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STRECTHED PENILE LENGTH NOMOGRAM AMONG SOUTH INDIAN CHILDREN OF 0-12 AGE GROUP Dissertation submitted for M.D., DEGREE EXAMINATION BRANCH VII PEDIATRIC MEDICINE THE TAMIL NADU DR.M.G.R MEDICAL UNIVERSITY CHENNAI INSTITUTE OF CHILD HEALTH AND HOSPITAL FOR CHILDREN MADRAS MEDICAL COLLEGE CHENNAI APRIL 2020 CERTIFICATE This is to certify that the dissertation titled “TO ESTABLISH STRETCHED PENILE LENGTH NOMOGRAM FOR SOUTH INDIAN CHILDREN IN 0-12 AGE GROUP” submitted by Dr. TAMIZHARASAN E to the Faculty of Paediatrics, THE TAMILNADU DR.M.G.R. MEDICAL UNIVERSITY, CHENNAI, in partial fulfillment of the requirements for the award of M.D., DEGREE (PEDIATRICS) is a bonafide research work carried out by him under our direct supervision and guidance. PROF.DR.K.JAYACHANDRAN, PROF.DR.ELILARASI.MD.,DCH, MD.,DCH, Director & Superintendent, Professor of Paediatrics, Institute of Child Health & Institute of child health & Hospital for Children, Hospital for children, Madras Medical College, Chennai- 600 008. Chennai – 600 008. PROF.DR.R.JAYANTHI M.D.,F.R.C.P(Glasg) The DEAN, Madras Medical College & Rajiv Gandhi Govt. General Hospital, Chennai – 600 003 DECLARATION I, DR.TAMIZHARASAN E solemnly declare that the dissertation titled “TO ESTABLISH STRETCHED PENILE LENGTH NOMOGRAM IN SOUTH INDIAN CHILDREN IN 0-12 AGE GROUP” has been prepared by me. This is submitted to the Tamil Nadu DR.M.G.R Medical University, in partial fulfillment of the rules and regulations for the M.D Degree examination in Paediatrics. Place : Chennai DR.TAMIZHARASAN E Date : SPECIAL ACKNOWLEDGEMENT My sincere thanks to PROF. DR.R.JAYANTHI M.D., F.R.C.P (Glasgow) , Dean, Madras Medical College, for allowing me to do this dissertation, utilizing the institutional facilities. ACKNOWLEDGEMENT It is with immense pleasure and privilege, I express my heartfelt gratitude, admiration and sincere thanks to PROF. Dr.S.EZHILARASI , M.D., DCH., Professor and Head of the Department of Paediatrics and our Director, for his guidance and support during this study. I am greatly indebted to my guide and teacher, PROF. Dr.K.JAYACHANDRAN, M.D., DCH., Professor of Paediatrics for his supervision, guidance and encouragement while undertaking this study. I would like to thank my Assistant Professors Dr.RAM KUMAR, Dr.N.BALAKRISHNAN, Dr.V.SEENIVASAN, Dr.M.S.MANI and Dr. S.P.KARAMATH for their valuable suggestions and support. I also thank all the members of the Dissertation Committee for their valuable suggestions. I gratefully acknowledge the help and guidance received from Dr. S. SRINIVASAN, DCH., Registrar at every stage of this study. I also express my gratitude to all my fellow postgraduates for their kind cooperation in carrying out this study and for their critical analysis. I thank the Dean and the members of Ethical Committee, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai for permitting me to perform this study. I thank all the parents and children who have ungrudgingly lent themselves to undergo this study without whom, this study would not have seen the light of the day. ETHICAL COMMITTEE APPROVAL FORM PLAGIARISM CERTIFICATE This is to certify that this dissertation work titled “TO ESTABLISH STRETCHED PENILE LENGTH NOMOGRAM IN SOUTH INDIAN CHILDREN IN 0-12 AGE GROUP” of the candidate DR. TAMIZHARASAN E with Registration Number 201717013 for the award of M.D. in the branch of PAEDIATRICS. I personally verified the urkund.com website for the purpose of plagiarism check. I found that the uploaded thesis file contains from introduction to conclusion pages and result shows 0 percentage of plagiarism in the dissertation. GUIDE AND SUPERVISOR SIGN WITH SEAL LIST OF ABBREVIATIONS ICH & HC - Institute of child health and hospital for children , Egmore. SPL - Stretched penile length T - testosterone LH - luetinising hormone FSH - follicular stimulating hormone GnRH - gonadotrophin releasing hormone AMH - anti mullerian hormone CAH - congenital adrenal hyperplasia INSL3 - insulin like growth hormone 3 GH - growth hormone DHT - dihydro testosterone HCG - human chorionic gonadotrophin IUGR - intra uterine growth restriction AD - Autosomal dominant AR - autosomal recessive DSD - disorders of sexual differentiation BETA HSD - hydroxy steroid dehydrogenase Cm - centimeter Mg - milligram Mm - millimeter M - months Y - years CONTENTS SL.NO. TITLES PAGE.NO. 1 INTRODUCTION 1 – 19 2 REVIEW OF LITERATURE 20 – 36 3 STUDY JUSTIFICATION 37 4 OBJECTIVE 38 5 MATERIALS AND METHODS 39 – 40 6 STUDY MANOEUVRE 41 7 STATISTICAL ANALYSIS 42 – 43 8 RESULTS 44 – 68 9 DISCUSSION 69 – 73 10 LIMITATIONS 74 11 CONCLUSION 75 12 BIBLIOGRAPHY 76 – 78 13 ANNEXURES 79 – 119 ● PROFORMA ● INFORMATION SHEET ● CONSENT FORM ● MASTER CHART WITH KEY INTRODUCTION One of the important concerns in neonates’ physical examination is the genital examination, especially penile size that is considered an important representation of hypothalamic or pituitary abnormality (hypogonadotropic hypogonadism); due to the role of androgen exposure in fetal sex development, disorders in genital system may be a sign of disorders of sex development (DSDs). Thus, penile measurement contributes to the diagnosis of the underlying genetic or endocrine disorders. In addition to diagnosis of hypothalamic and pituitary defects, measurement of PL is important in procedures, such as circumcision, as well. Micropenis is defined as a small penis, without epispadias or hypospadias, 2.5 SDs below the mean PL. Pure micropenis, not associated with DSDs, benefit from early intervention, especially in minipuberty stage of below 6 months of age, when low dose testosterone can improve the penile length significantly. Therefore, the accuracy of PL measurement and cut-off for the definition of micropenis are of great importance, commonly missed in early physical examination of the newborn. Stretched penile length (SPL) is basically put on nomograms, designed based on gestational age, weight, and height; accordingly, PL values are different for preterm infants, as well as different ethnicities. Paediatricians often encounter questions from parents regarding the normal size of their child's penis. Aberrant growth of male external genitalia may be the first sign of underlying biophysiologic or psychosocial illness. Medical 1 consultations regarding these have associated medical, sexual, psychological, and social implications. Morphological abnormalities of the penis can affect interpersonal relations and provoke emotional disturbances as the child grows into an adult. With relation to the genital size, it may be the child or parents who are suffering from a misconception, when all that is required is the knowledge of normal variation. At the same time, one must rule out micropenis, which is defined as a penis that is normal in terms of shape and function, but is 2.5 SD smaller than the mean size in terms of length for age of the child. MICROPENIS Micropenis is unusual but can result from multiple hormonal abnormalities. It constitutes 1.8% of all congenital penile anomalies .True micropenis is unusual and should be distinguished from a buried or webbed penis. 2 Webbed Penis When a web of skin obscures the penoscrotal angle in an otherwise normal sized penoscrotal shaft it is called a webbed penis. It is also known as penoscrotal fusion. This could be congenital or acquired. Congenital webbed penis is a very rare condition and is often associated with and is associated with penile and scrotal hypoplasia. Iatrogenic causes included excessive removal of skin during surgery. Embryological explanation for a webbed penis could be the failure of posterior migration of labioscrotal folds or there may be a disturbance in the development of the prepuce. During development the glans id initially devoid of any covering and as development progresses the ectodermal layer on the dorsal aspect of the penis grows and ultimately covers the entire penis. If there is a disturbance in this phase it may result in penoscrotal fusion. 3 Buried Penis When the penis lacking its proper sheath of skin ,lies buried beneath the integument of the abdomen , thigh or scrotum it is known as buried penis as defined by Keyes in 1919. These children may present with complains of poorly controlled urinary streams. They may have to undergo penile degloving and penile fixation 4 Normal penile development requires the appropriate timing and secretion of testosterone from Leydig cells. During the first 3 months of gestation, the process is mediated through placental hCG. In the 4th month, with the penis being fully formed, LH and follicle-stimulating hormone (FSH) stimulate testosterone secretion. This process is driven by gonadotropin-releasing hormone (GnRH), which is secreted by the fetal hypothalamus. Micropenis results from failure of the normal stimulation of penile growth after 14 weeks of gestation. Two major causes of abnormal hormonal stimulation are: 1. Inadequate secretion of GnRH (hypogonadotropic hypogonadism), which is the most common cause of micropenis. 2. Failure of the testes to produce testosterone. Micropenis strongly suggests central hypogonadism, a defect in androgen production (or) action (or) a defect in growth hormone signalling& as such should be evaluated early. Fetal hypogonadism: primary / central or combined of 2nd half of gestation typically results in micropenis & cryptorchidism reflecting the defective action of testosterone , INSL3 in a period where this testicular hormone are essential for testicular decent & growth of penis & scrotum. An isolated micropenis