Subject(s)
Humans , Male , Lung Diseases , Celiac Disease , Hemosiderosis/etiology , Hemosiderosis/therapyABSTRACT
Background: Twenty percent of patients with chronic hepatitis C evolve to cirrhosis in 10 to 20 years. The degree of steatosis and hepatic iron stores in liver biopsy increase the risk. Age, high body mass index, diabetes mellitus and alcohol consumption are factors associated to the severity of liver damage. Aim: To study the association of steatosis and increased iron stores in the liver biopsy and age, overweight, alcohol consumption and diabetes with the severity of liver damage in patients with hepatitis C virus infection. Patients and methods: Retrospective study of 84 liver biopsies of patients with chronic infection with hepatitis C virus were studied. The pathological appearance was classified as stage I when chronic hepatitis with mild activity without fibrosis was observed; as stage II when moderate chronic hepatitis with mild fibrosis was observed and as stage III when there was a moderate chronic hepatitis with fibrosis or cirrhosis. The amount of steatosis and iron deposition in the biopsy were also assessed. Results: Forty one percent of patients were in stage I, 32% in stage II and 27% in stage III. Patients in stage I were younger than those in stages II and III (40.7 and 52.2 years respectively, p <0,001). No association between the severity of liver damage and the degree of steatosis, hemosiderosis, body mass index or alcohol intake, was observed. The frecuency of diabetes mellitus increased along with pathological staging (3, 15 and 30% in stages I, II and III, respectively, p <0,05). Conclusions: This study confirms that severity of chronic hepatitis C is associated with age and the presence of diabetes mellitus.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Fatty Liver/pathology , Hepatitis C, Chronic/pathology , Alcohol Drinking/adverse effects , Biopsy , Body Mass Index , Diabetes Complications , Hemosiderosis/etiology , Hemosiderosis/pathology , Hepatitis C, Chronic/classification , Liver Cirrhosis/pathology , Overweight , Retrospective Studies , Severity of Illness IndexABSTRACT
La anemia de Diamond-Blackfan es una causa poco frecuente de anemia en el recién nacido y el lactante menor. Analizamos retrospectivamente las características clínicas y de laboratorio en 20 pacientes controlados en un período de 30 años. Encontramos que en el 85 por ciento de los casos el diagnóstico se planteó antes del año de edad y la mayoría eran de sexo femenino. En 45 por ciento existía el antecedente de bajo peso de nacimiento. El principal hallazgo al examen físico, además de la palidez, fue la talla baja y no se encontraron otras malformaciones asociadas en la mayoría de los pacientes. El 100 por ciento presentaba valores de hemoglobina por debajo de los valores normales para su edad, con macrocitosis y reticulocitopenia, sin compromiso del resto de las series hematológicas. La respuesta adecuada a corticoides se observó en 85 por ciento. De los 3 pacientes refractarios, 2 fallecieron y 1 sobrevive con dependencia a transfusiones de glóbulos rojos y hemosiderosis secundaria, ya que no fue posible efectuar trasplante de médula ósea por falta de donante. El trasplante debe ser considerado precozmente como alternativa terapéutica en el grupo no respondedor a corticoides, si existe donante compatible, ya que otros tratamientos son poco exitosos
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Fanconi Anemia/diagnosis , Infant, Low Birth Weight , Blood Transfusion , Fanconi Anemia/complications , Fanconi Anemia/therapy , Hemoglobins/deficiency , Hemosiderosis/etiology , Infant, Low Birth Weight , Prednisone/therapeutic use , Retrospective Studies , Bone Marrow TransplantationSubject(s)
Humans , beta-Thalassemia/complications , /pathology , /complications , Hemosiderosis/etiology , Liver/pathologyABSTRACT
Os autores mostram um caso de Hemossiderose Pulmonar Idiopatica na forma de inicio infantil, com uma sobrevida bem maior que a relatada na literatura. A crianca tem 5 anos de evolucao da doenca, e poucos sinais de cronicidade, apesar dos episodios graves de sangramento pulmonar, o que se opoe a descricao classica do quadro. Foi sugerida a alergia a proteina do leite de vaca como etiologia da doenca, e o tratamento foi feito com corticoide durante os surtos, com rapida resposta. Observou-se tambem varios episodios de recrudescen-cia por fuga da dieta, que nao ocorreram apos orientacao de uma equipe multidis-ciplinar. Os autores discutem ainda outros aspectos da doenca e fazem uma revi-sao da literatura.
Subject(s)
Child , Humans , Female , Hemosiderosis/etiology , Lung , Adrenal Cortex Hormones/drug therapy , Breast-Milk Substitutes/adverse effects , Hemosiderosis/therapyABSTRACT
Os autores relatam uma modificaçäo técnica nas hemisferectomias totais, com intuito de prevenir os sangramentos tardios e distorçöes cerebrais
Subject(s)
Humans , Cerebral Decortication/methods , Cerebral Decortication/adverse effects , Cerebral Hemorrhage/prevention & control , Craniotomy/methods , Hemosiderosis/etiology , Hemosiderosis/prevention & controlABSTRACT
É feita breve revisäo sobre o metabolismo do ferro no qual os Autores chamam a atençäo para o equilibrio que deve existir entre as quantidades ingeridas com a alimentaçäo e as condiçöes de absorçäo desse metal. Como no homem näo há via de excreçäo do ferro as trocas que se fazem entre o organismo e o meio externo devem ser bem conhecidas para evitar processo de depósito, condiçäo muito mais grave que sua carência. Revêm o mecanismo de absorçäo do ferro ao nível das células do intestino e sua distribuiçäo no organismo. O ferro é encontrado ligado a proteínas: hemoglobina, hemo-enzimas, hemo-pigmentos, mioglobina, ferritina e hemossiderina, estas duas últimas sob a forma de material armazenado. Abordam os tipos de carência: 1 - por falta de absorçäo; 2 - por perda hemorrágica e 3 - misto. Analisam os mecanismos envolvidos nestas situaçöes assim como seu tratamento. O excesso de depósito de ferro (hemossiderose e hemocromatose) é revisto, bem como as possibilidades terapêuticas. Chamam a atençäo para as campanhas comunitárias que se propöem a combater em geral, as carências de ferro das populaçöes de nível sócio-econômico mais baixo, atentando para os perigos que daí podem advir, ou seja, o aumento de incidência de hemossiderose e da hemocromatose
Subject(s)
Humans , Hemochromatosis/etiology , Hemosiderosis/etiology , Intestinal Absorption , Iron/metabolism , Chelating Agents/therapeutic use , Diet , Food, Fortified/adverse effects , Hemochromatosis/therapy , Hemosiderosis/therapy , Iron/deficiency , PhlebotomySubject(s)
Adolescent , Adult , Hypoxia/etiology , Arrhythmias, Cardiac/etiology , Blood Transfusion , Child , Heart Diseases/diagnosis , Heart Failure/etiology , Heart Valve Diseases/etiology , Hemochromatosis/etiology , Hemosiderosis/etiology , Humans , Pericarditis/etiology , Pulmonary Embolism/etiology , Rheumatic Heart Disease/etiology , Thalassemia/complicationsABSTRACT
Cardiac complications are the leading cause of death in patients with the thalassaemia syndromes. The cardiac abnormalities may be the result of chronic anaemia or iron overload. The cardiac status was evaluated in 30 patients with thalassaemia to determine whether the structural and functional changes could be related to the degree of anaemia or to other factors suggesting iron overload. Clinical, radiological, electrocardiographic and echocardiographic methods were employed. The study provided evidence that cardiac abnormalities were due to undertransfusion. Echocardiography provides a reliable, noninvasive means of assessing cardiac abnormalities in patients with thalassaemia.