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Biomedical subjects

M B Heyman

Publications and source records attributed to M B Heyman.

At least 55 records · Page 3Linked to original sources

Mediastinal pancreatic pseudocysts in children.

Mediastinal pseudocyst is an unusual complication of pancreatitis, with only four cases previously reported in children. The extent of the pseudocyst can be defined by computed tomography or magnetic resonance imaging scan and preoperative aspiration of cyst fluid for amylase level can establish the diagnosis. Endoscopic retrograde cholangiopancreatography to define ductal anatomy can help plan the appropriate drainage procedure. Although exceedingly rare, the diagnosis of pseudocyst should be considered for any cystic mass in the abdomen or thorax, even in the absence of elevated amylase or history suggesting pancreatitis.

Amylases↗

Hypersensitivity reaction in an infant fed hydrolyzed lactalbumin contained in a semielemental formula.

Following introduction of milk protein formula feedings, a 6-month-old male developed profuse, watery diarrhea progressing to shock, requiring cardiopulmonary resuscitation. Reinstitution of enteral feedings with a formula containing hydrolyzed lactalbumin (Travasorb STD) resulted in recurrence of diarrhea with fever. Intestinal and rectal biopsies showed only nonspecific inflammatory changes. He was discharged on an elemental formula (Vivonex). Twenty-three months later, while admitted for evaluation of hypophosphatemic rickets, immunologic testing using the lymphocyte migration inhibition factor (LIF) test demonstrated positive reactions, especially to alpha-lactalbumin (56% inhibition) and whole cow's milk (22%, normal of less than 20% inhibition). Skin tests revealed sensitivity to cow's milk and eggs. Soy formula also produced diarrhea and bloody stools. Protein hydrolysate formulas, touted as hypoallergenic diets, are useful in infants with intolerance to milk protein. This is the first documented case of an immunological reaction to the hydrolyzed whey protein, lactalbumin. Although protein hydrolysate formulas are effective treatment in most infants with milk protein intolerance, allergic reactions are possible. Caution and close observation should be exercised in immunologically sensitized infants rechallenged with any formula.

Food Hypersensitivity↗

Relapsing post-hepatitis aplastic anemia. Immunosuppressive therapy.

A 20-month-old Hispanic male developed severe aplastic anemia after an episode of non-A, non-B hepatitis. Prompt and complete recovery of all hematopoietic cell lines occurred after treatment with antithymocyte globulin (ATG) and high-dose corticosteroids. Severe aplasia recurred two months later coincident with a mild upper respiratory infection. A second course of immunosuppressive therapy was followed by complete, sustained improvement. The authors' experience provides clinical evidence indicating that immunologic mechanisms are important in the treatment of severe post-hepatitis aplastic anemia. Children in whom aplastic anemia recurs after immunosuppressive treatment may respond to a second course of therapy.

Adrenal Cortex Hormones↗

General and specialized parenteral amino acid formulations for nutrition support.

Advances in the understanding of amino acid metabolism and of the interaction of amino acids with skeletal muscle, liver, brain, and other tissues have led to refinements of parenteral amino acid solutions. Clinical situations may dictate the use of specific amino acid formulations. Branched-chain amino acid (BCAA) solutions may normalize aberrant amino acid profiles in patients with hepatic encephalopathy; however, controlled trials demonstrate little effect on clinical outcome, and the effectiveness in patients with acute liver failure or undergoing orthotopic liver transplantation is unproved. BCAA solutions have also been tried in septic and severely stressed patients with equivocal results. Renal failure has been treated with essential amino acid solutions, yet low-dose standard amino acid formulations are probably equally effective. Pediatric preparations have been tailored to "normalize" amino acid profiles to those of healthy term, breast-fed neonates. Recent studies suggest that premature infants receiving these formulations may achieve intrauterine growth rates, although the effect on long-term outcome is unknown. Glutamine may be essential for the preservation of intestinal mucosal structure and function; further study is indicated to determine the necessity of adding glutamine to parenteral amino acid solutions. Recently, amino acid infusions have been associated with enhanced ventilatory drive, possibly via stimulation of central ventilatory mechanisms. A variety of other side effects have been documented, including acidosis, hyperammonemia, hypercalciuria, and possibly bone disease and hepatotoxicity. Further understanding of the metabolism of intravenous infusion of amino acids is necessary to provide optimal nutritional protein support. Because full information regarding the complex effects of intravenous substrates is lacking, special amino acid formulations must be used with caution.

Amino Acids↗

Hyperplastic gastric polyps associated with persistent Helicobacter pylori infection and active gastritis.

We report two cases of patients with 3-yr histories of upper gastrointestinal symptoms, hyperplastic gastric polyps, and active chronic gastritis. Biopsies retrospectively stained with Giemsa revealed the persistent presence of Helicobacter pylori (HP) in gastric biopsies of both patients throughout the 3 yr. After treatment with amoxicillin and bismuth subsalicylate, both became asymptomatic, one demonstrating disappearance and recurrence of the gastric polyps in conjunction with the HP. These cases demonstrate 3 yr of hyperplastic gastric polyps associated with HP and active gastritis.

Aged↗

Gastrointestinal duplications causing relapsing pancreatitis in children.

Two cases of children with relapsing pancreatitis due to intramural gastrointestinal duplications with ductal communication to the pancreas are reported. A gastric duplication with ectopic pancreatic tissue was detected by endoscopic cholangiopancreatography in the gastric antrum of a 6-yr-old girl. A periampullary duodenal duplication was visualized preoperatively by duodenoscopy and computed tomography in a 10-yr-old boy. Resection of the duplication was curative in each case.

Animals↗

Elevated fasting breath hydrogen and abnormal hydrogen breath tests in children with sickle cell disease: a preliminary report.

Hydrogen breath tests were performed in eight children with sickle cell disease (Hgb S-S), four of whom were growth retarded. Average base-line breath H2 values after an overnight fast were elevated (22.4 +/- 10.8 ppm; normal = 7.1 +/- 5.0). Breath H2 concentrations increased significantly above base line within 30-40 min after lactulose ingestion in the four growth-retarded children whereas a negligible rise was observed in the four with normal growth indices. Breath H2 production for each time interval in the first 120 min was greater in all Hgb S-S then in normal children (p less than 0.01 for each time interval measured before 60 min). The results indicate that children with sickle cell disease have intestinal abnormalities favoring excess production of H2 in the fasted state combined with early elevations in postlactulose breath H2 in those with growth retardation. The possible role of disordered gastrointestinal motility and/or anomalously distributed intestinal flora in growth retardation of children with Hgb S-S requires further investigation.

Adolescent↗

Nutritional support for pediatric patients with inflammatory bowel disease.

Pediatric patients with ulcerative colitis and Crohn's disease often suffer from malnutrition and growth failure. This is particularly true in pubertal children. Chronic insufficient nutrient intake is most often the cause of growth failure. Both parenteral nutrition and defined enteral formulas are available to rehabilitate patients with malnutrition and growth failure. Assessment of nutritional status and growth and the use of parenteral nutrition and defined enteral formulas to reverse malnutrition, growth failure, and inflammation in pediatric patients with inflammatory bowel disease are discussed.

Adolescent↗

Altered glycine and taurine conjugation of bile acids following aluminum administration to rats.

Aluminum contaminates components of intravenous nutrient solutions and accumulates in the liver with parenteral feeding. Abnormalities in hepatic function associated with aluminum accumulation include increased serum bile acid concentration and glucuronyl transferase activity and reduced mixed function oxidase levels and bile flow. Whether there are other biochemical responses of the liver to aluminum is unclear. We report the effects of aluminum administration on bile acid conjugation in rats given aluminum intravenously as follows: group I, 1 mg/kg/day for 14 days; group II, 5 mg/kg/day for 14 days; and group III, 5 mg/kg/day for 7 days. Taurine-conjugated bile acids were reduced and glycine/taurine elevated in all groups compared with pair-fed controls. Glycine/taurine was greater in group II versus III and varied directly with serum bile acid concentration. These findings suggest that aluminum administration is associated with decreased taurine conjugation of bile acids, a phenomenon that may be associated with cholestasis.

Aluminum↗

Trends in Campylobacter pylori in pediatric and adult antral biopsies. A 5-year retrospective analysis.

Campylobacter pylori has been associated with chronic gastritis and antral ulceration in adults and has recently been reported in children with primary antral gastritis and duodenal ulceration. We reviewed all gastric antral biopsy specimens from children (n = 30) and adults (n = 77) over the past 5 years at the University of California, San Francisco. Tissue sections were stained with Giemsa to detect C pylori, and medical histories were obtained by chart review. The prevalence of C pylori in antral biopsy specimens with gastritis increased from 22% during the period 1983 to 1986 to 66% in 1987 (P less than .001). In all specimens showing gastritis, C pylori appeared more frequently in adults (31/67 [46%]) than in children (4/17 [24%]). In cases with primary gastritis, however, the prevalence of C pylori reversed to 57% (4/7) in children versus 49% (31/63) in adults. Most children (10/13) with gastritis and no C pylori had predisposing conditions. Infection with this organism was associated with more active inflammatory changes in adults than in children, but it may contribute to most cases of hitherto-unexplained gastritis in children. Further studies are needed to determine whether the prevalence of C pylori is increasing.

Adult↗

Polyethylene glycol-electrolyte solution for intestinal clearance in children with refractory encopresis. A safe and effective therapeutic program.

Severely constipated children with encopresis in whom outpatient management has failed frequently require several days of hospitalization, as well as conventional treatments involving cathartics and enemas. A balanced electrolyte solution of the nonabsorbable polymer polyethylene glycol (GoLytely, Braintree [Mass] Laboratories Inc) offers a safe and efficient method for clearing the intestine. Twenty-four patients with a mean age of 8.1 years (range, 0.8 to 17.6 years) and an average weight of 31.5 kg received polyethylene glycol-electrolyte solution: eight patients with encopresis were given it as treatment for severe fecal retention unresponsive to outpatient management; the other 16 were being prepared for colonoscopy. Weight, complete blood cell count, and serum electrolyte, serum urea nitrogen, and serum creatinine levels were measured before solution administration and two hours afterward. Abdominal roentgenograms were obtained from the encopretic patients. The two groups were comparable in age and weight. Polyethylene glycol-electrolyte solution was given at a rate of 14 to 40 mL/kg/h until clear fluid was excreted through the rectum. Intestines were cleaned out successfully in all patients, and side effects were minimal. Children with encopresis required an average of 11.8 L (574 mL/kg) given over 22.5 hours, while the other children needed only 4.0 L (128 mL/kg) given over 7.5 hours. Weight and serum electrolyte and creatinine levels did not change significantly in any patient. The hematocrit decreased only in encopretic patients (0.38 to 0.36 [37.6% to 35.8%]) and the serum urea nitrogen level decreased in the patients undergoing colonoscopy (3.6 to 2.0 mmol/L [10 to 8 mg/dL]).

Adolescent↗

Aluminum-associated hepatobiliary dysfunction in rats: relationships to dosage and duration of exposure.

Aluminum may contaminate parenteral nutrition solutions and accumulate in bone and liver of patients receiving this therapy. Although aluminum exposure is associated with low-turnover osteomalacia, there are few studies of hepatotoxicity. We therefore studied the effects of aluminum given to rats on total serum bile acid concentration and bile flow to determine if aluminum administration could produce abnormalities. Aluminum was given intravenously as follows: 5 mg/kg daily for 7 or 14 days and 1 mg/kg for 14 days. Hepatic aluminum was high in treated rats and undetectable in controls. Total serum bile acid concentrations were significantly higher in treated rats than in pair-fed controls with higher concentrations after 14 days than after 7 days. Bile flow was reduced by 33% in rats given 5 mg/kg but not in rats given 1 mg/kg. Hepatic aluminum correlated inversely with bile flow but not with serum bile acid concentration. Aluminum exposure in rats is associated with elevated serum bile acid concentration and diminished bile flow and may play a role in the pathogenesis of parenteral nutrition-induced hepatobiliary dysfunction.

Aluminum↗

Long-term percutaneous biliary drainage in an infant with hemangioendothelioma.

A 5-month-old female infant with jaundice (bilirubin = 11) and dilated intrahepatic and extrahepatic bile ducts was explored. A 3-cm mass in the head of the pancreas and portal region completely obstructed the cystic and common bile ducts. Cholecystectomy was done and biopsy of the tumor and regional nodes revealed hemangioendothelioma with no nodal involvement. After tissue diagnosis was made, the infant's biliary tract was decompressed percutaneously. External drainage was maintained for 22 months until the tumor regressed. This infant is the youngest patient ever reported to undergo percutaneous biliary drainage.

Catheters, Indwelling↗

Chronic nonspecific inflammatory bowel disease of the cecum and proximal colon in children with grossly normal-appearing colonic mucosa: diagnosis by colonoscopic biopsies.

The diagnosis of inflammatory bowel disease rests on radiologic, endoscopic, and histologic criteria. Five patients, 2 to 17 years of age, sought medical attention because of chronic abdominal pain, diarrhea, and heme-positive stools. Rectal biopsies, visual inspection of colonic mucosa through the colonoscope, and contrast radiographs of the large and small intestine yielded nonspecific results. Serial endoscopic biopsies demonstrated a gradient of inflammatory changes diminishing in severity distally from the ileocecal valve and cecum. The disease process was most evident in specimens from the cecum, whereas biopsies distal to the transverse colon had a normal histologic appearance in all five patients. Biopsies from the proximal colon may provide evidence of inflammatory bowel disease not detectable using standard techniques. The combination of chronic abdominal pain, diarrhea, and heme-positive stools associated with inflammatory changes in biopsy specimens obtained from the proximal colon, but normal findings on radiologic, colonoscopic, and rectal biopsy examinations, may represent an early stage in the evolution of chronic nonspecific inflammatory bowel disease, including ulcerative colitis or regional enteritis (Crohn disease).

Adolescent↗

Primary hemochromatosis in childhood.

Primary hemochromatosis is a genetic disorder rarely recognized in childhood; its long-term consequences include cirrhosis and liver cancer. We report a family with primary hemochromatosis affecting three generations, including a 7-year-old child and a 29-month-old child; these are the youngest children with primary hemochromatosis yet reported. The pathophysiology, genetics, and clinical findings of this disorder are reviewed. Serum ferritin and transferrin saturation are useful screening tests; definitive diagnosis, however, depends on determination of hepatic iron content. A plan for evaluating and treating affected patients is proposed. Physicians caring for children must learn to recognize this potentially treatable disorder.

Adult↗

Sclerosing cholangitis associated with chronic cryptosporidiosis in a child with a congenital immunodeficiency disorder.

Primary sclerosing cholangitis (PSC) in children is a rare and incurable disorder of unknown etiology. We report an immunodeficient child with chronic cryptosporidiosis of the biliary tract leading to clinical, pathological, and radiographic findings consistent with PSC. This case documents the ability of Cryptosporidium to disseminate to extraintestinal organs, and suggests that chronic cryptosporidial infection of the biliary tract may be one etiological mechanism producing sclerosing cholangitis in immunodeficient children. The increased incidence of PSC in immunodeficient children may in part be due to their inability to resolve infections of the biliary tract, which may result in sclerosing cholangitis mimicking PSC. We submit that an aggressive diagnostic workup should be performed to rule out an infectious etiology of sclerosing cholangitis in immunodeficient patients who have findings of PSC, because specific chemotherapy against the infecting organism would potentially arrest progressive biliary obliteration.

Adolescent↗

Dysphagia lusorum in children.

The association of difficulty in swallowing and an aberrant right subclavian artery is termed dysphagia lusorum. Dysphagia lusorum has been reported not to occur in children. We describe four children who had difficulty swallowing and aberrant right subclavian arteries. Esophageal manometry showed high pulsatile pressure in the area of the aberrant right subclavian artery in each child (12 to 100 mm Hg). Three of the four children underwent surgical correction, and their symptoms resolved. Postoperatively, esophageal manometric findings were normal. We conclude that dysphagia lusorum occurs in children and esophageal manometry shows persistently increased intraesophageal pressure, causing a functional partial obstruction in symptomatic children with dysphagia lusorum.

Cardiac Catheterization↗