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We report the results of treatment in 57 patients with renovascular hypertension associated with one poorly perfused, small kidney with less than 25 per cent of total 131I-hippurate uptake shown by renography. Arteriography in 29 patients demonstrated occlusion of the artery of the small kidney, and in 28 there was stenosis. In addition, 25 patients had stenosis of the artery supplying the larger contralateral kidney. Stenosis of the arteries of the contralateral kidneys was dilated by percutaneous transluminal arterioplasty in all but one of the 25 patients with bilateral disease of the artery, stenosis of the small kidney could be dilated successfully by percutaneous transluminal arterioplasty in 22 of the 28 patients, and cure or improvement of blood pressure was achieved in 12 of them. Percutaneous transluminal arterioplasty of occluded arteries was generally unsuccessful. In 17 patients with unilateral disease not manageable by percutaneous transluminal arterioplasty, nephrectomy of the small kidney improved blood pressure control without significant deterioration of renal function. Renal function improved in 10 patients with bilateral lesions treated by nephrectomy of the small kidney in combination with contralateral percutaneous transluminal arterioplasty. Histological examination of excised kidneys showed large infarcts or several cholesterol emboli whether percutaneous transluminal arterioplasty had been attempted or not. After observation periods ranging from two to 79 months, 48 patients were normotensive (21 without and 27 with medication) and nine patients were still hypertensive even with medication. This study showed that by using percutaneous transluminal arterioplasty initially if possible, supplemented with nephrectomy and/or medication, normotension without loss of renal function or immediate serious complications could be obtained in the majority of these severely hypertensive patients.

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Howie and Brewer recently described a novel glomerulopathy: the glomerular tip lesion (GTL). The characteristic feature of this entity is a collection of intracapillary foam cells and marked vacuolization of the epithelial cells of the glomerular segment adjacent to the origin of the proximal tubule. Although this lesion resembles focal segmental glomerulosclerosis (FSGS), Howie and Brewer suggested that it constitutes a distinct entity, differing also clinically from FSGS, in that it would have a better response to steroid treatment. We treated five patients fulfilling the criteria of Howie and Brewer. However, neither corticosteroids (1.5 mg/kg/day for 1 month in five patients) nor cyclosporin-A (5 mg/kg/day for three months in four patients) caused a decrease in proteinuria to below 4 g/day. In two patients, renal function deteriorated and in one of them, recurrence of classical FSGS was found in the renal transplant. A sixth patient was observed in whose biopsy a combination of GTL with membranous glomerulopathy was present. We conclude that GTL is not a distinct entity and that in the clinical course and response to treatment it does not differ from FSGS.

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Different histochemical techniques were compared on paraffin wax embedded tissue sections for routine classification of amyloid; the following methods were used: potassium permanganate, the indirect immunoperoxidase method using polyclonal anti-human amyloid antisera (anti-AA, anti-A lambda, anti-A kappa and anti-AF) and the peroxidase-antiperoxidase (PAP) method using antisera against human, bovine, hamster and canine AA amyloid. Anti-human AA antiserum appeared to be a useful tool in this respect. Polyclonal anti-AL antisera may be helpful in diagnosing AL amyloid, but were less of value than anti-AA serum. Strong cross reactivity between anti-bovine AA antiserum and human AA amyloid deposits was found. This indicates that animal amyloid AA antisera can also be used for the diagnosis of AA amyloid in human tissues.

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Two kindreds with the multiple endocrine neoplasia type 2A syndrome were studied. Of one of these we examined 150 members, 20 of whom were treated with thyroidectomy for medullary carcinoma and nine with bilateral adrenalectomy for pheochromocytoma. Of the second kindred 50 members were examined, seven of whom were thyroidectomized and seven treated with bilateral adrenalectomy. Pheochromocytomas were invariably found on both sides, even in four cases in which the adrenals on one side appeared to be completely normal, not only at preoperative roentgenologic examination but also on inspection during the operation. The microscopic finding of micronodules and a cluster of abnormal medullary cells identical with those found in pheochromocytomas in one of the apparently normal adrenals represents a first stage in the development of diffuse medullary hyperplasia as well as nodular hyperplasia. This is in accordance with the fact that in the MEN type 2A syndrome pheochromocytomas are always multicentric and multiple in origin. On the basis of these findings we conclude that all patients with the MEN 2A syndrome who show symptoms and signs of active pheochromocytoma should be subjected to bilateral adrenalectomy, even when one or both of the adrenals appear to be normal at roentgenologic investigation.

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Skin and kidney biopsies were performed on 262 patients with various nephropathies. In 45 skin biopsy specimens finely granular deposits of predominantly IgA and late-acting complement factors were detected in the walls of superficial capillaries, sometimes concomitantly with IgM, IgG, C4 or a combination of these proteins. Twelve of the 45 patients presented with anaphylactoid purpura and the majority of the other 33 patients had either recurrent macroscopic or microscopic hematuria. The renal lesions in 32 of these 45 patients consisted of focal segmental intracapillary proliferation. In 35 the kidney biopsy specimen showed mesangial deposits of IgA; in one case IgA was deposited along the glomerular basement membrane. In only three of the remaining 217 patients without cutaneous IgA deposits were typical mesangial IgA deposits found. The close correlation between IgA deposits in cutaneous vessels and focal segmental intracapillary proliferation with mesangial IgA deposits suggests that immunofluorescence examination of skin biopsy specimens could prove of diagnostic value. The results provide additional evidence for a close pathogenic relationship between IgA-associated glomerulonephritis and anaphylactoid purpura.

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Hypertension as a result of stenosis of a renal artery was diagnosed in three patients, aged 20, 17, and 9 respectively, among 14 siblings from one family. The oldest patient was treated by vasoplasty; a hypoplastic kidney had previously been removed in an unsuccessful attempt to control hypertension. Nephrectomy was performed in the other two patients. The pathological picture of the main renal artery that could be examined differed from that usually seen in the fibromuscular dysplasia group. The absence of microscopic signs of atrophy in the small kidneys behind the stenosis argues for a developmental defect. More than three years after operation blood pressure and renal function in all three patients have remained normal.

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A family is described many of whose members suffered from renal insufficiency, hypertension, gout, and hyperuricaemia in conjunction. Adequate information was obtained on 72 subjects from five generations. In 17, one or more of the above mentioned abnormalities was or had been present. The hereditary distribution suggested an autosomal dominant disease entity. The renal disease was characterized by an early loss of urinary concentrating power, minimal proteinuria, and death at a relatively early age dominating the clinical picture. The histological picture in three biopsies and one necropsy showed predominant tubular atrophy and interstitial fibrosis, with striking tubular basement membrane thickening. It is suggested that these patients suffered from a hereditary degenerative renal disease. The question whether hyperuricaemia was primary or secondary in these cases is discussed.

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