RESUMO
AIMS: To evaluate therapy and dose adjustments in patients with congenital hypothyroidism (CH), longitudinally followed up until 16 years old, according to aetiology, Beclard's nuclei presence, and thyroxine (T4) level at diagnosis. METHODS: L-T4/kg/day and dose change ratio (CR) were assessed in 74 CH patients. RESULTS: The dose was statistically larger in athyreosis than in dyshormonogenesis (1-10 and beyond 14 years) and in ectopy (2, 15, 16 years). The ectopic children required statistically larger L-T4/kg than the dyshormonogenetic ones (3-7 years). The L-T4/kg/day was increased, not statistically, in patients or with T4 <30 nmol/l or without Beclard's nuclei at diagnosis. The CR progressively dropped after the 6th month at each attendance, without any difference in terms of aetiology, T4 level at diagnosis, or Beclard's nuclei. The total CR was greater (significantly) in patients without Beclard's nuclei, and (not significantly) in those with T4 <30 nmol/l at diagnosis or with agenesia. CONCLUSION: The L-T4 dose in CH is highly affected by the aetiology. The CR is higher in patients with delayed bone maturation at diagnosis. We suggest that these latter patients need blood tests more frequently to obtain a proper titration of the therapy.
Assuntos
Hipotireoidismo Congênito/tratamento farmacológico , Tiroxina/uso terapêutico , Adolescente , Determinação da Idade pelo Esqueleto , Criança , Pré-Escolar , Coristoma , Hipotireoidismo Congênito/etiologia , Feminino , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Puberdade/fisiologia , Estudos Retrospectivos , Glândula Tireoide/anormalidades , Tiroxina/administração & dosagem , Tiroxina/sangueRESUMO
Catamenial hemoptysis is a rare condition characterized by cyclic pulmonary hemorrhage, synchronous with menses and associated with the presence of intrapulmonary or endobronchial endometrial tissue. Because of the paucity of cases reported in the literature, information regarding the natural history is limited and also the optimal diagnostic workup and management of these patients are not well defined. In this report, we present a case of endobronchial endometriosis in a 12-year-old female diagnosed by bronchoscopy and immunocytochemical assay, associated with type 1 von Willebrand disease.