Biomedical subjects

J C Partin

Publications and source records attributed to J C Partin.

At least 19 recordsLinked to original sources

PubMed1992

Painful defecation and fecal soiling in children.

Fecal soiling is a common complaint among school-age children. The fecal soiling is often accompanied by chronic constipation and so-called "idiopathic," "functional," or "psychogenic" megacolon, the cause of which is undetermined. The records of all children presenting to a pediatric gastroenterology clinic between 1981 and 1990 with difficult defecation were reviewed to determine the incidence of painful defecation and its relationship to chronic impaction and fecal soiling. There were 227 children; 74 were younger than 36 months of age and 153 were older than 36 months. Of the younger children, 86% presented with pain, 71% with impaction, and 97% with severe withholding. The younger children had painful defecation for a mean of 14 +/- 9 (SD) months before presentation. Of the older children, 85% presented with fecal soiling, 57% with pain, and 73% with fecal impaction, and 96% exhibited withholding; the older children had difficult defecation for a mean of 56 +/- 42 months before presentation. Sixty-three percent of the children presenting with fecal soiling had a history of painful defecation beginning before 36 months of age. Painful defecation frequently precedes chronic fecal impaction and fecal soiling in American children. Early, effective treatment of painful defecation in infancy might reduce the incidence of chronic fecal impaction and fecal soiling in school-age children.

Adolescent↗

PubMed1990

Plexiform neurofibromatosis of the liver and mesentery in a child.

Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11-yr-old boy who had a 2-yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium-surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver.

Biopsy, Needle↗

PubMed1985

Prognosis and diagnosis of Reye syndrome by discriminant analysis.

Discriminant analysis was used to discriminate between Reye syndrome (RS) patients and non-RS cases based either on conventional blood chemistry data obtained upon admission, or on the activities of hepatic mitochondrial enzymes in biopsy or necropsy tissue. The control group for blood chemistry measurements contained children with upper respiratory tract infections, varicella, etc. who did not develop RS, as well as healthy children. Subjects with no liver disorder (e.g., accidental death, sudden infant death, etc.) or with non-RS liver disorders were used as controls for hepatic enzyme studies. Hepatic damage indicators (aspartate aminotransferase, AST; alanine aminotransferase, ALT; and bilirubin) correctly classified 86-96% of non-RS cases and 61-71% of RS. By contrast, AST and ALT had little prognostic value (63% overall correct). Ammonia effectively classified favorable outcome cases (95% correct) but not unfavorable (14% correct). However, when ammonia was included with stage of coma information 88% of the favorable and 85% of the unfavorable outcome cases were correctly classified. Discriminant analysis of hepatic enzymes (glutamate dehydrogenase and monoamine oxidase activity) for a RS and a non-RS group correctly classified 80% of non-RS and 95% of RS specimens. The function was suitable for the direct evaluation of RS-like mitochondrial enzyme changes in rat liver.

Ammonia↗

PubMed1985

Quantitative evaluation of the extent of hepatic enzyme changes in Reye syndrome compared with normal liver or with non-Reye liver disorders: objective criteria for animal models.

Five enzymes were measured in 50 liver specimens (18 normal liver, 20 Reye liver, 12 diverse liver disorders other than Reye syndrome). The enzymes were: glutamic dehydrogenase (E.C. 1.4.1.3), monoamine oxidase (E.C. 1.4.3.4), lactate dehydrogenase (E.C. 1.1.1.27), D-glucose-6-phosphate dehydrogenase (E.C. 1.1.1.49), catalase (E.C. 1.11.1.6). The Reye syndrome group showed significant decreases in glutamic dehydrogenase (56%) and monoamine oxidase (70%) compared to normal control tissue and these changes were not characteristic of the non-Reye liver disorder group as a whole. Neither catalase nor lactate dehydrogenase appeared to be altered significantly in the Reye or in the abnormal control group compared with normal controls. Thus, only the prominent decreases in the mitochondrial enzyme activities appeared to be highly characteristic of Reye syndrome. Paradoxically, the means of the five hepatic enzymes and the admission levels of two serum enzymes indicative of liver damage (alanine and aspartate aminotransferase) were remarkably similar for both survivors and nonsurvivors of Reye syndrome.

Adolescent↗

PubMed1985

Hepatic and encephalopathic components of Reye's syndrome: factor analysis of admission data from 209 patients.

Factor analysis of admission data from 209 Reye's syndrome patients yielded three factors. Factor 1 was associated with encephalopathy, blood ammonia, creatinine kinase (CK), uric acid and, to a lesser extent, bilirubin. This factor was linked to the encephalopathy and hypermetabolic changes in muscle, possibly prostaglandin-mediated proteolysis. Factor 2 was associated with serum alanine aminotransferase (AlaAT) and aspartate aminotransferase (AspAT), and was identified as a hepatic lesion component. These factors correspond to two etiologic components of Reye's syndrome. Salicylate was only weakly associated with neuropathic and hypercatabolic indicators and not at all associated with the hepatic damage indicators.

Brain Diseases↗

PubMed1985

Acute hepatic failure in children.

Explore the source record for details and available documents.

Acute Disease↗

PubMed1985

Management of Reye's syndrome: need for early diagnosis and intravenous treatment of stage I non-comatose cases.

Clinicians and nurses should obtain a history of antecedent illness occurring within 2 weeks of the onset of vomiting. Ninety percent of school-age children will give a history of an antecedent illness (varicella or influenza-like respiratory illness) within 1 week of the onset of vomiting. The vomiting of Reye's syndrome is usually persistent, lasting for 24 to 96 hours before the onset of serious brain signs. We believe that any child with the history of flu or chickenpox within 1 week of the onset of vomiting, which lasts for more than 12 hours, and is unusually severe or is associated with lethargy, should have an SGPT (alanine aminotransferase). This laboratory measure is clearly elevated in most cases of Reye's syndrome.

Aspirin↗

PubMed1984

Grade I Reye's syndrome--outcome and predictors of progression to deeper coma grades.

We studied 83 biopsy-proved cases of Grade I Reye's syndrome to determine the outcome, possible clinical or laboratory predictors of progression to deeper coma grades, and hepatic ultrastructural findings. Seventy-eight patients had no change in coma grade during hospitalization, whereas five (6 per cent) had progression to deeper coma grades. All the patients survived without sequelae except one who sustained severe brain damage. The mean (+/- S.E.) level of serum ammonia on admission was significantly higher (P = 0.005) in patients whose disease progressed to deeper neurologic grades (291 +/- 42 micrograms per deciliter) than in those whose disease did not so progress (53 +/- 5 micrograms per deciliter), and the corrected prothrombin time was significantly more prolonged (P = 0.005) in patients with progressing coma (3.9 +/- 0.5 seconds) than in those whose coma grade did not change (1.6 +/- 0.2 seconds). The combination of a prothrombin time 3 seconds or longer than that of the control and a serum level of ammonia on admission of 100 micrograms per deciliter or more correctly predicted progression in 71.5 per cent of the cases (sensitivity, 100 per cent; specificity, 97.6 per cent). Our findings suggest that the prognosis is excellent for survival without sequelae in Grade I Reye's syndrome (98.8 per cent) when management includes hospital surveillance and intravenous glucose and electrolyte infusion.

Ammonia↗

PubMed1983

Hypocalcemia and steatorrhea--clues to etiology.

Two boys with idiopathic hypoparathyroidism had extensive studies of gastrointestinal function during hypocalcemia accompanied by steatorrhea. No evidence of generalized gastrointestinal moniliasis or abnormal mucosal structure or function was observed. Studies of pancreatic function and bile salt metabolism during hypocalcemia demonstrated deficient meal-stimulated intraluminal pancreatic enzyme concentrations in both subjects and reduced bile salt concentrations in one subject. However, following stimulation with exogenous octapeptide of cholecystokinin, intraluminal pancreatic enzyme and bile salt concentrations were normal in both. Cholic acid pool sizes were markedly increased in both subjects during hypocalcemia (9 and 12 times larger than during normocalcemia) and cholic acid turnover was reduced during hypocalcemia in one subject. Our findings suggest that during hypocalcemia, insufficient endogenous cholecystokinin is released by the duodenal mucosa during a meal stimulus to stimulate normal gallbladder contraction and pancreatic enzyme secretion.

Adolescent↗

PubMed1983

An ultrastructural study of enteropathogenic Escherichia coli infection in human infants.

Over the past 2 years, we have studied and treated 18 infants with protracted diarrhea due to an enteropathogenic Escherichia coli serogroup 0119. All patients had persistent stool escretion and jejunal over-growth with this pathogenic E. coli. Jejunal biopsy revealed atrophy of villi with a chronic inflammatory cell infiltrate in the lamina propria. E. coli 0119 adhered to the luminal surface of enterocytes. Electron microscopy showed disappearance of glycocalyx and microvilli at the areas of bacterial adherence. Intracellular damage was indicated by dilatation of rough endoplasmic reticulum, mitochondrial changes, and cytoplasmic pallor. Similar changes in histology and ultrastructure occurred in ileal epithelial cells. Glandular crypt epithelium showed prominent subnuclear vacuolation and separation of lateral intercellular junctions throughout the small intestine. Rectal mucosal biopsy showed mucus depletion and irregular atrophy of the epithelium, with E. coli 0119 adherent to the luminal surface. Ultrastructural damage paralleled that in the small intestine. E. coli 0119 causes damage to epithelial cells throughout the infant intestinal tract. This damage leads to atrophy of villi and a marked reduction in absorptive surface area, resulting in protracted diarrhea.

Colon↗

PubMed1982

Serum salicylate concentrations in Reye's disease. A study of 130 biopsy-proven cases.

Serum salicylate concentration was measured at admission in 130 children with liver-biopsy-confirmed Reye's disease. Mean serum salicylate was 12.3 mg/dl and mean salicylate concentrations by neurological grade (Lovejoy) were: stage I, 12, stage II, 13, stage III, 11, stage IV, 13, and stage V, 13 mg/dl. However, mean serum salicylate (15 mg/dl) at admission in 21 patients who died or had serious neurological deficits was significantly higher than that in 103 patients who survived without neurological sequelae (10 mg/dl). Serum salicylate in a group of 27 age-matched, community-matched control children collected consecutively over the period 1978-80 was less than 2 mg/dl, and children with varicella or influenza had salicylate concentrations indistinguishable from apparently well classmates or siblings. It is impossible to determine from this data whether salicylates are involved in the aetiology of or in determining the outcome of Reye's disease. Increased concentrations of salicylates at admission could be the result of excessive dosage because of a greater severity of the prodromal illness, or to diminished excretion because of impaired hepatic metabolism. It seems likely that serum salicylate concentrations entered the toxic range in many patients with Reye's disease before they presented for treatment. Most had been vomiting and had diminished oral intake for 33-55 h before hospital admission. Since the average number of hours from the beginning of vomiting to admission was no different in non-comatose and comatose cases, the time at which salicylate concentration was measured in relation to the last dose was probably similar in the two groups and therefore does not account for the higher levels in children with poor outcome. Salicylates are mitochondrial toxins and mitochondria are known to be significantly injured in Reye's disease; therefore, it seems wise to avoid the use of aspirin in children during outbreaks of Reye's disease.

Biopsy↗

PubMed1979

Refractory infantile diarrhea due to primary bile acid malabsorption.

A boy is described who had severe, refractory diarrhea beginning soon after birth. Prolonged parenteral alimentation was required to support life. Investigations of bile acid metabolism showed impaired intestinal absorption of bile acids. Contracted bile acid pool sizes resulted, with low intraluminal bile acid concentrations and severe, malabsorption of water and fat. Bile acid malabsorption is a cause of refractory infantile diarrhea.

Bile Acids and Salts↗

PubMed1979

Neuropsychologic consequences of Reye syndrome.

Behavioral measurement of brain function was conducted in 40 children, one or more years after their recovery of Reye syndrome. Test measures included standard indices of intelligence, school achievement, visual-motor coordination and social maturity, plus the Halstead-Reitan Neuropsychological Batteries. There was a strong correlation between the degree of impaired neuropsychologic function and clinical grade at admission, the duration of impaired consciousness, and the number of exchange transfusions required. Patients with milder disease had normal brain function and fewer school problems. Language and perceptual-motor performance significantly improved with increasing years in recovery, suggesting that some of the disturbances of brain functioning are transient. The statistical analysis indicated that there are lasting, often subtle disturbances of higher cognitive function as a result of Reye syndrome. These deficits, not always apparent on clinical examination, are clearly correlated with the extent of neurologic involvement. This quantitative assessment of neuropsychologic function is a basis for determining the "quality of survival" in Reye syndrome, and such measurements should be included in the comparative evaluation of Reye syndrome treatment programs.

Achievement↗

PubMed1979

Small intestinal mucosal fatty acid uptake and esterification in infants and children.

Oleic acid uptake and esterification in intact intestinal mucosa were studied in 14 infants and children with chronic non-specific diarrhea, but histologically normal small intestinal mucosal biopsies, using an in vitro technique. The uptake rate was 5.876 +/- 1.942 nmol fatty acid/mg Nigrogen/minute and the esterification rate was 4.060 +/- 1.010 nmol fatty acid/mg Nitrogen/minute, comparable to previous adult esterification studies. No effect of age on either esterification or uptake was present. Mucosal injury resulted in significant reductions in esterification (p less than 0.001) and uptake (p less than 0.05) compared to controls. Bile acid deficiencies led to reductions in mucosal esterification (p less than 0.05) but not uptake.

Bile Acids and Salts↗

PubMed1977

Abnormal lipid accumulation within the small intestinal mucosa of children with juvenile-onset diabetes mellitus.

The lipid content of the proximal jejunum, as studied by light microscopy with lipid histochemical staining methods, is determined in 14 fasting children with juvenile-onset diabetes mellitus. The results show that four children had greatly increased amounts of lipid, while six others had a mildly to moderately increased amounts of lipid present. The amount of lipid present appears to correlate most closely with the quality of control of the diabetes. These results indicate either that the clearance of exogenous lipid is impaired or that there is an increased endogenous synthesis of lipid within the intestine of the diabetic child, especially in those under poor control.

Adolescent↗

PubMed1977

Bile acid malabsorption--a consequence of terminal ileal dysfunction in protracted diarrhea of infancy.

Fecal excretion of labeled bile acid (14C-24-cholic acid) was distinctly increased in two infants with protracted diarrhea, whereas four patients with steatorrhea due to pancreatic or mucosal abnormalities and five patients with mild chronic diarrhea had no excess fecal loss of bile acid. The loss of 14C-24-cholic acid in our patients with intractable diarrhea was similar to that observed in four infants who had undergone ileal resection. The ratio of mean 24-hour excretion of bile acid to that of a non-absorbable marker, polyethylene glycol, confirmed the malabsorption of bile acid in the patients with intractable diarrhea or ileal resection. These results differ significantly (p less than 0.05) from excretion ratios obtained in patients with either steatorrhea or chronic diarrhea. The extent of the loss of bile acid was not significantly related to the rate of fecal fat excretion. There was no direct correlation of fecal weight with the rate of bile acid excretion. Ileal function, as further assessed by the Schilling test with exogenous intrinsic factor, was grossly abnormal in both of the patients with intractable diarrhea.

Bile Acids and Salts↗

PubMed1977

Hepatic encephalopathy and Reye's syndrome.

The encephalopathy of Reye's syndrome is a potentially reversible, treatable problem in many cases. Treatment requires (1) accurate, early diagnosis; (2) the prevention of anoxia; (3) the restitution of body glycogen stores through the administration of glucose; (4) the control of the complicating cerebral edema; (5) the avoidance of counterproductive treatment measures, such as the unwarranted infusion of sodium bicarbonate and the inappropriate use of respiratory depressing anticonvulsants; and (6) the early and aggressive use of exchange transfusion.

Child↗

PubMed1976

Isolation of influenza virus from liver and muscle biopsy specimens from a surviving case of Reye's syndrome.

Influenza virus (A/Ohio/7/76) was demonstrated by haemagglutination and by direct electron microscopy in chick embryo fluids inoculated with liver, muscle, cerebrospinal fluid, and nasotracheal secretions obtained from a boy with Reye's syndrome, 8 days after the onset of the upper-respiratory-tract symptoms and at the height of neurological and hepatic involvement. This finding accords with the hypothesis that Reye's syndrome may be an epiphenomenon of influenza-virus infection. It is postulated that the pathogenesis of Reye's syndrome may be related to the other influenzal eipiphenomena including influenzal pneumonia in previously healthy persons and influenza-associated myopathy, both of which, like Reye's syndrome, become clinically evident about 7 days after the first clinical signs of influenza.

Brain Diseases↗