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  • 1.
    Al-Mashhadi, Ammar Nadhom Farman
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dukic, Milena
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Rhabdomyomatous mesenchymal hamartoma presenting in a child as a perineal mass2019In: Journal of Pediatric Surgery Case Reports, ISSN 0022-3476, E-ISSN 2213-5766, Vol. 47, article id 101242Article in journal (Refereed)
    Abstract [en]

    Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare hamartomatous lesion in the dermis and subcutaneous tissue. It is mostly found in the face and neck region of children. We report a case of solitary RMH located in the perineum of an 8-month-old boy. Microscopic examination of specimen showed a disordered collection of mature adipose tissue, skeletal muscle, adnexal elements and nerve bundles, and immunohistochemistry confirmed a RMH. This case emphasizes the possibility of RMH in the perineum of the children. Even if RMH is a rare condition in the perineum it should be considered as a differential diagnosis of a perineal mass in children.

  • 2.
    Angsten, Gertrud
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Danielson, Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Kassa, Ann-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Lilja, Helene Engstrand
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Outcome of laparoscopic versus open gastrostomy in children2015In: Pediatric surgery international (Print), ISSN 0179-0358, E-ISSN 1437-9813, Vol. 31, no 11, p. 1067-1072Article in journal (Refereed)
    Abstract [en]

    Laparoscopic gastrostomy (LAPG) has gained popularity in children. The aim of this study was to compare the outcome of LAPG versus open gastrostomy (OG) in children with focus on complications, operative times and postoperative length of stay. Retrospective study of children who had gastrostomies inserted at our tertiary Pediatric Surgery Center from 2000 until 2013. The indications for a gastrostomy were an anticipated need for enteral support for at least 6 months. Totally 243 children were included in the study, 83 with LAPG and 160 with OG. We found a significant difference in postoperative length of stay, 3 days in the LAPG group versus 4 days in the OG group but no difference in a sub-group analysis from 2010 to 2013 when both techniques were used. There was no difference in median operative time or complications rates. Granuloma was the dominating complication in both groups. These two feeding-access techniques are comparable regarding complications, operative times and postoperative length of stay. The choice of surgical method should be individualized based on the patient's characteristics and the experience of the surgeon. The favorable results with LAPG in adults are not necessarily transferable to children since there are physiological and anatomical differences.

  • 3.
    Angsten, Gertrud
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Finkel, Yigael
    Lucas, Steven
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Pediatrics.
    Kassa, Ann-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Paulsson, Mattias
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Improved outcome in neonatal short bowel syndrome using parenteral fish oil in combination With ω-6/9 Lipid Emulsions2012In: JPEN - Journal of Parenteral and Enteral Nutrition, ISSN 0148-6071, E-ISSN 1941-2444, Vol. 36, no 5, p. 587-595Article in journal (Refereed)
    Abstract [en]

    Background:

    Newborn infants with short bowel syndrome (SBS) represent a high risk group of developing intestinal failure-associated liver disease (IFALD) which may be fatal. However, infants have a great capacity for intestinal growth and adaptation if IFALD can be prevented or reversed. A major contributing factor to IFALD may be the soybean oil-based intravenous lipid emulsions used since the introduction of parenteral nutrition (PN) 40 years ago.

    Methods:

    This retrospective study compares the outcome in 20 neonates with SBS treated with parenteral fish oil (Omegaven) in combination with omega-6/9 lipid emulsions (ClinOleic) with the outcome in a historical cohort of 18 patients with SBS who received a soybean oil-based intravenous lipid emulsion (Intralipid).

    Results:

    Median gestational age was 26 weeks in the treatment group and 35.5 weeks in the historical group. All patients were started on PN containing Intralipid that was switched to ClinOleic/Omegaven in the treatment group at a median age of 39 gestational weeks. In the treatment group, direct bilirubin levels were reversed in all 14 survivors with cholestasis (direct bilirubin >50 umol/). Median time to reversal was 2.9 months. Only 2 patients died of liver failure (10%).  In the historical cohort, 6 patients (33%) died of liver failure and only 2 patients showed normalization of bilirubin levels.

    Conclusions:

    Parenteral fish oil in combination with omega-6/9 lipid emulsions was associated with improved outcome in premature neonates with SBS. When used instead of traditional soybean-based emulsions, this mixed lipid emulsion may facilitate intestinal adaptation by increasing the IFALD-free period.

  • 4.
    Donoso, Felipe
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Engstrand Lilja, Heléne
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Risk Factors for Anastomotic Strictures after Esophageal Atresia Repair: Prophylactic Proton Pump Inhibitors Do Not Reduce the Incidence of Strictures2017In: European journal of pediatric surgery, ISSN 0939-7248, E-ISSN 1439-359X, Vol. 27, no 1, p. 50-55Article in journal (Refereed)
    Abstract [en]

    Background: Since 2005, infants with esophageal atresia (EA) in our unit are given prophylactic proton pump inhibitors (PPI) after repair until 1 year of age. The aims of this study were to identify risk factors for anastomotic strictures (AS) and to assess the efficacy of postoperative PPI prophylaxis in reducing the incidence of AS compared with symptomatic PPI. Methods Patients who underwent EA repair from 1994 to 2013 in our unit were included in this retrospective observational study approved by the local ethics review board. They were divided into two subgroups; symptomatic PPI-group with EA repair from 1994 to 2004 and prophylactic PPI-group with EA repair from 2005 to 2013. Data were collected from the patient records. Potential risk factors for AS analyzed were gender, long gap EA, birth weight, premature birth (< 37 gestational weeks), anastomotic tension, and anastomotic leakage. Number of dilatations until the age of 1 and 5 years were recorded. To evaluate risk factors for AS and the effect of prophylactic PPI Logistic, Cox and Poisson regression models were used. For descriptive statistics Fisher exact test and Wilcoxon rank sum test were used. Results A total of 128 patients were included. Patient characteristics, surgical method, grading of anastomotic tension, complications, and survival rates did not differ significantly between the symptomatic PPI-group (n = 71) and the prophylactic PPI-group (n = 57). Comparing the symptomatic and prophylactic PPI-group, there was no significant difference in the median age at the first AS (9.3 vs 6 mo), the number of dilatations until 1 year (2 vs 2) and 5 years (5 vs 4), or the incidence of anastomotic stricture (56.5% vs 50.9%). Long gap EA, high birth weight, and anastomotic tension were found to be independent risk factors. Conclusion Surgeons should aim to perform anastomosis under less tension at EA repair. Prophylactic PPI-treatment does not appear to reduce the rate of AS. Randomized controlled trials with larger study populations are needed to further evaluate the efficacy of prophylactic PPI.

  • 5.
    Donoso, Felipe
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Kassa, Ann-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Gustafson, Elisabet K.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Meurling, Staffan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Outcome and management in infants with esophageal atresia: a single centre observational study2016In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 51, no 9, p. 1421-1425Article in journal (Refereed)
    Abstract [en]

    Background/Purpose: A successful outcome in the repair of esophageal atresia (EA) is associated with a high quality pediatric surgical centre, however there are several controversies regarding the optimal management. The aim of this study was to investigate the outcome and management EA in a single pediatric surgical centre.

    Methods: Medical records of infants with repaired EA from 1994 to 2013 were reviewed.

    Results: 129 infants were included. Median follow-up was 5.3 (range 0.1-21) years. Overall survival was 94.6%, incidences of anastomotic leakage 7.0%, recurrent fistula 4.6% and anastomotic stricture 53.5% (36.2% within first year). In long gap EA (n = 13), delayed primary anastomosis was performed in 9 (69.2%), gastric tube in 3 (23.1%) and gastric transposition in one (7.7%) infants. The incidences of anastomotic leakage and stricture in long gap EA were, 23.1% and 69.2%, respectively. Peroperative tracheobronchoscopy and postoperative esophagography were implemented as a routine during the study-period, but chest drains were routinely abandoned.

    Conclusion: The outcome in this study is fully comparable with recent international reports showing a low mortality but a significant morbidity, especially considering anastomotic strictures and LGEA. Multicenter EA registry with long-term follow up may help to establish best management of EA.

  • 6.
    Engstrand Lilja, Helene
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Wefer, Hugo
    Karolinska Inst, Dept Microbiol Tumor & Cell Biol, S-17177 Stockholm, Sweden.;Karolinska Inst, Sci Life Lab, S-17177 Stockholm, Sweden..
    Nyström, Niklas
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Finkel, Yigael
    Karolinska Inst, Dept Clin Sci & Educ, S-11883 Stockholm, Sweden.;Sachs Childrens & Youth Hosp, S-11883 Stockholm, Sweden..
    Engstrand, Lars
    Karolinska Inst, Dept Microbiol Tumor & Cell Biol, S-17177 Stockholm, Sweden.;Karolinska Inst, Sci Life Lab, S-17177 Stockholm, Sweden.;Sci Life Lab, Clin Genom Facil, S-17165 Solna, Sweden..
    Intestinal dysbiosis in children with short bowel syndrome is associated with impaired outcome2015In: Microbiome, ISSN 0026-2633, E-ISSN 2049-2618, Vol. 3, article id UNSP 18Article in journal (Refereed)
    Abstract [en]

    Background: The composition of the intestinal microbiota seems to be an important factor in determining the clinical outcome in children with short bowel syndrome (SBS). Alterations in the microbiota may result in serious complications such as small bowel bacterial overgrowth (SBBO) and intestinal mucosal inflammation that lead to prolonged parenteral nutrition (PN) dependency with subsequently increased risk of liver failure and sepsis. To date, there are no reported mappings of the intestinal microbiome in children with SBS. Here, we present the first report on the intestinal microbial community profile in children with SBS. Findings: The study includes children diagnosed with SBS in the neonatal period. Healthy siblings served as controls. Fecal samples were collected, and microbial profiles were analyzed by using 16S rRNA gene sequencing on the Illumina MiSeq platform. We observed a pronounced microbial dysbiosis in children with SBS on PN treatment with an increased and totally dominating relative abundance of Enterobacteriacae in four out of five children compared to children with SBS weaned from PN and healthy siblings. Conclusions: The overall decreased bacterial diversity in children with SBS is consistent with intestinal microbiome mappings in inflammatory bowel diseases such as Crohn's disease and necrotizing enterocolitis in preterm infants. Our findings indicate that intestinal dysbiosis in children with SBS is associated with prolonged PN dependency.

  • 7.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology.
    Christofferson, Rolf H.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Carlsson, Per-Ola
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Transplantation and regenerative medicine.
    Lilja, Helene Engstrand
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Locally increased concentrations of inflammatory cytokines in an experimental intraabdominal adhesion model2014In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 49, no 10, p. 1480-1484Article in journal (Refereed)
    Abstract [en]

    Background: Peritoneal adhesions may cause bowel obstruction, infertility, and pain. This study investigated cytokines, proteins and growth factors thought to promote formation of adhesions in an experimental intraabdominal adhesion model. Methods: Male Sprague-Dawley rats were subjected to laparotomy, cecal abrasion, and construction of a small bowel anastomosis and examined at various time points after surgery. Concentrations of cytokines and growth factors in plasma and peritoneal fluid were analyzed using electrochemoluminescence and quantitative sandwich enzyme immunoassay technique. Results: Concentrations of interleukin-6 (IL-6), interleukin-1beta (IL-1 beta), and tumor necrosis factor alpha (TNF-alpha) increased in peritoneal fluid from 6 h after incision. Plasma concentrations of IL-6 increased at 6 h, but plasma concentrations of IL-1 beta and TNF-alpha remained low. Peritoneal fluid concentrations of platelet-derived growth factor-BB (PDGF- BB), transforming growth factor beta1 (TGF-beta 1), vascular endothelial growth factor (VEGF), tissue-type plasminogen activator (tPA) and plasminogen activator inhibitor-1 (PAI-1) were below detection levels at all time points. Conclusion: Early elevations of IL-6, IL-1 beta, and TNF-alpha concentrations in peritoneal fluid correlated to adhesion formation in this rodent model. Our model is relevant and reproducible, suitable for intervention, and indicates that antiadhesion strategies should be early, local and not systemic.

  • 8.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Christofferson, Rolf H.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Lilja, Helene Engstrand
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Adhesive small bowel obstruction after laparotomy during infancy2016In: British Journal of Surgery, ISSN 0007-1323, E-ISSN 1365-2168, Vol. 103, no 3, p. 284-289Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Intra-abdominal adhesions can cause adhesive small bowel obstruction, chronic abdominal pain and female infertility. Reports on long-term outcomes following laparotomy during infancy are scarce. The aims of this study were to investigate the incidence of and risk factors for long-term adhesive small bowel obstruction and associated morbidity after laparotomy during infancy.

    METHODS: Infants who underwent laparotomy between 1976 and 2011 were identified. Data were extracted from medical records and a questionnaire was sent to the patients.

    RESULTS: Some 898 of 1185 eligible patients were included, with a median follow-up time of 14·7 (range 0·0-36·0) years. Median age at first laparotomy was 6 (range 1·0-365·0) days. There were 113 patients (12·6 per cent) with adhesive small bowel obstruction who underwent relaparotomy, 79 (69·9 per cent) occurring during the first 2 years after the initial laparotomy. The highest incidence of small bowel obstruction was found in patients with Hirschsprung's disease (19 of 65, 29 per cent), malrotation (13 of 45, 29 per cent), intestinal atresia (11 of 40, 28 per cent) and necrotizing enterocolitis (16 of 64, 25 per cent). Lengthy duration of surgery (hazard ratio (HR) 1·25, 95 per cent c.i. 1·07 to 1·45), stoma formation (HR 1·72, 1·15 to 2·56) and postoperative complications (HR 1·81, 1·12 to 2·92) were independent risk factors. Chronic abdominal pain was reported in 180 (24·0 per cent) of 750 patients, and 17 (13·8 per cent) of 123 women reported infertility.

    CONCLUSION: The incidence of adhesive small bowel obstruction after laparotomy in infants is high.

  • 9.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Survival rates for surgically treated necrotising enterocolitis have improved over the last four decades2019In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 9, p. 1603-1608Article in journal (Refereed)
    Abstract [en]

    Aim Improved survival rates for premature infants have also increased the population at risk of necrotising enterocolitis (NEC). This study evaluated the outcomes of surgically treated NEC and identified risk factors for mortality, intestinal failure (IF) and IF associated liver disease (IFALD). Methods This was a retrospective observational study of 131 infants with surgically treated NEC from 1976 to 2016 in a Swedish tertiary referral centre: 20 in 1976-1996, 33 in 1997-2006 and 78 in 2007-2016. Data were extracted from medical records, and the Cox regression model was used to identify risk factors. Results When the first and last periods were compared, they showed decreases in both gestational age, from 30 to 26 weeks, and mortality rates, from 45% to 29%. IF was found in 67 patients (56%), IFALD in 41 patients (34%) and short bowel syndrome (SBS) in 13 (19%). The incidence of IF was high, even in infants without SBS. Low gestational age was an independent risk factor for mortality. No risk factors were identified for IF or IFALD. Conclusion Survival rates for NEC improved from 1976-2016, despite a decrease in gestational age. Clinicians should be particularly aware of the risk of infants without SBS developing IF.

  • 10.
    Fredriksson, Fanny
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Sellberg, Felix
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Bowden, Tim
    Uppsala University, Disciplinary Domain of Science and Technology, Chemistry, Department of Chemistry - Ångström, Polymer Chemistry.
    Engstrand, T.
    Karolinska Univ Hosp, Dept Reconstruct Plast Surg, SE-17176 Stockholm, Sweden.;Karolinska Inst, SE-17176 Stockholm, Sweden..
    Berglund, David
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Sutures impregnated with carbazate-activated polyvinyl alcohol reduce intraperitoneal adhesions2017In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 52, no 11, p. 1853-1858Article in journal (Refereed)
    Abstract [en]

    Background: Intraperitoneal adhesions cause significant morbidity. They occur after peritoneal trauma, which induces oxidative stress with production of inflammatory cytokines, peroxidized proteins (carbonyls) and lipids (aldehydes). This study aimed to investigate if carbazate-activated polyvinyl alcohol (PVAC), an aldehyde-carbonyl inhibitor, can reduce intraperitoneal adhesions in an experimental model.

    Material and methods: Male Sprague-Dawley rats (n = 110) underwent laparotomy, cecal abrasion and construction of a small bowel anastomosis. They either were treated with intraperitoneal instillation of PVAC or were sutured with PVAC-impregnated sutures. Thromboelastography analysis was performed using human blood and PVAC. The lipid peroxidation product malondialdehyde (MDA) and inflammatory cytokines IL-1 beta and IL-6 were quantified in peritoneal fluid. At day 7, bursting pressure of the anastomosis was measured and adhesions were blindly scored.

    Results: PVAC in human blood decreased the production of the fibrin-thrombocyte mesh without affecting the coagulation cascade. MDA, IL-1 beta and IL-6 were increased after 6 h without significant difference between the groups. PVAC-impregnated sutures reduced intraperitoneal adhesions compared to controls (p = 0.0406) while intraperitoneal instillation of PVAC had no effect. Anastomotic bursting pressure was unchanged.

    Conclusions: Intervention with an aldehyde-carbonyl inhibitor locally in the wound by PVAC-impregnated sutures might be a new strategy to reduce intraperitoneal adhesions.

  • 11.
    Hambraeus, Mette
    et al.
    Skane Univ Hosp, Dept Pediat Surg, Lund, Sweden;Lund Univ, Inst Clin Res, Lund, Sweden.
    Al-Mashhadi, Ammar
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Wester, Tomas
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Stockholm, Sweden.
    Svensson, Par-Johan
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Stockholm, Sweden.
    Stenstrom, Pernilla
    Skane Univ Hosp, Dept Pediat Surg, Lund, Sweden;Lund Univ, Inst Clin Res, Lund, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Functional outcome and health-related quality of life in patients with sacrococcygeal teratoma - a Swedish multicenter study2019In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 8, p. 1638-1643Article in journal (Refereed)
    Abstract [en]

    Background/Purpose: The aims of this study were to evaluate bowel and urinary tract function, to identify predictors for dysfunctional outcome and to evaluate health-related quality-of life (QoL) in patients treated for sacrococcygeal teratomas (SCT). Methods: Medical records of patients with SCT born between 1985 and 2015 treated at three Swedish pediatric surgical centers were reviewed. Questionnaires regarding urinary tract function, bowel function and QoL were sent to patients and parents. Different QoL instruments were used for the different age groups. Results: Totally 85 patients were identified. Four patients died in the neonatal period. Forty-nine patients answered the questionnaires (60%). Median age at follow-up was 8.9 years (range 3.6-28.8). Bowel dysfunction was reported by 36% and urinary tract dysfunction by 46% of the patients. Univariate analysis revealed that urinary tract dysfunction correlated with gestational age (p = 0.018) and immature histology (p = 0.008), and bowel dysfunction correlated with gestational age (p = 0.016) and tumor size (p = 0.042). Low gestational age was an independent predictor for both urinary tract and bowel dysfunction. Good or very good QoL was reported by 56% of children aged 4-7 years, 90% of children aged 8-17 years and 67% of the adults. Conclusion: Although a considerable proportion of bowel and urinary tract dysfunction was found, the reported QoL was good in a majority of the patients with SCT. Low gestational age was found to be a predictor for bowel-and urinary tract dysfunction. (C) 2018 Elsevier Inc. All rights reserved.

  • 12.
    Högberg, Niclas
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Carlsson, Per-Ola
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrinology, Diabetes and Metabolism.
    Hillered, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurosurgery.
    Stenbäck, Anders
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Intraluminal intestinal microdialysis detects markers of hypoxia and cell damage in experimental necrotizing enterocolitis2012In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 47, no 9, p. 1646-1651Article in journal (Refereed)
    Abstract [en]

    BACKGROUND/PURPOSE:

    Necrotizing enterocolitis (NEC) represents one of the gravest complications in premature infants and carries significant morbidity and mortality. There is a great need for improved diagnostic methods to reduce the severity and incidence of NEC. The aim of the study was to investigate if intraluminal microdialysis can detect intestinal ischemia in newborn rats with induced experimental NEC.

    METHODS:

    The studies were performed on 1-day-old Sprague-Dawley rat pups. Experimental NEC was induced using hypoxia/reoxygenation treatment. Microdialysis catheters were rectally inserted and placed in the rectosigmoid part of the colon. Microdialysate levels of glucose, lactate, pyruvate, and glycerol were measured. Intestinal specimens were collected at the end of the experiments for microscopic evaluation.

    RESULTS:

    Intraluminal microdialysis revealed signs of intestinal hypoxia and cellular damage, with a marked increase of lactate and glycerol. Microscopic evaluation confirmed intestinal damage in the NEC group.

    CONCLUSION:

    Intraluminal microdialysis can detect intestinal hypoxic stress and mucosal cell membrane decay in a rat model of NEC. Intestinal intraluminal microdialysis is easily accessible through the rectum and may be a useful noninvasive complement to other methods in the assessment of NEC.

  • 13.
    Högberg, Niclas
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Stenbäck, Anders
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Carlsson, Per-Ola
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Cell Biology. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Transplantation and regenerative medicine.
    Wanders, Alkwin
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Molecular and Morphological Pathology.
    Engstrand Lilja, Heléne
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Genes regulating tight junctions and cell adhesion are altered in early experimental necrotizing enterocolitis2013In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 48, no 11, p. 2308-2312Article in journal (Refereed)
    Abstract [en]

    Background/purpose:

    Necrotizing enterocolitis (NEC) represents one of the gravest complications in preterm infants and carries significant morbidity and mortality. Increased intestinal permeability may play an important role in the pathogenesis of NEC. In this study we investigated the genes regulating structural proteins such as tight junctions (TJ) and cell adhesion in a neonatal rat model of early NEC, as well as the expression of TJ proteins by immunohistochemistry staining.

    Methods:

    The studies were performed on Sprague-Dawley rat pups. Experimental NEC was induced using hypoxia/reoxygenation treatment on day 1 after birth. Intestinal specimens from the ileum were obtained, mRNA was purified and the transcriptome was analyzed using microarray. Immunohistochemistry staining was performed for TJ proteins.

    Results:

    We found several TJ genes such as claudins 1, 8, 14, 15 and gap junction protein to be affected. Immunohistochemistry staining for TJ protein claudin-1 revealed decreased levels in experimental NEC compared to controls. Alterations in genes involved in the inflammatory response was confirmed, along with several genes regulating proteins used as biomarkers for NEC.

    Conclusion:

    This study indicates that tight junctions and cell adhesion may play a critical role in the pathogenesis of early experimental NEC. Better understanding of the pathogenesis of NEC may lead to novel strategies for the prevention and treatment of NEC.

  • 14.
    Kadir, Darya
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden..
    Lilja, Helene Engstrand
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden..
    Risk factors for postoperative mortality in congenital diaphragmatic hernia- a single centre observational study2017In: Pediatric surgery international (Print), ISSN 0179-0358, E-ISSN 1437-9813, Vol. 33, no 3, p. 317-323Article in journal (Refereed)
    Abstract [en]

    The management of congenital diaphragmatic hernia (CDH) is a major challenge. The mortality is dependent on associated malformations, the severity of pulmonary hypoplasia, pulmonary hypertension and iatrogenic lung injury associated with aggressive mechanical ventilation. The aims of the study were to investigate the mortality over time in a single paediatric surgical centre, to compare the results with recent reports and to define the risk factors for mortality. The medical records of infants with CDH from two time periods: 1995-2005 and 2006-2016 were reviewed. Cox regression was used for statistical analysis. The study included 113 infants. The mortality rate was significantly decreased in the later time period, compared to the earlier, 4.4 and 17.9%, respectively. At the early time period five patients (7.5%) were treated with ECMO and in the later time period ECMO was used in three patients (6.5%). The mortality in ECMO-treated patients was 50% in both time periods. Prenatal diagnosis, intrathoracic liver, low Apgar score and low birth weight were defined as independent risk factors for mortality. Despite no significant differences in the incidence of independent risk factors and the use of ECMO between the two time periods, mortality decreased over time. The mortality was lower than previously reported. The results indicate that there are many important factors involved in a successful outcome after CDH repair. Large multicentre studies are necessary to define those critical factors and to determine optimal treatment strategies.

  • 15.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Akademiska sjukhuset, sektionen för barnkirurgi.
    Dahl, Margareta
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Strinnholm, Margareta
    Akademiska sjukhuset, Folke Bernadotte regionhabilitering.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Akademiska sjukhuset, sektionen för barnkirurgi.
    Attention difficulties and physical dysfunction common in children with complex congenital malformations:: a study of preschool children with VACTERL association.2018In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227Article in journal (Refereed)
    Abstract [en]

    AIM: Knowledge on the neurodevelopmental and physical function in children with vertebral defects, anorectal malformations, cardiac defects, tracheo-oesophageal fistula, renal and limb malformations (VACTERL) is scarce. We evaluated Swedish preschool children with VACTERL and identified whether they would need extra support in school.

    METHODS: From 2015 to 2017, we recruited children aged 5-7 with VACTERL association from the paediatric surgical centre at the University Children's Hospital at Uppsala. Neurodevelopmental function was assessed by age-appropriate intelligence and visual and auditory attention tests, and the children's behaviour and attention were observed by an experienced psychologist. Physical function was evaluated through parental interviews and examinations. Data on patient characteristics, including any surgery and anaesthesia, were extracted from medical records.

    RESULTS: Of the 13 eligible families, 10 agreed to participate. Intelligence was within the normal range for all children, but attention difficulties were found in eight of the children, requiring adjustments at school, and two of these were later diagnosed with attention deficit hyperactivity disorder. All children had physical dysfunctions that affected their daily nutrition, bowel or bladder functions.

    CONCLUSION: Attention difficulties and physical dysfunction were common in Swedish preschool children aged 5-7 with VACTERL and they would need support and adjustments when they started school.

  • 16.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Dellenmark-Blom, Michaela
    Insitutionen för kliniska vetenskaper, avd för pediatrik, Sahlgrenska akademin, Göteborgs universitet.
    Thorsell Cederberg, Jenny
    Institutionen för klinisk neurovetenskap, Karolinska Institutet, Stockholm.
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Public Health and Caring Sciences. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Children and adolescents with VACTERL association: health-related quality of life and psychological well-being in children and adolescents and their parentsIn: Article in journal (Refereed)
    Abstract [en]

    Purpose: VACTERL association is a rare and complex condition of congenital malformations, often requiring repeated surgery and entailing various physical sequelae. Due to scarcity of knowledge, the study aim was to investigate self-reported Health-Related Quality of Life (HRQoL), anxiety, depression and self-concept in children and adolescents with VACTERL association and self-reported anxiety and depression in their parents.

    Methods: Patients aged 8-17 years with VACTERL association and their parents were recruited from three of four Swedish paediatric surgical centres during 2015-2019. The well-established validated questionnaires DISABKIDS, Beck Youth Inventories, Beck Anxiety Inventory and Beck Depression Inventory were sent to the families. Data were analysed using descriptives, t-tests and multivariable analysis. Results were compared with norm groups and reference samples.

    Results: The questionnaires were returned by 40 patients, 38 mothers and 33 fathers. The mean HRQoL was M=80.4, comparable to children with asthma (M=80.2) and diabetes (M=79.5). Self-reported psychological well-being was comparable to the norm group of Swedish school children, and was significantly higher than a clinical sample. Factors negatively influencing children’s HRQoL and psychological well-being were identified. The parents’ self-reports of anxiety and depression were comparable to non-clinical samples.

    Conclusions: Although children and adolescents with VACTERL association reported similar HRQoL to those of European children with chronic conditions, their psychological well-being was comparable to Swedish school children in general. Nevertheless, some individuals among both children and parents were in need of extra support. This attained knowledge is valuable when counselling parents regarding the prognosis for children with VACTERL association.

  • 17.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Predictors of postnatal outcome in neonates with gastroschisis2011In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 46, no 11, p. 2108-2114Article in journal (Refereed)
    Abstract [en]

    Background/Purpose: The optimal management of neonates with gastroschisis is unclear and there is a significant morbidity. We performed a review of neonates with gastroschisis treated at our center of pediatric surgery over the last 21-years in order to determine predictive factors of outcome.

    Methods: Single-center retrospective analysis of 79 neonates with gastroschisis (1989 to 2009).  Length of hospital stay (LOS), days of parenteral nutrition (PN) and survival were outcome measures. Univariate and multiple regression analyses were used.

    Results: Overall survival was 92 % and primary closure was achieved in 80 %. Median LOS was 25 days and median duration on PN 17 days. Intestinal atresia, closed gastroschisis, secondary closure and sepsis were the primary variables associated with poor outcome independent of other variables, but prematurity also had an effect on outcome. Route of delivery and associated malformations were not related to poorer outcome. Necrotizing enterocolitis (NEC) did not occur in any of our patients.

    Conclusion: Outcome in our patients was favorable as measured by survival, LOS, and days on PN. Primary predictors of poor outcome were factors related to short bowel syndrome (SBS) and secondary closure, indicating a need to further improve treatment of SBS.

  • 18.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Pediat Surg, Uppsala, Sweden.
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    From crisis to self-confidence and adaptation; Experiences of being a parent of a child with VACTERL association: A complex congenital malformation2019In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 14, no 4, article id e0215751Article in journal (Refereed)
    Abstract [en]

    Aim Knowledge is scarce regarding mothers' and fathers' experiences of being a parent of a child with VACTERL association-a complex malformation. The aim of the study was to describe experiences of being a parent of a child with VACTERL association. Method Semi-structured interviews were performed with ten mothers and nine fathers face-to-face or by telephone and analyzed by using Qualitative content analysis. Results The parents described crisis reactions at the discovery of malformations in their child. Involvement in care was reported from the initial hospital admission until actively taking responsibility for treatments at home. Eventually the health condition became an integrated part of everyday life. The parents expressed the importance of meeting other families with a child with VACTERL. Descriptions were given of more or less professionalism with perceived discrepancies of knowledge and experience between the healthcare professionals in the tertiary hospital and those in the local hospital. Difficulties in receiving medical support during the initial period at home were described. Furthermore, emotional support and practical arrangements regarding parental accommodation and transportation varied. Conclusion Being a parent of a child with VACTERL association involves crisis, mixed emotional reactions and shared responsibility for the child ' s treatment and care with the professional care providers. Psychological processing, good medical care and support from experts, and peer support from other parents is essential in the parents' struggle to reach self-confidence and adaptation. A care plan with individualized tailored care for each child including a training and support plan for the parents is warranted. To reduce the described discrepancies in knowledge and experience between the local and tertiary hospital, video sessions with the parents and responsible professionals at the local and tertiary hospital could be an appropriate mode of transferring information at discharge and follow up of the child.

  • 19.
    Kassa, Ann-Marie
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Paediat Surg, SE-75185 Uppsala, Sweden..
    Engvall, Gunn
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Neuropediatrics/Paediatric oncology.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery. Univ Childrens Hosp, Dept Paediat Surg, SE-75185 Uppsala, Sweden..
    Young children with severe congenital malformations (VACTERL) expressed mixed feelings about their condition and worries about needles and anaesthesia2017In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 106, no 10, p. 1694-1701Article in journal (Refereed)
    Abstract [en]

    Aim: Our knowledge of the perceptions that children with severe congenital malformations have of their health, treatment and how to improve hospital care is limited. This study focused on patients with vertebral defects, anal atresia, cardiac defects, tracheo-oesophageal fistula, renal anomalies and limb abnormalities (VACTERL).

    Methods: We interviewed 10 children aged five to eight years with VACTERL association who were treated in a Swedish tertiary paediatric surgical centre, using a computer-assisted technique called In My Shoes. The interviews were analysed by qualitative content analysis.

    Results: The children described their awareness of their health history and said they felt proud but different due to their physical dysfunction. They were happy to visit the hospital to meet familiar staff, but expressed negative feelings about missing normal life. They were afraid of needle-related procedures and not wakening up after anaesthesia. Various ways of coping with difficult situations were expressed, and suggestions to improve hospital care were voiced.

    Conclusion: Careful follow-up of these children by multidisciplinary teams is crucial to optimise their health and functional status. Fear of medical procedures may be reduced by carefully delivered information, listening to the children, providing continuity of care and creating individual care strategies.

  • 20.
    Markasz, Laszlo
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Perinatal, Neonatal and Pediatric Cardiology Research.
    Wanders, Alkwin
    Umea Univ, Dept Biomed Sci, Umea, Sweden.
    Szekely, Laszlo
    Karolinska Inst, Dept Lab Med, Div Pathol, Stockholm, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Diminished DEFA6 Expression in Paneth Cells Is Associated with Necrotizing Enterocolitis2018In: Gastroenterology Research and Practice, ISSN 1687-6121, E-ISSN 1687-630X, article id 7345426Article in journal (Refereed)
    Abstract [en]

    Background. Necrotizing enterocolitis (NEC) is the most common gastrointestinal disorder in premature infants with a high morbidity and mortality. Paneth cell dysfunction has been suggested to be involved in the pathogenesis of NEC. Defensin alpha-6 (DEFA6) is a specific marker for Paneth cells acting as part of the innate immunity in the human intestines. The aim of this study was to investigate the expression of DEFA6 in infants with NEC. Materials and Methods. Infants who underwent bowel resection for NEC at level III NICU in Sweden between August 2004 and September 2013 were eligible for the study. Macroscopically vital tissues were selected for histopathological evaluation. All infants in the control group underwent laparotomy and had ileostomy due to dysmotility, and samples were taken from the site of the stoma. DEFA6 expression was studied by immunohistochemistry. Digital image analysis was used for an objective and precise description of the samples. Results. A total of 12 infants were included in the study, eight with NEC and four controls. The tissue samples were taken from the colon (n = 1), jejunum (n = 1), and ileum (n = 10). Both the NEC and control groups consisted of extremely premature and term infants (control group: 25-40 gestational weeks, NEC group: 23-39 gestational weeks). The postnatal age at the time of surgery varied in both groups (control group: 4-47 days, NEC group: 4-50 days). DEFA6 expression in the NEC group was significantly lower than that in the control group and did not correlate with gestational age. Conclusion. The diminished DEFA6 expression in Paneth cells associated with NEC in this study supports the hypothesis that alpha-defensins are involved in the pathophysiology of NEC. Future studies are needed to elucidate the role of alpha-defensins in NEC aiming at finding preventive and therapeutic strategies against NEC.

  • 21.
    Naji, Hussein
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Zetterlind, Liselotte
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Gustafson, Elisabet
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Lindblad, Kerstin
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Christofferson, Rolf
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Vet doktorn hur en barnrumpa ser ut?: Sent upptäckta anorektala missbildningar hos barn2011In: Läkartidningen, ISSN 0023-7205, E-ISSN 1652-7518, Vol. 108, no 46, p. 2380-2381Article in journal (Refereed)
    Abstract [sv]

    Av de 28 senaste barnen med anorektala missbildningar på vår klinik hade tolv fistel till perineum, analmembran eller fistel till vestibulum vaginae. Av dem upptäcktes fem sent – upp till sju månader efter födelsen.

    Tre av dessa fem hade uttalade förstoppningsbesvär med krystbeteende, och föräldrarna hade påpekat att anus hade avvikande utseende.

    Hos två av barnen visade utredning missbildningar i fler organ.

  • 22.
    Normann, Erik
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Fahlen, Annika
    Department of Microbiology, Tumor and Cell Biology, Karolinska institute, Stockholm, Sweden.
    Engstrand, Lars
    Department of Microbiology, Tumor and Cell Biology, Karolinska institute, Stockholm, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health.
    Intestinal microbial profiles in extremely preterm infants with and without necrotizing enterocolitis2013In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 102, no 2, p. 129-136Article in journal (Refereed)
    Abstract [en]

    Aim Necrotizing enterocolitis (NEC) represents one of the gravest complications in premature infants. The suggested role of intestinal microbiota in the development of NEC needs to be elucidated. Methods This prospective single-centre casecontrol study applied barcoded pyrosequencing to map the bacterial composition of faecal samples from extremely preterm infants. Ten patients were diagnosed with NEC and matched to healthy controls with regard to sex, gestational age and mode of delivery prior to analysis of the samples. Results Enterococcus, Bacillales and Enterobacteriaceae dominated the flora. Although not statistically significant, a high relative abundance of Bacillales and Enterobacteriaceae was detected at early time points in patients developing NEC, while healthy controls had a microbiota more dominated by Enterococcus. A low diversity of intestinal microbial flora was found without any differences between NEC patients and controls. In 16 healthy controls, Firmicutes (Enterococcus and Bacillales) dominated the faecal flora during the first weeks after birth and were then succeeded by Enterobacteriaceae. Conclusion No significant differences in the composition of intestinal microbiota of patients developing NEC were detected; however, some findings need to be scrutinized in subsequent studies.

  • 23.
    Pammi, Mohan
    et al.
    Baylor Coll Med, Dept Pediat, Sect Neonatol, Houston, TX 77030 USA.;Texas Childrens Hosp, Houston, TX 77030 USA..
    Cope, Julia
    Baylor Coll Med, Alkek Ctr Metagen & Microbiome Res, Houston, TX 77030 USA..
    Tarr, Phillip I.
    Washington Univ, Dept Pediat, St Louis Sch Med, St Louis, MO 63130 USA..
    Warner, Barbara B.
    Washington Univ, Dept Pediat, St Louis Sch Med, St Louis, MO 63130 USA..
    Morrow, Ardythe L.
    Univ Cincinnati, Cincinnati Childrens Hosp Med Ctr, Coll Med, Perinatal Inst,Dept Pediat, Cincinnati, OH 45221 USA..
    Mai, Volker
    Univ Florida, Coll Publ Hlth & Hlth Profess, Dept Epidemiol, Gainesville, FL USA.;Univ Florida, Coll Med, Gainesville, FL USA.;Univ Florida, Emerging Pathogens Inst, Gainesville, FL USA..
    Gregory, Katherine E.
    Brigham & Womens Hosp, Dept Newborn Med, Boston, MA 02115 USA..
    Kroll, J. Simon
    Imperial Coll London, Dept Med, Sect Pediat, London, England..
    McMurtry, Valerie
    Childrens Hosp, Dept Microbiol Immunol & Parasitol, New Orleans, LA USA..
    Ferris, Michael J.
    Childrens Hosp, Dept Microbiol Immunol & Parasitol, New Orleans, LA USA..
    Engstrand, Lars
    Karolinska Inst, Director Clin Genom, Stockholm, Sweden.;Karolinska Inst, Dept Microbiol Tumor & Cell Biol, Stockholm, Sweden..
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Hollister, Emily B.
    Texas Childrens Hosp, Texas Childrens Microbiome Ctr, Dept Pathol, Houston, TX 77030 USA.;Baylor Coll Med, Houston, TX 77030 USA..
    Versalovic, James
    Texas Childrens Hosp, Texas Childrens Microbiome Ctr, Dept Pathol, Houston, TX 77030 USA.;Baylor Coll Med, Houston, TX 77030 USA..
    Neu, Josef
    Baylor Coll Med, Dept Pediat, Sect Neonatol, Houston, TX 77030 USA.;Texas Childrens Hosp, Houston, TX 77030 USA..
    Intestinal dysbiosis in preterm infants preceding necrotizing enterocolitis: a systematic review and meta-analysis2017In: Microbiome, ISSN 0026-2633, E-ISSN 2049-2618, Vol. 5, article id 31Article, review/survey (Refereed)
    Abstract [en]

    Background: Necrotizing enterocolitis (NEC) is a catastrophic disease of preterm infants, and microbial dysbiosis has been implicated in its pathogenesis. Studies evaluating the microbiome in NEC and preterm infants lack power and have reported inconsistent results. Methods and results: Our objectives were to perform a systematic review and meta-analyses of stool microbiome profiles in preterm infants to discern and describe microbial dysbiosis prior to the onset of NEC and to explore heterogeneity among studies. We searched MEDLINE, PubMed, CINAHL, and conference abstracts from the proceedings of Pediatric Academic Societies and reference lists of relevant identified articles in April 2016. Studies comparing the intestinal microbiome in preterm infants who developed NEC to those of controls, using cultureindependent molecular techniques and reported a and beta-diversity metrics, and microbial profiles were included. In addition, 16S ribosomal ribonucleic acid (rRNA) sequence data with clinical meta-data were requested from the authors of included studies or searched in public data repositories. We reprocessed the 16S rRNA sequence data through a uniform analysis pipeline, which were then synthesized by meta-analysis. We included 14 studies in this review, and data from eight studies were available for quantitative synthesis (106 NEC cases, 278 controls, 2944 samples). The age of NEC onset was at a mean +/- SD of 30.1 +/- 2.4 weeks post-conception (n = 61). Fecal microbiome from preterm infants with NEC had increased relative abundances of Proteobacteria and decreased relative abundances of Firmicutes and Bacteroidetes prior to NEC onset. Alpha-or beta-diversity indices in preterm infants with NEC were not consistently different from controls, but we found differences in taxonomic profiles related to antibiotic exposure, formula feeding, and mode of delivery. Exploring heterogeneity revealed differences in microbial profiles by study and the target region of the 16S rRNA gene (V1-V3 or V3-V5). Conclusions: Microbial dysbiosis preceding NEC in preterm infants is characterized by increased relative abundances of Proteobacteria and decreased relative abundances of Firmicutes and Bacteroidetes. Microbiome optimization may provide a novel strategy for preventing NEC.

  • 24.
    Stadil, Tatjana
    et al.
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark.
    Koivusalo, Antti
    Univ Helsinki, Childrens Hosp, Dept Pediat Surg, Helsinki, Finland.
    Pakarinen, Mikko
    Univ Helsinki, Childrens Hosp, Dept Pediat Surg, Helsinki, Finland.
    Mikkelsen, Audun
    Oslo Univ Hosp, Rikshosp, Dept Gastr & Pediat Surg, Oslo, Norway;Oslo Univ Hosp, Ulleval, Dept Gastr & Pediat Surg, Oslo, Norway.
    Emblem, Ragnhild
    Oslo Univ Hosp, Rikshosp, Dept Gastr & Pediat Surg, Oslo, Norway;Oslo Univ Hosp, Ulleval, Dept Gastr & Pediat Surg, Oslo, Norway.
    Svensson, Jan F.
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Ehren, Henrik
    Karolinska Univ Hosp, Dept Pediat Surg, Stockholm, Sweden;Karolinska Inst, Dept Womens & Childrens Hlth, Stockholm, Sweden.
    Jonsson, Linus
    Queen Silvia Childrens Hosp, Dept Pediat Surg, Gothenburg, Sweden.
    Backstrand, Jakob
    Queen Silvia Childrens Hosp, Dept Pediat Surg, Gothenburg, Sweden.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Donoso, Felipe
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
    Thorup, Jorgen Mogens
    Copenhagen Univ Hosp, Rigshosp, Dept Pediat Surg, Copenhagen, Denmark.
    Saeter, Thorstein
    Trondhe Univ Hosp, St Olavs Hosp, Dept Pediat Surg, Trondheim, Norway.
    Rasmussen, Lars
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark.
    Pedersen, Rikke Neess
    Odense Univ Hosp, Hans Christian Andersen Childrens Hosp, Odense, Denmark.
    Stenstrom, Pernilla
    Lund Univ, Childrens Hosp, Dept Pediat, Lund, Sweden.
    Arnbjornsson, Einar
    Lund Univ, Childrens Hosp, Dept Pediat, Lund, Sweden.
    Oskarsson, Kristjan
    Univ Hosp, Childrens Hosp, Reykjavik, Iceland.
    Qvist, Niels
    Odense Univ Hosp, Surg Dept A, Sdr Blvd 29, DK-5000 Odense C, Denmark;Odense Univ Hosp, Hans Christian Andersen Childrens Hosp, Odense, Denmark;Odense Univ Hosp, Odense Patient Data Explorat Network, OPEN, Odense, Denmark.
    Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries2019In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 54, no 3, p. 423-428Article in journal (Refereed)
    Abstract [en]

    Background:

    Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year.

    Methods:

    Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life.

    Results:

    We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043).

    Conclusion:

    DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated.

    Type of study:

    Treatment study.

  • 25. Stenström, Pernilla
    et al.
    Brautigam, Matilda
    Borg, Helena
    Graneli, Christina
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Wester, Tomas
    Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.2017In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 52, no 8, p. 1302-1307Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years.

    METHODS: This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition.

    RESULTS: Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences.

    CONCLUSION: One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.

  • 26. Wester, T.
    et al.
    Borg, H.
    Naji, H.
    Stenstrom, P.
    Westbacke, G.
    Lilja, Helene Engstrand
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Serial transverse enteroplasty to facilitate enteral autonomy in selected children with short bowel syndrome2014In: British Journal of Surgery, ISSN 0007-1323, E-ISSN 1365-2168, Vol. 101, no 10, p. 1329-1333Article in journal (Refereed)
    Abstract [en]

    Background: Serial transverse enteroplasty (STEP) was first described in 2003 as a method for lengthening and tapering of the bowel in short bowel syndrome. The aim of this multicentre study was to review the outcome of a Swedish cohort of children who underwent STEP. Methods: All children who had a STEP procedure at one of the four centres of paediatric surgery in Sweden between September 2005 and January 2013 were included in this observational cohort study. Demographic details, and data from the time of STEP and at follow-up were collected from the case records and analysed. Results: Twelve patients had a total of 16 STEP procedures; four children underwent a second STEP. The first STEP was performed at a median age of 5 8 (range 0.9-19.0) months. There was no death at a median follow-up of 37.2 (range 3.0-87.5) months and no child had small bowel transplantation. Seven of the 12 children were weaned from parenteral nutrition at a median of 19.5 (range 2.3-42.9) months after STEP. Conclusion: STEP is a useful procedure for selected patients with short bowel syndrome and seems to facilitate weaning from parenteral nutrition. At mid-term follow-up a majority of the children had achieved enteral autonomy. The study is limited by the small sample size and lack of a control group.

  • 27.
    Wester, Tomas
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Barnkirurgi2016In: Kirurgi, Studentlitteratur AB, 2016, 4:1, , p. 717-736p. 717-738Chapter in book (Other (popular science, discussion, etc.))
  • 28.
    Wester, Tomas
    et al.
    Karolinska Univ Hosp, Dept Womens & Childrens Hlth, Unit Pediat Surg, Stockholm, Sweden.; Karolinska Inst, Stockholm, Sweden..
    Engstrand Lilja, Helene
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
    Stenström, Pernilla
    Skane Univ Hosp, Dept Pediat Surg, Lund, Sweden..
    Pakarinen, Mikko
    Helsinki Univ Hosp, Childrens Hosp, Pediat Liver & Gut Res Grp, Pediat Surg, Helsinki, Finland..
    Absent ileocecal valve predicts the need for repeated STEP in children2017In: Surgery, ISSN 0039-6060, E-ISSN 1532-7361, Vol. 161, no 3, p. 818-822Article in journal (Refereed)
    Abstract [en]

    Background. Serial transverse enteroplasty facilitates weaning from parenteral support in selected patients with short bowel syndrome, although repeated procedure is frequently required. Our aim was to evaluate the outcome of a series of patients after serial transverse enteroplasty and define predictors of repeated serial transverse enteroplasty and weaning off parenteral support. Methods. All children who underwent serial transverse enteroplasty at 4 Nordic pediatric surgery centers from 2004-2015 were included in this observational study. Data were collected from the patient records. The study was approved by the local ethics review boards. Results. Twenty-seven children with short bowel with initial median small bowel length of 26 cm (range, 10-100 cm) were included. Eleven patients had the ileocecal valve remaining. Serial transverse enteroplasty was performed at median age of 7.5 months (range, 0.9-224 months). Serial transverse enteroplasty made the small bowel 46% (0-233%) longer. Eleven patients (41 %) underwent a repeated serial transverse enteroplasty 12 months (1.0-72 months) later; 7 patients required additional operative procedures, but none were transplanted. At follow-up, 45.1 months (1.8-126 months) after the first serial transverse enteroplasty, 11 (41 %) patients needed parenteral support. The remaining 16 patients had been weaned off parenteral support. One patient had died. Absence of the ileocecal valve was the only factor, which predicted the need for a repeated serial transverse enteroplasty (odds ratio 16.7, 95 % confidence interval, 1.7-164.8, P =.007). No factor was identified predicting need for parenteral support at follow-up. Conclusion. A majority of children with short bowel syndrome can be weaned from parenteral support after serial transverse enteroplasty. The absence of the ileocecal valve predicts the need for a repeated serial transverse enteroplasty, which was required by 40% of the patients.

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