Index A description , 199 Abnormalities histology , 202 adiponectin , 187–188 ICU , 210–211 and bone loss, skeletal dynamics , 189–190 insulin-induced hypoglycemia , 209 growth hormone , 188 mechanism, glucocorticoid action , 200–201 hypothalamic-pituitary-ovarian axis , 188–189 metyrapone test , 209–210 insulin , 187 neonates , 211 leptin , 187 pathologic process , 202 thyroid hormone , 188 primary ( see Primary adrenal insuf fi ciency) Achondroplasia prognosis, nineteenth century , 199–200 average intelligence , 61 quality of life (see Quality of life, adrenal insuf fi ciency) cartilage growth , 56 regulation, HPA axis , 201 clinical features , 61 secondary and tertiary ( see Secondary and tertiary C-type natriuretic peptide(CNP) , 62 adrenal insuf fi ciency) management , 62 stimulation test mutation , 61–62 cosyntropin , 209 short stature , 57 CRH , 209 Acid-labile subunit (ALS) , 30, 34–35 glucagon , 209 Acromesomelic dysplasia, type maroteaux (AMDM) , 69 therapy ACTH. See Adrenocorticotropic hormone (ACTH) central failure , 213 ACTH dependent , 253, 255, 256 chronic replacement , 211–212 ACTH independent , 249, 253 newer , 213–214 Addison’s disease standard , 213 screening , 575 stress replacement , 212–213 type 1 diabetes , 574 X-linked leukodystrophy , 214 Adolescents. See Contraception Adrenocortical hyperplasias , 250 Adrenal Adrenocorticotropic hormone (ACTH) carcinomas , 256 adrenal insuf fi ciency Cushing syndrome , 249 baseline level, cosyntropin stimulation test , 209 GIPR , 249 changes , 202 hypothalamic–pituitary , 248 CRH , 209 ultrasound , 255 elevation , 208 Adrenal androgens dehydroepiandrosterone (DHEA) , 187 glucocorticoid , 204 Adrenal axis management , 211 cortisol , 592 metyrapone inhibits , 210 diagnosis , 593 primary , 207, 212 disadvantages, hormonal constellation , 592–593 regulation, HPA axis , 201–202 potential mediators , 592–593 secondary , 208, 213 treatment , 593 CRH , 249 Adrenal insuf fi ciency Cushing syndrome , 249 ACTH , 201, 202 elevation , 405 animal systems , 199 hypothalamic–pituitary–adrenal axis , 248 baseline hormone measurements , 208 independent and dependent activation , 249 CRH , 201 pituitary tumors , 254, 256 CT and MRI , 210 stimulation , 406, 412 S. Radovick and M.H. MacGillivray (eds.), Pediatric Endocrinology: A Practical Clinical Guide, 605 Second Edition, Contemporary Endocrinology, DOI 10.1007/978-1-60761-395-4, © Springer Science+Business Media New York 2013 606 Index Adrenoleukodystrophy , 215 Antithyroid medications , 282–283 Adult-onset growth hormone de fi ciency (AOGHD) AOGHD. See Adult-onset growth hormone de fi ciency autoimmune hypophysitis , 145–146 (AOGHD) BMI , 144 APS-1. See Autoimmune polyglandular syndrome bone density , 142 type 1 (APS-1) bone mineral density , 144 APS-2. See Autoimmune polyglandular syndrome clinical trail , 143–144 type 2 (APS-2) craniopharyngioma , 145 ARH. See Autosomal recessive hypercholesterolemia dose titration , 144–145 (ARH) glucagon stimulation test , 144 Arm span insulin hypoglycemia stimulation test , 144 AMDM , 69 mortality , 141 differences , 59–60 risk , 142 LWD , 63 therapy continuing , 141, 142 measurement , 58, 59 AIDS , 206 reference range , 60 Albumin , 292 Aromatase inhibitors , 79–80 ALS. See Acid-labile subunit (ALS) Atherosclerosis Ambiguous genitalia , 229, 234 adulthood , 545 AMDM. See Acromesomelic dysplasia, type maroteaux coronary, cerebral and peripheral arteries, (AMDM) adults , 552 Amenorrhea dyslipidemia and lesions, young adults , 557 DSD , 443 LDL in human , 549 hypothalamic , 445 Autoimmune endocrine disorders obesity , 445 APS-2 , 574–576 OCP , 458 APS-1 and APECED , 572–574 PCOS ( see Polycystic ovary syndrome (PCOS)) autoantibodies , 571, 572 steroidogenic blocks , 444 celiac disease , 571 Turner syndrome patients , 444 hemoglobin A1c , 571 Androgen hypothesis , 570 adrenal, production , 397, 401, 406 IPEX , 576–577 effects , 401 multiple genes , 570–571 estrogen ratios , 398, 402 syndromes , 569–570 hyperandrogenism , 405, 406 type 1 diabetes , 570 premature adrenarche , 405–406 Autoimmune polyendocrinopathy candidiasis and therapy , 411 ectodermal dystrophy (APECED). Androgen excess See Autoimmune polyglandular syndrome children and adults , 229, 231, 232 type 1 (APS-1) and APECED signs, males and females , 225 Autoimmune polyglandular syndrome type 2 (APS-2) treatment , 230 Addison’s disease , 574 virilizing disorders , 236 APS-3 and APS-4 , 574 Anorexia nervosa (AN) patients, type 1 diabetes , 574–575 clinical features , 186 screening , 575 description , 186 trearments , 575–576 DSM-IV criteria , 186 Autoimmune polyglandular syndrome type 1 (APS-1) endocrine dysfunction , 186 and APECED evaluation , 190–191 asplenia , 573 ghrelin and peptide YY , 189 autoantibodies , 574 HPA axis ( see Hypothalamic-pituitary-adrenal (HPA)) autoimmune endocrine disorders , 572–573 management , 191–192 autoreactive T cells , 572 oxytocin , 189 autosomal recessive disorder , 572 prolactin , 189 candidiasis association , 573–574 vasopressin , 189 diagnosis , 574 Anovulation disease associations , 573 chronic disease , 445 patients , 572–573 description , 443–444 treatment , 574 FSH levels , 444 Autoimmune thyroid disease galactorrhea , 445 chronic ( see Chronic autoimmune thyroiditis) gonadotropin de fi ciency , 444–445 Graves’ disease , 279–286 hypothalamic , 445 Autosomal recessive hypercholesterolemia PCOS , 445 (ARH) , 551 Index 607 B Cancer Beta-cell function, type 2 diabetes , 526–528 acute effects 3 b (Beta)-HSD/ D (delta)4,5-isomerase de fi ciency , 228–229 ACTH , 168 11 b (Beta)-hydroxylase de fi ciency , 232 ADG , 168 Birth trauma , 206 brain and spinal cord tumors , 168 Bisphosphonates , 67 cerebral salt wasting (CSW) , 169 Blomstrand chondrodysplasia , 68–69 chemotherapy , 169–170 BMD. See Bone mineral density (BMD) DI ( see Diabetes insipidus (DI)) Body composition, PWS pituitary gland , 168 GHD , 101 SIADH , 168–169 lean body mass , 101–102 description , 167–168 obesity , 101 endocrine disorders , 168 REE , 101 late effects ( see Late effects treatment, cancer) skinfold measurement, infants , 100, 101 skin , 17 strength and agility , 103–104 TN ( see Thyroid neoplasia) Body mass index (BMI) , 144 Cardiac surgery, children , 297–298 Bone health CDC. See Center for Disease Control (CDC) age , 186 Center for Disease Control (CDC) bone mass , 186, 188, 192 combination hormonal contraception , 471 formation , 187 contraceptive use, MEC , 467, 469 loss , 189–190, 192 guidelines resorption , 187 DMPA , 482, 483 younger age , 186 hormonal contraception , 472 Bone mineral density (BMD) thromboembolism , 473 adults , 144 VTE , 473 issue , 483 women, well-controlled disease , 471 signi fi cant loss , 482 MEC indicate , 475 temporary loss , 482 Central DI. See Diabetes insipidus (DI) Bone strength , 177–178 Central hypothyroidism thyroid function , 299 TSH , 290 C Central precocious puberty (CPP) CAH. See Congenital adrenal hyperplasia (CAH) bone age , 407 CAIS. See Complete androgen insensitivity syndrome (CAIS) bone mineral density (BMD) , 408 Calciopenic rickets breast and pubic hair development , 408 description , 363 children , 406 treatment CNS lesions , 406 anticonvulsant therapy , 369 differential diagnosis, gonadotropin dependent calcium de fi ciency , 368 precocious puberty , 401, 406 CKD , 369 GnRH ( see Gonadotropin-releasing hormone guidelines , 367–368 (GnRH)) heal , 367 hypothalamic–pituitary–gonadal axis , 407 knock-knee deformities , 367 identi fi cation , 406 monitoring and therapy complications , 370 “LH predominance” , 407 primary and secondary , 367 MRI , 407 severe hepatobiliary disease , 369 pelvic ultrasonography , 407 vitamin D ( see Vitamin D) progestational agents , 408 Calciotropic hormones , 340 Cerebral salt wasting (CSW) , 168–169 Calcitonin , 341–342 CETP. See Cholesteryl ester transfer protein (CETP) Calcium Challenges, DSD balance, children , 339–340 CAH , 433 calciotropic hormones , 340 differential diagnosis , 433, 434 calcitonin , 341–342 gender assignment , 435 distribution , 340 parents , 435 fetal and early neonatal period , 342 partial androgen insensitivity , 433 hypercalcemia (see Hypercalcemia) sexual hair , 436 hypocalcemia (see Hypocalcemia) surgical procedures , 435 parathyroid , 340–341 46,XX genetic males and female , 433 physiological functions , 339 46,XY infants with micropenis , 434–435 rickets ( see Calciopenic rickets) young women complains , 435 608 Index Chemotherapy, cancer side effects, combined hormonal contraception , bone strength , 177 478–479 childhood cancer , 168 Complete androgen insensitivity syndrome (CAIS) cytotoxic effects , 173, 175 babies , 432 diabetes mellitus , 170 individuals , 436 gastrointestinal side effects , 169 46,XY females , 436 GHD , 170 Congenital adrenal hyperplasia (CAH) glucocorticoids , 169–170 autosomal recessive disorders , 223 heterogeneous tumor type , 169 clinical and hormonal data , 225–227 high-dose steroid therapy , 170 cortisol synthesis , 223, 224 parenteral nutrition , 169 de fi ciency side effects , 169 3 b (Beta)-HSD/ D (delta)4,5-isomerase , Childhood obesity. See Type II diabetes mellitus 228–229 and obesity 11 b (Beta)-Hydroxylase , 232 Childhood-onset growth hormone de fi ciency (COGHD) 21-Hydroxylase , 230–232 AACE guidelines , 141 17-Hydroxylase/17,20-lyase , 229–230 adults ( see Adult-onset growth hormone de fi ciency P450-Oxidoreductase , 232–233 (AOGHD)) description , 223, 225 approved diagnosis, GHD , 139 diagnosis , 433 arginine/GHRH test , 140–141 enzymes and genes , 225 causes, transition patients , 138