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Biomedical subjects

M Frank

Publications and source records attributed to M Frank.

At least 127 records · Page 7Linked to original sources

[Cholestatic erythrohepatic protoporphyria: porphyrin metabolism before and after liver transplantation].

The ferrochelatase deficiency in protoporphyria leads to accumulation of protoporphyrin in erythrocytes and liver. Consequences are protoporphyrinemia with photosensitivity and liver damage (fibrosis, cirrhosis) with cholestasis. The latter are unpredictable and can be observed in about 10% of the patients. Protoporphyrin, the physiological main component of hepatocellular porphyrins, has a hepatotoxic effect in the high-concentrated crystalline storage form. The obligatory hepatobiliary excretion of the lipophil, erythropoietic increased accumulating protoporphyrin in protoporphyria strains the excretory function of the liver. Its restriction is followed by an exzessive protoporphyrin accumulation, which leads to protoporphyrin-induced, progressive cholestatic cirrhosis, icterus, and aggravation of the extrahepatic protoporphyrinemic cutaneous manifestation. In case of hepatobiliary complications a coproporphyria of diagnostic relevance develops with inversion of isomers. Simultaneously, the fecal protoporphyrin excretion decreases. After liver transplantation hyperbilirubinemia, protoporphyrinemia and coproporphyrinuria significantly went down. A protoporphyrinemia of about 20% of preoperative values reflects the persisting hereditary enzyme defect and the continuity of the metabolic disease.

Adult↗

[Liver transplantation in erythrohepatic protoporphyria].

A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver.

Cholestyramine Resin↗

Operative intervention in active endocarditis in children: report of a series of cases and review.

We describe in detail 14 children (age, less than or equal to 19 years) who underwent operative intervention during active infection of the heart and/or great vessels. The series included five children less than 6 years old, who constitute 10% of all such cases reported in this age group to date. We also review the 132 published reports in which children underwent operative intervention during active endocarditis. We found the following: (1) The survival rate for all cases was 77%. (2) Persistent infection, embolic phenomena, and increasing congestive heart failure were the most frequent indications for operative intervention. (3) Survival rates were independent of the duration of preoperative antibiotic treatment. (4) Survival rates were independent of positive results of cultures of blood or tissue obtained at operation. (5) The perioperative mortality in our series was 14%. (6) Only 67% of patients had conditions thought to predispose to endocarditis. (7) Except for removal of catheter-associated cardiac masses from neonates, operative intervention in active endocarditis was uncommon among children less than 4 years old. (8) Staphylococcus aureus and viridans streptococci were the etiologic agents in the majority of cases of endocarditis requiring operation during active infection in children.

Adolescent↗

Adaptation and renewal of the endocrine stomach.

At present at least seven different endocrine cell types have been identified in the stomach. According to their relative frequency and secretion products the antral gastrin producing G cell and somatostatin producing D cell and the fundic histamine producing ECL cell are the best characterized cell types. Total endocrine cell mass is controlled by various factors from inside and outside the stomach. Density of antral G and D cells depends on the presence and absence of food, on the antral pH and on additional humoral and/or neural factors. Gastrin and not gastric pH has been identified as the most important factor regulating the density of fundic ECL cells. Adaptation of gastric endocrine cells to gastric pH and to the presence, abundance or absence of humoral and neural regulators are well known phenomena though only partially understood. Antral G cells increase and antral D cells decrease during long-term achlorhydria which as a consequence leads to hypergastrinaemia. Examples are pernicious anaemia in man and drug-induced acid suppression under experimental conditions. Interestingly, achlorhydria-induced G cell hyperplasia never progresses to gastrinomas. Fundic ECL cell density increases markedly in the presence of long-lasting hypergastrinaemia independently of gastric pH. In contrast to G cells ECL cell hyperplasia may progress to rarely occurring ECLomas. However, this depends on additional conditioning factors as the presence of severe atrophic gastritis as in pernicious anaemia or a specific genetic trait present in patients with gastrinomas associated with the MEN I syndrome.

Adaptation, Physiological↗

Gambling and pathological gambling among university students.

Students from six colleges and universities in five states in the U.S. (New York, New Jersey, Oklahoma, Texas, and Nevada) were surveyed concerning their gambling behavior and the rate of pathological gambling. Type of gambling varied by state, with students in the northeast and Nevada gambling more than students in Oklahoma and Texas. Over 90% of males and 82% of females had gambled. One third of the males and 15% of females gambled once a week or more. Rates of pathological gambling ranged from 8% in New York to 4% in Nevada. The incidence of pathological gambling was high among males, Hispanics, Asians, and Italian-Americans (compared with among other whites), students with non-traffic arrests, those with parents who have gambling problems, and those who abuse alcohol and other drugs. Pathological gambling was only weakly correlated with age, religion, lower grade point average in school, overeating, living in neighborhoods that are "poorer than most," family income, and parental drug use. It was not correlated with academic year in college, marital status, parental occupation, parental alcohol, and bulimic behavior. The implications of the findings for further research and social policy are discussed.

Achievement↗

Pathological laughter.

A man experienced one hour and 40 minutes of continual, inappropriate, uncontrollable laughter. The onset was preceded by a single-inhalation exposure to an insecticide of very low toxicity. The episode was terminated by a single dose of IV diazepam. A discussion of pathological laughter, including its proposed pathophysiology, differential diagnosis, clinical relevance, and management, is presented.

Adult↗

Panic attacks and related disorders in alcohol-dependent, depressed, and nonclinical samples.

Although several studies have indicated that a substantial portion of alcoholics have an anxiety disorder, relatively little information exists specifically regarding panic disorders. In addition, prior studies have been marred by the absence of appropriate contrast groups. The present investigation compared the lifetime prevalence of panic attacks and panic-related disorders diagnosed according to DSM-III criteria in a group of 79 alcohol-dependent patients, 64 depressed patients, and 70 nonclinical subjects. Panic attacks, panic disorder, and agoraphobia with panic attacks were more prevalent in the alcohol-dependent and depressed samples than among nonclinical subjects. Men in both clinical samples were more likely than women to have had nonagoraphobic panic disorder, but male alcoholics were less likely to have developed agoraphobia than were female alcoholics or depressed patients of either sex. No consistent chronological relationship between onset of panic attacks and alcohol abuse was found. Results indicate that there is an unusually high prevalence of panic attacks and panic-related disorders among alcoholics, but comparable prevalence rates can be found in depressed and perhaps some other psychiatric populations. Implications for the assessment and treatment of alcohol-dependent and panic-disordered patients are discussed.

Adolescent↗

Hordenine: pharmacology, pharmacokinetics and behavioural effects in the horse.

Hordenine is an alkaloid occurring naturally in grains, sprouting barley, and certain grasses. It is occasionally found in post race urine samples, and therefore we investigated its pharmacological actions in the horse. Hordenine (2.0 mg/kg bodyweight [bwt]) was administered by rapid intravenous (iv) injection to 10 horses. Typically, dosed horses showed a flehmen response and defecated within 60 secs. All horses showed substantial respiratory distress. Respiratory rates increased about 250 per cent and heart rates were approximately double that of resting values. All animals broke out in a sweat shortly after iv injection, but basal body temperature was not affected. These effects were transient, and the animals appeared normal within 30 mins of dosing. Treated horses were tested in a variable interval responding apparatus 30 mins after dosing and no residual stimulation or depressant effects of hordenine were apparent. Animals dosed orally with 2.0 mg/kg bwt of hordenine showed no changes in heart rate, respiratory rate, basal body temperature or behaviour. After iv injection of hordenine, (2.0 mg/kg bwt) plasma reached a maximum value of about 1.0 micrograms/ml, and declined thereafter in a biexponential fashion. Kinetics of plasma concentration satisfied the concept of a two compartment open system, with an alpha-phase half-life of about 3 mins, and a beta-phase half-life of about 35 mins. Total urinary concentrations of hordenine (free and conjugated) peaked at about 400 micrograms/ml, and then declined exponentially to background levels by 24 h after dosing.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗