HIPOTIROIDISMO CONGENITO

PRONOSTICO NEUROLOGICO EN EL SEGUIMIENTO A

LARGO PLAZO

• Porque los neonatos son asintomaticos al nacerexisten los programasmundiales de tamizaje.

• Incidencia 1 in 2,000 • Es la causa mas comun

TRATABLE de retardomental

Untreated CH Patients at Risk for Severe Mental Retardation

Klein et al, J Pediatr 81:912-915, 1972

IQ of CH patients is normal if treatment early and adequate

NECHC. Lancet 2:1095-1098, 1981

Etiología

• Formas comunes de disgenesia tiroidea– Aplasia

– Hypoplasia

– Ectopia (66%)

• Disgenesias tiroideas usualmente son desconocidad (85% sporadicas, 15% hereditarias)

– Mutaciones

– Errores innatos de la sintesis de T4 su secrecion o utilizacion (2/3 de los casos son heredables)

• Hipotiroidismos transitorios– Tiroiditis materna

– Madre en tratamiento para enfermedad de Graves

• Cretinismo endémico

Clinical Manifestations

Infants protected for 1st few wks of life– Fraction of maternal thyroid

hormone crosses placenta

• >40 wk GA• HC Slightly higher % due to brain

myxedema• Large fontanels & wide sutures• Macroglossia• Distended abdomen with

umbilical hernia• Skin mottling• Goiters (5-10%)• Sensorineural deafness (10%)• Other congenital anomalies (10%)

• Slow to feed• Constipation• Lethargic• Sleep more, needs to be

awakened to feed• Hoarse cry• Cool to touch• Hypotonic with slow reflexes• Prolonged jaundice