Biomaterial Database

[Renovascular hypertension in neurofibromatosis von Recklinghausen (author's transl)]. 1978

D E Müller-Wiefel

The course of the illness in a six-year-old boy is reported. His recurrent headaches led to the detection of arterial hypertension (170/135 mmHg). Clinical and histological examination revealed neurofibromatosis von Recklinghausen. A bilateral paraumbilical murmur hinted at a renovascular form. Arteriography showed an aneurysm of the right renal artery and multiple bilateral intrarenal stenoses and aneurysmatic dilatations. Under conservative treatment with Propranolol and Dihydralazine blood pressure remained almost normal over two years. 38 paediatric cases of renovascular hypertension in childhood reported in the literature are analyzed with regard to clinical manifestation, morphology and localisation of the renovascular lesions.

UI	MeSH Term	Description	Entries
D006977	Hypertension, Renal	Persistent high BLOOD PRESSURE due to KIDNEY DISEASES, such as those involving the renal parenchyma, the renal vasculature, or tumors that secrete RENIN.	Hypertensions, Renal,Renal Hypertension,Renal Hypertensions
D008297	Male		Males
D009456	Neurofibromatosis 1	An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).	Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D011433	Propranolol	A widely used non-cardioselective beta-adrenergic antagonist. Propranolol has been used for MYOCARDIAL INFARCTION; ARRHYTHMIA; ANGINA PECTORIS; HYPERTENSION; HYPERTHYROIDISM; MIGRAINE; PHEOCHROMOCYTOMA; and ANXIETY but adverse effects instigate replacement by newer drugs.	Dexpropranolol,AY-20694,Anaprilin,Anapriline,Avlocardyl,Betadren,Dociton,Inderal,Obsidan,Obzidan,Propanolol,Propranolol Hydrochloride,Rexigen,AY 20694,AY20694,Hydrochloride, Propranolol
D012077	Renal Artery	A branch of the abdominal aorta which supplies the kidneys, adrenal glands and ureters.	Arteries, Renal,Artery, Renal,Renal Arteries
D012078	Renal Artery Obstruction	Narrowing or occlusion of the RENAL ARTERY or arteries. It is due usually to ATHEROSCLEROSIS; FIBROMUSCULAR DYSPLASIA; THROMBOSIS; EMBOLISM, or external pressure. The reduced renal perfusion can lead to renovascular hypertension (HYPERTENSION, RENOVASCULAR).	Renal Artery Stenosis,Obstruction, Renal Artery,Obstructions, Renal Artery,Renal Artery Obstructions,Renal Artery Stenoses,Stenoses, Renal Artery,Stenosis, Renal Artery
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D004078	Dihydralazine	1,4-Dihydrazinophthalazine. An antihypertensive agent with actions and uses similar to those of HYDRALAZINE. (From Martindale, The Extra Pharmacopoeia, 30th ed, p354)	Depressan,Dihydralazine Sulfate,Dihydrallazine,Dihydrazinophthalazin,Sulfate, Dihydralazine
D006261	Headache	The symptom of PAIN in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of HEADACHE DISORDERS.	Cephalgia,Hemicrania,Bilateral Headache,Cephalalgia,Cephalodynia,Cranial Pain,Generalized Headache,Head Pain,Ocular Headache,Orthostatic Headache,Periorbital Headache,Retro-Ocular Headache,Sharp Headache,Throbbing Headache,Unilateral Headache,Vertex Headache,Bilateral Headaches,Cephalalgias,Cephalgias,Cephalodynias,Cranial Pains,Generalized Headaches,Head Pains,Headache, Bilateral,Headache, Generalized,Headache, Ocular,Headache, Orthostatic,Headache, Periorbital,Headache, Retro-Ocular,Headache, Sharp,Headache, Throbbing,Headache, Unilateral,Headache, Vertex,Headaches,Headaches, Bilateral,Headaches, Generalized,Headaches, Ocular,Headaches, Orthostatic,Headaches, Periorbital,Headaches, Retro-Ocular,Headaches, Sharp,Headaches, Throbbing,Headaches, Unilateral,Headaches, Vertex,Ocular Headaches,Orthostatic Headaches,Pain, Cranial,Pain, Head,Pains, Cranial,Pains, Head,Periorbital Headaches,Retro Ocular Headache,Retro-Ocular Headaches,Sharp Headaches,Throbbing Headaches,Unilateral Headaches,Vertex Headaches
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man