2.  Growth can be easiest way to determine overall health of a child  Red Flag: Growth less than 5 cm/year  Most common cause of short stature is short parents

3.  Secreted in response to GHRH  Is secreted in bursts  Dopamine causes release of GHRH  Somatostatin inhibits the release of GH  Anything that effects the pituitary gland will usually result in GH Def.  Think midline defects, central incision, bifid uvula

4.  Height less than 3 SD below mean, slow growth velocity  Characteristic Features  Short stature  Normal body proportion  Increased adiposity around trunk and extremities  Boys may have small genitalia  Can be seen with midline defects  Hypoglycemia

5.  Tests  Height and bone age is delayed  Low IGF-1 and low GH levels  Can do GH stimulation tests ▪ Exercise ▪ L-Dopa, insulin, arginine, clonidine, glucagon  Treatment  GH replacement

8.  Hypoglycemia  Micropenis  Jaundice  Midline facial abnromalities

9.  TRH TSH T4/T3  Free T4 is the only active form

10.  Most are asymptomatic at birth because of maternal thyroxine  Newborn screen will check TSH  Clinical Presentation  Infant can present with goiter  Broad nasal bridge, thick lips, poor feeding  “hoarse cry”  Slow heart rate, low temperature  Umbilical hernia  Large posterior fontenelle, large sutures on X-ray

11.  Most common cause is thyroid dysgenesis  Other less common causes  Defect in the synthesis  Radioiodine given during pregnancy  TSH deficiency  Treatment should be started within 2 weeks  Thyroxine  Good prognosis if started early in life

13.  Most common cause Hashimoto thyroiditis  Dry skin, constipation, hair loss, depressed DTR, goiter  More common in girls  Sharp deceleration of growth with preserved  Tests  High TSH  Low thyroxine level  Often detect antibodies SCFE – high risk with rapid changes of growth. Can see because of rapid halt!

15.  Graves disease is most common cause  TSH receptor antibody stimulates thyroid cells  Symptoms  Tachycardia, weight loss, heat intolerance  Anxiety, muscle weakness, tremor  Exophthalmos,Goiter  Tests  Elevated thyroxine and triiodothyronine  Low TSH  Treatment  PTU or methimazole  Surgery – radioactive iodine/thyriodectomy

17.  Common cause of short stature in girls  Physical findings  Cubitus valgus, sheildlike chest, web neck  Gonadal failure ▪ Pubic and axillary hair with no breast development or menarche  WorkUp  Karyotype

20.  PTH  Increased Ca+ release from bones  Decrease renal excretion of Ca+  Increase conversion of Vit D to 1,25 OH-D  Decreases Phos reabsorption from kidney  1,25 OH-D  Increase Ca+ reabsorption from gut, bone, kidney  Increase phos reabsorption from gut and kidney

21.  “PTH antagonist”  Decrease Ca+ bone resorption  Increases renal Calcium clearance

22.  Due to aplasia/hypoplasia of parathyroid gland  Can be associated with DiGeorge  Also autoimmune forms  Presentation can vary  Muscle pain or cramps  Positive Chvostek or Trousseau sign  Seizures

23.  Labs:  Hypocalcemia  Elevated phos  Low PTH/low 1,25 OH D  EKG: prolonged QT  Treatment  Calcium  Vit D (Calcitriol)

24.  Pseudohypoparathyroidism  Round facies, short stature, obesity, skin hyperpigmentation, short thick necks  Short 4 th metacarpals and metatarsals  Decreased intelligence  Labs  Hypocalcemia  Hyperphosphatemia  Elevated PTH

26.  In children usually response to low Ca+ levels  Vit D deficiency  Malabsorption  Chronic renal disease  MEN I – neoplasia  Pancrease  Parathyroid  Pituitary

27.  Excess glucocorticoid secretion  Clinical features  Round face, obesity, buffalo hump, stirae, thin extremities, HTN, osteoporosis, decrease growth, hirsutism  Testing  Elevated urine free cortisol  Dexamethasone suppression test  Causes  Adrenal tumor, pituitary adenoma, ectopic ACTH

28.  ACTH-secreting pituitary tumor  Which results in excess glucocoriticoid secretion

30.  Acquired Deficiency of glucocorticoid and mineralcorticoid  Clinical Features  Thin body, hyperpigmentation of skin(bronzing)  Confusion, weakness  Can cause vascular colapse  Testing  Hyponatremia, hyperkalemia, low cortisol  Treatment  Replacement of hormones

32.  Autosomal recessive  Get increased ACTH  Causes:  21-Hydroxylase Def ▪ Most common cause ▪ Salt wasting ▪ Virilization ▪ Elevation 17-Hydroxyprogesterone  11ß-Hyroxylase Def ▪ No salt wasting – hypertension ▪ Virilization ▪ Elevated 11-deoxycortisol

34.  Presentation  Shock or septic in newborn  FTT  Males – normal genitalia (could have hyperpigmentation of scrotum)  Females- ambiguous genitalia  Lab findings  Low Na  Low Cl  High K