Hülya Nalçacıoğlu

• Pediatric Nephrology
• Ondokuz Mayis University Faculty of Medicine

Cystinuria, characterized by defective renal absorption of cystine causing recurrent nephrolithiasis, demands ongoing management. This study examines the effects of COVID-19-related disruptions in tiopronin availability on the clinical outcomes of pediatric cystinuria patients. This retrospective cohort study analyzed medical records of 11 pediatri...

Background Managing congenital nephrotic syndrome (CNS) remains a clinical challenge. While albumin infusions and nephrectomy have been long-standing treatments, a conservative approach is increasingly favored. This study aimed to compare clinical outcomes between nephrectomy (Nx) and non-Nx in patients with bi-allelic NPHS1 mutations. Methods Thi...

Objectives This study aimed to evaluate the predictability of the homozygous M694V genotype in pediatric familial Mediterranean fever (FMF) patients and to develop a clinical scoring system to enhance disease management strategies. Methods This nationwide, multicentre, cross-sectional study included 3,981 pediatric FMF patients with biallelic path...

Chronic kidney disease (CKD) is associated with growth retardation and delayed pubertal development in the pediatric population. This study aimed to investigate the impact of CKD on growth and pubertal development in children and adolescents by considering factors such as dialysis and transplantation. A cross-sectional retrospective study was condu...

Objectives Management of systemic lupus erythematosus (SLE) through new treatment options has improved lupus nephritis (LN) prognosis. The aim of this study was to compare the changes in the demographic, laboratory, and treatment characteristics, prognosis, and outcomes of pediatric-onset LN patients over 30 years. Methods We retrospectively revie...

Background Cyclophosphamide (CYC) is an inactive alkylating agent that transforms the alkyl radicals into other molecules and is used in combination with systemic corticosteroids in the treatment of many childhood rheumatic diseases, such as systemic lupus erythematosus (SLE), and ANCA-associated vasculitis (AAV). In recent years, rituximab (RTX),...

Vitamin ve Minerallerin Günlük Hayatımızdaki Yeri Murat ERSOY Vitamin ve Minerallerin İlave Olarak Alınması Gereken Durumlar Güldane Aylin İNAL Gebelikte Kullanılması Gereken Vitamin ve Mineraller Utkucan OKUDUCU Vitamin A Saliha BARUTÇU Adnan BARUTÇU Vitamin B1 (Tiamin) Burcu KÖŞECİ Vitamin B2 (Riboflavin) Nazmiye TÜZEL GÜNDÜZ Vitamin B3 (Niasin)...

The study evaluated the relationship between balance function and skeletal muscle mass index (ASMI), physical function, and fatigue in children with chronic kidney disease (CKD). A cross-sectional study of 83 children with CKD (stages 1–4, dialysis, transplant) and 71 healthy controls was conducted. Functional performance tests, including gait spee...

Background/Objectives: This study aims to evaluate the efficacy and outcomes of renal replacement therapy (RRT) in pediatric patients with metabolic diseases, specifically focusing on the impact of hemodialysis (HD) and peritoneal dialysis (PD) on clinical parameters, toxin reduction, and long-term survival. Methods: This retrospective study includ...

Background/Objectives: Eculizumab is a first-line treatment for atypical hemolytic uremic syndrome (aHUS), and patients undergoing eculizumab therapy may become more susceptible to infection caused by Neisseria meningitidis (Nm). While meningococcal vaccination is required for patients undergoing eculizumab therapy, there is limited knowledge about...

Congenital anomalies of the kidney and urinary tract (CAKUT) significantly contribute to pediatric morbidity, often necessitating ureterorenal surgery. This study explored the relationship between genetic mutations, renal surgery requirements, and prenatal, postnatal, and parental risk factors in children with CAKUT. A retrospective analysis of 651...

Introduction Continuous ambulatory peritoneal dialysis is an important modality of renal replacement therapy in children. Catheter dysfunction (commonly obstruction) is a major cause of morbidity and is a significant concern that hampers renal replacement therapy. As omentum is a significant cause of obstruction, some recommend routine omentectomy...

Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date. A total of 28 patients (21 girls, 7 boys) who were dia...

Aims: Hepatocyte nuclear factor 1β (HNF1B) mutations are the most common monogenic cause of congenital anomalies of the kidney and urinary tract (CAKUT). We aimed to investigate clinical and genetic characteristics of patients with HNF1B nephropathy to expand its phenotypic and genetic spectrum. Materials and methods: This retrospective cohort s...

Introduction We investigated the relationship between metabolic acidosis over time and allograft outcome in pediatric kidney transplantation (KTx). Methods This registry study collected data up to 10 years posttransplant. Survival analysis for a composite end point of graft loss or estimated glomerular filtration rate (eGFR) ≤ 30 ml/min per 1.73 m...

Background/Aims Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. Method A web-based software was prepared. Patient demographics, time under rubble (TUR), admission labora...

Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. Data from children ag...

Introduction This report provides insight into three distinct pediatric cases exhibiting a nexus between multisystem inflammatory syndrome in children (MIS-C) and thrombotic microangiopathy (TMA) triggered by COVID-19. The aim is to underscore the range of clinical presentations and the essentiality of early interventions. Case presentations This...

Objective: Chronic kidney disease (CKD) is associated with growth retardation and delayed pubertal development in the pediatric population. This study aimed to investigate the impact of CKD on growth and pubertal development in children and adolescents by considering factors such as dialysis and transplantation. Methods:A cross-sectional retrospect...

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal cystic diseases. Although its clinical manifestations usually occur in adulthood, hypertension (HT) is known to develop in most patients before the decline in renal function and it is associated with faster progression to end stage kidney disease (ESKD)....

Objective: Celiac disease (CD) is an autoimmune disorder among genetically predisposed subjects exposed to gluten-containing foods. It is now appreciated that CD is now recognized as a common condition among persons of various ethnic groups and both adults and children, and that affects many organ systems. This study was conducted to evaluate the d...

Background: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. Methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11...

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boy...

Background: The phenotypic and genotypic spectrum and kidney outcome of PLCε1-related kidney disease are not well known. We attempted to study 25 genetically confirmed cases of PLCε1-related kidney disease from 11 centers to expand the clinical spectrum and to determine the relationship between phenotypic and genotypic features, kidney outcome, an...

Introduction: Congenital nephrotic syndrome (CNS) is characterized by severe proteinuria, hypoalbuminemia, and edema within the first three months of life. CNS can occur due to perinatal infections or mutation of genes encoding structural or regulatory proteins of the glomerular filtration barrier. Treatment includes albumin infusions, nephrectomy,...

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational stud...

Anti-interleukin 1 agents are used successfully in colchicine-resistant or intolerant Familial Mediterranean Fever (FMF) patients. Sixty-five patients with FMF who received canakinumab treatment for at least 6 months due to colchicine resistance or intolerance between 2016 and 2020 in our department were retrospectively analyzed. Canakinumab treatm...

Background: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors and follow-up results of patients with NC in Turkey. Materials and methods: Patients diagnosed with NC in the Pediatric Nephrology Department Units of 19 centers from all geographical re...

Infectious complications are increasingly reported in patients with connective tissue diseases. These complications are both related to using immunosuppressive drugs and organ dysfunctions caused by diseases. Pyomyositis is a pyogenic infection of skeletal muscle associated with immunocompromised conditions, especially human immunodeficiency virus...

Introduction: Chronic kidney disease and its complications are among the most frequent cause of morbidity and mortality in patients with meningomyelocele. Objective: In this study, we aimed to determine the risk factors leading to chronic kidney disease progression in these patients. Material and Method: Fifty patients with meningomyelocele were an...

To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients...

Zinc is an essential micronutrient for the organism and is critical for the functional and structural integrity of cells. Determination of the amount of trace element concentrations in tissues such as hair samples have been used for the evaluation of the metabolic status especially in children. Unlike serum zinc, concentrations of zinc in hair are...

Background The aim of the present study was to evaluate the presence of cardiac systolic and diastolic dysfunction in pediatric patients with steroid sensitive nephrotic syndrome (NS). Methods The study population consisted of 19 patients with debut‐relapse of NS aged 1–18 years and 30 sex and age‐matched healthy controls. Blood, urine samples, tw...

Objective:Vesicoureteral reflux (VUR) is considered the most important predisposing factor for urinary tract infection (UTI). Renal damage due to VUR, subsequently renal scarring and the possibility of reflux nephropathy warrant early detection of VUR. Our aim was to evaluate the value of positioned instillation of contrast (PIC) cystography in the...

Paç Kısaarslan A, Sözeri B, Baştuğ F, Gündüz Z, Yel S, Nalçacıoğlu H, Şahin N, Özdemir Çiçek S, Poyrazoğlu H, Düşünsel R. Elemental mercury intoxication in 7 patients admitted to a pediatric rheumatology clinic. Turk J Pediatr 2019; 61: 786-790. Mercury (Hg) is a toxic heavy metal that can be classified into three groups; organic (methyl), inorgani...

Introduction: Brain natriuretic peptides, released in response to left ventricular stress, have a strong prognostic value in dialysis patients. However, their role in detecting abnormalities of fluid status is under debate; the relationship between volume status and brain natriuretic peptides (BNPs) differs among various studies. The aim of our st...

A 6-year-old Syrian boy presented with complaints of facial dysmorphism and difficulty of walking. He had coarse face, macrocephaly, pectus carinatum, x-bain deformity, kyphosis, corneal clouding, and claw hand deformity. Galactose-6 sulphatase enzyme level was 0.1 nmol/mg.17 h (reference range, > 68 nmol/mg.17 h), compatible with Morquio syndrome....

The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome (NCS). We also investigated the left renal vein Doppler ultrasonography (DUS) results, to determine whether or not there was an association between clinical symptoms and DUS findings among these patients....

Background COQ2 mutations cause a rare infantile multisystemic disease with heterogeneous clinical features. Promising results have been reported in response to Coenzyme Q10 treatment, especially for kidney involvement, but little is known about the long-term outcomes. Methods We report four new patients from two families with the c.437G→A (p.Ser1...

Baştuğ F, Nalçacıoğlu H, Baş VN, Tekatlı-Çelik B, Çetinkaya H, Yel S. Acute renal failure due to severe hypercalcemia and nephrocalcinosis treated with two doses of pamidronate in an infant with Williams-Beuren syndrome. Turk J Pediatr 2018; 60: 210-215. Infantile hypercalcemia has been reported in 15% of infants and children with Williams-Beuren s...

Renal transplant recipients are on long-term potent immunosuppressive therapy, which makes them highly vulnerable to opportunistic fungal infections. Dematiaceous, or dark-pigmented saprophytic fungi, are being increasingly seen as opportunistic pathogens of mycoses in immunosuppressed patients. One of these is Aureobasidium pullulans, which is a b...

Cystinosis is a rare autosomal recessive disorder resulting from defective lysosomal transport of cystine due to mutations in the cystinosin lysosomal cystine transporter (CTNS) gene. The clinical phenotype of nephropathic cystinosis is characterized by renal tubular Fanconi syndrome and development of end-stage renal disease during the first decad...

Background: Assessment of volume status and differentiating “underfill” and “overfill” edema is essential in the management of patients with nephrotic syndrome (NS). Objectives: Our aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children wit...

Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. We report a case of a soft tissue amyloidoma in the abdomen of a 16-year-old girl who was diagnosed as having systemic...

Background: Assessment of volume status and differentiating "underfill" and "overfill" edema is essential in the management of patients with nephrotic syndrome (NS). Objectives: Our aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children...

Renal involvement in non-Hodgkin lymphoma is a recognized development, but it mostly follows a diagnosis of lymphoma. We describe a rare case of a T-cell-type non-Hodgkin lymphoma that first presents as nephromegaly in a 5-year-old girl admitted to the emergency department with abdominal pain. Further investigation revealed bilateral nephromegaly,...

Purpose: To examine functional capacity and muscle strength in children and youth with familial Mediterranean fever (FMF) as compared with controls, and to assess whether these factors influence quality of life (QOL) in FMF. Methods: A total of 100 subjects with FMF and 55 control subjects (8-18 years old) without known health issues were enrolled...

Dense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the altern...

Nutcracker syndrome (NS), caused by compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta, results in elevation of LRV pressure and collateral vein development. This meso-aortic compression may present with hematuria, left flank pain and proteinuria. Several treatment options have been described to reli...

It is aimed to evaluate the clinical and laboratory characteristics of children applied to nephrology outpatient clinic with the complaint of bedwetting. The records of all patients who applied to Pediatric Nephrology outpatient clinic with the complaint of bedwetting between January 2006 and June 2012 were evaluated retrospectively. A total of 364...

Nutcracker syndrome (NS), caused by compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta, results in elevation of LRV pressure and collateral vein development. This meso-aortic compression may present with hematuria, left flank pain and proteinuria. Several treatment options have been described to reli...

Unlabelled: Bilateral obstructive nephrolithiasis is a rare cause of acute kidney injury (AKI) in early childhood. As soon as the identification of AKI secondary to ureteral stone is made, it will necessitate an emergency treatment. Patients: We report three infants with AKI caused by bilateral obstructive ureteral cystine stones. They were diag...

Infectious diseases, even vaccine preventable ones, might affect transplanting and the life course in pediatric solid-organ recipients. Owing to immunosuppression and decreased antibody production, susceptibility to infections is increased in these patients. The present study was designed to assess the vaccination and antibody status of the pediatr...

Gastrointestinal involvement is common in Henoch-Schönlein purpura, whereas life-threatening gastrointestinal bleeding is very rare. A seven-year-old male patient who was diagnosed with Henoch-Schönlein purpura four days before admitted with rectal bleeding and impaired general well-being. Palpable purpuric rash on the lower extremities and a clini...