Low serum pancreatic enzyme levels predict mortality and are associated with malnutrition-inflammation-atherosclerosis syndrome in patients with chronic kidney disease.

PURPOSE: Serum levels of amylase and lipase are frequently increased in patients with chronic kidney disease (CKD). Relatively low serum pancreatic enzyme levels in CKD may represent a state of pancreatic insufficiency and may contribute to protein-energy wasting (PEW). We aimed to investigate the relationships of serum pancreatic enzyme levels with PEW, inflammation, and mortality in CKD patients. METHODS: Two hundred and thirty CKD patients (male/female: 144/86; mean age, 59 ± 16 years) were enrolled. Serum total α-amylase and lipase activities were measured by enzymatic colorimetric assays. Mean follow-up time was 18 ± 10 months. Forty-seven patients (20 %) died during this period. RESULTS: Serum amylase levels were increased in 95 patients (41 %) and serum lipase levels were increased in 71 patients (30 %) out of the 230 patients. Diabetic patients had significantly lower serum amylase levels than non-diabetic ones (86 ± 46 vs. 111 ± 60 IU/L, p < 0.0001). Patients with ischemic heart disease also had significantly lower serum amylase (82 ± 37 vs. 108 ± 60 IU/L, p < 0.0001) and lipase levels (39 ± 36 vs. 57 ± 57 IU/L, p = 0.007). Serum amylase and lipase levels were directly correlated with serum creatinine (r = 0.173, p = 0.009 and r = 0.374, p < 0.0001) and albumin (r = 0.410, p < 0.0001 and 0.287, p < 0.0001), and inversely correlated with CRP (r = -0.223, p = 0.001 and r = -0.147, p = 0.027). The Kaplan-Meier analysis revealed survival advantages for both high-amylase and high-lipase groups in end-stage renal disease (ESRD) (CKD stage 5) patients (log rank, p < 0.001 and p = 0.02, respectively). In the Cox regression analysis, serum amylase was found to be an independent predictor for mortality. CONCLUSION: Serum amylase activity was found to be an independent predictor of mortality in ESRD patients. Relatively low serum pancreatic enzyme levels in CKD may be regarded as a novel component of the malnutrition-inflammation-atherosclerosis syndrome.

Clinical characteristics and predictors of progression of chronic kidney disease in autosomal dominant polycystic kidney disease: a single center experience.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease. The course and progression of the disease is highly variable. In this study, we aimed to investigate the impact of clinical characteristics and basic biochemical parameters on progression of chronic kidney disease (CKD) in ADPKD patients. MATERIALS AND METHODS: A total of 323 consecutive patients with ADPKD were enrolled into the study and followed with a mean duration of 100 ± 38 months. Patients were grouped as rapid progressors (RP) and slow progressors (SP) according to median rates of decline in glomerular filtration rate (ΔGFR) per year, namely 1 ml/min/year. RESULTS: History of macroscopic hematuria, urinary stone and smoking were more common in male patients; hepatic and other organ cysts were more common in female patients. ∆GFR/year was similar between males and females [0.95 (0-3.02) vs. 1.11 (0.10-2.74) ml/min/year, p = 0.21]. History of smoking and pack-year of cigarettes smoked were significantly higher in the RP compared to the SP group (36 vs. 18%, p = 0.01 and 5.24 ± 1.20 vs. 3 ± 1.32 pack-year, p = 0.02, respectively). Baseline 24 h-proteinuria was found to be significantly correlated with the percent decline of GFR (∆%GFR) per year (r = 0.303, 0.001). In Cox regression analysis for predicting the progression of CKD, age, hypertension, urinary stone and proteinuria were retained as the significant independent factors predicting progression of CKD in the model. CONCLUSION: Baseline proteinuria was significantly correlated with ∆%GFR per year. Hypertension and proteinuria were found to be the major treatable risk factors for the progression of CKD in ADPKD patients.

An unusual case of renal failure due to solitary plasmacytoma: parenchymal invasion of the kidney.

Solitary extramedullary plasmacytoma (EMP) is a rare plasma cell disorder mostly involving the upper airway; however, retroperitoneal infiltration is very rare. Kidney injury associated with EMP is exceptionally rare with only anecdotal reports. Herein we report a case of retroperitoneal EMP causing renal failure by the way of direct renal parenchymal infiltration. Renal parenchymal invasion should be considered in aggressive and refractory plasma cell dyscrasias with unexplained renal failure.

Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome.

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by headache, altered consciousness, seizures, and cortical blindness. The most frequent etiological factors are hypertension, kidney diseases, and immunosuppressive drugs such as steroids and cyclophosphamide. Herein we present a case of a 22-year-old female patient presented with alveolar hemorrhage and acute renal failure necessitating hemodialysis. In renal biopsy, necrotizing crescentic glomerulonephritis and immunofluorescence pattern compatible with Goodpasture syndrome were found. Anti-glomerular basement membrane antibody result was positive. At follow-up, respiratory failure ensued, steroid pulse treatment was started, and she was transferred to intensive care unit (ICU). In the ICU, she had visual disturbances and blindness together with seizures. Cranial magnetic resonance imaging (MRI) revealed irregular T2- and fluid-attenuated inversion recovery (FLAIR)-weighted lesions in bilateral occipital lobes. On clinical and radiological grounds, RPLS was diagnosed. With the supportive and anti-hypertensive treatment, RPLS was resolved without a sequela. Subsequent cranial MRI was totally normal. In the literature, RPLS associated with Goodpasture syndrome was reported only once. Hypertension and methylprednisolone might be the responsible etiologies in this case.

Cardiomyopathy mimicking left ventricular noncompaction in a patient with lupus nephritis.

A 37-year-old female patient was admitted with exertional dyspnea. Her serum creatinine was 2.4 mg/dL and anti-nuclear antibody was positive in a titer of 1/320. Renal biopsy revealed diffuse proliferative lupus nephritis. Echocardiography and cardiac magnetic resonance (MR) imaging showed increased apical trabeculations compatible with left ventricular noncompaction (LVNC), which is a rare genetic cardiomyopathy. The patient expressed a marked improvement in exertional dyspnea after the immune-suppressive treatment for systemic lupus erythematosus (SLE). Control echocardiography revealed a significant increase of ejection fraction. SLE may cause a kind of cardiomyopathy with high resemblance to LVNC. Discrimination of these two similar clinical entities is important because SLE-induced cardiomyopathy is potentially reversible after the immune-suppressive treatment for SLE.

Improvement of coronary flow velocity reserve with telmisartan in patients with autosomal-dominant polycystic kidney disease.

OBJECTIVES: Endothelial dysfunction (ED) has been reported in patients with autosomal-dominant polycystic kidney disease (ADPKD). Coronary flow velocity reserve (CFVR) is a noninvasive test showing endothelial function of epicardial coronary arteries and coronary microcirculatory function. The aim of this study was to investigate the effect of the angiotensin receptor blocker, telmisartan, on CFVR in patients with ADPKD. METHODS: Thirteen patients with ADPKD and well-preserved renal function and 22 healthy controls were included in the study. CFVR was measured at baseline and after dipyridamole infusion by echocardiography. CFVR was calculated as the ratio of hyperemic to baseline average peak diastolic velocities. After the baseline evaluation of CFVR, patients started telmisartan at a dose of 80 mg/day and were followed for 12 months. CFVR was remeasured after 6 and 12 months of therapy. RESULTS: Patients with ADPKD had significantly lower CFVR compared to healthy subjects. CFVR increased significantly after 6 months and 12 months of telmisartan therapy (P = 0.001) in patients with ADPKD. CONCLUSION: One year of telmisartan therapy significantly improved CFVR in patients with ADPKD. This finding suggests that the stimulation of the renin-angiotensin-aldosterone system contributes to the ED in these patients.

Coronary flow velocity reserve and carotid intima media thickness in patients with autosomal dominant polycystic kidney disease: from impaired tubules to impaired carotid and coronary arteries.

BACKGROUND AND OBJECTIVES: Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease. Endothelial dysfunction, an early and reversible feature in the pathogenesis of atherosclerosis, is associated with increased vascular smooth muscle tone, arterial stiffening, and increased intima-media thickness. Coronary flow velocity reserve is a noninvasive test showing endothelial function of epicardial coronary arteries and coronary microcirculatory function. The aim of the study was to investigate the carotid intima-media thickness and coronary flow velocity reserve in patients with autosomal dominant polycystic kidney disease. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Thirty normotensive patients with autosomal dominant polycystic kidney disease (10 male, 20 female) with well-preserved renal function and 30 healthy subjects (12 male, 18 female) were included in the study. Coronary flow velocity reserve was measured at baseline and after dipyridamole infusion by echocardiography. Coronary flow velocity reserve was calculated as the ratio of hyperemic to baseline diastolic peak velocities. RESULTS: Carotid intima-media thickness was significantly higher in patients than in control subjects (0.80 +/- 0.29 versus 0.54 +/- 0.14 mm, respectively; P < 0.001). Moreover, coronary flow velocity reserve was significantly lower in patients than in control subjects (1.84 +/- 0.39 versus 2.65 +/- 0.68, respectively; P < 0.001). CONCLUSIONS: Normotensive patients with autosomal dominant polycystic kidney disease with well-preserved renal function have significantly increased carotid intima-media thickness and significantly decreased coronary flow velocity reserve compared with healthy subjects. These findings suggest that atherosclerosis starts at an early stage in the course of their disease in patients with autosomal dominant polycystic kidney disease.

Ambulatory blood pressure and endothelial dysfunction in patients with autosomal dominant polycystic kidney disease.

Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Endothelial dysfunction (ED), which is an early manifestation of vascular injury, has been shown in patients with ADPKD. However, the association between ambulatory blood pressure and ED has not been investigated in these patients. Forty-one patients with ADPKD having well-preserved renal function were included in the study. Ambulatory blood pressure monitoring was performed in all patients. Patients were divided into dipper and non-dipper groups. Endothelial function of the brachial artery was evaluated by using high-resolution vascular ultrasound. Endothelial-dependent dilatation was expressed as the percentage change in the brachial artery diameter from baseline to reactive hyperemia. The mean 24-hour systolic blood pressure was similar in both groups (125.5 +/- 10.7 mmHg in dippers and 121.2 +/- 14.3 in non-dippers, p > 0.05). There was also no significant difference between the mean 24-hour diastolic blood pressures in both groups (82.3 +/- 9.6 mmHg in dippers and 77.1 +/- 8.6 mmHg in non-dippers, p > 0.05). The nocturnal fall rate in systolic blood pressure was 11.1 +/- 1.2% in dippers and 0.98 +/- 0.9% in non-dippers (p = 0.001). The nocturnal fall rate in diastolic blood pressure was 14.0 +/- 0.9% in dippers and 3.8 +/- 0.8% in non-dippers (p = 0.001). Endothelial-dependent dilatation was significantly higher in dippers compared to non-dippers (6.22 +/- 4.14% versus 3.57 +/- 2.52%, p = 0.025). Non-dipper patients with ADPKD show significant ED, which has an important impact on cardiovascular morbidity and mortality.

Improvement of endothelial dysfunction with simvastatin in patients with autosomal dominant polycystic kidney disease.

Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Endothelial dysfunction (ED), which is an early manifestation of vascular injury, has been shown in patients with ADPKD. Statins have a beneficial effect in the reversal of ED. The aim of this study was to investigate the effects of a statin, simvastatin, on ED in patients with ADPKD. Sixteen patients with ADPKD having well-preserved renal function were included in the study. Endothelial function of the brachial artery was evaluated by using high-resolution vascular ultrasound. Endothelial-dependent dilatation (EDD) was expressed as the percentage change in the brachial artery diameter from baseline to reactive hyperemia. After the baseline evaluations of EDDs, patients were started treatment with simvastatin at a dose of 40 mg/day and were treated for six months. EDDs were recalculated after one and six months of therapy. Interleukin-6 (IL-6) and high-sensitivity C-reactive protein were also measured as markers of inflammation. Baseline EDD was 11.3 +/- 6.9% in patients with ADPKD. After one month of simvastatin treatment, EDD increased significantly to 14.6 +/- 4.6 % (P = 0.016 versus baseline). Endothelial-dependent dilatation further increased significantly to 18.9 +/- 7.5 % (P = 0.011 versus baseline, P = 0.048 versus first month) after six months of therapy. There was also a significant decrease in the level of IL-6 from 21.6 +/- 21.7 pg/mL to 9.1 +/- 3.5 pg/mL (P= 0.002). Six months of simvastatin therapy resulted in a significant improvement of ED in patients with ADPKD. This finding may be in part related to the pleiotropic effects of simvastatin.

A rare post-transplant malignancy, cerebellar hemangioblastoma: a case report.

INTRODUCTION: Post-transplant malignancies are among the most important complications in organ transplantation. Hemangioblastoma (HB) is especially prevalent in the cerebellum. CASE REPORT: A 20-year-old male who first started dialysis therapy, and then underwent kidney transplantation from a living-relative donor. Five years after transplantation, the patient suffered from vertigo and imbalance when walking. On cranial magnetic resonance imaging (MRI), a mass lesion in the right cerebellar hemisphere was observed, 3 x 3 x 3 cm in size, which was pushing against the fourth ventricle, and the right cerebellar peduncle. The patient had significant hydrocephaly. The mass lesion was removed by craniectomy. The pathological diagnosis was cerebellar hemangioblastoma. The symptoms and clinical findings improved. The patient was diagnosed with sporadic hemangioblastoma. Rapamycin therapy was started instead of cyclosporine, and the patient is being followed up without further problems. DISCUSSION: HB causes 2% of all intracranial tumors in the general population. It is generally sporadic in nature and approximately 20% can be associated with von Hippel-Lindau (VHL) syndrome. As in this case, MRI is preferred for the diagnosis. There was no pathology related to VHL disease in this patient's physical examination, family history, routine biochemical tests and abdominal MRI. The treatment is surgical excision of the tumor, as in this case. CONCLUSION: When cerebellar symptoms occur or a cerebellar mass lesion is detected in an organ recipient, HB should be considered in the differential diagnosis. The examination of patients with HB for a possible association with VHL disease is also required.

Biventricular diastolic dysfunction in patients with autosomal-dominant polycystic kidney disease.

BACKGROUND: Left ventricular diastolic dysfunction has been shown in patients with autosomal-dominant polycystic kidney disease (ADPKD). However, there is no study evaluating right ventricular functions in these patients. METHODS: In the present study, diastolic functions of both ventricles in normotensive and hypertensive ADPKD patients with well-preserved renal function were investigated. Fifteen hypertensive and 16 normotensive patients with ADPKD with well-preserved renal function, 16 patients with essential hypertension, and 24 healthy subjects were included in the study. Conventional left and right ventricular echocardiographic measurements were performed in all subjects. Left and right ventricular functions were investigated both by myocardial performance index (MPI) [calculated by dividing the sum of isovolumic contraction time and isovolumic relaxation time (IVRT) by ejection time] and by tissue Doppler imaging (TDI). RESULTS: Left ventricular deceleration time and IVRT were significantly prolonged in hypertensive patients with ADPKD compared with patients with essential hypertension and even in normotensive patients with ADPKD compared with healthy subjects. Left and right MPIs were significantly higher in patients with ADPKD compared with healthy subjects, showing systolic and diastolic dysfunction. Moreover, by using TDI, the peak early diastolic mitral annular velocity (Em) to peak late diastolic mitral annular velocity (Am) ratio and the peak early diastolic tricuspid annular velocity (Et) to peak late diastolic tricuspid annular velocity (At) ratio were decreased in patients with ADPKD, suggesting biventricular diastolic dysfunction. CONCLUSION: Both hypertensive and normotensive patients with ADPKD show significant biventricular diastolic dysfunction, suggesting cardiac involvement very early in the course of ADPKD.

Endothelial dysfunction and increased carotid intima-media thickness in patients with autosomal dominant polycystic kidney disease.

BACKGROUND: Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease (ADPKD). Endothelial dysfunction (ED) and intima-media thickness (IMT) are predictors for the development and progression of atherosclerosis. In the present study, ED and IMT were investigated in patients with ADPKD. METHODS: Fifteen hypertensive and 16 normotensive patients with ADPKD with preserved renal function, 16 patients with essential hypertension, and 24 healthy subjects were included in the study. Endothelial function of the brachial artery was evaluated by means of high-resolution vascular ultrasound. Endothelial-dependent dilatation (EDD) was assessed by establishing reactive hyperemia, and endothelial-independent dilatation was determined by using sublingual isosorbide dinitrate. Carotid IMT was measured by means of high-resolution vascular ultrasound. RESULTS: EDD was significantly worse in hypertensive patients with ADPKD compared with patients with essential hypertension (9.1% +/- 4.1% versus 12.4% +/- 4.6%; P < 0.05) and even in normotensive patients with ADPKD compared with healthy subjects (13.1% +/- 5.2% versus 18.1% +/- 8.1%; P < 0.01). Moreover, carotid IMT was significantly greater in both hypertensive (0.71 +/- 0.10 mm; P < 0.01) and normotensive (0.57 +/- 0.14 mm; P < 0.001) patients with ADPKD compared with healthy subjects (0.45 +/- 0.10 mm). CONCLUSION: Both hypertensive and normotensive patients with ADPKD show significant ED and increased IMT, which are predictors of atherosclerosis.