The complement activation system in 37 patients with systemic lupus erythematosus (SLE) and 17 patients with acute glomerulonephritis (AGN) was studied with special respect to the relationship between classical pathway and altarnate pathway. Serum complement components, Clq, C4, C3, C3 proactivator (C3PA), C5 and C9, were measured immunochemically according to the method of Mancini using the antiserum to each component purchased from Behringwerke AG. (Clq, C4, C3PA, C9) and Hyland Laboratories (C3). Immuno-fluorescent studies on renal tissues were also carried out using antisera to IgG, IgM, IgA, C1q, C3, C3PA and C9. In the active phase of SLE, reduction of serum C3PA levels, significantly correlated with the levels of C4 and C3, was observed in 16 of 31 patients, and in renal tissues 7 of 13 patients showed the positive immunofluorescence of C3PA. Therefore, it was suggested that C3b feedback system in alternate pathway was activated with C3b, the cleaving product of C3, associated with the activation of classical pathway in SLE. On the other hand, in the initial phase of AGN, the serum levels of C3 and C5 were decreased, , though the reduction of C3PA and its positive immunofluorescence were observed in only 2 and 5 of 14 patients respectively. According to these results, alternate pathway in AGN might be activated through the pathway different from the C3PA activation system, probably through the pro perdin activation system shown by Spitzer et al. Some patients with the reduction of Clq and/or C4 levels, showing the glomerular deposition of Clq, suggested the activation of classical pathway. Although the change of serum C9 levels in both diseases was not significant, marked immu nofluorescence of C9 in the glomeruli of all studied revealed that the terminal component was activated through both pathways.

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Medullary cystic disease, first described by Smith and Graham in 1945, is a very rare cause of chronic renal failure. In this report, we described a 35-year-old male patient who was undergoing chronic hemodialysis. There was consanguinity among his parents. There was no family history of renal disease, but his brother and he were noticed nerve deafness, and no useful past history was available. In the spring of 1970, he first consulted a doctor, complaining of general malaise, occasional nausea with vomiting, and nasal bleedings. There were no abnormalities except for progressive anemia. Six months later, spasms of the both lower extremities started. He was admitted to another hospital, where a diagnosis of renal disorder was made. He was normotensive despite of severe reduction of renal function during his first admission. Laboratory data disclosed advanced anemia, low specific gravity of the urine, mild proteinuria, and almost normal findings of urinary sediments. Serum electrolytes were almost within normal ranges. Low protein diet was prescribed during initial hospitalization and his daily intake of salt was 15g. The blood-pressure values were normal or near normal during these diet therapies, but serum BUN and creatinine increased. Many symptoms of uremia appeared several months later and the indications of dialysis developed. He was transferred to our hospital. Chronic hemodialysis started in February, 1971. He definitely improved after several times of dialysis. Blood pressure had risen extremely since the summer of 1972. He began to have diarrhea, persistent headache, and cardiac failure. Digoxin and antihypertensive drugs were administrated and intensive hemodialysis was instituted every day. During these treatments, he complicated acute pneumonia and died in December, 1972. At autopsy, macroscopically, the both kidneys were of normal size with thin cortexes and numerous, small cysts (diameter 2-20 mm) were found at the corticomedullary junction. Microscopical changes revealed various degrees of hyalinization and sclerosis of glomeruli, hypertrophy and atrophy of tubules, periglomerular fibrosis, severe interstitial fibrosis, and infiltration of round cells. Vascular changes covered a wide spectrum. The parathyroid at the right lower position was a small finger tip-sized adenoma and was histologically composed of chief cells and oxyphil cells.

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We reported two cases of unilateral renal hypoplasia with hypertension and discussed similarity of their clinical findings to those of "Ask-Upmark" kidney. Morphologically, in both cases, right hypoplastic small kidney with cortical indentation was revealed on the nephrographic phase of renal arteriogram and renal scintigram. Typical clubbing of upper calyceal system was shown on IVP and this change coincided with grooves on the cortical surface, There was no renal artery stenosis. Functionally, ¹³¹IHippuran renogram showed decreased renal plasma flow and prolonged mean transit time on the right side and right renal vein renin was significantly higher than left renal vein renin in both cases. Right hypoplastic kidney might be related to pathogenesis of hypertension in both cases. However, those small kidneys could not be decided to be "Ask-Upmark" kidney because both cases were not operated even if they had many similar clinical features to those of "Ask-Upmak" kidney.

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