Hemolytic disease of the newborn due to anti-U

Anti-U is a rare red blood cell alloantibody that has been found exclusively in blacks. It can cause hemolytic disease of the newborn and hemolytic transfusion reactions. We describe the case of a female newborn presenting a strongly positive direct antiglobulin test due to an IgG antibody in cord blood. Anti-U was recovered from cord blood using acid eluate technique. Her mother presented positive screening of antibodies with anti-U identified at delivery. It was of IgG1 and IgG3 subclasses and showed a titer of 32. Monocyte monolayer assay showed moderate interaction of Fc receptors with maternal serum with a positive result (3.1%). The newborn was treated only with 48 hours of phototherapy for mild hemolytic disease. She recovered well and was discharged on the 4th day of life. We conclude that whenever an antibody against a high frequency erythrocyte antigen is identified in brown and black pregnant women, anti-U must be investigated.

Pulmonary hemorrhage as a manifestation of systemic lupus erythematosus

The authors report a case of a 19-year-old woman admitted for the investigation of fever and hemolytic anemia for the previous 2 months. As an inpatient, she had convulsions and sudden loss of consciousness, developing hemoptysis, hypoxia, and respiratory insufficiency. Examination showed pericardial effusions on the echocardiogram and bilateral alveolar condensations on the thoracic radiograph. A hypothetical diagnosis of systemic lupus erythematosus was made, and measurement of the antinuclear factor was requested along with daily pulse therapy methylprednisolone, in spite of which the outcome was fatal. Afterwards, the result of the antinuclear factor test was positive, with a titer of 1:5120, showing a fine punctiform pattern, fulfilling the criteria for systemic lupus erythematosus according to the American College of Rheumatology. Secondary pulmonary hemorrhage in this connective tissue disease is an uncommon but serious complication that involves a high level of mortality in spite of intensive treatment, as is also reported in the literature.

Anemia ferropriva em atletas adolescentes da Fundação Vila Olímpica de Manaus-AM

Considerando a escassez de informações quanto ao estado nutricional de atletas jovens, identificou-se a prevalência de anemia ferropriva e sua associação com indicadores nutricionais de atletas adolescentes participantes do Programa de Iniciação Esportiva da Fundação Vila Olímpica de Manaus-AM. A amostra foi constituída por 194 atletas adolescentes do gênero masculino, voluntários, praticantes de diferentes modalidades esportivas classificados como iniciantes. A coleta dos dados foi realizada mediante verificação de medidas antropométricas (peso corporal, estatura e espessuras das dobras cutâneas tricipital e subescapular), dosagem de hemoglobina utilizando o hemoglobinômetro portátil (Sistema Hemocue) e instrumento socioeconômico. No diagnóstico do estado nutricional foram utilizados os indicadores Estatura para a Idade (E/I) e Índice de Massa Corporal para a idade (IMC/I), assumindo pontos de corte propostos pela OMS. Os resultados mostraram que 9,4% dos adolescentes apresentaram desnutrição, 8,2% sobrepeso e 4,6% obesidade. A prevalência de anemia ferropriva encontrada na amostra foi de 41,7%, com predominância na classe socioeconômica mais baixa. Embora a prevalência de anemia tenha sido elevada, o estado nutricional dos adolescentes não influenciou sobre este resultado, considerando que a maioria dos adolescentes apresentou-se eutrófica. Diante do exposto, sugere-se a adoção de medidas efetivas de intervenção e de educação nutricional visando à minimização e/ou o controle da anemia ferropriva.

Anemia e marcadores séricos da deficiência de ferro em grávidas atendidas na rede pública municipal de Manaus, Amazonas, Brasil

O presente estudo teve como objetivo avaliar a anemia em grávidas, associando os resultados da dosagem de hemoglobina e hematócrito com a análise de marcadores do perfil sérico do ferro. Participaram do estudo 92 grávidas que estavam realizando pré-natal em unidades de atendimento à saúde no Município de Manaus, Amazonas, Brasil. Foi aplicado um formulário para obtenção dos dados antropométricos e informações sobre estilo de vida, além de serem realizadas dosagens dos níveis séricos de ferro, capacidade latente de ligação do ferro (CLLF), capacidade total de ligação do ferro (CTLF), índice de saturação da transferrina (IST), transferrina, ferritina e níveis sanguíneos de hemoglobina e hematócrito por metodologia automatizada utilizando kits comerciais disponíveis. Foram encontradas 26,1% de grávidas com níveis de hemoglobina abaixo de 11 g/dL. Observou-se que 17,4% das grávidas com níveis normais de hemoglobina apresentavam níveis inadequados de ferro sérico e 9,8% apresentavam níveis baixos de ferritina sérica. Os níveis de ferritina e de hemoglobina apresentaram diferença significativa entre os trimestres de gestação (p < 0,05, ANOVA). Os resultados sugerem que a dosagem da hemoglobina juntamente com outros marcadores do perfil sérico do ferro pode trazer uma avaliação mais precisa da deficiência de ferro na gravidez.

In vitro screening of Amazonian plants for hemolytic activity and inhibition of platelet aggregation in human blood

In the present study, different aerial parts from twelve Amazonian plant species found in the National Institute for Amazon Research's (INPA's) Adolpho Ducke Forest Reserve (in Manaus, Amazonas, Brazil) were collected. Separate portions of dried, ground plant materials were extracted with water (by infusion), methanol and chloroform (by continuous liquid-solid extraction) and solvents were removed first by rotary evaporation, and finally by freeze-drying which yielded a total of seventy-one freeze-dried extracts for evaluation. These extracts were evaluated initially at concentrations of 500 and 100 µg/mL for in vitro hemolytic activity and in vitro inhibition of platelet aggregation in human blood, respectively. Sixteen extracts (23 % of all extracts tested, 42 % of all plant species), representing the following plants: Chaunochiton kappleri (Olacaceae), Diclinanona calycina (Annonaceae), Paypayrola grandiflora (Violaceae), Pleurisanthes parviflora (Icacinaceae), Sarcaulus brasiliensis (Sapotaceae), exhibited significant inhibitory activity towards human platelet aggregation. A group of extracts with antiplatelet aggregation activity having no in vitro hemolytic activity has therefore been identified. Three extracts (4 %), all derived from Elaeoluma nuda (Sapotaceae), exhibited hemolytic activity. None of the plant species in this study has known use in traditional medicine. So, these data serve as a baseline or minimum of antiplatelet and hemolytic activities (and potential usefulness) of non-medicinal plants from the Amazon forest. Finally, in general, these are the first data on hemolytic and inhibitory activity on platelet aggregation for the genera which these plant species represent.

Sintomas depressivos em crianças e adolescentes com anemia falciforme

Objetivo: Determinar a frequência de sintomas depressivos em crianças e adolescentes com anemia falciforme, bem como caracterizar e associar tal sintomatologia aos dados individuais. Métodos: Realizou-se um estudo transversal com portadores de anemia falciforme dos 7 aos 17 anos, atendidos em um ambulatório de Hematologia Pediátrica, os quais preencheram o Inventário de Depressão Infantil (CDI). Resultados: Foram avaliados 76 pacientes, estabelecendo-se em 13 o ponto de corte. Vinte e seis pacientes (34,2%) apresentavam sintomas sugestivos de depressão. "Não ser tão bom quanto os outros colegas" foi o item mais pontuado, além de ter predominado significativamente naqueles com escore de CDI maior ou igual a 13, assim como as variáveis: ter pais separados ou viúvos e renda familiar mensal menor ou igual a R$ 510,00. O item "preocupação com dores" foi bem pontuado, independentemente da presença de sintomas depressivos (p = 0,1). Conclusão: A depressão em crianças e adolescentes com anemia falciforme ainda é pouco estudada. Os dados obtidos indicam frequência elevada de sintomas depressivos nessa população. Possivelmente, conviver precocemente com a separação dos pais e pertencer a uma família com baixa renda esteja relacionado ao desenvolvimento desses sintomas.

Doppler echocardiographic study in adolescents and young adults with sickle cell anemia

OBJECTIVE: Anatomical and functional assessment of the heart through Doppler and echocardiography in patients with cell anemia (SCA). METHODS: Twenty-five patients with SCA and ages ranging from 14 to 45 years were prospectively studied in a comparison with 25 healthy volunteers. All of them underwent clinical and laboratory evaluation and Doppler echocardiography as well.The measurements were converted into body surface indices. RESULTS: There were increases in all chamber diameters and left ventricle (LV) mass of the SCA patients. It was characterised an eccentric hypertrophy of the left ventricle. The preload was increased (left ventricle end-diastolic volume) and the afterload was decreased (diastolic blood pressure, peripheral vascular resistance and end-systolic parietal stress ESPS). The cardiac index was increased due to the stroke volume. The ejection fraction and the percentage of the systolic shortening , as well as the systolic time intervals of the LV were equivalent. The isovolumetric contraction period of the LV was increased. The mitral E-septum distance and the end-systolic volume index (ESVi) were increased. The ESPS/ESVi ratio,a loading independent parameter, was decreased in SCA, suggesting systolic dysfunction. No significant differences in the diastolic function or in the pulmonary pressure occurred. CONCLUSION: Chamber dilations, eccentric hypertrophy and systolic dysfunction confirm the evidence of the literature in characterizing a sickle cell anemia cardiomyopathy.

Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients

We report two cases of congenital atresia of the ostium of the left coronary artery. Case 1: a six-month-old infant presenting with serious cardiac insufficiency. A noninvasive diagnosis of dilated myocardiopathy was established and the clinical picture was pharmacologically compensated. When the patient was nine months of age, a hemodynamic study was performed that revealed congenital atresia of the ostium of the left coronary artery; the infant immediately underwent a successful anastomosis of the internal mammary artery with the left coronary artery. Case 2: an eleven-year-old asymptomatic boy with a history of heart murmur from the age of six months on, was refered for surgery with a diagnosis of anomalous origin of the left coronary artery from pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established during surgery. Successful surgical revascularization with the left internal mammary artery, and left ventricular aneurysmectomy were performed.

Epidemiological characteristics of congenital heart diseases in Londrina, Paraná South Brazil

OBJECTIVE: To determine the prevalence and other epidemiological characteristics of congenital heart diseases. METHODS: A retrospective population based study of children who were born in Londrina, from January '89 to December '98 (80,262 live births). Diagnoses were confirmed through autopsy, surgery, catheterization, or echocardiography. RESULTS: A total of 441 patients was as certained what corresponds to a prevalence of 5.494:1,000 live births. Ventricular septal defect was the commonest lesion. A small number of transpositions of the great vessels and of left ventricular hypoplasia was observed. A high propation of ventricular septal defect (28.3%) and atrioventricular septal defects (8.1%) occurred. Fifty-one (11.35%) affected children had syndromic diseases and 52 (12.01%) children had nonsyndromic anomalies. CONCLUSION: The prevalence of congenital heart diseases in Londrina is in accordance with that of other regions of the globe. This prevalence also may reflect the reality in the southern region of Brazil, because population characteristics are very similar in the 3 southernmost Brazilian states.

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67±3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases), stenosis 28.6% (6 cases), and double lesion 14.3% (3 cases). The perfusion 43.10±9.50min, and ischemia time were 29.40±10.50min. The average clinical follow-up in mitral insufficiency was 41.52±53.61 months. In the stenosis group (4 patients) was 46.39±32.02 months, and in the double lesion group (3 patients), 39.41±37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17±39.51 months, stenosis 42.61±30.59 months, and in the double lesion 39.41±37.51 months. RESULTS: Operative mortality was 9.5% (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3%) patients were asymptomatic (p=0.04). The majorit y with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

Echocardiographic Assessment of the Pulmonary Venous Flow: An Indicator of Increased Pulmonary Flow in Congenital Cardiac Malformations

OBJECTIVE: To identify the left inferior pulmonary vein as an indirect marker of increased pulmonary flow in congenital heart diseases.METHODS: We carried out a prospective consecutive study on 40 patients divided into 2 groups as follows: G1 - 20 patients diagnosed with congenital heart disease and increased pulmonary flow; G2 (control group) - 20 patients who were either healthy or had congenital heart disease with decreased or normal pulmonary flow. We obtained the velocity-time integral of the left inferior pulmonary vein flow, excluding the "reverse A" wave, with pulsed Doppler echocardiography.RESULTS: In G1, 19 out of the 20 patients had well-identified dilation of the left inferior pulmonary vein. No G2 patient had dilation of the left inferior pulmonary vein. Dilation of the left inferior pulmonary vein in conditions of increased pulmonary flow had sensitivity of 95%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 95% (1 false-negative case). The integral of time and velocity of the pulmonary venous flow obtained with pulsed Doppler echocardiography was greater in the G1 patients (G1=25.0±4.6 cm versus G2=14.8±2.1 cm, p=0.0001).CONCLUSION: The identification of dilation of the left inferior pulmonary vein suggests the presence of congenital heart disease with increased pulmonary flow. This may be used as an indirect sign of increased flow, mainly in malformations of difficult diagnosis, such as atrial septal defects of the venous sinus or coronary sinus type.

Supravalvular Congenital Mitral Stenosis

Congenital supravalvular mitral stenosis is a rare malformation characterized by the presence of a shelf-like fibrous membrane, with 1 or 2 small orifices, covering and obstructing the mitral valve. The membrane is positioned closely to the mitral valve (and sometimes it is attached to it); therefore, a preoperative diagnosis is inevitably difficult, even with the use of biplane echocardiography. Two patients with supravalvular mitral stenosis aged 3 years and 3 months are described. In 1 patient, a preoperative diagnosis was made, and both successfully underwent correction.