Prognostic impact of renal arterial resistance index upon renal allograft survival: the time point matters.

BACKGROUND: The renal arterial resistance index (RI) is reported to be a significant predictive parameter for renal allograft failure or death. The influence of the time point after renal transplantation on its predictive power has not been sufficiently evaluated. We performed a retrospective analysis of RI and its power to predict renal allograft failure or death with special emphasis on the time point of RI measurement. METHODS: The present analysis is based on ultrasonographically recorded intrarenal arterial RI measurements, routinely obtained in our outpatient department, over a period of 13 years. Altogether, 88 patients with an RI measurement 0-3, 3-6 and 12-18 months after transplantation were included and retrospectively stratified into two groups according to the RI: those with an index >0.75 and those with an index of ≤0.75. RESULTS: Twenty patients (23%) reached the combined end point, i.e. allograft failure with a return to dialysis or death. The RI measured early after transplantation (0-3 and 3-6 months) did not predict the end point, whereas the RI obtained between 12 and 18 months showed a significant predictive value for renal transplant failure or death in a univariate approach [Wald test, P = 0.0013, hazard ratio (HR) 4.787, 95% confidence interval (CI) 1.846-12.411]. At 12-18 months after transplantation, 14% (12 patients) of the study population had an RI >0.75. Seven (58%) of these patients reached the end point versus 13 of 76 patients (17%) with an RI ≤0.75. In a multivariate Cox model, the RI measured between 12 and 18 months after transplantation exhibited the highest hazard ratio (HR 6.191, 95% CI 2.288-16.756, P = 0.0003) for transplant failure or death. CONCLUSION: In our hands, the RI obtained during the first 6 months after transplantation failed to predict renal allograft failure or death, whereas the RI measured 12-18 months after transplantation appeared useful to predict long-term allograft outcomes.

Erratum: Trends of renal diseases in Germany: review of a regional renal biopsy database from 1990 to 2013.

[This corrects the article DOI: 10.1093/ckj/sfz023.][This corrects the article DOI: 10.1093/ckj/sfz023.].

Trends of renal diseases in Germany: review of a regional renal biopsy database from 1990 to 2013.

BACKGROUND: Several renal biopsy registries in Europe have shown geographical and temporal variations in the patterns of renal diseases. However, there is a lack of current data on trends of renal disease in Central Europe. METHODS: After exclusion of transplant and re-biopsies, the renal biopsy registry of the German RWTH Aachen University Hospital included data of 1208 biopsies over a period of 24 years (1990-2013). Trends in the biopsy rate and diagnosis of glomerular diseases were analysed. RESULTS: The average annual biopsy incidence was 6.1 biopsies per 100 000 population. The frequency of kidney biopsies increased significantly over the years (P < 0.001). Primary glomerulonephritis (GN) accounted for nearly two-thirds (58.4%) of all native kidney biopsies, and immunoglobulin A-nephropathy (IgAN) was the leading histological diagnosis (34.7%) followed by necrotizing GN (RPGN) at 18.7%. IgAN increased 2-fold over the study periods (+195%, P < 0.001). Focal segmental glomerulosclerosis accounted for 6.1% of all diagnoses, and its frequency rose to 3.9-fold (+388%, P < 0.001). Lupus nephritis showed a doubling in incidence (P = 0.0499), while acute tubular necrosis decreased to 3.5-fold (P = 0.0008). All other disease entities failed to exhibit linear trends over time. In children, the most common pathologies were IgAN (26.1%) and minimal change disease (21.7%), whereas RPGN (19.4%) dominated in the group of patients >60 years. CONCLUSION: IgAN was the most common primary glomerular disease in our centre and its prevalence increased over 24 years.

[Pulmonary alveolar microlithiasis--a rare cause of bilateral extensive pulmonary infiltrates]. / Pulmonale alveoläre Mikrolithiasis--eine seltene Ursache bilateraler ausgedehnter pulmonaler Infiltrate.

BACKGROUND: Pulmonary alveolar microlithiasis is a rare disease, which is characterized by pulmonary deposition of calcium phosphate microliths. The radiographic features can be pathognomonic with a "sandstorm"-like opacification throughout the lungs. CASE REPORT: A 29-year-old Moroccan presented for the first time 1988 with atypical chest pain. His history was characterized by nephrocalcinosis and pulmonary disease. Physical examination and laboratory values were unremarkable. Chest X-ray revealed extensive bilateral pulmonary infiltrates with "sandstorm"-like opacifications. Sonography showed medullary nephrocalcinosis. The diagnosis of pulmonary alveolar microlithiasis was confirmed by transbronchial biopsy. Over the next 13 years there was only a moderate expansion of the pulmonary infiltrates and a beginning restrictive pattern, whereas the patient was almost symptom-free. CONCLUSION: Pulmonary alveolar microlithiasis is a rare cause of bilateral pulmonary infiltrates and should be considered in the differential diagnosis. In some cases there is also an association with calcifications of extrapulmonary organs.

Heterotaxy syndrome with severe pulmonary hypertension in an adult.

Heterotaxy syndrome is a rare clinical entity in adults, characterized by situs ambiguus, congenital heart defects, and splenic malformations. We report the case of an adult with heterotaxy syndrome (including situs ambiguus, bilateral superior vena cava, hypoplastic right-sided spleen and portosystemic shunts) presenting with dyspnea due to severe pulmonary hypertension. Vasodilatory therapy was initiated, leading to marked reduction of clinical symptoms. This case exhibits 2 particular and partially novel features: primary diagnosis of heterotaxy syndrome may be delayed until adulthood, and heterotaxy syndrome may be associated with pulmonary hypertension, possibly on the basis of longstanding portosystemic shunts.