RESUMO
OBJECTIVE: To determine the influence of the new onset of esophageal variceal hemorrhage (EVH) on transplant-free survival in children with biliary atresia and to examine variables that predicted survival after the onset of EVH. METHODS: Retrospective chart review of 134 patients with biliary atresia who underwent portoenterostomy between 1973 and 1992 at a single institution; 29% had EVH. RESULTS: The risk of death or need for liver transplantation was 50% at 6 years after the initial episode of EVH. Patients with a serum bilirubin concentration < or =4 mg/dL at the first episode of EVH had transplant-free survival of >80% for 4 years after this episode, those with bilirubin levels >4 to 10 mg/dL had 50% survival at 1 year, and those with bilirubin levels >10 mg/dL had 50% survival at 4 months. The risk of death or transplant for a child with EVH and total serum bilirubin levels >10 mg/dL was 12.0 (95% CI: 6.0, 24.1), 4 to 10 mg/dL was 7.2 (3.1, 16.7), and < or =4 mg/dL was 0.6 (0.1, 3.1) times the risk of a same-aged child who did not have EVH. CONCLUSIONS: Children with biliary atresia and first EVH episode have a variable prognosis related to total serum bilirubin concentration at the time of the episode.
Assuntos
Atresia Biliar/cirurgia , Varizes Esofágicas e Gástricas/mortalidade , Hemorragia Gastrointestinal/mortalidade , Transplante de Fígado , Atresia Biliar/complicações , Bilirrubina/sangue , Criança , Pré-Escolar , Varizes Esofágicas e Gástricas/complicações , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Risco , Taxa de SobrevidaRESUMO
OBJECTIVES: The transglutaminase (TG) antibody test is accurate in identifying celiac disease in symptomatic children. We sought to determine the positive predictive value of this test in asymptomatic children at genetic risk for celiac disease. STUDY DESIGN: Asymptomatic children with a genetic risk for celiac disease were studied to investigate the relationships between TG antibody titer, small bowel histology, growth, and clinical features. Small bowel biopsy histology was graded by using the system of Marsh. RESULTS: Of 30 children with a positive TG antibody test result, 21 (70%) had definite (Marsh score 2 or 3) and 4 (13%) had possible (Marsh score 1) biopsy evidence of celiac disease. TG antibody titer correlated with Marsh score (r = 0.569, P <.01). There was an inverse correlation between Marsh score and height z score (r = -0.361, P =. 05). CONCLUSIONS: In this group of asymptomatic children screened because of a genetic risk, TG antibodies have a positive predictive value of 70% to 83% for biopsy evidence of celiac disease and may identify children before clinical features of celiac disease develop.
Assuntos
Autoanticorpos/análise , Doença Celíaca/enzimologia , Doença Celíaca/genética , Predisposição Genética para Doença , Transglutaminases/imunologia , Adolescente , Doença Celíaca/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Intestino Delgado/enzimologia , Intestino Delgado/imunologia , Intestino Delgado/patologia , Masculino , Valor Preditivo dos Testes , Radioimunoensaio , Estatísticas não ParamétricasAssuntos
Obesidade/epidemiologia , Alanina Transaminase/sangue , Aspartato Aminotransferases/sangue , Criança , Doença Crônica , Fígado Gorduroso/etiologia , Promoção da Saúde , Humanos , Estilo de Vida , Hepatopatias/tratamento farmacológico , Hepatopatias/etiologia , Obesidade/sangue , Obesidade/etiologia , Ácido Ursodesoxicólico/uso terapêuticoRESUMO
OBJECTIVE: To prospectively evaluate the biochemical status of vitamins A, D, and E in children with cystic fibrosis (CF). SUBJECTS: A total of 127 infants identified by the Colorado CF newborn screening program. DESIGN: Vitamin status (serum retinol, 25-hydroxy vitamin D, ratio of alpha-tocopherol/total lipids) and serum albumin were assessed at diagnosis (4 to 8 weeks), ages 6 months, 12 months, and yearly thereafter, to age 10 years. RESULTS: Deficiency of 1 or more vitamins was present in 44 (45.8%) of 96 patients at age 4 to 8 weeks as follows: vitamin A 29.0%, vitamin D 22.5%, and vitamin E 22.8%. Of these patients with initial deficiency, the percent that was deficient at 1 or more subsequent time points, despite supplementation, was vitamin A 11.1%, vitamin D 12.5%, and vitamin E 57.1%. Of the initial patients with vitamin sufficiency, the percent who became deficient at any time during the 10-year period was as follows: vitamin A 4.5%, vitamin D 14.4%, and vitamin E 11.8%. The percent of patients deficient for 1 or more vitamins ranged from 4% to 45% for any given year. CONCLUSIONS: Despite supplementation with standard multivitamins and pancreatic enzymes, the sporadic occurrence of fat-soluble vitamin deficiency and persistent deficiency is relatively common. Frequent and serial monitoring of the serum concentrations of these vitamins is therefore essential in children with CF.
Assuntos
Fibrose Cística/metabolismo , Deficiência de Vitamina A/epidemiologia , Deficiência de Vitamina D/epidemiologia , Deficiência de Vitamina E/epidemiologia , Criança , Pré-Escolar , Fibrose Cística/diagnóstico , Fibrose Cística/tratamento farmacológico , Seguimentos , Fármacos Gastrointestinais/uso terapêutico , Humanos , Lactente , Recém-Nascido , Triagem Neonatal , Pancrelipase/uso terapêutico , Estudos Prospectivos , Fatores de Tempo , Vitamina A/sangue , Deficiência de Vitamina A/diagnóstico , Vitamina D/sangue , Deficiência de Vitamina D/diagnóstico , Vitamina E/sangue , Deficiência de Vitamina E/diagnóstico , Vitaminas/uso terapêuticoRESUMO
OBJECTIVE: To describe clinical and histologic features of liver disease in infants and children with Navajo neuropathy (NN). METHODS: Physicians at Navajo Area Indian Health Service facilities and neurologists and gastroenterologists at regional referral hospitals were surveyed for identification of patients born between 1980 and 1994 with known or suspected NN. Clinical records and liver histologic findings were reviewed. RESULTS: Liver disease was present in all children with NN. Three clinical phenotypes of NN were observed, based on age at presentation and course: infantile NN presented in 5 infants before 6 months of age with jaundice and failure to thrive and progressed to liver failure before 2 years of age; childhood NN presented in 6 children between 1 and 5 years of age with liver dysfunction, which progressed to liver failure and death within 6 months; and classical NN presented in 9 children with variable onset of liver disease but progressive neurologic deterioration. Liver histologic findings were characterized by multinucleate giant cells, macrovesicular and microvesicular steatosis, pseudo-acini, inflammation, cholestasis, and bridging fibrosis and cirrhosis. Cases of all 3 phenotypes occurred within the same kindred. CONCLUSIONS: Liver disease is an important component of NN and may be the predominant feature in infants and young children. We propose changing the name of this disease to Navajo neurohepatopathy.
Assuntos
Indígenas Norte-Americanos , Hepatopatias/etnologia , Doenças do Sistema Nervoso/etnologia , Idade de Início , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Fígado/patologia , Fígado/ultraestrutura , Hepatopatias/complicações , Hepatopatias/diagnóstico , Hepatopatias/genética , Doenças do Sistema Nervoso/complicações , Doenças do Sistema Nervoso/genética , Linhagem , Fenótipo , Sudoeste dos Estados UnidosRESUMO
Most studies of prenatal cocaine exposure have found gestational age or intrauterine growth deficits but few, if any, cognitive effects. In a large, well-controlled study we detected cognitive deficits in relation to heavy cocaine exposure. These findings demonstrate that prenatal exposure to cocaine at sufficiently high doses early in pregnancy has the potential to produce cognitive changes in infants and that more focused, narrow-band tests may be necessary to detect these subtle neurobehavioral effects. A total of 464 inner-city, black infants whose mothers were recruited prenatally on the basis of pregnancy alcohol and cocaine use were tested at 6.5, 12, and 13 months of age. Standard analyses, based on presence or absence of cocaine use during pregnancy, confirmed effects on gestational age but failed to detect cognitive effects. A new approach to identifying heavy users found that heavy exposure early in pregnancy was related to faster responsiveness on an infant visual expectancy test but to poorer recognition memory and information processing, deficits consistent with prior human and animal findings. These persistent neurobehavioral effects of heavy prenatal cocaine exposure appear to be direct effects of exposure and independent of effects on gestational age.
Assuntos
Cocaína , Transtornos Cognitivos/etiologia , Transtornos Relacionados ao Uso de Opioides/complicações , Complicações na Gravidez , Efeitos Tardios da Exposição Pré-Natal , Alcoolismo/complicações , Análise de Variância , Desenvolvimento Infantil , Feminino , Humanos , Lactente , Masculino , Gravidez , Fumar/efeitos adversosRESUMO
OBJECTIVE: To describe fulminant hepatic failure (FHF) in children in the United States with clinical and histopathologic features distinctly different from those typical of FHF. PATIENTS: Seven young children were seen in early 1994 with encephalopathy, coagulopathy, and elevated aminotransferase levels. Liver failure was preceded by a prodromal viral illness that resulted in a period of fasting without dehydration. Unlike the majority of children with FHF, these patients had serum bilirubin levels < 171 mumol/L (10 mg/dl). All children had received therapeutic doses of acetaminophen during the prodromal illness. HISTOPATHOLOGIC FINDINGS: Histologic findings included zonal necrosis of hepatocytes in a centrilobular distribution, which is characteristic of toxic liver injury but is atypical for viral hepatitis and sporadic non-A non-B hepatitis. OUTCOME: Six patients recovered spontaneously, and one died of complications of liver failure and fungal sepsis. The cause of this disorder remains unknown, but we postulate a viral or environmental insult that preferentially damages zone 3 hepatocytes. The potential for this injury may have been augmented by ingestion of therapeutic doses of acetaminophen while patients were in a fasted state. The prognosis was good compared with typical FHF in children and correlated with the degree of liver necrosis on histologic examination.
Assuntos
Encefalopatia Hepática/diagnóstico , Fígado/patologia , Acetaminofen/efeitos adversos , Acetaminofen/sangue , Acetaminofen/uso terapêutico , Pré-Escolar , Feminino , Febre/tratamento farmacológico , Encefalopatia Hepática/sangue , Humanos , Lactente , Fígado/efeitos dos fármacos , Masculino , Necrose/etiologia , Necrose/patologia , Índice de Gravidade de DoençaRESUMO
Nine children aged 18 months to 17 years (mean 5.7 years) with chronic hepatitis b virus infection and chronic active hepatitis were treated with 5 to 6 million units/m2 of body surface area of interferon -alpha 2b administered subcutaneously three times per week for 4 months (n = 1) or 6 months (n = 8). At 12 months after the start of therapy, six children less than 3 years of age responded to the treatment (three completely and three partially), whereas only one of three children older than 7 years of age responded. We conclude that IFN treatment may be effective in children with chronic HBV infection, especially when administered while they are young.