H. Alpay - Academia.edu (original) (raw)

Papers by H. Alpay

Research paper thumbnail of Rituximab-associated hypogammaglobulinemia: Incidence, predictors and outcomes in patients with multi-system autoimmune disease

Journal of autoimmunity, Jan 31, 2014

Rituximab is a B cell depleting monoclonal antibody used to treat lymphoma and autoimmune disease... more Rituximab is a B cell depleting monoclonal antibody used to treat lymphoma and autoimmune disease. Hypogammaglobulinemia has occurred after rituximab for lymphoma and rheumatoid arthritis but data are scarce for other autoimmune indications. This study describes the incidence and severity of hypogammaglobulinemia in patients receiving rituximab for small vessel vasculitis and other multi-system autoimmune diseases. Predictors for and clinical outcomes of hypogammaglobulinemia were explored. We conducted a retrospective study in a tertiary referral specialist clinic. The severity of hypogammaglobulinemia was categorized by the nadir serum IgG concentration measured during clinical care. We identified 288 patients who received rituximab; 243 were eligible for inclusion with median follow up of 42 months. 26% were IgG hypogammaglobulinemic at the time that rituximab was initiated and 56% had IgG hypogammaglobulinemia during follow-up (5-6.9 g/L in 30%, 3-4.9 g/L in 22% and <3 g/L in...

Research paper thumbnail of Epidermolysis Bullosa with Focal Glomerulosclerosis: A Case Report

Research paper thumbnail of Positional installation of contrast (PIC) and Redo-PIC cystography for diagnosis of occult vesicoureteral reflux

The Turkish journal of pediatrics, 2018

Karadeniz-Cerit K, Thomas DT, Ergun R, Yıldız N, Alpay H, İnanır S, Dağlı ET, Tuğtepe H. Position... more Karadeniz-Cerit K, Thomas DT, Ergun R, Yıldız N, Alpay H, İnanır S, Dağlı ET, Tuğtepe H. Positional installation of contrast (PIC) and Redo-PIC cystography for diagnosis of occult vesicoureteral reflux. Turk J Pediatr 2018; 60: 180-187. To evaluate the value of Positional Installation of Contrast (PIC) and Redo-PIC cystography in patients with febrile recurrent urinary tract infection (f-UTI) where voiding cystourethrogram (VCUG) was negative. Patients with recurrent f-UTI with no reflux on VCUG referred to the outpatient clinic of Pediatric Urology, between June 2011 and June 2016 were included in the study. A PIC cystography was performed in all patients. When reflux was found, subureteric injection was performed. Urinary cultures were used for follow-up. Patients that continued having f-UTI, received redo-PIC cystography. PIC cystography was performed on 42 patients. The average age of patients was 8.0±3.6 years. Vesicoureteral reflux (VUR) was detected in 41 patients. Average fo...

Research paper thumbnail of The Timing of Primary Neurosurgical Repair Significantly Affects Neurogenic Bladder Prognosis in Children With Myelomeningocele

Journal of Urology, 2006

The correlation between timing of the primary neurosurgical repair and urological prognosis in in... more The correlation between timing of the primary neurosurgical repair and urological prognosis in infants with open spinal dysraphism remains unknown. We investigated the impact of primary repair timing on neurogenic bladder prognosis in children with myelomeningocele. Materials and Methods: We retrospectively reviewed the records of 401 children with myelomeningocele followed at our multidisciplinary clinic between 1996 and 2005. Among these patients 129 were included in the study based on the availability of urological followup data at age 3 years, which was chosen as an ideal point for the standardization of urological findings. Children were assigned to 1 of 2 groups-those who underwent primary neurosurgical repair within 72 hours of delivery (group 1) and those undergoing repair after 72 hours (group 2). We compared the incidence of febrile urinary tract infections, hydronephrosis, vesicoureteral reflux and secondary tethering of the spinal cord at age 3 years, as well as cystometric bladder capacity and detrusor leak point pressure between the 2 groups. Results: Urological followup data at age 3 years revealed significantly increased incidence of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering of the spinal cord in children in group 2 compared to group 1 (chi-square, p Յ0.05 for all comparisons). Urodynamic assessment also showed a significantly lower bladder capacity and a substantially higher detrusor leak point pressure in children who received primary repair at later than 72 hours (t test, p Ͻ0.05). A subgroup analysis of group 1 comparing children who underwent repair before and after 24 hours following delivery failed to demonstrate any significant difference in terms of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering (chi-square, p Ͼ0.05 for all comparisons), whereas mean bladder capacity was significantly higher (t test, p Ͻ0.05) and mean detrusor leak point pressure was lower (t test, p ϭ 0.08) in children undergoing repair within 24 hours of delivery. Conclusions: The timing of primary neurosurgical repair has a significant impact on neurogenic bladder prognosis in children with myelomeningocele. Closure of the spinal lesion on the first day of life seems to provide the best chance for favorable lower urinary tract function.

Research paper thumbnail of Autosomal recessive polycystic kidney disease in 115 children: clinical presentation, course and influence of gender

Research paper thumbnail of Are myelodisplastic children receiving sufficient health care in Turkey? An analysis of the problems in primary management and their impact on neurourological outcome

Purpose: We aimed to investigate the problems in the primary care of children with spina bifida a... more Purpose: We aimed to investigate the problems in the primary care of children with spina bifida and to analyze their impact on neuro-urological outcome in Turkey. Mothers' information about the disease, difficulties in getting medical services, availability and efficacy of prenatal ultrasound and folic acid prevention was also assessed. Patients: A retrospective review of 476 children registered in the pediatric urology section of our institutional multidisciplinary spina bifida clinic between 1996 and 2005 was made. All children were assessed for the time of primary repair, time to first neuro-urological visit, and compliance to follow-up. A phone interview was made with 166 mothers to obtain data regarding their educational status, supplementary folic acid intake before/during gestation, compliance to obstetric follow-up, prenatal diagnosis and counseling, information about the importance of neuro-urological surveillance, and difficulties in neuro-urological follow-up. Results...

Research paper thumbnail of Microalbumin excretion and outcome in children with multicystic dysplastic kidney

Turkish Journal of Medical Sciences

To present the long-term follow-up results of children with multicystic dysplastic kidney (MCDK) ... more To present the long-term follow-up results of children with multicystic dysplastic kidney (MCDK) and urinary microalbumin excretion levels in order to evaluate whether there is an increased risk of renal damage or not. Materials and methods: Th irty-three children with the diagnosis of MCDK who had been followed up by the nephrology outpatient clinic between 2002 and 2009 were invited to participate in the study. Twenty-six healthy children were investigated as a control group for microalbumin/creatinine ratio (μg/g creatinine). Th e mean age at diagnosis, the duration of follow-up, accompanying urinary tract abnormalities, attacks of urinary tract infection (UTI), contralateral kidney size, and urinary microalbumin levels were investigated. Results: Th e mean age of the patients with MCDK and the mean duration of follow-up were 6.5 ± 3.9 years and 35 months (range 2-96) months, respectively. Th e most common urinary tract abnormality was vesicoureteral refl ux (VUR), with a rate of 34%. Th irty-nine percent of the children experienced UTI during follow-up. Th e compensatory renal hypertrophy of the contralateral kidney was 24% at 6 months and 68% at 12 months. Sixteen patients (59%) had an increased microalbumin/creatinine ratio (>30 μg/mg creatinine). Microalbumin/creatinine ratio was higher in patients with MCDK than it was in the controls (P = 0.001). Conclusion: Microalbuminuria and VUR are not rare in children with MCDK. Systematic follow-up of these patients is recommended to identify those at risk of contralateral renal damage.

Research paper thumbnail of Clinical outcome of patients with antenatal diagnosis of urinary anomalies

Research paper thumbnail of Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism

Marmara Medical Journal, 2017

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerinin aldosterona duyarsızlığı ile karakteri... more Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerinin aldosterona duyarsızlığı ile karakterize, hiperkalemi, hiponatremi ve metabolik asidoz ile kendini gösteren nadir bir durumdur. Obstrüktif üropatisi olan bebeklerde ve piyelonefrit sırasında görülebilir. Gelişimi devam eden erken bebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisi ve enfeksiyonu sekonder PHA hastalarında tübüluslarda aldosteron direncine neden olan üç önemli faktördür. Genellikle hiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır, tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteron seviyelerinin saptanması ile tanı konur. Süt çoçuklarında biyokimyasal incelemede hiperpotasemi, hiponatremi ve metabolik asidoz saptandığında konjenital adrenal hiperplazi ayırıcı tanısında PHA göz önünde bulundurulmalı ve bu hastalarda üriner ultrasonografisi (USG) ve idrar analizi rutin olarak yapılmalıdır. Bu yazımızda sekonder PHA gelişen posterior üretral valv (PUV)'lı iki hasta sunuldu.

Research paper thumbnail of Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

The Journal of Pediatrics, 2018

for the ARegPKD consortium* Objective To identify prenatal, perinatal, and postnatal risk factors... more for the ARegPKD consortium* 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. AIC Akaike information criterion ARPKD Autosomal recessive polycystic kidney disease AUC Area under the curve CVVH Continuous venovenous hemofiltration PD Peritoneal dialysis Detailed affiliations available at www.jpeds.com (Appendix 1).

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

Balkan Medical Journal, 2017

Background: The causative agent spectrum and resistance patterns of urinary tract infections in c... more 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%), amoxicillinclavulanate (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.

Research paper thumbnail of Clinical and histopathological features of asymptomaticpersistent microscopic hematuria in children*

TURKISH JOURNAL OF MEDICAL SCIENCES, 2016

Background/aim: We analyzed the clinical and pathological features and prognosis of 106 children ... more 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.

Research paper thumbnail of Severe Neuropathic Ulcer and Fungal Dermatitis in a Patient with Spina Bifida

Research paper thumbnail of Use of Biphosphonates in Pediatric Nephrology Biphosphonates in Pediatric

Turkish Nephrology Dialysis Transplantation, 2011

Bifosfonatlar (BP) sentetik pirofosfat analoğu bileşiklerdir. Kemik yapımı sırasında osteoklast b... more Bifosfonatlar (BP) sentetik pirofosfat analoğu bileşiklerdir. Kemik yapımı sırasında osteoklast baskılanmasına yol açarak onların sayı ve işlevlerini azaltır, yaşam süresini kısaltır ve kemik rezorpsiyonunu önlerler. Erişkinlerde aşırı kemik rezorpsiyonuyla giden Paget hastalığı, metastatik kemik hastalığı, osteoporoz ve dirençli hiperkalsemi tedavisindeki etkileri iyi bilinmektedir. Son yıllarda kronik böbrek hastalıkları ve romatolojik hastalıklarda kullanımı yaygınlaşmıştır. Bifosfonatların pediatride kullanımı osteogenesis imperfekta, juvenil osteoporoz, fibröz displazi gibi kemik rezorpsiyonuyla seyreden hastalıklarda başlamış, klinik bulguları düzelttiği ve kemik kitlesini arttırdığı bildirilmiştir. Çocuklarda kronik böbrek hastalıklarında ve romatolojik hastalıklarda, özellikle glukokortikoid kullanımına bağlı osteoporoz tedavisinde yararlı olduğunu gösteren az sayıda çalışma vardır. Bu derleme ile çocukluk çağı böbrek hastalıklarında BP'ların kullanımı ile ilgili veriler incelenmiş, kullanım alanları, etkinliği ve yan etkileri değerlendirilmiştir.

Research paper thumbnail of Clinical and metabolic features of urolithiasis and microlithiasis in children

Pediatric Nephrology, 2009

We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis ... more 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.

Research paper thumbnail of Prognosis of acute poststreptococcal glomerulonephritis in children

Research paper thumbnail of Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

Pediatric nephrology (Berlin, Germany), Jan 29, 2018

Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in inf... more Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4-14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n = 5) or peritonitis (n = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static aft...

Research paper thumbnail of Pediatric stone disease in the first year of life: a single center study

Research paper thumbnail of Transition of the Adolescent Patient to the Adult Clinic

Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis, 2009

Transition is the purposeful, planned movement of adolescents and young adults with chronic and m... more Transition is the purposeful, planned movement of adolescents and young adults with chronic and medical conditions from child-centered to adult-oriented health care systems. For patients with chronic diseases that have begun early in life and continued into adulthood, transition is a very important period requiring not only medical, but also psychological and social support, which should begin on the day of diagnosis. Lack of coordination between pediatric and adult units, resistance of the adolescents and their families, and lack of planning and institutional support are a few of the numerous hardships that are encountered while trying to sustain a successful transition. This article reviews the steps of transition and also solutions for the potential barriers to a successful transition.

Research paper thumbnail of Cefepime-induced nonconvulsive status epilepticus as a cause of confusion in an elderly patient: A case report

Journal of the Formosan Medical Association, 2015

Acute confusion is a frequent emergency in elderly patients. Nonconvulsive status epilepticus (NC... more Acute confusion is a frequent emergency in elderly patients. Nonconvulsive status epilepticus (NCSE) should be included among the differential diagnoses, but is a diagnostic challenge. 1,2 NCSE has been associated with cefepime administration, and electroencephalography is an important tool for diagnoses. 3 The prognosis for elderly patients with untreated NCSE is poor. 2 We present the case of an 86-year-old patient with chronic kidney disease admitted to our hospital for right lower lobe pneumonia. Intravenous Unasyn 3 g was initiated every 12 hours for 2 weeks, but on Day 24, his fever flared again (38.4 C). The antibiotic regimen was changed to intravenous cefepime 2 g every 12 hours to combat possible nosocomial infections. On Day 28, the patient became confused and agitated. His temperature was 36.8 C, blood pressure 172/ 93 mmHg, heart rate 113 beats/min, and respiratory rate 20 breaths/min. Emergent laboratory findings were as follows: white blood cell count, 10,200/mm 3 ; serum sodium, 137 mEq/L; potassium, 4.1 mEq/L; calcium, 9.4 mg/dL; blood urea nitrogen, 32 mg/dL; creatinine, 2.5 mg/dL; alanine aminotransferase, 47 U/L; glucose, 148 mg/dL; thyroid-stimulating hormone, 2.67 mIU/mL; and free thyroxine, 1.35 ng/dL. Arterial blood gas measurements of oxygen delivered at a flow rate of 2 L/min via nasal cannula were as follows: pH, 7.41; partial pressure of carbon dioxide (pCO 2), 36 mmHg; partial pressure of oxygen

Research paper thumbnail of Rituximab-associated hypogammaglobulinemia: Incidence, predictors and outcomes in patients with multi-system autoimmune disease

Journal of autoimmunity, Jan 31, 2014

Rituximab is a B cell depleting monoclonal antibody used to treat lymphoma and autoimmune disease... more Rituximab is a B cell depleting monoclonal antibody used to treat lymphoma and autoimmune disease. Hypogammaglobulinemia has occurred after rituximab for lymphoma and rheumatoid arthritis but data are scarce for other autoimmune indications. This study describes the incidence and severity of hypogammaglobulinemia in patients receiving rituximab for small vessel vasculitis and other multi-system autoimmune diseases. Predictors for and clinical outcomes of hypogammaglobulinemia were explored. We conducted a retrospective study in a tertiary referral specialist clinic. The severity of hypogammaglobulinemia was categorized by the nadir serum IgG concentration measured during clinical care. We identified 288 patients who received rituximab; 243 were eligible for inclusion with median follow up of 42 months. 26% were IgG hypogammaglobulinemic at the time that rituximab was initiated and 56% had IgG hypogammaglobulinemia during follow-up (5-6.9 g/L in 30%, 3-4.9 g/L in 22% and <3 g/L in...

Research paper thumbnail of Epidermolysis Bullosa with Focal Glomerulosclerosis: A Case Report

Research paper thumbnail of Positional installation of contrast (PIC) and Redo-PIC cystography for diagnosis of occult vesicoureteral reflux

The Turkish journal of pediatrics, 2018

Karadeniz-Cerit K, Thomas DT, Ergun R, Yıldız N, Alpay H, İnanır S, Dağlı ET, Tuğtepe H. Position... more Karadeniz-Cerit K, Thomas DT, Ergun R, Yıldız N, Alpay H, İnanır S, Dağlı ET, Tuğtepe H. Positional installation of contrast (PIC) and Redo-PIC cystography for diagnosis of occult vesicoureteral reflux. Turk J Pediatr 2018; 60: 180-187. To evaluate the value of Positional Installation of Contrast (PIC) and Redo-PIC cystography in patients with febrile recurrent urinary tract infection (f-UTI) where voiding cystourethrogram (VCUG) was negative. Patients with recurrent f-UTI with no reflux on VCUG referred to the outpatient clinic of Pediatric Urology, between June 2011 and June 2016 were included in the study. A PIC cystography was performed in all patients. When reflux was found, subureteric injection was performed. Urinary cultures were used for follow-up. Patients that continued having f-UTI, received redo-PIC cystography. PIC cystography was performed on 42 patients. The average age of patients was 8.0±3.6 years. Vesicoureteral reflux (VUR) was detected in 41 patients. Average fo...

Research paper thumbnail of The Timing of Primary Neurosurgical Repair Significantly Affects Neurogenic Bladder Prognosis in Children With Myelomeningocele

Journal of Urology, 2006

The correlation between timing of the primary neurosurgical repair and urological prognosis in in... more The correlation between timing of the primary neurosurgical repair and urological prognosis in infants with open spinal dysraphism remains unknown. We investigated the impact of primary repair timing on neurogenic bladder prognosis in children with myelomeningocele. Materials and Methods: We retrospectively reviewed the records of 401 children with myelomeningocele followed at our multidisciplinary clinic between 1996 and 2005. Among these patients 129 were included in the study based on the availability of urological followup data at age 3 years, which was chosen as an ideal point for the standardization of urological findings. Children were assigned to 1 of 2 groups-those who underwent primary neurosurgical repair within 72 hours of delivery (group 1) and those undergoing repair after 72 hours (group 2). We compared the incidence of febrile urinary tract infections, hydronephrosis, vesicoureteral reflux and secondary tethering of the spinal cord at age 3 years, as well as cystometric bladder capacity and detrusor leak point pressure between the 2 groups. Results: Urological followup data at age 3 years revealed significantly increased incidence of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering of the spinal cord in children in group 2 compared to group 1 (chi-square, p Յ0.05 for all comparisons). Urodynamic assessment also showed a significantly lower bladder capacity and a substantially higher detrusor leak point pressure in children who received primary repair at later than 72 hours (t test, p Ͻ0.05). A subgroup analysis of group 1 comparing children who underwent repair before and after 24 hours following delivery failed to demonstrate any significant difference in terms of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering (chi-square, p Ͼ0.05 for all comparisons), whereas mean bladder capacity was significantly higher (t test, p Ͻ0.05) and mean detrusor leak point pressure was lower (t test, p ϭ 0.08) in children undergoing repair within 24 hours of delivery. Conclusions: The timing of primary neurosurgical repair has a significant impact on neurogenic bladder prognosis in children with myelomeningocele. Closure of the spinal lesion on the first day of life seems to provide the best chance for favorable lower urinary tract function.

Research paper thumbnail of Autosomal recessive polycystic kidney disease in 115 children: clinical presentation, course and influence of gender

Research paper thumbnail of Are myelodisplastic children receiving sufficient health care in Turkey? An analysis of the problems in primary management and their impact on neurourological outcome

Purpose: We aimed to investigate the problems in the primary care of children with spina bifida a... more Purpose: We aimed to investigate the problems in the primary care of children with spina bifida and to analyze their impact on neuro-urological outcome in Turkey. Mothers' information about the disease, difficulties in getting medical services, availability and efficacy of prenatal ultrasound and folic acid prevention was also assessed. Patients: A retrospective review of 476 children registered in the pediatric urology section of our institutional multidisciplinary spina bifida clinic between 1996 and 2005 was made. All children were assessed for the time of primary repair, time to first neuro-urological visit, and compliance to follow-up. A phone interview was made with 166 mothers to obtain data regarding their educational status, supplementary folic acid intake before/during gestation, compliance to obstetric follow-up, prenatal diagnosis and counseling, information about the importance of neuro-urological surveillance, and difficulties in neuro-urological follow-up. Results...

Research paper thumbnail of Microalbumin excretion and outcome in children with multicystic dysplastic kidney

Turkish Journal of Medical Sciences

To present the long-term follow-up results of children with multicystic dysplastic kidney (MCDK) ... more To present the long-term follow-up results of children with multicystic dysplastic kidney (MCDK) and urinary microalbumin excretion levels in order to evaluate whether there is an increased risk of renal damage or not. Materials and methods: Th irty-three children with the diagnosis of MCDK who had been followed up by the nephrology outpatient clinic between 2002 and 2009 were invited to participate in the study. Twenty-six healthy children were investigated as a control group for microalbumin/creatinine ratio (μg/g creatinine). Th e mean age at diagnosis, the duration of follow-up, accompanying urinary tract abnormalities, attacks of urinary tract infection (UTI), contralateral kidney size, and urinary microalbumin levels were investigated. Results: Th e mean age of the patients with MCDK and the mean duration of follow-up were 6.5 ± 3.9 years and 35 months (range 2-96) months, respectively. Th e most common urinary tract abnormality was vesicoureteral refl ux (VUR), with a rate of 34%. Th irty-nine percent of the children experienced UTI during follow-up. Th e compensatory renal hypertrophy of the contralateral kidney was 24% at 6 months and 68% at 12 months. Sixteen patients (59%) had an increased microalbumin/creatinine ratio (>30 μg/mg creatinine). Microalbumin/creatinine ratio was higher in patients with MCDK than it was in the controls (P = 0.001). Conclusion: Microalbuminuria and VUR are not rare in children with MCDK. Systematic follow-up of these patients is recommended to identify those at risk of contralateral renal damage.

Research paper thumbnail of Clinical outcome of patients with antenatal diagnosis of urinary anomalies

Research paper thumbnail of Salt wasting in two neonates with posterior urethral valves: secondary pseudohypoaldosteronism

Marmara Medical Journal, 2017

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerinin aldosterona duyarsızlığı ile karakteri... more Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerinin aldosterona duyarsızlığı ile karakterize, hiperkalemi, hiponatremi ve metabolik asidoz ile kendini gösteren nadir bir durumdur. Obstrüktif üropatisi olan bebeklerde ve piyelonefrit sırasında görülebilir. Gelişimi devam eden erken bebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisi ve enfeksiyonu sekonder PHA hastalarında tübüluslarda aldosteron direncine neden olan üç önemli faktördür. Genellikle hiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır, tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteron seviyelerinin saptanması ile tanı konur. Süt çoçuklarında biyokimyasal incelemede hiperpotasemi, hiponatremi ve metabolik asidoz saptandığında konjenital adrenal hiperplazi ayırıcı tanısında PHA göz önünde bulundurulmalı ve bu hastalarda üriner ultrasonografisi (USG) ve idrar analizi rutin olarak yapılmalıdır. Bu yazımızda sekonder PHA gelişen posterior üretral valv (PUV)'lı iki hasta sunuldu.

Research paper thumbnail of Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

The Journal of Pediatrics, 2018

for the ARegPKD consortium* Objective To identify prenatal, perinatal, and postnatal risk factors... more for the ARegPKD consortium* 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. AIC Akaike information criterion ARPKD Autosomal recessive polycystic kidney disease AUC Area under the curve CVVH Continuous venovenous hemofiltration PD Peritoneal dialysis Detailed affiliations available at www.jpeds.com (Appendix 1).

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

Balkan Medical Journal, 2017

Background: The causative agent spectrum and resistance patterns of urinary tract infections in c... more 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%), amoxicillinclavulanate (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.

Research paper thumbnail of Clinical and histopathological features of asymptomaticpersistent microscopic hematuria in children*

TURKISH JOURNAL OF MEDICAL SCIENCES, 2016

Background/aim: We analyzed the clinical and pathological features and prognosis of 106 children ... more 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.

Research paper thumbnail of Severe Neuropathic Ulcer and Fungal Dermatitis in a Patient with Spina Bifida

Research paper thumbnail of Use of Biphosphonates in Pediatric Nephrology Biphosphonates in Pediatric

Turkish Nephrology Dialysis Transplantation, 2011

Bifosfonatlar (BP) sentetik pirofosfat analoğu bileşiklerdir. Kemik yapımı sırasında osteoklast b... more Bifosfonatlar (BP) sentetik pirofosfat analoğu bileşiklerdir. Kemik yapımı sırasında osteoklast baskılanmasına yol açarak onların sayı ve işlevlerini azaltır, yaşam süresini kısaltır ve kemik rezorpsiyonunu önlerler. Erişkinlerde aşırı kemik rezorpsiyonuyla giden Paget hastalığı, metastatik kemik hastalığı, osteoporoz ve dirençli hiperkalsemi tedavisindeki etkileri iyi bilinmektedir. Son yıllarda kronik böbrek hastalıkları ve romatolojik hastalıklarda kullanımı yaygınlaşmıştır. Bifosfonatların pediatride kullanımı osteogenesis imperfekta, juvenil osteoporoz, fibröz displazi gibi kemik rezorpsiyonuyla seyreden hastalıklarda başlamış, klinik bulguları düzelttiği ve kemik kitlesini arttırdığı bildirilmiştir. Çocuklarda kronik böbrek hastalıklarında ve romatolojik hastalıklarda, özellikle glukokortikoid kullanımına bağlı osteoporoz tedavisinde yararlı olduğunu gösteren az sayıda çalışma vardır. Bu derleme ile çocukluk çağı böbrek hastalıklarında BP'ların kullanımı ile ilgili veriler incelenmiş, kullanım alanları, etkinliği ve yan etkileri değerlendirilmiştir.

Research paper thumbnail of Clinical and metabolic features of urolithiasis and microlithiasis in children

Pediatric Nephrology, 2009

We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis ... more 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.

Research paper thumbnail of Prognosis of acute poststreptococcal glomerulonephritis in children

Research paper thumbnail of Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

Pediatric nephrology (Berlin, Germany), Jan 29, 2018

Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in inf... more Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4-14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n = 5) or peritonitis (n = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static aft...

Research paper thumbnail of Pediatric stone disease in the first year of life: a single center study

Research paper thumbnail of Transition of the Adolescent Patient to the Adult Clinic

Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis, 2009

Transition is the purposeful, planned movement of adolescents and young adults with chronic and m... more Transition is the purposeful, planned movement of adolescents and young adults with chronic and medical conditions from child-centered to adult-oriented health care systems. For patients with chronic diseases that have begun early in life and continued into adulthood, transition is a very important period requiring not only medical, but also psychological and social support, which should begin on the day of diagnosis. Lack of coordination between pediatric and adult units, resistance of the adolescents and their families, and lack of planning and institutional support are a few of the numerous hardships that are encountered while trying to sustain a successful transition. This article reviews the steps of transition and also solutions for the potential barriers to a successful transition.

Research paper thumbnail of Cefepime-induced nonconvulsive status epilepticus as a cause of confusion in an elderly patient: A case report

Journal of the Formosan Medical Association, 2015

Acute confusion is a frequent emergency in elderly patients. Nonconvulsive status epilepticus (NC... more Acute confusion is a frequent emergency in elderly patients. Nonconvulsive status epilepticus (NCSE) should be included among the differential diagnoses, but is a diagnostic challenge. 1,2 NCSE has been associated with cefepime administration, and electroencephalography is an important tool for diagnoses. 3 The prognosis for elderly patients with untreated NCSE is poor. 2 We present the case of an 86-year-old patient with chronic kidney disease admitted to our hospital for right lower lobe pneumonia. Intravenous Unasyn 3 g was initiated every 12 hours for 2 weeks, but on Day 24, his fever flared again (38.4 C). The antibiotic regimen was changed to intravenous cefepime 2 g every 12 hours to combat possible nosocomial infections. On Day 28, the patient became confused and agitated. His temperature was 36.8 C, blood pressure 172/ 93 mmHg, heart rate 113 beats/min, and respiratory rate 20 breaths/min. Emergent laboratory findings were as follows: white blood cell count, 10,200/mm 3 ; serum sodium, 137 mEq/L; potassium, 4.1 mEq/L; calcium, 9.4 mg/dL; blood urea nitrogen, 32 mg/dL; creatinine, 2.5 mg/dL; alanine aminotransferase, 47 U/L; glucose, 148 mg/dL; thyroid-stimulating hormone, 2.67 mIU/mL; and free thyroxine, 1.35 ng/dL. Arterial blood gas measurements of oxygen delivered at a flow rate of 2 L/min via nasal cannula were as follows: pH, 7.41; partial pressure of carbon dioxide (pCO 2), 36 mmHg; partial pressure of oxygen