<prompt> Congenital adrenal hyperplasia is the most common cause ofTrue hermaphroditismMale pseudohermaphroditism underandrogenized male 46XYTurners syndromeFemale pseudohermaphroditism androgenized female 46XX  <response>: Ans. d. Female pseudohermaphroditism Ref. Guyton 11th ed., Table 23-1.Congenital Adrenal Hyperplasia Usually due to a congenital enzyme charactrized by cortisol deficiency and enlargement of adrenal glands There are 3 most common forms; all cause virillism and are called adrenogenital syndromes;- Partial 21-hydroxylase deficiency: normal aldosterone function and impaired cortisol production.- Salt-losing syndrome: near total 21-hydroxylase deficiency and aldosterone deficiency; infants present with vomiting, dehydration, hyponatremia, hyperkalemia- 11-hydroxylase deficiency: leads to excessive androgen production and buildup of 11-deoxycorticosterone strong mineralocorticoid, causing virilization, hypertension, hypokalemia.CLASSIFICATION OF THE MAJOR DISORDERS OF SEX DIFFERENTIATION IN HUMANS Chromosomal disorders- Gonadal dysgenesis XO and variants- Superfemales XXX- Seminiferous tubule dysgenesis XXY and variants- True hermaphroditism Developmental disorders- Female pseudohermaphroditism Congenital virilizing adrenal hyperplasia of fetus Maternal androgen excess Virilizing ovarian tumor Iatrogenic: Treatment with androgens or certain synthetic progestational drugs- Male pseudohermaphroditism Androgen resistance Defective testicular development Congenital 17-hydroxylase deficiency Congenital adrenal hyperplasia due to blockade of pregnenolone formation Various non-hormonal anomaliesCongenital Adrenal HyperplasiaEnzyme deficiencyGlucocorticoidsMineralocarticoidsAndrogensOther effects20, 22 desmolase Lethal if complete3 b-OH SDH DHEA Masculinization of females in utero Incomplete precocious puberty males Adrenal hyperplasia Hyponatremia and hypovolemia21b-OH Masculinization of females in utero Incomplete prococious puberty males Adrenal hyperplasia Hyponatremia and hypovolemia11 b-OH aldosterone DOC Mineralocorticoid excess DOC Hypervolemia and hypernatremia Sexual effects as above17 a-OHaldosterone DOC DOC excess Absent 2deg sexual aspects both sexes Amenorrhea17, 20LNormalNormal Absent 2deg sexual aspects both sexes Amenorrhea 