Presentation antineutrophil cytoplasmic autoantibody negative-associated granulomatosis with polyangiitis with pyuria

Pulmonary renal syndrome is a rare but serious complication of systemic vasculitis. The majority the cases are antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. In this case, a 58 years old female case having aworsening clinical picture of probable urinary infection and acute renal failure was presented. By means of proteinuria, pyuria, hematuria and unexplained acute renal failure, we review the relevant literature on pulmonary renal syndrome associated with granulomatosis with polyangiitis (GPA) with negative serum ANCA serology. This report demonstrates the difficulty of diagnosing granulomatosis with polyangiitis (GPA) until renal biopsy was done while initial diagnostic serological negative ANCA testing

Interrupted Aortic Arch in an Adult with Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is responsible for 8–10% of patients with end-stage renal failure. The major extrarenal complications of ADPKD are cardiovascular abnormalities. Interrupted aortic arch (IAA) is a lethal congenital cardiac abnormality seen with a frequency of 3/1000000 births and is defined as a segment of the arcus aorta being atresic. In the literature, there are no any reports showing that polycystic kidney disease and interrupted aortic arch occur together. In this study, we present a rare case in which the patient has polycystic kidney disease and IAA together and discuss whether IAA is a complication of ADPKD

Spontaneous Tumour Lysis Syndrome in a Multiple Myeloma

The tumor lysis syndrome (TLS) is a collection of metabolic abnormalities that occur in consequence of the release of intracellular contents following lysis of tumor cells. TLS occurs spontaneously or after chemotherapy. Spontaneous TLS is uncommon occurrence in multiple myeloma (MM). We define a case of a 70-year-old woman patient who was found to have MM with spontaneous TLS, following a compression fracture of the T-12 vertebrae. While serum uric acid and phosphorous levels were high, low calcium levels were identified. There were also acute kidney injury and metabolic acidosis. Upon the diagnosis of TLS, she was treated with hydration, allopurinol, sodium bicarbonate, and calcium gluconate. The improvement of her laboratory data was observed. We submitted this case in order to draw attention to the presentation of MM with spontaneous TLS

Prevalence of Fabry Disease in Familial Mediterranean Fever Patients from Central Anatolia of Turkey

WOS: 000379533500009PubMed ID: 27105876Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (AGALA) activity. FD and familial Mediterranean fever (FMF) have typical clinical similarities, and both diseases may progress to end-stage renal diseases. In this study, we aimed to determine the prevalence of FD in patients with FMF from Central Anatolia of Turkey. The study group consisted of 177 FMF patients, followed up by the Adult and Pediatric Nephrology Clinic of Cumhuriyet University Hospital. Screening for AGALA activity was performed by the dry blood spot method. Mutation analysis for GLA gene was carried out for patients having an AGALA enzyme activity value lower than the normal reference value. Low AGALA activity was detected in 23 (13 %) patients. Heterozygous GLA gene mutation c.[937G > T] p.[D313Y] was detected in one female patient (0.56 %). The patient was a 53-year-old female with proteinuria and who had undergone left nephrectomy; her glomerular filtration rate (GFR) by scintigraphy was found to be 70 ml/min. She had M694V mutation and no clinical manifestation of FD. In our study, the prevalence rate of FD was found as 0.56 % in FMF patients. The similarities between the symptoms of FMF and FD might lead to a diagnostic dilemma in physicians at countries where FMF is observed frequently. Although the prevalence of FD is rare, physicians should keep in mind that FD has an ambiguous symptomology pattern of FMF

Acute renal infarction in Turkey: a review of 121 cases

Kurultak, Ilhan (Trakya author)PurposeRenal infarction is a clinical condition which is caused by renal artery occlusion and leads to permanent renal parenchymal damage. In the literature, there are generally case reports on this subject, and few studies that include a large group of patients. Therefore, we aimed to present the data of a large group of patients who were diagnosed with acute renal infarction in our country in this retrospective study.MethodsThe data of patients who were diagnosed with acute renal infarction according to clinical and radiological findings in Turkey in the last 3years were examined. For this purpose, we contacted with more than 40 centers in 7 regions and obtained support from clinically responsible persons. Demographic data of patients, laboratory data at the time of diagnosis, tests performed for etiologic evaluation, given medications, and patients' clinical status during follow-up were obtained from databases and statistical analysis was performed.ResultsOne-hundred and twenty-one patients were included in the study. The mean age was 531.4 (19-91) years. Seventy-one (58.7%) patients were male, 18 (14.9%) had diabetes, 53 (43.8%) had hypertension, 36 (30%) had atrial fibrillation (AF), and 6 had a history of lupus+antiphospholipid syndrome (APS). Forty-five patients had right renal infarction, 50 patients had left renal infarction, and 26 (21.5%) patients had bilateral renal infarction. The examinations for the ethiologies revealed that, 36 patients had thromboemboli due to atrial fibrillation, 10 patients had genetic anomalies leading to thrombosis, 9 patients had trauma, 6 patients had lupus+APS, 2 patients had hematologic diseases, and 1 patient had a substance abuse problem. Fifty-seven (57%) patients had unknown. The mean follow-up period was 14 +/- 2months. The mean creatinine and glomerular filtration rate (GFR) values at 3months were found to be 1.65 +/- 0.16mg/dl and 62 +/- 3ml/min, respectively. The final mean creatinine and GFR values were found to be 1.69 +/- 0.16mg/dl and 62 +/- 3ml/min, respectively.Conclusions Our study is the second largest series published on renal infarction in the literature. More detailed studies are needed to determine the etiological causes of acute renal infarction occurring in patients

Pregnancy and its outcomes in hemodialysis patients in Turkey

Background/aim: This study aimed to investigate pregnancy frequency and evaluate the factors affecting live births in hemodialysis (HD) patients. Materials and methods: Female HD patients whose pregnancy was retrospectively reported between January 1, 2014, and December 31, 2019. The duration of HD, primary disease, and the information on whether the pregnancy resulted in abortion, stillbirth, or live birth, whether the HD duration was prolonged after diagnosing the pregnancy and whether it accompanied preeclampsia were recorded. Results: In this study, we reached 9038 HD female patients' data in the study. A total of 235 pregnancies were detected in 145 patients. The mean age was 35.42 (35 +/- 7.4) years. The mean age at first gestation was 30.8 +/- 6.5 years. The average birth week was 32 (28 -36) weeks. A total of 53.8% (no = 78) of the patients had live birth, 51.7% (no = 70) had at least one abortion in the first 20 weeks, and 13.1% (no = 19) had at least one stillbirth after 20 weeks. The rate of patients' increased numbers of dialysis sessions during pregnancy was 71.7%. The abortion rate was 22.4% in those with increased HD sessions, whereas 79.3% in those not increased HD sessions (p < 0.001). Live birth frequency was 67.2% in the increased HD sessions group and 3.4% in those who did not differ in HD sessions (p < 0.001). Conclusion: For the first time, we reported pregnancy outcomes in HD female patients, covering all regions of Turkey. It has been observed that; increasing the number of HD sessions in dialysis patients will decrease fetal and maternal complications and increase live birth rates

Pregnancy and its outcomes in hemodialysis patients in Turkey

Background/aim: This study aimed to investigate pregnancy frequency and evaluate the factors affecting live births in hemodialysis (HD) patients. Materials and methods: Female HD patients whose pregnancy was retrospectively reported between January 1, 2014, and December 31, 2019. The duration of HD, primary disease, and the information on whether the pregnancy resulted in abortion, stillbirth, or live birth, whether the HD duration was prolonged after diagnosing the pregnancy and whether it accompanied preeclampsia were recorded. Results: In this study, we reached 9038 HD female patients' data in the study. A total of 235 pregnancies were detected in 145 patients. The mean age was 35.42 (35 +/- 7.4) years. The mean age at first gestation was 30.8 +/- 6.5 years. The average birth week was 32 (28 -36) weeks. A total of 53.8% (no = 78) of the patients had live birth, 51.7% (no = 70) had at least one abortion in the first 20 weeks, and 13.1% (no = 19) had at least one stillbirth after 20 weeks. The rate of patients' increased numbers of dialysis sessions during pregnancy was 71.7%. The abortion rate was 22.4% in those with increased HD sessions, whereas 79.3% in those not increased HD sessions (p < 0.001). Live birth frequency was 67.2% in the increased HD sessions group and 3.4% in those who did not differ in HD sessions (p < 0.001). Conclusion: For the first time, we reported pregnancy outcomes in HD female patients, covering all regions of Turkey. It has been observed that; increasing the number of HD sessions in dialysis patients will decrease fetal and maternal complications and increase live birth rates

Body mass index and complications following major gastrointestinal surgery: a prospective, international cohort study and meta-analysis.

AIM: Previous studies reported conflicting evidence on the effects of obesity on outcomes after gastrointestinal surgery. The aims of this study were to explore the relationship of obesity with major postoperative complications in an international cohort and to present a meta-analysis of all available prospective data. METHODS: This prospective, multicentre study included adults undergoing both elective and emergency gastrointestinal resection, reversal of stoma or formation of stoma. The primary end-point was 30-day major complications (Clavien-Dindo Grades III-V). A systematic search was undertaken for studies assessing the relationship between obesity and major complications after gastrointestinal surgery. Individual patient meta-analysis was used to analyse pooled results. RESULTS: This study included 2519 patients across 127 centres, of whom 560 (22.2%) were obese. Unadjusted major complication rates were lower in obese vs normal weight patients (13.0% vs 16.2%, respectively), but this did not reach statistical significance (P = 0.863) on multivariate analysis for patients having surgery for either malignant or benign conditions. Individual patient meta-analysis demonstrated that obese patients undergoing surgery for malignancy were at increased risk of major complications (OR 2.10, 95% CI 1.49-2.96, P < 0.001), whereas obese patients undergoing surgery for benign indications were at decreased risk (OR 0.59, 95% CI 0.46-0.75, P < 0.001) compared to normal weight patients. CONCLUSIONS: In our international data, obesity was not found to be associated with major complications following gastrointestinal surgery. Meta-analysis of available prospective data made a novel finding of obesity being associated with different outcomes depending on whether patients were undergoing surgery for benign or malignant disease