[Autosomal dominant polycystic kidney disease. Case report of a 20 kg polycystic kidney]. / Autosomal-dominante polyzystische Nierenerkrankung. Ein Fallbericht von 20 kg schweren polyzystischen Nieren.

Polycystic kidneys can reach huge dimensions as a result of their continuous growth and thus lead to a "mechanical problem." We report on a 69-year-old patient with enormous polycystic kidneys having been treated for 4 years with standard hemodialysis. In the last 12 months, the patient was suffering from a deterioration of his condition and progressively developed cachexy. The preoperative status of the multi-morbid patient jeopardized operability. It was decided to perform bilateral nephrectomy with a Chevron incision on the right side. The right kidney weighed 11 kg, the left 9 kg. There were no postoperative problems. The massive relief resulted in an impressive improvement of this risk-patient's fitness and quality of life. There is a clear indication of unilateral nephrectomy in case of a renal transplantation. The role and necessity of a bilateral nephrectomy in the event of polycystic kidneys is discussed based on the respective literature.

[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia]. / Generalisierte AL-Amyloidose: Eine seltene Komplikation bei Makroglobulinämie Waldenström.

Waldenström's macroglobulinemia (W.M.) is a paraproteinemic disorder similar to multiple myeloma expressing a monoclonal IgM paraprotein. The course of W.M. is milder and the occurrence of renal disease is less frequent than in multiple myeloma. Amyloidosis occurs in less than 5% of patients with monoclonal IgM. We report a 65 year old patient with an asymptomatic long-term survival whose W.M. remained without any therapy. Amyloidosis developed twenty-one years after the initial diagnosis presenting with the nephrotic syndrome, renal and heart failure. W.M. combined with amyloidosis is a rare association. The deposition of amyloid causes progressive organ damage and indicates a bad prognosis. Most commonly nephrotic syndrome or heart failure are signs of amyloidosis. The development of amyloidosis does not seem to be influenced by the duration of W.M. disease or the paraprotein level. It is not known whether the amyloidosis can be prevented by an early chemotherapy.

[Peritoneal dialysis as kidney replacement therapy]. / Die Peritonealdialyse als Nierenersatztherapie.

Since the introduction of CAPD more than ten years ago, peritoneal dialysis has become an established technique of renal replacement therapy. The general practitioner must be aware of the important prerequisites, advantages, risks and complications of peritoneal dialysis, and should send the patient who is near end stage renal failure to a renal unit early enough. The nephrologist and dialysis instruction nurse will try to train the patient as far as possible on an outpatient basis. The aim is for the patient to be soon in a position to perform the dialysis technique at home and thus regain responsibility and independence. With proper training he can maintain himself in a stable condition with little risk of infection and a good prognosis.

[Parathyroid crisis. Fatal course despite control of hypercalcemia with mithramycin]. / Parathyreotoxische Krise. Letaler Verlauf trotz Kontrolle der Hypercalcämie mit Mithramycin.

A 73-year-old patient suddenly became confused and comatose due to excessive hypercalcemia. He developed acute respiratory distress syndrome (ARDS) and died even though the hypercalcemic state had been brought under control with mithramycin. Autopsy disclosed an adenoma of the parathyroid gland and calcifications in lung and kidney tissues as well as hyaline membranes. The case illustrates the poor prognosis of hyperparathyroid crisis whenever organ damage is present.

[Hydrochlorothiazide-induced lung edema with shock]. / Hydrochlorothiazid-induziertes Lungenödem mit Schock.

We report the case of a 74-year-old woman who developed pulmonary edema and shock shortly after ingestion of one tablet of hydrochlorothiazide/amiloride. The clinical findings and course of the disease were characteristic of hydrochlorothiazide-induced pulmonary edema. 14 similar case are reported in the literature. Attention is drawn to this rare but dangerous side effect of a frequently used diuretic.

[Osteomalacia after intestinal operations--therapeutic effect of calcitriol. Report of a case]. / Osteomalazie nach intestinalen Operationen--Therapieeffekt von Calcitriol. Ein Fallbericht.

An 82-year-old woman, with clinically and histologically documented osteomalacia associated with a history of several gastrointestinal operations and malabsorption, was successfully treated with low dose calcitriol. Osteomalacia, with particular reference to the gastrointestinal type, and the role of vitamin D and its metabolites are discussed. Therapy with calcitriol is compared with conventional vitamin D treatment. Calcitriol should be considered in the treatment of osteomalacia induced by gastrointestinal disorders.

Thymopentin as adjuvant to hepatitis B vaccination. Results from three double-blind studies.

The influence of adjuvant thymopentin therapy on the effect of vaccination with HB-Vax was investigated in three independent double-blind studies in which three different time/dose schedules of the adjuvant therapy were used. The first study was conducted with 30 hemodialyzed patients who had previously been non- or hyporesponders. Forty and 26 nonvaccinated hemodialyzed patients were chosen for the two additional studies. A 50-mg dose of thymopentin or placebo was administered subcutaneously in all studies. In one study, in which only one adjuvant injection was administered simultaneously with each vaccine injection, thymopentin inhibited the antibody response. On the contrary, in the other two studies, in which three injections of adjuvant were administered during the week following the vaccination (in one of these studies three injections were also given before the vaccination), no difference in the effect of vaccination was observed in patients on either placebo or thymopentin. Comparison of the results of the present studies with those of earlier observations emphasizes the importance of time/dose schedules of adjuvant therapy in vaccination.

[Non-A, non-B hepatitis in patients with chronic hemodialysis]. / Non-A-non-B-Hepatitis bei chronisch hämodialysierten Patienten.

A prospective study was conducted of the incidence and course of acute viral hepatitis in 69 chronic hemodialysis patients. During a mean observation period of 15.4 months, 5.6 cases/100 patients/year of non-A, non-B hepatitis and 1.1 cases/100 patients/year of hepatitis B virus infection occurred. 19 out of 38 patients with serologic evidence of immunity against hepatitis B virus infection developed the antibody to hepatitis B surface antigen after active immunization. All cases of non-A, non-B hepatitis had an asymptomatic course, but there was a tendency to chronicity. The overall impact of non-A, non-B hepatitis in patients on chronic hemodialysis is discussed.

Peritoneal access and related complications in continuous ambulatory peritoneal dialysis.

Over a four-year period, 50 patients underwent continuous ambulatory peritoneal dialysis. After 24 months, 48 percent of the patients continued to receive continuous ambulatory peritoneal dialysis treatment (including those who underwent continuous ambulatory peritoneal dialysis with bottled solutions from 1977 to 1978). Using solutions in plastic bags, 63 percent of patients continued to undergo continuous ambulatory peritoneal dialysis after 24 months. The mortality rate at 24 months was 23 percent overall and 15 percent for those using plastic bags. There was an overall average of 2.4 episodes of peritonitis a year per patient and 1.3 episodes a year per patient from 1979 through 1980, when only the technique with plastic bags was used. The number of days a patient was hospitalized averaged 48 per year, and 37 days per year in 1979 and 1980. Fifty-five percent of catheters remained functional at 24 months. Other complications included 15 hernias, 15 skin and tunnel infections, 12 leaks, and five cuff extrusions. Improved catheters and further reductions in the incidence of peritonitis will most likely result in a decreased number of patients who withdraw from continuous ambulatory peritoneal dialysis therapy.

Parathyroid hormone-independent adaptation of the renal handling of phosphate in response to renal mass reduction.

In man as well as in experimental animals progressive renal failure is associated with a decrease in the fractional reabsorption (FR) of inorganic phosphate (Pi). This response has been considered as an adaptation phenomenon and generally attributed to an increase in parathyroid hormone (PTH) secretion. One report indicates that in chronic thyroparathyroidectomized (TPTX) dogs treated with large doses of vitamin D progressive renal failure can also be associated with a fall in FRPi. However, in this latter study the concomittant administration of vitamin D could have accounted for the observed decrease in FRPi. In our study we investigated whether or not chronic reduction in renal mass leads to a similar decrease in maximal net tubular Pi reabsorption per volume of glomerular filtrate (maximal TRPi/ml GF) in the presence and absence of PTH and without pharmacological supplementation in vitamin D. Male rats were either TPTX or sham-operated (intact). One and two weeks later the animals of both groups were either subtotally nephrectomized (NX) in two stages or sham-operated (control). Four weeks after the second renal operation, the glomerular filtration rate (GFR) and the reabsorption of Pi were determined by clearance methodology under acute sodium chloride and Pi infusion, that is, at endogenous and increased plasma Pi concentrations ([Pi]Pl.). Thus maximal TRPi/ml GFR could be determined. In rats with intact parathyroid glands GFR was 1.56 +/- 0.10 (mean +/- SEM) and 0.54 +/- 0.10 ml/min in control and NX respectively, whereas maximal TRPi/ml GF was 2.24 +/- 0.07 in control and 1.57 +/- 0.18 mumol/ml (P less than 0.005) in NX. In TPTX rats GFR was 1.66 +/- 0.27 and 0.62 +/- 0.06 ml/min in control and NX respectively, whereas maximal TRPi/ml GF was 3.80 +/- 0.20 in control and 2.95 +/- 0.13 mumol/ml (P less than 0.005) in NX. The marked decrease in maximal TRPi/ml GF observed in TPTX after subtotal NX could not be ascribed to any consistent change in plasma calcium. Our study provides conclusive evidence that the decrease in maximal TRPi/ml GF in response to renal mass reduction can occur to the same degree in the presence or absence of PTH.

[Drug induced fluorosis in myeloma]. / Medikamentöse Fluorose bei Myelom.

Osteolytic lesions of bone in multiple myeloma may be treated by florides. A patient is reported who had IgA-Myeloma, was treated with high doses of sodium floride for 16 months and developed the clinical and radiological picture of advanced fluorosis. Within one year after discontinuing sodium fluoride therapy the soft tissue calcifications disappeared almost completely. High bone turnover may account for the accelerated deveopment and disappearance of fluorosis in myeloma. Progressive renal failure due to myeloma and, perhaps, increased susceptibility of destructed bone to fluorides advocate restricted use of fluorides in the treatment of osteolytic bone lesions in myeloma.

Resistance to the phosphaturic and calcemic actions of parathyroid hormone during phosphate depletion. Prevention by 1,25-dihydroxyvitamin D3.

Recent observations indicate that in thyroparathyroidectomized (TPTX) rats fed a low (0.2 g/100 g) phosphorus diet, the tubular phosphaturic response to parathyroid hormone (PTH) remains markedly blunted even when it is assessed at normal or high plasma concentration and filtered load of inorganic phosphate (Pi). Because 1,25-dihydroxyvitamin D(3) [1,25(OH)(2)D(3)] decreases the tubular capacity to reabsorb Pi when chronically administered to TPTX rats, we have studied whether this vitamin D(3) metabolite could specifically increase the phosphaturic response to PTH in phosphate-deprived animals. The results show that in Vitamin D-replete TPTX rats fed a low (0.2 g/100 g) phosphorus diet, 1,25(OH)(2)D(3) (2 x 13 pmol/d i.p. for 7 d) markedly enhanced the acute tubular phosphaturic response to PTH (2.5 IU/h i.v.) without affecting the action of the peptide hormone on Ca reabsorption and cyclic-3',5'-AMP excretion. The influence of 1,25(OH)(2)D(3) on the phosphaturic response to PTH could not be ascribed to an increased plasma concentration and(or) filtered load of Pi during the administration of the peptide hormone. However, it could be, at least in part, related to the elevation in the basal level of plasma Pi which was observed in the 1,25(OH)(2)D(3)-treated animals. The results also indicate that 1,25(OH)(2)D(3) significantly enhanced the calcemic response to PTH, which was blunted in these conditions of phosphate deprivation. Unlike 1,25-(OH)(2)D(3), 25-hydroxyvitamin D(3) did not unmask the phosphaturic effect of PTH in phosphate-depleted animals, even when given in doses 100 times larger. Thus, 1,25(OH)(2)D(3) displays a selective and powerful activity in preventing the occurrence of tubular resistance to the phosphaturic action of PTH during Pi depletion. This finding suggests the existence of an important interaction between dietary Pi, 1,25(OH)(2)D(3), and PTH in the homeostasis of phosphate.