RESUMEN
OBJECTIVE: To examine relationships among parenting styles, regimen adherence, and glycemic control for preschool and elementary school children who have Type I diabetes. METHODS: Parents of 55 children with diabetes completed parenting style and regimen adherence questionnaires. Glycosylated hemoglobin results were collected by chart review. RESULTS: Parental warmth was associated with better adherence ratings. Regression analyses showed that parental warmth explained 27% of the variance in adherence ratings. Parental restrictiveness was associated with worse glycemic control in univariate analyses. However, only Black ethnicity, not adherence or parenting variables, predicted glycemic control. Black ethnicity and lower socioeconomic status (SES) were associated with more parental restrictiveness and worse glycemic control. CONCLUSIONS: These results suggest that authoritative parenting, characterized by support and affection, may be advantageous for the regimen adherence and glycemic control of school-age and younger children with diabetes. Demographic characteristics are important and require further study in this context.
Asunto(s)
Diabetes Mellitus Tipo 1/complicaciones , Hipoglucemia/prevención & control , Responsabilidad Parental , Cooperación del Paciente , Niño , Preescolar , Etnicidad/estadística & datos numéricos , Femenino , Humanos , Hipoglucemia/diagnóstico , Hipoglucemia/etiología , Masculino , Factores Socioeconómicos , Encuestas y CuestionariosRESUMEN
We report on two adolescent boys with Kenny-Caffey syndrome and microorchidism. The first patient had elevated levels of serum follicle-stimulating hormone, but normal levels of luteinizing hormone and testosterone. There was no evidence of a microdeletion of the Y chromosome. The second patient had Leydig cell hyperplasia with normal seminiferous tubules and spermatogenesis, and normal pituitary histologic findings at autopsy. The presence of microorchidism in these patients confirms the previous observations and suggests subfertility, but does not fully clarify the pathogenesis.
Asunto(s)
Testículo/anomalías , Anomalías Múltiples/patología , Adolescente , Adulto , Estatura , Huesos/anomalías , Preescolar , Hormona Folículo Estimulante/sangre , Humanos , Hipoparatiroidismo/patología , Masculino , Cráneo/anomalías , SíndromeRESUMEN
Two unrelated male infants presented with brittle insulin-dependent diabetes mellitus in the first days of life. Subsequently they each developed severe secretory diarrhea, with stool volumes of more than 100 ml/kg/day. Extensive biochemical and serological investigation failed to reveal the etiology of the diarrhea. The infants, cared for at different institutions, underwent therapeutic trials of various agents including loperamide, cholestyramine, prednisone, indomethacin, and somatostatin analogue, without response. Both infants succumbed to septicemia and malnutrition related to diarrhea and poor control of glycemia. At autopsy, both were found to have absence of islets of Langerhans in the pancreas, and diffuse dysplastic changes in small and large intestinal mucosae. In particular, the entire alimentary tract in each case was lined by epithelia most typical of foregut mucosa: secretory-type glands, absent crypts of Lieberkuhn, and absent villi. These cases are contrasted with previously-reported infants with congenital diabetes mellitus, and the possible interrelation of these two highly unusual findings, congenital diabetes mellitus and diffuse intestinal dysplasia, is examined.
Asunto(s)
Diabetes Mellitus Tipo 1/congénito , Diarrea Infantil/complicaciones , Catéteres de Permanencia , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/patología , Diarrea Infantil/tratamiento farmacológico , Diarrea Infantil/patología , Humanos , Recién Nacido , Mucosa Intestinal/patología , Intestinos/patología , Hígado/patología , Masculino , Trastornos Nutricionales/etiología , Páncreas/patología , Sepsis/etiologíaRESUMEN
Transient neonatal thyroid disease is known to occur as a result of transplacental passage of maternal immunoglobulin G (IgG) that contains antibodies to the TSH receptor (TRAb). Thyroid-stimulating antibody (TSAb) produces hyperthyroidism, and antibody that blocks TSH binding (TBIAb) results in hypothyroidism. We have analyzed in detail the IgG from four women who gave birth to children with transient neonatal hypothyroidism and have shown stimulating and inhibiting antibodies to coexist in three. Human and/or rat thyroid (FRTL5) cells were used to show stimulatory effects in vitro. Inhibition was assessed as prevention of stimulation of these cells (by TSH or TSAb) or by the blocking of binding of [125I] TSH to its receptor. The IgG from two mothers was tested to identify whether the inhibitory and stimulating bioactivities resided in molecules characterized by either or both kappa- or lambda-light chains. Evidence for restricted heterogeneity (implying oligoclonality) was obtained, in that with one, purely inhibitory IgG all activity was with IgG kappa. With the other, stimulating and inhibitory activities were predominantly in IgG kappa and IgG lambda, respectively. In addition, the latter IgG contained a second stimulator that was not suppressed by either its own or other inhibitory IgG. Despite the presence of stimulatory antibodies in these IgG, the clinical effect was neonatal hypothyroidism, reflecting the greater potency of the inhibitory IgG in all instances. Based on the histories of these four women and their offspring it is apparent that TRAb, and in particular TBIAb, can develop at any point in the course of autoimmune thyroid disease, i.e. either at the onset or long after the autoimmune process has been established.
Asunto(s)
Anticuerpos/aislamiento & purificación , Hipotiroidismo/inmunología , Inmunoglobulina G/aislamiento & purificación , Embarazo/inmunología , Receptores de Tirotropina/inmunología , Adulto , Autoanticuerpos/farmacología , Sitios de Unión de Anticuerpos/efectos de los fármacos , Células Cultivadas , AMP Cíclico/metabolismo , ADN/metabolismo , Femenino , Humanos , Hipotiroidismo/etiología , Fragmentos de Inmunoglobulinas/aislamiento & purificación , Inmunoglobulina G/farmacología , Inmunoglobulinas Estimulantes de la Tiroides , Recién Nacido , Yodo/metabolismo , Glándula Tiroides/efectos de los fármacos , Glándula Tiroides/inmunología , Glándula Tiroides/metabolismo , Tirotropina/farmacologíaRESUMEN
Hyperthyroidism associated with subacute (painful, viral) thyroiditis is well-recognized as a clinical entity; the thyroid gland in Graves disease is minimally, if ever, tender and painful. We describe a 10-year-old girl with hyperthyroidism whose initial clinical presentation was predominantly a painful, tender goiter. Graves disease was established by high uptake of 131I with a diffuse pattern of distribution of radioactivity on scan and the presence of thyroid-stimulating antibody. Thyrotropin-binding inhibiting IgG and antibody to thyroid microsomal antigen were both positive. She responded well to treatment with propylthiouracil and had spontaneous regression of her thyroid pain. The cause of the severe pain and tenderness remains speculative.
Asunto(s)
Enfermedad de Graves/diagnóstico , Tiroiditis Subaguda/diagnóstico , Niño , Diagnóstico Diferencial , Femenino , Humanos , Hipertiroidismo/diagnóstico , Dolor/diagnósticoRESUMEN
We have observed isolated hepatotoxicity in two children treated with propylthiouracil (PTU) for hyperthyroidism. Neither patient had risk factors for or clinical evidence of preexisting liver disease. In one patient the drug was promptly discontinued when signs of liver disease were noted. This patient quickly recovered. The second patient continued to receive PTU for several days after developing symptoms. Her illness progressed to fulminant hepatic failure with encephalopathy, and she died. These are the third and fourth pediatric cases reported, and there have been 10 cases reported in adults in the English language literature. Thirteen of the 14 patients are female. The literature regarding all these patients is reviewed. Propylthiouracil may cause lethal hepatic damage. This drug should be discontinued immediately if signs or symptoms of hepatic injury are detected.