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1.
Arch. argent. pediatr ; 110(5): e91-e94, oct. 2012. ilus, tab
Artigo em Espanhol | LILACS | ID: lil-657481

RESUMO

Las hemoglobinopatías estructurales son variantes de la hemoglobina caracterizadas por la síntesis de una molécula cualitativamente diferente de la normal. La mayoría son inocuas, mientras que otras ocasionan cambios fisicoquímicos que determinan manifestaciones clínicas de gravedad variable. En el caso de las hemoglobinas inestables, las alteraciones reducen la solubilidad y facilitan la formación de complejos de hemoglobina desnaturalizada (cuerpos de Heinz) que precipitan, lo cual daña la membrana y destruye prematuramente al eritrocito. Hasta la actualidad se han descrito 142 hemoglobinas inestables, muchas de ellas ocasionan hemólisis crónica, que puede exacerbarse por infecciones o por la ingesta de medicamentos o drogas oxidantes. La hemoglobina Southampton (también conocida como hemoglobina Casper) es una variante inestable que resulta de la sustitución de un residuo de leucina por uno de prolina, en el codón ß106 (CTG ?CCG, como consecuencia de la mutación c.320 T>C. Presentamos una niña con anemia hemolítica grave, esplenomegalia y requerimiento transfusional debidos a hemoglobina Southampton.


Variant hemoglobins are the result of different types of mutations that occur in the globin genes. In many cases, these hemoglobinopathies are harmless, while in others they determine alterations in the physical and chemical properties raising clinical manifestations of variable severity. In the unstable hemoglobinopathies, the changes reduce solubility, inducing the formation of precipitates of denaturated hemoglobin (Heinz bodies), which damage the membrane and finally destroy the red blood cells prematurely. Up to now, more than 142 different unstable hemoglobins have been described, most of them cause chronic hemolysis, increased by infections or drugs. We report the clinical presentation of an unstable hemoglobin (hemoglobin Southampton) in a girl with severe hemolytic anemia, splenomegaly and red blood cell requirement.


Assuntos
Pré-Escolar , Feminino , Humanos , Anemia Hemolítica/etiologia , Hemoglobinas Anormais , Anemia Hemolítica/sangue , Anemia Hemolítica/diagnóstico , Hemoglobinas Anormais/análise , Índice de Gravidade de Doença
2.
Ginecol. obstet. Méx ; 70(7): 328-337, jul. 2002.
Artigo em Espanhol | LILACS | ID: lil-331079

RESUMO

INTRODUCTION: The principal causes of morbidity and mortality during pregnancy in Mexico, are preeclampsia/eclampsia, obstetric hemorrhage and puerperium complications; this is, 62 of maternal deaths in last years. HELLP syndrome was observed between 5 to 25 of the mortality in pregnancies of 36 weeks or less. OBJECTIVE: To analyze patients with HELLP syndrome in ICU's (Intensive Care Unit) of a Gynecology and Obstetric Hospital, related to the abnormal hematological, hepatic and renal results with the obstetric case history and the clinical complications. MATERIALS AND METHODS: A transversal study in patients with HELLP syndrome during 1998 and 1999 were carry out. CASE DEFINITION: Peripheral blood with Microangiopathic hemolysis, elevated liver enzymes: AST, ALT over 40 UI/L, even when were LDH lower than 600 UI/L. It was evaluated the hepatic and renal function, platelets count, microangiopathic hemolysis, arterial pressure, seizures, icteric skin color, blindness, visual disturbances, nausea, vomiting and upper quadrant right abdominal pain. In newborn we analyzed gestational age, sex, weight and APGAR. We studied for an association between maternal and biochemical variables with Correlation Pearson Test, and dependence between variables with lineal regression model. RESULTS: 2878 patients with hypertensives disorders in pregnancy (11.64). The 1.15 (n = 33) had HELLP syndrome with specific maternal mortality of 0.4 per 10,000 live birth, perinatal mortality of 1.62 per 10,000 live birth; and renal damage in 84.5. Coefficient beta was higher between number of pregnancies to platelets count (-0.33) and creatinine clearance (-0.401). CONCLUSION: We found an important renal damage, low platelets, elevated liver enzymes in women with two or more pregnancies. Then we propose there are similarities between HELLP syndrome and Systemic Inflammatory Response Syndrome (SIRS) because they could have the same pathophysiology.


Assuntos
Humanos , Masculino , Feminino , Gravidez , Recém-Nascido , Adulto , Anemia Hemolítica/epidemiologia , Complicações na Gravidez/epidemiologia , Hepatopatias , Pré-Eclâmpsia , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Trombocitopenia , Aborto Induzido , Anemia Hemolítica/sangue , Anemia Hemolítica/fisiopatologia , Cesárea , Comorbidade , Complicações na Gravidez/sangue , Complicações na Gravidez/fisiopatologia , Estudos Transversais , Suscetibilidade a Doenças , Doenças do Recém-Nascido/epidemiologia , Hipertensão/complicações , Mortalidade Infantil , Testes de Função Renal , Hepatopatias , Testes de Função Hepática , Idade Materna , Mortalidade Materna , México , Paridade , Pré-Eclâmpsia , Síndrome de Resposta Inflamatória Sistêmica/sangue , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Fatores Socioeconômicos , Trombocitopenia
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