Herencia conjunta de α+-talasemia y portador de hemoglobina S / Co-inheritance of α+-thalassemia and sickle trait

Resumen En este reporte de caso se describe el primer paciente doble heterocigoto para alfa+-talasemia tipo -3,7 y rasgo heterocigoto por hemoglobina S en Costa Rica, diagnosticado desde su nacimiento por medio del tamizaje neonatal como heterocigoto para hemoglobina S. Luego de la detección de la hemoglobina S por tamizaje, el paciente fue referido al servicio de Hematología del Hospital Nacional de Niños para su seguimiento, en donde se observa hemograma con índices y morfología de glóbulos rojos sugestivos de alfa talasemia, con presentación de electroforesis de hemoglobina con patrón AS cuya expresión relativa de HbS era menor de lo esperado, lo que motivó a efectuar estudio molecular del gen de alfa globina, que confirmó el diagnóstico de alfa talasemia con deleción heterocigota de tipo -3,7 en herencia conjunta con la heterocigosis de hemoglobina S.

Abstract In this case report we describe the first patient compound heterozygous for type -3.7 alpha+ thalassemia and sickle cell trait in Costa Rica, who was diagnosed from birth by neonatal screening as heterozygous for hemoglobin S. After detection of hemoglobin S by screening, the patient was referred to the Hematology service of the National Children`s Hospital for follow-up, where hemogram with indexes and morphology of red blood cells suggestive of alpha thalassemia is observed, presenting hemoglobin electrophoresis with AS pattern whose relative expression of hemoglobin S was lower tan expected, which led to a molecular study of the alpha globin gene confirming the diagnosis of alpha thalassemia with heretozygous deletion of type -3.7, in co-inheritance with hemoglobin S heterozygosis.

Anemia ferropénica en niños menores de un año usuarios de CASMU-IAMPP: prevalencia y factores asociados / Iron deficiency anemia in children younger than 1 year old users of CASMU-IAMPP: prevalence and associated factors

Resumen Introducción: la anemia ferropénica es un problema de salud nacional. Existen recomendaciones para su prevención. Para su diagnóstico temprano se incorporó a partir de 2014 la medición de la hemoglobina por punción digital entre los 8-12 meses de edad. Objetivo: determinar la prevalencia de anemia en lactantes usuarios de CASMU-IAMPP e identificar factores asociados. Material y métodos: se estudiaron lactantes entre 8-12 meses, a quienes se realizó medición de hemoglobina por punción digital, entre julio-diciembre 2014. Se describió la prevalencia de anemia. Se describieron las características de los niños con anemia y se compararon con las de un grupo control de niños sin anemia. Resultados: en el período evaluado se realizó hemoglobina digital a 95% de los lactantes entre 8-12 meses, 18,3% presentaba anemia. El 65,9% incorporó carne a la alimentación en forma tardía, 28,6% recibía dosis incorrecta de hierro suplementario y 23,4% no adhería al tratamiento. Los niños con anemia, en comparación con el grupo control, no presentaron mayor prevalencia de prematurez, peso al nacer menor a 3000 g, embarazo gemelar, anemia en el embarazo, suplementación con hierro en el embarazo, pecho directo exclusivo durante 6 meses, o inicio adecuado de alimentación complementaria. En los niños con anemia se detectó una falla en el inicio oportuno de la suplementación con hierro en dosis adecuada así como una mala adherencia al tratamiento. Discusión: debe alertar un 20% de niños con anemia en una población seleccionada en general con recursos económicos adecuados. Se debe insistir en la incorporación temprana de carne a la alimentación e inicio adecuado de la suplementación con hierro en dosis adecuada recomendando una buena adherencia al tratamiento.

Summary Introduction: iron deficiency anemia is a health problem in Uruguay. In 2014, hemoglobin measurement by digital puncture in infants between 8 and 12 months was introduced as a screening method for early diagnosis. Objective: to determine anemia prevalence in infants between 8 and 12 months in the institution (CASMU-IAMPP), and to identify associated factors. Method: infants between 8 and 12 months were studied with hemoglobin measurement by digital puncture from July to December of 2014. The characteristics of the children with anemia were described and compared with the control group. The data were obtained from the medical record and by means of a telephone survey. Results: 804 children were evaluated with hemoglobin measurement by digital puncture, 145 presented anemia (18.03%). The characteristics presented by children with anemia (n=143) were compared with the control group of children without anemia (n=143). There was no significant difference in the distribution by sex, prematurity prevalence, weight under 3.000 g, twin pregnancy, exclusive breastfeeding, and adequate initiation of replacement treatment. Children with anemia presented problems in the initiation of iron supplementation, doses of supplementary iron were adequate, although adherence to treatment was bad (p<0.05). Discussion: the fact that 20% of children from a socio-economic sector with adequate resources have anemia is rather alarming. Early consumption of meat and adequate initiation of iron supplementation in the right doses must be encouraged, as well as a good adherence to treatment.

A Gender-Specific Association between Self-Reported Snoring and Hemoglobin A1c Levels in a General Population without Type 2 Diabetes Mellitus

PURPOSE: We explored whether a gender difference was evident in terms of the associations of snoring with hemoglobin A1c (HbA1c) and homeostatic model assessment-insulin resistance (HOMA-IR) levels in a healthy population without type 2 diabetes mellitus (DM). MATERIALS AND METHODS: We analyzed 2706 males and 4080 females who participated in the baseline survey of the Namwon Study. In terms of self-reported snoring frequency, participants were classified as non-snorers or occasional (1–3 days/week), frequent (4–6 days/week), or constant (7 days/week) snorers. Participants with DM, defined as a fasting blood glucose level ≥126 mg/dL and/or use of insulin or hypoglycemic medication, were excluded from the analysis. RESULTS: In females, the fully adjusted mean (95% confidence interval) HbA1c levels in non-snorers and in occasional, frequent, and constant snorers were 5.53% (5.47–5.59%), 5.53% (5.47–5.59%), 5.57% (5.49–5.64%), and 5.57% (5.51–5.64%), respectively, reflecting a dose-response relationship (p trend=0.004). Compared with female non-snorers, the risk of an elevated HbA1c level (top quintile, ≥5.9%) in constant snorers remained significant (odds ratio 1.30, 95% confidence interval 1.02–1.66) after full adjustment. In addition, in females, a significant linear trend in HbA1c level odds ratio by increased snoring frequency was apparent (p trend=0.019 in model 3). In contrast, no significant association between snoring frequency and HbA1c level was identified in males. No significant association between snoring frequency and HOMA-IR was detected in either gender. CONCLUSION: We discovered a gender-specific association between snoring and HbA1c level in a healthy, community-dwelling population free of DM.

Vitamin D Status and Bone Mineral Density in Children with Inflammatory Bowel Disease Compared to Those with Functional Abdominal Pain

Low vitamin D has been implicated in reduced bone mineral density (BMD) in children with inflammatory bowel disease (IBD). Our study aimed to evaluate differences in serum 25-hydroxyvitamin D (25[OH]D) and total body less head (TBLH) BMD z-scores in children with Crohn's disease (CD), ulcerative colitis (UC), and those with abdominal pain-related functional gastrointestinal disorder (AP-FGID) as the control group. We also examined the correlation between serum 25(OH)D and TBLH BMD z-score, and factors that affect each of these parameters. A total of 105 children were included and divided into 3 groups: AP-FGID (n = 45), CD (n = 43), and UC (n = 17). Among the 3 study groups, TBLH BMD z-scores were found to be significantly different (0.5 ± 0.8 in CD vs. 0.1 ± 0.8 in UC vs. −0.1 ± 1.1 in FGID; P = 0.037), despite similar levels of serum 25(OH)D. Within each study group, correlation between serum 25(OH)D and TBLH BMD z-score was not observed. Factors found to affect the TBLH BMD z-score were sex (P = 0.018), age (P = 0.005) and serum hemoglobin (P = 0.041), while factors influencing serum 25(OH)D were sex (P = 0.018), CD with reference to AP-FGID (P = 0.020), and serum phosphorus (P = 0.018). Based on our results, vitamin D is a relatively small contributor to bone loss in pediatric IBD and clinicians should consider female sex, older age, and low hemoglobin as risk factors for low BMD in children with IBD.

The Influence of Self-care Behaviors, Empowerment and Social Support on Glycosylated Hemoglobin in Patients with Type 2 Diabetes

PURPOSE: The purpose of the study was to analyze the effects of self-care behavior, empowerment, and social support on glycosylated hemoglobin in patients with type 2 diabetes. METHODS: The data were collected during the period of July 1 to July 31, 2016. In total, 172 participants were recruited from outpatients who had been diagnosed with type 2 diabetes at a health care center, a health promotion center at National Health Insurance Corporation, and a tertiary hospital. Statistical data were analyzed with SPSS 20.0 using frequency analysis, t-test, ANOVA, Scheffé test, Pearson's correlation coefficients, and hierarchical regression analysis. RESULTS: The study results showed that self-care behavior (β=−.34, p<.001), empowerment (β=−.34, p<.001), and social support (β=−.20, p=.018) were found to be influential factors affecting glycosylated hemoglobin, with an overall descriptive power of 69%. CONCLUSION: Self-care behavior, empowerment, and social support are considered to be important factors in blood glucose management for the patients with type 2 diabetes. Therefore, self-caring blood glucose programs and internal synchronizing education through social support and empowerment need to be improved.

Glycosylated Hemoglobin Threshold for Predicting Diabetes and Prediabetes from the Fifth Korea National Health and Nutrition Examination Survey

We aimed to estimate the threshold level of glycosylated hemoglobin (HbA1c) for the fasting plasma glucose of 100 and 126 mg/dL in the Korean adult population, using the 2011 Korea National Health and Nutrition Examination Survey. A total of 4,481 participants over 19 years of age without diabetic medications and conditions to influence the interpretation of HbA1c levels, such as anemia, renal insufficiency, liver cirrhosis, and cancers, were analyzed. A point-wise area under the receiver operating characteristic curve was used to estimate the optimal HbA1c cutoff value. A HbA1c threshold of 6.35% was optimal for predicting diabetes with a sensitivity of 86.9% and a specificity of 99.1%. Furthermore, the threshold of HbA1c was 5.65% for prediabetes, with a sensitivity of 69.3% and a specificity of 71%. Further prospective studies are needed to evaluate the HbA1c cutoff point for diagnosing prediabetes and diabetes in the Korean population.

Association between Vitamin D Deficiency and Anemia in Patients with End-Stage Renal Disease: A Cross-Sectional Study

PURPOSE: Despite new treatment strategies, anemia remains the most prevalent complication in patients with end-stage renal disease (ESRD). We investigated whether 25-hydroxyvitamin D [25(OH)D3] deficiency was associated with anemia in ESRD patients. MATERIALS AND METHODS: We reviewed the medical records of 410 ESRD patients who had undergone renal transplantation (RTx) at Yonsei University Health System and who had 25(OH)D3 levels measured at the time of RTx. Patients were divided into two groups based on baseline 25(OH)D3 concentrations: group 1, 25(OH)D3 levels <10 ng/mL; and group 2, 25(OH)D3 levels ≥10 ng/mL. RESULTS: Using multivariate regression models, 25(OH)D3, age, and erythrocyte-stimulating agent (ESA) dose were found to be significantly associated with hemoglobin (Hb) levels [25(OH)D3: β=0.263, p<0.001; age: β=0.122, p=0.010; ESA dose: β=-0.069, p=0.005]. In addition, logistic regression analysis revealed that patients in group 1 had a significantly higher risk for developing anemia (Hb level <10 g/dL) compared to group 2 patients, even after adjusting for potential risk factors for anemia (odds ratio=3.857; confidence interval=1.091-13.632; p=0.036). CONCLUSION: 25(OH)D3 deficiency was significantly associated with anemia in patients with ESRD. Randomized controlled trials are needed to determine whether vitamin D supplementation can improve anemia in these patients.

Bases moleculares de alfa-talasemia en la Argentina

La α-talasemia, es uno de los desórdenes hereditarios más frecuentes mundialmente. Al presente, el diagnóstico molecular es la única herramienta que permite el diagnóstico certero. El propósito de este trabajo fue caracterizar las bases moleculares de estos síndromes en nuestro medio, y establecer relaciones genotipo-fenotipo. Mediante la complementación de distintas técnicas de biología molecular e hibridación fluorescente in situ (FISH), se logró poner en evidencia la presencia de mutaciones α-talasémicas en 145 de 184 (78.8%) pacientes estudiados con perfil hematológico compatible con α-talasemia. Dentro de este grupo, las deleciones correspondieron al defecto genético más frecuente, prevaleciendo la mutación -α3.7 en genotipos heterocigotas y homocigotas. Asimismo, en pacientes con fenotipo α0 las deleciones prevalentes fueron -MED y -CAL/CAMP. Este estudio permitió también describir una deleción de la región sub-telomérica en un paciente con α-talasemia y retraso mental. En el 7.6% de los pacientes caracterizados clínicamente como posibles α-talasémicos (microcitosis con valores de Hb A2 inferiores al 3.5%), se hallaron mutaciones β-talasémicas en estado heterocigota. Se lograron establecer perfiles hematológicos asociados a los genotipos α+ y α0 para pacientes adultos y niños. Esperamos que este trabajo pueda servir como guía para reconocer posibles portadores α-talasémicos. También permite destacar el trabajo en conjunto de médicos hematólogos, el laboratorio (bioquímico y de biología molecular) y de los médicos genetistas, con el fin de proporcionar adecuado consejo genético.

The α-thalassemia is one of the most common hereditary disorders worldwide. Currently, molecular diagnostics is the only available tool to achieve an accurate diagnosis. The purpose of this study was to characterize the molecular bases of these syndromes in our environment and to establish genotype-phenotype associations. Through a combination of different molecular techniques and fluorescent in situ hybridization (FISH),we were able to find α-thalassemic mutations in 145 of the 184 patients (78.8%) studied with hematological parameters compatible with α-thalassemia. Deletions of the a-globin genes resulted the major molecular cause of the disease, and the most frequent mutation was -α3.7, found in homozygous and heterozygous genotypes. In patients with α0 phenotypes, other prevalent mutations were -MED and -CAL/CAMP. The description of a sub-telomeric deletion in a patient with α-thalassemia and mental retardation was also achieved. β-thalassemic mutations in heterozygous state were found in 7.6% of the patients, who presented α-thalassemic clinical features (microcytosis and Hb A2 levels below 3.5%). Hematologic profiles for the α+ and α0 genotypes were established for adult and pediatric patients. Hopefully, this work will provide guidelines for the detection of possible α-thalassemic carriers. It also highlights the collaborative work of hematologists, the biochemical and molecular biology laboratory and genetists, in order to provide appropriate genetic counseling.

Effect and safety of testosterone undecanoate in the treatment of late-onset hypogonadism: a meta-analysis / 中华男科学杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of testosterone undecanoate (TU) in the treatment of late-onset hypogonadism (LOH) by meta-analysis.</p><p><b>METHODS</b>We searched Pubmed (until April 1, 2014), Embase (until March 28, 2014), Cochrane Library (until April 17, 2014), CBM (from January 1, 2001 to February 2, 2014), CNKI (from January 1, 2001 to February 2, 2014), Wanfang Database (from January 1, 2000 to February 2, 2014), and VIP Database (from January 1, 2000 to Febru ary 2, 2014) for randomized controlled trials of TU for the treatment of LOH. We evaluated the quality of the identified literature and performed meta-analysis on the included studies using the Rveman5. 2 software.</p><p><b>RESULTS</b>Totally, 14 studies were included after screening, which involved 1 686 cases. Compared with the placebo and blank control groups, TU treatment significantly increased the levels of serum total testosterone (SMD = 6.22, 95% CI 3.99 to 8.45, P < 0.05) and serum free testosterone (SMD = 4.35, 95% CI 1.86 to 6. 85, P < 0.05) but decreased the contents of luteinizing hormone (WMD = -2.23, 95% CI -4.03 to -0.42, P < 0.05), sex hormone binding globulin (WMD = 2.00, 95% CI 1.38 to 2.63, P < 0.05). TU also remarkably reduced the scores of Partial Androgen Deficiency of the Aging Males (WMD = -9.49, 95% CI -12.96 to -6.03, P < 0.05) and Aging Males Symptoms rating scale (WMD = -2.76, 95% CI -4.85 to -0.66, P <0.05) but increased the hemoglobin level (SMD = 2.35, 95% CI 0.29 to 4.41, P < 0.05) and packed-cell volume (SMD = 4.35, 95% CI 1.36 to 7.33, P < 0.05). However, no significant changes were shown in aspertate aminotransferase, alanine transaminase, prostate-specific antigen, or prostate volume after TU treatment (P > 0.05).</p><p><b>CONCLUSION</b>TU could significantly increase the serum testosterone level and improve the clinical symptoms of LOH patients without inducing serious adverse reactions. However, due to the limited number and relatively low quality of the included studies, the above conclusion could be cautiously applied to clinical practice.</p>

Low haemoglobin levels increase unilateral spatial neglect in acute phase of stroke / Níveis baixos de hemoglobina aumentam a negligência espacial unilateral na fase aguda do acidente vascular cerebral

Objective The objective of this study was to evaluate the relationship between unilateral spatial neglect (USN) and haemoglobin (Hb) level in acute phase of stroke. Method Cross-sectional study was performed after right hemisphere ischemic stroke. Independent variable: Hb level (mg/dL); Outcome: USN; Potential confounding factors: Age, National Institutes of Health Stroke Scale (NIHSS), and glycaemia (mg/dL); Characterization variables were obtained from electronic medical records, Hb, mean corpuscular volume (MCV) and glycaemia by laboratory exams, and USN by cancellation and bisection tasks. The relationship between Hb and USN was assessed by Spearman correlation and linear regression model. Results 40 individuals were evaluated; it was observed that the higher the Hb level, the better the USN test performance, with the two being negatively correlated. There was no significant correlation between VCM level and USN performance. Conclusion Low hemoglobin levels may indicate a worse performance in USN cancellation and bissection tests in acute phase of stroke. .

Objetivo O objetivo do estudo foi avaliar a relação entre a negligência espacial unilateral (NEU) e hemoglobina (Hb) na fase aguda do acidente vascular cerebral (AVC). Método Foi realizado estudo transversal em pacientes com AVC de hemisfério direito dentro das primeiras 48 horas do ictus. Variáveis independentes: nível de Hb (mg/dL); Desfecho: NEU; Fatores potenciais de confundimento: Idade, National Institutes of Health Stroke Scale (NIHSS) e glicemia (mg/dL); A caracterização das variáveis foram obtidas por meio de prontuários eletrônicos, Hb e glicemia por exames laboratoriais, e NEU por meio do Line Cancellation (LCT), Star Cancelation (SCT), e Line Bisection Tasks (LBT). Resultados 40 indivíduos foram avaliados e foi observado que quanto maior os níveis de Hb, melhor o desempenho nos testes de NEU, demonstrando correlação negativa entre eles. Conclusão Níveis baixos de hemoglobina podem indicar pior desempenho nos testes de NEU na fase aguda do AVC. .

Influence of age on the haemoglobin concentration of malaria-infected patients in a reference centre in the Brazilian Amazon

Anaemia is amongst the major complications of malaria, a major public health problem in the Amazon Region in Latin America. We examined the haemoglobin (Hb) concentrations of malaria-infected patients and compared it to that of malaria-negative febrile patients and afebrile controls. The haematological parameters of febrile patients who had a thick-blood-smear performed at an infectious diseases reference centre of the Brazilian Amazon between December 2009-January 2012 were retrieved together with clinical data. An afebrile community control group was composed from a survey performed in a malaria-endemic area. Hb concentrations and anaemia prevalence were analysed according to clinical-epidemiological status and demographic characteristics. In total, 7,831 observations were included. Patients with Plasmodium falciparum infection had lower mean Hb concentrations (10.5 g/dL) followed by P. vivax-infected individuals (12.4 g/dL), community controls (12.8 g/dL) and malaria-negative febrile patients (13.1 g/dL) (p < 0.001). Age, gender and clinical-epidemiological status were strong independent predictors for both outcomes. Amongst malaria-infected individuals, women in the reproductive age had considerably lower Hb concentrations. In this moderate transmission intensity setting, both vivax and falciparum malaria are associated with reduced Hb concentrations and risk of anaemia throughout a wide age range.

Evaluación del desempeño analítico de tres métodos de cuantificación de hemoglobina A1c / Analytical performance evaluation of three methods for hemoglobin A1c quantification / Avaliação do desempenho analítico do três métodos de quantificação de hemoglobina A1c

El laboratorio debe garantizar la exactitud de los resultados de HbA1c cumpliendo con los requisitos analíticos internacionales de calidad, cada vez más estrictos y asegurar que una variación de HbA1c de 0,5 puntos porcentuales (%-NGSP) o más entre dos controles consecutivos de un paciente diabético se deba a una variación clínica y no a una variación analítica. En este trabajo se evaluó el desempeño analítico de tres métodos comerciales para HbA1c: inmunoturbidimétrico, enzimático y cromatográfico de intercambio catiónico. Para tal fin, se procesaron por cada método distintos controles comerciales de HbA1c, con trazabilidad al método de referencia IFCC, determinándose en cada caso Coeficiente de Variación Total, Bias, Error Total, Valor de Referencia del Cambio y cambio clínico significativo de HbA1c en el punto crítico 7,0%-NGSP. En las condiciones analíticas de este trabajo, solamente el método inmunoturbidimétrico tuvo un desempeño analítico aceptable, permitiendo atribuir un cambio de 0,5%-NGSP a una variación clínica significativa del paciente. Frente a las recomendaciones internacionales sobre el uso de HbA1c en el control y diagnóstico de diabetes, es indiscutible la importancia de elegir un método que satisfaga los requerimientos analíticos mínimos de calidad para asegurar la utilidad clínica del resultado de HbA1c.

The laboratory must guarantee the accuracy of HbA1c results meeting the increasingly strict international analytical quality standards and assuring that an HbA1c variation of 0.5 percentage points (%-NGSP) or more between two consecutive controls of a diabetic patient is due to a clinical variation and not to an analytical variation. In this paper, the analytical performance of three commercial methods for HbA1c: Immunoturbidimetric, Chromatographic and Enzymatic Cation Exchange, were evaluated. For this purpose, commercial controls with assigned values traceable to the IFCC reference method for HbA1c were processed. For each methodology, total Coefficient of Variation (CV%), Bias%, Total Error (TE%), Change Reference Value and Clinically Significant Change (CSC) at the critical point of HbA1c 7.0%-NGSP were determined. Within the analytical conditions of this study, only the immunoturbidimetric method had an acceptable analytical performance, allowing attribute a change in 0.5%-NGSP to a significant clinical variation. Faced with international recommendations on the use of HbA1c on control and diagnosis of diabetes, the importance of choosing a method that meets the minimum analytical quality requirements to ensure the clinical utility of HbA1c result is undeniable.

O laboratório deve garantir a precisão dos resultados da HbA1c cumprindo com os requisitos analíticos internacionais de qualidade cada vez mais exigentes e garantir que uma variação de HbA1c de 0,5 pontos percentuais (% - NGSP) ou mais entre duas verificações consecutivas de um doente diabético seja devido a uma variação clínica e não a uma variação analítica. Neste trabalho foi avaliado o desempenho analítico de três métodos comerciais para HbA1c: imunoturbidimétrico, enzimático e cromatográfico de intercâmbio catiônico. Para esse fim, foram processados diversos controles comerciais de HbA1c por cada método, com rastreabilidade ao método de referência IFCC, determinando em cada caso Quociente de Variação Total, Bias, Erro Total, Valor de Referência da Alteração e Alteração Clinicamente Significativa de HbA1c no ponto crítico 7,0%-NGSP. Nas condições de análise deste estudo, apenas o método imunoturbidimétrico teve um desempenho analítico aceitável, permitindo atribuir uma alteração de 0,5%-NGSP a uma variação clínica significativa do paciente. Perante as recomendações internacionais sobre o uso da HbA1c no controle e diagnóstico da diabetes, é inegável a importância de escolher um método que atenda os requisitos analíticos mínimos de qualidade de análise para garantir a utilidade clínica do resultado HbA1c.

The effect of hookworm infection and urinary scistosomiasis on blood hemoglobiin concentration of schoolchildren living in northern Mozambique / Infecções por ancilostomídeos e Schistosoma haematobium e sua correlação com a concentração sanguínea de hemoglobina em crianças moçambicanas

This study aims to assess the association between schistosomiasis and hookworm infection with hemoglobin levels of schoolchildren in northern Mozambique. Through a cross-sectional survey, 1,015 children from five to 12 years old in the provinces of Nampula, Cabo Delgado and Niassa were studied. Hookworm infection and urinary schistosomiasis were diagnosed, through Ritchie and filtration methods, with a prevalence of 31.3% and 59.1%, respectively. Hemoglobin levels were obtained with a portable photometer (Hemocue®). The average hemoglobin concentration was 10.8 ± 1.42 g/dL, and 62.1% of the children presented levels below 11.5 g/dL, of which 11.8% of the total number of children had hemoglobin levels below 9 g/dL. A multiple linear regression analysis demonstrated negative interactions between hemoglobin levels and ancylostomiasis, this being restricted to the province of Cabo Delgado (β = -0.55; p < 0.001) where an independent interaction between hemoglobin levels and urinary schistosomiasis was also observed (β = -0.35; p = 0.016). The logistical regression model indicated that hookworm infection represents a predictor of mild (OR = 1.87; 95% CI = 1.17-3.00) and moderate/severe anemia (OR = 2.71; 95% CI = 1.50 - 4.89). We concluded that, in the province of Cabo Delgado, hookworm and Schistosoma haematobium infections negatively influence hemoglobin levels in schoolchildren. Periodical deworming should be considered in the region. Health education and improvements in sanitary infrastructure could achieve long-term and sustainable reductions in soil-transmitted helminthiases and schistosomiasis prevalence rates.

Este estudo tem como objetivo avaliar a relação entre a ancilostomíase e a esquistossomíase urinária com as concentrações sanguíneas de hemoglobina em crianças escolares no norte de Moçambique. Em estudo transversal, 1.015 crianças com idade entre cinco e 12 anos foram incluídas, nas Províncias de Nampula, Cabo Delgado e Niassa. A ancilostomíase e a esquistossomíase urinária foram diagnosticadas através das técnicas de Ritchie e de filtração da urina, respectivamente; prevalências de 31,3% e 59,1% foram observadas. As concentrações sanguíneas de hemoglobina foram obtidas com um fotômetro portátil (Hemocue). A concentração média de hemoglobina foi 10,8 ± 1.42 g/dL, 62,1% das crianças apresentaram concentração abaixo de 11,5 g/dL e 11,8% apresentaram nível abaixo de 9 g/dL. A regressão linear múltipla demonstrou interações negativas entre os níveis de hemoglobina e i) a infecção por ancilostomídeos (β = -0,55; p < 0,001) e ii) a esquistossomíase urinária (β = -0,35; p = 0,016), ambas associações restritas à Província de Cabo Delgado. Também em Cabo Delgado, o modelo de regressão logística demonstrou que a infecção por ancilostomídeos representa um preditor de anemia leve (OR = 1,87; 95% CI = 1,17-3,00) e anemia moderada/grave (OR = 2,71; 95% CI = 1,50 - 4,89). O estudo conclui que em Cabo Delgado, Moçambique, as infecções por ancilostomídeos e Schistosoma haematobium estão significativamente associadas a uma menor concentração sanguínea de hemoglobina em crianças em idade escolar. A administração periódica de anti-helmínticos deve ser feita regularmente. Melhorias na infraestrutura sanitária das regiões estudadas são as medidas mais eficazes para controle destas parasitoses.

Embolization of splenic artery aneurysm associated with gastrointestinal bleeding: case report / Embolização de aneurisma de artéria esplênica associado à hemorragia digestiva: relato de caso

The splenic artery is the visceral vessel that is most often affected by aneurysmal disease. Occasionally, gastrointestinal bleeding may signify that the aneurysm is in communication with the digestive tract. We report on the case of a 64-year-old multiparous patient with intermittent digestive bleeding caused by a splenic artery aneurysm who was successfully treated with endovascular embolization.

A artéria esplênica é o vaso visceral mais acometido pela doença aneurismática. Ocasionalmente, um sangramento gastrointestinal pode refletir uma comunicação entre o aneurisma de artéria esplênica e o trato digestivo. Relatamos o caso de uma paciente de 64 anos com hemorragia digestiva intermitente devida a aneurisma de artéria esplênica, a qual foi submetida ao tratamento endovascular por embolização com sucesso.

Relationships between Diabetic Knowledge, Self-Care Behaviors and HbA1c in Diabetic Patients using public hospitals / 기본간호학회지

PURPOSE: The purpose of this study was to investigate diabetic knowledge, self-care behaviors, and HbA1c of patients with diabetic mellitus in public hospitals. METHODS: Participants were 287 adult patients who had been diagnosed with diabetes in two public hospitals in Seoul. Collected data were analyzed using t-test, ANOVA, Pearson's correlation with the SPSS (PASW) 21.0 program. RESULTS: For general characteristics of the participants, there were significant differences in the level of diabetic knowledge according to age (F=11.28, p<.001), educational level (F=11.07, p<.001), type of health insurance (F=9.38, p<.001), and monthly income (t=-4.58, p<.001) and in the self-care behaviors according to age (F=8.61, p<.001) and in HbA1c according to age (F=6.72, p=.001). As for disease related characteristics of the participants, significant differences were found for self-care behaviors according to education about diabetes (t=3.90, p<.001) and in HbA1c according to education about diabetes (t=3.26, p=.001) and current diabetic therapy methods (F=13.51, p<.001). The study results showed that there was no correlation between diabetic knowledge and self-care behavior, or between self-care behavior and HbA1c. CONCLUSION: Results indicate that when developing preliminary data on nursing intervention education programs on diabetes, increasing diabetic knowledge and self-care behaviors would help to improve blood glucose levels.

Anemia e nível de hemoglobina como fatores prognósticos da mortalidade entre idosos residentes na comunidade: evidências da Coorte de Idosos de Bambuí, Minas Gerais, Brasil / Anemia and hemoglobin level as prognostic factors of mortality in community-dwelling elderly: evidence from the Bambuí Cohort Study on Aging, Minas Gerais State, Brazil / Anemia y nivel de hemoglobina como factores pronósticos de la mortalidad entre ancianos residentes en la comunidad: Cohorte de Ancianos de Bambuí, Minas Gerais, Brasil

O objetivo deste trabalho foi examinar a influência de baixos níveis de hemoglobina (Hb) e anemia na mortalidade entre idosos ao longo de dez anos de seguimento (1997 a 2007). Entre os 1.742 idosos participantes da linha de base da coorte de Bambuí, Minas Gerais, Brasil, 1.322 (82,3%) foram acompanhados pelo tempo médio de 8,9 anos. A probabilidade de sobrevida foi estimada pelo método de Kaplan-Meier e o modelo de riscos proporcionais de Cox foi utilizado, estimando-se os hazard ratios (HR) e intervalos de 95% de confiança (IC95%), considerando-se os potenciais fatores de confusão. Idosos anêmicos e com baixos níveis de Hb (primeiro tercil) apresentaram maior risco de óbito (HR = 2,63; IC95%: 1,83-3,76 e HR = 1,38; IC95%: 1,07-1,79, respectivamente) comparados aos não anêmicos e aos de maior nível de Hb. Os resultados demonstram que a ocorrência de anemia e/ou baixos níveis de Hb (independente do diagnóstico de anemia) devem ser interpretados e tratados como determinante independente da mortalidade nessa população idosa.

The aim of this study was to examine the influence of low hemoglobin (Hb) and anemia on mortality in the elderly in a ten-year follow-up (1997-2007). Among the 1,742 elderly participants in the baseline cohort from Bambuí, Minas Gerais State, Brazil, 1,322 (82.3%) were followed for an average of 8.9 years. Probability of survival was estimated by Kaplan-Meier and Cox's proportional hazard ratios (HR), and confidence intervals (95%CI) were used, considering potential confounders. Anemia and low Hb (lowest tertile) were associated with increased risk of death (HR = 2.63, 95%CI: 1.83-3.76 and HR = 1.38, 95%CI: 1.07-1.79, respectively). The results show that anemia or low Hb (regardless of a diagnosis of anemia) should be interpreted and managed as independent determinants of mortality in this elderly population.

El objetivo de este trabajo fue evaluar la influencia de los bajos niveles de hemoglobina (Hb) y anemia sobre la mortalidad de ancianos a lo largo de diez años (1997-2007). De los 1.742 ancianos participantes de la cohorte de Bambuí, Minas Gerais, Brasil, a 1.322 (82,3%) se les realizó un seguimiento promedio de 8,9 años. Se utilizó el método de Kaplan-Meier para estimar la probabilidad de supervivencia y el modelo de riesgos proporcionales de Cox para poder realizar una estimación del cociente de riesgo - hazard ratios (HR) - con intervalos de confianza de un 95%. Asimismo, fueron considerados factores potenciales de confusión. Ancianos anémicos y con niveles bajos de Hb (primer tercil) presentaron un riesgo superior de morir (HR = 2,63; IC95%: 1,83 a 3,76 y HR = 1,38; IC95%: 1,07 a 1,79, respectivamente), en comparación con los individuos no anémicos y con los que presentaron niveles superiores de Hb. Los resultados indican que la ocurrencia de anemia y/o bajos niveles de Hb (independiente del diagnóstico) se debe interpretar y manejar como factores determinantes independientes de la mortalidad entre una determinada población geriátrica..

Distribución de tipos de paraproteínas en una muestra de enfermos con mieloma múltiple / Distribution of types of paraproteins in a sample of patients with multiple myeloma

Introducción: el mieloma múltiple (MM) es una enfermedad caracterizada por una proliferación monoclonal de inmunoglobulinas que representa aproximadamente el 15 por ciento de las hemopatías malignas. Métodos: se realizó un estudio de la distribución de las clases, sub clases y tipos de cadenas ligeras de inmunoglobulinas en 285 enfermos con el diagnóstico de MM. Se emplearon tres métodos: electroforesis de proteínas en suero para la detección de la inmunoglobulina monoclonal o paraproteína, electroforesis de inmunofijación y doble inmunodifusión para identificar las clases, sub clases y tipo de cadenas ligeras. Resultados: se encontraron 206 enfermos (72.28 por ciento) con MM IgG; 73 (25.62 por ciento) con MM IgA y 6 (2.1 por ciento) con MM IgM. La distribución de sub clases de IgG fue: 130 casos (63.11 por ciento) IgG1, 43 (20.87 por ciento) IgG2, 21 (10.19 porciento) IgG3 y 12 (5.83 por ciento) IgG4; y la de sub clases de IgA fue de 59 enfermos (80.82 por ciento) IgA1 y 14 (19.18 por ciento) IgA2. Del total de enfermos 187 (65.61 por ciento) mostraron cadenas ligeras tipo kappa y 98 (34.38 por ciento) tipo lambda. Conclusiones: los datos obtenidos en nuestro estudio permitieron identificar la frecuencia de distribución de las clases, subclases y cadenas ligeras en una muestra de enfermos con MM

Introduction: multiple mieloma (MM) is a disease characterized by a monoclonal proliferation of immunoglobulins representing approximately 15 percent of malignant hemopathies. Methods: the distribution of classes, subclasses and light chains of monoclonal immunoglobulins was studied in 285 patients with MM. Three methods were used: serum protein electrophoresis for the detection of monoclonal immunoglobulins or paraproteins, immunofixation electrophoresis and double immunodiffusion to identify classes, subclasses and light chain types. Results: 206 patients (72.28 percent) with IgG MM, 73 (25,62 percent) with IgA MM, and 6 (2,1 percent) with IgM MM were found. The distribution of IgG subclasses was: 130 cases (63,11 percent) IgG1; 43 (20,87 percent) IgG2; 21 (10,19 percent) IgG3: and 12 (5,83 percent) IgG4. Distribution of IgA subclasses was: 59 patients (80,82 percent) IgA1 and 14 (19,18 percent) IgA2; 187 patients (65,62 percent) showed kappa light chains and 98 (34,38 percent) were lambda. Conclusions: the data obtained in our study allowed us to identify the frequency of distribution of classes, subclasses and light chains in a sample of patients with MM

Alpha chain hemoglobins with electrophoretic mobility similar to that of hemoglobin S in a newborn screening program

OBJECTIVE: To characterize alpha-chain variant hemoglobins with electric mobility similar to that of hemoglobin S in a newborn screening program. METHODS: βS allele and alpha-thalassemia deletions were investigated in 14 children who had undefined hemoglobin at birth and an electrophoretic profile similar to that of hemoglobin S when they were six months old. Gene sequencing and restriction enzymes (DdeI, BsaJI, NlaIV, Bsu36I and TaqI) were used to identify hemoglobins. Clinical and hematological data were obtained from children who attended scheduled medical visits. RESULTS: The following alpha chain variants were found: seven children with hemoglobin Hasharon [alpha2 47(CE5) Asp>His, HbA2:c.142G>C], all associated with alpha-thalassemia, five with hemoglobin Ottawa [alpha1 15(A13) Gly>Arg, HBA1:c.46G>C], one with hemoglobin St Luke's [alpha1 95(G2) Pro>Arg, HBA1:c.287C>G] and another one with hemoglobin Etobicoke [alpha212 84(F5) Ser>Arg, HBA212:c.255C>G]. Two associations with hemoglobin S were found: one with hemoglobin Ottawa and one with hemoglobin St Luke's. The mutation underlying hemoglobin Etobicoke was located in a hybrid α212 allele in one child. There was no evidence of clinically relevant hemoglobins detected in this study. CONCLUSION: Apparently these are the first cases of hemoglobin Ottawa, St Luke's, Etobicoke and the α212 gene described in Brazil. The hemoglobins detected in this study may lead to false diagnosis of sickle cell trait or sickle cell disease when only isoelectric focusing is used in neonatal screening. Additional tests are necessary for the correct identification of hemoglobin variants.