Pubertal development and adult height in patients with congenital hypothyroidism detected by neonatal screening in southern Brazil.

Objectives Adequate treatment of congenital hypothyroidism (CH) is required for normal growth and sexual development. To evaluate pubertal development in patients with permanent CH detected by a statewide Neonatal Screening Program of Paraná and, secondly, to evaluate adult height (AH) in a subgroup of patients. Methods Clinical, laboratory, and auxological data obtained from medical records of 174 patients (123 girls). Results Median chronological age (CA) at treatment initiation was 24 days, and mean initial levothyroxine dose was 11.7 ± 1.9 µg/kg/day; mean CA at puberty onset was 11.5 ± 1.3 years (boys) and 9.7 ± 1.2 years (girls); mean CA in girls who underwent menarche (n=81) was 12.1 ± 1.1 years. Thyroid-stimulating hormone (TSH) values above the normal range were observed in 36.4% of the boys and 32.7% of the girls on puberty onset, and in 44.6% around menarche. Among 15 boys and 66 girls who had reached the AH, the median height z-score value was significantly greater than the target height (TH) z-score value in boys (p=0.01) and in girls (p<0.001). Boys with normal TSH values at puberty onset had greater mean AH z-score compared with boys with TSH values above the normal range (p=0.04). Conclusions In this group, pubertal development in girls with CH was not different from that reported in healthy girls in the general Brazilian population. Boys with higher TSH at puberty onset may have an increased risk of not reaching their potential height compared with those with normal TSH during this period. In a subgroup who attained AH, the median AH z-score was greater than the median TH z-score.

[Growth hormone therapy for children with chronic diseases]. / Tratamento com hormônio de crescimento em crianças com doenças crônicas.

Growth disorders are commonly observed in children suffering from chronic diseases. The pathogenesis of growth failure is multifactorial. In chronic inflammatory diseases such as juvenile idiopathic arthritis and inflammatory bowel disease, growth is also affected by pro-inflammatory cytokines. Patients with chronic diseases might also become growth hormone (GH) deficient. However, normal or increased GH secretion with reduced plasma concentrations of insulin-like growth factor-I indicate a degree of GH insensitivity in some patients. Growth damage can increase with specific treatments, especially if glucocorticoids are used. GH therapy has been used to reduce the consequences of the disease and long-term steroid therapy in these patients. In this review, it is reported the encouraging results of GH treatment in growth-retarded children with chronic diseases, both in well defined indications as well in situations still under investigation.

Tratamento com hormônio de crescimento em crianças com doenças crônicas: [revisão] / Growth hormone therapy for children with chronic diseases: [review]

Crianças com doenças crônicas freqüentemente apresentam crescimento inadequado e baixa estatura. A falência do crescimento é multifatorial. Nas doenças inflamatórias, como na artrite juvenil idiopática e nas doenças inflamatórias intestinais, o crescimento é comprometido também pelo processo inflamatório. Muitas vezes, o tratamento da doença de base compromete o crescimento, especialmente quando é necessário glicocorticóides. Em algumas situações é possível comprovar a deficiência associada de hormônio de crescimento (GH, do inglês growth hormone). Em outras, os exames sugerem certo grau de insensibilidade ao GH. O tratamento destes pacientes com GH tem se mostrado útil e seguro com melhora do crescimento e da qualidade de vida. Nesta revisão, são apresentados resultados do tratamento com GH em pacientes com baixa estatura decorrente de doenças crônicas, algumas indicações já bem definidas e outras ainda em investigação.

Growth disorders are commonly observed in children suffering from chronic diseases. The pathogenesis of growth failure is multifactorial. In chronic inflammatory diseases such as juvenile idiopathic arthritis and inflammatory bowel disease, growth is also affected by pro-inflammatory cytokines. Patients with chronic diseases might also become growth hormone (GH) deficient. However, normal or increased GH secretion with reduced plasma concentrations of insulin-like growth factor-I indicate a degree of GH insensitivity in some patients. Growth damage can increase with specific treatments, especially if glucocorticoids are used. GH therapy has been used to reduce the consequences of the disease and long-term steroid therapy in these patients. In this review, it is reported the encouraging results of GH treatment in growth-retarded children with chronic diseases, both in well defined indications as well in situations still under investigation.