A Little Known but Very Common Phenotype in Patients with Severe Congenital Neutropenia due to HAX1 Deficiency: Premature Ovarian Insufficiency.

Background: Autosomal recessive severe congenital neutropenia (SCN) has been associated with homozygous variants in the HAX1 gene. The aim of this cross-sectional study was to evaluate the gonadal function and pubertal development in pediatric patients with SCN due to HAX1 gene variant (HAX1-SCN).

Methods: Forty-five patients, including 24 females (median age 11.

Evaluation of Clinical and Hematological Findings of Patients with Acute and Reactivated EBV Infection.

Epstein-Barr virus (EBV) is a latent DNA virus that infects B lymphocytes, can cause widespread hematological findings, and can cause various disease manifestations. In our study, it was aimed to the clinical and hematological findings of those in the pediatric age group patients with a primary EBV infection and those with EBV reactivation. The files of the patients whose EBV serology was evaluated and followed up with the diagnosis of EBV.

Long-term evaluation of neurological outcomes in chemotherapy-associated posterior reversible encephalopathy (PRES) syndrome in children.

Background: Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome with numerous etiologies, mostly characterized by magnetic resonance imaging (MRI) abnormalities in the posterior cerebral white and gray matter and acute neurological symptoms.

Aim: To examine the predisposing factors, clinical results, and radiological features of PRES in children diagnosed with malignancy.

Materials And Methods: The study included 20 patients (7 F/13 M) aged 4-16 years at the time of diagnosis who were diagnosed with malignancy and developed PRES during chemotherapy.

Risk factors for neurologic sequelae in children and adolescents with hemophilia after intracranial hemorrhage.

Background: Intracranial hemorrhage (ICH) is reportedly rare but has high morbidity and mortality risk in persons with hemophilia. Although the risk factors that facilitate bleeding are known, the factors affecting the sequelae are not well known.

Objectives: We planned to investigate the risk factors for neurologic sequelae in children and adolescents with hemophilia suffering from ICH.

Assessment of Mothers' Shaking Behavior of their Babies and Related Factors: An Experimental Approach Using an Accelerometer and an Infant Manikin.

Background: Our study aimed to investigate the factors associated with mothers' shaking behavior of their babies.

Methods: Sixty-three mothers who stated that they shook their babies (Group B) and 91 mothers who stated that they did not (Group A) among those who applied to or were followed up from the Pediatric Outpatient Clinics of our University Hospital were included. The mothers (with a baby aged 0-30 months) completed the Brief Symptom Inventory (BSI) and psychiatric rating scales measuring attachment styles, empathic tendency, perceived social support, and self-esteem.