Pedia_M5L1_PediaEndocrinology.pdf - PEDIATRICS II Module 5...

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[ GUZMAN, ATIENZA] Checked by: [VILLANUEVA] Page 1 of 12 DR. RUBY ANN L. PUNONGBAYAN Module 5 1 January 11, 2020 PEDIATRIC ENDOCRINOLOGY PEDIATRICS II LEGEND Book Recordings Important points to remember** HORMONES Hormones are generally regulated in a feedback loop so that production of a hormone is linked to its effect or its circulating concentration Endocrine disorders can generally manifest from of four ways: o By excess hormone o By deficient hormone o By an abnormal response of end organ to hormone o By gland enlargement that may have effects as a result of size rather than function HYPOTHALAMIC PITUITARY ORGAN AXIS Hypothalamus controls many endocrine systems either directly or through the pituitary gland Hypothalamic releasing or inhibiting factors travel through capillaries of the pituitary portal system to control the anterior pituitary gland, regulating the hormones specific for the factor The pituitary hormones enter the peripheral circulation and exert their effects on target glands, which produce other hormones that feedback to suppress their controlling hypothalamic and pituitary hormones SHORT STATURE Abnormal linear growth Causes: 1. Familial (genetic) - 80% 2. Constitutional 3. Disproportionate a. Rickets b. Achondroplasia (short legs) c. Spondyloepiphyseal dysplasia (short trunk) 4. Proportionate causes a. Prenatal Insult - IUGR - Placental dysfunction - Intrauterine infections and teratogens - Chromosomal abnormalities b. Postnatal Insult - Malnutrition - Chronic systemic diseases - Psychosocial deprivation - Drugs - Endocrine disorders (hypothyroidism, GH deficiency (5%) , glucocorticoid excess, and precocious puberty) HYPOPITUITARISM Deficiency of growth hormone with or without a deficiency of other pituitary hormones CLINICAL MANIFESTATIONS Normal size and weight at birth Atrophy of adrenal cortex, thyroid and gonads Outline Hypothalamic Pituitary Organ Axis Hypopituitarism and Hyperpituitarism Diabetes Insipidus Precocious puberty Graves disease and Thyroid Storm Hypothyroidism Thyroiditis Hypoparathyroidism and Hyperparathyroidism Adrenal Glands Diabetes Mellitus CONGENITAL HYPOPITUITARISM Those with MPHD and genetic defects of the GH1 or GHR gene have birth lengths that average 1 SD below the mean Severe defects in GH production or action typically fall more than 4 SD below the mean for length by 1yr of age May present with neonatal emergencies such as apnea, cyanosis, or severe hypoglycemia with or without seizures Prolonged neonatal jaundice is common, nystagmus can suggest septooptic dysplasia, micropenis, hypoadrenalism, and hypothyroidism, as well as gonadotrophin deficiency Head is round and the face is short and broad. Frontal bone is prominent, and the bridge of the nose is depressed and saddle shaped. Nose is small, and the nasolabial folds are well-developed. Eyes are somewhat bulging. Mandible and the chin are undeveloped, and the teeth, which erupt late, are often crowded
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