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Biomedical subjects

C A Riely

Publications and source records attributed to C A Riely.

At least 19 recordsLinked to original sources
PubMed1992

Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count)

OBJECTIVE: Our objective was to categorize the histologic findings in the liver in patients with HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count) and to correlate these findings with the severity of clinical laboratory abnormalities. STUDY DESIGN: Eleven patients with laboratory criteria for HELLP syndrome who required cesarean delivery underwent needle biopsy of the liver under direct visualization. RESULTS: Eight patients had periportal hemorrhage, and six had fibrin deposition. Fatty infiltration was seen in four, one with large-droplet fat, three with microvesicular fat. There was no statistically significant correlation between the severity of the histologic findings of periportal hemorrhage and fibrin deposition and the clinical laboratory findings. Fatty infiltration did not correlate with the severity of the HELLP syndrome's histologic condition, but, in contrast, did correlate with thrombocytopenia and aminotransferase elevations. CONCLUSIONS: Laboratory abnormalities do not accurately reflect the severity of the underlying histopathologic condition in HELLP syndrome. We propose that all patients with HELLP syndrome, regardless of the degree of their laboratory abnormalities, be treated aggressively, primarily with delivery.

Adolescent↗
PubMed1992

Baking powder pica mimicking preeclampsia.

We report a case of baking powder pica during pregnancy that was associated with maternal hypertension, hypokalemia, and elevated liver function tests. After discontinuation of baking powder ingestion and correction of electrolyte abnormalities, the blood pressure and the liver function tests normalized.

Adult↗
PubMed1992

Hyperemesis gravidarum.

Hyperemesis gravidarum is a poorly understood disorder and one for which many physicians have little sympathy, perhaps because we find it difficult to understand its pathogenesis and to treat. It may be associated with dysfunction in a variety of organs, including the thyroid and liver. Many theories, prominently including psychological or behavioral abnormalities, have been proposed to explicate this syndrome. On examination, however, no single theory seems to provide an adequate explanation for hyperemesis gravidarum. Treatment for hyperemesis gravidarum includes fluid and electrolyte supplementation, nutritional support, and total parenteral nutrition, as well as psychological and behavioral therapies and, in selected cases, pharmacotherapy.

Female↗
PubMed1991

Herpes simplex virus hepatitis in pregnancy. Two patients successfully treated with acyclovir.

Two cases of herpes simplex virus hepatitis in pregnancy are presented. Each case was characterized by extremely high serum aminotransferase levels with minimal bilirubin elevation. In both cases, liver biopsy was instrumental in arriving at the diagnosis. In addition, computed tomography showed a radiographic appearance of the liver not characteristically seen in other hepatic disorders of pregnancy. A high index of suspicion in the second case led to early recognition and treatment. Despite the presence of fulminant liver failure and evidence of herpes encephalitis in the other case, institution of therapy with acyclovir was associated with complete recovery in both patients. The present cases are compared and contrasted with the literature. The incidence of two cases within a 6-month period suggests that herpes simplex virus hepatitis in pregnancy may occur more frequently than previously reported.

Acyclovir↗
PubMed1991

Insulin resistance in noncirrhotic idiopathic portal hypertension.

To explore further the pathogenesis of glucose intolerance and insulin resistance observed in patients with cirrhosis and portal hypertension, we studied a 35-year-old woman with presinusoidal portal hypertension without cirrhosis due to nodular regenerative hyperplasia of the liver. After oral glucose ingestion, glucose tolerance remained normal; however, this occurred at the expense of a markedly hyperinsulinemic plasma response, suggesting the presence of insulin resistance. To examine this question more directly, we performed a stepwise euglycemic insulin clamp study in combination with an infusion of [6-3H]glucose and [1-14C]palmitate and indirect calorimetry. The ability of insulin to promote total body (primarily muscle) glucose uptake was markedly impaired, whereas its effect to suppress hepatic glucose production was normal compared with results obtained in nine healthy subjects. Moreover, insulin failed to normally suppress plasma free fatty acid turnover and oxidation in this patient. This informative case demonstrates that portal hypertension alone, without hepatic dysfunction from cirrhosis, is associated with impaired insulin-mediated glucose and plasma free fatty acid metabolism and may also play a predominant role in the development of insulin resistance in many cirrhotic patients.

Adult↗
PubMed1991

Effect of physiologic hyperinsulinemia on glucose and lipid metabolism in cirrhosis.

Insulin secretion and insulin sensitivity were evaluated in eight clinically stable cirrhotic patients and in 12 controls. OGTT was normal in cirrhotics but plasma insulin response was increased approximately twofold compared with controls. Subjects received a three-step (0.1, 0.5, 1.0 mU/kg.min) euglycemic insulin clamp with indirect calorimetry, [6-3H]-glucose, and [1-14C]-palmitate. During the two highest insulin infusion steps glucose uptake was impaired (3.33 +/- 0.31 vs. 5.06 +/- 0.40 mg/kg.min, P less than 0.01, and 6.09 +/- 0.50 vs. 7.95 +/- 0.52 mg/kg.min, P less than 0.01). Stimulation of glucose oxidation by insulin was normal; in contrast, nonoxidative glucose disposal (i.e., glycogen synthesis) was markedly reduced. Fasting (r = -0.553, P less than 0.01) and glucose-stimulated (r = -0.592, P less than 0.01) plasma insulin concentration correlated inversely with the severity of insulin resistance. Basal hepatic glucose production was normal in cirrhotics and suppressed normally with insulin. In postabsorptive state, plasma FFA conc (933 +/- 42 vs. 711 +/- 44 mumol/liter, P less than 0.01) and FFA turnover (9.08 +/- 1.20 vs. 6.03 +/- 0.53 mumol/kg.min, P less than 0.01) were elevated in cirrhotics despite basal hyperinsulinemia; basal FFA oxidation was similar in cirrhotic and control subjects. With low-dose insulin infusion, plasma FFA oxidation and turnover failed to suppress normally in cirrhotics. During the two higher insulin infusion steps, all parameters of FFA metabolism suppressed normally. In summary, stable cirrhotic patients with normal glucose tolerance exhibit marked insulin resistance secondary to the impaired nonoxidative glucose disposal. Our results suggest that chronic hyperinsulinism may be responsible for the insulin resistance observed in cirrhosis.

Adult↗
PubMed1990

Clinical and pathological characteristics of hepatotoxicity associated with occupational exposure to dimethylformamide.

The clinical characteristics, laboratory results, and liver biopsy findings of seven workers with toxic liver injury associated with exposure to several solvents, including substantial levels of the widely used solvent dimethylformamide, are presented. Three patients had short exposure (less than 3 months), four long exposure (greater than 1 year). Among those with brief exposure, symptoms included anorexia, abdominal pain, and disulfiram-type reaction. Aminotransferases were markedly elevated with the ratio of alanine aminotransferase to aspartate aminotransferase always greater than 1. Liver biopsy showed focal hepatocellular necrosis and microvesicular steatosis with prominence of smooth endoplasmic reticulum, complex lysosomes, and pleomorphic mitochondria with crystalline inclusions. Among workers with long exposure, symptoms were minimal and enzyme elevations modest. Biopsies showed macrovesicular steatosis, pleomorphic mitochondria without crystalloids, and prominent smooth endoplasmic reticulum, but no evidence of persisting acute injury or fibrosis. Abnormal aminotransferases in both groups may persist for months after removal from exposure, but progression to cirrhosis in continually exposed workers was not observed. We conclude that exposure of these workers to solvents, chiefly dimethylformamide, may result in two variants of toxic liver injury with subtle clinical, laboratory, and morphological features. This may be readily overlooked if occupational history and biopsy histology are not carefully evaluated.

Adult↗
PubMed1990

The effects of early treatment of hereditary tyrosinemia type I in infancy by orthotopic liver transplantation.

Two infants with hereditary tyrosinemia secondary to fumarylacetoacetate hydrolase (FAH) deficiency underwent orthotopic liver transplantation at 14 and 16 weeks of age due to poor clinical and biochemical response to medical therapy. Prompt clearance of abnormal metabolites with improved mental alertness and appetite occurred with minimal perioperative complications. Both infants tolerated rapid institution of normal diets and have shown progressive growth and development in the first 36 months after transplantation. Early liver transplantation should be considered as an option for infants with certain inherited metabolic disorders with poor prognosis, such as tyrosinemia type I, who fail to respond to medical therapy.

Amino Acid Metabolism, Inborn Errors↗
PubMed1989

Automated bibliographic retrieval based on current topics in hepatology: hepatopix.

The Hepatopix computer program helps the physician go from a computerized clinical record directly to a computerized search of the medical literature. The program uses a hierarchical list of current (key) topics in hepatology to offer "intelligent" searches. Each topic has associated "selection logic" and a tested Medline search. Starting with a liver biopsy case record, Hepatopix evaluates the selection logic to determine which topics may be pertinent to the case (based on clinical findings, lab tests, and critical words or phrases in the summary). The physician then picks those topics which are interesting enough to warrant a literature search. The citations are retrieved using search strategies stored for each topic and presented. Hepatopix is operational with over 200 topics in the realm of liver neoplasms, operating on liver biopsy case summaries from the Klatskin Database of Liver Biopsies. Besides demonstrating clinical case-directed bibliographic retrieval, it demonstrates the utility of a "key topics" list as a bridge between medical databases.

Bibliography of Medicine↗
PubMed1989

Pseudoportal Doppler signal in the partially obstructed inferior vena cava.

The Doppler spectrum of the normal inferior vena cava (IVC) shows a majority of flow toward the right atrium, with wide variations in flow velocity and direction owing to effects of the cardiac and respiratory cycles. Seventeen subjects underwent duplex sonography: nine children after liver transplantation, two children with right upper quadrant neuroblastoma, and six healthy control subjects. In six children the spectrum obtained from the IVC bore a strong resemblance to the unidirectional, low-velocity, continuous-flow pattern associated with the portal vein; these children included two children with biliary atresia and cirrhosis, two liver transplant recipients, and two children with IVC compression by neoplasm. In healthy children and adults, a pseudoportal Doppler signal in the IVC can be generated by subcostal right upper quadrant compression. The pseudoportal IVC appears to be a sign of partial IVC obstruction. Knowledge of this potential pitfall and meticulous morphologic imaging can help prevent mistaking the IVC for a patent portal vein.

Adult↗
PubMed1988

Semantic relationships and medical bibliographic retrieval: a preliminary assessment.

This paper describes a project exploring whether semantic relationships between bibliographic terms may effectively partition the clinical literature. To address this question, a set of semantic relationships was identified between pairs of bibliographic terms taken from four categories: (1) diseases, (2) treatments, (3) tests, and (4) patient characteristics. The MEDLINE system of the National Library of Medicine was used to generate lists of abstracts relating to pairs of clinical terms. Each list of abstracts was examined to identify the semantic relationships, if any, which applied to the two terms in each paper. The study suggests that semantic relationships may play a potentially valuable role in assisting computer-based medical bibliographic retrieval. The degree to which relationships partition the literature is strongly dependent on the underlying semantics of the particular bibliographic terms involved.

Information Systems↗