The Relationship Between Serum Lactate Dehydrogenase Enzyme Levels and Myeloid Engraftment in Hematopoietic Stem Cell Transplantation.

Background: The early detection of myeloid engraftment after hematopoietic stem cell transplantation (HSCT) is of clinical importance for clinicians. In this study, we evaluated whether serum lactic dehydrogenase enzyme levels are significant predictors in the early detection of successful myeloid engraftment after HSCT.

Methods: The study included 74 patients, all of whom underwent HSCT between February 2014 and June 2020.

Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?

Background: 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.

The Relationship Between Premature Adrenarche and Platelet Aggregation.

Objective: Premature adrenarche (PA) has been associated with an increase in adrenal androgens, and the hyperandrogenic hormonal environment is known to lead to increased platelet (PLT) aggregation. Here, we evaluated the effects of PA on PLT aggregation in PLT-rich plasma samples from female patients.

Methods: The study included 40 female patients diagnosed with PA between February, 2014 and June, 2018 and 30 healthy female individuals as a control group.

Valproic acid-induced DRESS in a child responding to cyclosporine with HLA analysis.

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome, is a potentially life-threatening rare reaction that causes a severe rash and can lead to multiorgan failure. As in other severe drug eruptions, drug-specific T lymphocytes play a crucial role in DRESS. The hapten/pro-hapten model, pharmacological interaction model, and altered peptide repertoire model are three different models developed to describe the relationship/interaction between a medication or its metabolites and the immune system.

The impact of initial hematocrit values after birth on peri-/intraventricular hemorrhage in extremely low birth weight neonates.

Aim: Peri-/intaventricular hemorrhage (P/IVH) is a common condition in preterm neonates and is responsible for substantial adverse neurological outcome especially in extremely low birth weight infants. As hematocrit after birth is a surrogate marker for blood volume, this study aimed to evaluate the effect of initial hematocrit values after birth on P/IVH development in extreme low birth weight (ELBW) neonates.

Patients And Methods: A prospective cohort analysis of 92 eligible ELBW neonates was performed.

Tolvaptan Response in a Hyponatremic Newborn with Syndrome of Inappropriate Secretion of Antidiuretic Hormone.

The use of tolvaptan to treat both euvolemic and hypervolemic hyponatremia has rapidly increased in recent years. However, data on its effects on children, especially newborns and infants, are limited. Here, we present a newborn who developed syndrome of inappropriate secretion of antidiuretic hormone following an intracranial hematoma drainage operation who was unresponsive to conventional treatments.

Collapsing Glomerulopathy in a Child with Galloway-Mowat Syndrome.

Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome.

Elevated Urinary T Helper 1 Chemokine Levels in Newly Diagnosed Hypertensive Obese Children.

Objective: Increasing evidence suggests that T helper (Th) cells play a significant role in the pathogenesis of hypertension. The aim of this study was to evaluate the effect of obesity and anti-hypertensive treatment on urinary Th1 chemokines.

Methods: The study groups consisted of three types of patients: hypertensive obese, healthy, and non-hypertensive obese.

A new mutation in blau syndrome.

Blau syndrome is a rare, autosomal dominant, granulomatous autoinflammatory disease. The classic triad of the disease includes recurrent uveitis, granulomatous dermatitis, and symmetrical arthritis. Blau syndrome is related to mutations located at the 16q12.

Acquired bartter-like syndrome associated with colistin use in a preterm infant.

Acquired Bartter-like syndrome (BLS), characterized by hypokalemic metabolic alkalosis, hypomagnesemia, hypocalcemia, and normal kidney function, can be induced by diuretics or antibiotics. It is a very rare condition and only anecdotal cases mostly in adults were reported. Although tubulopathy associated with colistin was reported in adults, to the best of our knowledge, colistin-associated BLS neither in adults nor in children has been reported in the literature.

Two novel deletions in hypotonia-cystinuria syndrome.

Hypotonia-cystinuria syndrome (HCS) is an autosomal recessive disorder caused by combined deletions of SLC3A1 and PREPL. Clinical features include cystinuria, neonatal hypotonia with spontaneous improvement, poor feeding in neonates, hyperphagia in childhood, growth hormone deficiency, and variable cognitive problems. Only 14 families with 6 different deletions have been reported.