Yasar Kandur

Hypertension in Pediatric Patients With Renal Scarring in Association With Vesicoureteral Reflux

OBJECTIVE To examine the reflux nephropathy rate and severity as well as the hypertension rate in pediatric patients with vesicoureteral reflux (VUR). METHODS The study included 240 patients with VUR. Renal scarring (RS) was demonstrated by renal parenchymal examination using technetium-99m-labeled dimercaptosuccinic acid (99mTc-DMSA) scintigraphy. Office measurements of arterial blood pressure and ambulatory blood pressure monitoring (ABPM) of VUR patients were done during the follow-up period. RESULTS Follow-up was a mean duration of 24 months. Rates of RS and hypertension increased parallel to increases in the degree of VUR. A gradual elevation in hypertension rates was evident during the follow-up period. All patients with hypertension had RS. Severe RS in 56 patients was associated with increasing blood pressure readings by 24-hour ABPM or office measurements in 19 patients (33.9%). ABPM measurements enabled us to detect additional patients compared with office measurements alone. CONCLUSION Hypertension is a serious complication in children with reflux nephropathy and is associated with the severity of RS and VUR grade. ABPM seems to be superior over office measurements of blood pressure in identifying patients with hypertension.

How often do we face side effects of sirolimus in pediatric renal transplantation

We analyzed 25 pediatric renal transplantation patients on sirolimus (SRL) therapy to assess changes in serum creatinine, glomerular filtration rate, electrolytes, triglycerides, cholesterol, and side effects. Mean time to initiate SRL therapy was 3.2 years. The serum creatinine levels of patients on SRL treatment at 1, 6, 12, and 24 months were 1.67 ± 1.15 mg/dL, 1.18 ± 0.52 mg/dL, 1.24 ± 0.32 mg/dL, 1.15 ± 0.31 mg/dL, and 1.17 ± 0.12 mg/dL, respectively. We observed proteinuria in 3, hyperlipidemia in 5, and anemia in 2 patients, but none had the treatment discontinued. We diagnosed interstitial pneumonia in (n = 1), nasal acneiform lesions (n = 1), and lower extremity edema (n = 1). Hypokalemia developed in 1 subject with high blood SRL levels. In the follow-up period there was no case of acute rejection episode during SRL therapy.

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever – a randomized controlled noninferiority trial

BackgroundIn this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF).MethodsIn this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement.ResultsA total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once- and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group.ConclusionsUsing colchicine with either a once- or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage.Trial Registration IDClinicalTrials.gov identifier NCT02602028. Registered 5 November 2015.

Evaluation of urinary KIM-1, NGAL, and IL-18 levels in determining early renal injury in pediatric cases with hypercalciuria and/or renal calculi.

AIM One has to measure urinary enzymes and proteins to determine renal dysfunction and tubular injury in earlier stage during the course of disease. The aim of this study was to investigate the role of urinary NGAL (neutrophil gelatinase-associated lipocalin), IL-18 (Interleukin 18), and KIM-1 (kidney injury molecule-1) levels in determining early renal injury in pediatric patients with hypercalciuria (HC) and/or nephrolithiasis (NL) and to compare the levels of these markers between the sick and healthy subjects. MATERIAL AND METHODS Urinary NGAL, KIM- 1, and IL- 18 levels were measured in 40 pediatric patients diagnosed with NL, 23 patients with HC, and 20 healthy controls. RESULTS A significant difference was found between patient groups (NL and HC) and healthy children with respect to urinary NGAL/cr ratio (p < 0.001). There were no significant differences between patient and control groups with respect to urinary IL18/cr and KIM1/cr ratios. CONCLUSIONS We found that urinary NGAL is a useful marker for determining renal tubular damage in NL and HC. To our knowledge, this is the first study to report significantly increased urinary NGAL levels in NL and HC.

Posterior Reversible Encephalopathy Syndrome in Henoch-Schonlein Purpura and Hemolytic Uremic Syndrome

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome, composed of symptoms such as headache, seizures, visual disturbances, lethargy, confusion, stupor, focal neurologic findings and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres. PRES is associated with significant morbidity and mortality if it is not expeditiously recognized. Magnetic resonance image (MRI) represents the most sensitive imaging technique for recognizing PRES. PRES has been seen in various clinical settings including renal disorders such as acute glomerulonephritis, lupus nephritis, nephrotic syndrome, and drug usage such as calcineurin inhibitors. We aimed to present two study cases for such clinical setting. In this report, we present two patients with PRES in whom the primary diagnosis was hemolytic uremic syndrome (HUS) and Henoch-Schonlein purpura (HSP). Both of them were treated with anticonvulsant and proper antihypertensive drugs. A repeated MRI scan of the head, an ophthalmologic assessment, and a follow-up electroencephalogram produced normal results with no sequelae. Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate the appropriate treatment, so that intensive treatment should be performed as soon as possible to avoid neurological sequelae.

The evaluation of bone metabolism in children with renal transplantation

This study aims to evaluate BMD and bone biomarkers and to investigate the effects of immunosuppressives on bone disease after RTx. Thirty‐three RTR aged 16.7 ± 3.7 yr and healthy controls (n = 32) were enrolled. There was no difference between pre‐RTx BMD and BMD at the time of study (45.9 ± 30.9 months after RTx), while both values were lower than controls (p < 0.01 and p < 0.05, respectively). Worst BMD scores were obtained at sixth month after RTx (−0.2 ± 0.9) and best at fourth year (1.4 ± 1.3). 25‐hydroxy‐(OH) vitamin D and OPG were higher in RTR (p < 0.001). BMD z scores negatively correlated with OPG and cumulative CS doses at the time of study (r = −0.344, p < 0.05 and r = −0.371, p < 0.05, respectively). Regression analysis revealed OPG as the only predictor of BMD (β −0.78, 95% CI −0.004 to −0.013, p < 0.001). The increase in OPG, a significant predictor of BMD, could either be secondary to graft dysfunction or for protection against bone loss. CS doses should be minimized to avoid their untoward effects on bone metabolism.

Neonatal hyperbilirubinemia in a Turkish cohort: association of vitamin B12.

Background Deficiency of vitamin B12 (VitB12) causes failure of erytrocyte maturation leading to cell lysis. Red blood cell lysis causes excess heme production that ends with hyperbilirubinemia. In this study, we aimed to evaluate the role of VitB12 in neonatal hyperbilirubinemia (NNH) with prolonged jaundice and to compare patients with control group who did not develop hyperbilirubinemia. Methods A total of 20 patients (M/F = 13/7) with jaundice and 20 healthy controls (M/F = 11/9) were included in the study. Results The mean indirect bilirubin level of patient group was 9.91 ± 1.90 mg/dL (6.71 - 15.2 mg/dL) and control group was 3.18 ± 1.24 mg/dL (1.16 - 4.96 mg/dL). The mean VitB12 level of patient group was 119.9 ± 43.9 ng/L (42.35 - 178 ng/L) and the control group was 286.17 ± 97.43 ng/L (207.90 - 624.10 ng/L). There was a statistically significant difference in terms of VitB12 level (< 0.001) between the study groups. Conclusion To our knowledge, this study is the first study showing that low VitB12 level has been observed as a risk factor in NNH for the first time in the literature. We suggest that prophylactic use of VitB12 by pregnant women so will greatly benefit to prevent VitB12 deficiency and its complications in the first years of life such as NNH.

Rhabdomyolysis and acute kidney injury in two children: Questions

A 15-year-old male presented with respiratory distress, dark urine, and an inability to walk following tiredness and widespread muscle pain after playing football. History revealed that the patient occasionally had weakness after exercise and infections, and was hospitalized for widespread muscle pain and difficulty in breathing 3 years previously. In addition, his parents were second-degree relatives. On physical examination, the patient’s general well-being was moderate, he was conscious, but restless and he looked pale. His blood pressure was 160/89 mmHg. Muscle strength in the upper and lower extremities was 2/5. Urine output was 1.5 ml/kg/h and urine color was dark brown. The urine examination by dipstick showed 3+ blood in the urine, but microscopic examination did not show erythrocytes. Whole blood count showed Hb 9.5 g/dl, white cell count 22,600/mm, and platelet count 283,000/mm. Blood chemistry results were as follows: blood glucose 86 mg/dl, BUN 16 mg/dl, creatinine 1.2 mg/dl, creatinine phosphokinase (CPK) 2,458 U/l (normal range: 10–145 U/l), AST 898 U/l, ALT 181 U/l, lactate dehydrogenase (LDH) 2,570 U/l, uric acid 10 mg/dl, Na 139 mg/dl, K 4.2 mg/dl, Cl 100 mg/dl, phosphate 3.9 mg/dl, calcium 8.8 mg/dl. He had mild metabolic acidosis (PH: 7.35 and HCO3: 19 BE: –4.0). Thyroid function tests were normal (FreeT4, FreeT3, TSH). Telecardiography, electrocardiography, and echocardiography were normal. There was increased echogenicity in the parenchyma of both kidneys on renal ultrasonography. During follow-up, laboratory analysis showed deterioration: BUN 85 mg/dl, creatinine 4.8 mg/dl, uric acid 9.6 mg/dl, urinary output 0.3 cc/kg/h.

Therapeutic Plasma Exchange in Pediatric Patients with Nephrologic Diseases: Results from a Single Center

OBJECTIVE: Therapeutic plasma exchange (TPE) has been used in many diseases as primary or adjunctive therapy. We present our TPE experience in a pediatric nephrology practice setting. MATERIAL and METHODS: We retrospectively evaluated the indications and outcomes of TPE performed between 2008-2013 on the basis of the 2013 American Society for Apheresis Guidelines. RESULTS: One hundred and sixteen TPEs were performed in 15 patients (6 male / 9 female, mean age 12.9±3.5 years). The indications were hemolytic uremic syndrome (HUS) in 7 (four atypical) patients, pre-transplant TPE in 3 patients with focal segmental glomerulosclerosis (FSGS), treatment-resistant membranoproliferative glomerulonephritis (MPGN) in 1 patient, antibody mediated rejection (AMR) in 3 patients, and thrombotic microangiopathy (TMA) in 1 renal transplantation patient. Six months after TPE, hypertension persisted in two of seven and proteinuria in three of seven HUS patients, although all HUS patients had normal creatinine levels. Similarly, serum creatinine and urinary protein excretion were within the normal range in all FSGS patients and in one patient with AMR. Thrombocytopenia and anemia resolved and the blood creatinine level decreased in a patient with TMA. CONCLUSION: Although adherence to adult TPE guideline indications is around 50%, treatment results of TPE are satisfactory in 2/3 of our pediatric nephrology patients. Pediatric TPE Guidelines based on pediatric evidence-based data will help achieve better clinical outcomes in children. KEy wORDS: Plasmapheresis, Nephrology, Pediatric

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (sapho) syndrome with subcutaneous methotrexate: a case report

Akçaboy M, Bakkaloğlu-Ezgü SA, Büyükkaragöz B, Isıyel E, Kandur Y, Hasanoğlu E, Buyan N. Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report. Turk J Pediatr 2017; 59: 184-188. SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is defined as a syndrome that is related to various osteoarticular manifestations and chronic dermatological conditions especially severe acne. SAPHO syndrome is a rare and unusual clinical entity in childhood and treatment choices are variable. We report an 11-year-old girl who suffered from SAPHO syndrome and successfully treated with subcutaneous methotrexate. We report our case in order to take attention to this rare clinical condition in evaluating patients and also to point out that treatment options beyond biologic agents should be the first line treatment in childhood.