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Biomedical subjects

D A Gremse

Publications and source records attributed to D A Gremse.

34 records · Page 2Linked to original sources

Sunflower seed bezoar leading to fecal impaction.

We report the cases of two children, ages 8 and 10 years, with painful defecation and constipation due to impaction of sunflower seed shells that did not respond to laxative and enema therapy. Physical examination revealed a prickly mass in the rectal vault. Proctoscopy revealed bezoars of sunflower seed shells, which were removed by endoscopic biopsy forceps followed by a combination of rectal irrigation and manual disimpaction. The rectal pain resolved, and bowel habits returned to normal with laxatives. Patients with rectal bezoars who fail to respond to enemas or colonic lavage may require endoscopic management for resolution of their symptoms.

Bezoars↗

Collagenous colitis in children.

Collagenous colitis, a disorder characterized by increased subepithelial collagen deposition associated with an inflammatory infiltrate in the lamina propria, has been reported infrequently in children. An 8-year-old girl with collagenous colitis is described who presented with chronic watery diarrhea and abdominal pain. Biopsy specimens of the colonic mucosa showed the pathological features of collagenous colitis. The patient's symptoms resolved following corticosteroid therapy. Collagenous colitis should be considered in the differential diagnosis of children with chronic diarrhea.

Child↗

Symptoms of acid-peptic disease in children.

Esophagogastroduodenoscopy (EGD) is a useful procedure for the evaluation of gastrointestinal disease in pediatric patients. Indications for EGD in children are similar to those for adults. However, symptoms of acid-peptic disease reported by children may differ from those expressed by adults. Therefore, we sought to identify symptoms associated with acid-peptic disease in pediatric patients. The medical records of 114 children having EGD with biopsy were reviewed. The diagnosis, which included esophagitis, gastritis, duodenitis, ulcer, or normal, was based on histologic findings. Six symptoms correlated significantly with acid-peptic disease: epigastric pain, nocturnal pain, postprandial pain, water brash, weight loss, and a family history of peptic ulcer disease. Epigastric pain and nocturnal pain are the most sensitive symptoms of acid-peptic disease in pediatric patients. Fecal occult blood and hematemesis, although specific, are not sensitive indicators of acid-peptic disease in children.

Abdominal Pain↗

Monoethylglycinexylide formation in assessing pediatric donor liver function.

Lidocaine metabolism to monoethylglycinexylide (MEGX) has been described as a novel method to assess liver function in adult transplant donors and recipients. While this assay appears to offer a number of advantages over existing liver function tests, limited work has been done to evaluate its potential in the pediatric population. This study evaluated MEGX formation in potential pediatric liver donors (n = 35) and a control group of children (n = 16). The mean MEGX formation was significantly higher in pediatric donors than in the control group (156 +/- 62 vs 106 +/- 33 ng/ml, p < 0.05). No correlation with age, total bilirubin, liver transaminases, or alkaline phosphatase could be made within each group. Significant differences in MEGX levels were noted when each group was compared to its adult counterpart. Both pediatric donors and controls had greater mean MEGX formation than has been reported for adult donors and controls (156 +/- 62 vs 127 +/- 61 ng/ml, p < 0.05 and 106 +/- 33 vs 72 +/- 36 ng/ml, p < 0.05, respectively). Drugs that alter lidocaine pharmacokinetics and their potential influence on MEGX formation were evaluated in the pediatric donor group. Donors exposed to hepatic enzyme-inducing drugs had a higher mean MEGX formation (187 +/- 60 vs 146 +/- 63 ng/ml). No significant differences were noted between donors receiving and not receiving vasopressors. In conclusion, the significant differences between pediatric and adult MEGX formation should be noted when establishing reference or normal ranges for this diagnostic test. Furthermore, concomitant drug therapy may significantly alter MEGX formation.

Adult↗

Assessment of lidocaine metabolite formation as a quantitative liver function test in children.

Lidocaine, an aminoethylamide, undergoes deethylation in the liver after intravenous injection, resulting in the formation of monoethylglycinexylidide. Serum monoethylglycinexylidide concentration can be measured by a simple, rapid fluorescent polarization immunoassay. We sought to determine whether lidocaine metabolism, as indicated by monoethylglycinexylidide formation, could be used as a quantitative index of hepatic function. Therefore we measured monoethylglycinexylidide formation in 10 healthy children and in 28 children with chronic liver disease. Patients with chronic liver disease were divided by disease severity as being at low, moderate or high risk for deterioration based on a clinical scoring system for liver transplant candidates. In healthy controls, the peak monoethylglycinexylidide concentration 15 min after injection of lidocaine (1 mg/kg) was 97 +/- 12 micrograms/L (mean +/- S.E.). However, monoethylglycinexylidide concentration was decreased in 28 children with chronic liver disease (32 +/- 5 micrograms/L, p less than 0.001). Furthermore, this value was inversely proportional to the severity of liver disease. The mean difference in monoethylglycinexylidide concentration between the low-risk and high-risk groups was statistically significant (42 +/- 6 vs. 11 +/- 4 micrograms/L, respectively, p less than 0.01). We conclude that the rate of formation of the lidocaine metabolite monoethylglycinexylidide is decreased in patients with chronic liver disease and is inversely related to disease severity. Therefore measurement of monoethylglycinexylidide concentration after lidocaine injection may be used as a quantitative assessment of liver function in children. We speculate that monoethylglycinexylidide formation may be a useful prognostic index in children with liver disease.

Adolescent↗

Efficacy of cornstarch therapy in type III glycogen-storage disease.

Type III glycogen-storage disease (GSD-III), due to decreased activity of the glycogen debranching enzyme amylo-1,6 glucosidase, may cause hepatic dysfunction, growth failure, and myopathy. The prevention of hypoglycemia by nocturnal intragastric formula infusion has been shown to enhance growth and improve the metabolic abnormalities associated with GSD-III. Cornstarch therapy was effective in preventing hypoglycemia in a few patients with GSD-III who were previously treated with nocturnal enteral formula infusion, but oral cornstarch had not been evaluated as an initial treatment. We studied three patients with GSD-III who exhibited growth failure, elevated serum aminotransferase concentrations, and asymptomatic hypoglycemia. Cornstarch therapy was associated with maintenance of normoglycemia, increased growth velocity, and decreased serum aminotransferase concentrations in all patients. Our experience suggests that cornstarch therapy can be effective as an initial treatment for patients with GSD-III.

Blood Glucose↗

Hypophosphatemia in the nutritional recovery syndrome.

We studied the incidence of hypophosphatemia in patients during the nutritional recovery syndrome. The charts of 150 patients receiving a complete nutritional assessment for 18 months were reviewed; 45 met established nutritional risk criteria. Only 9 of these 45 had serial phosphorus values measured during nutritional repletion, and 5 of these 9 patients had hypophosphatemia (phosphorus levels less than 0.97 mmol/L). Anthropometric measurements of arm circumference and arm muscle circumference were less than the fifth percentile in all patients developing hypophosphatemia. We concluded that hypophosphatemia is an underrecognized complication of nutritional repletion and that anthropometric measurements may be predictive of patients at risk. All patients with significant malnutrition should be evaluated for this complication of refeeding.

Adolescent↗

Papillary stenosis and sclerosing cholangitis in an immunodeficient child.

Sclerosing cholangitis, an inflammatory disease of the biliary tree that occurs infrequently in childhood, has been recognized in combination with papillary stenosis in adults with the acquired immunodeficiency syndrome. A 10-yr-old child with a familial immunodeficiency syndrome characterized by defective T-cell function and deficiencies of immunoglobulins A and G developed papillary stenosis and sclerosing cholangitis associated with cryptosporidium enteritis. The patient presented with fever, jaundice, right upper quadrant pain, and elevated serum concentrations of transaminases and alkaline phosphatase. The pain and jaundice resolved after endoscopic sphincterotomy, but the biochemical abnormalities persisted. This case demonstrates that the combination of papillary stenosis and sclerosing cholangitis can occur in children as well as adults and may be associated with immunodeficiency syndromes other than the acquired immunodeficiency syndrome. Endoscopic sphincterotomy can provide symptomatic treatment for papillary stenosis in children with this condition, although the effect of sphincterotomy on the natural history of the sclerosing cholangitis is uncertain.

Ampulla of Vater↗

Sulfasalazine hypersensitivity with hepatotoxicity, thrombocytopenia, and erythroid hypoplasia.

Sulfasalazine, a commonly prescribed drug for the treatment of inflammatory bowel disease, can cause an allergic reaction with hepatotoxicity, lymphadenopathy, and rash. In addition, many hematologic complications have been observed with sulfasalazine, including aplastic anemia, thrombocytopenia, and erythroid hypoplasia. However, thrombocytopenia in association with sulfasalazine hypersensitivity has not been previously reported. We report a child who developed severe thrombocytopenia and anemia during a hypersensitivity reaction to sulfasalazine.

Anemia↗

Neonatal gallbladder enlargement and alpha 1-antitrypsin deficiency.

Patients with clinical signs of alpha 1-antitrypsin deficiency in the neonatal period usually present with prolonged obstructive jaundice. We report a patient with alpha 1-antitrypsin deficiency who presented with gallbladder enlargement in the neonatal period. This gallbladder enlargement may be due to cystic duct hypoplasia or atresia, which has been reported in association with alpha 1-antitrypsin deficiency. The diagnosis of alpha 1-antitrypsin deficiency should be considered in neonates with gallbladder enlargement and prolonged obstructive jaundice.

Gallbladder Diseases↗