The relationship between the length of umbilical cord and neonatal outcomes

Maternal outcomes and prenatal outcomes depend on the mutual reactions between maternal and fetal and environmental factors. One of the influential cases in this field is the funis length. A descriptive analytic study was conducted on 300 pregnant women, who had been admitted in labor section. After placenta expulsion, the length of the funis, placental and birth weight were measured. Gestational age was determined according to maternal LMP and sonography of pregnancy. Meconial amniotic fluid was determined through labor examination in the amniotomy. The result revealed meaningful differences between neonatal height, placental and neonatal weight. No relationship was revealed between the length of funis and the sex of neonate, the situation of Amniotic fluid, and the first minute Apgar score. Considering the deficient that may be available in this study, it is recommended that a study be done in this fiend in other regions of the country and in other countries, in employed women, and in larger samples so that we can achieve comparative or new results in this regard

Clinical Features and Management of Cartilage-Hair Hypoplasia: A Narrative Review

Context: Cartilage-hair hypoplasia is a rare hereditary cause of short stature. The aim of this study was to familiarize physicians with this rare but important disease. Evidence Acquisition: This article is a narrative review of the scientific literature to inform about clinical features and management of Cartilage-hair hypoplasia. A systematic search identified 127 papers include original and review articles and case reports. Results: Cartilage-Hair Hypoplasia characterized by short-limb dwarfism associated with metaphyseal chondrodysplasia. The inheritance is autosomal recessive. Other findings include hair hypoplasia, anemia, immunodeficiency, propensity to infections, gastrointestinal disorders (Hirschsprung disease, anal stenosis, esophageal atresia and malabsorption), defective spermatogenesis, increased risk of malignancies and higher rate of mortality. Immunodeficiency in cartilage-hair hypoplasia may be an isolated B-cell or isolated T-cell immunodeficiency or combined B and T-cell immunodeficiency; however, severe combined immunodeficiency is rare. There is no known treatment for hair hypoplasia. Growth hormone was used with conflicting results for short stature in children with Cartilage-hair hypoplasia. Skeletal problems must be managed with physiotherapy and appropriate orthopedic interventions. Hirschsprung disease, anal stenosis and esophageal atresia should be surgically corrected. Patients with severe hypoplastic anemia require repeated transfusions. Bone marrow transplantation may be required for patients with severe combined immunodeficiency or severe persistent hypoplastic anemia. Treatment with G-CSF is useful for neutropenia. Patients should be monitored closely for developing malignancy such as skin neoplasms, lymphomas and leukemias. Conclusions: Cartilage-hair hypoplasia is an important hereditary disease with different medical aspects. The high rate of consanguineous marriages in Iran necessitates considering CHH in any child with severe short stature or other clinical features of disorder

Clinical Features and Management of Cartilage-Hair Hypoplasia: A Narrative Review

Context: Cartilage-hair hypoplasia is a rare hereditary cause of short stature. The aim of this study was to familiarize physicians with this rare but important disease. Evidence Acquisition: This article is a narrative review of the scientific literature to inform about clinical features and management of Cartilage-hair hypoplasia. A systematic search identified 127 papers include original and review articles and case reports. Results: Cartilage-Hair Hypoplasia characterized by short-limb dwarfism associated with metaphyseal chondrodysplasia. The inheritance is autosomal recessive. Other findings include hair hypoplasia, anemia, immunodeficiency, propensity to infections, gastrointestinal disorders (Hirschsprung disease, anal stenosis, esophageal atresia and malabsorption), defective spermatogenesis, increased risk of malignancies and higher rate of mortality. Immunodeficiency in cartilage-hair hypoplasia may be an isolated B-cell or isolated T-cell immunodeficiency or combined B and T-cell immunodeficiency; however, severe combined immunodeficiency is rare. There is no known treatment for hair hypoplasia. Growth hormone was used with conflicting results for short stature in children with Cartilage-hair hypoplasia. Skeletal problems must be managed with physiotherapy and appropriate orthopedic interventions. Hirschsprung disease, anal stenosis and esophageal atresia should be surgically corrected. Patients with severe hypoplastic anemia require repeated transfusions. Bone marrow transplantation may be required for patients with severe combined immunodeficiency or severe persistent hypoplastic anemia. Treatment with G-CSF is useful for neutropenia. Patients should be monitored closely for developing malignancy such as skin neoplasms, lymphomas and leukemias. Conclusions: Cartilage-hair hypoplasia is an important hereditary disease with different medical aspects. The high rate of consanguineous marriages in Iran necessitates considering CHH in any child with severe short stature or other clinical features of disorder

Etiology and clinical features of precocious puberty in children referred to the endocrinology clinic of Kashan University of Medical Sciences, Iran

Background and Aim: Precocious puberty presents a variety of causes and clinical features, with its prevalence on the rise. This study aimed at investigating the etiology and clinical characteristics of precocious puberty in children referred to the endocrinology clinic of Kashan University of Medical Sciences, Iran. Methods: In this retrospective study, the medical records of children diagnosed with precocious puberty who attended the pediatric endocrinology clinic at Kashan University of Medical Sciences, Iran, between 2007 and 2014, were retrospectively reviewed. A comprehensive history and clinical examination were conducted by a pediatric endocrinologist, along with left hand X-rays and specific hormonal tests. Results: Among the 357 children meeting the inclusion criteria, only two were boys, one with isolated adrenarche and the other with central precocious puberty. Out of the 355 girls, 315 (88.2%) had central precocious puberty, 19 (5.3%) presented with premature thelarche, 20 (5.6%) exhibited pubarche, and three (0.8%) had transient thelarche. Only 172 children maintained regular follow-up visits, with an average follow-up duration of 23±16 months. Of these, 42 were undergoing treatment, while 130 were monitored for the progression of puberty and the necessity for intervention. Over a 3-6-month period, no change in pubertal stage was observed in 49.6% of children, while 42.6% progressed in thelarche or pubarche, and 7.8% advanced in both pubarche and thelarche. Additionally, 24.8% of children experienced a height growth rate exceeding the 97th percentile. Conclusion: Central precocious puberty emerged as the predominant cause of precocious puberty symptoms in this study. Furthermore, more than half of girls with true precocious puberty displayed a slow progressive course that did not warrant therapeutic interventions

Metabolic Bone Disease in Very Low-Birth-Weight Neonates

Background: Metabolic bone diseases (MBD), including rickets and osteopenia, are important neonatal complications among preterm infants. This study aimed to determine the prevalence and risk factors of MBD in neonates with very low birth weight (VLBW). Methods: This prospective study was conducted on VLBW infants from January 2012 to July 2013. Inclusion criteria were birth weight of ≤1500 g and age of ≤7 days, and the exclusion criteria were cholestatic disorders, skeletal anomalies and genetic syndromes. Serum calcium, phosphorus and alkaline phosphatase (ALP) concentrations were measured regularly until the 12th week of birth. In addition, wrist and chest radiographs were obtained from the neonates at 8-12 weeks of age. Results: In total, 58 neonates with the mean gestational age of 30.6±2.65 weeks, weight of 1265±262 g and height of 38.06±2.49 cm were enrolled in this study. The correlation between biochemical parameters in multiple analysis and radiological findings of rickets was examined, and a significant association was observed between serum phosphorus level at the first week of age and the incidence of rickets. Moreover, 14 infants had only one radiologic sign of rickets (e.g. fraying, cupping, widening or lack of provisional zone of calcification (PZC), and 8 subjects (13.7%) showed at least two radiologic signs. The prevalence of osteopenia and rickets among infants with birth weight of 1200 g had normal X-rays (P=0.036). Conclusion: Despite the remarkable advances in the management of VLBW infants, MBD is still a prevalent complication during the neonatal period. According to the results of this study, birth weight and gestational age are the most significant risk factors for MBD