[Diagnosis of glomerular and non-glomerular erythrocyturia using phase contrast microscopy of the urine sediment]. / Diagnose glomerulärer und nicht-glomerulärer Erythrozyturien mit Hilfe der Phasenkontrastmikroskopie des Urinsedimentes.

Recently the appearance of deformed polymorphous erythrocytes in the urinary sediment has been described as characteristic of glomerular bleeding. We studied 30 patients with histologically confirmed glomerular disorders and 25 patients with urological diseases and with hematuria. In the sediment of 10 ml urine 200 erythrocytes were counted under phase-contrast microscopy and evaluated relative to their morphology. The number of glomerular erythrocytes was expressed as a percentage. In all groups of glomerular disorders (mesangial-proliferative, membranous and membrano-proliferative glomerulonephritis, focal segmental glomerulosclerosis, glomerulonephritis of systemic disease, thinning of the glomerular basement membrane) the percentage of glomerular erythrocytes varied widely between 2 and 100%. In 7 cases less than 10% of glomerular erythrocytes were found. There was no correlation between the percentage of glomerular erythrocytes and the degree of renal insufficiency, hematuria or proteinuria. On the other hand, in patients with hematuria from the lower urinary tract, erythrocytes were uniformly non-glomerular in shape (95-100%). We conclude that 10-20% or more of glomerular erythrocytes in the urinary sediment are a good indicator of glomerular disease, whereas lower figures do not definitely rule out a glomerular origin for hematuria.

Adjuvant intravesical chemotherapy of superficial bladder cancer with monthly doxorubicin or intensive mitomycin. A comparison of two consecutive series.

120 patients with the first occurrence of TNM stage Ta or T1 bladder carcinoma were given adjuvant intravesical treatment in 2 consecutive series. The first 60 received doxorubicin monthly for 6 months, then on the 9th and 12th month following surgery. The second series was given mitomycin weekly for 2 months, then monthly to the end of the first year. Median follow-up is 48 months in the first series and 15 in the second. In the doxorubicin group, 28 patients had recurrences. The recurrence rate is 1.86/100 patient-months. Seventeen of these first relapses occurred within the first year. In the 'intensive' mitomycin series, where 36 patients were observed for 12 months or more, 3 relapsed. The recurrence rate to date is 0.35. The relapse-free survival probability at 12 months is 75% in the first series and 93% in the second. Treatment was stopped due to toxicity in 4 patients in the doxorubicin group and in 10 of the mitomycin group.

[Chronic fatal vitamin A poisoning with hemolytic anemia]. / Chronische, letal verlaufende Vitamin-A-Vergiftung mit hämolytischer Anämie.

A case of chronic, lethal vitamin A intoxication is reported, the diagnosis of which was not established intra vitam. The patient presented with chronic mixed prehepatic and hepatic icterus and hypersplenism with hemolytic anemia. On post mortem histological examination, abundant deposits of neutral lipids in the reticuloendothelial cells of liver, spleen and bone marrow were found. Chemical analysis of the lipids extracted from these organs revealed esterified fatty acids. Triglycerides could be ruled out since the triglyceride content of the patient's liver was significantly lower than that of a normal control. By means of thinlayer chromatographic analysis it was possible to identify retinol, i.e. retinylester (vitamin A) in the deposits. Vitamin A was determined photometrically and found to be very elevated in the liver and, to a lesser degree, in the spleen of the patient. These findings lead to a definite diagnosis of chronic hypervitaminosis A. Unique features of the case presented are both hemolytic anemia and the lethal outcome of the chronic hypervitaminosis A.

Mixed medullary and follicular carcinoma of the thyroid.

We report a case of medullary carcinoma of the thyroid which on light microscopy showed not only the well known arrangement of cells in sheets and nests but also unequivocal follicular structures. These follicular structures are present both in the primary tumor and in lymph node metastases. Immunohistochemical investigations revealed that the cells lining the follicles produce thyroglobulin, whereas the remaining tumor tissue is positive for calcitonin and carcinoembrionic antigen. This case represents a medullary carcinoma of the thyroid with an atypical pattern consisting of both thyroglobulin and calcitonin producing cells.

Generalized gangliosidosis: acid beta-galactosidase deficiency with early onset, rapid mental deterioration and minimal bone dysplasia.

This report concerns a 3-month-old girl with rapidly progressive psychomotor retardation, hepatomegaly, vacuolated lymphocytes, minimal bone dysplasia and normal excretion of acid mucopolysaccharides. A deficiency of acid beta-galactosidase was demonstrated in isolated leucocytes and in a liver biopsy. The diagnosis of generalized gangliosidosis due to deficiency of beta-galactosidase was also based on the absence of the enzyme activity from cultured fibroblasts. The diagnosis was confirmed on autopsy at 16 months by typical histology, electron microscopy and biochemistry of the organs. beta-galactosidase deficiency has been demonstrated in various clinical conditions ranging from generalized gangliosidosis with severe mental retardation to clinical pictures resembling Morquio's disease and normal intelligence. The heterogeneity of the clinical manifestations in beta-galactosidase deficiency could be explained by different residual activities of a structurally mutated enzyme towards its various substrates.

High amniotic fluid alpha-1-fetoprotein in a case of fetal sacrococcygeal teratoma.

Amniocentesis was performed in a pregnancy of 26 weeks because of hydramnios. The amniotic fluid was examined for fetal karyotype and AFP content. The latter was elevated to 300 mug/ml. Fetal death occurred shortly after amniocentesis and a malformed male fetus with a large sacrococcygeal teratoma was stillborn 3 days later.

Ring chromosome 13 in a polymalformed anencephalic.

In the 33rd week of pregnancy an amniocentesis was performed because of low estriol. X-ray indicated the presence of anencephaly and a premature delivery was induced. Necropsy, in addition to anencephaly, showed a wide variety of malformations. The fetal karyotype determined from cultured amniotic fluid cells revealed a ring chromosome 13.