Retrospective Analysis of the Factors Affecting Growth Parameters in Turkish Children With Systemic Lupus Erythematosus.

Objectives: This study aims to investigate the growth parameters in Turkish children with systemic lupus erythematosus (SLE) and to compare these growth parameters according to the presence or absence of cyclophosphamide, rituximab treatment, cumulative corticosteroid dose, proliferative nephritis, and the last visit disease activity and damage index.

Patients And Methods: Medical data, growth parameters including z-scores for weight, height, and body mass index and parent-adjusted height z-scores of 45 juvenile SLE (jSLE) patients (5 males, 40 females; mean age 12.3±3.

Growth Parameters of Turkish Children With an Autoinflammatory Disease Before and After Canakinumab Treatment.

Objective: To evaluate the effect of canakinumab on growth parameters of patients with autoinflammatory diseases.

Methods: This retrospective study included Colchicine resistant familial Mediterranean fever (FMF), Mevalonate kinase deficiency (MKD), Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), Deficiency of adenosine deaminase 2 (DADA2) patients treated with canakinumab for at least six consecutive months.

Results: Eleven patients with FMF, 9 with MKD, 3 with TRAPS, and 1 with DADA2 were included.

Newborn Screening for Cystic Fibrosis in Mersin Province: Yearly Assessment of the National Program.

Objectives: A national newborn screening program for cystic fibrosis (CF) was started using immunoreactive trypsinogen (IRT) test on January 1, 2015, in Turkey. We aimed to analyze the characteristics of newborn screen-positive (NBSP) infants in Mersin province.

Materials And Methods: The data on NBSP infants were retrospectively analyzed between 2015 and 2017 from records of Mersin Women & Children's Hospital and Mersin City Training and Research Hospital.

Etanercept for the Treatment of Chronic Arthritis Related to Chronic Granulomatous Disease: A Case.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency, in which infections are the main presenting symptoms. Moreover, patients may also experience inflammatory and autoimmune manifestations. With proper management of infections, patients now survive to adulthood, and inflammatory manifestations have become more prominent problems.

Efficacy of Magnesium Sulfate Treatment in Children with Acute Asthma.

Objective: Systemic administration of magnesium sulfate (MgSO4) has been proposed as a treatment for pediatric patients with acute asthma. However, previous trials show mixed results and uncertain evidence of benefit. The aim of the study was to ascertain whether intravenous (IV) MgSO4 improves lung function parameters in children with acute asthma.

A comparative study of the pancreas in pediatric patients with cystic fibrosis and healthy children using two-dimensional shear wave elastography.

Purpose: To compare sonographic parameters of the pancreas between healthy children and pediatric cystic fibrosis (CF) patients with pancreatic involvement using shear wave elastography (SWE) and to investigate the efficacy of SWE in the diagnosis of pancreatic involvement in pediatric CF patients.

Methods: The pancreas was evaluated in 38 patients with CF and 38 healthy children using conventional B-mode ultrasonography (US) and two-dimensional (2D)-SWE.

Results: The pancreatic 2D-SWE values of the CF group were significantly lower than those of the healthy control group (1.

Two years of newborn screening for cystic fibrosis in Turkey: Çukurova experience.

Şaşihüseyinoğlu AŞ, Altıntaş DU, Bişgin A, Doğruel D, Yılmaz M, Serbes M. Two years of newborn screening for cystic fibrosis in Turkey: Çukurova experience. Turk J Pediatr 2019; 61: 505-512.

Clinical findings and genetic analysis of the patients with IL-12Rβ1 deficiency from southeast Turkey.

Doğruel D, Gündeşlioğlu ÖÖ, Yılmaz M, Alabaz D, Altıntaş DU, Kocabaş E. Clinical findings and genetic analysis of the patients with IL- 12Rβ1 deficiency from southeast Turkey. Turk J Pediatr 2019; 61: 174-179.

Twenty-Year Experience of a Single Referral Center on Pediatric Familial Mediterranean Fever: What Has Changed Over the Last Decade?

Background/objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease and is characterized by recurrent fever and serositis episodes. We aimed to share our 20-year FMF experience, clarify a phenotype-genotype correlation, and compare the characteristics and outcomes of pediatric FMF patients over the last 2 decades in this study.

Methods: This medical record review study included 714 pediatric FMF patients (340 females, 374 males), diagnosed by Tel Hashomer diagnostic criteria between January 2009 and January 2019 and followed up in our department.

Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience.

Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses.

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever.

Objectives: This study aims to analyze the growth parameters in children with familial Mediterranean fever (FMF) according to disease characteristics including genotype and disease severity by a recently validated tool in relatively more patients.

Patients And Methods: This retrospective study included 126 patients with FMF (70 males, 56 females; mean age 7.3±3.

Canakinumab in Children with Familial Mediterranean Fever: A Single-Center, Retrospective Analysis.

Introduction: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by interleukin (IL)-1 overproduction. Colchicine is the mainstay drug in the treatment of FMF; however, a minority of patients do not respond despite the highest tolerated doses. We aimed to share our experience with canakinumab, a human monoclonal antibody against IL-1β, in pediatric FMF patients.

Symptomatic multifocal avascular necrosis in an adolescent with neuropsychiatric systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by malar rash, oral ulcers, arthralgia, photosensitivity and nephritis. Herein, we report a rare comorbidity, multiple avascular necrosis (AVN), in an adolescent SLE patient and also highlight the importance of risk factors for this comorbidity with a brief literature review. A 13-year-old female patient was admitted with severe headache, visual plus auditory hallucinations, polyarthritis and a history of recurrent oral ulcers.

Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency.

Background: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established.

Objective: To determine the clinical and immunologic features of LRBA deficiency and long-term efficacy of abatacept treatment in controlling the different disease manifestations.

Do practical laboratory indices predict the outcomes of children with Henoch-Schönlein purpura?

: Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis of childhood and often has a self-limiting course. We aimed to study whether practical laboratory parameters at the diagnosis predict disease course including recurrence and nephritis in addition to severe gastrointestinal involvement in children with HSP. : This retrospective cohort study included 214 HSP patients, 43.

Hyperimmunoglobulinemia D syndrome with recurrent perianal abscess successfully treated with canakinumab.

Hyperimmunoglobulinaemia D syndrome is an autoinflammatory disease usually representing recurrent episodes of fever, arthralgia/arthritis, cervical lymphadenopathy, vomiting, diarrhoea, abdominal pain and skin rashes lasting 3-7 days every 4-8 weeks since their infancy. Recent reports suggested a link between perianal fistulae/abscess and severe colitis with hyperimmunoglobulinaemia D syndrome resembling an inflammatory bowel disease phenotype. Herein, we report an 18-month-old patient with recurrent attacks of fever and pharyngitis lasting 2-3 days every 10-15 days since the first two weeks of life.

Frequency of functional gastrointestinal disorders in children with familial Mediterranean fever.

Introduction: Familial Mediterranean fever (FMF) is characterized by self-limiting fever episodes usually accompanied by serositis, arthralgia, and arthritis. Functional gastrointestinal disorders (FGIDs) are diseases in which brain-gut axis and low-grade inflammation take part in pathogenesis. We aimed to study the FGIDs frequencies and possible risk factors for FGIDs in children with FMF.

Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey.

Objectives: This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey.

Methods: Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2005 and June 2018 were included in the present study.

Results: The median age at the diagnosis was 12.

MEFV gene variants in children with Henoch-Schönlein purpura and association with clinical manifestations: a single-center Mediterranean experience.

Objectives: Henoch-Schönlein purpura (HSP) is characterized by non-thrombocytopenic palpable purpura, abdominal pain, and arthralgia/arthritis. We aimed to describe the clinical presentations of children with HSP in a single center and compare the prevalence of each manifestations between patients with MEFV variants, particularly in exon 10 and those without.

Methods: This cohort retrospectively included 144 HSP (59 females, 85 males) patients without Familial Mediterranean Fever (FMF) symptoms and followed for at least 6 months.

Recurrent Henoch Schönlein purpura without renal involvement successfully treated with methotrexate.

Introduction: Henoch Schönlein purpura is characterised by palpable purpura, abdominal pain, arthritis/arthralgia, often with a self-limiting course. Herein, we report a patient with recurrent Henoch Schönlein purpura and severe gastrointestinal involvement, successfully treated with methotrexate.

Case Presentation: A 12-year-old boy was admitted to our department with palpable purpura, abdominal pain and arthralgia.

Renal Amyloidosis in Deficiency of Adenosine Deaminase 2: Successful Experience With Canakinumab.

Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease that was firstly described in patients with early-onset strokes, livedo reticularis, and periodic fever resembling polyarteritis nodosa. In reported case series, researchers described highly variable manifestations, including autoimmunity, immunodeficiency, hepatosplenomegaly, pancytopenia, ichthyosiform rash, and arthritis, in patients with DADA2. A thirteen-year-old female patient who was born to consanguineous parents was admitted to our hospital with generalized edema and leg pain.

Is Henoch-Schönlein purpura a susceptibility factor for functional gastrointestinal disorders in children?

Henoch-Schönlein purpura (HSP), the most common childhood vasculitis is characterized by non-thrombocytopenic palpable purpura, arthritis/arthralgia, abdominal pain and renal involvement. Functional gastrointestinal disorders (FGIDs) are heterogeneous disease spectrum with unclear etiology and include the most common subtypes: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain and functional constipation. Formerly, FGIDs were known as non-organic disorders; however, recent advances revealed that low-grade inflammation may also play a role.