Hypertension and bilateral stenosis of the renal artery associated with congenital hypoplasia of the intrahepatic bile ducts

Exss, R; Rotthauwe, H. W.

doi:10.1007/bf00443067

Cited by 6 publications

(2 citation statements)

References 40 publications

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“…Interestingly, this patient's son, who also suffered from AS, had a hypoplastic abdominal aorta, which was attributed to retroperitoneal fibrosis [5]. Exss and Rottahauwe [19] described a patient with renal artery stenosis considered secondary to hyperlipidemic arteriosclerosis. However, the lipid profile abnormality in AS patients reportedly does not place them at increased risk for arteriosclerosis [21].…”

Section: Discussionmentioning

confidence: 97%

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Renovascular hypertension and vascular anomalies in Alagille syndrome

Bérard

Sarles

Triolo

et al. 1998

Pediatric Nephrology

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Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, peripheral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubulointerstitial nephritis, renal tubular acidosis, and mesangiolipidosis have been noted in AS. The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least four of the five major features of AS who developed secondary hypertension of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis and various other abnormalities of the main arteries in all five patients: aorta (3 cases), celiac artery (4 cases), superior mesenteric artery (1 case), subclavian artery (1 case). Our findings underscore the value of arterial blood pressure monitoring in patients with AS. If hypertension occurs, a renovascular origin should be sought. The diffuse vascular abnormalities which appeared to be a feature of AS in these patients should prompt larger studies of vascular abnormalities in AS.

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Section: Discussionmentioning

confidence: 97%

“…Renovascular hypertension has rarely been reported in patients with AS [5,19,20]. Severe hypertension was described in a patient with AS and a congenital single kidney who died at 67 years of renal failure [5].…”

Section: Discussionmentioning

confidence: 99%

Renovascular hypertension and vascular anomalies in Alagille syndrome

Bérard

Sarles

Triolo

et al. 1998

Pediatric Nephrology

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Glomerular mesangiolipidosis in Alagille syndrome (arteriohepatic dysplasia)

et al. 1987

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Alagille syndrome is characterized by the association of chronic cholestasis with a paucity of interlobular bile ducts and a distinctive facies together with cardiovascular, skeletal and eye abnormalities. We examined the kidneys of 26 patients with this syndrome; 22 were under 3 years of age and 4 were 4, 6, 12 and 17 years old, respectively. Eighteen showed glomerular lesions of variable severity characterized by a mesangiolipidosis. In the 8 lesser affected patients light microscopy (LM) disclosed a fibrillar appearance of the mesangium, and electron microscopy (EM) showed lipid vacuoles widespread in the mesangial matrix. In the 10 patients who were affected to a greater degree LM and EM showed, in addition to the mesangial matrix changes, the presence of mesangial foam cells. Clinical signs of renal involvement were mild in all patients except for one who died from chronic renal failure at 8 months of age. The extent of mesangiolipidosis was not related to age but to the degree of cholestasis, the most severe lesions being observed in patients aged 3, 6, 8, and 14 months. The glomerular lesions observed in Alagille syndrome are strikingly similar to those observed in adults with lecithin-cholesterol acyl transferase deficiency and other conditions characterized by an increase in plasma lipoproteins rich in free cholesterol and in phospholipids. We conclude that glomerular involvement should be added to the characteristic features of Alagille syndrome. Also we found that the lipid deposition in the glomeruli of patients with Alagille syndrome is related to an abnormal lipid metabolism, which is the consequence of severe cholestasis. The most striking feature of our study is the early detection of the glomerular lesions, contrasting with the lack of overt clinical renal disease. Renal failure may be a major complication for patients with this syndrome in adulthood.

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Middle Aortic Syndrome in a Boy with Arteriohepatic Dysplasia (Alagille Syndrome)

1997

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Middle aortic syndrome is increasingly recognized as a cause of hypertension in adolescents and young adults. Alagille syndrome is a genetic disorder characterized by hepatic, eye, and bony abnormalities. Cardiovascular lesions occur in most of the patients, but narrowing of the abdominal aorta has not been previously recorded. We present an unusual association between middle aortic syndrome and Alagille syndrome, with a similar vascular lesion seen in two generations.

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Hypertension and bilateral stenosis of the renal artery associated with congenital hypoplasia of the intrahepatic bile ducts

Cited by 6 publications

References 40 publications

Renovascular hypertension and vascular anomalies in Alagille syndrome

Renovascular hypertension and vascular anomalies in Alagille syndrome

Glomerular mesangiolipidosis in Alagille syndrome (arteriohepatic dysplasia)

Middle Aortic Syndrome in a Boy with Arteriohepatic Dysplasia (Alagille Syndrome)

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