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Results for “Nephritis, Hereditary”

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At least 19 recordsLinked to original sources
PubMed1979

Blood coagulation and hereditary nephritis.

Nineteen patients with hereditary nephritis - members of six families - were studied for haemostasis on 11 occasions by the use of four capillary tests, 14 different blood coagulation tests and two different platelet function tests. Systemic capillaropathy was demonstrable in 8 out of 15 cases of the non-uraemic, and in all the 5 cases of the uraemic group. No completely normal blood coagulation was found in any of the cases and the coagulation disorders were never of exclusively haemorrhagic pattern. On the evidence of the complex studies, hypercoagulability as well as hyper-hypocoagulability were found in the uraemic and non-uraemic groups alike. Reduction in plasma fibrinolytic activity, elevation of the fibrinogen level and a shift of the thrombelastogram in the direction of thrombosis were demonstrable in the majority of the cases.

Adolescent↗
PubMed1977

Hereditary nephritis and the heart.

Eleven cases of hereditary nephritis were studied for cardiac abnormalities by means of ECG and BCG. With the exception of two cases no significant abnormalities were demonstrable, which indicates that this genetically transmitted process generally leaves the heart unaffected. It is thus to be expected that intermittent haemodialysis, as far as the heart is concerned, will be well tolerated by these patients.

Adolescent↗
PubMed1978

Hereditary nephritis: a re-examination of its clinical and genetic features.

In order to re-evaluate current concepts of hereditary nephritis we studied the urinary findings, the course of the disease, and its genetic transmission in two large pedigrees. We identified 150 patients with hereditary nephritis. Our data show that microscopic hematuria is the most reliable urinary criterion for diagnosing hereditary nephritis in both male and female patients. The hematuria is frequently accompanied by erythrocyte casts indicating that the renal lesion is a glomerulitis. Men are more severely affected than women. They have striking urinary abnormalities, which are present in early childhood, and they progress to renal failure in adult life. Affected women have less obvious urinary abnormalities and rarely develop uremia. In these two families a sex-linked dominant mode of genetic transmission was present. The demonstration that hereditary nephritis is X-linked, at least in some families, helps to explain the difference in severity between men and women and the variable expression among affected women.

Adolescent↗
PubMed1977

Development of the characteristic ultrastructural lesion of hereditary nephritis during the course of the disease.

The diagnosis of hereditary nephritis in the absence of a clear family history or the presence of characteristic ancillary abnormalities is difficult, but it has considerable prognostic importance. The recent recognition of an ultrastructural lesion characteristic of this disease, although not present in all families, is of considerable value. In this report we describe a kindred with the lesion-associated disease that differs from those previously described in that the lesion developed during the course of the disease, was not present in all affected members and appeared to be the result of paternal transmission.

Adolescent↗