A 67-year-old female patient had been suffering for a year from such a severe pain syndrome that she could no longer walk. Although she had presented herself to several doctors from different disciplines, the cause of her illness was not identified. It was osteomalacia caused by a drug. This was not recognized, although the disease is common. The aim of this article is to raise awareness of a common bone disease. In contrast to osteoporosis, this disease is very accessible to therapy. The differential diagnosis to osteoporosis and the different causes of osteomalacia, hypophosphatemia and Fanconi syndrome are explained in detail. With the correct diagnosis and consequent treatment, the patient could be helped in such a way that she could even walk again without a walking aid. Overlooking phosphaturia can cause severe skeletal consequences.

The aim of this study was to evaluate the effect of ibandronate administration on long-term graft function and graft survival after successful renal transplantation.

A 59-year-old man with end-stage renal disease and haemodialysis since 2008 was admitted with painful, non-healing ulcerations on his legs. The lesions developed without known trauma 2 months before admission (figure). Pedal pulses were palpable on both sides and there were no signs of chronic venous insufficiency. Radiography of the soft tissue revealed diffuse calcified arterioles in a mesh-like pattern (figure). Histological examination of skin biopsy showed sclerosis and thrombosis of blood vessels (figure) and von Kossa stains were positive for calcium deposits (figure), confirming the diagnosis of calciphylaxis. Calciphylaxis is a syndrome of systemic medial calcification of the arteries leading to tissue necrosis. Skin biopsy and radiographic features are helpful in the diagnosis; but negative results do not necessarily exclude the diagnosis. Although the pathogenesis remains unclear, several comorbid conditions are known to increase its development. Associated risk factors in our patient were: end-stage renal disease, secondary hyperparathyroidism, and oral anticoagulation with phenprocoumon. Recent reports indicate that low parathyroid hormone levels in association with adynamic bone diseases can also be associated with calciphylaxis. Unfortunately, there is still no standardised treatment for calciphylaxis. Based on previous reports, phenprocoumon was withdrawn in our patient and he was treated with cinacalcet, daily haemodialysis, sodium thiosulfate, and bisphosphonate. After 4 weeks, skin condition improved and the patient was discharged home.

Angesichts ihrer hohen Prävalenz besitzen kognitive Auffälligkeiten bei Patienten mit chronischer Nierenerkrankung unmittelbare praktische Relevanz. Charakterisiert durch kognitive Verlangsamung, Aufmerksamkeitsdefizite, Störungen der Planung, Initiierung und Umsetzung von Handlungen, sowie Sprach- und Gedächtnisstörungen ist die kognitive Beeinträchtigung bei Patienten mit chronischer Niereninsuffizienz meist Folge einer subkortikalen arteriosklerotischen Enzephalopathie. Letzterer liegt in der Regel eine systemische Mikroangiopathie zugrunde, die sich im Gehirn vor allem in der weißen Substanz manifestiert und kernspintomographisch durch Hyperintensitäten in T2-gewichteten Sequenzen nachgewiesen werden kann. Die Therapie umfasst die Behandlung der chronischen Niereninsuffizienz, der gemeinsamen Risikofaktoren von chronischer Nierenerkrankung und subkortikaler arteriosklerotischer Enzephalopathie sowie, bei Vorliegen des Vollbilds einer Demenz, ggf. die Verordnung von Antidementiva.

Unfortunately it remains unclear whether the intention was to describe an independent pathology (hypocomplementemic urticarial vasculitis syndrome) or whether the scenario was one of immunovasculitis (leukocytoclastic vasculitis) with a particularly pronounced reduction of complement factors. It would be interesting to know whether hypocomplementemia exists in such patients before disease onset or for a long period of time after symptoms have disappeared. Further, it is well known that diverse medications are the most common cause of immunovasculitis, which is always accomxpanied by a reduction in complement factors. No mention was made in the case report about drug or topical treatment preceding (or during) the illness.

BACKGROUNDChronic urticaria often points the way to the diagnosis of a systemic disease, particularly when urticarial vasculitis can be demonstrated. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is considered to be an independent immunological disease.METHODSelective literature review and consideration of the author's own clinical experience.RESULTS AND CONCLUSIONSThe main manifestation of HUVS is chronic urticarial vasculitis with complement deficiency and the demonstration of C1q antibody in the serum. Multiple other organs are involved, sometimes severely. The diagnosis is confirmed by skin biopsy, which reveals leukocytoclastic vasculitis as a pathogenetic correlate of this systemic disease. Although HUVS is relatively rare, the medical specialists that might encounter it-ophthalmologists, rheumatologists, nephrologists, dermatologists, general practitioners, and pediatricians-should include it in their differential diagnoses whenever appropriate. Awareness of HUVS and rational diagnostic evaluation will lessen the chance of it being misdiagnosed as another type of systemic immunological disease and will reduce superfluous diagnostic testing in patients suffering from it.

Drug Prescribing for Patients with Chronic Kidney Disease in General Practice: a Cross-Sectional Study

Obstructive sleep apnea and arterial hypertension are frequent diseases, but they are also often overlooked. There is a causal relationship of sleep apnea and hypertension. Undiagnosed sleep apnea is probably the most important reason for "essential" hypertension. It is important to identify these patients. All hypertensive patients should be asked for snoring, breathing arrest and daytime sleepiness, neck circumference should be measured, and an ambulant sleep apnea monitoring should be performed, if necessary. Especially patients with refractory hypertension or non-dippers should be screened for sleep apnea and patients with sleep apnea should be examined for arterial hypertension. Continuous positive airway pressure (CPAP) can effectively lower blood pressure in the hypertensive sleep apnea patient. This is especially true for the obstructive sleep apnea patient with refractory hypertension. CPAP therapy is probably the best therapy for sleep apnea-induced nocturnal blood pressure rises.

Die obstruktive Schlafapnoe und die arterielle Hypertonie sind sehr häufige, aber leider auch oft nicht erkannte Erkrankungen. Der ursächliche Zusammenhang von Schlafapnoe und Hypertonie ist gesichert. Wahrscheinlich ist die undiagnostizierte Schlafapnoe die häufigste Ursache der früher so genannten „essentiellen“ Hypertonie. Besonders wichtig erscheint daher die Identifikation dieser Patienten. Bei allen Hypertonikern sollten nach Schnarchen, Atemstillständen und Tagesmüdigkeit gefragt werden, der Halsumfang gemessen und ggf. eine ambulante Messung der nächtlichen Atmung (kardiorespiratorische Polygraphie) erfolgen. Insbesondere sollten alle Patienten mit schwer einstellbarer Hypertonie oder fehlender Nachtabsenkung in der 24-h-Blutdruckmessung auf ein obstruktives Schlafapnoesyndrom gescreent werden. Umgekehrt sollten alle Patienten mit Schlafapnoe gezielt auf eine arterielle Hypertonie untersucht werden. Da bei diesen Patienten auch nur eine nächtliche arterielle Hypertonie vorliegen kann, reichen Einzelmessungen am Tage nicht aus, um einen Bluthochdruck sicher auszuschließen.

BACKGROUND:Chronic hepatitis C virus (HCV) infection is closely associated with mixed cryoglobulinemia. Cryoglobulins can activate complement leading to vascular damage. We examined whether cryoglobulinemia and complement turnover is associated with HCV infection in renal transplant recipients and whether this has an adverse effect on graft outcome.METHODS:Sera and fresh plasma from 31 HCV-RNA-positive patients after renal transplantation (group I) were studied for cryoglobulins, complement hemolytic activity (CH50), and complement split product C3d. In total, 80 HCV-negative renal transplant recipients (group II) and 72 untreated patients with chronic hepatitis C (group III) without renal transplantation served as controls.RESULTS:Cryoglobulins were detected in 45, 28, and 26% of the patients in group I, II, and III, respectively. A high cryocrit ( > 5%) was present only in patients of group III (p < 0.01%). Mean CH50 values were lower and C3d levels higher in HCV-positive patients (group I and III) compared with HCV-negative patients (p < 0.0001). Cryoglobulins were not associated with extrahepatic manifestations or graft dysfunction, except in five patients of group III demonstrating cryoglobulinemic vasculitis. HCV-positive renal transplant recipients with signs of complement activation showed a significantly greater increase of serum creatinine (0.88 +/- 1.14 mg/dL) when compared with baseline than patients without complement activation (0.34 +/- 0.37 mg/dL; p = 0.035). There was also a tendency toward a higher extent of proteinuria in patients with complement activation (1.38 +/- 2.17 g/d vs. 0.50 +/- 0.77 g/d; p = 0.25, NS).CONCLUSIONS:Cryoglobulins are common in renal allograft recipients, but do not affect graft function. However, complement activation appears to be involved in chronic allograft dysfunction in HCV-infected recipients.

Background. Aspirin treatment has an undoubted beneficial impact on the progression of cardiovascular diseases. We hypothesized that aspirin also protects allograft function and survival in the context of chronic renal allograft dysfunction, which displays decisive pathophysiologic features that are similar to those involved in atherogenesis.Methods. A retrospective, multivariate analysis was performed to assess the effect of low-dose aspirin treatment (100 mg/day) on allograft function and survival of 830 renal transplant recipients. Allograft function was evaluated by serum creatinine levels, urine protein levels, and the presence of hematuria.Results. Median allograft; survival time was significantly longer in patients receiving low-dose aspirin therapy compared with patients receiving no aspirin treatment (n = 205, 13.8 +/- 2.6 vs. 7.8 +/- 0.3 years, n=625; adjusted relative risk=0.443, 95% confidence interval [0.323-0.608], P<0.0001). Statin treatment and a recent time point of transplantation, reflecting the qualitative advances of the applied immunosuppressive therapy, were further positive determinants of renal allograft survival. The number of antihypertensive agents, representing the extent of hypertension, was a negative determinant of allograft survival. Transplant function was better preserved in aspirin-treated patients, who displayed a slower increase of serum creatinine and less proteinuria and hematuria during the observation period. The duration of aspirin treatment was positively associated with better allograft function.Conclusions. Low-dose aspirin therapy substantially improves renal allograft function and allograft survival. These findings suggest that aspirin should be considered to complement long-term posttransplant medical treatment regimens.

Background: Patients with Wilson’s disease may present with cirrhosis, acute hepatitis or fulminant hepatic failure. Without urgent orthotopic liver transplantation, a fulminant Wilson crisis has a mortality of 100%. We report on an 18-year-old female patient with fulminant hepatic failure due to Wilson crisis. Methods: The molecular adsorbent recirculating system (MARS) was used to eliminate albumin-bound toxins and to bridge waiting until an organ became available. Results: A total of 18 MARS sessions and 4 plasma exchange sessions were performed. Bilirubin levels and hepatic encephalopathy improved under MARS therapy. A total of 75 mg copper was removed until serum copper levels were within the normal range. Copper elimination was measured in 15 MARS treatments, which removed a total of 12.9 mg copper. Four plasma exchange sessions, with a total exchange of 11 liters of plasma, removed 12 mg copper. Urinary copper elimination with penicillamine was 50 mg. Conclusion: MARS was an effective method to stabilize a patient with Wilson crisis, contributed to copper elimination and gained time for liver transplantation. The risk of high-urgency transplantation could be avoided. Liver support was easy in the hands of nephrologists familiar with extracorporeal therapy.

Background. En bloc kidneys from pediatric donors are regarded as questionable with respect to the safety and quality of the transplant outcome. Therefore, we retrospectively studied graft outcome and graft function of our 56 en bloc kidneys transplanted in paraaortal position between 1992 and 1999.Methods. Graft outcome of en bloc kidneys (group A) was compared with graft outcome of single cadaveric adult donor kidneys (group B). Matched pairs were generated regarding HLA-missmatch, cold ischemic time, recipient age, body mass index, and systolic arterial blood pressure.Results. Allograft survival rates of pediatric en bloc kidneys at 1, 3, and 5 years were significantly lower (group A. 78, 70, 70% vs. group B: 92, 92, 81%, P<0.05). Lower survival rate was caused by a higher number of graft losses in the early postoperative period (group A: 21% vs. group B: 4%, P<0.01) due to vascular complications. Main risk factor for graft loss was donor age of less than 12 months. Five years after transplantation serum creatinine of pediatric en bloc kidneys was significantly better than of adult kidneys (0.9+/-0.06 vs. 1.8+/-0.2 mg/dl, P<0.001).Conclusion. En bloc kidneys show a high percentage of graft survival with excellent long-term graft function. However, the early postoperative period carries a higher risk of graft loss in very young donors due to vascular complications. In the face of donor shortage en bloc kidneys from pediatric donors can successfully be transplanted in a paraaortal position.

Background: Mortality or graft loss after renal transplantation might be influenced by hepatitis virus infection.

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism characterized by an excessive accumulation of copper in many organs, particularly liver and brain. Mortality rate of patients with fulminant hepatic failure is expected to be 100 %, if the patients can not be transplanted.