Biomedical subjects
Publications and source records attributed to F Daum.
A 19-year-old white male with Crohn's disease, who complained of passing urine per rectum and having retrograde ejaculations, was noted to have a urethroperineal-rectal fistula. The fistulous communication remained patent despite pharmacologic therapy, a diversion ileostomy, and a total proctocolectomy. A fistulectomy and definitive urethral repair finally resulted in resolution of the problem.
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Chronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11-year-old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.
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A new type of tube is described for measuring lower esophageal sphincter pressures in nonsedated infants and children. Pressure measurements may help in predicting those infants who will require surgery for gastroesophageal reflux.
The diagnosis of Hirschsprung's disease in the newborn does not mandate the performance of a preliminary colostomy. Enterocolitis can be adequately and safely treated by a precise regimen of colonic irrigations. The endo-rectal "pull-through" procedure is safe and effective when performed in the neo-natal period. Long-term follow-up is necessary to evaluate possible late complications.
Sixty-three children with seizure disorders receiving phenobarbital and/or diphenylhydantoin for more than 12 months had liver function tests evaluated. All 56 whose serum anticonvulsant concentrations were in the therapeutic range had elevations of their serum gamma glutamyl transpeptidase activity. Of the 11 who had elevated SGOT and SGPT concentrations initially, six had persistent transaminase abnormalities for more than 20 weeks. Liver tissue from these six patients revealed by light microscopy uniform swelling of the hepatocytes without cell necrosis, inflammation, fibrosis, or disturbance of hepatic architecture. Electron microscopy demonstrated proliferation of the smooth endoplasmic reticulum without other ultrastructural alterations. All six patients were maintained on the same dosages of PB and/or DPH, and their transaminase activities returned to normal within eight to 14 months. The clinical well being of these patients, the transient nature of their SGOT and SGPT elevations, and the absence of specific histopathology suggest that chronic treatment with PB and/or DPH does not result in hepatotoxicity but rather in enzyme induction. The data indicate that liver biopsies are not warranted in such children and that PB and DPH may be continued despite mild elevations of SGOT and SGPT, without concern for hepatic damage.
Zinc status was studied in 30 patients with chronic inflammatory bowel disease (CIBD) as well as in 17 normal children, 13 primordial short stature, and 17 anorexia nervosa patients. Basal serum and urinary excretion levels of zinc were measured in all patients. In addition, a zinc loading test was performed in 16 CIBD patients, 21 normal and/or short stature children, and nine patients with anorexia nervosa. Eleven of 30 patients with CIBD had serum zinc values less than 0.7 microgram/ml, whereas none of the other patients had hypozincemia. In addition, the mean urinary zinc excretion of CIBD patients was significantly lower than that of patients with primordial short stature and with anorexia nervosa. An altered response to oral zinc load was the most frequent abnormality in CIBD patients. Those with moderate and severe clinical disease activity had a decreased serum rise of zinc after the oral load of this ion. Urinary excretion of zinc after oral load was also marked by deficiency in all CIBD patients. The abnormalities of zinc metabolism were more frequent among the CIBD patients with growth abnormalities, although they were also found in patients who had normal growth. Among the 14 patients with CIBD and growth abnormalities, seven were hypozincemic and four hypozincuric. Hypozincemia was only found in four patients who had normal height; however, the growth velocity was not known. The zinc tolerance test revealed abnormalities in four of five CIBD patients with short stature and in two of three patients with slow growth. On the other hand, similar alterations in zinc tolerance tests were seen in three of seven CIBD patients with normal height and growth.
Infants undergoing phototherapy may develop loose stools. Acquired lactase deficiency secondary to this treatment modality was suggested as a cause for the diarrhea. The effects of light energy directly on intestinal enzymes and indirectly through bilirubin photooxidation products were studied. Adult homozygote and heterozygote Gunn rats were treated with light for varying periods while littermate controls were kept in the dark. Intestinal lactase, sucrase and GGTP activities and serum bilirubin concentrations were determined. Jaundiced and non-jaundiced 1-week-old suckling Gunn rats were treated with light for 96 h, and intestinal lactase activity determined. No decreases in lactase, sucrase or GGTP activities were observed suggesting the reported diarrheal states following phototherapy are not related to light energy or photooxidation products.
Although acute anterior uveitis has been noted in children with inflammatory bowel disease, it has not been appreciated in the absence of ocular symptoms. To determine the presence of asymptomatic uveitis, slit-lamp examinations were performed in 19 children with granulomatous bowel disease and seven with ulcerative colitis. In the former group, six had uveitis, while no abnormalities were noted in those with ulcerative colitis. Abnormalities consisted of cells and flare in the anterior chamber. In the group with asymptomatic uveitis, all were male, three were black, and all had colonic involvement. No positive correlations were noted between the presence of uveitis and bowel symptoms, duration of illness, extraintestinal manifestations, or specific treatment regimens. None of the six children with uveitis had evidence of spondylitis, and five were HLA-B27-negative. Repeated eye examinations six to 12 months later disclosed no evidence of uveitis in four of five children and improvement in the remaining child. These data suggest that asymptomatic transient uveitis is common in children with granulomatous bowel disease, but progression to severe adult uveal disease remains unclear.
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Three children, 30 to 36 months of age, had solitary juvenile polyps in the sigmoid colon and rectal bleeding of varying severity, In all three patients, the polyps were removed without the use of general anesthesia through a standard 100-cm colonoscope used to examine adult patients. No untoward effects were encountered. Colonoscopic polypectomy is an effective therapeutic procedure and obviates the need for either anxious waiting in the mildly symptomatic patient or colotomy and polypectomy in the child with profuse rectal bleeding.
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In children with extrahepatic biliary atresia, impaired hydroxylation and defective intestinal absorption of cholecalciferol may lead to a deficiency of vitamin D and rickets. The data presented herein demonstrate that in such patients serum levels of vitamin D measured as 25-hydroxycalciferol are reduced. A moderate therapeutic oral dose of 25-hydroxycholecalciferol, by circumventing the hepatic conversion of cholecalciferol to 25-hydroxycholecalciferol, will replete vitamin D stores and maintain the serum concentration of 25-hydroxycalciferol required to prevent or heal rickets in these patients.
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Adverse reactions to aspirin, a potentially hepatotoxic drug, are believed to be dose related. This article reviews the medical literature and described two children with aspirin hepatitis. Hepatic dysfunction recurred in one of these patients at salicylate concentrations in serum previously considered nontoxic.