RESUMO
Existing descriptions of liver abnormalities in ataxia-telangiectasia have been associated with co-existent hepatitis virus infection. Here we report veno-occlusive disease of the liver in 2 patients with ataxia telangiectasia that is not attributable to bone marrow transplantation or coincidental hepatitis infection.
Assuntos
Ataxia Telangiectasia/complicações , Veias Hepáticas/patologia , Trombose Venosa/complicações , Adolescente , Ascite/complicações , Ascite/terapia , Ataxia Telangiectasia/sangue , Criança , Feminino , Humanos , Masculino , Albumina Sérica/análise , Albumina Sérica/uso terapêutico , Trombose Venosa/patologiaAssuntos
Colo/inervação , Gânglios/patologia , Megacolo/patologia , Biópsia , Humanos , Lactente , Masculino , Megacolo/etiologia , Reto/inervação , Reto/patologiaRESUMO
Acquired monosaccharide intolerance is characterized by the malabsorption of all carbohydrates, resulting in profuse acidic diarrhea. Five infants with this syndrome, ranging from two to six months of age, were studied by measuring their ability to absorb glucose given by intestinal perfusion. Jejunal biopsies obtained at the time of perfusion were studied by light and electron microscopy, and surface area measurements were obtained. Glucose absorption and surface area were significantly decreased (P less than 0.005) during the acute phase of the disease and improved during convalescence. A linear correlation (r = 0.8757) was observed between the ability to absorb glucose and intestinal surface area. It is concluded that the loss of absorptive surface is a major cause of the reduced carbohydrate absorption and consequent diarrhea.
Assuntos
Intestinos/patologia , Síndromes de Malabsorção/patologia , Monossacarídeos/metabolismo , Atrofia , Convalescença , Feminino , Glucose/metabolismo , Humanos , Lactente , Recém-Nascido , Doenças do Recém-Nascido , Absorção Intestinal , Mucosa Intestinal/patologia , Mucosa Intestinal/ultraestrutura , Jejuno/patologia , Síndromes de Malabsorção/metabolismo , Masculino , Microvilosidades/ultraestruturaRESUMO
Modular Formula is a dietary preparation for use in treating infants with complex malabsorptive disorders and for weaning infants from total parenteral nutrition. With this formula the physician can alter the quality of the various nutrients normally fed to infants as well as concentrations of those nutrients. Infants who cannot tolerate existing proprietary formulas will often thrive with the judicious use of this flexible formula. Modular Formula provides the clinician with a useful aid in the diagnosis and treatment of chronic diarrhea of infancy.
Assuntos
Diarreia Infantil/dietoterapia , Alimentos Infantis/efeitos adversos , Diarreia Infantil/diagnóstico , Carboidratos da Dieta , Gorduras na Dieta , Proteínas Alimentares , Humanos , Lactente , Recém-Nascido , Síndromes de MalabsorçãoRESUMO
Eight patients with inflammation of the skin about the cutaneous stoma responded to local application of a 20% cholestyramine ointment in a short period of time. Two children treated with the hydrophilic base (Aquaphor) alone did not respond to treatment. The ileal bile acid patterns of two children with ileostomies were found to be different from that of the control group. Ileostomy patients had a higher concentration for total bile acids and a higher percentage of free bile acids.