Publications by authors named "Fatos Yalcinkaya"

118 Publications

Chronic non-bacterial osteomyelitis: another disease associated with MEFV gene mutations.

Clin Exp Rheumatol 2020 Sep-Oct;38 Suppl 127(5):112-117. Epub 2020 Nov 25.

Department of Paediatric Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey.

Objectives: Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disease of unknown aetiology. The relationship between CNO and familial Mediterranean fever (FMF) is not clearly documented so far. This cross-sectional study aims to evaluate the clinical and laboratory characteristics of a cohort of CNO patients within the context of its relationship with FMF and MEFV gene mutations.

Methods: Demographic and clinical data were extracted from electronic medical records of patients with CNO. The MEFV gene analysis was performed for all patients.

Results: A total number of 18 patients with CNO with a median follow-up of 36.50 (13.00-84.00) months were included in the study. Five patients (27.8%) were found to have at least one exon 10 mutations (four with M694V and one with M680I). Four of them (22.2%) had homozygous or compound heterozygous mutations of the MEFV gene. Two patients had a previous diagnosis of FMF and developed CNO while FMF was under control. Patients with MEFV mutations had an earlier onset of CNO, higher acute phase reactants, lower haemoglobin concentrations, and a higher number of bone lesions at disease onset with a persistent course of disease more frequently.

Conclusions: Our results demonstrated an increased frequency of MEFV gene mutations in CNO and a more severe disease phenotype of CNO in patients with MEFV gene mutations. Physicians practicing in regions where FMF is prevalent should be aware of this relationship and ask about the symptoms of FMF in detail in patients with CNO. Moreover, FMF should be included in CNO-associated conditions.
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December 2020

Active vitamin D is cardioprotective in experimental uraemia but not in children with CKD Stages 3-5.

Nephrol Dial Transplant 2021 Feb;36(3):442-451

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

Background: Uraemic cardiac remodelling is associated with vitamin D and Klotho deficiency, elevated fibroblast growth factor 23 (FGF23) and activation of the renin-angiotensin system (RAS). The cardioprotective properties of active vitamin D analogues in this setting are unclear.

Methods: In rats with 5/6 nephrectomy (5/6Nx) treated with calcitriol, the cardiac phenotype and local RAS activation were investigated compared with controls. A nested case-control study was performed within the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study, including children with chronic kidney disease (CKD) Stages 3-5 [estimated glomerular filtration rate (eGFR) 25 mL/min/1.73 m2] treated with and without active vitamin D. Echocardiograms, plasma FGF23 and soluble Klotho (sKlotho) were assessed at baseline and after 9 months.

Results: In rats with 5/6Nx, left ventricular (LV) hypertrophy, LV fibrosis and upregulated cardiac RAS were dose-dependently attenuated by calcitriol. Calcitriol further stimulated FGF23 synthesis in bone but not in the heart, and normalized suppressed renal Klotho expression. In the 4C study cohort, treatment over a mean period of 9 months with active vitamin D was associated with increased FGF23 and phosphate and decreased sKlotho and eGFR compared with vitamin D naïve controls, whereas LV mass index did not differ between groups.

Conclusions: Active vitamin D ameliorates cardiac remodelling and normalizes renal Klotho expression in 5/6Nx rats but does not improve the cardiac phenotype in children with CKD Stages 3-5. This discrepancy may be due to further enhancement of circulating FGF23 and faster progression of CKD associated with reduced sKlotho and higher serum phosphate in vitamin D-treated patients.
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http://dx.doi.org/10.1093/ndt/gfaa227DOI Listing
February 2021

The changing resistance patterns of bacterial uropathogens in children.

Pediatr Int 2020 Sep;62(9):1058-1063

Departments of Division of Nephrology, Ankara University School of Medicine, Ankara, Turkey.

Background: Increased antimicrobial resistance is a problem in managing urinary tract infections (UTI). With this study we assessed the resistance patterns of urinary isolates in children with UTI between January 2017 and January 2018.

Methods: A retrospective cohort study was conducted. Among 5,443 isolates, a total of 776 UTI episodes in 698 patients were included. Patients' gender, age, voiding dysfunction, UTI history, prophylaxis status, and presence of vesicoureteral reflux were noted. Patients were divided into three age groups: group 1 for ages ≤12 months; group 2 for ages 13-60 months; and group 3 for ages >60 months. The susceptibilities of etiologic agents to different antimicrobials were explored.

Results: Median age was 54 months (range 1 month-21 years); male to female ratio was 1:5. The most common causative agent was Escherichia coli (83% of the cases), followed by Klebsiella pneumoniae (7.5%). Resistance to ampicillin (62.6%) and co-trimoxazole (39.8%) were remarkable in all isolates. Overall extended-spectrum beta-lactamase (ESBL) positivity was 23.5%. The highest resistance rates, higher ESBL positivity (28.6%), and K. pneumoniae frequency (13.5%) were observed in group 1. Ceftriaxone resistance was significantly low (0.5%) in the ESBL (-) group, which constituted the majority of the isolates. Higher resistance rates were observed among the patients on prophylaxis compared to those off prophylaxis (P < 0.001).

Conclusion: Ceftriaxone can still be used for empirical treatment; however, initial urine culture results are crucial due to high ESBL positivity. Special consideration must be taken for patients under 1 year of age. Periodical surveillance studies are needed to explore the changing resistance patterns of uropathogens and modify treatment plans.
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http://dx.doi.org/10.1111/ped.14270DOI Listing
September 2020

Clinical characteristics and outcome of childhood vesicoureteral reflux.

Arch Argent Pediatr 2020 02;118(1):e16-e21

Ankara University School of Medicine, Department of Pediatrics, Division of Nephrology.

Introduction: The aim of the study was to assess the clinical features and outcome parameters of children with vesicoureteral reflux (VUR) based on gender and VUR grade.

Population And Methods: Patients with VUR who were seen during routine follow-up visits at Ankara University Children's Hospital between January 2014-January 2015 were included in this retrospective study. Patient demographics, clinical course, laboratory investigations, imaging were noted.

Results: Two hundred and twenty patients were recruited. Mean age at the time of diagnosis was 3,17 ± 3,08 years. Boys were diagnosed at younger ages as compared to girls (2.00 ± 2,59 vs. 3,81 ± 3.15, p < 0.001). Urinary tract infection (UTI) was the most common presentation. The second presentation form was antenatal hydronephrosis (AHN) which was more common in males (25.6 %, p < 0.001). Twenty-two percent of the patients had grade 1-2, 51 % grade 3 and 27 % grade 4-5 reflux. Patients with grade 4-5 reflux had more abnormal ultrasound (US) and Tech 99m dimercaptosuccinic acid scintigraphy (DMSA) findings and surgery was performed more frequently in this group (p < 0.001). In males, grade 4-5 reflux (43.6 % vs. 18.3 %), abnormal US (77 % vs. 54 %) and DMSA (77 % vs. 59 %) findings were more frequent (p < 0.05). In girls higher rates of UTIs, lower urinary tract dysfunction (LUTD) and spontaneous reflux resolution were seen (p < 0.05).

Conclusions: Despite younger age at diagnosis, spontaneous resolution was found lower in boys and they had more frequent AHN, more severe reflux, and radiological abnormalities.
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http://dx.doi.org/10.5546/aap.2020.eng.e16DOI Listing
February 2020

The effect of genotype on musculoskeletal complaints in patients with familial Mediterranean fever.

Postgrad Med 2020 Mar 22;132(2):220-224. Epub 2020 Jan 22.

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

: Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent, self limited attacks of fever with serositis. The aim of this study was to describe the frequency of musculoskeletal complaints in children with FMF and to investigate the effect of genotype on these findings.: Files of patients who had been seen in our department (during routine follow-up visits) were retrospectively evaluated. Comparisons regarding musculoskeletal findings were performed between patients with homozygous/compound heterozygous and heterozygous mutations. Thereafter, patients with two mutations were divided into three groups; M694V/M694V, M694V/other mutation, and patients carrying two mutations other than M694V. Patients with single mutation were divided into two groups; M694V and non M694V carriers.: The study group comprised 317 FMF patients (170 females) with a mean age of 12.2 ± 5.7 years. Arthralgia (42.6%), leg pain (42.9%), and heel pain (35.6%) were the most common musculoskeletal complaints in children with FMF. Frequency of musculoskeletal findings of the patients who had two mutations did not differ from the patients with single mutations (p > 0.05). Leg and heel pain were more frequently detected in patients with homozygous M694V mutation (p = 0.001). Among patients with heterozygous mutations; children with M694V mutation had more frequently arthralgia, leg, and heel pain (p < 0.05).: Musculoskeletal problems were commonly encountered in patients with FMF. Genotype seems to affect the frequency of these problems and M694V mutation is a predisposing factor for musculoskeletal complaints.
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http://dx.doi.org/10.1080/00325481.2019.1708147DOI Listing
March 2020

Management of antenatal hydronephrosis.

Pediatr Nephrol 2020 12 6;35(12):2231-2239. Epub 2019 Dec 6.

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

Antenatal hydronephrosis (AHN) is the most frequently detected abnormality by prenatal ultrasonography. Differential diagnosis of AHN includes a wide variety of congenital abnormalities of the kidney and urinary tract ranging from mild abnormalities such as transient or isolated AHN to more important ones as high-grade congenital vesicoureteral reflux or ureteropelvic junction obstruction. It is well known that the outcome depends on the underlying etiology. Various grading systems have been proposed for the classification of AHN on prenatal and postnatal ultrasonography. Mild isolated AHN represents up to 80% of cases, is considered to be benign, and majority of them resolve, stabilize, or improve during follow-up. Controversies exist regarding the diagnosis and management of some important and severe causes of AHN such as high-grade vesicoureteral reflux and ureteropelvic junction obstruction. Current approach is becoming increasingly conservative during diagnosis and follow-up of these patients with less imaging and close follow-up. However, there is still no consensus regarding the clinical significance, postnatal evaluation, and management of infants with AHN. The aim of this review is to discuss the controversies and provide an overview on the management of AHN.
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http://dx.doi.org/10.1007/s00467-019-04420-6DOI Listing
December 2020

Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children.

Kidney Int 2019 07 20;96(1):214-221. Epub 2019 Mar 20.

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany. Electronic address:

Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. We performed a post hoc analysis of the Cardiovascular Comorbidity in Children with CKD (4C) study, which prospectively follows children aged 6-17 years with baseline estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m. uEGF levels were measured in archived urine collected within 6 months of enrollment. Congenital abnormalities of the kidney and urinary tract were the most common cause of CKD, with glomerular diseases accounting for <10% of cases. Median eGFR at baseline was 28 ml/min/1.73 m, and 288 of 623 participants (46.3%) reached the composite endpoint of CKD progression (50% eGFR loss, eGFR < 10 ml/min/1.73 m, or initiation of renal replacement therapy). In a Cox proportional hazards model, higher uEGF/Cr was associated with a decreased risk of CKD progression (HR 0.76; 95% CI 0.69-0.84) independent of age, sex, baseline eGFR, primary kidney disease, proteinuria, and systolic blood pressure. The addition of uEGF/Cr to a model containing these variables resulted in a significant improvement in C-statistics, indicating better prediction of the 1-, 2- and 3-year risk of CKD progression. External validation in a prospective cohort of 222 children with CKD demonstrated comparable results. Thus, uEGF may be a useful biomarker to predict CKD progression in children with CKD.
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http://dx.doi.org/10.1016/j.kint.2019.01.035DOI Listing
July 2019

Alemtuzumab as a Successful Antirejection Therapy: Experience in a Pediatric Renal Transplant Patient.

Exp Clin Transplant 2019 02 22. Epub 2019 Feb 22.

From the Department of Pediatrics, Division of Nephrology, Ankara University School of Medicine, Ankara, Turkey.

Alemtuzumab is a monoclonal antibody against CD52 that is being increasingly used in renal transplantation as a lymphocyte-depleting agent. Data on alemtuzumab use in resistant rejection episodes are scarce, especially in children. Here, we present a 14-year-old renal transplant patient with acute cellular and humoral rejection who was treated with pulse steroids, plasmapheresis, and intravenous immunoglobulin with no success. He had 2 previous rejection episodes that were treated with antithymocyte globulin. In the third episode, alemtuzumab was given as a rescue therapy, and the patient benefited from the treatment. No complications were observed. Alemtuzumab can be a treatment option in pediatric patients with refractory rejection episodes.
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http://dx.doi.org/10.6002/ect.2018.0203DOI Listing
February 2019

Clinical features and disease severity of Turkish FMF children carrying E148Q mutation.

J Clin Lab Anal 2019 May 4;33(4):e22852. Epub 2019 Feb 4.

Department of Pediatric Rheumatology and Nephrology, Ankara University School of Medicine, Ankara, Turkey.

Background: Familial Mediterranean fever (FMF) is the most common hereditary monogenic autoinflammatory disease caused by mutations in the MEFV gene. It is controversial whether E148Q alteration is an insignificant variant or a disease-causing mutation. The aim of this study was to evaluate the clinical features and disease severity of FMF patients carrying E148Q mutation.

Methods: Files of FMF patients were retrospectively evaluated. Patients with at least one E148Q mutation were included to the study. The clinical characteristics and disease severity of the patients who were carrying only E148Q mutation were compared with the patients who were compound heterozygous for E148Q and homozygous for M694V mutation.

Results: The study group comprised 33 patients who were homozygous or heterozygous for E148Q; 34 with compound heterozygous E148Q mutations and 86 patients who had homozygous M694V mutation. Patients who had only E148Q mutation were found to have the oldest mean age of disease onset and lowest mean disease severity score. Attack frequency and colchicine doses were lower in patients with only E148Q mutation as compared with the other two groups. The frequency of clinical findings such as fever, abdominal pain, arthralgia, and arthritis among the three groups was similar.

Conclusion: Familial Mediterranean fever patients with only E148Q mutation are presenting with late-onset and milder disease course despite having similar clinical findings as compared with patients who had other mutations. Finally, we imply that E148Q is a mutation and colchicine treatment should be given.
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http://dx.doi.org/10.1002/jcla.22852DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6528560PMC
May 2019

Neonatal onset familial Mediterranean fever.

Mod Rheumatol 2019 Jul 18;29(4):647-650. Epub 2018 Oct 18.

a Department of Pediatric Rheumatology , Ankara University School of Medicine , Ankara , Turkey.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that FMF patients with early disease onset have more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had disease onset during the neonatal period. Medical records of all patients diagnosed as FMF and had been seen in the outpatient clinic of Paediatric Rheumatology department between January 2013 and January 2014 were retrospectively evaluated. Patients with disease onset during the first month of life were included to the study. Among 317 patients; 19 (12 males) were included to the study. Approximately 60% of the patients had family history of FMF. Homozygous p.M694V mutation was detected in 42% of the cases. Thirteen patients present with attacks of fever and remaining had attacks in the form of restlessness, resembling infantile colic starting in the neonatal period. Majority of these patients developed classical abdominal attacks between the ages of 1 and 2.5 years. The diagnosis of FMF was significantly delayed; the median age at onset of therapy was 3.5 years (range 7 months-17 years). Patients with FMF could have complaints even in the neonatal period. Homozygous p.M694V mutation is a prominent mutation in this group of patients. In order to prevent diagnostic delay physicians dealing with these type of patients should be more vigilant.
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http://dx.doi.org/10.1080/14397595.2018.1500874DOI Listing
July 2019

Transplantation within the era of anti-IL-1 therapy: case series of five patients with familial Mediterranean fever-related amyloidosis.

Transpl Int 2018 10 13;31(10):1181-1184. Epub 2018 Jul 13.

Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, Ankara, Turkey.

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http://dx.doi.org/10.1111/tri.13312DOI Listing
October 2018

Sacroiliitis in Children With Familial Mediterranean Fever.

J Clin Rheumatol 2019 Mar;25(2):69-73

Nephrology, Department of Pediatrics, Ankara University School of Medicine.

Background/objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent, self-limited attacks of fever with serositis. Various diseases were reported to be associated with FMF. The aim of this study was to investigate the frequency and characteristics of sacroiliitis in children with FMF.

Methods: Files of FMF patients who had been seen in 2 reference hospitals in Ankara were retrospectively evaluated. Patients with FMF and concomitant sacroiliitis were included to the study. All patients had magnetic resonance imaging evidence of sacroiliitis.

Results: Among 650 FMF patients, 17 (11 females, 6 males; mean age, 13.32 ± 4.24 years) (2.6%) of them were found to have sacroiliitis. Familial Mediterranean fever diagnosis was done prior to sacroiliitis diagnosis in 11 patients (65%) and concurrently or afterward in 6 patients (35%). Ten patients had isolated sacroiliitis, and 7 had associated diseases (5 enthesitis-related arthritis, 1 psoriatic arthritis, and 1 ulcerative colitis). Arthritis (59%), arthralgia (77%), leg pain (71%), heel pain (41%), and enthesitis (29%) were common complaints. Sacroiliac tenderness was detected in 77%, and M694V mutation in almost 90% of the patients. All patients received colchicine therapy. Additionally, 14 of them were treated with nonsteroidal anti-inflammatory drugs, 10 were on sulfasalazine treatment, and 7 of them were on biological agents.

Conclusions: Sacroiliitis can be seen in patients with FMF during childhood, and M694V mutation seems to be a susceptibility factor for its development. Inflammatory low-back pain and leg and heel pain could suggest sacroiliitis.
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http://dx.doi.org/10.1097/RHU.0000000000000770DOI Listing
March 2019

Outcome of renal transplantation in small infants: a match-controlled analysis.

Pediatr Nephrol 2018 06 13;33(6):1057-1068. Epub 2018 Mar 13.

Department of Paediatrics I, University Children's Hospital, Heidelberg, Germany.

Background: Infants with a body weight of less than 10 kg are often not considered to be suitable candidates for renal transplantation (RTx). The objective of this study was to evaluate this arbitrary weight threshold for pediatric RTx.

Methods: We conducted a multicenter, retrospective, match-controlled cohort study on infants weighing less than 10 kg at time of engrafting (low-weight group [LWG], n = 38) compared to a matched control group (n = 76) with a body weight of 10-15 kg, using data from the first 2 years post-transplant derived from the CERTAIN Registry.

Results: Patient survival was 97 and 100% in the LWG and control groups, respectively (P = 0.33), and death-censored graft survival was 100 and 95% in the LWG and control groups, respectively (P = 0.30). Estimated glomerular filtration rate at 2 years post-transplant was excellent and comparable between the groups (LWG 77.6 ± 34.9 mL/min/1.73 m; control 74.8 ± 29.1 mL/min/1.73 m; P = 0.68). The overall incidences of surgery-related complications (LWG 11%, control 23%; P = 0.12) and medical outcome measures (LWG 23%, control 36%, P = 0.17) were not significantly different between the groups. The medical outcome measures included transplant-related viral diseases (LWG 10%, control 21%; P = 0.20), acute rejection episodes (LWG 14%, control 29%; P = 0.092), malignancies (LWG 3%, control 0%; P = 0.33) and arterial hypertension (LWG 73%, control 67%; P = 0.57).

Conclusions: These data suggest that RTx in low-weight children is a feasible option, at least in selected centers with appropriate surgical and medical expertise.
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http://dx.doi.org/10.1007/s00467-018-3895-5DOI Listing
June 2018

Atypical Hemolytic Uremic Syndrome in Children Aged <2 Years.

Nephron 2018 13;139(3):211-218. Epub 2018 Mar 13.

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

Background: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS.

Materials And Methods: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry.

Results: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study. Age at disease onset was ≤1 year in 29 of the patients. In all, 21 (40%) of the patients developed neurological symptoms. Disease-causing mutations were noted in 14 (36%) of the 39 patients in which genetic analysis was performed. Plasma therapy was performed in 42 (79%) patients; eculizumab therapy was administered to treat the first episode of aHUS in 33 (62%) patients and in 5 patients as the first- line therapy. In total, 38 (72%) patients received renal replacement therapy (RRT), 3 (6%) died due to acute illness, and 4 (8%) were discharged from hospital with RRT. Follow-up visit data were available for 46 patients and the median duration was 23 months (range 3-129 months). End-stage renal disease developed only in 1 patient. Proteinuria and hypertension persisted in 17 (37%) and 20 patients (44%) respectively. Eculizumab treatment was continued in 25 of the 39 patients during the follow-up period.

Conclusion: One-third of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities.
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http://dx.doi.org/10.1159/000487609DOI Listing
September 2019

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

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

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

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

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

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

Conclusions: Vitamin D supplementation normalized Klotho and sclerostin in children with mild to moderate CKD but further increased FGF23 in advanced CKD.
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http://dx.doi.org/10.1093/ndt/gfy012DOI Listing
December 2018

Fatigue in pediatric patients with familial Mediterranean fever.

Mod Rheumatol 2018 Nov 2;28(6):1016-1020. Epub 2018 Feb 2.

a Department of Pediatrics, Division of Pediatric Rheumatology , Ankara University School of Medicine , Ankara , Turkey.

Objectives: Familial Mediterranean fever (FMF) is characterized by recurrent, self-limited attacks of fever with serositis involving the peritoneum, pleura and joints. Fatigue is a common problem in many pediatric rheumatic diseases; however, has not been evaluated systematically in FMF patients. Accordingly, the aim of this study was to evaluate fatigue and its possible allied factors in patients with FMF.

Methods: Patients with FMF, aged between 10 and 21 years, were assessed by completed validated fatigue questionnaire (Checklist Individual Strength-20). As a control group, patients with chronic rheumatic diseases and healthy children without any chronic disease were included.

Results: The study group comprised 111 patients with FMF, 54 with other chronic rheumatic diseases and 79 healthy subjects. While the CIS-20 total score and subscale scores (including subjective experience of fatigue) were similar between patients with FMF and those with other chronic rheumatic diseases (p > .05); both groups had significantly higher scores when compared with healthy subjects (p < .05). FMF patients with musculoskeletal complaints had significantly higher scores of subjective experiences of fatigue when compared to those without those complaints.

Conclusions: Fatigue is a common but unrecognized complaint in patients with FMF. Familial Mediterranean fever seems to be a chronic disease with inter attack ongoing complaints.
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http://dx.doi.org/10.1080/14397595.2018.1427459DOI Listing
November 2018

Proteinuria in pediatric renal transplant recipients.

Pediatr Transplant 2018 02 9;22(1). Epub 2017 Oct 9.

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

Proteinuria has been shown to be an important and potentially treatable risk factor for graft loss. The aim of this study was to evaluate prevalence, etiology, and outcome of proteinuria during the follow-up of children with renal transplantation. We retrospectively reviewed the files of renal transplanted children between 2006 and 2016 in our center. All patients were interpreted with respect to the demographic data and clinical and laboratory features including information about proteinuria. Chi-square test and Mann-Whitney U test were used for analysis. Fifty-two children were eligible for the study. Proteinuria was observed in 34 (65%) and nephrotic range proteinuria was detected in 5 (9.6%) patients. Etiology of proteinuria could be identified in 21 patients. Acute rejection and uncontrolled hypertension were the most frequent causes of proteinuria. Proteinuria had resolved during the follow-up in 59% of the patients. We found that children with and without proteinuria had similar glomerular filtration rate at the end of 50 months of follow-up period. Proteinuria seems to be a common complication in renal transplant recipients. Graft functions can be preserved by immediate evaluation of increasing proteinuria, and by fixing treatable causes rapidly and efficiently during the follow-up in majority of the patients.
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http://dx.doi.org/10.1111/petr.13068DOI Listing
February 2018

Hypertension and improved left ventricular mass index in children after renal transplantation.

Pediatr Transplant 2017 Dec 5;21(8). Epub 2017 Oct 5.

Department of Pediatric Cardiology, Medical School, Ankara University, Ankara, Turkey.

This study was conducted to evaluate the changes in BP and LVH after the transplantation and to evaluate the effect of BP changes in LVH. Forty-three pediatric renal transplant patients, with a mean age of 16.99 ± 3.88 years, were enrolled in this study. Twenty-three (53.5%) of the patients were male. Medical records for pretransplantation period (closest to the time of transplantation) and for post-transplantation period (9-12 months after transplantation) were reviewed. All the patients had BP measurements and echocardiographic evaluation in pre- and post-transplantation period. Hypertension was defined as an average systolic and/or diastolic BP that is ≥95th percentile for sex, age, and height. Although the number of patients with hypertension increased from 30 (69.76%) to 35 (81.4%), the number of patients with LVH decreased from 19 (44.1%) to 9 (20.9%) after the transplantation. Although the only significant difference in BP measurements was between the mean Z scores of 24 hour and nighttime mean DBP before and after the transplantation; the mean LVMI, and the prevalence of LVH was significantly lower after the transplantation. There was no significant correlation between the LVMI and the BP measurements. Even though hypertension may persist, there is significant improvement in LVH after renal transplantation.
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http://dx.doi.org/10.1111/petr.13066DOI Listing
December 2017

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

Pediatr Nephrol 2018 Jan 4;33(1):117-124. Epub 2017 Aug 4.

Department of Pediatric Nephrology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

Background: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data.

Methods: Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD).

Results: Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups.

Conclusions: This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.
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http://dx.doi.org/10.1007/s00467-017-3770-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5700229PMC
January 2018

Acute kidney injury in congenital cardiac surgery: Pediatric risk-injury-failure-loss-end-stage renal disease and Acute Kidney Injury Network.

Pediatr Int 2017 Dec;59(12):1252-1260

Pediatric Cardiology, Ankara University Faculty of Medicine, Ankara, Turkey.

Background: Acute kidney injury (AKI) is associated with an increased risk of mortality, especially in pediatric intensive care units. The aim of this study was to determine the risk factors of AKI in children undergoing cardiac surgery for congenital heart disease and to compare two different classification systems: pediatric risk-injury-failure-loss-end-stage renal disease (pRIFLE) and Acute Kidney Injury Network (AKIN).

Methods: We retrospectively analyzed 145 patients undergoing pediatric congenital heart surgery who were between 1 month and 18 years of years, and treated at a cardiovascular surgery department from January 2009 to October 2011.

Results: One hundred and thirty-seven patients (mean age, 36.6 ± 43.3 months) were enrolled: 84 (61.3%) developed AKI according to the pRIFLE criteria (25.5%, risk; 20.4%, injury; 15.3%, failure); and 65 patients (47.4%) developed AKI according to the AKIN criteria (15.3%, stage I; 18.2%, stage II; and 13.9%, stage III). Children younger than 11 months were more likely to develop AKI (P < 0.005). Longer cardiopulmonary bypass time was associated with an increased risk of AKI (P < 0.05). pRIFLE identified AKI more frequently than AKIN (P < 0.0005). pRIFLE may help in the early identification of patient at risk for AKI and seems to be more sensitive in pediatric patients (P < 0.05). Any degree of AKI in both classifications was associated with increased mortality (pRIFLE: OR, 15.1; AKIN: OR, 11.2; P = 0.007).

Conclusion: pRIFLE identified AKI more frequently than the AKIN criteria. pRIFLE identified patients at risk for AKI earlier, and was more sensitive in pediatric patients. Any degree of AKI in both classifications was associated with increased mortality. Both sets of criteria had the same association with mortality.
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http://dx.doi.org/10.1111/ped.13359DOI Listing
December 2017

A questionnaire survey of radiological diagnosis and management of renal dysplasia in children.

J Nephrol 2018 02 24;31(1):95-102. Epub 2017 Jun 24.

Department of Pediatric Nephrology, University Children's Hospital Marburg, Philipps-University Marburg, Marbug, Germany.

Background: The condition called renal dysplasia is considered to be a frequent cause of chronic kidney disease in children. Formally, it is defined by histological parameters. In current nephrology practice, however, the appearance of the kidneys on ultrasound scanning is often used as a basis for the diagnosis.

Methods: The European Society for Pediatric Nephrology Working Group on Congenital Anomalies of the Kidney and Urinary Tract hypothesized that the current diagnostic approach with regard to renal dysplasia was not homogeneous. Accordingly, we here report the results of a survey targeting pediatric nephrologists with 12 questions regarding their perceptions of the ultrasonographic characteristics of renal dysplasia and further tests that they might undertake.

Results: Of almost 1200 physicians who successfully received the invitation, 248 from 54 countries completed the survey. There was a notable lack of homogeneity regarding the ultrasonographic diagnosis of renal dysplasia and also of follow-up tests, including genetic testing and further radiology.

Conclusions: Based on the responses to this large survey, a picture emerges of nephrologists' current clinical practice with regard to renal dysplasia. The Working Group considers that these results serve as an important sounding board which can provide the basis for more definitive recommendations regarding the challenges to clinical diagnosis and diagnostic follow-up of this important condition.
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http://dx.doi.org/10.1007/s40620-017-0417-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5778166PMC
February 2018

Dyslipidemia after pediatric renal transplantation-The impact of immunosuppressive regimens.

Pediatr Transplant 2017 May 28;21(3). Epub 2017 Mar 28.

Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, Cologne, Germany.

Dyslipidemia contributes to cardiovascular morbidity and mortality in pediatric transplant recipients. Data on prevalence and risk factors in pediatric cohorts are, however, scarce. We therefore determined the prevalence of dyslipidemia in 386 pediatric renal transplant recipients enrolled in the CERTAIN registry. Data were obtained before and during the first year after RTx to analyze possible non-modifiable and modifiable risk factors. The prevalence of dyslipidemia was 95% before engraftment and 88% at 1 year post-transplant. Low estimated glomerular filtration rate at 1 year post-transplant was associated with elevated serum triglyceride levels. The use of TAC and of MPA was associated with significantly lower concentrations of all lipid parameters compared to regimens containing CsA and mTORi. Immunosuppressive regimens consisting of CsA, MPA, and steroids as well as of CsA, mTORi, and steroids were associated with a three- and 25-fold (P<.001) increased risk of having more than one pathologic lipid parameter as compared to the use of TAC, MPA, and steroids. Thus, amelioration of the cardiovascular risk profile after pediatric RTx may be attained by adaption of the immunosuppressive regimen according to the individual risk profile.
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http://dx.doi.org/10.1111/petr.12914DOI Listing
May 2017

Familial Mediterranean fever gene mutation frequencies in a sample Turkish population.

Clin Exp Rheumatol 2016 Sep-Oct;34(6 Suppl 102):97-100. Epub 2016 Oct 17.

Department of Paediatric Nephrology and Rheumatology, School of Medicine, Ankara University, Ankara, Turkey.

Objectives: Our knowledge about the frequencies of mutations in the Turkish population is based on the studies on the affected patients and hospital-based control groups. We aimed to determine the frequencies of MEFV gene mutations in a population-based field study in Turkey.

Methods: Turkish citizens aged between 5 and 65 years were included in the study. Cities from seven regions of Turkey were studied. Blood samples were obtained from individuals who gave permission for laboratory experiments, and they were analysed for 10 MEFV gene mutations.

Results: Among 500 participants, MEFV mutations were found in 74 (14.8%). Sixty four (12.8%), 7 (1.4%), and 3 (0.6%) participants were heterozygous, compound heterozygous, and homozygous, respectively. Among inhabitants with heterozygous mutations, the most common heterozygous mutations were E148Q/- and M694V/-. Sixteen participants were found to be heterozygous for M694V, 2 were compound heterozygous for M694V/E148Q, and one was homozygous for M694V/M694V mutation; in total, the frequency of M694V allele was 4% (n=20). Twenty-three (4.6%) individuals were heterozygous for common mutations (M694V, M680I, V726A). Total allelic frequency was 8.4%.

Conclusions: Our study, which describes the MEFV mutational spectrum and distribution in a healthy Turkish population, found a carrier rate that is much higher than expected.
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February 2017

Childhood polyarteritis nodosa: diagnosis with non-invasive imaging techniques.

Clin Rheumatol 2017 Jan 5;36(1):165-171. Epub 2016 Oct 5.

Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, Ankara, Turkey.

There is limited number of publications about the use of non-invasive imaging modalities in the diagnosis of childhood polyarteritis nodosa (cPAN). The aim of this study was to present the clinical and imaging findings of the patients with cPAN who were diagnosed with non-invasive imaging techniques. Files of patients who had been diagnosed as cPAN in our department from 2005 to 2015 were reviewed, retrospectively. Demographic, clinical, laboratory, and imaging findings of the patients were evaluated. Nine patients (8M, 1F; age at disease onset: 12.5 years (7-16)) had been diagnosed as cPAN in our clinic with non-invasive imaging techniques within the last 10 years. Abdominal pain, fever, fatigue, and myalgia were the most frequent complaints. Doppler ultrasonography (US) was used in the diagnosis of seven patients and computed tomography (CT) angiography was done in four patients. Duration between admission to our center and diagnosis was median of 5 days (8 h-10 days), including four patients who were diagnosed within 24 h of admission. Approximately 80 % of our patients with cPAN had MEFV gene mutations and 90 % had elevated anti-streptolysin O levels. All of them had the involvement of the gastrointestinal tract. Hepatic and cystic arterial involvements were detected in seven and six patients, respectively. This report included the largest cPAN series that were diagnosed with non-invasive imaging modalities. We suggest that non-invasive modalities, especially Doppler US, should be considered in first line approach in the diagnosis of these patients, particularly in children.
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http://dx.doi.org/10.1007/s10067-016-3440-5DOI Listing
January 2017

Digestion of Chromatin in Apoptotic Cell Microparticles Prevents Autoimmunity.

Cell 2016 Jun 9;166(1):88-101. Epub 2016 Jun 9.

Department of Pathology, New York University School of Medicine, New York, NY 10016, USA; Department of Microbiology and Immunology, Columbia University Medical Center, New York, NY 10032, USA; Department of Medicine, New York University School of Medicine, New York, NY 10016, USA. Electronic address:

Antibodies to DNA and chromatin drive autoimmunity in systemic lupus erythematosus (SLE). Null mutations and hypomorphic variants of the secreted deoxyribonuclease DNASE1L3 are linked to familial and sporadic SLE, respectively. We report that DNASE1L3-deficient mice rapidly develop autoantibodies to DNA and chromatin, followed by an SLE-like disease. Circulating DNASE1L3 is produced by dendritic cells and macrophages, and its levels inversely correlate with anti-DNA antibody response. DNASE1L3 is uniquely capable of digesting chromatin in microparticles released from apoptotic cells. Accordingly, DNASE1L3-deficient mice and human patients have elevated DNA levels in plasma, particularly in circulating microparticles. Murine and human autoantibody clones and serum antibodies from human SLE patients bind to DNASE1L3-sensitive chromatin on the surface of microparticles. Thus, extracellular microparticle-associated chromatin is a potential self-antigen normally digested by circulating DNASE1L3. The loss of this tolerance mechanism can contribute to SLE, and its restoration may represent a therapeutic opportunity in the disease.
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http://dx.doi.org/10.1016/j.cell.2016.05.034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5030815PMC
June 2016

Anti-VEGF-related thrombotic microangiopathy in a child presenting with nephrotic syndrome.

Pediatr Nephrol 2016 Jun 29;31(6):1029-32. Epub 2016 Feb 29.

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

Background: Targeting the vascular endothelial growth factor (VEGF) signaling pathway has become an important approach to current cancer therapy. Anti-VEGF therapy-related renal adverse effects may present as hypertension, non-nephrotic proteinuria, and rarely as nephrotic syndrome (NS) and acute kidney injury.

Case-diagnosis/treatment: In this report, we present a 15-year-old boy who had developed nephrotic syndrome and thrombotic microangiopathy 26 months after administration of anti-VEGF therapy. Treatment was discontinued and nephrotic syndrome remitted spontaneously within 3 months.

Conclusions: Nephrologists should be aware of the side effects of anti-VEGF therapy. Early diagnosis and prompt management with withdrawal of the agents will result in spontaneous remission.
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http://dx.doi.org/10.1007/s00467-016-3355-zDOI Listing
June 2016

Vesicoureteral Reflux and Renal Scarring Risk in Children after the First Febrile Urinary Tract Infection.

Nephron 2016 23;132(3):175-80. Epub 2016 Feb 23.

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

Background/aims: There are many controversies regarding the best approach for evaluating children after their first febrile urinary tract infection (UTI). The aim of this study was to define the clinical, laboratory, and radiological features of patients with their first febrile UTI and to investigate the factors that might predict the presence of vesicoureteral reflux (VUR) and renal scarring.

Methods: The files of patients who were followed due to their first febrile UTI between 2008 and 2013 were retrospectively reviewed (n = 300). Patients were divided into groups based on their age, the resistance state of microorganisms, the presence of VUR, and scarring on Tc99m dimercaptosuccinic acid scintigraphy. The chi-square test and Mann-Whitney U test were used for analysis.

Results: The median age at the first febrile UTI was 11 months and girls constituted 77% of the patient population. VUR and renal scarring were detected in 30.9 and 19.4% of the patients, respectively. C-reactive protein levels and the presence of renal scarring were significantly higher in patients with VUR (p < 0.05). Abnormal ultrasonography findings, VUR and recurrent UTIs were significantly higher in patients with renal scars (p < 0.001). In multivariate analysis, we did not detect any factor that might predict the presence of VUR and renal scarring.

Conclusion: A majority of children had their first febrile UTI at a young age. Although we could not find any factor that might predict the VUR and scar risk in patients with their first febrile UTI, an abnormal renal scan at 6 months after infection was closely related with the presence of VUR and recurrent UTIs.
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http://dx.doi.org/10.1159/000443536DOI Listing
December 2016

Late-onset disease is associated with a mild phenotype in children with familial Mediterranean fever.

Clin Rheumatol 2016 Jul 3;35(7):1837-40. Epub 2016 Feb 3.

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

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that patients with early disease onset during childhood period had more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had late-onset disease during childhood period and to compare them to those with earlier onset patients. Files of patients who had been seen in our department between January 2013 and January 2014 were retrospectively evaluated. Patients were divided into two groups according to age of disease onset (group I, ≤8 years; group II, >8 years), and clinical findings were compared between the two groups. The study group comprised 317 FMF patients. There were 267 patients in group I and 50 patients in Group II. Median attack frequency was 24/year in group I and 12/year in group II (p < 0.05). Fever and M694V homozygosity were less frequently detected in group II (p = 0.003 and p = 0.022). Median delay in diagnosis was 24 months in group I and 12 months in group II (p = 0.002). Disease severity scores and final colchicine dosages were lower in group II (p < 0.001 and p = 0.003). Only a small number of FMF patients had disease onset at older ages in childhood period. It seems that FMF patients with late-onset disease have milder illness. However, more readily expression of their clinical findings in older ages yields earlier diagnosis in this group.
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http://dx.doi.org/10.1007/s10067-016-3196-yDOI Listing
July 2016

Cytomegalovirus Infection in Pediatric Renal Transplantation and the Impact of Chemoprophylaxis With (Val-)Ganciclovir.

Transplantation 2016 Apr;100(4):862-70

1 Department of Pediatrics I, University Children's Hospital, Heidelberg, Germany. 2 Hanover Medical School, Hanover, Germany. 3 IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy. 4 Faculty of Medicine, Department of Pediatric Nephrology, Hacettepe University, Ankara, Turkey. 5 University Children's Hospital, Department of General Pediatrics, Pediatric Nephrology, Münster, Germany. 6 Olga Children's Hospital, Clinic of Stuttgart, Stuttgart, Germany. 7 Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Center, Manchester, United Kingdom. 8 Baskent University, Adana Teaching and Research Center, Department of Pediatric Nephrology, Adana, Turkey. 9 Great Ormond Street Hospital, Great Ormond Street, London, United Kingdom. 10 Ankara University Faculty of Medicine, Dikimevi, Ankara, Turkey. 11 University Children's Hospital, Hamburg, Germany. 12 University Children's Hospital, Tübingen, Germany. 13 University Children's Hospital, Freiburg, Germany. 14 Children's Hospital, Memmingen, Germany. 15 Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany.

Background: Cytomegalovirus (CMV) replication and disease, with its associated morbidity and poor transplant outcome, represents a serious threat to transplant recipients. The pediatric kidney transplant population is at a particularly increased risk of CMV infection.

Methods: We therefore analyzed CMV epidemiology in a large cohort of pediatric renal transplant recipients (n = 242) and assessed the impact of antiviral chemoprophylaxis with valganciclovir (VGCV) or ganciclovir (GCV) on CMV replication and morbidity.

Results: While antiviral chemoprophylaxis with VGCV or GCV in patients with a high (D+/R-) or intermediate (D+/R+) CMV risk (n = 82) compared to preemptive therapy (n = 47) had no significant effect on the incidence of CMV syndrome or tissue-invasive disease, chemoprophylaxis was associated with a better preservation of transplant function at 3 years posttransplant (loss of estimated glomerular filtration rate in the chemoprophylaxis cohort, 16.0 ± 3.4 vs. 30.1 ± 4.7 mL/min per 1.73 m(2) in the preemptive therapy cohort, P < 0.05).CMV replication was associated with a more pronounced decline of graft function (difference in estimated glomerular filtration rate of 9.6 mL/min per 1.73 m(2) at 3 years) compared to patients without CMV replication. However, patients undergoing VGCV or GCV chemoprophylaxis had more leukocytopenia.

Conclusion: Antiviral chemoprophylaxis with VGCV or GCV in recipients with a high or moderate CMV risk is associated with a better preservation of transplant function. Hence, the prevention of CMV replication in this patient population has the potential to improve transplant outcome.
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http://dx.doi.org/10.1097/TP.0000000000000888DOI Listing
April 2016

Bone marrow aspiration complications: Iliopsoas abscess and sacroiliac osteomyelitis.

Turk J Pediatr 2016 ;58(5):562-565

Divisions of Pediatric Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.

After bone marrow aspiration procedure; some complications like pain and bleeding at the puncture site may be expected but some serious complications like osteomyelitis and soft tissue infections may also rarely occur. In this case we present a boy with recurrent fever. During etiologic investigation, familial Mediterranean fever (FMF) gene M694V mutation was +/+. Patient was treated with oral colchicine however fever persisted. The patient was considered as colchicine resistant FMF and steroid treatment was planned. Bone marrow aspiration procedure was executed to rule out malignancy. Three months after bone marrow aspiration, he was readmitted with complaint of left pelvic pain, difficulty in walking without support and standing on his left foot. Radiological imaging demonstrated left iliopsoas abscess and left sacroiliac osteomyelitis. Patient was successfully treated with intravenous ampicillin-sulbactam and clindamycin treatment for 6 weeks. Then oral amoxicillin-clavulanic acid treatment was continued for 2 weeks. Patient was discharged without any surgical procedure. On 1-year follow-up he could walk without any support.
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http://dx.doi.org/10.24953/turkjped.2016.05.019DOI Listing
September 2017