Biomedical subjects
Publications and source records attributed to M B Heyman.
A method was developed to obtain purified cryptosporidium oocysts from fecal samples. Oocysts were initially collected by centrifugation through a sucrose density gradient and further purified by passage through glass bead columns. The purified oocysts were antigenically active and sufficiently pure for immunological studies.
The effect of increased nutritional intake was evaluated in 5 growth-retarded children with sickle-cell disease. Growth on recommended daily calorie and protein intakes had been inadequate in all 5. Fat absorption and intestinal mucosal morphology were normal in all 5. 2 children were given nutritional supplementation by nasogastric intubation, 1 received nightly oral formula supplements, and 2 were supplemented with zinc, iron, folate, and vitamin E only. Nutritional supplementation by the nasogastric route produced a rapid sustained increase in growth rate, associated with striking reductions in pain crises and infections which had previously necessitated many hospital admissions. Oral supplementation improved the clinical course but had no effect on growth rate. Mineral and vitamin supplements influenced neither the growth rate nor the clinical course. The observations indicate that nasogastric nutritional supplementation may accelerate growth and reduce the incidence and severity of complications in growth-retarded children with sickle-cell disease.
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A newborn infant was found to have diffuse esophageal spasm in association with apnea and bradycardia. This association has not been physiologically documented before. The infant's condition improved after the administration of glycopyrrolate, an anticholinergic agent. It is speculated that the association of esophageal spasm and bradycardia may be mediated by the vagus.
Infection with cryptosporidium occurred in 12 immunocompetent persons who had direct contact with the feces of infected calves during three unrelated outbreaks of calf cryptosporidiosis. Nine of the twelve subjects had diarrhea and abdominal cramps that lasted 1 to 10 days. Infections were diagnosed and monitored by detection of oocysts in feces, with a modified Sheather's flotation technique and phase-contrast microscopy. Oocysts of cryptosporidium were isolated from calves but not from other animals with which these subjects had been in contact. Oocysts of cryptosporidium were also detected during repeated examinations of feces from two immunodeficient patients with persistent cryptosporidiosis. An apparently identical infection was transmitted to calves and mice, using oocysts from infected calves and human beings. Oocysts from an immunodeficient person also produced infections in kittens, puppies, and goats. This study shows that cryptosporidium may produce a moderate self-limited illness in immunocompetent persons, which contrasts sharply with the prolonged severe diarrhea in immunocompromised patients who contract cryptosporidiosis. Calves with diarrhea should be considered a potential source of human infection, and immunocompromised persons should avoid contact with such animals.
A 1-year-old boy was evaluated because of failure-to-thrive and persistent diarrhea. His illness was characterized by autonomic dysfunction sharing some features of both familial dysautonomia and congenital sensory neuropathy with anhidrosis, but was consistent with neither diagnosis. The gastrointestinal tract was involved: esophageal motility was abnormal and moderate secretory diarrhea was present. This report documents an unusual case of congenital autonomic dysfunction with secretory diarrhea.
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A 10-month-old infant with hypoplasia of the intestinal mucosa had the fat overload syndrome develop while receiving intravenous fat emulsion at a dosage of 5 g/kg/day of fat for five weeks. This syndrome was characterized by fever, jaundice, easy bruisability, increased levels of serum transaminases, conjugated hyperbilirubinemia, and abnormal results of clotting studies. Management consisted of withdrawal of parenteral nutrition for 72 hours, followed by gradual reinstitution of protein and subsequent introduction of fat at a lower dosage.
A 2-year-old girl with persistent vomiting of solid foods was evaluated; the diagnosis was esophageal muscular ring. This rare congenital anomaly and its differentiation from esophageal stricture are discussed. This case represents a variation of the esophageal malformation associated with the VACTERL association.
OBJECTIVE: To investigate the effects of aging on mechanisms of body energy regulation and thereby determine the causes of unexplained weight loss in older persons, a factor predisposing to premature death and disability. DESIGN: Dietary intervention study. SETTING: Metabolic ward and outpatient. PARTICIPANTS: The subjects were 35 healthy younger and older men of normal body weight consuming a diet of typical composition and performing usual activities. MAIN OUTCOME MEASURES: Subjects were either overfed by a mean (+/- SD) of 4.09 (+/- 0.26) MJ/d (n = 17) or underfed by 3.17 (+/- 0.68) MJ/d (n = 18) for 21 days. Measurements were made of changes in body weight, body composition, and energy expenditure during overfeeding or underfeeding, and of subsequent voluntary nutrient intakes and changes in body weight. RESULTS: There was no significant effect of aging on changes in body composition, body weight, or energy expenditure with overfeeding or underfeeding. However, following overfeeding, younger men exhibited spontaneous hypophagia, whereas the older men did not (mean [+/- SD] changes in energy intake relative to control values were -2.11 [+/- 2.18] and 1.55 [+/- 2.11] MJ/d, respectively; P = .006). As a result, the younger men lost the excess body weight gained during overfeeding but the older men did not. Similarly, following underfeeding, the younger men exhibited hyperphagia while the older men did not (mean [+/- SD] changes in energy intake relative to control values were 1.88 [+/- 2.31] and -0.52 [+/- 1.54] MJ/d, respectively; P = .02), and as a result the older men failed to regain the weight lost during underfeeding. CONCLUSIONS: These results in 35 men suggest that aging may be associated with a significant impairment in the ability to control food intake following overeating or undereating. Since overeating and undereating occur routinely as part of the normal pattern of energy regulation, the findings reported herein may help to explain the vulnerability of older persons to unexplained weight gain and weight loss.
The differentiation of infantile biliary malformations from primary parenchymal diseases is difficult. The recent development of a pediatric side-viewing endoscope (PJF Endoscope; Olympus Corporation of America) provided an opportunity to investigate the usefulness of endoscopic retrograde cholangiography (ERC) for precise visualization of the extrahepatic biliary passages in infants with persistent cryptogenic cholestasis. ERC was performed in 12 patients, with visualization of the existing extrahepatic bile ducts in 4. The entire biliary system was visualized in one, excluding extrahepatic biliary atresia and choledochal cyst. The reduced caliber of the intrahepatic bile ducts and histological observations in a percutaneous liver biopsy supported the diagnosis of intrahepatic biliary hypoplasia in this case. An intact hepatic portochole cystostomy was documented in one, although the intrahepatic biliary system was not delineated. Atresia of the hepatic bile ducts proximal to the gallbladder was documented in two. Of the eight patients in whom extrahepatic bile ducts were not visualized by ERC, six had extrahepatic biliary atresia confirmed at exploratory laparotomy. The papilla of Vater could not be located in four of these six infants. The remaining two had neonatal hepatitis. ERC may offer a useful alternative to operative cholangiography in selected infants with persistent cholestasis and acholic stools.
Prolonged total parenteral nutrition (TPN) with solutions containing hydrolyzed casein has been associated with aluminum accumulation in patients with bone disease. We investigated the effects of free amino acids in TPN solution on plasma, urine, and bone aluminum in six patients, five of whom had symptoms of bone disease or documented demineralization. No evidence of aluminum accumulation was found. TPN with free amino acids, containing 42 micrograms aluminum per liter or less, does not lead to aluminum loading in adolescents or adults.
Cholestasis is a common complication of total parenteral nutrition (TPN) in infants. A contributing factor to the hepatic dysfunction may be a contaminant of the TPN solution, such as aluminum, that accumulates in liver and may act as a hepatotoxin. To study the hepatic effects of aluminum, growing piglets were given daily intravenous injections of aluminum, 1.5 mg/kg, for 50 days; pair-fed controls were given heparinized saline. At sacrifice, liver and serum were obtained. Liver was analyzed for histopathology and for aluminum content and localization. The hepatocyte lysosomes of the experimental group showed aluminum peaks by x-ray microanalysis, whereas the control group did not. No differences in ultrastructure were noted between the two groups when examined by electron microscopy. Mean serum total bile acid levels (27.8 +/- 15.9 SD vs 6.3 +/- 1.5 mumol/liter, p less than 0.05), mean alkaline phosphatase (309 +/- 108 vs 180 +/- 27 IU/liter, p = NS), and mean hepatic copper content (24.8 +/- 4.5 vs 14.4 +/- micrograms/g dry weight, p less than 0.01), were elevated in the aluminum-loaded piglets, indicating that cholestasis may have been produced. Also, a small but significant reduction in serum levels of 25 hydroxy-vitamin D was found in the aluminum-loaded piglets, suggesting that vitamin D hydroxylation may be impaired. Inasmuch as lysosomal contents are excreted into the bile, aluminum accumulation in lysosomes may alter lysosomal function and possibly affect bile flow or content.
Two infants were diagnosed with dumping syndrome by a radionuclide gastric emptying method. Both patients presented with anorexia, weight loss, agitation and diaphoresis following bolus feeding by gastrostomy tube. One had documented hyperglycemia and glycosuria. Symptoms and signs of dumping in one patient were due to a gastrostomy placed in the antrum, whereby bolus tube feedings were inadvertently introduced directly into the duodenum. The second patient developed dumping symptoms after a Waterston colonic interposition was performed to correct a long gap esophageal atresia. Gastric emptying, measured by administering 99mTc-sulfur colloid-labeled formula, demonstrated an initial extremely rapid appearance of isotope in the small intestine, with greater than one-third of the formula leaving the stomach in less than 2 min. The gastric emptying pattern in both patients appeared biphasic; after the initial "dumping" phase, the remaining formula emptied slowly, with monoexponential decay kinetics.
Gastrostomy tubes have improved technically over the last 10 years. New to the market are skin-level devices, which are low-profile in design and avoid many problems of standard gastrostomy tubes. Two skin-level devices, the Button and the MIC-KEY, are appropriately designed for infants and children and are compared.
Eleven patients (4 males, 7 females) with Wilson's disease who presented before 18 years of age are described. The mean age onset of symptoms was 11.2 +/- 3.9 (SD) years. The mean age at diagnosis was 13.3 +/- 3.4 (SD) years. All patients had hepatic manifestations of the disease when diagnosed: cirrhosis (6 patients), chronic hepatitis (2) and fulminant hepatic failure (3). Three patients were asymptomatic at diagnosis. Two of the symptomatic patients presented with new undescribed manifestations: one with blurred vision and the other with acalculous cholecystitis. At diagnosis, 6 patients had Kayser Fleischer rings and 5 had hemolytic anemia. The three patients with fulminant hepatic failure had hemolysis with relatively low serum aminotransferase and alkaline phosphatase levels, possibly helpful findings for rapid diagnosis of Wilson's disease in such presentation. Ten patients were treated with penicillamine. Liver transplantation was performed in 4 patients, 2 of which presented with fulminant hepatic failure. One patient died while waiting for liver transplantation, the remainder of the patients live free of symptoms. It is important to be aware of the different manifestations of Wilson's disease in the pediatric population, in order to make appropriate evaluations in a timely manner to facilitate early diagnosis and appropriate treatment.