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At the beginning of the four chapters on phaenomena, analysis, pathophysiology and therapy of cystinuria the essentials of the published literature are summarized. The frequency of cystinuria is in the order of 1:10,000. Besides the cystine lithiasis occurring in nine tenths of all cystinuria patients neurological diseases may also be observed. All commonly applied methods to analyze cystine detect the sum of cystine and cysteine. Cystinuria is characterized by a higher cystine excretion, up to the 100-fold of the normal. Also the concentrations of lysine, arginine and ornithine in the urine of cystinuria patients are elevated, caused by intestinal and renal transport defects. Inevitable damage of renal parenchyma by multiple operations can drastically be reduced by the therapy with D-penicillamine or α-mercaptopro-pionylglycine. The disadvantages of that formation of soluble asymmetric disulfides are the side effects, such as nausea, gastric difficulties and dermatosis, occurring in up to 50% of the patients. Using the especially developed method with HPLC separation and electrochemical detector with a mercury electrode, cystine and cysteine are analyzed simultaneously. In the urine of healthy persons the molar concentration of cysteine is in the same order as cystine. But in cystinuria the cysteine concentration in urine is about a thousand times less than that of cystine. These results are evidence that a shifted redox-equilibrium of cystine-cysteine is also typical of cystinuria. The molar cysteine percentage of cysteine in healthy persons is increased from 30 to 50% by oral ascorbic acid administration. Therefore a vitamine C therapy for cystinuria is developed. 31 cystinuria patients who receive 5 g of vitamine C a day show a decrease in the cystine concentration of about 40%. Up to now, no side effects have been observed. The most obvious sign of the positive effect of the proposed vitamine C therapy for cystinuria is the missing cystine sediment in fresh urine.

Stress is a response state of the body which may be evoked by various stimuli. The effect of such stress factors on the quantitative composition of 24-hour urine in young male Wistar rats is examined using short, repetitive, weak electric impulses. Changes in body development and, in particular, of the absolute and relative increases in size and weight of the adrenal glands compared with control groups are used as evidence of the endured stress procedure. Three 24-hour urine groups can be clearly distinguished. While concentrations of calcium and sodium remain unaltered by the adaptation syndrome of the body, the concentrations of potassium and magnesium are clearly reduced during the first days of the stress period compared with the control group. Inorganic phosphorus and uric acid concentrations, however, are markedly increased during the experimental period. The combination of increased uric acid and inorganic phosphorus concentrations and reduced potassium and magnesium levels favors a disposition to urolithiasis since uric acid and inorganic phosphorus are recognized promoters, and potassium and magnesium are known inhibitors, of stone formation.

A 40-year-old man with two normally descended testicles and no signs of hormonal anomalies developed a fist-sized extraperitoneal tumor to the left of the urinary bladder. Histologic diagnosis was that of a probably malignant gonadal stromal tumor because of considerable mitotic activity and widespread vascular invasion. Retroperitoneal lymphadenectomy showed no metastatic disease, and the patient is well two years after surgery. By use of an immunoperoxidase method we could demonstrate estradiol and testosterone in the tumor cells. The findings in this case are unique in regard to the extratesticular location of the tumor and support the current view that gonadal stromal tumors arise from a primitive cell with a multidirectional potential for differentiation.