Association of von Recklinghausen's neurofibromatosis with adult polycystic disease of kidneys and liver.
نویسندگان
چکیده
Introduction Von Recklinghausen's neurofibromatosis and adult polycystic disease of the kidneys (APDK) are both heredofamilial disorders (Preiser and Davenport, 1918; Dalgaard, 1971) although sporadic cases also occur (Wander and Dsgupta, 1977; Hatfield and Pfister, 1972). Both the conditions are known to occur in association with a number of other hereditary disorders (Dalgaard, 1971; Koch, 1977). However, their occurrence together is a rarity (Siegelman, Javed and Hecht, 1971).
منابع مشابه
Spontaneous hemothorax caused by a ruptured intercostal artery aneurysm in von Recklinghausen's neurofibromatosis.
Aneurysms arising from an intercostal artery are very rare vascular malformations in von Recklinghausen's neurofibromatosis, which often have a silent clinical presentation and are difficult to diagnose before rupture. We report a case of von Recklinghausen's neurofibromatosis with massive hemothroax caused by spontaneous rupture of an intercostal artery aneurysm in a 29-year-old man. The diagn...
متن کاملA case report of neurofibromatosis
Introduction:Neurofibromatosis is a genetic disease characterized by multifocal benign tumors of peripheral nerves, called neurofibromas, and pigmented spots on the skin which inherited as autosomal-dominant. The most common form of the disease is neurofibromatosis type 1, also known as von Recklinghausen's disease of the skin. When an individual has small number of lesions in a limited region ...
متن کاملVon Hippel's disease in association with von Recklinghausen's neurofibromatosis.
Ten members of a large family who showed manifestations of either von Hippel-Lindau disease or von Recklinghausen's neurofibromatosis were examined. Three of 10 members were found to have retinal angiomas which had not been present on fundus examination 3 years previously. These angiomas were associated with ocular and systemic signs of neurofibromatosis. These cases show overlapping manifestat...
متن کاملAn unusual association of recurrent pyogenic granuloma on nevus flammeus in a patient with Von Recklinghausen's disease.
Pyogenic granuloma is a common benign vascular lesion of the skin and mucosa. There are a few reports on the rare association between it and port wine stain, but there is no clear description of an association with neurofibromatosis type 1 in the literature. This report presents a 29-year-old Saudi male with Von Recklinghausen's disease with recurrent pyogenic granuloma on the nevus flammeus ov...
متن کاملSpontaneous haemothorax: a cause of sudden death in von Recklinghausen's disease.
Vasculopathy is a relatively frequent but poorly recognised manifestation of von Recklinghausen's neurofibromatosis. One of its more dramatic presentations is as spontaneous haemothorax. Clinicians and pathologists should be aware of this syndrome as a cause of sudden death in patients with neurofibromatosis.
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Postgraduate medical journal
دوره 58 676 شماره
صفحات -
تاریخ انتشار 1982