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1.
Endocrinol Metab Clin North Am ; 28(4): 709-29, viii, 1999 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-10609116

RESUMO

Type 2 diabetes mellitus is a disease of adults and has been considered rare in the pediatric population. Over the last decade, however, there has been a disturbing trend of increasing cases of type 2 diabetes in children, particularly adolescents, and with a greater proportion of minority children being affected. This article reviews the clinical characteristics of youth with type 2 diabetes, presents the risk factors associated with insulin resistance and type 2 diabetes, discusses treatment options, and projects future directions in research. The ultimate goal is to raise awareness of this challenging entity among healthcare professionals.


Assuntos
Diabetes Mellitus Tipo 2/epidemiologia , Grupos Minoritários , Adolescente , Criança , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/terapia , Feminino , Hispânico ou Latino , Humanos , Indígenas Norte-Americanos , Masculino , México/etnologia
3.
J Pediatr ; 133(1): 151-3, 1998 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-9672532

RESUMO

The upper lip of 17 consecutive individuals with various forms of holoprosencephaly were examined either at autopsy or during clinical evaluation. A total of 88% of cases were missing the superior labial frenulum regardless of the severity of holoprosencephaly or other associated craniofacial defects. Because the frenulum was found to be missing across a complete clinical spectrum of holoprosencephaly including cases exhibiting only minimal craniofacial features, it should be inspected as part of the craniofacial examination, and its absence should be prompt imaging studies of the brain. Absence of the frenulum in holoprosencephaly also provides evidence that its embryonic origin is that of the medial nasal process.


Assuntos
Holoprosencefalia , Freio Labial/anormalidades , Holoprosencefalia/diagnóstico , Holoprosencefalia/embriologia , Humanos
4.
West J Med ; 168(1): 11-6, 1998 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-9448482

RESUMO

To define the clinical and metabolic characteristics of children with non-insulin-dependent diabetes mellitus (NIDDM), we reviewed the medical records of 18 children and adolescents who met either or both of the following criteria for the diagnosis of the disease: evidence of continued endogenous secretion of insulin beyond that expected in insulin-dependent diabetes mellitus and satisfactory glycemic control with diet alone or in combination with an oral hypoglycemic agent more than 2 years from the time of diagnosis. Patients who met these criteria but had islet cell antibodies or insulin autoantibodies were eliminated from the study group. Patients with NIDDM constituted 8% of all patients with diabetes seen in our pediatric clinics and 19% of diabetic patients of Central or South American ancestry. Of the 18 patients, 12 (67%) were Mexican American. The mean age of onset was 12.8 years (range, 5 to 17). Obesity (n = 9) and acanthosis nigricans (n = 12) were common findings. Ketonuria was present at diagnosis in 5 (33%) of 15 patients and acidosis in 2 of 14 (14%). Challenge with a nutritional supplement (Sustacal, Mead Johnson Nutritionals) (n = 10) showed a mean fasting serum C-peptide concentration of 1.19 nmol per liter (3.6 ng per ml). A family history of NIDDM was present in 13 (87%) of 15 patients, with 7 (47%) having 3 or more generations affected. Children with NIDDM are an important subset of those with diabetes, and this disease should be suspected in diabetic children presenting without ketoacidosis and with acanthosis nigricans, obesity, and a strong family history, particularly among those of Mexican-American ethnicity. Children with these characteristics should undergo testing of endogenous insulin secretion for appropriate therapeutic intervention.


Assuntos
Diabetes Mellitus Tipo 2/etnologia , Americanos Mexicanos , Adolescente , Distribuição por Idade , Idade de Início , California/epidemiologia , Criança , Pré-Escolar , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/epidemiologia , Feminino , Humanos , Masculino , Prevalência , Sistema de Registros , Fatores de Risco , Distribuição por Sexo
5.
J Pediatr ; 131(5): 718-21, 1997 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-9403652

RESUMO

OBJECTIVE: To assess general intellectual functioning in children with histories of heavy prenatal alcohol exposure, with or without the facial features and growth deficiencies characteristic of fetal alcohol syndrome (FAS). DESIGN: Forty-seven alcohol-exposed children were recruited on evaluation at a dysmorphology clinic and evaluated as part of a university research project using standard tests of IQ. Thirty-four of the alcohol-exposed patients met the traditional diagnostic criteria for FAS. The other 13 alcohol-exposed children lacked both the pattern of facial features and prenatal or postnatal growth deficiency characteristic of the diagnosis. RESULTS: Compared with normal control subjects matched for age, sex, and ethnicity, both groups of alcohol-exposed children displayed significant deficits in overall IQ measures and deficits on most of the subtest scores. Although those in the nondysmorphic group usually obtained marginally higher IQ scores than those in the FAS group, few significant differences were found between the two alcohol-exposed groups. CONCLUSIONS: These results indicate that high levels of prenatal alcohol exposure are related to an increased risk for deficits in intellectual functioning and that these can occur in children without all of the physical features required for a diagnosis of FAS. They also emphasize the need for conducting a thorough history of prenatal alcohol exposure in children with intellectual deficits.


Assuntos
Transtornos Cognitivos/etiologia , Etanol/efeitos adversos , Transtornos do Espectro Alcoólico Fetal/diagnóstico , Transtornos do Espectro Alcoólico Fetal/etiologia , Inteligência/efeitos dos fármacos , Efeitos Tardios da Exposição Pré-Natal , Transtornos Relacionados ao Uso de Substâncias/complicações , Criança , Transtornos Cognitivos/diagnóstico , Feminino , Humanos , Masculino , Gravidez , Índice de Gravidade de Doença , Escalas de Wechsler
6.
J Pediatr ; 123(6): 983-5, 1993 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8229534

RESUMO

Two infants with congenital syphilis and persistent hypoglycemia were found to have hypopituitarism. Hypopituitarism should be recognized as a potential complication of congenital syphilis; affected infants with persistent hypoglycemia should receive a prompt evaluation of pituitary function.


Assuntos
Hipoglicemia/etiologia , Hipopituitarismo/complicações , Doenças do Prematuro/diagnóstico , Sífilis Congênita/complicações , Humanos , Hipopituitarismo/diagnóstico , Recém-Nascido , Recém-Nascido Prematuro , Masculino
7.
J Pediatr ; 116(2): 258-61, 1990 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-2299497

RESUMO

The medical records of 102 live-born children with a congenital diaphragmatic defect were reviewed to determine the frequency and nature of underlying chromosomal, genetic, and nongenetic patterns of malformation. Overall, 40 children (39%) had a major nonpulmonary malformation, and 14 of these children (14%) had a previously recognized pattern of malformation. A group of 18 children (18%) with cardiac anomalies had an increased mortality rate in comparison with those children without cardiac defects (72% vs 38%). The frequency and severity of nonpulmonary abnormalities in children with congenital diaphragmatic defects suggest that examination of affected children should include cardiac evaluation, a karyotype when the defect is one feature of a broader pattern of altered development, and a careful evaluation for minor anomalies, which may provide clues to an overall diagnosis.


Assuntos
Anormalidades Múltiplas/epidemiologia , Eventração Diafragmática/complicações , Hérnias Diafragmáticas Congênitas , Anormalidades Múltiplas/classificação , Criança , Eventração Diafragmática/mortalidade , Hérnia Diafragmática/complicações , Hérnia Diafragmática/mortalidade , Humanos , Incidência
8.
J Pediatr ; 111(4): 538-43, 1987 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-3655984

RESUMO

The association of ectrodactyly and absence of long bones of the upper or lower extremities has been recognized previously in nine families. We report 24 additional individuals in four families who are similarly affected. Two of these families manifest the unusual feature of unilateral preaxial polydactyly of a lower extremity. Data from these four families plus the nine previously reported suggest that ectrodactyly associated with absence of long bones of the upper or lower limbs is a genetically determined disorder, inherited as an autosomal dominant trait with widely variable expression or nonpenetrance.


Assuntos
Braço/anormalidades , Deformidades Congênitas do Pé/genética , Deformidades Congênitas da Mão/genética , Perna (Membro)/anormalidades , Adulto , Aberrações Cromossômicas/genética , Transtornos Cromossômicos , Feminino , Genes Dominantes , Humanos , Recém-Nascido , Masculino , Linhagem
9.
J Pediatr ; 111(2): 236-9, 1987 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-3612395

RESUMO

The term hemifacial microsomia refers to unilateral defects in development of structures derived from the first and second branchial arches. Recently we evaluated three unrelated children who had a similar pattern of unilateral craniofacial defects that was associated with other structural abnormalities having a disruptive vascular pathogenesis. The clinical findings in these patients suggest that one cause of hemifacial microsomia is in utero interruption of blood flow.


Assuntos
Região Branquial/irrigação sanguínea , Ossos Faciais/anormalidades , Crânio/anormalidades , Adolescente , Artérias Carótidas/anormalidades , Artérias Carótidas/diagnóstico por imagem , Pré-Escolar , Feminino , Humanos , Recém-Nascido , Masculino , Microftalmia/etiologia , Radiografia , Síndrome
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