Aminosalicylates for the treatment of inflammatory bowel disease.
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Aminosalicylic Acids↗
Biomedical subjects
A M Leichtner
Publications and source records attributed to A M Leichtner.
Long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children.
BACKGROUND: The long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children are unknown. METHODS: Five children were evaluated in a clinical research center 2.5 to 10 years after pancreaticoduodenectomy to assess their nutritional status based on patterns of growth and to assess their gastrointestinal function. Investigation included vitamin levels, a bentiromide study, and serum immunoreactive trypsinogen levels to evaluate pancreatic function and a d-xylose absorption and a radionuclide gastric emptying scan for intestinal absorption and motility. RESULTS: Children were able to grow after pancreaticoduodenectomy. Three remained in low percentile groups for height/weight ratio, and two were near or above normal. Low normal levels of the fat-soluble vitamins were present. Very low levels of pancreatic function were found based on the bentiromide and trypsinogen studies, whereas intestinal absorption of d-xylose was normal except for one patient with extremely rapid gastric emptying. CONCLUSIONS: After pancreaticoduodenectomy children can grow and develop normally if given adequate levels of oral pancreatic supplements to replace the severely decreased level of endogenous pancreatic enzymes after operation. Routine supplementation of the fat-soluble vitamins should be considered.
4-Aminobenzoic Acid↗
Allergic proctocolitis in infants: a prospective clinicopathologic biopsy study.
Allergic proctocolitis is a major cause of rectal bleeding in infants, but its clinical features and laboratory results are often nonspecific. Our previous retrospective study demonstrated that large numbers of eosinophils in colonic mucosal biopsy specimens were highly associated with cases of allergic proctocolitis. Therefore, we prospectively examined 60 colonic mucosal biopsy specimens from the same sites (4, 8, and 12 cm from the anal verge) in 20 infants with clinically confirmed allergic proctocolitis to validate this morphologic feature, to characterize its distribution, and to correlate these data with the clinical information. The patients (age range, 4 to 304 days) were fed breast milk or a variety of formulas and all presented with rectal bleeding. Sigmoidoscopic examination was abnormal in 19 cases, typically characterized by focal areas of mucosal erythema. The major histologic finding was a strikingly focal increase in the number of eosinophils in all mucosal compartments, with a predilection to aggregate in close association with lymphoid nodules. Eosinophilic infiltration varied not only between biopsies at different sites, but also within individual biopsy specimens. Only 12 of 20 patients (60%) had all three of their biopsy specimens categorized as abnormal; in the remainder, only one (four patients) or two (four patients) of the three biopsy specimens were abnormal. The average number of eosinophils per high-power field of lamina propria for all cases was 15.6. No significant correlation was identified between the number of eosinophils in the mucosa and the patient's age, length of illness, endoscopic appearance, or type of inciting formula. In summary, eosinophils appear to be an excellent marker for infantile allergic proctocolitis. Given the focal distribution of the eosinophils, multiple mucosal biopsy specimens should be obtained and several levels of each examined to confirm the diagnosis.
Biopsy↗
The antegrade colonogram: extending the small bowel follow through for children suspected of having colonic disease.
Fifty pediatric patients suspected of having colonic disease were evaluated by antegrade barium study ("antegrade colonogram") and by colonoscopy with multiple biopsy specimens. Radiographic patterns of mucosal irregularity, nodularity, or ulceration, haustral asymmetry or edema, and narrowing of the colonic lumen were easily recognized. Correlation between colonographic patterns, biopsy findings, and gross appearance at colonoscopy showed the colonogram to have an overall accuracy of 77-88%. The antegrade colonogram is easily performed and often provides information regarding areas of the colon not seen by the endoscopist. In some cases it may also be useful in the evaluation of response to therapy for inflammatory bowel disease.
Adolescent↗
Longitudinal assessment of type I procollagen in children with inflammatory bowel disease subjected to surgery.
Using radioimmunoassay we have measured the serum concentrations of the C-terminal propeptide of type I procollagen (pColl-I-C) in 12 Tanner I-II subjects (aged 9-16 years) with severe Crohn's disease (8 patients) or ulcerative colitis (4 patients) and markedly decreased growth velocity who were subjected to surgery and 50 similarly aged children with either ulcerative colitis (20 patients) or Crohn's disease (30 patients) and normal growth. Prior to operation, the mean growth velocity and pColl-I-C concentration in the former group of 12 children were 0.03 +/- 0.02 cm/month (normal greater than or equal to 0.5 cm/month) and 14.1 +/- 1.9 micrograms/dl, respectively. This pColl-I-C concentration is comparable with that previously reported for adults (5-17 micrograms/dl) and significantly lower than found in the 50 normally growing children with inflammatory bowel disease (IBD) (46.9 +/- 2.0 micrograms/dl) (p less than 0.001). All 12 children subjected to surgery had a marked increase in growth velocity and pColl-I-C concentration to 0.73 +/- 0.08 cm/month and 59.1 +/- 5.6 micrograms/dl, respectively (p less than 0.001 compared with preoperative values). Changes in pColl-I-C concentrations antedated measurable changes in linear growth. These data suggest that pColl-I-C concentrations can reflect growth velocity in children with IBD subjected to surgery and may provide a rapidly available measure of current "growth activity."
Adolescent↗
Relationship of type I procollagen to corticosteroid therapy in children with inflammatory bowel disease.
We determined the serum concentration of the C-terminal propeptide of type I procollagen (pColl-I-C) in 60 children and adolescents (ages 4 to 17 years) with inflammatory bowel disease (24 ulcerative colitis, 36 Crohn disease) and in seven children (ages 2 to 15 years) with nongastrointestinal disease (asthma) during varying regimens of corticosteroid therapy. Patients with inflammatory bowel disease were grouped according to disease severity (mild, and moderate to severe). Significantly lower pColl-I-C concentrations and growth velocities were found in each severity group among those subjects receiving daily corticosteroid therapy compared with those receiving alternate-day or no corticosteroid therapy (P less than 0.01). When daily corticosteroid therapy was initiated and then maintained for 7 to 14 days in 11 patients with exacerbation of inflammatory bowel disease clinical improvement resulted, but mean procollagen concentrations decreased significantly (P less than 0.001). In seven children with asthma receiving methylprednisolone intravenously, significant decreases in pColl-I-C concentrations were noted within 24 to 48 hours of therapy (P less than 0.001). These data indicate that serum procollagen values decrease during both short- and long-term daily administration of corticosteroid therapy. Longitudinal assessment of procollagen concentrations may provide rapid assessment of the effects of different corticosteroid regimens on growth.
Adolescent↗
Carbohydrate malabsorption following fruit juice ingestion in young children.
We performed breath hydrogen analyses in 13 healthy children (9 to 36 months of age) and seven children (14 to 27 months of age) with chronic nonspecific diarrhea after they had ingested pear, grape, and apple juices and a 2% sorbitol solution. Excess breath H2 excretion was found in virtually all study subjects following the ingestion of either pear juice (with approximately 2% sorbitol content) or the 2% sorbitol solution, in approximately 50% of those ingesting apple juice (0.5% sorbitol), and in 25% of those ingesting grape juice (no sorbitol) (P less than .001, analysis of variance). No differences were noted between the healthy children and those with chronic nonspecific diarrhea. Forty percent of all study subjects in whom excess breath hydrogen excretion occurred also had diarrhea and abdominal cramping. Carbohydrate malabsorption appears to be frequent following the ingestion of common fruit juices and in some children may be associated with nonspecific gastrointestinal symptoms.
Breath Tests↗
Cyclosporine treatment of autoimmune chronic active hepatitis.
A 14-yr-old boy with a 5-yr history of autoimmune chronic active hepatitis refractory to corticosteroid therapy was given cyclosporin A (5 mg/kg X day). Before cyclosporine therapy, serum aminotransferase levels were 20 times normal and immunoglobulin G was 4 g/dl. Within 2 wk of starting cyclosporine therapy, aminotransferase levels decreased; by 2 mo they were almost normal, and at 1 yr into therapy they were normal. A decrease in cyclosporine dosage was associated with an increase in aminotransferase levels, which then again decreased as the dose was increased. Severe growth failure observed during previous corticosteroid therapy reversed during cyclosporine treatment and the patient displayed "catch-up" growth. No significant side effects were noted after 1 yr of cyclosporine therapy. Further evaluation of cyclosporine in the treatment of corticosteroid-unresponsive autoimmune chronic active hepatitis appears warranted.
Adolescent↗
Hypertension associated with unilateral hydronephrosis as a complication of Crohn's disease.
Obstructive uropathy with hydronephrosis is a well-known complication of Crohn's disease. The treatment for this condition is still controversial. This is the case study of a 14-year-old girl with documented right-sided obstructive uropathy secondary to Crohn's disease associated with renin-mediated hypertension secondary to her obstructive uropathy. The patient had complete resolution of her hypertension following surgery, which involved only resection of the involved bowel without ureterolysis.
Adolescent↗
Type 1 procollagen as a biochemical marker of growth in children with inflammatory bowel disease.
Using radioimmunoassay, we measured the levels of the C-terminal propeptide of type I procollagen (pColl-I-C) in sera from 69 children with functional bowel disease (control population), 18 children with ulcerative colitis, and 35 children with Crohn disease. Sexually mature fully grown adolescents from all three patient groups had mean pColl-I-C concentrations (12.0 +/- 0.8 micrograms/dl) similar to those previously reported for adults (5 to 17 micrograms/dl). Children with functional bowel disease and normal growth had significantly higher concentrations (32.8 +/- 1.7 micrograms/dl) (P less than 0.001) than did the fully grown adolescents. In patients with inflammatory bowel disease a significant relationship between growth velocity and pColl-I-C concentrations was noted (P less than .001). Lower pColl-I-C concentrations were found in patients receiving daily prednisone therapy compared with those receiving alternate-day therapy (P less than 0.01) or those not taking the drug (P less than 0.01). These data suggest that pColl-I-C concentrations reflect growth activity in children. Repeated determinations may allow rapid assessment of the effects of various therapeutic modalities on growth in children with inflammatory bowel disease.
Adolescent↗
Pseudopheochromocytoma and cardiac arrest associated with phenylpropanolamine.
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Adolescent↗
Apple juice. An unappreciated cause of chronic diarrhea.
Chronic nonspecific diarrhea (CNSD) remains a common pediatric problem. Previous reports have suggested disordered small intestinal motility, food intolerances, dietary fat restriction, and excessive fluid consumption as possible contributory factors. We have recently encountered a subset of children with CNSD in whom nonexcessive apple juice intake seemed to cause their diarrhea. In five subjects, ingestion of 240 mL of apple juice disclosed evidence of significant carbohydrate malabsorption by breath-hydrogen testing and resulted in diarrhea. Withdrawal of apple juice from the diets of these subjects was curative in all cases. Before embarking on an expensive and time-consuming evaluation for CNSD in otherwise healthy children, a brief restriction of apple juice intake may be warranted.
Breath Tests↗
Chronic diarrhea associated with hypogammaglobulinemia and enteropathy in infants and children.
In order to define the gastrointestinal manifestations and small intestinal structure and function in a group of infants with chronic nonspecific diarrhea and hypogammaglobulinemia, we retrospectively identified 55 such patients from a population of 518 children evaluated for chronic diarrhea over a 6-year span (10.6%). All patients had IgG levels 2.0 SD or more below the mean values for age. Patients with biochemical evidence of protein loss (enteropathy or nephropathy) were excluded. There was a 50% incidence of small intestinal mucosal injury among these patients, with a spectrum of morphological findings ranging from healing enteritis to severe active enteritis. Carbohydrate malasorption, and infection with Giardia lamblia or Clostridium difficile occurred in 34% and 24% of patients tested, respectively. These structural, functional, and infectious complications were all statistically more common in patients than in a control group of children with chronic diarrhea, normal growth, and normal immunoglobulin levels. This study suggests that immunoglobulin determination, in children who would otherwise carry a diagnosis of chronic nonspecific diarrhea, identifies a group with hypogammaglobulinemia, having an increased incidence of treatable intestinal dysfunction or infection, and a spectrum of small intestinal histologic abnormalities.
Agammaglobulinemia↗
Recent advances in diagnosis and treatment of gastrointestinal hemorrhage in infants and children.
Gastrointestinal bleeding is a common and occasionally life-threatening problem in infants and children. A careful history and physical examination as well as the application of new endoscopic and radiographic techniques will reveal the source of hemorrhage in most patients. The utility of recently introduced pharmacologic agents and endoscopic techniques in the treatment of peptic disease and variceal hemorrhage in children remains to be determined. A cooperative effort among pediatricians, radiologists, and surgeons should minimize the morbidity and mortality from gastrointestinal tract bleeding in this population.
Angiography↗
Discordance for biliary atresia in two sets of monozygotic twins.
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Bile Ducts↗
Addition of a mannose-6-phosphate-containing oligosaccharide alters cellular processing of low density lipoprotein by parental and LDL-receptor-defective Chinese hamster ovary cells.
Low density lipoprotein (LDL) was chemically modified by the addition of omega-(6-phospho)-tetra(alpha 1-3)mannosyl-(alpha 1-2)mannose (M56P), a phosphorylated oligosaccharide containing a terminal mannose 6-phosphate residue. Uptake and degradation of this modified LDL (M56P-LDL) by Chinese hamster ovary (CHO) cells occurred via the lysosomal enzyme (mannose 6-phosphate) receptor pathway. Cellular processing of M56P-LDL was saturable, specific for the mannose 6-phosphate marker, and occurred with approximately threefold higher affinity than that of native LDL by the LDL receptor pathway. Mannose 6-phosphate receptor activity, as measured by degradation of M56P-LDL, was ninefold lower than the LDL receptor activity. Degradation of M56P-LDL was more sensitive to inhibition by the lysosomotropic agent chloroquine than was degradation of LDL, suggesting differences in the intracellular processing of mannose 6-phosphate-bearing ligands and LDL. Previously isolated CHO cell lines defective in LDL receptor activity resembled parental CHO cells in their ability to process M56P-LDL. The potential use of M56P-LDL in the isolation of cells with pleiotropic mutations affecting receptor-mediated endocytosis is discussed.
Alkaline Phosphatase↗
Horizontal nonparenteral spread of hepatitis B among children.
Two families with an unusually high incidence of hepatitis B infection (15 of 21 persons) were investigated over an 18-month period. Serologic evidence of past or present infection--hepatitis B surface antigen (HBsAg), antibody to hepatitis B surface antigen (anti-HBs), or antibody to hepatitis B core antigen (anti-HBc)--was found in 11 of the 12 members of one family, four of whom were chronic HBsAg carriers, and in four of nine members of a contact family. Anti-HBc was the only serologic marker of infection in five persons. Histocompatibility leukocyte antigen (HLA) typing failed to show an association between carriage of HBsAg and specific HLA markers. Chewing gum was a potential vehicle as HBsAg was detected in gum samples from three of four children who were chronic HBsAg carriers. Horizontal, nonparenteral transmission of hepatitis B virus probably accounted for the clustering of infection in these families, especially via the exchange among children of objects contaminated with oral secretions.
Adolescent↗
Regulation of low density lipoprotein receptor function in a human hepatoma cell line.
Low density lipoprotein (LDL) processing was investigated in a human hepatoma-derived cell line, Hep G2. Hep G2 cells bound, internalized and degraded LDL via a saturable, high affinity (Kd approximately 2 X 10(-8)M) pathway similar to that present in other mammalian cells. Although 80% of the uptake and degradation of 125I-LDL was inhibited by 40-fold excess native LDL, the same concentration of methylated LDL, which cannot bind to LDL receptors, had virtually no effect on processing. When added at low concentrations, the lysosomotropic agent, chloroquine, inhibited degradation (I50 approximately 15 microM) without affecting the rate of lipoprotein internalization. Receptor activity was decreased 60% by preincubation of the cells in medium containing a source of cholesterol (LDL or unesterified cholesterol) and increased 1.7-fold by preincubation with compactin, a competitive inhibitor of 3-hydroxy-3-methylglutaryl coenzyme A reductase. The Hep G2 cell line may prove a useful system both for the further study of hepatic lipoprotein metabolism and for the evaluation of new antihypercholesterolemic agents.
Carcinoma, Hepatocellular↗