Occult Adrenal Insufficiency in Renal Amyloidosis Patients.

OBJECTIVE: Systemic amyloidosis may affect many organs, and may cause endocrinologic problems which may result in adrenal insufficiency. However, assessment of adrenocortical reserve is challenging in amyloidosis patients with renal involvement. We aimed to evaluate adrenocortical reserve with various methods of cortisol measurement to determine any occult clinical condition. METHODS: Patients with renal amyloidosis and healthy subjects were evaluated in this cross-sectional study. Basal cortisol, corticosteroid-binding globulin (CBG), and albumin levels were measured. Serum free cortisol (cFC) level was calculated. Cortisol response tests performed after ACTH stimulation test (250 µg, intravenously) were evaluated, and free cortisol index (FCI) was calculated. RESULTS: Twenty renal amyloidosis patients, and 25 healthy control subjects were included in the study. Patients and control subjects had similar median serum baseline cortisol levels [258 (126-423) vs 350 (314-391) nmol/L, p=0.169)] whereas patients' stimulated cortisol levels at the 60th minute were lower [624 (497-685) vs 743 (674-781) nmol/L, p=0.011)]. The 60th-minute total cortisol levels of 8 of the 20 (40%) amyloidosis patients were <500 nmol/L, but only three of these 8 patients had stimulated FCI <12 nmol/mg suggesting an adrenal insufficiency (15%). CONCLUSION: ACTH stimulation test and cortisol measurements should be considered in renal amyloidosis patients with severe proteinuria to avoid false positive results if only ACTH stimulation test is used. It will be appropriate to evaluate this group of patients together with estimated measurements as FCI.

Characteristics of primary glomerular diseases patients with hematuria in Turkey: the data from TSN-GOLD Working Group.

PURPOSE: Hematuria is one of the most common laboratory findings in nephrology practice. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular disease (PGD) patients with hematuria in our country. METHODS: Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. Demographic characteristics, laboratory and biopsy findings were also recorded. RESULTS: Data of 3394 PGD patients were included in the study. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Patients with hematuria had statistically higher systolic blood pressure, serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria. However, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-h proteinuria, serum total, HDL and LDL cholesterol, and C3 levels when compared with patients without hematuria. Hematuria was present 609 of 1733 patients (35.8%) among the patients presenting with nephrotic syndrome, while it was presented in 1090 of 1661 (64.2%) patients in non-nephrotics (p < 0.001). CONCLUSION: This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

Urinay neutrophil gelatinase-associated lipocalin as a biomarker in different renal problems

Background/aim: Neutrophil gelatinase-associated lipocalin (NGAL) is used previously to estimate the etiology, severity, and clinical outcomes of acute kidney injury (AKI). However, the role of urinary NGAL (uNGAL) in the postrenal setting is not clear. In our study, we aimed to discover the cut-off value of uNGAL that can be used in the differential diagnosis of underlying AKI etiologies. Materials and methods: In this prospective cross-sectional study, we examined 82 subjects in four groups: patients that had (1) postrenal AKI; (2) AKI other than postrenal etiologies; (3) stable chronic kidney disease; and (4) healthy subjects. A renal function assessment was carried out by measuring serum creatinine (sCr) and uNGAL at the time of diagnosis [0th min (T0)]. We followed the study group for three months. Results: At the time of diagnosis, sCr (T0) was highest in the postrenal AKI and AKI groups in contrast to stable chronic kidney disease patients and healthy subjects (P < 0.001), as expected. T0 median uNGAL was highest in the postrenal group (P < 0.001). Area under curve (AUC) of uNGAL to estimate postrenal AKI presence was 0.957 (95% CI, 0.897­1.000; P < 0.001). The cut-off point of uNGAL was 42.625 ng/mL for this estimation. Conclusion: Patients with AKI must be classified according to the underlying etiologies as soon as possible. uNGAL may be useful to estimate the etiologies, and whether the problem is acute or chronic in the course. In postrenal kidney problems, to plan the urgency of the urologic procedures, it is crucial.

The effect of Helicobacter pylori eradication on proteinuria in patients with primary glomerulonephritis.

INTRODUCTION: Glomerulonephritis is still the primary cause among the diseases causing end stage renal disease. Helicobacter pylori (HP), also having a local proinflammatory effect on gastric mucosa, can trigger a local and systemic inflammatory response, and consequently have a role in the development of extragastrointestinal defects. MATERIAL AND METHODS: The study was composed of patients diagnosed with primary glomerulonephritis who had dyspeptic complaints throughout the diagnosis. Patients who received endoscopic biopsy upon the determination of pathologic findings in their upper gastrointestinal endoscopy were HP positive in their biopsy material. A triple eradication therapy was initiated for HP. RESULTS: The study included 14 female and 19 male patients, 33 in total, whose biopsy material was determined to be HP positive. Before the eradication for HP, we found serum albumin to be 34.0 (19.0-51.0) g/l, serum total protein 58.6 ±12.9 g/l, serum creatinine 0.9 (0.5-1.2) and proteinuria 3069 (652-12392) mg/day in 24-hour urine. After the eradication, however, serum albumin was found to be 40 (20-52) g/l, serum total protein 62.3 ±11.1 g/l, serum creatinine 1.02 (0.6-1.29) mg/dl and proteinuria was 2850 (172-15181) mg/day in 24-hour urine. A comparison of the results showed that a statistically significant difference is established between the serum albumin, total protein and creatinine values (p = 0.001, p = 0.001 and p = 0.021, respectively), but not between proteinuria values in 24-hour urine (p = 0.990). CONCLUSIONS: Patients with primary glomerulonephritis, HP eradication treatment has an effect on serum albumin levels.

Unknown aspect of the old disease: does dyslipidemia in systemic AA amyloidosis differ from the dyslipidemia in primary glomerulonephritis?

AIM: To investigate the nature of dyslipidemia and its diversity in patients with systemic AA amyloidosis. METHODS: The reports of the kidney biopsies performed due to nephrotic proteinuria (>3.5 g/day/1.73 m(2)) with preserved renal function [glomerular filtration rate (GFR) >60 mL/min/1.73 m(2)] were reviewed. Clinical and laboratory data of the patients with systemic AA amyloidosis and primary glomerulonephritis (PG) were analyzed. RESULTS: A total of 104 (systemic AA amyloidosis: 43, PG: 61) patients were included in the study. Proteinuria and GFR levels were similar in both the groups. Patients with systemic AA amyloidosis group had lower serum albumin (p = 0.002), lower hemoglobin levels (p = 0.001), higher platelet counts (p = 0.002) and higher C-reactive protein levels (p = 0.001) compared to patients in PG group. Although the frequency of dyslipidemia was similar in the groups (86.0 vs. 93.4%), patients with systemic amyloidosis had both lower values of LDL-C (4.56 ± 2.05 vs. 5.49 ± 2.23 mmol/L, p = 0.028) and HDL-C (1.19 ± 0.36 vs. 1.35 ± 0.39 mmol/L, p = 0.035). Serum lipid levels were correlated with serum total protein, albumin and proteinuria levels in PG group. However, in the systemic amyloidosis group, only one clear correlation between serum lipid and hemoglobin levels was estimated. A multivariate analysis demonstrated that LDL-C was independently associated with the etiology of nephrotic proteinuria, serum total protein, serum albumin (inversely) and hemoglobin levels. CONCLUSIONS: Although dyslipidemia is closely associated with serum total protein, albumin and proteinuria in patients with PG, there is no clear such association in patients with systemic amyloidosis. Correlation between serum lipid and hemoglobin levels in this group and other findings point out that probably complex mechanisms take place in dyslipidemia of nephrotic syndrome caused by systemic AA amyloidosis.

Prognostic factors in crescentic glomerulonephritis: a single-center experience.

INTRODUCTION: Crescentic glomerulonephritis (CGN) is a fatal disease, rapidly leading to end-stage renal disease. Diagnosis should be accurate and treatment should be started immediately. We investigated the factors associated with the renal prognosis in CGN patients. MATERIALS AND METHODS: Forty-one patients with CGN who were followed up at the Nephrology Clinic of Ankara Numune Education and Research Hospital were divided into 2 arms of the dialysis-dependent group after treatment and the group that was followed up without dialysis. Demographic and clinical features along with biopsy findings during time of diagnosis were evaluated for both groups. RESULTS: The mean age was 41.3 ± 17.2 years old and 26 were men. Twenty patients developed end-stage renal disease, requiring long-term dialysis. The dialysis-dependent group had higher serum creatinine levels (8.2 ± 3.6 mg/dL versus 2.6 ± 2.5 mg/dL) and percentages of glomeruli with crescent (83.1 ± 19.1% versus 56.4 ± 11.9%), were more likely to have oligoruia-anuria (90.5% versus 9.5%) and be dialysis-dependent at admission (86.4% versus 13.6%), and had longer elapsed time until the beginning of treatment (18.9 ± 10.4 days versus 10.6 ± 3.0 days) after treatment. At admission, their serum creatinine was greater than 4.2 mg/dL and the rate of crescentic glomeruli was greater than 63%. CONCLUSIONS: In patients with CGN, renal prognosis is poor and the time of admission to the hospital, degree of renal insufficiency, presence of oligo-anuria, dialysis requirement, and the percentage of crescentic glomeruli on biopsy are closely related to progression to end-stage renal disease.

Coexistence of autosomal dominant polycystic kidney disease and amyloidosis in a patient with nephrotic-range proteinuria.

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the development and growth of cysts in the kidneys. Non-nephritic-range proteinuria is a common presentation in ADPKD patients; however, nephrotic syndrome is a rare coincidence. A 52-year-old man is described who was diagnosed with secondary amyloidosis with ADPKD. To our knowledge, this is the first case of amyloidosis associated with frequently infected renal cysts. Patients with ADPKD who show massive proteinuria should be investigated in terms of concomitant glomerular disease.

Evaluation of the mean platelet volume in secondary amyloidosis due to familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an inflammatory disorder that is leading cause of secondary amyloidosis (AA). This study was designed to investigate the level of mean platelet volume (MPV) in AA. Seventy-four FMF, 29 AA patients and 180 healthy controls, were included. There was no significant difference between the cases in terms of sex and age. MPV levels were measured in all groups. In the FMF group, MPV level was significantly higher when compared to the control group. MPV level was significantly lower in AA group in comparison with the FMF and healthy control groups. In summary, our present study showed low MPV values in AA due to FMF.

Revisiting secondary amyloidosis for an inadequately investigated feature: dyslipidemia.

Secondary amyloidosis is the most frequent form of the systemic amyloidosis around the world. Data on frequency and nature of dyslipidemia in patients with secondary amyloidosis are not conclusive. We evaluated the lipid abnormalities and their association with clinical and laboratory characteristics of the patients with secondary amyloidosis. The reports of the kidney biopsies performed in our hospital were reviewed. Clinical and laboratory data of the patients with biopsy-proven secondary amyloidosis were analyzed retrospectively. A total of 102 patients were diagnosed as having secondary amyloidosis. Familial Mediterranean fever was the leading cause of secondary amyloidosis accounting for 42.2 % of the cases. The most frequent indication for kidney biopsy was the nephrotic range proteinuria. The most common clinical and laboratory characteristics at the time of the diagnosis were edema, proteinuria and impaired renal function. The frequency of the nephrotic range proteinuria and microscopic hematuria were 75.5 and 18.6 %, respectively. Dyslipidemia was found in 88 % of the cases. Serum lipids significantly correlated with estimated glomerular filtration rate (eGFR), but not with serum albumin or urine protein levels. We demonstrated that majority of the patients with secondary amyloidosis had serum lipid abnormalities. Dyslipidemia was closely associated with GFR in a manner that patients with advanced stage kidney disease had lower serum lipid levels.

An uninvestigated risk factor for contrast-induced nephropathy in chronic kidney disease: proteinuria.

BACKGROUND: Contrast-induced nephropathy (CIN) is one of the most frequent causes of acute renal failure in hospitalized patients with the incremental use of contrast media. We aimed to investigate whether proteinuria may act as a risk factor for CIN in patients with chronic kidney disease. METHODS: Seventy hospitalized patients (37 men, 33 women) with chronic kidney disease, proteinuria, and/or estimated glomerular filtration rate (eGFR) of <60 mL/min/1.73 m2, who were exposed to contrast media were investigated prospectively. Thirty patients were diabetic. All patients received prophylaxis against CIN with acetylcysteine and 0.9% intravenous saline. CIN is defined as either a 25% higher increase in serum creatinine (sCr) from the baseline levels or a 0.5 mg/dL increase in sCr at 72 h after contrast media exposure. RESULTS: CIN was detected in 26 (37.1%) patients. Advanced age, diabetes, heart failure, anemia, baseline sCr of >1.5 mg/dL, baseline eGFR of <60 mL/min/1.73 m(2), proteinuria of ≥1 g/day, hypoalbuminemia, and the volume of contrast media of ≥100 mL correlated significantly with CIN. The frequency of CIN was significantly higher in patients with proteinuria of ≥1 g/day compared to patients with proteinuria of <1 g/day (p = 0.009). CONCLUSION: Proteinuria may be a new risk factor for the development of CIN in patients with chronic kidney disease.

Major barriers against renin-angiotensin-aldosterone system blocker use in chronic kidney disease stages 3-5 in clinical practice: a safety concern?

Renin-angiotensin-aldosterone system (RAAS) blockers are underutilized in patients with chronic kidney disease (CKD). We aimed to determine barriers against the use of RAAS blockers in these patients. Patients with stage 3-5 CKD referred to Hacettepe University Hospital Nephrology Unit during a 1 year period were evaluated for RAAS blocker use. Two hundred and seventy-nine patients (166 male, 113 female) were analyzed. The mean age of the patients was 56.7 ± 15.2 years, mean serum creatinine was 2.45 ± 1.44 mg/dL, and mean glomerular filtration rate was 33.3 ± 15.1 mL/min. The mean follow-up time was 22.0 ± 21.9 months and the clinical visit number was 4.0 ± 3.5. Angiotensin-converting-enzyme inhibitors or angiotensin receptor blockers were used by 68.8% of all patients and 67.7% of diabetic patients at the time of analysis. In 82.1% of patients, RAAS blockers had either been used earlier or were being used. Hyperkalemia was the principal reason for both not starting and also discontinuing these drugs in patients with CKD. In 37.4% of patients, reasons for not starting RAAS blockers were unclear. This study showed that hyperkalemia is the major barrier against the use of RAAS blockers in patients with CKD. There was, however, a subset of patients who did not receive RAAS blockers even without clear contraindications.

Acute thrombotic thrombocytopenic purpura following orthopedic surgery: case report and review of the literature.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon cause of hematological and renal abnormalities in the postoperative period. An association between TTP and orthopedic surgery, a rare entity, has been reported in the literature. It has the strong possibility of being fatal and therefore should be treated immediately, mostly by plasmapheresis. We report a 15-year-old girl of TTP following a high tibial valgus osteotomy (HTO).

Use of mesenchymal stem cells and darbepoetin improve ischemia-induced acute kidney injury outcomes.

BACKGROUND: Interest has recently been focused on the possible role of bone marrow-originating stem cells and the therapeutic role of erythropoietin in the recovery of ischemia-induced acute kidney injury (AKI). The aim of the present study was to compare treatment with mesenchymal stem cells (MSCs) to treatment with darbepoetin-α (DPO) or both concomitantly in a rat model of ischemia/reperfusion (I/R) AKI. METHODS: Forty male Sprague-Dawley rats were included, and 28 of them were randomly assigned to controls (treated with serum physiologic) or one of the three treatment groups treated with either DPO, MSCs, or both (MSCs and DPO concomitantly) after the induction of I/R injury. Hematocrit, serum creatinine, and BUN levels were obtained at 0, 24, 48, and 72 h of surgery, and renal tissue was obtained at 72 h after nephrectomy for histological analysis. Tissue injury was quantified by standardized histological scoring systems, using light and electron microscopes. RESULTS: Treatment with MSCs or DPO improved renal function compared with controls. However, the improvement observed in renal function in the MSC/DPO group was better than that in the other groups. Histological analysis demonstrated that tissue injury was significantly decreased in rats in the MSC or DPO groups compared to that of the controls; however the best recovery was observed in rats treated with MSCs and DPO concomitantly. CONCLUSION: These results suggest that concomitant application of DPO and MSCs may be a potential novel renoprotective therapy for patients after having sustained an ischemic renal insult.

High frequency of aspirin resistance in patients with nephrotic syndrome.

BACKGROUND: Aspirin has a beneficial role in prevention of cardiovascular and thromboembolic events. Patients may experience thromboembolic events despite aspirin treatment, a phenomenon called aspirin resistance. We evaluated the frequency of aspirin resistance and its correlation with clinical and biochemical parameters among patients with nephrotic syndrome (NS). METHODS: A total of 83 patients (50 males, 33 females, age range 18-79 years) with NS using aspirin 100 mg/day were included in the study. Demographic information and aetiology of NS based on the histology of a renal biopsy were recorded for each patient. Blood samples were drawn to investigate the association of aspirin resistance with inflammation and thrombotic risk factors. Aspirin resistance was defined as a normal collagen/epinephrine closure time<159 s using a platelet function analyzer (PFA-100). RESULTS: Aspirin resistance was determined in 51 patients (61.4%). The number of patients exposed to azathioprine therapy was significantly higher in the aspirin-sensitive group (P=0.043), whereas patients exposed to cyclosporine therapy were significantly higher in the aspirin-resistant group (P=0.017). More patients in the aspirin-resistant group were on angiotensin-converting enzyme inhibitor therapy compared with the aspirin-sensitive group (P=0.024). The aspirin-resistant group showed significantly higher serum low-density lipoprotein cholesterol (LDL-C) (151±47 versus 104±21 mg/dL; P<0.001), triglyceride levels (192±116 versus 134±82 mg/dL; P=0.015) and glomerular filtration rates (91.8±43.0 versus 74.0±35.6 mL/min/1.73 m2; P=0.044) compared with the aspirin-sensitive group. In multivariate analysis, LDL-C was the only parameter associated independently with aspirin resistance [odds ratio (OR) 1.04, 95% confidence interval (CI) 1.02-1.06; P=0.004]. CONCLUSIONS: A significant number of patients with NS are resistant to aspirin therapy. Serum LDL-C level is closely associated with aspirin resistance in NS.

Coexistence of medullary sponge kidney and renal AA amyloidosis in a patient with nephrotic range proteinuria.

We report a patient with medullary sponge kidney (MSK) who presented with hematuria and nephrotic-range proteinuria. Renal biopsy revealed a diagnosis of renal AA amyloidosis. No secondary factors contributing to renal amyloidosis were demonstrated. To the best of our knowledge, this is the first reported case that demonstrates the coexistence of MSK and renal AA amyloidosis.

Renal cell carcinoma extending from superior vena cava into right atrium.

Tumoral invasion of inferior vena cava by renal cell carcinoma is reported to be relatively frequent. Usually the tumor grows intraluminally into the renal vein and inferior vena cava as an extension of primary tumor. In this report, we present an unusual case of venous system involvement, invasion of renal cell carcinoma into superior vena cava.

Endobronchial metastasis of thyroid follicular carcinoma.

Well-differentiated forms of thyroid cancer, including follicular carcinoma, usually have good prognoses. But they are also known to metastasize to the bones, lungs and central nervous system. Endobronchial metastasis is exceptionally rare. In this paper, we report on a patient with endobronchial metastasis of follicular thyroid carcinoma. A 77-year-old male patient presented to our hospital with hemoptysis and a growing mass over the right clavicula. Computerized tomography (CT) of the chest revealed multiple lung metastases. Flexible bronchoscopy revealed a fragile polypoid mass of 1.5 cm, 6 cm distal to the vocal cords. Biopsy from the supraclavicular mass was consistent with follicular thyroid carcinoma. Taking into account the advanced nature of the disease as well as the general condition and age of the patient, aggressive treatment modalities were not considered in the management. Palliation for hemoptysis was attained by external radiotherapy. After radiotherapy, hemoptysis did not recur, and the patient was discharged. Although endobronchial metastasis of thyroid follicular carcinoma is very rare, the presence of endobronchial metastasis may be life threatening due to massive hemoptysis, and such a lesion must be suspected in any cancer patients presenting with hemoptysis.