Publications by authors named "Ibrahim Gokce"

32 Publications

Treatment and long-term outcome in primary nephrogenic diabetes insipidus.

Nephrol Dial Transplant 2020 Dec 26. Epub 2020 Dec 26.

Department of Renal Medicine, University College London, London,UK.

Background: Primary nephrogenic diabetes insipidus (NDI) is a rare disorder and little is known about treatment practices and long-term outcome.

Methods: Paediatric and adult nephrologists contacted through European professional organizations entered data in an online form.

Results: Data were collected on 315 patients (22 countries, male 84%, adults 35%). Mutation testing had been performed in 270 (86%); pathogenic variants were identified in 258 (96%). The median (range) age at diagnosis was 0.6 (0.0-60) years and at last follow-up 14.0 (0.1-70) years. In adults, height was normal with a mean (standard deviation) score of -0.39 (±1.0), yet there was increased prevalence of obesity (body mass index >30 kg/m2; 41% versus 16% European average; P < 0.001). There was also increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (32%) and adults (48%). Evidence of flow uropathy was present in 38%. A higher proportion of children than adults (85% versus 54%; P < 0.001) received medications to reduce urine output. Patients ≥25 years were less likely to have a university degree than the European average (21% versus 35%; P = 0.003) but full-time employment was similar. Mental health problems, predominantly attention-deficit hyperactivity disorder (16%), were reported in 36% of patients.

Conclusion: This large NDI cohort shows an overall favourable outcome with normal adult height and only mild to moderate CKD in most. Yet, while full-time employment was similar to the European average, educational achievement was lower, and more than half had urological and/or mental health problems.
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http://dx.doi.org/10.1093/ndt/gfaa243DOI Listing
December 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

Sclerostin and osteoprotegerin: new markers of chronic kidney disease mediated mineral and bone disease in children.

J Pediatr Endocrinol Metab 2020 Nov;33(11):1383-1390

Marmara University Medical Faculty, Department of Pediatrics, Division of Pediatric Nephrology, Istanbul, Turkey.

Background Sclerostin and osteoprotegerin (OPG) are new markers of chronic kidney disease (CKD) mediated mineral bone disease (CKD-MBD) which were extensively evaluated in adult population. We aimed to evaluate the associations between serum levels of sclerostin/OPG and parameters of bone turnover and compare the serum levels of sclerostin/OPG in different stages of CKD in children. Methods 70 children with CKD stage 1-5, aged 2-21 years were examined. Serum levels of alkaline phosphatase (ALP), creatinine, total calcium, phosphorus , intact parathyroid hormone (iPTH) and vitamin D were measured. Serum sclerostin and OPG levels were measured in children with different levels of CKD stage and their association with bone turnover parameters were noted. Results We did not observe any significant correlation between serum levels of sclerostin and OPG and stages of CKD. A negative relationship was present between serum sclerostin and 25-OH vitamin D levels. Osteoprotegerin was positively and significantly correlated with ALP but serum sclerostin was negatively correlated with ALP. Conclusion Our study, which includes only children and adolescents with a growing skeleton under uremic conditions and excluding diabetes and atherosclerosis interference, is very valuable. We couldn't find any significant relationship between either sclerostin or OPG levels among different stages of CKD. Also our study demonstared a strong negative relationship between ALP and sclerostin levels and a strong positive relationship between ALP and OPG levels, reminding the importance of ALP levels to predict the bone-mineral status of the children with CKD.
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http://dx.doi.org/10.1515/jpem-2020-0140DOI Listing
November 2020

Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD).

Sci Rep 2020 09 29;10(1):16025. Epub 2020 Sep 29.

Department of Pediatrics, Faculty of Medicine, University Hospital Cologne and University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany.

To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (≤ 3 months; VEBNE) and early (4-15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ≤ 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort.
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http://dx.doi.org/10.1038/s41598-020-71956-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7525474PMC
September 2020

Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome.

Pediatr Dev Pathol 2020 Sep-Oct;23(5):362-371. Epub 2020 May 14.

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

Background: The aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value.

Methods: Renal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome.

Results: The mean age at presentation and follow-up period was 4.9 ± 3.9 and 3.9 ± 3.0 years, respectively. The median time interval from the first symptom to biopsy was 10 days. Vascular thrombosis and interstitial fibrosis were significantly related to chronic kidney disease (CKD) requiring dialysis or kidney transplantation during follow-up (5.6-fold, for both). Glomerular necrosis, cortical necrosis, and glomerular sclerosis were markedly associated with CKD without dialysis (6.2-fold, 13.3-fold, and 8.8-fold, respectively). However, presence of endothelial swelling, subendothelial widening, and fragmented erythrocytes was found to be correlated with a favorable final outcome.

Conclusions: Presence of vascular thrombosis, cortical necrosis, and glomerular sclerosis in histopathological evaluation correlated with developing CKD. Chronic changes in the interstitial compartment were also related to poor prognosis, a finding that has been shown for the first time in pediatric aHUS cases.
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http://dx.doi.org/10.1177/1093526620925947DOI Listing
May 2020

Postural Stability in Children with High Sacral Level Spina Bifida: Deviations from a Control Group.

J Mot Behav 2020 21;52(6):676-686. Epub 2019 Oct 21.

Department of Neurosurgery, Marmara University School of Medicine, Pendik, Istanbul, Turkey.

To assess static and dynamic postural stability changes in children with high sacral level spina bifida. Thirty-five children with high sacral level spina bifida and 35 age-matched healthy controls were enrolled. Their lower extremity muscle strengths and static and dynamic postural stability parameters were measured with the use of a dynamometer and the NeuroCom Balance Master device, respectively. Functional gait and balance were evaluated using the five times sit-to-stand test (5STS) and the 6-minute walk test (6MWT). Spinal, hip, and ankle deformities of the patient group were measured by radiologic evaluation. In comparison with controls, patients were found to have lower ankle dorsiflexion and plantar-flexion strength, increased 5STS duration, and decreased 6MWT distance while both static and dynamic postural stability parameters were significantly different. Bilateral ankle muscle strengths were found to be negatively correlated with postural stability parameters. The presence of hydrocephalus or meningomyelocele in the patient group was found to have negative effects on static postural stability. Static and dynamic postural stability is affected even in children with high sacral level spina bifida who are expected to have best condition in this patient population. The ankle muscle strength is the main factor influencing these changes.
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http://dx.doi.org/10.1080/00222895.2019.1676189DOI Listing
October 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

Polyarteritis nodosa in case of familial Mediterranean fever.

Turk J Pediatr 2018 ;60(3):326-330

Division of Pediatric Nephrology, Department of Pediatrics, Marmara University Faculty of Medicine, İstanbul, Turkey.

Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Protracted febrile myalgia syndrome (PFMS) is a rare form of vasculitic disease which is an uncommon dramatic manifestation of FMF, characterized by severe crippling myalgia and high fever. Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium or small arteries. It is rarely observed in children, but its incidence increases in the presence of FMF. In this article we described a 14-year-old child diagnosed with FMF associated with PAN. Physicians should be aware of this possible association.
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http://dx.doi.org/10.24953/turkjped.2018.03.016DOI Listing
February 2019

Outbreak of Shiga toxin-producing Escherichia-coli-associated hemolytic uremic syndrome in Istanbul in 2015: outcome and experience with eculizumab.

Pediatr Nephrol 2018 12 29;33(12):2371-2381. Epub 2018 Aug 29.

Pediatric Nephrology, Cerrahpaşa Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Background: This study aims to identify epidemiological and clinical characteristics of patients and report our experience with eculizumab treatment during an outbreak of hemolytic uremic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) in Istanbul in 2015.

Methods: Thirty-two children (21 females, median age 3.25 years) were included in this study. Demographic, clinical and laboratory data, and treatment details were retrospectively collected. Renal outcomes were assessed at last follow-up visit. To assess the effect of eculizumab on prognosis of STEC-HUS, subgroup analysis was performed on patients who required dialysis.

Results: A high number of cases occurred within a certain region of Istanbul. Stool samples were cultured from 21 patients (65%), and enteroaggregative E. coli (EAEC; n = 7) and enterohemorrhagic E. coli (EHEC; n = 3) strains were detected. Rates of dialysis treatment, neurological manifestations, and death were 59%, 25%, and 3%, respectively. Mean follow-up duration was 8.6 ± 2.6 months (range 3-12 months). None of the patients (n = 25) was on dialysis at the final visit. The complete renal recovery rate was 54%. Nine patients were treated with eculizumab. At final follow-up visit, no differences in estimated glomerular filtration rate, proteinuria level, or hypertension incidence were observed between patients treated with eculizumab and those not treated with eculizumab.

Conclusions: An outbreak of EAEC occurred in a specific region of Istanbul. Livestock markets were suspected as the source. Evidence for beneficial effects of eculizumab on renal outcome was not clear in this cohort.
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http://dx.doi.org/10.1007/s00467-018-4033-0DOI Listing
December 2018

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

The Clinical and Mutational Spectrum of Turkish Patients with Cystinosis.

Clin J Am Soc Nephrol 2017 Aug 9. Epub 2017 Aug 9.

Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.

Background And Objectives: Infantile nephropathic cystinosis is a severe disease that occurs due to mutations in the cystinosis gene, and it is characterized by progressive dysfunction of multiple organs; >100 cystinosis gene mutations have been identified in multiple populations. Our study aimed to identify the clinical characteristics and spectrum of cystinosis gene mutations in Turkish pediatric patients with cystinosis.

Design, Setting, Participants, & Measurements: We identified the clinical characteristics and spectrum of cystinosis gene mutations in Turkish patients with cystinosis in a multicenter registry that was established for data collection. The data were extracted from this registry and analyzed.

Results: In total, 136 patients (75 men and 61 women) were enrolled in the study. The most common clinical findings were growth retardation, polyuria, and loss of appetite. None of the patients had the 57-kb deletion, but seven novel mutations were identified. The most common mutations identified were c.681G>A (p.Glu227Glu; 31%), c.1015G>A (p.Gly339Arg; 22%), and c.18_21 del (p.Thr7Phefs*7; 14%). These mutations were associated with earlier age of disease onset than the other mutations. To understand the effects of these allelic variants on clinical progression, the mutations were categorized into two major groups (missense versus deletion/duplication/splice site). Although patients with missense mutations had a better eGFR at the last follow-up visit, the difference was not significant. Patients in whom treatment began at age <2 years old had later onset of ESRD (=0.02). Time to ESRD did not differ between the patients with group 1 and group 2 mutations.

Conclusions: The most common cystinosis gene mutations identified in Turkey were c.681G>A (p.Glu227Glu), c.1015G>A (p.Gly339Arg), and c.18_21 del (p.Thr7Phefs*7). Patients with less severe cystinosis gene mutations tend to have better kidney outcome.
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http://dx.doi.org/10.2215/CJN.00180117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5628704PMC
August 2017

Invasive aspergillosis in a patient with end stage renal disease.

Med Mycol Case Rep 2017 Dec 11;18:12-14. Epub 2017 Jul 11.

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

Invasive aspergillosis caused by A. Fumigatus, almost occurs in immunocompromised hosts and has a poor prognosis. We report a case of invasive Aspergillosis in a 15-year-old boy with ESRD. He was initially diagnosed as lobar pneumonia and peritonitis. When he complained for lower extremity weakness and had convulsions, a solid mass originated from right lung compresses the spinal cord and intracranial hemorrhagic abscesses were found on MRI. The biopsy specimen showed hypae of aspergillus-spp and he died on 12th day.
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http://dx.doi.org/10.1016/j.mmcr.2017.06.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5524294PMC
December 2017

Changes in Bacterial Resistance Patterns of Pediatric Urinary Tract Infections and Rationale for Empirical Antibiotic Therapy.

Balkan Med J 2017 Sep 6;34(5):432-435. Epub 2017 Apr 6.

Department of Pediatrics, Division of Pediatric Nephrology, Marmara University School of Medicine, İstanbul, Turkey.

Background: The causative agent spectrum and resistance patterns of urinary tract infections in children are affected by many factors.

Aims: To demonstrate antibiotic resistance in urinary tract infections and changing ratio in antibiotic resistance by years.

Study Design: Retrospective cross-sectional study.

Methods: We analysed antibiotic resistance patterns of isolated Gram (-) bacteria during the years 2011-2014 (study period 2) in children with urinary tract infections. We compared these findings with data collected in the same centre in 2001-2003 (study period 1).

Results: Four hundred and sixty-five uncomplicated community-acquired Gram (-) urinary tract infections were analysed from 2001-2003 and 400 from 2011-2014. Sixty-one percent of patients were female (1.5 girls : 1 boy). The mean age of children included in the study was 3 years and 9 months. Escherichia coli was the predominant bacteria isolated during both periods of the study (60% in study period 1 and 73% in study period 2). Bacteria other than E. coli demonstrated a higher level of resistance to all of the antimicrobials except trimethoprim-sulfamethoxazole than E. coli bacteria during the years 2011-2014. In our study, we found increasing resistance trends of urinary pathogens for cefixime (from 1% to 15%, p<0.05), amikacin (from 0% to 4%, p<0.05) and ciprofloxacin (from 0% to 3%, p<0.05) between the two periods. Urinary pathogens showed a decreasing trend for nitrofurantoin (from 17% to 7%, p=0.0001). No significant trends were detected for ampicillin (from 69% to 71%), amoxicillin-clavulanate (from 44% to 43%), cefazolin (from 39% to 32%), trimethoprim-sulfamethoxazole (from 32% to 31%), cefuroxime (from 21% to 18%) and ceftriaxone (from 10% to 14%) between the two periods (p>0.05).

Conclusion: In childhood urinary tract infections, antibiotic resistance should be evaluated periodically and empiric antimicrobial therapy should be decided according to antibiotic sensitivity results.
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http://dx.doi.org/10.4274/balkanmedj.2015.1809DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5635630PMC
September 2017

Clinical and histopathological features of asymptomaticpersistent microscopic hematuria in children.

Turk J Med Sci 2016 Dec 20;46(6):1707-1711. Epub 2016 Dec 20.

Department of Pediatric Nephrology, School of Medicine, Marmara University, İstanbul, Turkey.

Background/aim: We analyzed the clinical and pathological features and prognosis of 106 children with persistent asymptomatic microscopic hematuria (PAMH) with or without mild proteinuria.

Materials And Methods: This was a retrospective study of 106 children who were referred to our clinics from 2000 to 2013 for evaluation of PAMH.

Results: Among the 106 patients, 69 (65%) were female and 37 (35%) were male. The patients were divided into two groups: 101 patients with isolated microscopic hematuria (IMH) and 5 patients with asymptomatic microscopic hematuria and mild proteinuria (AMHP). Renal biopsy was performed in all 5 children with AHMP: 2 patients had hereditary nephropathy and 2 patients had focal segmental glomerulosclerosis (FSGS). One biopsy specimen revealed nonspecific findings. Renal biopsy was performed in 9 children with IMH: 4 patients had hereditary nephropathy and 5 patients had nonspecific findings. None of the patients received any specific treatment prior to renal biopsy. During the follow-up period, none of the patients developed impaired renal function. Among all the children, only one patient with AMHP developed hypertension and 2 patients with IMH developed proteinuria.

Conclusion: Long-term follow-up must be done carefully for isolated microscopic hematuria and renal biopsy should be performed in selected cases.
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http://dx.doi.org/10.3906/sag-1511-10DOI Listing
December 2016

Leptospirosis in a child with acute respiratory distress syndrome.

Turk J Pediatr 2017 ;59(6):688-692

Department of Pediatric Nephrology, Marmara University Medical Faculty Hospital, Istanbul, Turkey.

Yeşilbaş O, Şevketoğlu E, Kıhtır HS, Hatipoğlu N, Yıldırım HM, Akyol MB, Aktay-Ayaz N, Gökçe İ. Leptospirosis in a child with acute respiratory distress syndrome. Turk J Pediatr 2017; 59: 688-692. Leptospirosis is an infectious vasculitis, which can occur with different clinical features. While it is generally a subclinical and self-limited infection; kidney and liver dysfunction, pulmonary hemorrhage, thrombocytopenia, and cardiovascular collapse may occurr. A six-year-old boy presented with acute respiratory distress syndrome, shock, thrombocytopenia-associated multiple organ failure, and persistent high fever secondary to leptospirosis. Persistent high fever was resistant to intravenous immunoglobulin and pulse steroid therapy. He was successfully treated with plasmapheresis and hemofiltration with endotoxin-cytokine cleaning filter. In conclusion; leptospirosis may cause thrombocytopenia-associated multiple organ failure, persistent high fever, and acute respiratory distress syndrome. Plasmapheresis and hemofiltration should be considered in cases of severe leptospirosis with multiorgan failure.
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http://dx.doi.org/10.24953/turkjped.2017.06.011DOI Listing
January 2017

Vitamin D receptor gene polymorphisms in children with kidney stone disease.

Turk J Pediatr 2017 ;59(4):404-409

Division of Pediatric Nephrology, Marmara University Medical Faculty, İstanbul, Turkey.

Subaşı B, Gökçe İ, Delil K, Alpay H. Vitamin D receptor gene polymorphisms in children with kidney stone disease. Turk J Pediatr 2017; 59: 404-409. Kidney stone disease has a multifactorial etiology involving the interaction of genetic and environmental factors. There is an increased risk of stone formation in the relatives of idiopathic stone patients, which can be explained up to 60% by genetic factors. This study was conducted to explore the association of vitamin D receptor (VDR) gene polymorphisms with the risk of urolithiasis (UL) in Turkish children. We investigated the VDR gene polymorphisms: ApaI, BsmI, TagI, Cdx2, FokI, in 52 children (26 boys, 26 girls) with UL and in 51 healthy children (22 boys, 29 girls) without UL. Apa I, BsmI, TagI, Cdx2, FokI genotypes were analyzed by Apa I, BsmI, TagI, Cdx2, FokI restriction enzyme digestion, respectively. The resulting alleles are designated as ABTCF (ApaI, BsmI, TagI, Cdx2, and FokI restriction site is absent), or abtcf (ApaI, BsmI, TagI, Cdx2, FokI restriction site is present), respectively. Genotype and allele frequencies were calculated, and the association with UL, hypercalciuria and hypocitraturia was investigated. Our data provide no statistically significant evidence for an association between UL and VDR ApaI, BsmI, TagI, Cdx2, and FokI genotype and allele frequencies. Patients with hypocitraturia and hypercalciuria were compared with the control group and no statistically significant difference was detected in terms of VDR gene ApaI, BsmI, TagI, Cdx2, and FokI polymorphisms and allele frequencies. Our data suggest that the VDR ApaI, BsmI, TagI, Cdx2, and FokI polymorphisms do not indicate a significant risk for UL.
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http://dx.doi.org/10.24953/turkjped.2017.04.006DOI Listing
December 2018

The effect of systemic corticosteroids on the innate and adaptive immune system in children with steroid responsive nephrotic syndrome.

Eur J Pediatr 2016 May 30;175(5):685-93. Epub 2016 Jan 30.

Research and Training Hospital, Division of Pediatric Allergy and Immunology, Marmara University, Mimar Sinan Cad. No: 41, 34890, Istanbul, Turkiye.

Unlabelled: The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic nephrotic syndrome (NS) treated with 2 mg/kg/day prednisolone and healthy control (HC) were enrolled. Blood samples were drawn for immunologic analyses at baseline and at the first and second weeks and first, second, and third months of CS therapy in addition to first and second weeks and first, second, and third months of discontinuation. Fourteen patients (M/F, 7/7) between 1 and 8 years old were evaluated. Untreated NS exhibited high absolute lymphocyte count (ALC)(p = 0.010), absolute CD3(+) T cells (p = 0.020) and absolute CD8(+) T cells (p = 0.006) compared to HC. Suppression in ALC was observed and nadir value was noted at first month of therapy compared to baseline (p = 0.002). The CD4(+) (p = 0.036) and CD8(+) T cell (p = 0.013) counts decreased significantly at the first week of treatment compared to baseline. While baseline B cell counts was indifferent from HC, gradually increased in 2 weeks of CS initiation and decreased during the treatment with a statistical significance compared to HC (p = 0.010). However, after cessation of CS, B cell counts continued to decline and found to be significantly different than baseline at first week (p = 0.008) and at third month (p = 0.040).

Conclusion: Apart from baseline lymphocyte subset changing observed in untreated NS patients, our data implies that T cells were suppressed very early in the CS treatment. Interestingly, depressed B cell counts were detected later but persisted even after CS cessation. Due to early decrease in T cells, it would be beneficial to assume the patients as immunosuppressed at the very beginning of CS treatment to avoid infections.

What Is Known: • Corticosteroids (CSs) are widely used for a variety of diseases including nephrotic syndrome, which is related with complex immune disturbance including T and B cells dysfunctions. • CSs induce neutrophilic leukocytosis concomitant with lymphopenia and eosinopenia leading to immunosupression. What is New: • T cell subsets and proliferation are susceptible to CSs more than B cells; however, the reversibility is faster with dose reduction in CS. • The change of B cells and B cell subtypes (CD27 (+) memory) shows prolonged effect of CSs on B cells which may alter antibody production even after 3 months of CSs cessation.
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http://dx.doi.org/10.1007/s00431-016-2694-xDOI Listing
May 2016

Is late-preterm birth a risk factor for hypertension in childhood?

Eur J Pediatr 2014 Jun 29;173(6):751-6. Epub 2013 Dec 29.

Department of Pediatrics, Marmara University Medical Faculty Hospital, Istanbul, Turkey.

Unlabelled: Late-preterm birth is associated with higher rates of neonatal morbidity and mortality and higher health care utilization, but its impact on later life is not well known. In this study, we aimed to evaluate whether late-preterm birth affects blood pressure, renal function, and urinary protein excretion in children later in life. Sixty-five children aged 4 to 13 years born as late-preterm and 65 age- and sex-matched children born full term were evaluated with 24-h ambulatory blood pressure monitoring (ABPM), urinary microalbumin excretion (UAE), and glomerular filtration rate (GFR). All subjects underwent ABPM prospectively. For each gender, daytime, nighttime, and 24-h systolic and diastolic and mean blood pressures (SBP, DBP, and MAP) were transformed to standard deviation scores (SDS). Blood pressure profiles (SBP DBP, and MAP) were considered abnormal when the corresponding SDS values exceeded 1.63. Urinary microalbumin excretion was expressed as milligrams per day, and the value between 30 and 300 mg/day was defined as microalbuminuria (MA). There was no significant difference in the mean GFR and MA levels between late-preterm and term children. 24-h systolic BP SDS, daytime systolic BP SDS, nighttime systolic BP SDS, 24-h diastolic BP SDS, nighttime diastolic BP SDS, 24-h MAP BP SDS, daytime MAP BP SDS, and nighttime MAP BP SDS were found to be significantly higher in late-preterm children compared to term children.

Conclusion: We conclude that late-preterm children have higher BP levels, so those children should be followed up carefully by the pediatrician regarding probable hypertension in their future life.
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http://dx.doi.org/10.1007/s00431-013-2242-xDOI Listing
June 2014

Effects of rapamycin and tacrolimus on mature endothelial cells and endothelial progenitor cells.

J Pak Med Assoc 2012 Aug;62(8):822-5

Department of Nephrology, Marmara University Medical Faculty, Altunizade, Istanbul, Turkey.

Objective: To investigate the effects of the potent immunosuppressive agents tacrolimus and rapamycin on the number of circulating mature endothelial cells and circulating endothelial progenitor cells in an experimental model.

Methods: It was an experimental study performed from December 2007 to January 2008 in which the effects of the immunosuppressive agents tacrolimus and rapamycin on endothelial progenitr cells and circulating mature endothelial cells were analysed on 24 male wistar albino rats in a controlled environment model. Circulating cell populations were measured by flow-cytometric analysis. Maun-Whitney U test and analysis of vartiance were used for statistical purposes.

Results: Rapamycin increased the number of circulating mature endothelial cells approximately 2-fold compared to tacrolimus. The number of endothelial progenitor cells also was increased in the peripheral blood of rats treated with rapamycin compared to those treated with tacrolimus.

Conclusion: The study showed that treatment with rapamycin is associated with an increase in endothelial progenitor cells and circulating mature endothelial cells. This increase may be associated with endothelial cell damage and repair.
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August 2012

Urinary stone disease in the first year of life: is it dangerous?

Pediatr Surg Int 2013 Mar 25;29(3):311-6. Epub 2012 Dec 25.

Division of Pediatric Nephrology, Department of Pediatrics, Medical Faculty, Marmara University, Istanbul, Turkey.

Purpose: We have evaluated the clinical, radiological and metabolic features of infantile urolithiasis (UL).

Materials And Methods: We have reviewed the medical records of 93 children who were diagnosed as having UL before 1 year of age. We recorded patient demographics, the age at diagnosis, presenting symptoms, family history, the localizations and dimensions of stones, urinary metabolic examinations, as well as physical, laboratory, and radiologic findings. Our secondary objective was to compare some features of this group with those of older children with UL followed-up in the same clinic which were previously reported.

Results: We evaluated 93 children referred to our pediatric nephrology clinics. A family history of UL was 56.2 % in the study group. Resolution of stones was observed in 30.1 % of the cases. Urinary tract infections (UTIs) were detected in 65.9 % of females and 46.2 % of males. At least one urinary metabolic abnormality was found in 79.5 % of all the children. Most commonly seen metabolic abnormality was hypercalciuria. In all patients stones were located in kidneys except one infant who had an ureteral stone together with a kidney stone. Fifteen (16.1 %) children had an accompanying systemic disorder.

Conclusions: Among pediatric urinary stone diseases infantile UL can be regarded as a separate clinical entity. Coexistence of systemic disorders and anatomic anomalies at high frequencies may indicate a role of distinct pathogenetic mechanisms. In addition, high rates of UTIs and metabolic abnormalities in this age group justify screening for these parameters during follow-up of these children.
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http://dx.doi.org/10.1007/s00383-012-3235-yDOI Listing
March 2013

Inhaled iloprost in the treatment of pulmonary hypertension in very low birth weight infants: a report of two cases.

J Pak Med Assoc 2012 Apr;62(4):388-91

Department of Paediatrics, Erzurum District Training and Research Hospital, Erzurum, Turkey.

We treated 2 very low birth weight (VLBW) infants with respiratory distress syndrome suffering from refractory hypoxic respiratory failure complicated with severe pulmonary hypertension with inhaled iloprost. The first infant was an 800 gram male and the second case was a 920 gram female. Echocardiography revealed a right to left shunt through patent duct in the first case; suprasystemic pulmonary arterial pressure was estimated by using tricuspid regurgitation of moderate severity in the second case. Inhaled iloprost was started in those infants when conventional therapies including the administration of exogenous surfactant and high-frequency oscillatory ventilation failed. After the commencement of therapy, the clinical condition of the infants improved dramatically. Pulmonary arterial pressure returned to normal levels within five days. We suggest that inhaled iloprost may be helpful by improving oxygenation and reducing the need for aggressive mechanical ventilation in some cases of severe hypoxaemic respiratory failure in VLBW infants.
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April 2012

Clinical spectrum of antenatally detected urinary tract abnormalities with respect to hydronephrosis at postnatal ultrasound scan.

Pediatr Surg Int 2012 May 18;28(5):543-52. Epub 2012 Mar 18.

Division of Pediatric Nephrology, Department of Pediatrics, Marmara University Medical Faculty Hospital, Istanbul, Turkey.

Aim: The purpose of this study was to compare the outcome of infants having antenatally detected urinary tract abnormalities (AUTAs) with respect to the presence of hydronephrosis in postnatal ultrasonography (US) examination.

Patients And Methods: Between January 1999 and October 2009, 256 infants diagnosed with AUTAs were prospectively followed. Infants were divided into two groups according to the presence of hydronephrosis in postnatal US examination: Group 1, infants with hydronephrosis; Group 2, infants without hydronephrosis (including renal cyst, agenesis, ectopic kidney). The events of interest were the presence and diagnoses of uropathy, AUTA resolution, urinary tract infection (UTI), development of renal parenchymal defects (RPDs)--focal or global scarring, dysplasia--, acute kidney injury (AKI) and chronic kidney disease (CKD), and the need for surgery and dialysis treatment.

Results: The most commonly detected underlying abnormalities were ureteropelvic junction obstruction (44.8 %), vesicoureteral reflux (VUR) (30.0 %) and megaureter (9.5 %) in patients with postnatal hydronephrosis. On the other hand, multicystic dysplastic kidney (43.5 %), renal agenesis (19.4 %) and VUR (19.4 %) were mostly encountered abnormalities in patients without postnatal hydronephrosis. RPDs were significantly more common among patients with postnatal hydronephrosis compared to those without hydronephrosis (37 vs. 21 %, P = 0.02). The incidence of UTI and VUR was higher in infants with postnatal hydronephrosis than in infants without hydronephrosis. There was no statistically significant difference in terms of the development of AKI and CKD and the need for surgery and dialysis treatment between patients with hydronephrosis and those without hydronephrosis.

Conclusion: Infants with AUTAs should be investigated postnatally. The findings from this study will help to identify the natural history and outcome of infants with AUTAs according to the postnatal US parameters.
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http://dx.doi.org/10.1007/s00383-012-3072-zDOI Listing
May 2012

Early Transient Neonatal Cyanosis Related to Interatrial Right-to-Left Shunting at an Altitude of 1890 Meters: A Report of Five Cases.

Iran J Pediatr 2011 Jun;21(2):239-43

Erzurum District Training and Research Hospital, Department of Pediatric Cardiology, Erzurum, Turkey.

Background: We report five term neonates born at an altitude of 1890 meters with transient early neonatal cyanosis due to right-to-left shunting at atrial level through patent foramen ovale.

Case Presentation: The five neonates with no clinical sign or symptom other than marked cyanosis were examined in two neonatal units of Erzurum city. Hematologic and radiologic examinations were normal. Partial oxygen pressure (PO(2)) in the arterial blood samples was lower than 45 mmHg in all of the patients, and did not increase more than 15 mmHg in any of the patients after inhalation of 100% oxygen. Echocardiography revealed normal intracardiac structure. The right-to-left interatrial shunt at diastole was detected through a patent foramen ovale in all of these infants. By only observation with no treatment, diastolic right-to-left shunt disappeared in 40.15±9.52 hours. Oxygen saturation was increased from 69.80±9.55 percent to 90.40±8.80 percent. The patients were discharged from the hospital at 5.6±0.4 days of life. Follow up for 6 months revealed no clinical problem in any of the cases.

Conclusion: Transient cyanosis can be seen in the very early neonatal period because of interatrial right-to-left shunting in some healthy term neonates born at an altitude of 1890 meters. Decreased right atrial compliance due to relative hypoxia at that altitude can be speculated to be the causative mechanism.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3446169PMC
June 2011

Serum alkaline phosphatase levels in healthy children and evaluation of alkaline phosphatase z-scores in different types of rickets.

J Clin Res Pediatr Endocrinol 2011 23;3(1):7-11. Epub 2011 Feb 23.

Marmara University, Department of Pediatric Endocrinology, İstanbul, Turkey.

Objective: Serum alkaline phosphatase (ALP) levels show great variation with age and sex in children and adolescents. Additionally, different buffers used even in the same method cause variable results. This detail is not usually taken into account in the evaluation. We aimed to study pediatric age- and sex-specific reference ranges for ALP by colorimetric assay using p-nitrophenyl phosphate as substrate and diethanolamine as buffer and also to compare the ALP levels in patients with different types of rickets.

Methods: 1741 healthy children and adolescents (904 girls) were included in the study for normative data. 77 different ALP measurements from 38 nutritional rickets (NR), 7 vitamin D-dependent rickets (VDDR) and 8 hypophosphatemic rickets (HR) patients were included.

Results: Reference values for ALP were constructed. ALP levels demonstrated a tetraphasic course with two peaks at infancy and puberty. There was no difference in ALP levels between boys and girls until puberty. However, higher ALP levels were noted at 10-11 years in girls (p=0.02) and at 12-13, 14-15, 16-17 years in boys (p<0.001). ALP levels start to decline after age 12 and 14 in girls and boys, respectively. Serum ALP levels were highest in the VDDR group and lowest in the HR group (median z-score values in HR, VDDR and NR were 3.6, 10.4 and 6.5, respectively; p<0.001). Similarly, plasma parathormone(PTH) levels ranged from highest to lowest in the VDDR, NR and HR groups (median values: 525, 237 and 98 pg/mL, respectively; p<0.001).

Conclusions: This normative data will provide a basis for better evaluation of ALP levels determined by the described method. Furthermore, use of z-scores gives a more precise assessment of changes in ALP levels in rickets and other bone disorders.
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http://dx.doi.org/10.4274/jcrpe.v3i1.02DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3065317PMC
December 2011

Familial Mediteranean fever with protein-losing enteropathy due to constrictive pericarditis.

World J Pediatr 2011 Nov 5;7(4):365-7. Epub 2011 Jan 5.

Department of Pediatric Nephrology and Rheumatology, Gülhane Military Academy of Medicine, School of Medicine, 06018 Etlik, Ankara, Turkey.

Background: Constrictive pericarditis (CP) represents a rare cause of protein-losing enteropathy (PLE) resulting from intestinal lymphangiectasia (IL). In this report, we describe an 8-year-old Turkish boy with IL and PLE secondary to CP.

Methods: The boy was introduced to our clinic due to bilateral pretibial edema and swelling of the eyelids caused by hypoproteinemia. Physical examination revealed a distended right jugular vein. Laboratory investigation revealed PLE with fecal concentration of alpha-1 antitripsin of 4.87 mg/g. Histopathologic examination of random biopsies obtained from the duodenum revealed markedly dilated lymphatics compatible with IL. Constrictive pericarditis was diagnosed by tagged cine cardiac magnetic resonance imaging.

Results: Pericardiectomy was performed for the patient. Genetic analysis was done and heterozygous mutation E148Q was detected as a disease-causing Mediterranean fever (MEFV) mutation. Colchicine was started after the operation. Six months after the initiation of regular colchicine therapy, echocardiography revealed disappearance of CP.

Conclusion: This is the first reported case of PLE with a distended right jugular vein due to CP secondary to familial Mediterranean fever associated with E148Q heterozygosity in the MEFV gene.
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http://dx.doi.org/10.1007/s12519-011-0255-yDOI Listing
November 2011

A comparison of different methods for the determination of glomerular filtration rate in elderly patients with chronic renal failure.

Int Urol Nephrol 2011 Mar 20;43(1):257-63. Epub 2010 Oct 20.

Department of Nephrology, Erzurum District Training and Research Hospital, 25070, Erzurum, Turkey.

Objective: The aim of the present study was to asses the accuracy of prediction equations in elderly patients with advanced renal disease (ARD).

Patients And Methods: Twenty-three elderly patients (>60 years) with chronic kidney disease (CKD) stages 3-5 underwent technetium-99 m-diethylenetriaminepentaacetic acid (Tc-99m-DTPA) radionuclide measurement of glomerular filtration rate (GFR). To predict GFR, estimation formulas [Cockcroft-Gault (CG), 6-variable Modification of Diet in Renal Disease (6-var-MDRD) and the corrected 24-hour creatinine clearance (24-h CCL) method] were used. Estimates of bias and precision were obtained to compare prediction equations of GFR with standard radionuclide measurement.

Results: In the present study, all the prediction equations overestimated the GFR measured with the radionuclide method. Six-var-MDRD and 24-h CCL methods correlated better with standard radionuclide measurements compared to the CG formula (Rc of 0.53 and 0.50 vs. 0.41, respectively) as for GFR prediction, but they were still insufficiently accurate.

Conclusion: Glomerular filtration rate prediction equations correlate poorly with standard radionuclide measurements and their use may lead to late initialization of renal replacement therapy in elderly patients with ARD. Therefore, measurement by standard radionuclide method (Tc-99m-DTPA) is recommended to accurately assess GFR in elderly patients with ARD.
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http://dx.doi.org/10.1007/s11255-010-9846-0DOI Listing
March 2011

Urinary levels of interleukin-6 and interleukin-8 in patients with vesicoureteral reflux and renal parenchymal scar.

Pediatr Nephrol 2010 May;25(5):905-12

Pediatric Nephrology Department, Marmara University Medical Faculty, Istanbul, Turkey.

The objective of this study was to assess the urine levels of interleukin-6 (IL-6) and interleukin-8 (IL-8) as noninvasive markers of vesicoureteral reflux (VUR) and renal parenchymal scarring (RPS) in children in the absence of a recent urinary tract infection (UTI) episode. Urine concentrations of IL-6 and IL-8 in 114 children aged 1 month to 16 years were evaluated. The children were divided into four groups: group 1, 26 children with VUR and RPS; group 2, 27 children with VUR without RPS; group 3, 34 children with RPS without VUR, group 4, 27 children without VUR and RPS, as the control group. After the first assessment, the children were divided into four larger groups for comparison purposes: group A (groups 1+2), 53 children with VUR; group B (groups 3+4), 61 children without VUR; group C (groups 1+3), 60 children with RPS; group D (groups 2+4), 54 children without RPS. Urinary IL-6 and IL-8 concentrations were determined. To avoid dilution effects and to the standardize samples, urinary levels of IL-6 and IL-8 were expressed as the ratio of cytokine to urinary creatinine (pg/mg). The median urine IL-6/creatinine was significantly higher in patients with VUR than in those without VUR (5.72 vs. 3.73). In patients with VUR, there was a significant but rather weak correlation between IL-6/creatinine concentrations and there flux grade (p<0.05, R=0.305). The median urine IL-8/creatinine was significantly higher in patients with RPS than in those without RPS (43.12 vs. 16.36). In patients with RPS, there was a significant but rather weak correlation between IL-8/creatinine concentrations and the renal scar grade (p<0.05, R=0.251). The results of this study provide preliminary evidence that children with VUR have a high urine IL-6 concentration, whereas children with RPS have a high urine IL-8 concentration.
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http://dx.doi.org/10.1007/s00467-009-1396-2DOI Listing
May 2010

Hereditary spherocytosis with immunoglobulin A nephropathy.

Iran J Kidney Dis 2010 Jan;4(1):78-81

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

Hereditary spherocytosis is a familial hemolytic anemia. Immunoglobulin A (IgA) nephropathy associated with hereditary spherocytosis has not been reported in children. Here, we report a case of a 17-year-old boy with IgA nephropathy and hereditary spherocytosis. The patient was diagnosed with hereditary spherocytosis at the age of 12 years and splenectomy was done at the age of 15 years. Later, the patient presented with macroscopic hematuria and proteinuria. Kidney biopsy of the boy was consistent with IgA nephropathy. Treatment with angiotensin-converting enzyme inhibitor was started. The patient became free of proteinuria after the 6th month of therapy.
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January 2010

Clinical and metabolic features of urolithiasis and microlithiasis in children.

Pediatr Nephrol 2009 Nov 15;24(11):2203-9. Epub 2009 Jul 15.

Department of Pediatric Nephrology, Marmara University, Istanbul, Turkey.

We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis or microlithiasis who had been referred to our pediatric nephrology clinics between 1998 and 2008 with suspected urolithiasis. The medical histories of these children (78 girls, 84 boys), who ranged in age from 2 months to 16 years (mean age 5.59 +/- 0.35 years), were reviewed retrospectively for clinical and metabolic features of urinary tract calculi. Urinary tract infections (UTI) were present in 45.9% of the cases. The most common presenting symptoms were flank pain or restlessness (25.3%) and hematuria (21.6%), followed by UTI (16%), whereas 23.5% of the cases were detected incidentally during evaluation for other medical conditions. Other symptoms at presentation included dysuria, passing stones, penile edema, enuresis, vomiting and anorexia. Urine analysis revealed metabolic abnormalities in 87% of the cases, including hypercalciuria (33.8%), hypocitraturia (33.1%), hyperoxaluria (26.5%), hyperuricosuria (25.4%), hypocitraturia + hypercalciuria (21.1%), hyperphosphaturia (20.8%) and cystinuria (5.7%). Almost 50% of the patients had a positive family history for urolithiasis. The most frequently involved site was in the kidneys (86%). Ureters and bladder were involved in 12 and 2% of the cases, respectively. A family history of urolithiasis, presenting symptoms and underlying metabolic abnormalities were similar for microlithiasis and the patients with larger stones. However, in our study population, microlithiasis was mainly a disease of young infants, with a greater chance for remission and often not associated with structural changes. The presenting symptoms of urolithiasis show a wide spectrum, so that a high index of suspicion is important for early detection. A metabolic abnormality can be identified in 87% of cases of urolithiasis. Detection of microlithiasis may explain a number of symptoms, thus reducing invasive diagnostic procedures and allowing early recognition of metabolic abnormalities. These results draw attention to the importance of screening for UTIs in patients with urolithiasis.
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http://dx.doi.org/10.1007/s00467-009-1231-9DOI Listing
November 2009

The co-existence of membranoproliferative glomerulonephritis type 1 and coeliac disease: a case report.

Pediatr Nephrol 2009 Jun 31;24(6):1247-50. Epub 2009 Jan 31.

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

Coeliac disease is an autoimmune enteropathy characterised by chronic inflammation of the small intestinal mucosa and the presence of typical autoantibodies. Coeliac disease may be a risk factor for renal disease. Immunoglobulin A (IgA) nephropathy is reported in the majority of these cases. Only one adult patient had been reported with membranoproliferative glomerulonephritis (MPGN) and coeliac disease. Here, we report a case in a 12-year-old girl with coeliac disease who presented with severe anaemia and later developed nephrotic syndrome. Renal biopsy of the patient was consistent with MPGN type 1, which has not been previously reported in children with coeliac disease. A gluten-free diet was started. After 6 months of this diet, her nephrotic syndrome resolved completely. This case is presented to draw attention to the rare association of coeliac disease and MPGN type 1.
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http://dx.doi.org/10.1007/s00467-009-1118-9DOI Listing
June 2009