Herein, a case of malignant hypertension was reported in a 7 year-old girl, who complained of hypertension (180/130) and nasal bleeding. Right contracted kidney was found on IVP, right renal artery stenosis on renal arteriogram and bilateral vesico-ureteral reflex (VUR) on voiding cystogram. Peripheral vein renin was 11.8 ng/ml/hr, however, there was no lateralization in the value of renal vein renin. Anti-VUR operations were performed and she was followed up with an antihypertensive medication. With a sudden cessation of the antihypertensive drugs (Inderal, Apresolin and Serpasil), hypertension was accelerated (260/180), associated with hematuria, edema and oliguria. Hypertensive encephalopathy appeared during peritoneal dialysis and she died in uremia . At autopsy, malignant nephrosclerosis was noticed and arteriosclerotic vascular changes were found in adrenal glands, liver, spleen and pancreas. This case was supposed to be the seventh case of malignant nephrosclerosis in the Japanese children. Right renovascular hypertension and bilateral pyelonephritis with superimposed acceler ated nephrosclerosis was discussed for the pathogenesis of hypertension in this case.

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Plasma renin activity (PRA) and plasma aldosterone (PAL) were measured in six patients with chronic renal failure before and after nephrectomy and in 3 renal allograft recipients during the first month after transplantation. In controls, PRA and PAL were measured in one patient with a juxtaglomerular cell tumor after nephrectomy and in two patients with renovascular hypertension after autotransplantation of the ischemic kidney. PRA was decreased to zero within 12 hours after nephrectomy. In only one male patient, it showed 0 to 0.23 ng/ml/h even a month after nephrectomy. Therefore, it is inferred that the biological half life of renin is about 5 hours and renin is also released from the tissue other than the kidney. PRA and PAL were elevated in most patients within 24 hours after transplantation. The values in patients with a retarded function of the grafted kidney were significantly higher than in patients whose kidney worked well from the transplantation on, After three to four days, most PRA and PAL values were within the normal range. It is suggested, therefore, that the ischemic during transplantation may be the cause of renin elevation, The elevation of PAL is most likely a consequence of the increased angiotensin II generated by high PRA, indicating that the renin-angiotensin-aldosterone relationship is unaltered in many patients after renal transplantation. A consistent relationship could be demonstrated between acute graft rejection and PRA. Further, there was no significant correlation between PRA and blood pressure after transplantation.

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The urinary excretion of fibrin/fibrinogen degradation products (FDP) were measured by the method of the tanned red cell hemagglutination inhibition immunoassay (TRCHII) in 54 patients with nephrotic syndrome. More than 4.Eg/ml of urinayFDP was regarded as positive. The results obtained were as follows : (1) Idiopathic and nephritic nephrotic syndrome (a) Urinary FDP positive cases were found in nephrotic patients more than in non-nephrotic patients. (b) The urinary FDP excretion did not correlated with fibrin on immunofluorescent studies. (c) The values of serum FDP in nephrotic patients were within normal limit and did not correlated with the amount of urinary FDP. (d) Serum fibrinogen and serum lipid levels were higher in nephrotic patients than in nonnephrotic patients, but there was not significant difference between urinary FDP positive cases and negative cases. (e) In six patients whose urinary FDP showed persistent positive five patients showed partial remission but one patient became uremic. (2) Lupus nephritis Nephrotic syndrome due to lupus nephritis had more urinary FDP positive cases than nonnephrotic syndrome. Urinary FDP positive cases with nephrotic syndrome showed active lesions by the histological examination. (3) Diabetic nephropathy Two positive cases of urinary FDP showed decreased renal function. (4) Renal amyloidosis Five of seven nephrotic patients showed urinary FDP positive.

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Twelve renal allotransplants were performed in twelve children, 7 to 19 years of age between January 1975 and September 1976 at Tokyo Metropolitan Children's Hospital, Kiyose. Twelve Kidneys were donated from four fathers and eight mothers . At the end of May 1977, there was no death and no graft loss (follow up period 9 months to 2 years 4 monthsaverage 19.4 months). All twelve children rehabilitated to full school activity in ordinary school and ten out of twelve are continuing their normal daily life. One patient was readmitted because of recurent chronic rejection and slowly deteriorating kidney function. The remaining one developped bilateral aseptic necrosis of Femurs which restricted his daily excercise, even though function of his transplanted kidney has been excellent. Procedures of renal allotransplant were followed modified Hume & Williams' method. Two recipients weighed less than 20kg recieved adult kidneys without difficulty. Average warm ischemic time was ten minutes, cold ischemic time was thirty eight minutes and initial diuresis was seen twenty two minutes after revascularization, including two cases of double renal arteries. Nine out of twelve recipients underwent bilateral (uretero-) nephrectomy and appendectomy 2 to 39 weeks prior to transplant surgery, six underwent splenectomy simultaneously with bilateral nephrectomy. One patient underwent bilateral nephrectomy 9 months after transplant surgery because of intractable hypertension. Clinial course of the twelve transplant recipients were reported and basic immunosuppressive therapy, treatment of acute and cronic rejection, complication, management of pre- and post-transplant patients, effect of pretransplant blood transfusions to the outcome of graft function and comparison of patients'survival and grafts' survival were also discussed.

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Twelve kidneys transplanted into children from living related donors were examined for histo pathological deviations 1hr., Ca. 2 months and Ca. 1 year after the transplantation by use of light microscopy, electron microscopy and fluorescence techniques. 1. In kidneys examined 1hr. after the transplantation, 5 out of 12 cases showed some identifiable histological deviations which may have existed in the donor before the transplantation. 2. The influence of these changes on the clinical course after transplantion should be examined in the future. 3. Histological deviations compatible with acute rejection were observed even in cases which showed no apparent clinical episode of rejection. 4. Recovery from the histological deviations of rejection were observed in accordance with the clinical recovery from rejection. 5. Electron microscopic findings of transplant glomerulopathy were observed in the glomerulus of cases which had reccurent episodes of rejection. 6. By fluorescence techniques, IgC, IgM, and .A1C were seen in many cases. 7. To date, we have seen no cases of reccurence of the original glomerular diseases.

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To obtain more sensitive and more stable method for determination of methylguanidine (MG), the Sakaguchi reaction was carried out in an ice bath by adding of urea and thymine to the reagents. MG concentration in the human blood cells of healthy and uremic subjects, and the tissue concentration of MG in the blood cells, liver, muscle, colon, kidney and brain in the uremic rats made by the Platt method and normal rats were determined respectively. In the blood cells of uremic patients, MG was concentrated about three fold comparing to the serum level. In addition, except for brain, all tissue concentrations of MG in the uremic rats were three to four times higher than that of serum. These tissue concentrations seem to be sufficient for MG to exert as a uremic toxin. In the uremic rats fed with protein free diet, the production rate of MG was reduced. On the other hand, in the uremic rats fed with high protein diet the production rate of MG was increased. Although the serum creatinine concentration was not changed by these dietary conditions. These results implied that the metabolic production of MG has a close relation to the protein metabolism in uremia

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In 77 patientswith primary plomerular disease, fibrin/fibrinogen degradation products (FDP) have been measured at the time of a renal biopsy, in 299 patients with primary glomerular disease, histological and immunohistological examination of biopsy specimens have been performed. The serum FDP was proved in 94.3% of patients. In only 1 of 20 normal adults, the serum FDP was detected. The urinary FDP appeared in 72.7% of patients, and in none of normall adults. There was a high degree of correlation renal histological grading (cell proliferation) and immunofluorescent antifibrinogen staning of renal biopsies. The relationship of serum FDP or urinary FDP to plasma fibrinogen levels, proteinuria and histological grading of biopsy specimens were investigated. The results are described as follows: 1) Urinary FDP levels had no correlation to serum FDP levels. 2) There was a high degree of correlation between urinary FDP levels and proteinuria, but no correlation was proved between urinary FDP levels and plasma fibrinogen levels, Serum FDP levels had no correlation proteinuria and plasma fibrinogen levels. 3) There was some correlation between urinary FDP levels and renal histological grading (cell proliferation) of biopsy specimens, also, there was a high degree of correlation between urinary FDP levels and immunofluorescent antifibrinogen staning of renal biopsy specimens. Serum FDP levels had no correlation to histological grading and immunofluorescent antifibrinogen staning of renal biopsy specimens. 4) The clinical significance of urinary FDP was to be assessed rather in the ratio of urinary FDP to proteinuria (u-FDP/u-p) than in daily amount (mg/day) or in concentration (μg /ml). In cases showing u-FDP/u-P exceeding 50ppm the urinary FDP is estimated to be due to intraglomerular fibrin deposition.

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