Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
➤ Gửi thông báo lỗi ⚠️ Báo cáo tài liệu vi phạmNội dung chi tiết: Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
Delayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 rm through normal vaginal delivery, and his developmental milestones were normal. He was average in scholastic performance and studied up to tenth standard. He had history of learning disabilities but no behavioral abnormalities. He was tallest among his peers during late teenage. He noticed appeara Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 nce of pubic and axillary hair by 15 years of age. but failed to develop facial or body hair or increase in penile length or size of the testes. ThereEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
was no history of head trauma, surgery for midline defects, chronic systemic illness, testicular trauma, mumps, or drug abuse. He had no history of aDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 rtility, or gynecomastia. He did not receive any medical treatment prior to visit to this hospital. On examination, his height was 170 cm (height - I SDS. height age 15 years, target height 173 cm), weight was 55 Kg (weight age 15 years), and blood pressure was 110/70 mmHg. Anthropometry showed eunu Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 choi-dal proportions with upper segment/lower segment ratio (US: LS. 80:90 cm) 0.88 and arm span exceeding height by 10 cm. There was no gynecomastia.Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
Tanner stage of pubertal development was A+, p2. and both testes were present within poorly developed scrotal sac and soft in consistency and measureDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 axia, and visual deficits. On investigations, complete blood count and liver and renal function tests were normal. Hormonal profile revealed serum LH 0.29 miu/tnl (N 1.7-8.6), FSH 0.69 miu/ml (N 1.5-12.4), testosterone 0.44 nmol/L (N 9.9-27.8). estradiol 12.3 pg/ml (N 7.6-42.6). prolactin 9.6 ng/ ml Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 (N 4-15.2). T4 7.32 pg/dl (N 4.8-12.7). TSH 1.9 plư/ml (N 0.27-4.2), and 080011 cortisol 447 nmol/L (N 171-536). His bone age was 15 years. CEMRi selEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
la and olfactory region did not display any abnormality. LH response to triptorelin at 411 was 2.8 miu/ml. Scrum testosterone at baseline was 0.45 nmoDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 li et al.. Clinical Rounds in Endocrinology.Ml in mni/mc ÍỈ1 m c 72127 Delayed Pubertyclinical and biochemical profile, a diagnosis of congenital idiopathic hypogonado-tropic hypogonadism (IHH) was considered, and he was initiated with testosterone enanthate 100 mg intramuscularly every fortnightly. Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 The doses of testosterone were increased gradually to 200 mg every fortnightly over a period of 2 years. On testosterone therapy, he developed gynecoEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
mastia. He is planned for gonadotropin therapy for induction of spermatogenesis after the attainment of virilization (Fig. 7.1).Fig. 7.1 (a) A 19-yearDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 2W MR image shows nor-7 Delayed Puberty2137.2Stepwise AnalysisThe index case presented al the age of 19 years with delayed pubertal development. Delayed puberty in boys is defined as lack of pubertal development by the age of 14 years which is in correspondence with 2.5 SD above the mean for the pop Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 ulation. The main differentials for the delayed pubertal development between the age of 14-18 years are constitutional delay in growth and puberty (CDEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
GP) and hypogonadism. However, the adolescents with CDGP enter into puberty by the age of 18 years: hence, the possibility of CDGP after this age is vDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 hypogonadism as a cause of delayed puberty was considered. Development of secondary sexual characteristics results from both adrenarche and gonadarche which may overlap or come in succession. Though the first sign of puberty in boys is testicular enlargement (TV > 4 ml), only in 1 % of boys* pubarc Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 he precede the gonadarche. On the contrary, in 20% of girls, pubarche precedes the thclarche. Patients with hypogonadism usually have normal onset ofEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
adrenarche. but pubarche is delayed as was seen in our patient who had appearance of pubic hair at the age of 15 years without evidence of gonadarche.Delayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 nosmia, midline defects, synkinesia, eunuchoidal proportions, small soft testes, skeletal anomalies, and neurological deficits (nystagmus and ataxia) usually suggests the diagnosis of IHH. Further, the manifestations of IHH vary according to the age of presentation; infants present with micropenis a Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 nd cryptorchidism, adolescents with delayed or arrested puberty and gynecomastia, and adults with infertility. Long-leggedness, gynecomastia, small fiEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
rm testes, learning disabilities/behavioral abnormalities. and some degree of virilization favor the diagnosis of Klinefelter’s syndrome which is consDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 tes w hich support the diagnosis of hypogonadotropic hypogonadism. Low LH and low testosterone below the reference range confirm the diagnosis of hypogonadotropic hypogonadism. LH response to short-acting GnRH agonist (triptorclin) and testosterone response to hCG were prepubertal in our patient, fu Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 rther substantiate the diagnosis of hypogonadotropic hypogonadism. However, these dynamic tests help in differentiating between CDGP and 1HH and are nEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
ot required if the patient is above the age of 18 years. High LH. FSH. and low testosterone indicate hypergonadotropic hypogonadism and require furtheDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 y lesion or due to familial or sporadic genetic mutations. The index patient was diagnosed to have isolated hypogonadotropic hypogonadism, as other pituitary hormone profile was normal and MR brain imaging was unremarkable. Patients of IHH w ith anosmia or hyposmia are termed as Kallmann syndrome. D Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 efective migration of olfactory neurons from olfactory placode to bulb results in impaired development of olfactory bulb and consequent anosmia. ThisEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
is evident in MR1 as olfactory bulb aplasia/hypoplasia and absent olfactory sulci. Since our patient did not have hypos-mia/anosmia, he was consideredDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 l characteristics and to improve the fertility prospects. For induction of secondary sexual characteristics, testosterone therapy is initiated with a low dose of testosterone esters (testosterone enanthate 50-100 mg) intramuscularly every month which is gradually built up to 200-250 mg every fortnig Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 htly over a period of 2-3 years. Improvement in libido, mood, and quality of life is observed over a period of 3-6 months, whereas increase in body haEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
ir, muscle mass and strength, and deepening of voice lake longer lime over a period of 1-2 years. Scrum testosterone should be measured midway betweenDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 tosterone just prior to the next injection to decide about the dosing interval. The adverse effects associated with testosterone therapy include gynecomastia, aggressive behaviour, priapism, mood swings, acne, and androgenic alopecia. hCG has also been tried for the induction of puberty which is ass Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 ociated with stable level of serum testosterone, minimal fluctuation in hypogo-nadal symptoms, and initiation of spermatogenesis; however, frequent inEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
jections and cost preclude its routine use in clinical practice. In addition, limited data is available regarding the use of gonadotropins as a primarDelayed Puberty7.1Case VignetteA 19-year-old male presented with complaints of failure to develop secondary sexual characteristics. He was born at ter Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 ater the dose frequency was increased to fortnightly. At 6 months of follow-up. his serum testosterone was 5 nmol/L and he had improvement in generalized well-being. The dose was further increased to 150 mg fortnightly. Gonadotropin therapy is indicated when fertility is desired. hCG is initiated at Ebook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2 a dose of 1.000-2.000 11) twice or thrice a week with monthly monitoring of serum testosterone to achieve and sustain testosterone in eugonadal rangeEbook Clinical rounds in endocrinology (Volume II - Pediatric endocrinology): Part 2
. If the target is not achieved the doses can be increased up to 5,000 1U thrice a week. Once the serum testosterone level is maintained >9 nmol/L. seGọi ngay
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