Publications by authors named "Engin Melek"

33 Publications

Influenza and pneumococcus vaccination rates in pediatric dialysis patients in Europe: Recommendations vs reality A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group Study.

Turk J Med Sci 2021 02 4. Epub 2021 Feb 4.

Background: Children on dialysis are under increased risk of influenza and invasive pneumococcal disease. Although, vaccination against these microorganisms are recommended in dialysis patients and despite the fact that these vaccines can reduce disease burden and rates of hospitalization due to infection, vaccination rates are below expected and desired. We aimed to evaluate influenza and pneumococcal vaccination and infection rates in European pediatric dialysis centers.

Methods: In 16 centers from 11 countries, 357 pediatric dialysis patients were evaluated retrospectively during one year of observation period between 01.01.2014 and 01.01.2015.

Results: In all centers, vaccination policy included immunization of dialysis patients with inactive influenza vaccine and pneumococcal conjugate vaccine (PCV). 50% of centers recommended pneumococcal polysaccharide vaccine following routine PCV series. Significantly higher pneumococcal vaccination rate (43.9 %) was seen in PD patients compared to those on HD (32.9 %) (p=0.035), while the rates for influenza were similar (42.4 % and 46.1 respectively, p=0.496). Among all dialysis patients, 2,2 % (n=8) developed pneumonia and 6.4 % (n=23) infected by influenza. Pneumococcic pneumonia rate was 5 % for 140 patients who received anti-pneumococcal vaccine, while only one pneumonia episode was recorded out of 217 unvaccinated patients (p=0.007). The influenza virus infection rates were similar for patients vaccinated and non-vaccinated (7 % and 6 %, respectively).

Conclusions: Although influenza and pneumococcal vaccines are highly recommended in pediatric dialysis patients, vaccination rates were lower than expected. Pneumococcal vaccination rates were higher in PD compared to the patients on HD. The rate of children with influenza infection was higher than pneumonia. The efficacy of influenza and pneumococcal vaccines was highlighted by the low infection rates. Higher pneumonia rates in patients vaccinated against pneumococcus compared to unvaccinated ones might be due to coexisting risk factors.
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http://dx.doi.org/10.3906/sag-2012-26DOI Listing
February 2021

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

Predictors of poor kidney outcome in children with C3 glomerulopathy.

Pediatr Nephrol 2021 May 31;36(5):1195-1205. Epub 2020 Oct 31.

Department of Pediatric Nephrology, Erciyes University, Faculty of Medicine, Kayseri, Turkey.

Background: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G.

Methods: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model.

Results: Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development.

Conclusions: Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract.
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http://dx.doi.org/10.1007/s00467-020-04799-7DOI Listing
May 2021

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

Evaluation of phenotypic and genotypic features of children with distal kidney tubular acidosis.

Pediatr Nephrol 2020 12 1;35(12):2297-2306. Epub 2020 Jul 1.

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

Background: The present study aimed to assess genotype-phenotype correlations with long-term prognosis in children with distal kidney tubular acidosis (dKTA). The kidney function of children with dKTA could be impaired in the long-term.

Methods: Thirty-one children with dKTA from 23 families were included in the present study. Demographic features, growth parameters, clinical manifestations, follow-up results, and genetic analysis results of the patients were recorded.

Results: Eighteen children (58.1%) were male. The median age at diagnosis was 3 months. The median follow-up period was 77 months and the longest was 23.5 years. Eight (28.8%) patients had chronic kidney disease (CKD) stage 2 or 3. Three patients aged 24, 23, and 19 years had CKD stage 3 with an estimated glomerular filtration rate of 54, 57, and 48 mL/min/1.73 m, respectively. Thirteen patients had mutations in the ATP6V0A4 gene, eight had mutations in the ATP6V1B1 gene, and three had mutations in the SLC4A1 gene. There was no significant correlation between molecular diagnosis and CKD. Growth retardation with a height below a standard deviation (SD) score of - 2 was found in 14 patients (45.1%) at the time of diagnosis. The mean height SD score at the last visit was significantly higher in patients who had adequate metabolic control at > 75% of all visits as compared with that in patients who did not.

Conclusion: Patients with dKTA usually have a good clinical prognosis in childhood with appropriate treatment; however, dRTA is characterized by deterioration of kidney function in adulthood, particularly after puberty.
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http://dx.doi.org/10.1007/s00467-020-04685-2DOI Listing
December 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.
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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.
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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.

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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.

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

Metformin Intoxication with Severe Renal Damage: An Adolescent Case Report.

J Pediatr Intensive Care 2019 Dec 28;8(4):255-258. Epub 2019 Aug 28.

Division of Pediatric Intensive Care Unit, Faculty of Medicine, Çukurova University, Adana, Turkey.

Metformin is an oral antidiabetic medication which belongs to the class biguanide. Its most hazardous and life-threatening side effect is lactic acidosis which has been increasingly reported in recent years. Early diagnosis and aggressive treatment approaches have significantly reduced morbidity and mortality rates in metformin intoxication. In this case report, we emphasize the importance of early continuous renal replacement therapy in metformin-associated lactic acidosis in a 16-year-old patient who developed severe renal damage during the follow-up period of renal replacement therapy due to lactic acidosis after taking metformin in a suicide attempt.
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http://dx.doi.org/10.1055/s-0039-1695046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821537PMC
December 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.
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http://dx.doi.org/10.1080/14397595.2019.1687074DOI Listing
November 2020

Phenotypic variability in two patients with tumor necrosis factor receptor associated periodic fever syndrome emphasizes a rare manifestation: Immunoglobulin A nephropathy.

Eur J Med Genet 2020 Apr 3;63(4):103780. Epub 2019 Oct 3.

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

Tumor necrosis factor receptor associated periodic fever syndrome (TRAPS) is caused by heterozygote mutations in TNFRSF1A, characterized by recurrent inflammatory attacks. In this report, we described two patients with different heterozygote mutations in TNFRSF1A. Patient 1, a 15-year-old male, had suffered from recurrent fever attacks accompanied by abdominal pain, eye manifestations, and myalgia with increased acute phase reactants since the age of 6-month. He had been unsuccessfully treated with colchicine for having familial Mediterranean fever without an identifiable MEFV mutation since the age of 4-year. At the age of 15 years, he was diagnosed with immunoglobulin (Ig) A nephropathy due to massive proteinuria and renal biopsy findings. Next generation sequencing revealed NM_001065.3: c.236C>T; p. (Thr79Met); T50M heterozygote mutation in TNFRFS1A. He was treated with methylprednisolone and cyclosporine for IgA nephropathy, thereafter with canakinumab for TRAPS. Patient 2, a 17-year-old female, had recurrent arthritis attacks accompanied by increased acute phase reactants for the last two months. She had neither fever attacks nor rashes or myalgia. Her physical examination was normal between attacks. Magnetic resonance imaging of both knees and ankles showed no signs of chronic arthritis. MEFV analyzes showed no mutation. Next generation sequencing revealed NM_001065.3: c.362G>A; p.(Arg121Gln); R92Q heterozygote mutation in TNFRFS1A. Arthritis attacks were treated successfully with ibuprofen thereafter. In conclusion, we wish to emphasize the diversity of the clinical manifestations between these two patients with distinct sequence variants in TNFRSF1A. Moreover, we presented a rare manifestation of TRAPS, IgA nephropathy.
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http://dx.doi.org/10.1016/j.ejmg.2019.103780DOI Listing
April 2020

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.

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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.

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

Treatment and long-term outcome in primary distal renal tubular acidosis.

Nephrol Dial Transplant 2019 06;34(6):981-991

Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.

Background: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome.

Methods: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form.

Results: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate.

Conclusion: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.
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http://dx.doi.org/10.1093/ndt/gfy409DOI 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.
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http://dx.doi.org/10.1007/s10067-019-04433-4DOI Listing
May 2019

Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis.

Pediatr Nephrol 2019 05 4;34(5):847-854. Epub 2019 Jan 4.

Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).

Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically.

Results: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007).

Conclusions: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis.
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http://dx.doi.org/10.1007/s00467-018-4162-5DOI Listing
May 2019

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

J Pediatr 2018 08 9;199:22-28.e6. Epub 2018 May 9.

Department of Pediatrics, University Hospital of Cologne, Cologne, Germany; Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany.

Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis.

Study Design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life.

Results: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys.

Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.
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http://dx.doi.org/10.1016/j.jpeds.2018.03.052DOI Listing
August 2018

Extra-Renal manifestations of atypical hemolytic uremic syndrome in children.

Pediatr Nephrol 2018 08 2;33(8):1395-1403. Epub 2018 Apr 2.

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

Background: Atypical 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.

Methods: This 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.

Results: The 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.

Conclusions: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.
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http://dx.doi.org/10.1007/s00467-018-3933-3DOI Listing
August 2018

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

Nephron 2018 12;138(4):280-286. Epub 2017 Dec 12.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

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.
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http://dx.doi.org/10.1159/000485398DOI Listing
September 2019

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

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

Pediatr Transplant 2017 Mar 29;21(2). Epub 2016 Nov 29.

Division of General Surgery, Baskent University, Ankara, Turkey.

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.
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http://dx.doi.org/10.1111/petr.12860DOI Listing
March 2017

Timing for Removal of Peritoneal Dialysis Catheters in Pediatric Renal Transplant Patients.

Exp Clin Transplant 2016 11;14(Suppl 3):74-77

From the Department of Pediatric Nephrology, Baskent University, Ankara, Turkey; and the Department of Pediatric Nephrology, Cukurova University, Adana, Turkey.

Objectives: Peritoneal dialysis, the preferred long-term renal replacement modality in the pediatric population, can also be used during the post transplant period. Although it is well known that peritonitis or other complications may occur related to the peritoneal dialysis catheter, less is known about complications related to the peritoneal dialysis during the posttransplant period. Our objective was to evaluate the complications related to use of a peritoneal dialysis catheter during the posttransplant period and to determine the optimum time for removal of the peritoneal dialysis catheter.

Materials And Methods: We retrospectively analyzed 33 chronic peritoneal dialysis patients. Pretransplant and posttransplant demographics and clinical and laboratory data for each patient were recorded, including incidence of peritonitis and incidence of peritoneal dialysis catheter requirement after transplant.

Results: Mean age of patients at transplant was 12.8 ± 4.0 years (range, 3.5-18.0 y). Mean catheter removal time was 81.1 ± 36.2 days (range, 22.0-152.0 d). The peritoneal dialysis catheter was used in 6 of 33 patients (18.2%); none of these patients developed peritonitis. In contrast, 2 of the 27 patients who did not use the peritoneal dialysis catheter developed peritonitis. Our data suggest that the need for catheter use occurs predominantly during the first month, and infectious complications usually happen later.

Conclusions: Previously, the trend was to not remove the peritoneal dialysis catheter at the time of transplant. However, in light of recent literature and our present study, we recommend that the time of catheter removal should be modified and decided for each patient on an individual basis.
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November 2016

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

Exp Clin Transplant 2016 05 2. Epub 2016 May 2.

From the Department of Pediatric Nephrology, Baskent University School of Medicine, Ankara, Turkey.

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.
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http://dx.doi.org/10.6002/ect.2016.0120DOI Listing
May 2016

Labial adhesion and urinary tract problems: The importance of genital examination.

J Pediatr Urol 2016 Apr 23;12(2):111.e1-5. Epub 2015 Oct 23.

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

Background: Urinary tract infection (UTI) is a common bacterial illness in children. Delay in the treatment of UTI may lead to acute renal parenchymal damage and subsequent renal scarring. It is well established that several risk factors increase the tendency for UTI - one being labial adhesion (LA).

Objective: The purpose of this study was to emphasize the importance of genital examination in girls with nephrourologic symptoms, particularly UTIs, in order to detect LA.

Material And Methods: Data were collected from the files of 46 girls with LA, including: the girl's age, thickness of LA, any recurrence and treatment options of LA, and the reason for admission to hospital. The LAs were grouped in terms of thickness as thin, moderate and dense, and also partial or complete.

Results: The average age of the girls at the first visit was 51.9 ± 37.57 months (min-max: 3.5-157 months). Twenty-seven (58.7%) of the girls had history of recurrent UTI. There was a marked association between the presence of UTI and the type of adhesions. The percentages of UTIs in girls with complete and partial LA were 84.0% and 28.6%, respectively (P < 0.05). The percentages of UTIs in girls with thick and thin LA were 100% and 44.1%, respectively (P < 0.05). None of the girls' primary care physicians or pediatricians recognized LA at the time of a periodic health examination.

Discussion: In the present study, girls with complete and thick LA had a greater tendency towards having UTIs than those with partial and thin LA, respectively. After treatment of LA, the UTIs did not recur in any girls. None of the girls in this study had undergone a previous genital examination. Therefore, this study suggests that physicians do not often perform genital examinations. Limitations of this study were the small sample size and the short follow-up period. In addition, although all of the girls were examined by the same physician, the thickness of the membrane is subjective and solely depends on the physician's experience.

Conclusions: This study showed that although genital examination is a routine part of a physical examination, it is not always performed. Therefore, it is recommend that genital examination should be performed in girls with nephrourologic complaints, particularly for UTI. By timeously determining the presence of LA, many unnecessary and invasive investigations could be avoided in these children.
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http://dx.doi.org/10.1016/j.jpurol.2015.10.002DOI Listing
April 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.

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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.

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

Parvovirus B19-induced multisystemic vasculitis and acute endocapillary proliferative glomerulonephritis in a child.

Ren Fail 2010 May;32(4):506-9

Department of Pediatric Nephrology, Baskent University, Faculty of Medicine, Ankara, Turkey.

A 10-year-old girl was admitted with fever, cough, maculopapular rash, hemoptysis, dark-colored urine, edema, multiple lymphadenopathies, and hepatosplenomegaly. She developed acute glomerulonephritis during the course of these complex clinical features. Laboratory data showed hematuria, proteinuria, and hypocomplementemia. Serological tests showed positive human parvovirus B19 (HPVB19)-specific immunoglobin M (IgM) and HPVB19 DNA was detected in the patient's serum using polymerase chain reaction (PCR). Renal biopsy revealed acute endocapillary proliferative glomerulonephritis (AEPGN) with coarse granular C3 depositions in a "starry sky pattern," which is more peculiar to poststreptococcal glomerulonephritis. Electron microscopy showed subendothelial and small hump-shaped subepithelial electron-dense deposits in glomerular capillary walls. There was no evidence of either any mycobacterial, staphylococcal, or streptococcal infection, and the diagnosis of Goodpasture syndrome and connective tissue disorders was excluded during clinical and laboratory investigations. A diagnosis of HPVB19-induced pleuropneumonitis and glomerulonephritis was made. Through a literature search there was no documented pediatric case of AEPGN induced by HPVB19, and this case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and AEPGN has been demonstrated in a child.
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http://dx.doi.org/10.3109/08860221003728713DOI Listing
May 2010

Urofacial (ochoa) syndrome: can a facial gestalt represent severe voiding dysfunction?

Ren Fail 2009 ;31(7):589-92

Department of Pediatrics, Başkent University Faculty of Medicine, Ankara, Turkey.

Urofacial (Ochoa) syndrome is a rare autosomal-recessive disorder that features an unusual "inverted" facial expression, such that patients appear to be crying when they smile. This syndrome also involves serious urinary tract disorders, though the diagnosis may be missed because of variability of these problems and failure to recognize the characteristic facial grimacing. The urinary issues usually result in enuresis, urinary tract infection, and hydronephrosis, and some severely affected patients become hypertensive and progress to end-stage renal disease. Early diagnosis is very important for management of urinary problems and best prognosis in these patients. We report the first published case of urofacial syndrome in Turkey. The patient was diagnosed at 16 years of age, after having been followed with the diagnosis of recurrent urinary tract infection and vesico-ureteral reflux. Physicians should keep this syndrome in mind for any patient who presents with dysfunctional voiding, particularly in countries with high rates of consanguineous marriage.
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http://dx.doi.org/10.1080/08860220903003370DOI Listing
January 2010

Acute, reversible nonoliguric renal failure in two children associated with analgesic-antipyretic drugs.

Pediatr Emerg Care 2009 Apr;25(4):263-6

Departments of Pediatrics, and daggerPediatric Nephrology, Baskent University, Ankara, Turkey.

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 2 patients 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.
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http://dx.doi.org/10.1097/PEC.0b013e31819e38d4DOI Listing
April 2009