Angel R. Colon

Lipase and pepsin activity in the gastric mucosa of infants, children, and adults

The range of activity and the location of lipase and pepsin were determined in the stomach and duodenum of infants, children, and adults. The range of lipase activity in biopsy specimens from the gastric body, in 29 subjects aged from 3 months to 26 years, was 1.8-5.3 U/mg protein (1 U is 1 mumol [3H]oleic acid released from tri-[3H]olein per minute). There were no significant differences among age groups (5-19 months, 2-4 years, 6-10 years, 11-13 years, and 15-26 years). Lipase activity was low or undetectable in the gastric antrum of all subjects. Pepsin activity in specimens from the gastric body ranged from 180 to 780 pepsin units/mg protein (using hemoglobin as substrate). The antrum had significantly lower pepsin activity (P less than 0.001) than the gastric body. As with lipase activity, there were no statistically significant differences in pepsin activity among age groups. Lipase and pepsin activity was also quantified in pinch biopsy specimens from the duodenum and duodenal bulb in 13 subjects. Contrary to lipase activity, which was almost completely absent from the duodenum or duodenal bulb, these sites contained low pepsin activity (9-78 pepsin units/mg protein). The data show that in infants and children, as previously reported in adults, gastric lipase is localized primarily in the gastric body. Tissue pepsin levels and localization, reported here for the first time, are similar to those of lipase, although, contrary to lipase, the gastric antrum has considerable pepsin activity. The identical levels of lipase and pepsin activities in infants, children, and adults indicate that the gastric phase of nutrient digestion is well developed at birth.

Using the radionuclide salivagram to detect pulmonary aspiration and esophageal dysmotility

That the radionuclide “milk” scan is insensitive for aspiration has been demonstrated. Here the authors review their experience with the radionuclide salivagram in its ability to detect aspiration in children. Tc-99m sulfur colloid, 0.5 to 1.0 mCi in less than 1 mL, is instilled into the mouth and sequential supine posterior images of the thorax are obtained for an hour with delayed images until the oropharynx is cleared of radiotracer. Fourteen studies have been performed in 13 patients aged 1 month to 6.5 years. There are scintigraphic findings consistent with aspiration in 4 of 14 studies (28%); dysmotility (prolonged retention of activity in the esophagus) in 7 of 14 studies (50%); and normal studies in 3 of 13 patients (22%). Eight of 13 patients had milk scans; all were negative for aspiration. One patient studied twice had aspiration on the first examination, and dysmotility on the second study. It is concluded that the salivagram can detect aspiration of oral secretions, is superior to the milk scan in detecting aspiration, and can demonstrate esophageal dysmotility.

Chronic Fibrosing Pancreatitis in Childhood: Report of a Case and Literature Review

Chronic fibrosing pancreatitis in childhood is an uncommon condition of unknown etiology with a variety of clinical presentations, histopathologic features, and outcomes. The diagnosis is one of exclusion (of hereditary or secondary pancreatitis), which should include histological assessment. The histological features of this condition have been described, to our knowledge, in nine published cases. We report a case in a 13-year-old male, who presented with obstructive jaundice and subsequently had evidence of endocrine and exocrine pancreatic insufficiency, despite a surgical decompression of the pancreatic-biliary duct system.

Efficacy of Hepatobiliary Scintigraphy (HIDA) in the Evaluation of Cholestasis in Very Low Birth Weight (VLBW) and Extremely Low Birth Weight(ELBW) Infants 586

Efficacy of Hepatobiliary Scintigraphy (HIDA) in the Evaluation of Cholestasis in Very Low Birth Weight (VLBW) and Extremely Low Birth Weight(ELBW) Infants 586

413 HEMOCARBOPERFUSION IN CHILDHOOD HEPATIC COMA

Fulminant hepatic failure and coma in children has a mortality rate of 60 to 70%. Hemocarboperfusion (HCP) employing an acrylic hydrogel polymer coated charcoal column was used in three children ages 8,11, and 13 years presenting with acute encephalopathy and hepatic failure. Two children were in stage IV hepatic coma and one in stage V Reye syndrome. Following HCP all patients showed significant improvements in deranged serum chemistries. Serum ammonia and amino acid concentrations decreased dramatically. Serial examinations revealed no evidence of hemolysis, thrombocytopenia, or microembolization secondary to HCP. One of three patients survived and had no evidence of cerebral edema. The other two patients developed posturing and pupillary changes suggestive of severe cerebral edema prior to HCP. Continuous intracranial pressure monitoring (employing Richmond screw) in one patient showed > 50mmHg (N:0-10) which transiently diminished with mannitol infusion but failed to respond to HCP. The patients who expired had nearly isoelectric EEGs which showed no improvement following HCP.The patient who responded to HCP and had no evidence of cerebral edema is well without complications after a two year period. It is concluded that for effective HCP in children with hepatic encephalopathy, the therapy should be initiated before the onset of cerebral edema.

535 RING CHROMOSOME 12 SYNDROME

Advances in the development of banding techniques have facilitated the identification of a number of new chromosomal abnormalities. We studied a 13 mo. old girl with a history of failure to thrive, developmental delay and dysmorphic features. Chromosomal analysis revealed ringed chromosome 12. J.T. was the product of an uncomplicated second pregnancy of a 24 yr. old woman. Birth weight and height were close to the 3rd percentile. At 13 mos. her weight age was 1 mo., height age was 3 mos. and head circumference was at 3rd percentile for 3 mos. She had epicanthal folds, mildly cupped low set ears, high arched palate, short neck with low set hairline. Total hand length was that of a 1.5 mo. old and clinodactyly and single crease of left 5th finger. Developmentally she functioned between 5-8 mos. The chromosome number was 46 in all cells and trypsin banding showed a ring 12 chromosome. Twelve percent of cells showed variable chromosome loss and 4 showed gaps or breaks in chromatids. The purpose of this report is to delineate the clinical findings and natural history of ring chromosome 12 syndrome. There are only two known cases of this syndrome with which we compared our findings. It appears that the amount of genetic material deleted from chromosome 12 determines the degree of physical abnormalities and developmental delay. The relatively high rate of the loss of the ring 12 points out the fragility of the ring chromosome.