Collagenous gastritis in children: A national cohort.

BACKGROUND: Collagen gastritis is a rare disease that manifests in children mainly as isolated gastric involvement associated with martial deficiency anemia. There are no recommendations for the management and follow-up of these patients. We aimed to describe the clinical data, endoscopic findings, and treatments deployed in France's children with collagenous gastritis. METHODS: All French pediatric gastroenterology centers and pediatric centers for rare digestive diseases (Centres de Maladies Rares Digestives) were contacted to collect cases of collagenous gastritis, defined on gastric biopsies and diagnosed before 18 years of age. RESULTS: A total of 12 cases diagnosed (4 males and 8 females) between 1995 and 2022 could be analyzed. The median age at diagnosis was 12.5 years (7-15.2). The most frequent clinical presentation was abdominal pain (6/11) and/or nonspecific symptomatology attributed to anemia (8/10). Anemia was present in all children (11/11; Hb 2.8-9.1 g/dL). Nodular gastritis was present in 10 patients (antrum: 2; fundus: 4; in antrum and fundus: 4). All patients had a basement membrane thickening (from 19 to 100 µm). The treatments received were PPI (11), oral or intravenous martial supplementation (12), budesonide (1), and prednisone (1). Martial supplementation improved anemia in all cases. At discontinuation, nine of 10 patients had a recurrence of anemia. CONCLUSION: Collagenous gastritis is an exceptional condition, clinically manifested in children as abdominal pain and iron deficiency anemia probably of hemorrhagic origin. Patients require long-term follow-up and monitoring of their disease to describe the risk of progression better.

Gastrostomy for infants with severe epidermolysis bullosa simplex in neonatal intensive care.

INTRODUCTION: Severe epidermolysis bullosa simplex (EBS sev) is a rare genodermatosis characterized by congenital generalized blistering and mucosal involvement. Increased needs and decreased intake quickly lead to nutritional imbalance. Enteral nutrition support is proposed, but classical nasogastric tubes are not well tolerated in these patients and gastrostomy is preferred. OBJECTIVE AND METHODS: To report the experience with EBS sev in neonatal units of French reference centers for gastrostomy. In this retrospective multicentric study, we included all patients with EBS sev who had gastrostomy placement before age 9 months during neonatal care hospitalization. RESULTS: Nine infants (5 males/4 females) with severe skin and mucosal involvement were included. A gastrostomy was decided, at an early age (mean 3.7 months, range 1.4 to 8 months) in infants with mean weight 4426 g (range 3500 to 6000 g). Techniques used were endoscopy with the pull technique for 5 infants and surgery under general anesthesia for 4. Main complications were local but resolved after treatment. All infants gained weight after gastrostomy. The mean withdrawal time (n = 7) for the gastrostomy was 35.8 months (range 10.5 months to 6.5 years). Seven children had persistent oral disorders. CONCLUSIONS: Gastrostomy in infants with EBS sev can be necessary in neonatal intensive care units. Both surgical and endoscopic pull techniques seem efficient, with good tolerance.

[Knowledge of adolescents with inflammatory bowel disease: Results of a multicenter cross-sectional survey]. / Connaissances des adolescents atteints de maladie inflammatoire chronique intestinale : résultats d'une enquête transversale multicentrique.

AIM: To assess knowledge acquired by adolescents about their inflammatory bowel disease (IBD). METHODS: An anonymous questionnaire was given during consultation to adolescents followed for IBD by pediatricians from 13 hospitals between 1 September 2012 and 1 July 2013. After parental consent, these physicians completed a form at the inclusion of each patient, in which the characteristics of IBD were detailed. The patients mailed back their questionnaire. RESULTS: A total of 124 patients from 12 to 19 years of age were included with a response rate of 82% (all anonymous); 23% of the patients thought that diet was a possible cause of IBD and 22% that one of the targets of their treatment was to cure their disease for good. Of the patients reported having Crohn disease, 46% knew the anoperineal location and 14% knew that Crohn disease can affect the entire digestive tract. Twenty-five percent of the patients were able to name one side effect of azathioprine (88% had already received this treatment), 24% were able to name one side effect of infliximab (54% had already received this treatment), 70% of the adolescents knew that smoking worsens Crohn disease, 68% declared they had learned about their IBD from their pediatrician, and 81% said they would like to receive more information. CONCLUSION: Adolescents with IBD have gaps in their general knowledge and the different treatments of their disease. Their main source of information is their pediatrician, warranting the implementation of customized patient education sessions.

[Congenital portosystemic shunts: experience of the western region of France over 5 years]. / Shunts porto-systémiques congénitaux : expérience du Grand-Ouest sur cinq ans.

INTRODUCTION: Congenital portosystemic shunts (CPS) are rare vascular malformations that lead to liver metabolic modifications. The aim of this study was to describe the management and outcome of CPS in children who were followed in the western region of France and to compare our experience to a literature review. PATIENTS AND METHODS: This retrospective study reports nine children with CPS who were followed in the western region of France between 2008 and 2013. RESULTS: Among these nine children, six were detected by prenatal ultrasonography, one had cholestatic jaundice during the postoperative period, one had cholestatic jaundice during the first days of life, and one had dyspnea and fatigue. Two children had liver tumors. The CPS was intrahepatic for four children and extrahepatic for two children. Three others had a patent ductus venosus. Among the intrahepatic shunts that were discovered before the age of 2 years, three closed spontaneously. The five other shunts were closed surgically or by interventional radiology techniques, after angiography for four children. After closure of eight shunts, the outcome was favorable for six children. Two children presented complications after surgery: portal hypertension and portal thrombus. CONCLUSION: When CPS is suspected, the diagnosis should be confirmed and complications should be investigated. Shunt closure restores portal flow and prevents complications.

Factors influencing outcome after intestinal transplantation in children.

We evaluated 131 patients (6 months-14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n=12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx.

Fecal tumor necrosis factor-alpha and calprotectin as differential diagnostic markers for severe diarrhea of small infants.

OBJECTIVE: The etiology of early-onset diarrhea of neonates and small infants that persists despite bowel rest is heterogeneous. Two different categories of disorders presenting with diarrhea in the first weeks of life can be distinguished: constitutive intestinal epithelial disorders (microvillus atrophy [MVA] or epithelial dysplasia [ED]) and immune-inflammatory disorders, (autoimmune enteropathy [AIE] or inflammatory colitis [IC]). We aimed to evaluate in a prospective manner the use of fecal inflammatory markers in the differential diagnosis of severe persistent diarrhea. MATERIAL AND PATIENTS: Twenty-five patients (17 males) were enrolled in this study (median age 8 months). Fourteen children had a constitutive enterocyte disorder (group 1: MVA = 8, ED = 6), and 11 patients had an immuno-inflammatory disease (group 2: AIE = 5, IC = 6). Stool samples were collected at the time of diagnosis and stored at -80 degrees until tumor necrosis factor (TNF)-alpha and calprotectin were measured by enzyme-linked immunoadsorbent assay. RESULTS: No significant differences in age at onset of diarrhea or in stool volumes were observed between both groups. In group 1, fecal TNF-alpha was undetectable/normal in 14 of 14 children, whereas group 2 showed dramatically elevated TNF-alpha levels (mean 3,104, range 237-18,078 pg/g) in 8 of 11 patients. Similarly, calprotectin levels were undetectable/normal in 14 of 14 patients in group 1 and highly raised in 11 of 11 patients in group 2 (median 1,145, range 375-3,095 mug/g), P < 0.01. Under therapy, these inflammatory parameters normalized. CONCLUSIONS: Determination of fecal inflammatory markers is a simple method helping to distinguish constitutive from immuno-inflammatory etiologies of severe persistent diarrhea. These data also suggest that constitutive enterocyte disorders are not accompanied by an inflammatory mucosal reaction.

[Malnutrition screening in hospitalized children: influence of the hospital unit on its management]. / Dépistage de la malnutrition chez l'enfant hospitalisé: influence de la structure d'accueil dans la prise en charge.

OBJECTIVES: The aims of this study were to assess the prevalence of malnutrition in a pediatric population hospitalized in a French regional hospital and to evaluate the influence of type of hospital unit (pediatric or not) in the screening and the management of malnutrition. PATIENTS AND METHODS: This one-day cross-sectional survey was performed in three different seasons during 2003. Every child aged 2 months to 16 years old, hospitalized for more than 48 hours was included. Weight for height, Z-score and Body Mass Index Z-score were used for nutritional assessment. Type of hospitalisation unit, date of admission, associated diagnosis, screening and treatment of malnutrition were also taken into account. RESULTS: Two hundred and eighty hospitalized children were undernourished (11%) and thirty-one children were obese (11%) with no difference in prevalence of malnutrition between pediatric and non-pediatric units. At the time of the study, malnutrition was recognized in one third of the children, at a similar rate whatever the type of hospitalized unit. The children hospitalized in pediatrics wards benefited more frequently from nutritional intervention, i.e. dietician care (43 vs. 16% P < 0.01). CONCLUSION: Prevalence of malnutrition in hospitalized children is low and the same in pediatric or non-pediatric units. Screening of malnutrition remains unsatisfactory in hospital. However, malnutrition is more frequently treated in pediatric unit compared with non-pediatric unit.

Persistence of gastrocutaneous fistula after removal of gastrostomy tubes in children: prevalence and associated factors.

BACKGROUND AND STUDY AIMS: The aims of this study were to determine the prevalence of gastrocutaneous fistula after removal of gastrostomy tubes in children and to identify associated risk factors. PATIENTS AND METHODS: The records of children who had undergone removal of gastrostomy tubes between January 1992 and December 2002 were reviewed retrospectively. Persistent gastrocutaneous fistula was defined as the absence of closure of the gastrostomy 1 month after tube removal. Factors that might influence spontaneous closure of the gastrostomy were studied, including age, underlying disease, nutritional status, type of gastrostomy, replacement of the gastrostomy tube by a button, abdominal wall thickness, duration of gastrostomy tube or button placement, and complications related to the presence of the gastrostomy (infection, granulation tissue). RESULTS: A total of 44 patients were included in the study (mean age 20 months, range 1 day to 14 years). Of these, 28 had undergone percutaneous endoscopic gastrostomy and 16 surgical gastrostomy. The mean time to spontaneous closure was 6 +/- 7 days. Persistent gastrocutaneous fistula developed in 11 patients (25 %) and in seven of these patients this required surgical closure (16 %). The mean duration of gastrostomy placement was significantly longer in patients who went on to develop a gastrocutaneous fistula than in patients who did not develop a fistula (39 +/- 19 months vs. 22 +/- 23 months, respectively, P < 0.03). No other significant association was found between the time required for spontaneous closure and the characteristics of patients or the type of gastrostomy. CONCLUSIONS: Persistent gastrocutaneous fistula is common after removal of gastrostomy tubes in children. Surgical closure should be considered when a gastrostomy has not closed spontaneously 1 month after removal of the gastrostomy tube.