Dyshormonogenesis Emerging As a Predominant Cause of Congenital Journal of Hypothy-Roidism in India - a Mini Review
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Prabhu SR. Dyshormonogenesis emerging as a predominant cause of Congenital Journal of Hypothy-roidism in India - A Mini Review. J Pediatr Pediatr Med. 2018 2(4): 1-6 Journal of ; Pediatrics and Journal of Pediatrics and Pediatric Medicine Pediatric Medicine Mini Review Open Access Dyshormonogenesis Emerging as a Predominant Cause of Congenital Hypothyroidism in India - A Mini Review Sudha Rathna Prabhu* The Tamil Nadu Dr MGR Medical University , Chennai , Tamil Nadu , India ABSTRACT Article Info Congenital hypothyroidism (CH) is one of the most common preventable and Article Notes Received: May 02, 2018 treatable cause of intellectual impairment in children. Among known etiologies Accepted: July 04, 2018 dysgenesis due to abnormal anatomical development and dyshormonogenesis as a result of deranged physiological functioning of fetal thyroid gland. account *Correspondence: for at least 90 % of causes of CH. While review of literature report thyroid Dr. Sudha Rathna Prabhu, MBBS, DNB, (PhD), Pediatrician dysgenesis as the most common etiology of CH currently focus is on iodine and Research Scholar in Maternal And Child Health at status adequacy in women before and during pregnancy and after delivery and Institute of Obstetrics and Gynaecology Hospital for Women & Institute of Child Health and Hospital for Children, Egmore, lactation. Worldwide reports of previously iodine sufficient countries declared Chennai, Tamil Nadu, India; Affiliated to The Tamil Nadu Dr presently as iodine insufficient and recent publications of several Indian states MGR Medical University, Chennai, Tamil Nadu, India; consuming lesser amounts of iodized salt seem to indicate that iodine related Email: [email protected]; pathophysiological factors are emerging as predominant etiologies of CH. The [email protected]. most vulnerable pregnant women and newborns are prone to be affected with iodine imbalances leading to pregnancy and perinatal related complications. © 2018 Prabhu SR. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License. Analysis of demographic, biochemical, clinical and statistical data by Indian Council of Medical Research (ICMR) in a multi-centric pilot study on congenital hypothyroidism has clearly shown that dyshormonogenesis is a leading cause of CH in neonates born in India. The need of the hour is to consider mandatory newborn screening for CH in all live newborns and further conduct country wise Keywords Congenital hypothyroidism ethnic and culture oriented research studies with special emphasis on iodine Iodine deficiency disorders status, genetic predispositions and lifestyle changes impact on all vulnerable Dyshormonogenesis populations and design effective early therapeutic strategies for management Intellectual impairment of CH to prevent intellectual impairment. Iodized salt; Goiter Urinary iodine levels Introduction Globally, malnutrition both as under and over nutrition is a major health hazard and the most vulnerable are women and children1. Among essential micronutrients iodine is vital for normal development of thyroid gland. Most of the iodine uptake takes place inside thyroid gland and hormones synthesis and functions are entirely dependent on the bioavailability of iodine in consumed diet. Risk factors causing iodine imbalances are thus prone to affect thyroid gland and initiate a cascade of biochemical changes. This leads further to a spectrum of endocrine related metabolic vulnerable human biological systems and pose a constant threat to thedisorders entire referredlife time ofto anas individualiodine deficiency from in disordersutero conception (IDD) within stage cause of preventable brain damage2,3 and most of consequences of till senility. Worldwide iodine deficiency constitute the single1 .largest When predisposing socio demographic factors favouring malnutrition operateIDD are irreversiblewithin vulnerable but preventable population when groups, intervened detrimental early effects are inevitable and most severe health impact occurs in maternal Page 1 of 6 Prabhu SR. Dyshormonogenesis emerging as a predominant cause of Congenital Journal of Pediatrics and Pediatric Medicine Hypothyroidism in India - A Mini Review. J Pediatr Pediatr Med. 2018; 2(4): 1-6 and neonatal populations. Congenital hypothyroidism disorders may be dysgenetic due to anatomic defects or dyshormonogenetic due to impaired physiological endocrine cause of mental impairment in children and is maturation , synthesis and functioning of thyroid hormones (CH) is one of the most common preventable1-3 resulting and in decreased treatable and / or absence of biological actions of the thyroid gland. usually caused by iodine deficiency cells.Thyroid form gland thyroid is usually anlage visible by embryonic day (E)evolves E20 Risk Factors in CH to E22 when “specification”15 of endodermal epithelium While Western literature report dysgenesis as most thyroid gland development and.Normal maturation embryology with evolution in its first stage around two processes which are fetal in this process results in thyroid agenesis. As a second common etiology of CH, dyshormonogenesis (DH) seems stageof the ofhypothalamic-pituitary-thyroid early morphogenesis thyroid anlageaxis and forms any defecta bud tocommoner dyshormonogenesis in Indian population. particularly Recently in Asian published population studies4-6. that proliferates and migrates and then becomes bilobed are reporting increase in CH prevalence predominantly due results in hemi agenesis while an impaired descent causes Iodine deficiency is on the rise and as per National Health anaround ectopic day typeE48 ofto thyroidE50. Any tissue error or. Thefaulty physiological lobulation and Nutrition Examination Survey (NHANES 2005–2008) component of thyroid hormones secretions15-17 begins around survey in USA ,57% of pregnant women had urinary iodine levels7 less than 150 mcg /L suggestive of iodine receptors initiate their production. The maturity of gland haveinsufficiency an inadequate.As per iodine recent intake global and estimate, over half the1.88 children billion 10-11 weeks of age when thyroid peroxidase thyrotropin people are at risk of iodine deficiency. 241 million children Africa , occurs in and around 2516. weeksAt this of stagegestation defects as is inevidenced protein therewith insufficient 8,9is a gradual iodine rise in intake number live of in children South-East with Asia thyroid and1,6,8 synthesisby a progressive will result rise inin thedyshormonogenesis ratio of free thyroxine a disorder (FT4) dyshormonogenesis. Among Indian compared populations to thyroid based dysgenesis.studies ofto thethyrotropin thyroid gland(TSH) resulting in a deranged maturation Major regional risk factors attributed and observed in synthesis or disorderly functioning of gland resulting in are consanguineous impaired thyroid hormones synthesis . marriages, practice of marriages 10,11among natives of the 16,17 ICMR pilot study at Chennai centre Global status: According to WHO and International same district and caste groups particularly 12in South India. in siblings has been reported by the author and this mini Council for the Control of Iodine Deficiency Disorders A scientific letter on dyshormonogenesis as etiology of CH differences in incidence of congenital hypothyroidism review is an attempt to further unfold etiopathogenesis of (ICCIDD) worldwide data on iodine status, regional 1,2. are suggested to be more likely due to iodine deficiency congenitalConcept hypothyroidism of “Golden Period” in Indian for population. intervention: The time period from conception till two years of age represents thethyroid globe disorders , research rather studies than on etiopathologicalethnic affiliation factorsIodine of deficiency is the main cause of goiter in all ages. Across environmentalthe first 1000 days factors of a baby during and isembryonic referred to asand “GOLDEN foetal primary congenital hypothyroidism ( PCH ) report that developmentPERIOD “when with fetal risk programming of diseases in links later nutritionallife13 and dyshormonogenesis80 % to 85 % are due as to athyroid result agenesis of defective , dysgenesis enzymatic and deiodinationectopic thyroid metabolic while remaining disorders 15 %within to 20 %the are thyroid due to period provide best of health outcomes of affected. Detection babies. gland and treatment of specific diseases during this crucial from 7-9Turkey will help develop their cognitive abilities. This normal .In contrast18 to several previous results , a study. In normal children exposure to time oriented stimuli has reported thyroid dysgenesis in 52.2% andTamam dyshormonogenesis et al in which dyshormonogenesisin 47.8% of the study was population etiology becomephenomenon adults. occurs The same because phenomenon a direct link applies exists to betweenchildren Similar results19 were observed in another study by early exposure and level of success of these children as they who are diagnosed with congenital hypothyroidism during hypothyroidism. Multiple factors which include mainly familial,in 44% ofmaternal, subjects neonatalwith permanent and environmental primary congenital factors is available for potential interventions and for effective seem to have combined etiological roles in occurrence of healththe same outcomes GOLDEN14. PERIODA mini review when on “window thyroid of gland opportunity” anatomy, physiological factors operating during embryonic stages of CH.Current status in India: developmentembryology