Engin Melek

Acute, Reversible Nonoliguric Renal Failure in Two Children Associated With Analgesic-Antipyretic Drugs

Abstract Analgesic-antipyretic agents and nonsteroidal anti-inflammatory drugs are the most commonly used medications worldwide for the treatment of pain and fever in children. Acute renal failure is commonly seen in adults after treatment with analgesic agents. This complication has rarely been reported in children. Here, we describe 2patients admitted to our hospital with acute nonoliguric renal failure temporally associated with ingestion of analgesic-antipyretic drugs at therapeutic doses. The first case was a 16-year-old adolescent boy, who had taken acetaminophen (APAP) and mefenamic acid for the indication of upper respiratory tract infection with daily doses of 1500 and 500 mg, respectively. His serum urea nitrogen and creatinine values were 16 and 1.6 mg/dL. The second case was a 12-year-old boy, who had taken APAP with a daily dose of 500 mg for abdominal pain. His serum urea nitrogen and creatinine values were 21 and 2.29 mg/dL. Both of them recovered with appropriate hydration within a week. Over-the-counter analgesic-antipyretic agents seem innocent but carry the risk of acute renal failure even at therapeutic doses. We believe that increased caution and awareness of the toxic effects of APAP and nonsteroidal anti-inflammatory drugs are needed. We suggest that clinicians should be careful while using analgesic-antipyretic-anti-inflammatory drugs especially in children with subclinical dehydration.

The predictive value of resistive index obtained by Doppler ultrasonography early after renal transplantation on long‐term allograft function

DUSG is a useful diagnostic tool for the follow‐up of renal transplant recipients. The measurement of intrarenal arterial RI by DUSG has been proven to predict short‐term AF. The aim of the study was to evaluate the predictive value of DUSG performed during the early after RTx on long‐term AF. Seventy patients were enrolled into study. DUSG was performed at third and seventh days after RTx. Patients were divided into two groups according to rate of recovery of graft function as patients with normal graft function and abnormal graft function. Although the RI values were correlated with the AF early after transplantation, they were not correlated with long‐term AF. However, the rate of recovery of graft function at early period after RTx was correlated with creatinine level at first year and with glomerular filtration rate at first year and last visit. Although the RI has no predictive value for long‐term AF, the rate of recovery of graft function at early post‐transplantation period has predictive value for long‐term AF; patients with higher RI values early after RTx should be followed carefully for the development of chronic allograft injury.

Extra-Renal manifestations of atypical hemolytic uremic syndrome in children

BackgroundAtypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood.MethodsThis study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded.ResultsThe most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates.ConclusionsThe most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.

Favorable Outcomes of Renal Transplant in Children With Abnormal Lower Urinary Tract.

OBJECTIVES Chronic kidney disease caused by lower urinary tract abnormalities is a significant complication in pediatric care. Although there are conflicting reports about clinical outcomes in the past, favorable outcomes have been reported in recent years. Despite this, many centers still refrain from performing renal transplant in these patients. Here, we compared clinical outcomes of renal transplant recipients with and without lower urinary tract abnormalities. MATERIALS AND METHODS Our study included 71 renal transplant recipients who were divided into 3 groups: 17 patients with abnormal lower urinary tracts having vesicoureteral reflux (group 1), 7 patients with abnormal lower urinary tracts having bladder dysfunction (group 2), and 47 patients with anatomically and functionally normal lower urinary tracts (group 3). We retrospectively compared demographic features, clinical course, graft survival, pre- and posttransplant incidence of urinary tract infections, and final graft function among the groups. RESULTS There were no statistically significant differences among groups regarding median age at time of transplant, graft survival, median creatinine level, and median glomerular filtration rate (P > .05). Significant differences were shown in incidence of urinary tract infections between patients in groups 1 and 2 (abnormal lower urinary tracts) and group 3 (normal lower urinary tracts) before transplant (P < .05). Although frequency of urinary tract infections in groups 1 and 2 were moderately higher than shown in group 3 after transplant, this difference was not statistically significant. CONCLUSIONS Although the children with abnormal lower urinary tracts had slightly higher incidence of urinary tract infections, there were no differences between patients with abnormal and normal lower urinary tracts regarding allograft survival and function. In addition, proper follow-up of patients before and after transplant, based on our experience, should include educating patients and their parents about potential complications after transplant for the best outcome of renal transplant.

Analysis of Genitourinary Anomalies in Patients with Fanconi Aplastic Anemia

Objective: Fanconi aplastic anemia (FAA) is a rare genetic disease that causes bone marrow failure. It is the most common bone marrow failure syndrome among the hereditary bone marrow failure syndromes. Genitourinary system anomalies occur in about one third of patients with FAA. Renal malformations that can lead to end stage chronic kidney disease can be one of the major causes of morbidity and mortality in these patients and requires detailed review. Material and Methods: In order to evaluate genitourinary system malformations in FAA patients, eleven FAA patients who were diagnosed with bone marrow aspiration and mutation analysis, accompanied by genitourinary system anomalies, and followed at Cukurova University Department of Pediatric Hematology were retrospectively analyzed, The presence of genitourinary system anomalies were detected by renal ultrasonography, dimercaptosuccinic acid (DMSA) scintigraphy and voiding cystourethrogram. Results: Five of the eleven patients with FAA were girls (45.5%) and six of them were boys (54.5%). Seven patients (63.6%) had bilateral vesicoureteral reflux (VUR) and one patient (9%) had unilateral VUR. Four patients (36.4%) had unilateral renal agenesis and two patients (18.2%) had an ectopic kidney. Three patients (27.2%) had neurogenic bladder requiring clean intermittent catheterization. One of the patients had a penile deformity and urethral stricture.

Early Onset Gitelman Syndrome: A Case Report

Gitelman syndrome is characterized by hypokalemic metabolic alkalosis with hypomagnesemia and hypocalciuria. It is the most frequent inherited tubulopathy in adulthood. The cause is a genetic defect in the SLC12A3 gene that encodes the thiazide-sensitive Na-Cl co-transporter in the distal convoluted tubule cells. Gitelman syndrome is usually diagnosed at adulthood. Most cases present after six years of age and it is only rarely clinically defined before this age. We report a five-year-old patient who presented with tetany and was diagnosed with Gitelman syndrome, and review the literature.

Vaccination Practices in Pediatric Dialysis Patients Across Europe. A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group Study

Background: Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. Methods: A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization. The immunization practices and center protocols for monitoring antibody titers after vaccination in dialysis patients were obtained through a questionnaire. Results: All centers included in the study recommended immunization against hepatitis B virus (HBV), diphtheria, tetanus, pertussis, Hemophilus influenzae type b (Hib), poliomyelitis, measles, mumps, rubella (MMR), and streptococcus pneumonia in dialysis patients. In 16 centers, dialysis patients were vaccinated against influenza virus annually. HBV protective antibody titers were measured in 17 centers (during dialysis period in 14 centers, during pre-renal transplantation preparations in 14 centers or in both times in 11 centers). Hepatitis A virus (HAV) was reported to be followed in 13 centers, in 8 centers during dialysis period, and in 11 centers during pre-RTx preparations. MMR and varicella-zoster virus (VZV) protective antibody titers were measured during the dialysis period or before renal transplantation (RTx) in 12 and 15 centers, respectively, and in 6 centers both titers were checked both times. Conclusion: There are variations in vaccination practice across Europe. Children with CKD, those undergoing dialysis, and transplant candidates should receive age-appropriate vaccinations before RTx as well as before the transition to adult nephrology clinics and antibody levels should be monitored to evaluate the immunization status before and after RTx.

Konjenital nefrotik sendromlu bir infantta periton diyalizine bağlı olarak görülen nadir bir komplikasyon olarak şiloz asit

Şiloz asit; periton diyalizi yapilan hastalarda nadir gorulen fakat onemli bir non-enfeksiyoz komplikasyondur. Konjenial nefrotik sendromlu 1,5 aylik erkek hasta genel durumunda kotulesme, diffuz odematoz gorunum ve solunum sikintisi nedeni ile cocuk yogun bakim unitesine kabul edildi. Yatisinin ucuncu gununde periton diyaliz kateteri takilarak periton diyalizi uygulanmaya baslandi. Periton diyaliz kateteri takildiktan bes gun sonra (yatisinin sekizinci gununde) diyaliz sivisi sut gorunumu almaya basladi. Sut gorunumundeki sivinin biyokimyasal analizi siloz asit karakteri gosteriyordu: trigliserit 468 mg/dl. Şiloz asit kisa zincirli yag asitlerinden zengin dusuk lipidli diyet ve somatostatin analogu (octreotid) infuzyonu ile tedavinin 5. gununde duzeldi ve hasta tedavinin devami icin pediatri servisine transfer edildi.

Urolithiasis Frequency and Risk Factors in Home Ventilated Patients with Tracheostomy

©Telif Hakkı 2017 Çocuk Acil Tıp ve Yoğun Bakım Derneği Çocuk Acil ve Yoğun Bakım Dergisi, Galenos Yayınevi tarafından basılmıştır. Yazışma Adresi/Address for Correspondence: Dr. İlknur Tolunay, Çukurova Üniversitesi Tıp Fakültesi, Çocuk Yoğun Bakım Bilim Dalı, Adana, Türkiye E-posta: ilknurtolunay@gmail.com ORCID ID: orcid.org/0000-0002-3454-8483 Geliş Tarihi/Received: 30.07.2017 Kabul Tarihi/Accepted: 14.09.2017 Giriş: Bu çalışmada, ev tipi ventilatör ile izlenen trakeostomili hastalarda ürolitiazis sıklığı ve risk faktörlerinin değerlendirilmesi amaçlanmıştır. Yöntemler: Ocak 2014 ve Aralık 2015 tarihleri arasında çocuk yoğun bakım ünitemize yatırılan ve ev tipi ventilatörü olan trakeostomili 30 hasta geriye dönük olarak değerlendirilmiştir. Hastalara ait yaş, cinsiyet, altta yatan hastalık, kullanmakta olduğu ilaçlar, beslenme şekli, beslenme ürünü, kan biyokimyası, parathormon, 25(OH) vitamin D düzeyi, spot idrarda kalsiyum/kreatin, üriner sistem ultrasonografisi bilgileri hastaların arşiv dosyaları ve bilgisayar sisteminden elde edilmiştir. Bulgular: Hastaların %30’u (9/30) kız, %70’i (21/30) erkek olup yaş ortanca 70 ay (15-197 ay) idi. Motor fonksiyon sınıflama sistemine göre hastaların %33,3’ü (10/30) skor 4, %66,7’si (20/30) skor 5 olup tamamı immobil hastalar idi. Üriner sistem ultrasonografisi ile hastaların %30’unda (9/30) üst üriner sistem taşı saptandı. Hastaların %56,7’sinde (17/30) spot idrar kalsiyum/kreatin 0,21’in üzerinde bulundu. Yaşa göre spot idrar kalsiyum/kreatin hastaların %66,6’sında (20/30) yüksek saptandı. Hastaların günlük kalsiyum alımı 541±182 (336-1200) mg/gün; vitamin D alımı 25,9±10,6 (7,8-51,4) μg/gün idi. Hastaların tamamında kan kalsiyum düzeyi normal sınırlarda (8,5-10,5 mg/dL) olup kan 25(OH) vitamin D düzeyi 8 hastada >30 ng/mL, 13 hastada 20-30 ng/mL ve 9 hastada <20 ng/mL idi. Sonuç: Ev tipi ventilatör ile izlenen trakeostomili hastalarda immobilizasyon ürolitiazis açısından önemli bir risk faktörü olup bu hastaların eşlik eden metabolik bozukluklar açısından düzenli olarak takip edilmesi gerekmektedir. Anahtar Kelimeler: Çocuk, immobilizasyon, ürolitiazis, ev tipi ventilatör hastas

An ignored cause of red urine in children: rhabdomyolysis due to carnitine palmitoyltransferase II (CPT-II) deficiency.

Abstract Carnitine palmitoyltransferase II (CPT-II) deficiency is an autosomal recessively inherited disorder involving the β-oxidation of long-chain fatty acids, which leads to rhabdomyolysis and subsequent acute renal failure. The clinical phenotype varies from a severe infantile form to a milder muscle form. Here, we report a 9-year-old boy referred to our hospital for the investigation of hematuria with a 2-day history of dark urine and malaise. As no erythrocytes in the microscopic examination of the urine and hemoglobinuria were present, myoglobinuria due to rhabdomyolysis was the most probable cause of dark urine. After excluding the other causes of rhabdomyolysis, with the help of metabolic investigations, the patient was suspected to have CPT-II deficiency, the most common cause of metabolic rhabdomyolysis. Our aim in presenting this case is to emphasize considering rhabdomyolysis in the differential diagnosis of dark urine in order to prevent recurrent rhabdomyolysis and renal injury.