Clinical outcomes and the impact of treatment modalities in children with carbapenem-resistant Enterobacteriaceae bloodstream infections: a retrospective cohort study from a tertiary university hospital.

Background: The increasing prevalence of carbapenem-resistant Enterobacteriaceae (CRE) infections among children represents a significant global concern, leading to elevated mortality rates. The aim of this study was to evaluate the risk factors, outcomes, 30-day mortality rates and contributing factors in children with CRE bloodstream infections (CRE-BSIs).

Methods: Data regarding demographic characteristics, treatment approaches and outcomes of hospitalized children aged 0-18 years diagnosed with CRE-BSIs between January 2018 and December 2022 were extracted from medical records.

Thrombotic Microangiopathy Following Hematopoietic Stem Cell Transplantation: A Case of Purtscher-like Retinopathy.

Purtscher-like retinopathy (PLR) is a rare entity related with retinal ischemia due to endothelial dysfunction and embolization. We present a case of a 17-year-old patient who presented with PLR associated with transplant-associated thrombotic microangiopathy. Visual acuity was finger counting at 1 meter in both eyes.

MHC Class II Deficiency: Clinical, Immunological, and Genetic Insights in a Large Multicenter Cohort.

Background: Major histocompatibility complex class II deficiency, a combined immunodeficiency, results from loss of HLA class II expression on antigen-presenting cells. Currently, hematopoietic stem cell transplantation stands as the sole curative approach, although factors influencing patient outcomes remain insufficiently explored.

Objectives: To elucidate the clinical, immunologic, and genetic profiles associated with MHC-II deficiency and identify prognostic indicators that affect survival rates.

COVID-19 disease in children and adolescents following allogeneic hematopoietic stem cell transplantation: A report from the Turkish pediatric bone marrow transplantation study group.

Background: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited.

Objectives: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection.

Method: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022.

Successful Treatment of a Child with Hemoglobin Hammersmith with Hematopoietic Stem Cell Transplantation.

Hemoglobin (Hb) Hammersmith, formed by serine substitution for phenylalanine at residue 42 in the beta-globin chain, is a very rare variant of unstable hemoglobin with low oxygen affinity. For patients with hemoglobinopathies, it is well-established that hematopoietic stem cell transplantation provides a complete cure, but the literature on its role for those with Hb Hammersmith is limited. A seven-month-old girl who was examined for anemia and splenomegaly was followed up for congenital hemolytic anemia.

Hematopoietic stem cell transplantation with reduced toxicity conditioning regimen in mitochondrial neurogastrointestinal encephalopathy syndrome.

Background: Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disorder due to mutations in the TYMP gene. Clinical findings are characterized by neurologic manifestations and severe gastrointestinal dysfunction. The syndrome is usually fatal, the most effective treatment appears to be hematopoietic stem cell transplantation (HSCT).

Long-term kidney outcomes in children after allogeneic hematopoietic stem cell transplantation assessed with estimated glomerular filtration rate equations, creatinine levels, and cystatin C levels.

Background And Objective: With the widespread use of allogeneic hematopoietic stem cell transplantation (allo-HSCT), long-term complications have come to the fore. The aim of this study was to determine the prevalence and risk factors of chronic kidney disease (CKD) developing in the long term in patients who underwent allo-HSCT in childhood and also to investigate the superiority of eGFR formulas.

Methods: The present study evaluated CKD in patients who underwent allo-HSCT.

Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience.

We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for thalassemia major (TM) patients in Turkey on behalf of the Turkish Pediatric Stem Cell Transplantation Group. We retrospectively enrolled 1469 patients with TM who underwent their first HSCT between 1988 and 2020 in 25 pediatric centers in Turkey. The median follow-up duration and transplant ages were 62 months and 7 years, respectively; 113 patients had chronic graft versus host disease (cGVHD) and the cGVHD rate was 8.

Combined immunodeficiency with marginal zone lymphoma due to a novel homozygous mutation in gene and successful treatment with hematopoietic stem cell transplantation.

Mutations in the interleukin-21 receptor (IL-21R) gene are recently defined as primary immunodeficiency diseases. IL-21R defects result in combined immunodeficiency by affecting the functions of innate and adaptive immune system components.A six-year-old girl was admitted to our hospital with complaints of chronic diarrhea that started after the newborn period and generalized rash over the last three months.

Metabolic syndrome and risk factors after hematopoietic stem cell transplantation in children and adolescents.

Objectives: The early and late complications after hematopoietic stem cell transplantation (HSCT) determine the patients' prognosis and life quality. We aim to determine the metabolic syndrome development frequency after HSCT in children to find out the risk factors and compare them with healthy adolescents.

Methods: Thirty-six children who underwent HSCT at least two years ago were analyzed prospectively and cross-sectionally.

Severe Rare Bleeding Disorders: A Single-center Experience.

Although rare bleeding disorders (RBDs) are not common diseases, they are important for life-threatening bleedings and prophylaxis approaches, especially in severe forms. In this retrospective study, the authors have analyzed data from children with severe RBDs who were examined at the center over a period of 10 years to describe the distribution, clinical features, treatment patterns, and outcome of severe RBDs in patients. Data from all children (age under 18 y) with RBDs who were examined in the center between 2005 and 2015 were retrospectively reviewed.

Non-typhoidal bacteraemia in paediatric leukaemia patients.

Non-typhoidal (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by in paediatric leukaemia patients, which has been reported for the first time in both Europe and the US. According to our research, NTS might be a cause of serious infections in paediatric haematology-oncology patients.

LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY.

Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis.

Case Description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later.

Varicella-Zoster Virus Infections in Pediatric Malignancy Patients: A Seven-Year Analysis.

Primary varicella-zoster virus (VZV) infection is a benign self-limited disease. In this study, we review our experience in focusing on the outcome and treatment of VZV infection in pediatric malignancy patients. During the study period, a total of 41 patients with pediatric malignancy had been hospitalized with the diagnosis of VZV infection.

Pediatric langerhans cell histiocytosis: single center experience over a 17-year period.

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.

A 7-year study of the distribution of nosocomial candidemia in children with cancer.

Candidemia is an important cause of morbidity and mortality in cancer patients. The incidence of candidemia has been reported to have shifted toward nonalbicans species. The aim of this study was to determine the distribution of Candida species resulting in bloodstream infections or catheter-related blood stream infections (CRBSIs) in pediatric hematology-oncology (PHO) patients over a 7-year-period.

Successful immune tolerance induction with low-dose coagulation factor VIII in a patient with hemophilia A from a developing country.

Inhibitor development is the most frequent and serious complication of the treatment in patients with hemophilia. Immune tolerance induction (ITI) is the only option of treatment for the eradication of factor VIII (FVIII) inhibitor. We would like to present our case with hemophilia whose FVIII inhibitor eradication was done by a low-dose ITI regimen.

Half-and-half nails in a pediatric patient after chemotherapy.

Half-and-half nail, characterized by a reddish brown distal band with a sharply demarcated white proximal band, is a specific manifestation of chronic kidney disease, but it is unusual to occur after chemotherapy. We report a seven-year-old girl who developed half-and-half nails in her fingers one month after treatment with modified Berlin-Frankfurt-Munster protocol followed by maintenance therapy with oral methotrexate and 6-mercaptopurine for pre-B acute lymphoblastic leukemia.

Acute generalized exanthematous pustulosis induced by ceftriaxone use.

Acute generalized exanthematous pustulosis (AGEP) is a rare cutaneous rash characterized by the abrupt onset of a generalized pustular rash often accompanied by fever. There is a history of drug use in 90% of the cases. Here we have reported a 15-year-old male patient with sickle cell anemia who developed AGEP after the use of ceftriaxone.

Congenital amegakaryocytic thrombocytopenia: three case reports from patients with different clinical diagnoses and somatic abnormalities.

The congenital amegakaryocytic thrombocytopenia (CAMT) is a syndrome characterized by preservation of granulocytic and erythroid cells during genesis, with a gradual or progressive decrease in the number of megakaryocytic series of cells in the bone marrow. At later times, most patients develop aplastic anemia. It is important to rule out specific causes of thrombocytopenia that develop in the early stages of CAMT.

The effectiveness of tools for monitoring hemophilic arthropathy.

Introduction: Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health.