[Cystic fibrosis: transition from child to adult. The stakes and the challenges].
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Biomedical subjects
Publications and source records attributed to D Turck.
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UNLABELLED: Heterotopic or aberrant pancreas is a rare congenital anomaly, usually asymptomatic. The preoperative diagnosis is difficult but most cases of gastric ectopic pancreas have a unique appearance that often makes possible a diagnosis in the absence of histologic confirmation. A well-delineated submucosal, firm mass with central umbilication is characteristic of gastric ectopic pancreas. To the best of our knowledge, the association of esophageal atresia and gastric heterotopic pancreas has never been reported previously. CASE REPORTS: We report two cases of heterotropic pancreas associated with esophageal atresia. Both diagnoses were incidental findings and concerned children operated on at birth for esophageal atresia. Although a definitive diagnosis is histologic, the endoscopic appearance made it possible to maintain this diagnosis. CONCLUSION: Some symptoms have been attributed to ectopic pancreas, and malignant degeneration has been reported in adults. Management continues to be debated: some authors recommend surgical resection while others, as we do, prefer initial therapeutic abstention.
UNLABELLED: Anastomotic stricture is the most common complication following the surgical repair of esophageal atresia, and is usually treated by esophageal dilation. OBJECTIVES: The aims of this study were to assess in an infant population operated on at birth for type III or IV esophageal atresia: 1) the frequency of esophageal stenosis following the repair of esophageal atresia, and associated factors; 2) the efficacy of esophageal dilation by the Savary-Gaillard bougie technique. MATERIALS AND METHODS: The medical records of 52 children presenting with esophageal atresia over a 5-year period were retrospectively reviewed. Gestional age and birth weight, duration of mediastinal and transanastomotic drainage, and anastomotic complications including leakage, stricture, and the presence of gastroesophageal reflux were recorded and analysed. Patients presenting with anastomotic stricture were compared with a group of children without stricture. The number of esophageal dilations, their efficacy and the complication rate were analyzed. RESULTS: Anastomotic stricture developed in 20 (40%) of the 50 patients undergoing primary repair for esophageal atresia. The occurrence of anastomotic stricture was related to anastomotic tension during esophageal surgical repair (p < 0.03). Young children required esophageal dilation at a mean age of 142 days (24-930 days). Stricture resolution occurred after a mean of 3.2 dilations (1-15) over an average period of 7.9 months (range: 0-30 months). Dilation was successful in 90% of the 20 patients. Seven patients required only one dilation. Perforation of the esophagus occurred in one case, and this severe complication led to the death of the child. Esophageal dilation was unsuccessful in two patients, who presented prolonged severe dysphagia. CONCLUSION: Anastomotic stricture following repair of esophageal atresia is connected with the length of the gap that has to be repaired, and tension during suture. Esophageal dilation by the Savary-Gaillard bougie technique is an effective method for treating esophageal stricture. Several dilations are usually needed before the disappearance of dysphagia.
UNLABELLED: Prognosis of extrahepatic biliary atresia depends on an early surgical treatment. This survey studied the delay to surgery of infants treated for biliary atresia and analysed the causes of late diagnosis and referral. MATERIAL AND METHODS: Medical files of 21 infants treated for biliary atresia between 1988 and 1998 were retrospectively analysed. RESULTS: Median age at biliary operation was 57 days and did not change during time. In only 3/21 cases, surgery was performed before 45 days of age. The first clinical or biological sign of cholestasis was noted at a median age of 12 days. In eight cases, the first medical visit for cholestasis (median age of 21 days) was not followed by a blood test. In 11 cases, infants presenting with biological cholestasis were referred to hospital more than seven days later. False diagnosis was noted in seven cases and delayed significantly the operation. CONCLUSION: The delay to surgical treatment is too long and does not decrease whereas a majority of infants visit their physician early. Medical information is mandatory for all physicians taking care of infants.
Chemotherapy administered during childhood may induce dental abnormalities, such as acquired amelogenesis imperfecta, microdontia, hypodontia and altered root morphology. The magnitude of the defect varies according to the cytotoxic agents, the duration of their use and the stage of tooth development at the time of chemotherapy. Patients who received high-dose chemotherapy before the age of 5 are particularly concerned. The dental supervision of these children is based upon three orthopantomograms: the first one has to be performed before starting chemotherapy and will be used as a reference; the second is done soon after the drug therapy in order to evaluate the first consequences; the third is performed after the eruption of all permanent teeth (age 12-13 in average) in order to determine the dental abnormalities. In case of hypodontia, orthodontic treatment must be considered, but it is necessary to take into account the fact that it may increase the risk of root resorption. Preventive dental care is important for these children. It involves meticulous oral hygiene and frequent dental visits to assess and maintain dental health.
For many years soy bean-based formulas (SBBF) were the only dietary product used for infants with cow's milk intolerance. At the present time, their place in infant nutrition is reduced as a result of the availability of new dietary products without lactose and/or cow's milk proteins and the recognition of soy bean protein allergy. There is no evidence that SBBF have any efficiency in infant colic. SBBF have no indication in the prevention of allergy, nor in premature infants' nutrition. Their main indication is the feeding of infants of vegetarian parents who do not want to use cow's milk products. Studies have shown that SBBF contain large quantities of phytoestrogens, particularly isoflavone. Because of experimental data suggesting a possible deleterious effect of phytoestrogens on the neuroendocrine maturation, the reduction of their content in SBBF must be considered.
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BACKGROUND: Percutaneous or surgical insertion of gastrostomy tubes for feeding children has been well described. However, there is no report of percutaneous gastrostomy for chronic decompression in children with chronic gastrointestinal obstruction. The objective of this study was to evaluate this technique. METHODS: Eighteen gastrostomies were performed for gastrointestinal decompression in children. The patients ranged in age from 2 to 125 months (median: 15.5), and all had prolonged hospital stays for severe disease: severe gastroparesis and/or duodenal dysmotility (n = 8), duodenal stenosis (n = 2), chronic intestinal pseudo-obstruction (n = 4), enterocolitis (n = 2), and metastatic abdominal carcinomatosis (n = 2). The duration of symptoms before gastrostomy placement ranged from 0.5 to 44 months (median: 2), with major symptoms including epigastric pain, early satiety, nausea, vomiting, and bloating. RESULTS: The goals of gastric decompression and removal of the nasogastric tube were achieved in all patients, and all had significant relief of both nausea and emesis. Oral intake of liquids and soft foods was possible in 17 of 18 of the children. The tubes were kept in place for a median of 22.5 months (range, 2-73). There was neither long-term morbidity nor mortality associated with the presence of the tube. Seventeen patients returned home with gastric decompression and cyclic parenteral nutrition, two of them for terminal care. Six patients died. In all patients, gastrostomy was used throughout and did not contribute to the death of the patients. For seven children, tubes were removed because of resolution of small bowel or gastric outlet obstruction. CONCLUSIONS: Gastrostomy is an efficient and well-tolerated method of achieving long-term gastric decompression in children with abdominal obstruction not amenable to surgery and/ or resistant to medical treatment.
BACKGROUND: The aim of the study was to identify familial and community environmental risk factors associated with Helicobacter pylori infection in a pediatric population. METHODS: Children requiring diagnostic upper endoscopy were included in the study during a 2-year period. During endoscopy, five gastric biopsies were performed for the histologic or bacteriologic diagnosis, or both, of H. pylori infection. Epidemiologic data collected by a questionnaire were analyzed using the chi-square test or Fisher test and stepwise logistic regression. RESULTS: The authors included 436 patients (242 boys), aged 2 days to 17.9 years (median, 2.7 years). H. pylori prevalence was 7.3%. Univariate analysis found H. pylori was more common in older patients (P < 0.00001), in children who had at least one parent born in a developing country (P < 0.02) or with a low socioeconomic status (P < 0.02), and in those living in crowded conditions (P < 0.02). Children whose mother worked at home were more frequently infected than children whose mother worked outside the home (P < 0.02). Attendance at nursery or school before the age of 6 years was not associated with infection. Logistic regression showed a strong association with H. pylori only for age and number of persons at home. CONCLUSIONS: The source of H. pylori is intrafamilial rather than from a community, such as nursery and school attended at a young age. The number of persons in the home influences the infection status of children but not by the presence of the mother in home. These data suggest that H. pylori infection transmission occurs from siblings or the father rather than from mother.
BACKGROUND: Omeprazole is a proton pump inhibitor, acting selectively on the gastric parietal cell H+K+-adenosine triphosphatase. Data on the intravenous route are limited in children and not available in infants. OBJECTIVE: This study was designed to determine the pharmacokinetics and the optimal dosage of intravenous omeprazole in patients younger than 30 months of age. METHODS: Nine children (three girls), aged 4.5 to 27 months, with normal liver and renal functions requiring intravenous omeprazole were studied. After enrollment in the study and randomization, omeprazole was administered once daily, at 8 am, as a 1-hour infusion. Group 1, consisting of the first four patients, received 20 mg/1.73 m2, and group 2, consisting of the following five patients, received 40 mg/1.73 m2. At day 3, a 24-hour intragastric pH and a pharmacokinetic study of omeprazole were performed. Plasma concentrations were measured by high-performance liquid chromatography. RESULTS: Patients in group 2 had a significantly higher median pH (6.99 vs. 3.35; P = 0.01) and percent of monitored time with gastric pH >4 than children given 20 mg/1.73 m2 (90.6% vs. 44.8%; P < 0.01). Four had a pH more than 4 during more than 90% of the time versus none of the patients of group 1. The plasma concentration versus time curves showed rapid elimination of the drug. The median area under the curve of omeprazole was 0.78 microg. mL-1. h-1 (range, 0.55-1.64 microg. mL-1. h-1) and 3.95 microg. mL-1. h-1 (range, 1.9-4.9 microg. mL-1. h-1), respectively, in groups 1 and 2 (P < 0.05). Systemic clearance was not different between the two groups: median values were 0.68 and 0.42 L. kg-1. h-1 (P = 0.22). CONCLUSIONS: In critical situations, intravenous administration of omeprazole may be required in infants. The authors demonstrate that the dose of 20 mg/1.73 m2 is not effective in maintaining 24-hour gastric pH of more than 4 and that a dose of 40 mg/1.73 m2 is required.
BACKGROUND: There are few data concerning the risk of contamination of enteral feeding systems via gastrostomy in children, and none for conditions that pertain to home-based care. METHODS: To investigate the risk of contamination of enteral feeding systems during the home-based care of 20 children receiving gastrostomy tube feeding, five samples were taken for analysis: two samples before the enteral feeding period (gastrostomy, enteral feeding system) and three after this period (gastrostomy, distal giving set, liquid remained in container). Microorganisms were identified and counted. Different factors were studied to elucidate their role in bacterial colonization: acid suppressive therapy, gastrostomy tube or button, hanging feeding time, rate of enteral feeding, gastric pullulation and retrograde contamination, manipulation error, and use of open or closed enteral feeding systems. RESULTS: Overgrowth was defined as a microorganismal load exceeding 10(4) colony-forming units (cfu)/mL. Overgrowth was present in 85% of gastrostomy samples before enteral nutrition started. Most microorganisms belonged to gastric flora. Some bacteria had an environmental origin or derived from cutaneous flora. Forty-five percent of the lines showed overgrowth at the end of enteral nutrition period, mainly with the same microorganism found in the gastrostomy. Closed enteral bags remained sterile, even if manipulation error occurred. Duration, rate of enteral feeding, and acid suppression treatment were not risk factors for overgrowth. CONCLUSIONS: Retrograde contamination of gastrostomy feeding systems occurs frequently. The preferential use of closed enteral feeding systems is recommended for home-based enteral nutrition programs.
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PURPOSE: The aim of this study was to report the late morbidity of percutaneous endoscopic gastrostomy (PEG) in a pediatric population and to identify possible risk factors for complications developing after PEG insertion. METHODS: A PEG was placed in 110 children between 1 May 1990 and 1 January 1997 using the pull technique. A retrospective study of late-onset complications was performed, with a follow-up period ranging from 1 to 8 years. All the complications occurring more than 6 days after PEG insertion were recorded, except for gastrostomy tube obstruction and accidental tube dislodgement. RESULTS: The prevalence of late-onset complications related to PEG in our patients varied from 3.8 to 4.4 per 10 5 days. The overall rate of late-onset complications was 44% (48 complications observed in 29 patients [26%]). Seventy-five percent of the complications appeared during the first 2 years after PEG insertion. Nine different types of complication have been identified: intragastric buried or extruded gastrostomy (n = 24), gastric metaplasia granulation tissue around the site of gastrostomy (n = 8), intragastric pseudotumoral proliferative gastric mucosa (n = 4), intragastric mucosal ulceration (n = 3), cutaneous necrosis (n = 3), cologastric fistula (n = 2), gastrostomy closure delay after tube removal (n = 2), subcostal neuralgia (n = 1), and peritonitis (n = 1). Wilcoxon and chi-square tests were used to compare the clinical characteristics of the patients and the type of material used in the two populations, with and without complications. No clinical risk factor for the development of complications could be identified. Intragastric buried or extruded gastrostomy appeared more frequently with the use of one type of button than with the use of tubes. CONCLUSIONS: The authors' experience suggests that PEG is associated with significant late morbidity, which is mainly observed within the first 2 years after PEG insertion. However, no risk factor for the development of such complications could be identified.
A case of severe and protracted diarrhoea is reported, which started in the neonatal period and progressively associated with neurological impairment, dysmorphy, hepatosplenomegaly, and hepatic insufficiency, from which the patient died at 2 years of age. Isoelectric focusing of serum transferrin showed a congenital disorder of glycosylation type I pattern but the basic defect could not be identified. This observation shows that congenital disorder of glycosylation is a cause of intractable diarrhoea in neonates.