[Disseminated pulmonary adiasporomycosis. (Report of one case) (author's transl)].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to C Scheiner.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Hb F values were estimated in 336 adults on 525 occasions. A variable elevation appeared not only in patients with a variety of haematological diseases, but also in conjunction with malignant neoplasms, liver disease etc. The highest values were encountered in cases of pernicious anaemia, haemolytic anaemia, panmyelopathy and plasmocytoma. The determination of Hb F in peripheral blood smears by means of this extremely simple elution method (Betke and Kleihauer) represents a useful tool in the diagnosis of haematological disorders in adults, especially the haemoglobinopathies. The possible causes of this biochemical redifferentiation of erythropoiesis are discussed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
In this geographically based study the findings on 158 abnormal fetuses, primarily diagnosed by routine antenatal ultrasound, are correlated with the results of the examinations subsequently carried out by a fetopathologist and a clinical geneticist. Ninety fetuses (57%) had a single malformation, 66 were polymalformed (42%) and 2 had no malformations. In 90% of all these cases, the prenatally and postnatally detected anomalies were identical; in 3% the defect established at necropsy was different from that diagnosed prenatally, and in 7% the predicted anomaly was absent. These values did not depend on whether single or multiple malformations were involved. In 57% of the polymalformed cases, however, the ultrasound examination missed at least one other diagnosable anomaly. On the basis of pathological and clinical genetic expertise, a risk of recurrence of the anomaly was revised in 13% of the single malformed cases and in 53% of the multiple ones, i.e., in 30% of all the cases of malformation on average. This study confirms the need for the fetus to be examined by a pathologist and a clinical geneticist after termination of a not 'at risk' pregnancy in order to check the accuracy of the sonographic procedure, to confirm the reasons for terminating the pregnancy to the parents, and to be able to monitor the next pregnancy based on an accurate assessment of the risk of recurrence.
A review of 78 renal tumors in patients under 6 months of age revealed 35 congenital mesoblastic nephromas (CMNs). Based on cellular criteria, 14 were classified as classical, 4 as partly cellular, and 17 as cellular CMN. The mean ages were 24, 11, and 70 days, respectively. There were 13 intrarenal tumors (stage I) but 9 classical, 3 partly cellular, and 5 cellular CMNs extended to the perirenal fat (stage II) and 5 cellular tumors ruptured (stage III). In order to assess cellular proliferative activity, silver staining of nucleolar organizer region (Ag-NOR) proteins was performed on 19 CMNs. The number of Ag-NOR dots per cell was significantly lower in classical and partly cellular CMN than in cellular CMN, whatever the stage (P < .01). Within the cellular CMNs, the mean number of Ag-NOR dots was statistically higher in the single case that recurred with fatal outcome (P < .02). Counting of Ag-NOR dots appears to be a reproducible method for evaluating the biologic potential of CMNs. The number of Ag-NOR dots, DNA content measurements, the histological subclassification, and the presence or absence of tumor at the surgical margins may be useful features in selecting those patients who will benefit from further treatment after nephrectomy.
The authors report a case of Menetrier's disease revealed in a 51 years woman before oedomas by systematic gastroscopy. Somes particularities of this case authorize to argue problems put by this disease: a thyroid nodule treated by L + triodothyronin; an eosinophilia; a biological study of gastric secretion, gastrin and intrinsec factor level; an hormonal, immunologic and genetic exploration; an histochimic and ultra-structural study of operative portion; its favorable evolution after total gastrectomy.
Explore the source record for details and available documents.
BACKGROUND: IgA nephropathy may occur in adults with alcoholic cirrhosis. It has also been reported in children with alpha-1-antitrypsin deficiency. A case of IgA nephropathy associated with cirrhosis due to Wilson's disease is reported. CASE REPORT: A 10 year-old girl, was admitted for cirrhosis. She had suffered from ascitis and hematuria since the age of 6 years and vascular purpura since the age of 9 yr 6 mo. At admission, she had atrophic liver cirrhosis, ascitis splenomegaly and petechiae on her legs. There was microcytic anemia due to iron deficiency. The Coombs test was positive. There were hypoalbuminemia (2 g/dl) and polyclonal hyperglobulinemia (IgA 750 mg/dl, IgG 1670 mg/dl and IgM 250 mg/dl). Her serum concentrations of complement factors were normal and no immune complexes were detected in serum. She also showed biological changes due to kidney failure, hematuria and proteinuria. A liver biopsy showed micronodular cirrhosis with numerous plasma cells containing IgA. The search for an etiology showed ceruloplasmin levels of 5 mg/dl, elevated urinary copper excretion (> 150 micrograms/day) and above normal liver copper (255 micrograms/g dry weight); Kayser-Fleischer ring was also seen. The renal biopsy showed membranoproliferative glomerulonephritis, and immunofluorescent microscopy revealed C3 and IgA in the mesangial and subendothelial regions. The patient was given spironolactone and furosemide followed by triethylenetetramine dihydrochloride. CONCLUSIONS: The IgA nephropathy, vascular purpura and elevated serum IgA levels could be due to the liver changes. If so, they should be improved by a liver transplant, although our patient would be a better candidate for combined liver and kidney transplantation.
A collaborative study was performed to determine the different types and mechanisms of intestinal abnormalities during gestation. Cases had to fulfill one or more of the following three criteria: (1) meconium ileus, (2) intestinal stenosis or atresia, and (3) meconium peritonitis. Esophageal atresia, anorectal atresia, and abdominal wall defects were excluded. One hundred two cases were reviewed from the autopsies of 42 induced abortions, 22 stillborns, and the surgical findings in 38 neonates. Meconium ileus was detected mainly during the second trimester (28/38), and was associated with cystic fibrosis (15), fetal blood deglutition (4), infection (6), or multiple-abnormalities (10), in which three chromosomal aberrations were found. Intestinal stenosis or atresia was more commonly detected during the third trimester of gestation (46/56). Sixteen of the 30 duodenal malformations were associated with trisomy 21, whereas in the 26 small intestinal atresias, signs of distress or ischemia were most frequently detected. Only 8 of 25 meconium peritonitis cases were isolated. A total of 20 cystic fibrosis cases could be proved. In this series, functional abnormalities were observed predominantly in the second trimester and associated mainly with cystic fibrosis or amniotic fluid abnormalities. Anatomic lesions were commonly detected later on and associated with ischemic conditions, chromosomal aberrations, and even cystic fibrosis.