Publications by authors named "Aysun Karabay Bayazit"

43 Publications

Retrospective Analysis of the Factors Affecting Growth Parameters in Turkish Children With Systemic Lupus Erythematosus.

Arch Rheumatol 2020 Sep 8;35(3):357-365. Epub 2020 Jan 8.

Department of Pediatrics, Division of Pediatric Rheumatology, Çukurova University School of Medicine, Adana, Turkey.

Objectives: This study aims to investigate the growth parameters in Turkish children with systemic lupus erythematosus (SLE) and to compare these growth parameters according to the presence or absence of cyclophosphamide, rituximab treatment, cumulative corticosteroid dose, proliferative nephritis, and the last visit disease activity and damage index.

Patients And Methods: Medical data, growth parameters including z-scores for weight, height, and body mass index and parent-adjusted height z-scores of 45 juvenile SLE (jSLE) patients (5 males, 40 females; mean age 12.3±3.2 years; range 7.1 to 18 years) were retrospectively evaluated. Growth parameters were calculated by anthropometric references in Turkish children. The disease activity was assessed by the SLE Disease Activity Index 2000 (SLEDAI-2K). The disease outcome was measured by the Pediatric version of Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index.

Results: The median diagnostic delay was two months (range, 0-36 months). The median follow-up duration was 3.21 years (range, 0.63-9.48 years). Mean height z-score was significantly lower at last visit than at the time of diagnosis. The growth parameters did not differ according to age at disease onset, diagnostic delay, presence of proliferative nephritis, having cyclophosphamide and rituximab treatment and the last visit (SLEDAI-2K) scores. The median duration of corticosteroid treatment was 3.2 years (range, 0.6-9.4 years) and the median cumulative corticosteroid dosage was 13.9 g (range, 1.9-58 g) of methylprednisolone. The mean height z-score at last visit was significantly lower in those who received at least 10 g of cumulative dose of corticosteroid. The last visit mean parent-adjusted height z-scores did not differ significantly regarding the cumulative corticosteroid dose.

Conclusion: The last visit height and parent-adjusted height z-scores of jSLE patients were significantly lower. The patients treated with at least 10 g of cumulative dose of corticosteroids had lower mean height z-score.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.46497/ArchRheumatol.2020.7573DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7788651PMC
September 2020

From infancy to adulthood: challenges in congenital nephrogenic diabetes insipidus.

J Pediatr Endocrinol Metab 2020 Jul 4. Epub 2020 Jul 4.

Department of Pediatric Nephrology, Cukurova Univsersity Faculty of Medicine, Adana, Turkey.

Objectives Congenital nephrogenic diabetes insipidus (NDI) is a rare hereditary disorder which is characterized by unresponsiveness to arginine vasopressin (AVP) in collecting ducts and leads to polyuria and polydipsia. The wide clinical spectrum of congenital NDI can cause difficulties in early diagnosis. We aimed to evaluate clinical prognosis of children with congenital NDI in long-term period. Methods Nineteen children with congenital NDI followed up in Pediatric Nephrology Department were enrolled to the study. This study is a single-center retrospective study, which reports clinical follow-up and genetic results of children with congenital NDI. Results Presenting symptoms of patients were mostly dehydration and fever due to polyuria and polydipsia. Four male patients had bilateral nonobstructive hydroureteronephrosis (HUN) and neurogenic bladder which requires clean intermittent catheterization (CIC). One patient had intracranial calcification which is a rarely seen complication in congenital NDI due to recurrent hypernatremic dehydration and severe brain dehydration. The causative mutations were identified in all patients. The identified mutations in six of them (31.6%) were hemizygous mutations in AVPR2 gene and homozygous mutations of AQP2 gene in the rest 13 cases (68.4%). More than that, four of these mutations (two in AVPR2 and two in AQP2) were novel mutations. Noncompliance with the treatments is associated with high risk of morbidity due to neurogenic bladder and chronic kidney disease (CKD). Conclusions The prognosis of congenital NDI is good when diagnosis can be made early and treatment is started immediately. Genetic counseling and prenatal testing for hereditary diseases are recommended especially in regions with relatively higher rates of consanguineous marriages.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1515/jpem-2019-0529DOI Listing
July 2020

Long-term follow-up results of patients with ADCK4 mutations who have been diagnosed in the asymptomatic period: effects of early initiation of CoQ10 supplementation.

Turk J Pediatr 2019 ;61(5):657-663

Division of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara.

Atmaca M, Gülhan B, Atayar E, Karabay Bayazıt A, Candan C, Arıcı M, Topaloğlu R, Özaltın F. Long-term follow-up results of patients with ADCK4 mutations who have been diagnosed in the asymptomatic period: effects of early initiation of CoQ10 supplementation. Turk J Pediatr 2019; 61: 657-663. ADCK4-related glomerulopathy is a recently recognized clinical entity associated with insidious onset in young children and a high potential to progress to chronic kidney disease in adolescents. Early initiation of exogenous coenzyme Q10 (CoQ10) supplementation in the asymptomatic period could be protective on renal functions. In the present study, we aimed to investigate long-term follow-up of patients that we have diagnosed during the asymptomatic period and in whom we started CoQ10 treatment. We analyzed long-term effects of CoQ10 on proteinuria and estimated glomerular filtration rate (eGFR) in this patient population. A total of 8 patients (4 female, 4 male) from 6 different families were included. The mean age at diagnosis and at last visit were 16.8±11.2 years and 20.7±11.7 years, respectively. None of the patients had extrarenal system involvement. At the time of initiation of treatment; median eGFR was 107.8 ml/min/1.73 m2, median proteinuria was 1008 mg/m2/day. After a median follow-up period of 25.3±5.8 months, median proteinuria decreased to 318.5 mg/m2/day (p=0.03) and median eGFR remained stable at 99.6 ml/min/1.73 m2 (p=0.21). Coenzyme Q10 treatment is effective for reducing proteinuria and seems to be renoprotective.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2019.05.003DOI Listing
July 2020

Renal features of Bardet Biedl syndrome: A single center experience.

Turk J Pediatr 2019 ;61(2):186-192

Departments of Pediatric Nephrology, Çukurova University Faculty of Medicine, Adana, Turkey.

Atmış B, Karabay-Bayazıt A, Melek E, Bişgin A, Anarat A. Renal features of Bardet Biedl syndrome: A single center experience. Turk J Pediatr 2019; 61: 186-192. Bardet Biedl syndrome (BBS), is a multisystemic disorder which is described as a ciliopathy. BBS is a rare autosomal recessive disorder and 21 different BBS genes have been defined to date. BBS is characterized with dysmorphic extremities, retinitis pigmentosa, obesity, hypogenitalism, intellectual disabilility and renal structural abnormalities. Renal symptoms in patients with BBS, are nonspecific and often undetected until end-stage renal disease. Here, we were reported 23 children with BBS (12 females, 11 males) with renal abnormalities from a single center and defined their features. Age at diagnosis were very variable (2 days-16 years). Median age at diagnosis was 84 months. Mean follow-up period was 42 months. All 23 children had urinary tract abnormalities on renal ultrasonography. These abnormalities were polycysts (34.8%), hyperechogenic kidneys (34.8%), fetal lobulation (21.7%), hypoplasia on at least one kidney (21.7%) and hydronephrosis in at least one kidney (17.4%). Vesicoureteral reflux and neurogenic bladder detected 11.1% and 22.2% of patients who recieved a voiding cystourethrogram, respectively. Proteinuria was found in 39 % of patients. Hypertension was defined in 21.7% of patients. Six of 23 children (26%) in our cohort had proven mutations in BBS genes. Five of them (83.3%) had homozygous mutations in BBS10 gene and one of them had homozygous mutation in BBS2 gene. All of 23 children had retinitis pigmentosa, twenty two of them (95.6%) had learning disabilities/cognitive impairment and seventeen of them (82.6%) had obesity. Renal involvement is now accepted as a cardinal feature and the most important factor causing mortality in BBS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2019.02.006DOI Listing
August 2020

Concomitance of Familial Mediterranean Fever and Gitelman syndrome in an adolescent.

Turk J Pediatr 2019 ;61(3):444-448

Departments of Pediatric Nephrology, Çukurova University Faculty of Medicine, Adana, Turkey.

Atmış B, Kışla-Ekinci RM, Melek E, Bişgin A, Yılmaz M, Anarat A, Karabay-Bayazıt A. Concomitance of Familial Mediterranean Fever and Gitelman syndrome in an adolescent. Turk J Pediatr 2019; 61: 444-448. Gitelman syndrome is a renal tubular salt-wasting disorder characterized by hypokalemic metabolic alkalosis with hypomagnesemia and hypocalciuria. Patients occasionally have symptoms in childhood, while diagnosis is often in adulthood. It is inherited by an autosomal recessive manner through SLC12A3 gene mutations. Familial Mediterranean Fever (FMF) is the most common autoinflammatory disorder, inherited by an autosomal recessive manner and characterized by recurrent fever and pleuritis, peritonitis, and synovitis. Mutations in MEditerrenean FeVer (MEFV) gene, coding pyrin protein are responsible for FMF. Both MEFV and SCL12A3 genes were located on chromosome 16. A 9-year-old boy was admitted to our department because of recurrent abdominal pain, fever, joint pain and swelling since he was three years old. He was diagnosed as FMF and MEFV gene sequencing revealed homozygous M694V (c.2080A > G) mutation. At the age of 14 years, polyuria, polydipsia, hypokalemia and mild hypomagnesemia had occurred. Patient was successfully treated with oral supplementation of potassium and magnesium along with colchicine. Molecular genetic analysis including SCL12A3 gene sequencing revealed homozygote IVS4-16G > A (c.602-16G > A) intronic splicing site mutation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2019.03.021DOI Listing
July 2020

Proptosis in a child with chronic kidney disease: Questions.

Pediatr Nephrol 2020 05 11;35(5):787-788. Epub 2019 Dec 11.

Department of Pediatric Nephrology, Cukurova University Faculty of Medicine, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04422-4DOI Listing
May 2020

Proptosis in a child with chronic kidney disease: Answers.

Pediatr Nephrol 2020 05 10;35(5):789-791. Epub 2019 Dec 10.

Department of Pediatric Nephrology, Cukurova University Faculty of Medicine, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04423-3DOI Listing
May 2020

Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease.

Indian J Crit Care Med 2019 Oct;23(10):489-490

Department of Pediatrics, Division of Pediatric Nephrology, Cukurova University, Faculty of Medicine, Adana, Turkey.

Background: Drugs are very important in the etiology of nontraumatic rhabdomyolysis.

Case Descriptions: A 16-year-old male patient with Wilson's disease was admitted for myoclonic contractions. Oral trientine was started for neurological problems and tremor on the hands due to D-penicillamine 1 month ago. Patient was oligoanuric, and his creatine kinase level was 15197 U/L. Rhabdomyolysis was associated with trientine, and trientine treatment was stopped. Hemodiafiltration was performed. The patient began to urinate on the 24th day.

Conclusion: This is the first pediatric patient with rhabdomyolysis induced by trientine. Drugs used should be questioned carefully in patients with rhabdomyolysis.

How To Cite This Article: Aslan N, Yavuz S, Yildizdas D, Horoz OO, Coban Y, Tumgor G, et al. Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease. Indian J Crit Care Med 2019;23(10):489-490.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5005/jp-journals-10071-23271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6842836PMC
October 2019

Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience.

Mod Rheumatol 2020 Nov 14;30(6):1039-1046. Epub 2019 Nov 14.

Faculty of Medicine, Department of Pediatric Rheumatology, Cukurova University, Adana, Turkey.

Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis ( = 244, 58.1%), abdominal pain ( = 235, 56%), subcutaneous edema ( = 163, 38.8%), and renal involvement ( = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/14397595.2019.1687074DOI Listing
November 2020

Isolated nocturnal and isolated daytime hypertension associate with altered cardiovascular morphology and function in children with chronic kidney disease: findings from the Cardiovascular Comorbidity in Children with Chronic Kidney Disease study.

J Hypertens 2019 11;37(11):2247-2255

Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Heidelberg, Germany.

Introduction: Prevalence of isolated nocturnal hypertension (INH) and isolated daytime hypertension (IDH) is around 10% in adults. Data in children, especially in chronic kidney disease (CKD), are lacking. The aim of this cross-sectional multicenter cohort study was to define the prevalence of INH and IDH and its association with cardiovascular morphology and function, that is, pulse wave velocity (PWV), carotid intima-media thickness (cIMT), or left ventricular mass index (LVMI) in children with CKD.

Methods: Ambulatory blood pressure (BP) monitoring profiles were analyzed in 456 children with CKD stages III-V participating in the Cardiovascular Comorbidity in Children with Chronic Kidney Disease Study (64.3% males, 71.3% congenital anomaly of the kidney and urinary tract, age 12.5 ± 3.2 years, estimated glomerular filtration rate 29 ± 12 ml/min per 1.73 m). Baseline PWV, cIMT, and LVMI were compared in normotension, INH, IDH, or sustained 24-h hypertension.

Results: Prevalence of sustained hypertension was 18.4%, of INH 13.4%, and of IDH 3.7%. PWV SDS (SD score) and cIMT SDS were significantly higher in sustained hypertension and INH, and PWV SDS was significantly higher in IDH, compared with normotension. LVMI was significantly increased in sustained hypertension, but not in INH or IDH. Determinants of INH were smallness for gestational age, older age, higher height SDS and parathyroid hormone, and shorter duration of CKD. In logistic regression analysis, day/night-time hypertension or ambulatory BP monitoring pattern (normal, INH, IDH, sustained hypertension) were independently associated with cardiovascular outcome measures: elevated night-time BP was associated with increased cIMT, PWV, and left ventricular hypertrophy; INH was associated with cIMT.

Conclusion: INH is present in almost one out of seven children with predialysis CKD; INH and nocturnal hypertension in general are associated with alterations of arterial morphology and function.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/HJH.0000000000002160DOI Listing
November 2019

An infant with hyponatremia, hyperkalemia, and metabolic acidosis associated with urinary tract infection: Answers.

Pediatr Nephrol 2019 10 3;34(10):1739-1741. Epub 2019 May 3.

Faculty of Medicine, Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04254-2DOI Listing
October 2019

An infant with hyponatremia, hyperkalemia, and metabolic acidosis associated with urinary tract infection: Questions.

Pediatr Nephrol 2019 10 3;34(10):1737. Epub 2019 May 3.

Faculty of Medicine, Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-019-04252-4DOI Listing
October 2019

Vascular Access Choice, Complications, and Outcomes in Children on Maintenance Hemodialysis: Findings From the International Pediatric Hemodialysis Network (IPHN) Registry.

Am J Kidney Dis 2019 08 19;74(2):193-202. Epub 2019 Apr 19.

Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany. Electronic address:

Rationale & Objective: Arteriovenous fistulas (AVFs) have been recommended as the preferred vascular access for pediatric patients on maintenance hemodialysis (HD), but data comparing AVFs with other access types are scant. We studied vascular access choice, placement, complications, and outcomes in children.

Study Design: Prospective observational cohort study.

Setting & Participants: 552 children and adolescents from 27 countries on maintenance HD followed up prospectively by the International Pediatric HD Network (IPHN) Registry between 2012 and 2017.

Predictor: Type of vascular access: AVF, central venous catheter (CVC), or arteriovenous graft.

Outcome: Infectious and noninfectious vascular access complication rates, dialysis performance, biochemical and hematologic parameters, and clinical outcomes.

Analytical Approach: Univariate and multivariable linear mixed models, generalized linear mixed models, and proportional hazards models; cumulative incidence functions.

Results: During 314 cumulative patient-years, 628 CVCs, 225 AVFs, and 17 arteriovenous grafts were placed. One-third of the children with an AVF required a temporary CVC until fistula maturation. Vascular access choice was associated with age and expectations for early transplantation. There was a 3-fold higher living related transplantation rate and lower median time to transplantation of 14 (IQR, 6-23) versus 20 (IQR, 14-36) months with CVCs compared with AVFs. Higher blood flow rates and Kt/V were achieved with AVFs than with CVCs. Infectious complications were reported only with CVCs (1.3/1,000 catheter-days) and required vascular access replacement in 47%. CVC dysfunction rates were 2.5/1,000 catheter-days compared to 1.2/1,000 fistula-days. CVCs required 82% more revisions and almost 3-fold more vascular access replacements to a different site than AVFs (P<0.001).

Limitations: Clinical rather than population-based data.

Conclusions: CVCs are the predominant vascular access choice in children receiving HD within the IPHN. Age-related anatomical limitations and expected early living related transplantation were associated with CVC use. CVCs were associated with poorer dialysis efficacy, higher complication rates, and more frequent need for vascular access replacement. Such findings call for a re-evaluation of pediatric CVC use and practices.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.ajkd.2019.02.014DOI Listing
August 2019

Acute dialysis in children: results of a European survey.

J Nephrol 2019 Jun 4;32(3):445-451. Epub 2019 Apr 4.

Nephrology and Dialysis Unit, Pediatric Subspecialties Department, Institute for Scientific Research, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165, Rome, Italy.

The number of children with acute kidney injury (AKI) requiring dialysis is increasing. To date, systematic analysis has been largely limited to critically ill children treated with continuous renal replacement therapy (CRRT). We conducted a survey among 35 European Pediatric Nephrology Centers to investigate dialysis practices in European children with AKI. Altogether, the centers perform dialysis in more than 900 pediatric patients with AKI per year. PD and CRRT are the most frequently used dialysis modalities, accounting for 39.4% and 38.2% of treatments, followed by intermittent HD (22.4%). In units treating more than 25 cases per year and in those with cardiothoracic surgery programs, PD is the most commonly chosen dialysis modality. Also, nearly one quarter of centers, in countries with a gross domestic product below $35,000/year, do not utilize CRRT at all. Dialysis nurses are exclusively in charge of CRRT management in 45% of the cases and pediatric intensive care nurses in 25%, while shared management is practiced in 30%. In conclusion, this survey indicates that the choice of treatment modalities for dialysis in children with AKI in Europe is affected by the underlying ethiology of the disease, organization/set-up of centers and socioeconomic conditions. PD is utilized as often as CRRT, and also intermittent HD is a commonly applied treatment option. A prospective European AKI registry is planned to provide further insights on the epidemiology, management and outcomes of dialysis in pediatric AKI.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s40620-019-00606-1DOI Listing
June 2019

Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey.

Clin Rheumatol 2019 May 16;38(5):1459-1468. Epub 2019 Jan 16.

Department of Pediatric Rheumatology, Faculty of Medicine, Cukurova University, Adana, Turkey.

Objectives: This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey.

Methods: Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2005 and June 2018 were included in the present study.

Results: The median age at the diagnosis was 12.8 (range, 5.1-17.7) years. The female to male ratio was 9.6:1. The most prevalent clinical features were mucocutaneous involvement (96.2%) and constitutional manifestations (94.3%). Renal manifestations, hematological manifestations, and neuropsychiatric involvement were detected in 40 (75%), in 38 (71.7%), and in 13 (24.5%) patients, respectively. Renal biopsy was performed to 49 patients (92.5%). Class IV lupus nephritis (LN) (34%) and class II LN (20.4%) were the most common findings. Mycophenolate mofetil, cyclophosphamide with corticosteroid were the main treatment options. Eighteen patients received rituximab and one tocilizumab. The mean SLE Disease Activity Index (SLEDAI) score at the time of diagnosis was 22.47 ± 8.8 (range = 3-49), and 1.34 ± 1.85 (range = 0-7) at last visit. Twenty-one patients (39.6%) had damage in agreement with Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (PedSDI; mean = 0.60 ± 0.94; range = 0-5) criteria. Growth failure was the most prevalent cause of damage (n = 13, 26%). One patient deceased due to severe pulmonary hemorrhage and multiple cerebral thromboses.

Conclusion: jSLE patients in this cohort have severe disease in view of the higher frequency of renal and neurologic involvement. Nevertheless, multicenter studies are needed to make a conclusion for all Turkish children with jSLE.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10067-019-04433-4DOI Listing
May 2019

Time-averaged hemoglobin values, not hemoglobin cycling, have an impact on outcomes in pediatric dialysis patients.

Pediatr Nephrol 2018 11 13;33(11):2143-2150. Epub 2018 Aug 13.

Department of Pediatric Nephrology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.

Background: During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients.

Methods: Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed. More than 1 g/dL decrease or increase in Hb level was considered as HC. Patients were divided into two groups according to 12-month Hb trajectory as rare cycling (RC) (≤ 3) and frequent cycling (FC) (> 3 fluctuation) as well as three groups based on T-A-Hb levels: < 10, 10-11, and > 11 g/dL.

Results: Two hundred forty-five dialysis (160 peritoneal dialysis (PD) and 85 hemodialysis (HD)) patients aged 12.3 ± 5.1 (range 0.5-21) years were enrolled in this study. Fifty-two percent of the patients had RC, 45% had FC, and only 3% had no cycling. There were no differences between HC groups with respect to age, dialysis modality, having anemia, hospitalization rate, residual urine volume, and mortality. Although left ventricular mass index (LVMI) tended to be higher in RC than FC group (65 ± 37 vs 52 ± 23 g/m, p = 0.056), prevalence of LVH was not different between the groups (p = 0.920). In regression analysis, FC was not a risk factor for LVH, but low T-A Hb level (< 10 g/dL) was a significant risk for LVH (OR = 0.414, 95% CI 0.177-0.966, p = 0.04). The target Hb levels were more often achieved in PD patients, and the number of deaths was significantly lower in non-anemic patients (Hb level > 11 g/dL).

Conclusion: Hb cycling is common among dialysis patients. Severity of anemia rather than its cycling has more significant impact on the prevalence of LVH and on inflammatory state.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-018-4013-4DOI Listing
November 2018

Peritoneal dialysis in neonates: six years of single center experience

Turk J Med Sci 2018 Apr 30;48(2):231-236. Epub 2018 Apr 30.

Background/aim: Peritoneal dialysis (PD) is generally considered the practical dialysis modality for neonates in the treatment of acute kidney injury (AKI) and metabolic disturbances. The aim of this study was to evaluate the indications, complications, and outcomes of PD between January 2011 and December 2016. Materials and methods: This study included all 56 neonates that underwent PD over six years in our neonatal intensive care unit (NICU). A retrospective chart review was performed for all patients in our institution. Results: The incidence of PD was 1.32% (56/4230 patients) during this period. Mean birth weight of the patients was 2267.77 ± 1060.63 (540–5050) g. Thirty-four (60.7%) patients were premature. Fourteen patients (25%) were in the postoperative period of cardiac surgery due to congenital cardiac defects, fourteen patients (25%) were diagnosed with metabolic diseases, one patient had hypoxic ischemic encephalopathy, and another patient had severe pulmonary hypertension. Conclusion: In the acute setting of a neonatal intensive care unit, PD performed in a neonate provides a technically simple method of steady fluid, solute removal, and correction of electrolyte abnormalities. A trocar catheter appears safe even in premature babies, and closed three-way stopcock system seems to be reliable at the bedside in NICUs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3906/sag-1705-175DOI Listing
April 2018

Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child.

J Pediatr Hematol Oncol 2018 07;40(5):401-404

Departments of Pediatric Nephrology.

Background: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult.

Case: We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed. The patient was treated with eculizumab, agressive blood pressure control, and antiepileptic drugs. At the fifth month of follow-up, the patient was diagnosed with acute B-cell lymphoblastic leukemia with fever, bone pain, hepatosplenomegaly, and pancytopenia. After initiation of ALL treatment, he had no episodes of HUS, despite cessation of eculizumab.

Conclusion: In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPH.0000000000001121DOI Listing
July 2018

Effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism in children with chronic kidney disease.

Nephrol Dial Transplant 2018 12;33(12):2208-2217

Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany.

Background: We investigated the effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism, i.e. serum levels of fibroblast growth factor 23 (FGF23), Klotho, bone alkaline phosphatase (BAP) and sclerostin, in two cohorts with chronic kidney disease (CKD).

Methods: In all, 80 vitamin D-deficient children were selected: 40 with mild to moderate CKD from the ERGO study, a randomized trial of ergocalciferol supplementation [estimated glomerular filtration rate (eGFR) 55 mL/min/1.73 m2], and 40 with advanced CKD from the observational Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study (eGFR 24 mL/min/1.73 m2). In each study, vitamin D supplementation was started in 20 children and 20 matched children not receiving vitamin D served as controls. Measures were taken at baseline and after a median period of 8 months. Age- and gender-related standard deviation scores (SDSs) were calculated.

Results: Before vitamin D supplementation, children in the ERGO study had normal FGF23 (median 0.31 SDS) and BAP (-0.10 SDS) but decreased Klotho and sclerostin (-0.77 and -1.04 SDS, respectively), whereas 4C patients had increased FGF23 (3.87 SDS), BAP (0.78 SDS) and sclerostin (0.76 SDS) but normal Klotho (-0.27 SDS) levels. Vitamin D supplementation further increased FGF23 in 4C but not in ERGO patients. Serum Klotho and sclerostin normalized with vitamin D supplementation in ERGO but remained unchanged in 4C patients. BAP levels were unchanged in all patients. In the total cohort, significant effects of vitamin D supplementation were noted for Klotho at eGFR 40-70 mL/min/1.73 m2.

Conclusions: Vitamin D supplementation normalized Klotho and sclerostin in children with mild to moderate CKD but further increased FGF23 in advanced CKD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/ndt/gfy012DOI Listing
December 2018

Effect of the timing of dialysis initiation on left ventricular hypertrophy and ınflammation in pediatric patients.

Pediatr Nephrol 2017 09 10;32(9):1595-1602. Epub 2017 Apr 10.

Department of Pediatric Nephrology, Tepecik Training and Research Hospital, İzmir, Turkey.

Background: The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis.

Methods: The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed. In addition to demographic data, we retrieved anthropometric measurements, data on dialysis treatment modalities, routine biochemical parameters, complete blood count, serum ferritin, parathormone, C-reactive protein (CRP), and albumin levels, as well as echocardiographic data and hospitalization records. The patients were divided into two groups based on their estimated glomerular filtration rate (eGFR) levels at dialysis initiation, namely, an early-start group, characterized by an eGFR of >10 ml/min/1.73 m, and a late-start group, with an eGFR of < 7 ml/min/1.73 m. The collected data were compared between these groups.

Results: A total of 245 pediatric dialysis patients (mean age ± standard deviation 12.3 ± 5.1 years, range 0.5-21 years) were enrolled in this study. Echocardiographic data were available for 137 patients, and the mean LV mass index (LVMI) was 58 ± 31 (range 21-215) g/m. The LVMI was 75 ± 30 g/m(n = 81) and 34 ± 6 g/m(n = 56) in patients with or without LV hypertrophy (LVH) (p < 0.001). Early-start (eGFR >10 ml/min/1.73 m) versus late-start dialysis (eGFR < 7 ml/min/1.73 m) groups did not significantly differ in LVMI and LVH status (p > 0.05) nor in number of hospitalizations. Serum albumin levels were significantly higher in the early-dialysis group compared with the late-dialysis group (3.3 ± 0.7 vs. 3.1 ± 0.7 g/dl, respectively; p < 0.05). The early-start group had relatively higher time-averaged albumin levels (3.2 ± 0.5 vs. 3.1 ± 0.5 g/dl; p = > 0.05) and relatively lower CRP levels (3.64 ± 2.00 vs. 4.37 ± 3.28 mg/L, p > 0.05) than the late-start group, but these differences did not reach statistical significance.

Conclusion: Although early dialysis initiation did not have a significant effect on important clinical outcome parameters, including LVH, inflammatory state, and hospitalization, in our pediatric dialysis patients, this area of study deserves further attention.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3660-1DOI Listing
September 2017

Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment.

Pediatr Nephrol 2017 Aug 24;32(8):1369-1375. Epub 2017 Mar 24.

Department of Pediatric Nephrology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey.

Background: ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration.

Methods: A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation.

Results: Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04-19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4-39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117-155) ml/min/1.73m, proteinuria was 1,008 (IQR 281-1,567) mg/m/day. After a median follow-up of 11.5 (range 4-21) months following CoQ10 administration, proteinuria was significantly decreased (median 363 [IQR 175-561] mg/m/day, P=0.025), whereas eGFR was preserved (median 137 [IQR 113-158] ml/min/1.73m, P=0.61).

Conclusions: ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-017-3634-3DOI Listing
August 2017

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

J Pediatr Endocrinol Metab 2017 Feb;30(2):237-239

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1515/jpem-2016-0324DOI Listing
February 2017

Impaired glucose tolerance in Fanconi-Bickel syndrome: Eight patients with two novel mutations.

Turk J Pediatr 2017 ;59(4):434-441

Departments Pediatric Metabolism, Balcali Hospital, Cukurova University, Adana.

Şeker-Yılmaz B, Kör D, Bulut FD, Yüksel B, Karabay-Bayazıt A, Topaloğlu AK, Ceylaner G, Önenli-Mungan N. Impaired glucose tolerance in Fanconi-Bickel syndrome: Eight patients with two novel mutations. Turk J Pediatr 2017; 59: 434-441. Fanconi-Bickel syndrome (FBS) is a rare, autosomal recessive disorder of carbohydrate metabolism caused by defects in the facilitative glucose transporter 2 (GLUT2 or SLC2A2) gene. Prominent findings are failure to thrive, renal tubular acidosis, hypoglycemia and postprandial hyperglycemia even mimicking diabetes mellitus. Eight patients from 6 families with FBS were included in this study. c.482_483insC homozygous mutation was detected in six patients from four different families. Mutation analysis of SLC2A2 gene revealed two novel homozygous mutations; c.1069delGinsAATAA and c.575A > G. Standard oral glucose tolerance test with 1.75 g/kg oral glucose was performed in six of the patients who were older than 3-years of age. Impaired glucose tolerance was found in all patients as expected and two of them had overt diabetes. None of the antidiabetic medications were given to them in order to avoid significant hypoglycemia. Beside the conservative treatment, follow up with frequent oral glucose tolerance tests are planned. We report these cases of FBS, as GSD XI is rare, two novel mutations were detected and also to highlight the risk of diabetes mellitus; although there is not a consensus about the treatment.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2017.04.010DOI Listing
December 2018

Genotypic and phenotypic features of the cystinosis patients from the South Eastern part of Turkey.

Turk J Pediatr 2016 ;58(4):362-370

Divisions of Pediatric Nutrition and Metabolism, Department of Pediatrics, Çukurova University Faculty of Medicine, Adana.

We have conducted this study for the purposes of demonstrating the spectrum of mutations and of identifying their effects on the phenotype, with a particular focus on the clinical course, prognosis and response to treatment. A total of 25 patients from 20 families, who have been treated and followed up after being diagnosed with cystinosis. Nine patients were identified with mutations of homozygous c.451A > G, 7 patients with homozygous c.681G > A, 6 patients with homozygous c.834_842del, 2 patients with homozygous c.18_21delGACT and 1 patient with compound heterozygous for c.451A > G/ c.1015G > A. The c.834_842del mutation identified in six patients from four families has not been previously identified. Progression to renal failure occurred earlier in the patients identified with the new mutation, despite treatment. Larger patient series are required to demonstrate the genotypic properties of the patients with cystinosis and their relationship with the clinical course.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2016.04.003DOI Listing
July 2017

Crescentic glomerulonephritis in a child with Heiner syndrome.

Turk J Pediatr 2014 Nov-Dec;56(6):661-4

Division of Pediatric Nephrology, Çukurova University Faculty of Medicine, Adana, Turkey.

Heiner syndrome is a food-induced pulmonary hypersensitivity disease that predominantly affects infants. Chronic respiratory symptoms with pulmonary infiltrates on radiography, positive milk precipitins and resolution of findings upon removal of cow's milk constitute the main features. Severe cases may present with pulmonary hemosiderosis. Few renal manifestations associated with this syndrome have been reported so far. Here we report the first case of Heiner syndrome complicated by crescentic glomerulonephritis after 5 years of follow-up.
View Article and Find Full Text PDF

Download full-text PDF

Source
March 2016

A rare manifestation of renal osteodystrophy in a non-compliant child on hemodialysis: Answers.

Pediatr Nephrol 2016 09 13;31(9):1451-3. Epub 2015 May 13.

Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-015-3124-4DOI Listing
September 2016

A rare manifestation of renal osteodystrophy in a non-compliant hemodialysis child: Questions.

Pediatr Nephrol 2016 09 13;31(9):1449-50. Epub 2015 May 13.

Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00467-015-3121-7DOI Listing
September 2016

Medullary nephrocalcinosis in Schimke immuno-osseous dysplasia.

Pediatr Int 2015 Apr;57(2):310-3

Division of Pediatric Nephrology, Cukurova University Hospital, Adana, Turkey.

Schimke immuno-osseous dysplasia (SIOD) is a rare hereditary disease characterized by skeletal dysplasia, immune deficiency and progressive renal disease. Kidney involvement mainly determines the prognosis. The most common renal pathology is focal segmental glomerulosclerosis (FSGS). Medullary nephrocalcinosis refers to the diffuse deposition of calcium salts in renal medulla and has not previously been identified in SIOD. Here we report the first case of a pediatric patient having typical features of SIOD with medullary nephrocalcinosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ped.12455DOI Listing
April 2015

Membranous nephropathy presenting with nephrotic syndrome in a child with thalassemia major.

Pediatr Int 2015 Aug 24;57(4):711-3. Epub 2015 Feb 24.

Histology and Embryology, Cukurova University School of Medicine, Adana, Turkey.

Few data on the renal effects of thalassemia syndrome are available in the literature. Recent clinical studies identified proximal tubular damage and glomerular filtration abnormalities in thalassemia. Iron-chelating agents might be nephrotoxic as well, but proven glomerular injury, either due to anemia or chelating therapy, has not previously been demonstrated in thalassemia patients. Here, we report the first thalassemia patient presenting with nephrotic syndrome to be diagnosed with membranous nephropathy in the literature.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ped.12572DOI Listing
August 2015

Markers of bone metabolism are affected by renal function and growth hormone therapy in children with chronic kidney disease.

PLoS One 2015 6;10(2):e0113482. Epub 2015 Feb 6.

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

Objectives: The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort.

Methods: Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group.

Results: Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score.

Conclusion: Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0113482PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4319910PMC
January 2016