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Purpose: The incidence of persistent gastrocutaneous fistula (GCF) after removal of gastrostomy tubes in pediatric patients is estimated to be up to 44 %. Our aim was to review the outcomes of GCF closure by an endoscopic technique that utilizes cautery and endoclips., Methods: A retrospective analysis of patients who underwent endoscopic treatment for persistent GCF from January 2010 to September 2013 was performed. This technique utilized esophagogastroduodenoscopy with cauterization of the fistula track and endoclipping of the gastric mucosa., Results: Sixteen patients underwent endoscopic treatment for persistent GCF. Mean age at time of endoscopy was 7.5 ± 5.5 (1.1-17) years. Gastrostomy tubes were in place for mean of 5.4 ± 5.2 (0.5-14.2) years prior to removal. The average time from gastrostomy tube removal to first endoscopic clipping was 6.7 ± 9 (0.1-28.9) months. Seven patients (44 %) had successful closure after one endoclipping procedure. Six patients underwent a second endoclipping procedure, with three successful closures. Four patients (25 %) required surgical closure for persistent fistulas and 2 (13 %) have continued drainage., Conclusions: While endoscopy with cautery and endoclipping proves to be safe, many patients require multiple procedures and may require surgical closure. Patient selection and refinement of this technique may improve outcomes.

Isoniazid (INH) monotherapy has gained widespread acceptance as an efficacious therapy for latent tuberculosis infection (LTBI) especially in low-prevalence settings. Although INH related hepatotoxicity is well recognized, progression to severe liver dysfunction requiring care at a transplant center remains unpredictable. We report the management of a five year-old girl who developed progressive liver failure due to INH prophylaxis. This highlights the potential severity of INH related hepatic injury and underscores the significance of vigilant clinical monitoring throughout the duration of the therapy in children.

Background: Little information has been reported regarding the frequency and type of complications arising from removal of percutaneous endoscopic gastrostomy (PEG) tubes in children., Methods: The records of 397 patients who had PEG tubes placed from 1993 through 1998 were reviewed for complications after removal. Data collected included length of time the tube was in place, age of the patient at insertion, type of tube removed, and patient diagnosis., Results: Fifty-four children had the PEG tube removed by traction or endoscopy. The only complication was persistent leaking through a gastrocutaneous fistula in 13 patients (24%). Leaking ceased in 6 children coincident with H2-antagonist therapy and silver nitrate cautery, and surgical closure of the fistula was required in 7 patients. Comparison of these 7 children with those who did not require surgery (n = 47) showed a longer duration of tube placement (mean +/- SE of 20.6+/-3.6 months, range 11-31 months vs. 11.1+/-1.3 months, range 1-35 months; P<0.05). Further analysis showed no child with a PEG tube removed before 11 months (n = 23) after insertion required surgery, whereas 7 (23%) of 31 children with a PEG tube removed after 11 or more months required surgery. Age at insertion, type of feeding device removed, and patient diagnoses were not different between the two groups., Conclusions: These data indicate that persistent leaking necessitating surgical closure of a gastrocutaneous fistula does not occur in children with a PEG tube removed within 11 months of insertion. In contrast, 23% of children with a PEG tube removed 11 or more months after insertion require surgery. In patients identified as candidates for tube removal, this time frame may be important in clinical decision making.

Type IV glycogenosis is due to branching enzyme deficiency and is usually manifested clinically by progressive liver disease with cirrhosis and hepatic failure between the second and fourth years of life. We describe a 5-year-old boy who, following an acute febrile illness at 2 years of age, was first noted to have hepatomegaly with mildly elevated serum transaminase levels. Liver biopsy revealed hepatic fibrosis with periodic-acid Schiff-positive, diastase-resistant inclusions in hepatocytes and fibrillar inclusions characteristic of amylopectin by electron microscopy. Enzymatic assay revealed deficient hepatic branching enzyme activity with normal activity of glucose-6-phosphatase, debranching enzyme and phosphorylase activities. During the succeeding 3 years, he grew and developed normally with apparent resolution of any clinical evidence of liver disease and only intermittent elevation in serum transaminase levels associated with fever and prolonged fasting. Repeat liver biopsy at 4 years of age showed persistence of scattered hepatocellular periodic-acid Schiff-positive, diastase-resistant inclusions, but no progression of hepatic fibrosis in spite of persistent deficiency of hepatic branching enzyme activity. Skeletal muscle and skin fibroblasts from the patient also showed deficient enzyme activity. Skin fibroblasts from both parents exhibited half the normal control activity, suggesting a heterozygote state. This is the first documented patient with deficiency of branching enzyme but without evidence of progressive hepatic disease. This patient, coupled with reports of other patients with late onset hepatic or muscle disease with branching enzyme deficiency, suggests that the defect resulting in Type IV glycogen storage disease is more heterogenous and possibly more common than previously suspected.

Foreign bodies of the esophagus may penetrate the wall and migrate extraluminally, requiring a thoracotomy for removal. We report two children who swallowed coins that went undetected for several months and slowly eroded into the esophageal wall. These coins could not be retrieved by upper endoscopy, because of proximal strictures and granulation tissue at the perforation sites. Following surgical gastrostomy and retrograde flexible endoscopy, the coins were successfully removed. Retrograde endoscopy should be considered for removal of foreign esophageal objects before undertaking a thoracotomy.

An 8-week-old infant presented with vomiting and failure to thrive due to small bowel obstruction caused by a diffusely enlarged pancreas. Surgical bypass of the obstruction was followed by secretory diarrhea, hypokalemia, and dehydration. Plasma vasoactive intestinal peptide (VIP) (823pg/ml), pancreatic polypeptide (4,500 pg/ml), and neurotensin (680 pg/ml) concentrations were markedly elevated. No neoplastic process was identified. Therapy with the long-acting somatostatin analogue SMS 201-995 was followed by decline in VIP concentrations (900 to 200-300 pg/ml), decrease in stool frequency, and normalization of serum electrolytes. During 12 months of somatostatin analogue therapy, length and weight progressed along the 3rd percentile on the Tanner growth chart.

Intestinal allergic states are common, but so are misconceptions about them. Since cow milk is one of the most common foods to produce an adverse immunologic reaction, it serves here as a prototype of food allergy in general. The mechanism of this reaction and its clinical and laboratory manifestations vary considerably from patient to patient. Once the diagnosis is established, however, treatment is relatively straightforward.

The clearance of circulating IgA immune complexes following acute bile duct obstruction was investigated in this study. IgA immune complexes were formed in vitro from MOPC-315, an IgA M-component with anti-dinitrophenyl (DNP) specificity, and 125I-DNP10 bovine serum albumin (BSA). Eighteen hours after laparotomy during which the common bile duct was either identified only or identified and ligated, the IgA immune complexes were injected intravenously. Groups of bile duct-ligated and bile duct-patent rats were also injected intravenously with IgG anti-DNP-125I-DNP10BSA immune complexes and 125I-bovine liver beta-glucuronidase to assess the hepatic clearance of materials not dependent on an intact biliary system. Clearance of IgA immune complexes was delayed after bile duct ligation. Although the clearance of IgA immune complexes was delayed, only 10% of these complexes remained in the circulation at 3 hr. The clearance of IgG immune complexes and beta-glucuronidase was not affected by ligation. These experiments demonstrate the physiologic importance of a patent bile duct in the normal clearance of IgA immune complexes in the rat. The observation that clearance is delayed, but not completely inhibited by bile duct ligation suggests that alternate mechanisms exist for removing IgA immune complexes from the circulation.