Drug treatment in endocrine disorders

Disorder of sex development DSD In human sexual development, the female phenotype represents the default pathway. therefore, a failure of testis determination results in the development of the female phenotype, while genetic alterations resulting in partial testicular development can give rise to a wide spectrum of masculinization. In addition to defects in peptide hormones and their receptors, timing of hormonal exposure is also critical to appropriate development. Genital anomalies are estimated to occur in 1 in 4500 births. Disorder of sex development DSD is a medical term referring to congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical. Patients with ambiguous genitalia have disorders of sexual development DSD, that previously termed intersex conditions. The term DSD in lieu of potentially pejorative and confusing terms such as intersex, pseudohermaphroditism, hermaphroditism, sex reversal, and gender-based diagnostic labels. For clinical purposes, DSDs in newborns are classified according to karyotype. Based on the various steps of sex differentiation and development, we suggest the following classification: A DSD with a 46,XY karyotype formerly referred to as male pseudohermaphroditism, undervirilization of an XY male and undermasculinization of an XY male. In 46,XY DSD the bipotential gonads differentiate to variable degrees into testes, B DSD with a 46,XX karyotype formerly referred to as female pseudohermaphroditism or masculinization of an XX female. In 46,XX DSD the bipotential gonads usually differentiate into ovaries but with abnormally increased androgen exposure and C DSD with an unusual karyotype such as mosaicism 45,XO46,XY or 46,XX46,XY, transposition of genes 46,XX, SRY+ or chromosome deletions that include one of the genes necessary for sex determination and differentiation. For optimal clinical management of individuals with DSD, the consensus statement recommends the following: Avoid gender assignment in newborns before expert evaluation. Conduct evaluation and long-term management at a center with an experienced multidisciplinary team, including pediatric subspecialists in endocrinology, surgery or urology or both, psychologypsychiatry, gynecology, genetics, neonatology, and, if available, social work, nursing, and medical ethics. Give a gender assignment to all individuals. Openly communicate with patients and families and encourage their participation in decision making. Respect patient and family concerns, and address them in strict confidence.

10. Drug treatment in endocrine disorders

In physiological state, the function of body organs adopts to the activity of the body. Organs that are involved in physiologic adaptation of the body especially in metabolism are the endocrine glands such as the hypophysis, thyroid gland, pancreas and adrenal glands. Dysfunction of the endocrine glands leads to metabolic disturbances, depending on the function of the hormone of the gland and produces specific syndrome. The syndrome can be manage by using specific drugs: hormone or anti hormonal drugs. Many drugs act through pharmacodynamic and pharmakinetics mechanism. For example, drugs are used for managing diabetes mellitus and lipid metabolism disorders. Udayana University Departement of Medicine Education, DME 18 Case : A woman with decreased body weight A woman 22 year old, complained of reduced body weight aggressively in a few weeks, tremble, and diarrhea 2 or 3 times a day. She has slightly enlargement of her neck. Laboratory result showed high level of thyroid hormone FT4free thyroxine and very low level of thyroid stimulating hormone TSHs. Learning Task: 1. Explain the classical endocrine glands and each hormone be secreted. 2. Explain the concept of endocrine, paracrine, and autocrine in hormone action; and each examples of hormone or molecules 3. Explain the concepts of ligand, receptor, effector, and second messenger 4. Based on location of receptor in hormone action, how many kinds of hormone are there? Explain briefly the mechanism each hormone action and example of its hormones 5. Explain two major types of control of endocrine gland function 6. Associated with hypothalamic-pituitary-target gland system, what level of the disorder was occurred at hypothalamus, pituitary or thyroid gland in above case? Draw a figure of the disorder and explain briefly. Self assessment: 1. What is the hormone function in our body, and give example of hormone for each function? 2. Explain the differences between signaling by hormones, local mediators, and neurotransmitters 3. Describe the transport of hormone in the blood 4. What do you know about ligand, receptor, effector, first-second messenger? 5. Explain about the control of hormone secretion Give an example of feed back negative mechanism of hormone secretion 6. Make a list of the endocrine disease Learning Task: 1. Discuss the structure of thyroid and parathyroid gland, include the vascularisation and identify their position in the neck 2. Discuss anatomical structures of pituitary gland and hypothalamus 3. Discuss anatomical structures of pancreas 4. Discuss anatomical structures of adrenal gland Self assessment: 1. Decribe the structure of thyroid and parathyroid gland, include the vascularisation and identify their position in the neck 2. Describe anatomical structures of pituitary gland and hypothalamus 3. Describe anatomical structures of pancreas 4. Describe anatomical structures of adrenal gland Udayana University Departement of Medicine Education, DME 19

12. LEARNING TASKS