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Biomedical subjects

A Nemeth

Publications and source records attributed to A Nemeth.

At least 19 recordsLinked to original sources
PubMed1992

Liver transplantation in a boy with acute porphyria due to aminolaevulinate dehydratase deficiency.

The clinical and biochemical outcome of a liver transplantation in a seven-year-old boy with acute porphyria due to aminolaevulinate dehydratase deficiency is described. Before transplantation standard liver function tests were normal and the rationale for transplantation was that the new liver would reduce the metabolic disturbance and thus avert the porphyric symptoms. During the year after the transplantation, the functioning of the new liver has been excellent. Basal excretion of porphyrin and porphyrin precursors has remained unchanged but, with the new liver transplant the patient has been able to withstand several porphyrinogenic challenges without increasing the excretion. Episodes of neurological and respiratory crises may have been due to persistent porphyric vulnerability. Alternatively, two early attacks may have been caused by neurotoxic effects of cyclosporin in combination with the existing damage to nervous tissue.

Acute Disease↗
PubMed1992

Descriptive analysis of the tumor registry experience for malignant melanoma from 1977 to 1986 at the University of Miami School of Medicine/Jackson Memorial Hospital.

A descriptive analysis was performed of malignant melanoma data ascertained by the University of Miami School of Medicine/Jackson Memorial Hospital (UM/JMH) Tumor Registry. A total of 376 melanoma cases were collected and reviewed. Most of the melanoma lesions occurred on the trunk, especially in the 40- to 49- and 50- to 54-year-old age groups. Local-stage cases had the best 5-year survival--77%. The difference in survival between local-stage case and regional- and distant-stage cases was statistically significant (p = 0.0000). In males with local-stage disease, lesions on the trunk were associated with better survival than lesions at other sites (p = 0.04). In females with local-stage disease, survival was 68% for 5 years for trunk sites vs. 87% for other sites (p = 0.05). In local-stage disease, the overall 5-year survival was 85%.

Adult↗
PubMed1991

Analysis of multiple-choice items.

This paper deals with scoring and analysis of multiple-choice exam questions. Data may be read by an optical mark reader scanner or from a file created by a system editor and stored in a format convenient to be analysed by a specially designed program 'MCQ'. In addition the data file created can be linked with the package SPSSx for pertinent statistical methods or SPSS Graphics for illustration of score and item distributions. The MCQ program comprises: (1) scoring; (2) item analysis: difficulty and discrimination indices; (3) testing the goodness of alternatives attached to each question. The method is applicable to multiple-choice questions with 4.5 greater than 5; true/false and combined exam questions. The method should prove useful for instructors to build up a balanced discriminant questions bank.

Computer Graphics↗
PubMed1990

[Chronic atrophic gastritis and the risk of cancer].

Chronic gastritis, which is frequent in subjects over 50 years old, is caused by the concurrence of predisposing and congenital conditions and exogenous harmful factors, in particular foods. In etiopathogenetic terms it is worth considering autoimmune diseases and duodenogastric back-flow separately. Lesions develop progressively from superficial gastritis to atrophic gastritis and finally to gastric atrophy; they are frequently found together with intestinal metaplasia, formed by areas of the epithelium with the morphological and histochemical characteristics of intestinal mucosa, which are the expression of a modified regeneration of the gastric wall. It is acknowledged that chronic atrophic gastritis is a precancerous phenomenon which is the majority of cases leads to the onset of intestinal cancer, passing through the stages of chronic gastritis, metaplasia and dysplasia. Identification of this lesion may therefore help to prevent cancer: diagnosis is essentially performed using endoscopy (together with histocytological tests and bioptic staining) and laboratory tests (enzyme and CEA assays in the gastric juices). Rather than prescribing generic medical therapy or surgical treatment, which is only possible in selected cases of alkaline gastritis, attention is focused on curing unhealthy habits and on an endoscopic follow-up (every 2 years in cases of gastritis, and more frequently in cases of metaplasia or dysplasia).

Gastritis, Atrophic↗
PubMed1990

Phenobarbital can aggravate a cholestatic bile acid pattern in infants with obstructive cholangiopathy.

The effect of phenobarbital on urinary bile acid excretion in intrahepatic cholestasis was studied in four boys 4-43 months of age who received 10 mg/kg of body weight of phenobarbital for a period of 3 weeks-3 years. One child was observed at two different periods: with and without histologically proven cirrhosis. Before the treatment period, the infants excreted 10-fold higher amounts of bile acids in urine than healthy children. The primary bile acids predominated, and there were also increased amounts of polyhydroxylated bile acids, 3 beta-hydroxy-5-cholenoic acid, and ketonic bile acids but small amounts of secondary bile acids. After the phenobarbital treatment, the patients further increased their urinary bile acid excretion, including all kinds of bile acids except the secondary ones. The sulfated fraction did not increase in absolute amounts, and its relative percentage decreased from a mean of 60-33%. Liver function test results generally did not improve, although serum concentration of bilirubin decreased. Most of these changes suggested a worsening of the cholestatic state after phenobarbital treatment. The results indicate that at our present state of knowledge, phenobarbital should not be given routinely to infants or children with intrahepatic cholestasis.

Bile Acids and Salts↗
PubMed1990

Liver damage in juvenile inflammatory bowel disease.

During a 12-year period, 46 children and adolescents with inflammatory bowel disease were followed from the time of diagnosis with regular biochemical tests of liver function. Thirty-four patients had ulcerative colitis and 12 had Crohn's disease. Mean age at the time of diagnosis was 10.2 years (range 7 months-17 years) and the mean follow-up period was 5.2 years (range 1-11 years). Pathological liver function tests were found in 60% of the 34 patients with ulcerative colitis: 9 of these 20 patients demonstrated more severe disturbance, usually at the time of diagnosis. Liver damage was most frequent in patients with total colitis. Liver biopsy was performed in eight patients, demonstrating "pericholangitis", fibrosis and in one case cirrhosis. Morphometry of electron microscopical pictures revealed a significantly increased number of lysosomes and dilated cisternae of the rough endoplasmic reticulum. ERCP was performed in two patients, verifying primary sclerosing cholangitis in one. Four of the 12 patients with Crohn's disease had mildly pathological liver function tests. No correlation was found to the extent, duration or treatment of bowel disease. In our series of juvenile inflammatory bowel disease, liver damage occurred frequently, especially in ulcerative colitis. The more severe changes tended to coincide with the onset of bowel disease.

Biopsy↗
PubMed1989

Gallbladder function is altered in sickle hemoglobinopathy.

Despite comparable rates of hemolysis, only 50% of patients with sickle hemoglobinopathy (SH) develop pigment gallstones by age 20 yr. Thus, pathogenetic factors, other than hemolysis, may contribute to gallstone formation. In the present study we determined whether gallbladder function, measured by real-time ultrasonography or bile acid metabolism, determined by isotope dilution-mass spectrometry, were altered in adolescents and young adults with SH. Compared with healthy controls, SH subjects had larger fasting (27 +/- 16 vs. 15 +/- 5 ml, p less than 0.02), and residual (8 +/- 6 vs. 4 +/- 2 ml, p less than 0.03) volumes of the gallbladder, but similar rates of emptying (0.029 +/- 0.016 vs. 0.034 +/- 0.029 min-1) and percentage of fasting volume emptied (71% +/- 13% vs. 72% +/- 14%). In SH subjects, the volume and emptying of the gallbladder were similar between those with and without gallstones. Some SH subjects had stasis of bile within the gallbladder, as demonstrated by isotopic disequilibrium between the circulating bile acid pool and bile stored in the gallbladder. Subjects with SH with gallstones tended to have smaller bile acid pools than SH subjects without gallstones (81 +/- 11 vs 163 +/- 91 mumol/kg, p = 0.051). We conclude that adolescents and young adults with SH have enlarged gallbladders that retain an increased postprandial volume of bile. Bile retention within the gallbladder may lead to stasis and contribute to the pathogenesis of pigment gallstones.

Adolescent↗
PubMed1989

Immunofluorescence detection of digoxin with monoclonal digoxin specific antibody.

The present study primarily focuses on the analysis of digoxin binding of the heart muscle cells. The primary aim of the investigation was to demonstrate the cardiac glycoside morphologically. The direct immunofluorescence staining technique with digoxin specific monoclonal antibody or Fab fragments and FITC or Texas-Red conjugated antisera are useful for morphological demonstration of digoxin binding and localization in cardiac cells. With the immunofluorescence method, linkage can be observed on the sarcolemma membrane and on the wall of capillaries and arterioles in myocardial cells treated by cardiac glycoside. The specificity of reaction is provided by the negative reaction of cells, not treated by digoxin. Intensity of reaction depends on concentration. The photometric measuring of fluorescence enables the quantitative analysis of cardiac glycoside. It shows the sensitivity of the method in that cardiac glycoside linked to the cell membrane can be detected in the upper sphere of a therapeutic dose. Application of the immunofluorescence method is manifold and relatively simple, and this quick method can be used in diagnoses and in the study of cardiac glycoside receptors of cell membrane. On the basis of our own experiments it is possible to study the kinetics of digoxin linkage. The use of this method is demonstrated for investigation of single cell suspension and cryostat sections.

Animals↗
PubMed1989

[Comparative analysis of the technics of preoperative localization of the parathyroid glands].

Parathyroid surgery needs an appropriate diagnosis and a preoperative localization. We conducted a prospective study to compare the efficacy of 4 different imaging modalities in 17 patients: thallium-technetium subtraction scintigraphy, ultrasonography, computed tomography and arteriography. The sensitivity was: scintigraphy 58%, echotomography 86%, Tc 92% and arteriography only 33%. Neck exploration confirmed the imaging results. We found 15 cases of adenomas (2 cases of double adenoma) and 1 case of hyperplasia; in 1 patient, no lesions were found. We conclude that the association of such techniques appears to be the optimal strategy in about 100% of the patients.

Adenoma↗
PubMed1989

Cell-mediated immunity in pigeon breeders' lung: the effect of removal from antigen exposure.

Eighteen precipitin-positive pigeon breeders, thirteen symptomatic (SPB), with extrinsic allergic alveolitis (EAA), and five asymptomatic (APB), without lung disease, underwent bronchoalveolar lavage (BAL). Cytospins were prepared on which differential cell counts were performed. Immunocytological methods, using monoclonal antibodies, were performed to identify lymphocyte and macrophage subsets. Marked abnormalities in cell populations were observed in both groups but with no suggestion of differences between the groups. All subjects had a lymphocytosis in BAL (SPB 45%; APB 29%). These lymphocytes were almost exclusively T-cells. The cluster designation CD4/CD8 ratio was decreased (SPB 0.86; APB 1.13) and a significantly higher proportion of these cells than normal expressed UCHL1 (an antigen associated with the common leucocyte antigen complex) indicating immune commitment. In the macrophage population increased proportions of cells expressing antigens associated with interdigitating cells (RFD1+) and mature macrophages (RFD7+) were also abnormal. When six SPB patients were relavaged after isolation from pigeons for three weeks, there was a significant reduction in the lymphocytosis and in the proportion of UCHL1+ lymphocytes. This was accompanied by reductions in the percentage of macrophages expressing RFD1 and UCHL1. We suggest that EAA in pigeon breeders is associated with a cell-mediated immune response which is down-regulated by isolating patients from exposure to pigeon derived antigens.

Adult↗
PubMed1987

HLA in juvenile liver disease with alpha-one-antitrypsin deficiency.

The possible role of HLA phenotypes was investigated in the development of juvenile liver disease in persons with alpha 1-antitrypsin deficiency. Seventeen patients were investigated between the ages of 3-25 years. All of them had alpha 1-antitrypsin phenotype PiZ. After a longer follow-up a clinical diagnosis was established by the help of physical status, biochemical liver function tests and--in the cases of suspected liver disease--liver biopsy. The clinical course was correlated to the HLA phenotypes of the patients. In 2 cases all first degree relatives were investigated, as well. Our studies on the 17 unrelated patients indicated no correlation between HLA and juvenile liver disease in alpha 1-antitrypsin deficiency. The family studies confirmed these findings.

Adolescent↗
PubMed1984

Persistent tyrosinemia associated with low activity of tyrosine aminotransferase.

A son of related Turkish parents had grossly elevated serum tyrosine concentration and excreted tyrosine and p-hydroxyphenolic acids into the urine, whereas neither succinylacetone nor succinylacetoacetate could be demonstrated. The tyrosine concentration was normalized by a proper diet. This was not followed strictly at home. During the first 2 years of life, the patient had severe undulating nystagmus that disappeared later. No skin lesions were present and there was only slight corneal clouding of the eyes. At the age of 5, the patient had attained the maturity of a 4-year-old, showing a balanced profile. Specific tyrosine aminotransferase (EC 2.6.1.5) was present in the liver; the Km value for tyrosine was normal. However, the total activity was less than 10% of normal, a situation similar to that observed in fetal human liver. A younger sister of the patient also has tyrosinemia and low hepatic tyrosine aminotransferase activity.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

Early case of acquired immunodeficiency syndrome in a child from Zaire.

An eight-year-old child from Zaire died in Sweden in 1982 after a clinical course compatible with the acquired immunodeficiency syndrome (AIDS). In 1975, at the age of 5 months, the infant had an acute viral infection with a rash; this illness was followed by a chronic cough. During the course of the disease he had recurrent septicemia, fever (frequently with miliary lung infiltrates), disseminated lymphadenopathy, hepatosplenomegaly, candidiasis, and diarrhea. Late in the illness the child developed lethal disseminated disturbances of the central nervous system. Immunologic investigations revealed a pronounced hypergammaglobulinemia, normal C3 but low C4 values, decreased number of T-lymphocytes, and decreased lymphocyte stimulation with T-cell and B-cell mitogens. Samples of serum taken in 1981 and 1982 were analyzed and found to be positive for antibodies to HTLV-III virus. The course of the disease in this child was more prolonged than most of the pediatric cases described earlier. It is likely that this child developed AIDS early in 1975, long before the AIDS epidemic was apparent in the United States.

Acquired Immunodeficiency Syndrome↗