Morphometric properties of the tensor fascia lata muscle in human foetuses

Background: In neonatal and early childhood surgeries such as meningomyelocele repairs, closing deep wounds and oncological treatment, tensor fasciae lata (TFL) flaps are used. However, there are not enough data about structural properties of TFL in foetuses, which can be considered as the closest to neonates in terms of sampling. This study’s main objective is to gather data about morphological structures of TFL in human foetuses to be used in newborn surgery. Materials and methods: Fifty formalin-fixed foetuses (24 male, 26 female) with gestational age ranging from 18 to 30 weeks (mean 22.94 ± 3.23 weeks) were included in the study. TFL samples were obtained by bilateral dissection and then surface area, width and length parameters were recorded. Digital callipers were used for length and width measurements whereas surface area was calculated using digital image analysis software. Results: No statistically significant differences were found in terms of numerical value of parameters between sides and sexes (p > 0.05). Linear functions for TFL surface area, width, anterior and posterior margin lengths were calculated as y = –225.652 + 14.417 × age (weeks), y = –5.571 + 0.595 × age (weeks), y = –4.276 + 0.909 × age (weeks), and y = –4.468 + 0.779 × age (weeks), respectively. Conclusions: Linear functions for TFL surface area, width and lengths can be used in designing TFL flap dimensions in newborn surgery. In addition, using those described linear functions can also be beneficial in prediction of TFL flap dimensions in autopsy studies

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM)

PubMedID: 30300449Objectives: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (?100 mL/kg of pRBC or a serum ferritin [SF] level >1000 µg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 µg/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 µg/L), SCA (1655.5 to 1260 µg/L), and across age groups of 2-6 years (1971.5 to 1499 µg/L), 7-12 years (1688.5 to 1159.8 µg/L), and 13-18 years (1496.5 to 1107 µg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ?30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (?30 mg/kg/d) may be required to achieve iron balance. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Lt

Neonatal effects of thyroid diseases in pregnancy and approach to the infant with increased TSH: Turkish neonatal and pediatric endocrinology and diabetes societies consensus report

Thyroid functions in the fetus and newborn carry importance in terms of the baby’s health and development of the central nervous system. Maternal iodine deficiency, exposure to iodine, thyroid diseases (Hashimoto thyroiditis, Graves’) and drugs used by the mother affect thyroid functions in the fetus. Reflections of these effects are observed immediately after delivery. Investigation of the mother in terms of thyroid diseases during pregnancy, recognition and appropriate assessment of the required conditions, screening of all newborns in the first days of life in terms of congenital hypothyroidism, timely and appropriate evaluation of the screening results, early diagnosis and appropriate treatment of cases of congenital hypothyroidism, assessment and management of cases of transient thyroid hormone disorders and close monitoring of the thyroid functions and development of patients in whom treatment has been initiated with a diagnosis of hypothyroidism are crucial in terms of developmental outcomes of the babies who have thyroid function disorders or hypothyroidism. This guideline was written with the objective of guiding pediatricians, neonatologists and pediatric endocrinologists in the issue of assessment, diagnosis and management of thyroid function disorders and thyroid diseases concerning the fetus and baby during gestation and neonatal period. © Copyright 2018 by Turkish Pediatric Association

Mental status and physical activity in patients with homozygous familial hypercholesterolemia: A subgroup analysis of a nationwide survey (A-HIT1 registry)

BACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening disease due to high serum low-density lipoprotein (LDL) cholesterol levels. LDL cholesterol-lowering interventions are fundamental for patients with HoFH

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM)

Objectives: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (≥100 mL/kg of pRBC or a serum ferritin [SF] level >1000 μg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 μg/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 μg/L), SCA (1655.5 to 1260 μg/L), and across age groups of 2-6 years (1971.5 to 1499 μg/L), 7-12 years (1688.5 to 1159.8 μg/L), and 13-18 years (1496.5 to 1107 μg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ≥30 mg/kg/d (n = 120, −579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (≥30 mg/kg/d) may be required to achieve iron balance. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Lt

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM).

OBJECTIVES: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. METHODS: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (≥100 mL/kg of pRBC or a serum ferritin [SF] level >1000 μg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. RESULTS: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 μg/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 μg/L), SCA (1655.5 to 1260 μg/L), and across age groups of 2-6 years (1971.5 to 1499 μg/L), 7-12 years (1688.5 to 1159.8 μg/L), and 13-18 years (1496.5 to 1107 μg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ≥30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. CONCLUSIONS: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (≥30 mg/kg/d) may be required to achieve iron balance