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Malignant hypertension in three siblings
S Kuriyama1, T Kawamura, G Tokutome
1Second Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan.
Insights
This study identified a rare familial form of malignant hypertension (MH) in three siblings. Clinical and pathological findings suggest a genetic basis for this severe condition, leading to renal failure in two brothers.
Area of Science:
- Nephrology
- Genetics
- Pathology
Background:
- Malignant hypertension (MH) is a severe form of hypertension with significant morbidity and mortality.
- Familial clustering of severe hypertension suggests potential genetic underpinnings.
- Understanding the etiology of MH is crucial for developing targeted therapies.
Abstract:
We encountered three siblings (one female and two males) whose clinical manifestations were consistent with those of malignant hypertension (MH). Renal biopsies were obtained from two of the patients, and an autopsy from the third. The light microscopic findings from all three cases were characterised by marked intimal thickening with concentric layering of collagen and cellular proliferation in the interlobular arteries. Findings, on examination of optic fundi, including retinal haemorrhages, exudates and papilloedema were also consistent with malignant hypertension. The two male patients progressed to develop renal failure. These cases strongly suggest the existence of a rare familial form of primary malignant hypertension.