Biomedical subjects

J C Partin

Publications and source records attributed to J C Partin.

At least 37 records · Page 2Linked to original sources

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↗

PubMed1976

Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.

Since Reye's syndrome is associated with hyperammonemia, we measured the urea-cycle enzymes in hepatic tissue of 13 patients. Expressed as nanomoles of citrulline per milligram of hepatic protein per minute, mean activity of carbamyl phosphate synthetase (6.27 +/- 2.45 S.D.) and ornithine transcarbamylase (136.19 +/- 41.83) in Reye's syndrome was reduced significantly (P less than 0.005) when compared with that of 25 "normal" controls (11.54 +/- 4.24 and 307.49 +/- 94.15, respectively). Activity was maximally reduced during the first days of clinical symptoms; it returned toward normal during the following week regardless of whether the disease ended in death or recovery. The activity of the two enzymes was normal in patients with salicylate intoxication or heritable argininosuccinic acid synthetase deficiency. The apparent Km of hepatic ornithine transcarbamylase for ornithine was in the normal range in patients with Reye's syndrome (mean 0.24 mM). These observations indicate that Reye's syndrome is associated with acquired and transient dysfunction of hepatic mitochondrial urea-cycle enzymes.

Age Factors↗

PubMed1975

Reye's syndrome.

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Adolescent↗

PubMed1975

Reye syndrome: treatment by exchange transfusion with special reference to the 1974 epidemic in Cincinnati, Ohio.

The treatment of 66 children with Reye syndrome proved by hepatic biopsy or autopsy is described. Prior to the utilization of exchange transfusion early in the course of the disease, our case fatality rate was 100% of nine patients. With early diagnosis and early exchange transfusion, the case fatality rate was reduced to 27% of 44 patients. During the 1974 epidemic of Reye syndrome, 26 children were treated. In 18 children the diagnosis was established by hepatic biopsy; 16 received one or more exchange transfusions. There were no deaths among these 26 patients. In the 1974 epidemic, the national case fatality rate was estimated to be 40%. Exchange transfusion appears to have been an important factor in the reduction of the case fatality rate among our patients.

Biopsy, Needle↗

PubMed1975

Reye's syndrome: epidemiologic and viral studies, 1963-1974.

Fifty-eight children with Reye's syndrome (RS) confirmed by liver biopsy were treated at the University of Cincinnati between 1963 and 1974. Cases were clustered in the winter and spring with the peak in February and March. These coincided with the occurrence of influenza and, numerically, were associated more closely with influenza B than with influenza A. Six of the 58 cases were associated with chickenpox. Twenty-six children with RS were seen from 1963 to 1971, before the beginning of a systematic epidemiologic and virologic study. Viral infection was documented in either the patient or a contact in only 19% of those studied. Between 1971 and 1973, after the study was initiated, 16 cases of RS were diagnosed and viral infection was confirmed in 56% of these. In 1974, an epidemic of RS occurred during an influenza B epidemic and viral infection was found in either the patient or a contact in 81% of 16 cases. This study demonstrates that an association with viral infection can be proven in the majority of cases of RS when an intensive investigation is undertaken. During this study no significant environmental toxic exposures could be idenified. Most children had taken aspirin and other medications, and seven children had a history of excessive aspirin ingestion.

Adolescent↗

PubMed1975

Brain ultrastructure in Reye's syndrome.

Cerebral biopsies were obtained for electron microscopy 48 and 72 hours after the onset of encephalopathy from a child with severe Reye's syndrome. Gravely ill at the time of craniectomy to relieve cerebral hypertension, the child survived and recovered good brain function; therefore, the biopsy findings appear to reflect the organelle pathology of the brain at a severe yet reversible stage in the disease process. The cardinal ultrastructural changes in the brain in Reye's syndrome are astrocyte swelling and partial deglycogenation, myelin bleb formation and universal injury of neuron mitochondria. The mitochondrial injury consists of matrix disruption with moderate but not massive swelling. Dilatation of rough endoplasmic reticulum and nuclear changes occurred only in neurons with severely altered mitochondria. The organelle pathology of the brain in this case did not resemble the organelle pathology of the brain in human "hepatic encephalopathy" or in experimental ammonia intoxication in primates. The mitochondrial ultrastructure of the cerebral neurons resembled the unique mitochondrial ultrastructural changes seen in the liver parenchyma in Reye's syndrome.

Brain↗

PubMed1975

Reye's syndrome (encephalopathy and fatty liver). Diagnosis and treatment.

Reye's syndrome is an acute encephalopathy and fatty liver which has mainly been recognized in children through the age of about 16 years. Since the patients are not jaundiced and the liver may be relatively small at the moment of admission, the diagnosis must be sought by a high index of suspicion and the routine use of liver function studies, especially the SGOT, in all unexplained cases of encephalopathy. Early treatment and appropriate management of cerebral edema seem to reduce the over-all mortality of Reye's syndrome from more than 50% to less than 20%. Alert gastroenterologists may find some adult cases of Reye's syndrome masquerading as acute neurological disease or supposed acute drug reactions.

Adolescent↗

PubMed1975

The hepatic lesion in Reye's syndrome.

The hepatic lesion in Reye's syndrome (acute encephalopathy with fatty degeneration of viscera) was studied by light microscopy of sequential biopsy specimens obtained in 49 children. The hepatic lesion is a morphologically characteristic, rapidly evolving, and reversible toxic hepatitis. In specimens obtained with 48 hr of onset of neurological deterioration, the severity of the diffuse microvesicular steatosis is best appreciated in frozen sections stained for lipid content. Variation in severity of hepatocyte glycogen depletion in early biopsies correlates with other histological measures of severity, and with the occurrence of hypoglycemia, severity of the encephalopathy at the time of admission, and mortality rate. Histochemical studies suggest that the hepatic lesion is attributable to mitochondrial injury and other evidence that supports this hypothesis is briefly reviewed. The etiology of the syndrome and its relationship to the viral disease which usually precedes it are unknown.

Brain Diseases↗

PubMed1974