Diffuse Alveolar Damage Correlation with Clinical Diagnosis of Pediatric Acute Respiratory Distress Syndrome.

Diffuse alveolar damage (DAD) is one of the pathological hallmarks of acute respiratory distress syndrome (ARDS). We aimed to compare pathological findings of DAD with clinical ARDS criteria. We re-evaluated 20 patients whose clinical autopsy revealed DAD.

Inflammatory milieu of muscle biopsies in juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is an inflammatory myopathy which causes severe morbidity and high mortality if untreated. In this study, we aimed to define the T-helper cell profile in the muscle biopsies of JDM patients. Muscle biopsies of twenty-six patients (50% female) were included in the study.

LARGE expression in different types of muscular dystrophies other than dystroglycanopathy.

Background: Alpha-dystroglycan (αDG) is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin globular domains and certain arenaviruses. An important enzyme, known as Like-acetylglucosaminyltransferase (LARGE), has been shown to transfer repeating units of -glucuronic acid-β1,3-xylose-α1,3- (matriglycan) to αDG that is required for functional receptor as an extracellular matrix protein scaffold. The reduction in the amount of LARGE-dependent matriglycan result in heterogeneous forms of dystroglycanopathy that is associated with hypoglycosylation of αDG and a consequent lack of ligand-binding activity.

Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department.

Isıyel E, Tekşam Ö, Foto-Özdemir D, Özmert E, Tümer AR, Kale G. Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department. Turk J Pediatr 2018; 60: 625-632.

Clinical features and treatment results in children with anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) tends to have frequent relapse and good response to salvage chemotherapy. The frequency of ALCL among 1486 Non-Hodgkin's lymphoma (NHL) cases followed-up since 1972 was 1.5%, however, the percentage was 9.

Final diagnosis of children and adolescents with musculoskeletal complaints.

Background: Musculoskeletal complaints (MSCs) are one of the leading causes of outpatient admissions. However, analytical and epidemiological data are limited. The aim of this study is to identify the etiology of MSCs (excluding acute traumatic conditions) in children and adolescents, and to identify clues for the differential diagnosis.

The efficacy of delayed surgery in children with high-risk neuroblastoma.

Context: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies.

Aims: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma.

Placental findings of IUGR and non-IUGR.

This study aims to define the placental findings in intrauterine growth restriction (IUGR). The study group consisted of 40 neonates without IUGR and 24 neonates with IUGR, including their placentas. The cases were separated into three groups according to gestational age.

Burnout syndrome during residency in internal medicine and pediatrics in a country without working time directive.

Purpose: The purpose of this paper is to demonstrate burnout syndrome among internal medicine and pediatrics residents in a country that does not have the working time directive (WTD) and also to determine the risk factors and consequent impact on efficient functioning in clinical areas.

Design/methodology/approach: A 57-item questionnaire was given to internal medicine and pediatrics residents. Responses from 22 pediatrics and 33 internal medicine residents were evaluated.

Studying cytokines of T helper cells in the kidney disease of IgA vasculitis (Henoch-Schönlein purpura).

Background: Immunoglobulin A vasculitis (IgA-V), formerly known as Henoch-Schönlein purpura (HSP), is the most common small vessel vasculitis in children. In recent years, the role of T cells in the pathogenesis of HSP/IgA-V has become a focus of research.

Methods: Renal biopsy specimens from 22 pediatric patients diagnosed with Henoch-Schönlein nephritis (patient group) were compared to normal renal tissue in nephrectomy specimens from 20 pediatric patients diagnosed with Wilms tumor (control group).

Clinical features and treatment results of children with diffuse large B-cell lymphoma.

The demographic, clinical characteristics, and treatment groups of 33 children with diffuse large B-cell lymphoma (DLBCL) were recorded and analyzed among 1486 non-Hodgkin lymphoma (NHL) cases since 1972. The median age was 9.7 years (range 1.

Primary liver tumors in children: Hacettepe experience.

We aimed to review our experience with the clinical characteristics and outcome in childhood liver tumors. We investigated the clinical, laboratory and pathological characteristics, treatments and outcome in hepatoblastomas (HBL) and hepatocellular carcinomas (HCC). We identified 91 HBL and 42 HCC cases.

Studying IFN-gamma, IL-17 and FOXP3 in pediatric lupus nephritis.

Background: We studied the cytokines secreted by the inflammatory T cell subgroups (IFN-γ and IL-17) and FOXP3 expression in lupus nephritis (LN) and analyzed associations with clinical and histopathological parameters.

Methods: Renal tissue samples of 39 LN patients were studied. Immunohistochemical staining was carried out with antibodies against IFN-γ, IL-17, and FOXP3.

Munchausen syndrome by proxy presented as recurrent respiratory arrest and thigh abscess: a case study and overview.

While many physicians are familiar with the sexual or physical abuse of children, there is little awareness about Munchausen syndrome by proxy (MSBP). As case reports of MSBP increase, awareness among physicians is thought to increase as well. We thus present herein a 16-month-old girl who admitted to Hacettepe University İhsan Doğramacı Children's Hospital with the complaint of seizure, recurrent apnea and thigh abscess, who was later diagnosed as MSBP.

Serum alpha-fetoprotein levels in neonatal cholestasis.

Alpha-fetoprotein (AFP) is used as a tumor marker for hepatocellular carcinoma, hepatoblastoma and germ cell tumors. It may also be elevated in infants with some hepatobiliary disorders. The mechanism of AFP elevation in neonatal cholestasis is not known.

Clinicopathological and immunohistological features in childhood IgA nephropathy: a single-centre experience.

Background: IgA nephropathy is a glomerular disease diagnosed by renal biopsy and is characterized by a highly variable course ranging from a completely benign condition to rapidly progressive renal failure. We aimed to evaluate the clinical, histopathological and inflammatory characteristics of children with IgA nephropathy.

Methods: Data of 37 patients with IgA nephropathy diagnosed between the years 1980 and 2008 were retrospectively reviewed.

Mutations in KLHL40 are a frequent cause of severe autosomal-recessive nemaline myopathy.

Nemaline myopathy (NEM) is a common congenital myopathy. At the very severe end of the NEM clinical spectrum are genetically unresolved cases of autosomal-recessive fetal akinesia sequence. We studied a multinational cohort of 143 severe-NEM-affected families lacking genetic diagnosis.

The analysis of child abuse and neglect cases assessed by a multidisciplinary study group between 2005-2008.

This study aimed to evaluate the diagnosis, treatment and follow-up of child abuse and neglect (CAN) cases. Subjects were 102 cases that were clinically assessed by the multidisciplinary hospital team. Early and late childhood cases (according to the age at first abuse) were compared by means of characteristics of abuse.

Malignant sacrococcygeal germ cell tumors in children: a 30-year experience from a single institution.

Background: Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors.

Procedure: Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded.

Role of CXCR1 (CKR-1) in inflammation of experimental mesangioproliferative glomerulonephritis.

CXCR1 (CKR-1), a receptor of IL-8, is expressed in various cells including neutrophils and monocytes, both of which play a major role in proliferating glomerular diseases. We investigated time-dependent expression of CXCR1 and the effect of single-dose cyclosporine A (CsA) treatment on this expression in experimental mesangioproliferative glomerulonephritis induced by anti-thymocyte serum (ATS). Wistar rats were divided into three groups.

Thirty-three-year experience on childhood poisoning.

By comparing our data for the period 1985-2008 with findings from a previous report covering the period 1975-1984, we aimed to share our experience with poisoning cases in order to contribute toward its prevention, diagnosis and treatment. The records of patients admitted to the Pediatric Intensive Care Unit with acute poisoning between November 1985 and October 2008 were evaluated retrospectively. The records of 2251 patients with acute poisoning could be retrieved.

Different features of lung involvement in Niemann-Pick disease and Gaucher disease.

Background: Niemann-Pick disease (NPD) and Gaucher disease (GD) are well-known lysosomal storage diseases. Respiratory system involvement is an important cause of morbidity and mortality in patients with NPD and GD.

Objectives: We tried to assess the clinical, radiological, and histological features of GD and NPD patients with lung involvement.

Varying clinical features of Turkish Kawasaki disease patients.

Kawasaki disease (KD) is an acute, systemic and self-limited vasculitis that is complicated with the development of coronary artery (CA) aneurysms. We present the clinical features of Turkish KD patients from a tertiary referral center. When 33 KD patients were assessed, a number of features stood out as differing from the expected, for example, periungual peeling 7.

Solid tumors in Turkish children: a multicenter study.

Background: This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions.

Methods: The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out.

A congenital muscular dystrophy with mitochondrial structural abnormalities caused by defective de novo phosphatidylcholine biosynthesis.

Congenital muscular dystrophy is a heterogeneous group of inherited muscle diseases characterized clinically by muscle weakness and hypotonia in early infancy. A number of genes harboring causative mutations have been identified, but several cases of congenital muscular dystrophy remain molecularly unresolved. We examined 15 individuals with a congenital muscular dystrophy characterized by early-onset muscle wasting, mental retardation, and peculiar enlarged mitochondria that are prevalent toward the periphery of the fibers but are sparse in the center on muscle biopsy, and we have identified homozygous or compound heterozygous mutations in the gene encoding choline kinase beta (CHKB).