RESUMO
We describe an infant who had a dilated cardiomyopathy and who was later found to have congenital adrenal hyperplasia. The cardiomyopathy resolved after replacement of glucocorticoid and mineralocorticoid. We believe that glucocorticoid deficiency may have played a direct role in the evolution of this cardiomyopathy.
Assuntos
Hiperplasia Suprarrenal Congênita/complicações , Cardiomiopatia Dilatada/etiologia , Hiperplasia Suprarrenal Congênita/metabolismo , Cardiomiopatia Dilatada/metabolismo , Humanos , Recém-Nascido , MasculinoRESUMO
We examined the results of the Northwest Regional Screening Program (NWRSP) over its first 10 years to determine whether the detection of hypopituitary hypothyroidism is a justified advantage of the primary thyroxine (T4)-supplemental thyroid-stimulating hormone (TSH) screening strategy, and to determine whether all such infants will be detected by this screening approach. Between May 1975 and May 1985, the NWRSP screened 850,431 infants, detecting 192 infants with primary hypothyroidism (1:4429) and eight with hypopituitary hypothyroidism (1:106,304). In 11 additional infants, TSH deficiency, not detected by the screening program, was diagnosed on recognition of clinical features over the same period. Thyroid hormone treatment was begun in seven of the 11 infants prior to obtaining the screening sample results because of clinical symptoms of hypopituitarism, including hypoglycemia, persistent jaundice, microgenitalia, diabetes insipidus, midface hypoplasia, cleft lip or palate, or abnormalities of vision. The other four infants were not detected despite clinical features of hypopituitarism (in retrospect) and low serum T4 with TSH concentration below assay sensitivity on at least one screening sample. The most accurate assessment of total cases comes from Oregon, where all cases of congenital hypopituitarism are referred to our center; we estimate a frequency of 1:29,000. In our experience, a combination of newborn T4-supplemental TSH screening measurements and recognition of clinical features of hypopituitarism is the optimal strategy for detecting infants with congenital hypopituitary hypothyroidism.
Assuntos
Hipopituitarismo/epidemiologia , Hipotireoidismo/epidemiologia , Programas de Rastreamento , Hipotireoidismo Congênito , Feminino , Humanos , Hipopituitarismo/sangue , Hipopituitarismo/complicações , Hipopituitarismo/congênito , Hipotireoidismo/sangue , Hipotireoidismo/complicações , Lactente , Recém-Nascido , Masculino , Tireotropina/sangue , Tiroxina/sangueAssuntos
Síndrome da Sela Vazia/induzido quimicamente , Hipopituitarismo/induzido quimicamente , Hipotireoidismo/tratamento farmacológico , Criança , Humanos , Hipotireoidismo/diagnóstico por imagem , Masculino , Hormônios Tireóideos/sangue , Tiroxina/efeitos adversos , Tomografia Computadorizada por Raios XRESUMO
To determine the frequency with which hypothyroidism develops during human growth hormone therapy and to corroborate its onset with blood lipid changes, we measured growth rate, serum T4 and T3, and plasma cholesterol, triglyceride, and lipoprotein concentrations at 4-month intervals for a year in two subgroups of hGH-deficient children. The first group was initially euthyroxinemic (n = 16), and the second was TSH deficient and therefore already receiving thyroxine (n = 15). Basal plasma concentrations of total and low-density lipoprotein cholesterol and, to a lesser extent, plasma triglycerides were increased in both groups compared with an age-matched reference group. Basal plasma cholesterol levels were not statistically different in the euthyroxinemic and thyroxine-treated subgroups, and hGH treatment for a year did not lower lipid values in either subgroup. With hGH replacement, 25% of the euthyroxinemic patients experienced a slowdown in growth rate (3.2 +/- 0.7 cm/yr) associated with decreasing T4 (4.8 +/- 1.1 micrograms/dl) and increasing cholesterol concentrations (218 +/- 23 mg/dl); with thyroxine treatment, the growth rate improved (6.9 +/- 2.2 cm/yr), T4 increased (10.0 +/- 4.0 micrograms/dl), and cholesterol decreased (173 +/- 44 mg/dl, P less than 0.05). Although our results do not justify routine thyroid replacement, they do indicate that hypothyroxinemia and hypercholesterolemia may precede the growth slowdown during hGH treatment, and the need to monitor thyroid function at this time.