Oligomeganephronia (OMN) is a rare, renal hypoplasia, consisting of reduced number of hypertrophied nephrons. This disorder has been considered to be a congenital but not a genetic disease. We describe the first report, to the best of our knowledge, of familial cases of OMN; two male siblings ran rapidly downhill courses and died 11 and 8 days after births, respectively. In addition, the two patients had similar multiple anomalies; microcephaly, prominent glabella, hypertelorism, antimongoloid slant, epicanthal folds, broad nose, cleft lip and palate, downturned mouth, short philtrum, micrognathia, low set ears, hypospadias, and cryptorchism. Although the patients and the parents had normal G‐banded karyotypes, 4p monosomy syndrome is suggested from clinical features. The implications of this are discussed briefly. ACTA PATHOL. JPN. 35: 449–457, 1985.
An autopsy case of primary hepatic fibrosarcoma with severe hypoglycemia is reported. Only two cases have been previously reported. Histological features were well differentiated and partly indistinguishable from those of fibrous mesothelioma. ACTA PATHOL. JPN. 33 : 177-182, 1983.
A case of urethral clear cell adenocarcinoma (mesonephric carcinoma) in a 62‐year‐old woman is reported. The patient consulted our hospital because of acute urinary retention. Fine needle aspiration cytology showed a few atypical cells; loose cluster cells with finely vacuolated cytoplasm, large nuclei and prominent nucleoli, and small cluster cells with finely vacuolated, delicate cytoplasm, small pale nuclei and small nucleoli. Cytologic diagnosis was adenocarcinoma and no diagnosis of clear cell adenocarcinoma was obtained. The neoplasia was localized at the posterior wall of the urethra and deep muscular layer of the vaginal wall. Histologically, the tumor revealed mainly small, elongated glands consisting of single‐layered cuboidal or columnar cells with scanty cytoplasm and a focally hobnail appearance. In addition, a small cystic lesion resembling nephrogenic adenoma was observed. This finding raises the possibitily that clear cell adenocarcinoma may be a malignant counterpart of nephrogenic adenoma. ACTA PATHOL JPN 38: 217–223, 1988.
Nephrotoxic activity of rabbit antisera to glomerular basement membrane (GBM) from three different donors (rat, gerbil, and man) was examined in mice of various strains. Animals receiving a small dose (0.1 ml) of anti-rat GBM or a large dose (0.3 mlx2) of anti-gerbil GBM developed massive proteinuria and irreversible glomerular lesions which were identical to those seen in rats receiving anti-rat GBM, i.e. rat Masugi nephritis. Linear deposition of rabbit IgG, host's immunoglobulins and C3 was characteristically seen. Necrotizing changes and crescent formation accompanied by plasmic exudation, cellular proliferation and/or infiltration of PMNs and monocytes occurred after the injection. In animals receiving anti-human GBM, linear deposition of rabbit IgG and less marked glomerular changes were seen. The finding of no specificity in species between GBM antigens of rat and mouse indicates the usefulness of rat GBM for mouse Masugi nephritis or anti-GBM disease. ACTA PATHOL. JPN. 31: 85-92, 1981.
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