Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma, is characterized by presence malignant cells (Sézary cells, SCs) in both blood and skin. Insights into molecular progression SS have revealed substantial inter- intra-tumoral heterogeneity. Identifying common gene expression signature from different compartments elucidating disease pathogenesis could facilitate diagnosis t...

Background: Children affected with sickle cell disease (SCD) are at increased risk for severe morbidity and mortality, especially during the first 3-5 years of life. It is suggested that early treatment can improve the condition. The aim of this pilot study was to estimate the incidence of hemoglobin S (HbS) by umbilical cord blood screening in Khorramshahr and Abadan cities in southwest of Ira...

The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complicatio...

A young West Indian woman with established sickle cell disease developed a severe episode of sickle chest syndrome. Conventional treatment including exchange transfusions and mechanical ventilation was to no avail, and an infusion of epoprostenol also failed to halt her worsening condition. When her arterial oxygen tension (PaO2) had fallen to 6.5 kPa (49 mm Hg) extracorporeal membrane oxygenat...

Objective(s): Predegeneration is a standard technique to obtain mitotically activated and enriched cultures of Schwann cells (SCs). This study, for the first time, evaluated the impact of various duration of predegeneration on cell yield and enrichment of SCs from dog peripheral nerve. Materials and Methods: Dog sural nerves were subjected to 5, 10, 15 day-long in vitro predegeneration. The tot...

Subclinical Cushing syndrome (SCS) is a hypothalamic-pituitary-adrenal axis abnormality characterized by autonomous cortisol secretion in patients with no typical signs or symptoms of Cushing syndrome. SCS patients may have adverse metabolic and cardiovascular effects due to slight, but continuous glucocorticoid secretion. Glucocorticoids also affect behavior, mood, neural activity, and a numbe...

INTRODUCTION The presence of subclinical Cushing's syndrome (SCS) and some features of the metabolic syndrome were evaluated in adrenal incidentaloma patients. MATERIAL AND METHODS 165 patients were studied. Plasma cortisol, ACTH, DHEA-S, 17-OH-P, aldosterone, renin activity and 24-h urinary methoxycatecholamines were measured. Fasting concentrations of glucose, insulin, triglycerides, T-chol...

The efficacy of hydroxyurea (HU) and its role in the reduction in mortality in sickle cell patients has been established. Nevertheless, many patients still die of complications of this disease while on HU. Of the 226 patients treated with HU at our center, 38 died (34 of sickle cell-related causes). Acute chest syndrome (ACS) was the most common (35%) cause of death. Deceased and surviving pati...