Congenital hepatic fibrosis with polycystic disease of the kidneys.

Three cases involving simultaneous occurrence of congenital hepatic fibrosis (CHF) and polycystic kidneys (PCK) are reported. In two infantile forms hepatic fibrosis accompanying polycystic renal lesion was revealed only on autopsy; the third patient has been kept under observation for seven years. Emphasis is laid on the importance of early diagnosis if serious complications are to be prevented. The pathogenesis of the disease is discussed on the basis of light and electron microscopic investigations of repeatedly taken liver and kidney biopsy material.

[Prevention of testosterone-vasopressin induced necrosis of the kidney cortex by cyproterone acetate]. / Tesztoszteron+vazopresszin adagolást követo vesekéreg-nekrózis kivédése cyproteron acetáttal.

After a ten days pretreatment with testosteron-propyonate administration of vasopressin induces bilateral cortical necrosis of kidneys in rats. When testosteron and cyproteron-acetate an androgen-blocking agent were given simultaneously vasopressin did not induce necrosis of the renal cortices. These findings suggest that androgen receptors of the kidney may take a part in eliciting this phenomenon.

Cyproterone acetate-promoted prevention of renal cortical necrosis following testosterone and vasopressin administration.

Bilateral focal renal cortical necrosis was observed after vasopressin administration in rats pretreated for 10 days with testosterone phenyl propionate. When the androgen-receptor blocking-agent cyproterone acetate was administered together with the testosterone, the subsequent vasopressin treatment did not cause renal cortical necrosis. The results suggest the role of the androgen receptors in the kidney in the induction of the phenomenon.

Histomorphologic and histochemical investigations in mannosidosis. A light and electron microscopic study.

Morphologic and histochemical studies have been performed at light and electron microscopic level on needle-liver biopsy specimens, circulating blood lymphocytes and fibroblast cultures from patients with mannosidosis. The findings demonstrated generalized storage phenomena of varying degrees in the various tissues examined. Histochemical findings were in agreement with the biochemical nature of the stored material. Enzyme histochemical methods indicated storage in the lysosomes, at least in the hepatocytes. The ultrastructural appearance of mannosidosis in itself has but a limited diagnostic significance since the morphology and distribution of vacuoles have characteristics in common with other storage diseases. Repeated liver biopsy disclosed extensive storage in the hepatic tissue. However, the progression of the disease was not accompanied by severe mechanical destruction or microcirculatory disturbances.

Familial occurrence of giant cell hepatitis in infancy.

The repeated occurrence within one family or giant cell hepatitis terminating in death is reported. The familial form of giant cell hepatitis has a poorer prognosis than that occuring sporadically. In the first case, reported earlier, primary liver cancer developed within a short time. In the second case, reported here, the progression of the course of the disease which terminated in cirrhosis was followed in a series of histological examinatons made at five different points of time. Electronmicroscopic examination were also conducted.