Nutritional response and clinical complications in patients with post-pyloric and gastric enteral tubes / Resposta nutricional e complicações clínicas em pacientes com uso de sonda gástrica ou pós-pilórica

ABSTRACT Objective The present study assessed the differences in nutritional markers (albumin, transferrin, total body fat, and body mass index) and clinical complications (diarrhea, vomiting, and diet interruptions) associated with enteral nutrition. Methods This is an open-label, randomized, two-arm parallel-group controlled clinical trial. Out of 105 patients assessed for eligibility, 35 adult patients were randomly divided into two groups and followed for 28 days. The sample comprised a heterogeneous group of severely ill individuals initially treated in the intensive care units. Neurological conditions (i.e., strokes and brain tumours) were the most common reasons for hospitalization. Patients had one singularity: the clinical need for exclusive enteral nutrition therapy. One group received the diet via gastric tube and the other via a post-pyloric tube. Results The groups presented increases in the calories prescribed and administered, as well as reduced diet discontinuation. Although similar values were observed up to day 21, the post-pyloric group showed increased albumin levels compared to the gastric group on Day 28. Transferrin levels increased over time in both groups. Conclusion There were no differences in the complications recorded between groups, albeit serum albumin significantly increased in the post-pyloric group.

RESUMO Objetivo Avaliar diferenças tanto nos marcadores nutricionais (albumina, transferrina, gordura corporal e índice de massa corporal) quanto nas complicações clínicas (diarreia, vômitos e interrupções na dieta) associadas à nutrição enteral administrada através de duas vias tradicionais. Métodos Este é um ensaio clínico de tratamento, paralelo de dois braços, aberto e randomizado controlado. Dos 105 participantes avaliados para elegibilidade, 35 pacientes adultos foram divididos aleatoriamente em dois grupos e seguidos por 28 dias. A amostra foi formada por um grupo heterogêneo e gravemente enfermo, tratados inicialmente em unidades de terapia intensiva. Condições neurológicas, como acidente vascular e tumores cerebrais foram as razões principais para hospitalização. Os pacientes tinham em comum um aspecto, a saber, a necessidade clínica exclusiva de receber nutrição enteral. Um grupo recebeu a dieta via sonda gástrica e o outro através de sonda pós-pilórica. Resultados Os grupos apresentaram aumento de calorias prescritas e administradas, bem como redução da descontinuação da dieta. Embora valores semelhantes tenham sido observados até o 21° dia, o grupo pós-pilórico apresentou aumento dos níveis de albumina em relação ao grupo gástrico no dia 28. Os níveis de transferrina aumentaram ao longo do tempo em ambos os grupos. Conclusão Não houve diferenças nas complicações registradas entre os grupos, embora níveis séricos de albumina aumentaram significativamente no grupo pós-pilórico.

Transferrin isoelectric focusing for the investigation of congenital disorders of glycosylation: analysis of a ten-year experience in a Brazilian center / Isoeletrofocalização da transferrina para investigação das doenças congênitasda glicosilação: análise de dez anos de experiência de um centro brasileiro

Abstract Objectives: To characterize cases of suspected congenital disorders of glycosylation (CDG) investigated in a laboratory in southern Brazil using the transferrin isoelectric focusing TfIEF test from 2008 to 2017. Method: Observational, cross-sectional, retrospective study. The laboratory records of 1,546 individuals (median age = 36 months, 25-75 IQR = 10-108; males = 810) submitted to the TfIEF test during the period were reviewed. Results: Fifty-one individuals (3%) had an altered TfIEF pattern (5 ± 2.8 cases/year; median age = 24 months, 25-75 IQR = 11-57 months; males = 27, 53%). For 14 of them, data on diagnosis conclusion were available (classic galactosemia = 4; hereditary fructose intolerance = 4; peroxisomal diseases = 2; PMM2-CDG = 2; MPDU1-CDG = 1; SLC35A2-CDG = 1).Comparing the cases with the normal and altered TfIEF patterns, there was a higher prevalence of altered cases in the age group from 11 months to 3 years. There was an increase in the likelihood of change in TfIEF, especially in the presence of inverted nipples or liver disease. Conclusions: The data suggest that the investigation of a case with suspected CDG is a complex problem, being aggravated by the existence of other IEMs (inborn errors of metabolism) associated with altered TfIEF pattern and lack of access to confirmatory tests. The presence of inverted nipples and liver disease, especially in individuals aged 11 months to 3 years, should suggest the need for TfIEF investigation.

Resumo Objetivos: Caracterizar os casos com suspeita de CDG investigados em laboratório do sul do Brasil pelo exame de IEFTF de 2008 a 2017. Metodologia: Estudo observacional, transversal, retrospectivo. Foram revisadas as fichas laboratoriais de 1.546 indivíduos (mediana de idade = 36 meses, IQ 25-75 = 10-108; sexo masculino = 810) que fizeram o exame de IEFTF no período. Resultados: Cinquenta e um indivíduos (3%) apresentaram padrão alterado na IEFTF (5 ± 2,8 casos/ano; mediana de idade = 24 meses, IQ 25-75 = 11-57 meses; sexo masculino = 27, 53%). Para 14 deles, estavam disponíveis dados sobre a conclusão do diagnóstico (galactosemia clássica = 4; intolerância hereditária à frutose = 4; doenças peroxissomais = 2; PMM2-CDG = 2; MPDU1-CDG = 1; SLC35A2-CDG = 1). Comparando os casos com padrão normal e alterado na IEFTF, houve maior prevalência de casos alterados na faixa etária de 11 meses a 3 anos. Verificou-se um aumento na probabilidade de alteração na IEFTF principalmente na presença de mamilos invertidos ou de hepatopatia. Conclusões: Os nossos dados sugerem que a investigação de um caso com suspeita de CDG é complexa, é agravada pela existência de outros EIM associados a padrão alterado na IEFTF e pela falta de acesso a exames confirmatórios. A presença principalmente de mamilos invertidos e de hepatopatia em indivíduos na faixa etária de 11 meses a 3 anos deve sugerir a necessidade de investigação por IEFTF.

Transferrina como marcador de maturação pulmonar em cordeiros nascidos a termo ou prematuros / Transferrin as a marker of pulmonary maturation in lambs born at term or premature

O objetivo do estudo foi procurar proteínas de fase aguda que possam indicar sinais de maturação no neonato prematuro, por meio da quantificação sérica delas. Identificou-se a imunoglobulina A, a ceruloplasmina, a haptoglobina, a glicoproteína ácida, a transferrina, a albumina e as imunoglobulinas G de cadeias leve e pesada, pela comparação do perfil dos proteinogramas de cordeiros nascidos a termo com os prematuros submetidos a diferentes protocolos terapêuticos, a fim de estimular a atividade respiratória. Constituíram-se seis grupos: PN (n= 9): nascidos de parto normal; CN (n= 7): nascidos de cesariana em tempo normal de gestação; CP (n= 6): nascidos de cesariana prematura sem nenhum tipo de tratamento; DEX (n= 9): prematuros cujas mães receberam dexametasona pré-parto; SURF (n= 6): prematuros tratados com surfactante; e DEXSURF (n= 6): prematuros tratados com surfactante cujas mães receberam dexametasona pré-parto. As avaliações foram realizadas nos momentos imediatamente após o nascimento (M0), após 24 (M24) e após 48 horas (M48). As amostras foram processadas por meio de eletroforese em gel de poliacrilamida contendo dodecil sulfato de sódio (SDS-PAGE). A albumina, as imunoglobulinas e a proteína total dos cordeiros tiveram elevação após a ingestão de colostro. Maiores valores séricos de transferrina são referentes a maior período gestacional, podendo essa proteína ser utilizada como marcador de maturação neonatal.(AU)

The aim of this study was to search for acute phase proteins that could indicate signs of maturation in the premature neonate by quantifying them in serum. Immunoglobulin A, ceruloplasmin, haptoglobin, acid glycoprotein, tranferrin, albumin, light and heavy chain immunoglobulin G were quantified, comparing the profile of proteinograms from term to preterm lambs submitted to different protocols that stimulate respiratory activity. Six groups were used: PN (n= 9): born from normal birth; CN (n= 7): born from caesarean section at normal time of gestation; CP (n= 6): born from premature cesarean without any type of treatment; DEX (n= 9) preterm whose mothers received prepartum dexamethasone; SURF (n= 6) preterm treated with surfactant; DEXSURF (n= 6): preterm treated with surfactant whose mothers received prepartum dexamethasone. The evaluations were performed immediately after birth (M 0), after 24 and 48 hours (M 24 and M 48). Samples were processed with sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE). Albumin, immunoglobulins, and serum total protein of the lambs were elevated, after colostrum ingestion. Higher serum transferrin values refer to a longer gestational period, and this protein may be used as a marker of neonatal maturation.(AU)

Parâmetros hematológicos e bioquímicos de bezerros neonatos da raça Holandesa tratados com ferro suplementar / Hematological and biochemical parameters of neonatal Holstein calves supplemented with iron

A deficiência de ferro em bezerros neonatos está associada ao desenvolvimento de anemia, que favorece o aparecimento de outras enfermidades como pneumonia e diarreia. Avaliou-se o efeito da suplementação de ferro sobre o eritrograma, teores séricos de ferro, ceruloplasmina e transferrina, bem como o potencial para toxicidade do protocolo utilizado por meio da avaliação dos teores de ureia, creatinina e enzimas hepáticas. Para tal avaliação foram utilizados 40 bezerros neonatos da raça Holandesa, alocados em cinco grupos experimentais com oito animais em cada grupo, que foram submetidos aos seguintes protocolos: administração intramuscular de 5mL de solução fisiológica estéril no 5º dia de idade (grupo controle G1), e administração intramuscular de 5mL de ferro dextrano 10% nos seguintes momentos: no 5º dia de idade (G2); no 5o e no 20º dias de idade (G3); no 5o e no 30º dias de idade (G4) e no 5o, 20o e 45º dias de idade (G5). Foram coletadas amostras de sangue até 8 horas após o nascimento e aos 5, 10, 20, 30, 60 e 90 dias de idade para realização do eritrograma, avaliação dos teores séricos de ferro, ceruloplasmina, transferrina, ureia, creatinina, bilirrubina total e direta, e das atividades das enzimas aspartato aminotransferase (AST), fosfatase alcalina (ALP) e gamaglutamiltransferase (GGT). Os animais que receberam ferro suplementar apresentaram menor oscilação nos parâmetros eritrocitários, embora os animais do grupo controle não tenham desenvolvido anemia. Notou-se também aumento, embora não significativo, nos teores séricos de ferro e das proteínas de fase aguda ceruloplasmina e transferrina, cuja atividade está relacionada ao metabolismo desse mineral. Os teores séricos de ureia, creatinina, bilirrubina total e direta e as atividades das enzimas GGT, AST e ALP não foram influenciados pelos protocolos de administração de ferro suplementar. Os protocolos de tratamento empregados não ocasionaram hepatoxidade ou nefrotoxidade aos animais. Concluiu-se que a suplementação com ferro dextrano por via parenteral em bezerros que recebem outras dietas que não apenas leite não traz benefícios que justifiquem sua indicação, embora sejam necessários mais estudos que avaliem a influência da suplementação com ferro sobre o tempo necessário para a recuperação, custos com o tratamento e impacto sobre a vida produtiva dos animais na idade adulta.(AU)

Iron deficiency in newborn calves is associated with the development of anemia, which favors the development of other infirmities such as pneumonia and diarrhea. The present study evaluated the effect of iron supplementation on erythrogram, serum levels of iron, ceruloplasmin and tranferrin, as well as potential toxicity of the protocol used by means of evaluation of urea, creatinine and hepatic enzyme activities. 40 newborn Holstein calves were allocated into 5 experimental groups comprising 8 calves each, which were subjected to the following treatment protocols: intramuscular administration of 5mL of sterile saline on the 5th day of age (control group G1), intramuscular administration of 5mL of 10% dextran iron in the following moments: on the 5th day of age (G2); on the 5th and in the 20th day of age (G3); on the 5th and 30th day of age (G4); on the 5th, 20th and 45th days of age (G5). Blood samples were taken until 8 hours after birth and with 5, 10, 20, 30, 60, and 90 days of age, and subjected to hemogram, evaluation of serum levels of iron, ceruloplasmin, transferrin, urea, creatinine, total and direct bilirrubin, and serum activities of aspartate aminotransferase (AST), alcaline phosphatase (ALP), and gamma-glutamyltransferase (GGT). Calves that received iron supplementation at any time presented less variation in the erythrocyte parameters, although calves in the control group did not develop anemia. Serum concentration of iron and acute phase protein ceruloplasmin and transferrin, which activities are related to iron metabolism, also increased, although not significantly. Serum levels of urea, creatinine, bilirubins and activities of AST, ALP, and GGT were not influenced by the administration protocols used in this experiment. The results of the experiment led to the conclusion that the supplementation with parenteral dextran iron in calves that receive diets other than exclusive milk does not bring sufficient advantages to be indicated, although more studies are necessary to evaluate the influence of iron supplementation on the outcome of infections in newborn calves, especially its influence on cost of treatment, time necessary for discharge and impact on its productive life.(AU)

Ingesta de hierro y gen HFE en varones adultos de Buenos Aires / Iron intake and HFE gen in male adults from Buenos Aires

El consumo excesivo de hierro (Fe) en portadores de mutaciones en el gen HFE puede resultar en sobrecarga. Para evaluar el riesgo de sobrecarga de Fe fueron investigados 166 varones adultos donantes de sangre de la ciudad de Buenos Aires. Se estimó la ingesta diaria de Fe (IFe), de Fe hemínico y de Fe proveniente de harinas enriquecidas con SO4Fe. Se determinó ferritina sérica y porcentaje de saturación de transferrina (criterio de sobrecarga de Fe: ferritina sérica > 300 ng/ml y saturación de transferrina ≥ 50%). Las mutaciones C282Y, H63D y S65C fueron investigadas en sangre mediante PCR-RFLP. Todos los participantes cubrieron ampliamente el requerimiento estimado promedio de Fe (6 mg Fe/día) y 3.0% superó el máximo tolerable (45 mg Fe/día). El Fe hemínico correspondió al 9.4% de la IFe y el de harinas enriquecidas al 47.7%. Se observó una asociación entre el aumento de IFe y el de ferritina sérica (p = 0.0472), y el 2.3% de los donantes presentaron ferritina sérica > 300 ng/ml y saturación de transferrina ≥ 50%. El 29.3% de los donantes eran portadores de los genotipos H63D, S65C o C282Y, asociados a hemocromatosis hereditaria, y tenían valores de saturación de transferrina significativamente mayores a los de los donantes wild type (p = 0.0167). Si bien la incidencia clínica de hemocromatosis hereditaria fue baja en el grupo estudiado (1.2%), el consumo excesivo de Fe plantea un riesgo potencial para la salud de individuos que ignoran sus antecedentes familiares de sobrecarga de Fe.

Excess iron (Fe) intake in subjects carrying certain mutations in the HFE gene may result in Fe overload. To estimate risk of Fe overload, 166 male blood donors (19-65 years) from Buenos Aires city were investigated. Daily Fe intake (FeI), hem Fe intake, and Fe intake from SO4Fe enriched flours were estimated (SARA Computer Program and Food Composition Table, USDA). Serum ferritin and transferrin saturation were determined; criteria for Fe overload was serum ferritin > 300 ng/ml and transferrin saturation ≥ 50%. HFE genotypes C282Y, H63D and S65C were analyzed by PCR-RFLP in blood samples. No participant presented FeI lower than the estimated average requirement (6 mg Fe/day) and 3.0% was over the upper level (45 mg Fe/day). Hem Fe and Fe from flour enrichment were 9.4% and 47.7% of daily Fe intake, respectively. A significant association was observed between the increase in serum ferritin (ng/ml) and the increase in FeI (p = 0.0472); 2.3% of the donors presented serum ferritin > 300 ng/ml and transferrin saturation ≥ 50%. Genotypes associated with hereditary hemochromatosis (H63D, S65C and C282Y) were found in 29.3% of the donors. The percentage of transferrin saturation was higher in subjects carrying mutation than in wild type subjects (p = 0.0167). Although penetrance of hereditary hemochromatosis in the studied group was only 1.2%, an excessive Fe intake could enhance adverse effects in individuals unaware of any family history of Fe overload.

key regulator for iron homeostasis in chronic hepatitis C

Hepcidin is a small, cysteine-rich cationic peptide produced by hepatocytes. There is a single human hepcidin gene; whose essential role in iron homeostasis was confirmed by identifying homozygous frameshift or nonsense mutations in affected individuals with severe Juvenile hemochromatosis. IL-6 may be the mediator of hepcidin induction by inflammation. Hypoferremia is a common response to systemic infections or generalized inflammatory disorders, anemia of chronic disease occurs in patients with acute and chronic immune activation and represents an important clinical problem. The study will attempt to determine the hepatic hepcidin expression levels in patients with chronic hepatitis C virus infection. Fifty patients with chronic hepatitis C virus infection [CHCV], their age between [20- 55] years, selected from the National Hepatology and Tropical Medicine Research Institute were included in this study, before interferon and Ribavirin therapy, and ten healthy individuals were included to serve as controls. All the patients and controls were subjected to the following history, clinical examination, abdominal ultrasonography and collection of blood samples for routine laboratory investigations. CBCs and serological assay for serum ferritin, iron, transferrin [s-TFR] levels, Liver biopsy for hepcidin mRNA levels and iron deposits in liver by [PCR] polymerase chain reaction. All subjects gave written informed consent for enrolment in the study, which was approved by the Research Ethical Committee of the General Organization for Teaching Hospitals and Institutes. Liver biopsy was taken from healthy subjects during abdominal surgery. Our results revealed that hepatic hepcidin expression is considered highly valid marker in case of CHCV infection. Our study concluded that there's a highly significant inverse correlation between hepcidin versus liver iron, serum iron and serum transferrin but there's no significant correlation versus ferritin. Hepcidin measuring and manipulating hepcidin levels will, in the future, have a role in diagnosing and treating any number of iron related disorders. Hepcidin itself has antimicrobial properties of uncertain importance so that careful clinical trials will be required to define appropriate indications of hepcidin antagonists

Evolución del estado nutricional en pacientes en hemodiálisis durante 4 años de seguimiento

La malnutrición proteíco calorica asi como la inflamación sistémica y metabólica son trastornos frecuentes entre los pacientes con insuficiencia renal crónica sometidos a tratamiento renal sustitutivo (Hemodiálisis), lo que contribuye a su morbilidad y mortalidad. En este trabajo se ha seguido a 90 pacientes de ambos sexos con insuficiencia renal crónica que fueron tratados con hemodiálisis periódicamente en nuestra unidad durante cuatro años. A todos los pacientes se le realizaron mediciones trimestrales de albúmina plasmática (Alb), colesterol total (CT), proteínas totales (PT) y mensuales de transferrina (Tr), y se les efectuaron mediciones antropométricas de peso, altura e índice de masa corporal calculado mediante la formula peso/talla², y agrupada según la clasificación de la OMS en IMC < 18,50 infrapeso, 18,50 a 24,99 normal, 1,25 a 29,99 sobrepeso y >30 del IMC s/OMS. El objetivo de este trabajo fue evaluar el estado nutricional de estos pacientes mediante la valoración de parámetros bioquímicos y parámetros antropométricos y determinar si estos pacientes sufren alteraciones que sugieran deterioro nutricional directamente relacionado con el tiempo en diálisis. Durante los 4 años todos los pacientes manifestaron un importante descenso de los parámetros bioquímicos, en cambio el IMC no presentó cambios significativos en relación a la desnutrición. Por lo tanto la desnutrición de los pacientes en diálisis es un hecho patente, el IMC no se corresponde con lo parámetros bioquímicos observados, por lo que el deterioro nutricional de estos pacientes se manifiesta principalmente mediante los parámetros bioquímicos estudiados.

Protein-calorie malnutrition as well as systemic inflammation and metabolic disorders are common among patients with chronic renal failure undergoing renal replacement therapy (hemodialysis), which contributes to their morbidity and mortality. This work has followed 90 patients of both sexes with chronic kidney disease who were treated with hemodialysis periodically in our unit for four years. All patients were performed quarterly measurements of plasma albumina (Alb), total cholesterol (TC), total protein (TP) and monthly transferrin (Tr), Anthropometric measurements of height and weight were taken on all patients by using a balance/stadiometer (Perperson 113481); weight was measured in kilograms and height in centimetres. BMI was calculated with this formula: weight/height2 and classified according to the WHO criteria: BMI.

Soapstone utensils may improve iron status in adult women. A preliminary study

Soapstone is among the first heat-resistant materials to have been used by man for the manufacture of culinary utensils, but its impact on human nutrition has merited little attention. Here, we present a preliminary evaluation of the impact of soapstone pans on the iron status in humans. Five nuns (37.6 ± 6.31y) replaced the soapstone for their traditional aluminum cookware, without any alteration of the diet itself. Comparison of the hematological data, determined before and 10 weeks after switching the pans, revealed that hemoglobin rose from 13.26 to 14.0 mg•dL-1 (p=0.0048), and hematocrit increased from 38.14 to 40.71% (p=0.0002), while the transferrin saturation index went from 28.04 to 28.96% (p=0.0147) and ferritin, from 31.5 to 34.74ìg•L-1 (p=0.0681). By simply replacing the soapstone for the traditional aluminum cookware, substantially improved the iron status in all subjects in a relatively short period of time.

La piedra-jabon (jabon-de-sastre, piedra de talco), uno de los primeros materiales termo-resistentes usados en la manufactura de utensilios culinarios, ha recibido poca atencion sobre el impacto de su uso en el estado nutricional de la poblacion. El presente estudio piloto evalua el uso de utensilios de piedra-jabon en el estado nutricional en hierro de mujeres jovenes. Cinco monjas (37,6 } 6,31 anos) reemplazaron sus tradicionales utensilios de aluminio por ollas de piedra-jabon, sin cualquier otra alteracion en la dieta. La comparacion de los parametros sanguineos, determinados antes y despues de 10 semanas de intervencion, mostro que la hemoglobina aumento, de 13,26 a 14,00 mg.dL-1 (p=0,0048) y el hematocrito, de 38,14 a 40,71 (p=0,0002), mientras el indice de saturacion de transferrina subio, de 28,04 a 28,96%, y la ferritina, de 31,50 a 34,74 ƒÊgEL-1 (p=0.0681). Se concluye que mediante la simple eliminacion de los utensilios de aluminio y su substitucion por los de piedrajabon, posibilito una substancial mejoria del estado nutricional en hierro de todos los sujetos, en un termino relativamente corto.

Deficiência de ferro em lactentes brasileiros com doença falciforme / Iron deficiency in Brazilian infants with sickle cell disease

OBJETIVO: Avaliar a deficiência ou sobrecarga de ferro em lactentes com doença falciforme, a fim de embasar a decisão de recomendar (ou não) a suplementação profilática de ferro nessa população. MÉTODOS: Estudo retrospectivo transversal envolvendo 135 lactentes menores de 2 anos (66 meninos e 69 meninas), com genótipos SS e SC (77/58), nascidos entre 2005 e 2006 em Minas Gerais. Os indicadores de uma possível deficiência de ferro foram: volume corpuscular médio (VCM), hemoglobina corpuscular média (HCM), saturação da transferrina (ST) e ferritina. Dezessete lactentes [12,6 por cento, intervalo de confiança de 95 por cento (IC95 por cento) 7,0-18,2 por cento] haviam recebido hemotransfusão antes da coleta dos exames. RESULTADOS: ST e ferritina estavam significativamente mais baixas nos lactentes com hemoglobina SC (p < 0,001). Quando dois indicadores foram utilizados para definir a deficiência de ferro (VCM ou HCM baixos mais ST ou ferritina baixas), 17,8 por cento das crianças (IC95 por cento 11,3-24,3 por cento) tinham deficiência de ferro, predominando naquelas com perfil SC (p = 0,003). Análise das crianças que não haviam sido transfundidas (n = 118) mostrou prevalência de ferropenia em 19,5 por cento. Constatou-se aumento de ferritina em 15 lactentes (11,3 por cento; IC95 por cento 5,9-16,7 por cento); a maioria havia sido transfundida. CONCLUSÕES: A maior parte dos lactentes com doença falciforme não desenvolve deficiência de ferro, mas alguns têm déficit significativo. Este estudo indica que lactentes com doença falciforme, principalmente aqueles com hemoglobinopatia SC, talvez possam receber ferro profilático; no entanto, a suplementação deve ser suspensa após a primeira hemotransfusão.

OBJECTIVE: To assess iron deficiency or overload in infants with sickle cell disease in order to support the decision to recommend (or not) iron prophylactic supplementation in this population. METHODS: Cross-sectional and retrospective study with 135 infants below 2 years old (66 boys and 69 girls), 77 with SS and 58 with SC hemoglobin, born between 2005 and 2006 in Minas Gerais, Brazil. Indicators of possible iron deficiency were: mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), transferrin saturation (TS), and ferritin. Blood transfusions had been given to 17 infants (12.6 percent, 95 percent confidence interval [95 percentCI] 7.0-18.2 percent) before laboratory tests were done. RESULTS: Ferritin and TS were significantly lower in SC infants (p < 0.001). When two indices were considered for the definition of iron deficiency (low MCV or MCH plus low ferritin or TS), 17.8 percent of children (95 percentCI 11.3-24.3 percent) presented iron deficiency, mainly those with SC hemoglobin (p = 0.003). An analysis of infants who were not given transfusions (n = 118) showed that 19.5 percent presented iron deficiency. Fifteen infants (11.3 percent, 95 percentCI 5.9-16.7 percent) presented increased ferritin; the majority had been transfused. CONCLUSIONS: Most infants with sickle cell disease do not develop iron deficiency, though some have a significant deficit. This study indicates that infants with sickle cell disease, mainly those with SC hemoglobin, may receive prophylactic iron; however, supplementation should be withdrawn after the first blood transfusion.

Optimización del tratamiento con hierro endovenoso en hemodiálisis: Estudio clínico prospectivo a largo plazo / Optimizing iron therapy in hemodialysis: a prospective long term clinical study

A pesar del amplio uso del hierro endovenoso en hemodiálisis, resta aún identificar un índice adecuado para optimizar esta terapéutica en el largo plazo. Con ese objetivo, se diseñó un estudio prospectivo de cohorte, de larga duración, que consistió en un período basal (PB) y dos períodos experimentales: PI y PII. Se infundió hierro dextran de bajo peso molecular a 100, 150 y 200 mg/mes, respectivamente, durante 6 meses y al final de cada periodo se determinaron: saturación de transferrina (TSAT), ferritina (FERR), porcentaje de eritrocitos hipocrómicos (HYPO) y contenido de hemoglobina en reticulocitos (HCr). Durante el estudio la albúmina aumentó significativamente, pero la Hgb, la dosis de EPO y la proteína C-reactiva se mantuvieron sin cambios. Los cambios en HYPO y FERR fueron inespecíficos. Sólo TSAT (desde 21.4 ± 6 en PB a 34 ± 7.1% en PII, p = 0.01) y HCr (desde 27.5 ± 1.3 en PB a 29.3 ± 1.7 pg en PII, P = 0.045) respondieron específicamente, pero el porcentaje de aumento de TSAT fue de 65% (IC95% 22), y el de HCr sólo 6% (IC95% 2.3; p = 0.0002). Esta diferencia a favor de TSAT se observó en todos los pacientes. Los resultados sugieren la utilización de 200 mg/FeIV/mes y que, de los índices estudiados, TSAT sería el más adecuado para optimizar el uso a largo plazo del hierro endovenoso en hemodiálisis.

The usefulness of intravenous iron therapy in hemodialysis is evidence-based. However, controversy still arises about the most suitable iron marker to optimize this treatment in the long term. We aimed to determine the most suitable marker with a prospective, cohort study, designed to comprise a basal period (BP) and two consecutive experimental periods (PI, PII). Low molecular weight iron dextran was infused at 100, 150 and 200 mg/month respectively, on a biweekly basis, during 6 months. At the end of each period, the following were determined: transferrin saturation (TSAT), ferritin (FERR), percentage of hypochromic eritrocytes (HYPO) and haemoglobin content in reticulocytes (HCr). During the study, albumin increased significantly, whereas no significant changes in hemoglobin, EPO doses and C-reactive protein were observed. Changes in HYPO and FERR were unspecific. Only TSAT (from 21.4 ± 6 in PB to 34 ± 7.1% in PII, p < 0.01) and HCr (from 27.5 ± 1.3 in PB to 29.3 ± 1.7 pg in PII, P < 0.05 ) responded specifically to changes in Fe doses, but change of TSAT was 65% (CI 95% 22), whereas change of HCr was just 6% (CI 95% 2.3; p = 0.0002). The difference was observed in all patients. Results suggest that 200 mg/FeIV/month is effective and that, of the markers tested in this study, TSAT would be the most suitable one to the practicing nephrologist to optimize intravenous iron in the long term.

Indicadores bioquímicos del hierro materno en el tercer trimestre de la gestación y su relación con la antropometría materna y el peso al nacer* / Biochemical indicators of maternal iron in the third trimester of pregnancy and their relation with maternal anthropometry and neonatal weight

Objetivos: evaluar el hierro y la transferrina séricos, la capacidad de fijación de hierro de la transferrina y el porcentaje de saturación de la misma, en una submuestra de gestantes del Bajo Cauca antioqueño, y asociar estos indicadores con la antropometría materna y el peso al nacer. Métodos: estudio descriptivo, transversal en 16 mujeres en el tercer trimestre de la gestación; la transferrina sérica se midió por nefelometría, el hierro sérico y la capacidad de fijación de la transferrina por fotocolorimetría; el porcentaje de saturación se calculó con la fórmula estándar. Para la antropometría materna se tuvieron en cuenta el Índice de Masa Corporal (IMC) y la ganancia de peso; se pesó a los recién nacidos en la sala de partos, con un equipo de alta precisión. Resultados: el hierro sérico y el porcentaje de saturación de la transferrina indicaron eritropoyesis deficiente en hierro y anemia, pese a que la hemoglobina estaba por encima de 11,3 g/dL. La concentración de hemoglobina fue menor en las madres con IMC bajo, y el peso al nacer se correlacionó positivamente con este indicador. Conclusión: el hierro sérico y el porcentaje de saturación de la transferrina pueden ser biomarcadores del estado del hierro de más pronta respuesta que la hemoglobina a la deficiencia de este mineral; para mejorar el peso de los neonatos se debe monitorizar el hierro materno y satisfacer las necesidades de este mineral.

Objectives: To evaluate seric iron, transferrin iron binding capacity and its saturation index in a group of pregnant women in Antioquia, northwestern Colombia, and to associate these indicators with maternal anthropometry and neonatal weight. Methodology: This was a descriptive, cross sectional study of 16 women in the third trimester of pregnancy. Seric transferrin was determined by nephelometry, seric iron and transferrin iron binding capacity were measured by photocolorimetry; the saturation index of transferring was calculated by a standard chemical formula. Maternal anthropometry was based on the body mass index (BMI) and total body weight gain; neonatal weight was measured in the delivery room, with a high precision equipment. Results: Seric iron concentration and transferring saturation index indicated iron-deficient erythropoiesis and anemia, even though the average haemoglobin concentration was above 11.3 g/dL. Hemoglobin concentration was lower in mothers with low BMI, and neonatal weight correlated positively with maternal hemoglobin. Conclusion: Seric iron and transferrin saturation index may be biomarkers of the iron status with earlier response than hemoglobin to the deficiency of this mineral. Maternal iron should be monitorized and iron needs satisfied in order to improve the weight of newborns.

Anemia ferropriva e estado nutricional de crianças de creches de Guaxupé / Anemia and nutritional status of children in day-care centers in Guaxupé

OBJETIVO: Avaliar o estado nutricional e a prevalência de anemia ferropriva de crianças que freqüentam creches no município de Guaxupé, MG. MÉTODOS: Foram incluídas no estudo crianças de ambos os sexos, de 6 a 72 meses de idade, matriculadas em creches do município de Guaxupé. O estado nutricional foi aferido por meio do escore Z das relações estatura para idade e peso para a estatura. Para o diagnóstico de anemia utilizou-se a dosagem de hemoglobina, ferro sérico, capacidade total de ligação de ferro e o índice de saturação de transferrina. RESULTADOS: Escore Z < -2 foi observado em 3,3 por cento das crianças para a relação estatura para a idade e em 0,4 por cento para o peso para a estatura. A prevalência total de anemia foi de 16,1 por cento. Na faixa etária de 6 a 36 meses foi de 44,6 por cento. No total da amostra, observaram-se 45 por cento com ferro sérico diminuído, 37,9 por cento com capacidade total de ligação de ferro aumentada e 43,1 por cento com baixos índices de saturação de transferrina. CONCLUSÃO: A desnutrição não se constitui em problema, visto sua baixa prevalência; entretanto, se observa prevalência importante de deficiência de ferro. A anemia é mais prevalente em crianças de 6 a 36 meses, confirmando ser essa faixa etária mais vulnerável. Não há associação entre anemia e o escore Z das relações antropométricas estudadas.

OBJECTIVE: To evaluate the nutritional status and the prevalence of iron deficiency anemia in children attending public day care centers in the city of Guaxupé (MG), Brazil. METHODS: Children of both genders, from 6 to 72 months, registered in public day-care centers in the city of Guaxupé, were studied. Nutritional evaluation was conducted using the Z-score for height-to-age and weight-to-height relationships. Hemoglobin dosage, serum iron, total iron binding capacity and the transferrin saturation index were used for diagnosis of anemia. RESULTS: Z score < - 2 was observed in 3.3 percent of the children for the height-to-age relationship and of 0.4 percent for the weight-to-height relationship. Prevalence of total iron deficiency anemia was of 16.1 percent. In the age span from 6 to 36 months it was of 44.6 percent. The entire sample showed 45 percent with low serum iron, 37.9 percent with high total iron binding capacity and 43.1 percent with a low transferrin saturation index. CONCLUSION: Malnutrition was not considered to be a problem due to its low prevalence; however, an important prevalence of iron deficiency was observed. Iron deficiency anemia is more prevalent in children between 6 and 36 months confirming that this age span is more vulnerable. No association between iron deficiency anemia and the Z score of the studied anthropometry relationships was found.

Two pedigrees with restless legs syndrome in Brazil

Numerous studies have suggested a substantial genetic contribution in the etiology of the primary form of restless legs syndrome (RLS) and periodic leg movements (PLM). We describe the symptoms, the sleep profiles and physiological parameters of two families in which several members present RLS/PLM. The proband of family 1 is a 70-year-old woman and the proband of family 2 is a 57-year-old woman; both have exhibited the symptoms since the age of 20 years. All patients in both families were diagnosed with RLS according to the criteria of the International RLS Study Group. Polysomnographic recordings were performed to quantify and to describe PLM during sleep. Sleep parameters showed decreased sleep efficiency, increased sleep latency in the arousal index and the presence of PLM in all subjects. One of the families showed an exact profile of dominant inheritance with anticipation of age at onset. In the other family, the founders were blood relatives and there was no affected member in the third generation suggesting a recessive mode of inheritance. RLS/PLM is a prevalent sleep disorder affecting about 5 to 15 percent of the population and one that substantially impairs healthy sleep patterns. Efforts to understand the underlying pathophysiology will contribute to improve the sleep and life quality of these patients.

Avaliação do estado nutricional de ferro e anemia em crianças menores de 5 anos de creches públicas / Nutritional assessment of iron status and anemia in children under 5 years old at public daycare centers

OBJETIVO: Avaliar o estado nutricional de ferro e a prevalência de anemia em crianças menores de 5 anos de creches públicas da cidade do Recife (PE). MÉTODOS: Estudo transversal, com amostra aleatória sistemática de 162 crianças, de 6 a 59 meses. O estado nutricional de ferro foi avaliado em termos de reservas corporais (ferritina sérica), transferrinemia (ferro sérico, capacidade total de ligação do ferro e por cento de saturação da transferrina), eritropoiese (protoporfirina eritrocitária livre) e hemoglobinogênese (hemoglobina). RESULTADOS: A prevalência de anemia (hemoglobina < 11,0 g/dL) foi de 55,6 por cento (IC95 por cento 47,3-63,5), a redução dos estoques de ferro (ferritina sérica < 12,0 ng/mL) foi evidenciada em 30,8 por cento (IC95 por cento 22,9-39,3), baixa transferrinemia ( por cento de saturação da transferrina < 16) em 60,1 por cento (IC95 por cento 51,7-68,0) e eritropoiese deficiente (protoporfirina eritrocitária livre > 40 æmol/mol heme) em 69,6 por cento (IC95 por cento 61,0-77,1) das crianças. Os parâmetros de ferro não apresentaram correlação com o gênero (p > 0,05). No entanto, crianças < 24 meses apresentaram concentrações mais baixas de hemoglobina (p < 0,00) e níveis mais elevados de protoporfirina eritrocitária livre (p < 0,000) e de capacidade total de ligação do ferro (p < 0,001), quando comparadas às crianças > 24 meses. A significante correlação observada entre reserva, transferrinemia e eritropoiese representa achado compatível com o esperado ciclo de vida do ferro no organismo. CONCLUSÕES: A deficiência de ferro e a anemia parecem ser um importante problema de saúde pública entre as crianças menores de 5 anos de creches públicas do Recife. Logo, ações efetivas direcionadas à prevenção e ao controle dessa deficiência são fortemente recomendadas nesse contexto ecológico.

OBJECTIVE: To assess nutritional iron status and anemia prevalence in children less than 5 years old at public daycare centers in the city of Recife, PE, Brazil. METHODS: A cross-sectional study, with a systematic random sampling of 162 children aged 6 to 59 months. Nutritional iron status was assessed in terms of body iron reserves (serum ferritin), transferrinemia (serum iron, total iron binding capacity, and transferrin saturation percent), erythropoiesis (free erythrocyte protoporphyrin) and hemoglobin production (hemoglobin). RESULTS: The prevalence of anemia (hemoglobin < 11.0 g/dL) was 55.6 percent (95 percentCI 47.3-63.5), evidence was found of depleted iron stocks (serum ferritin < 12.0 ng/mL) in 30.8 percent (95 percentCI 22.9-39.3), low transferrinemia levels (transferrin saturation percent < 16) in 60.1 percent (95 percentCI 51.7-68.0) and deficient erythropoiesis (free erythrocyte protoporphyrin > 40 æmol/mol heme) in 69.6 percent (95 percentCI 61.0-77.1) of the children. Iron parameters were not correlated with sex (p > 0.05). However, children < 24 months exhibited lower hemoglobin concentrations (p < 0.00) and higher levels of free erythrocyte protoporphyrin (p < 0.000) and total iron binding capacity (p < 0.001) when compared with children > 24 months. The significant correlation observed between reserves, transferrinemia and erythropoiesis is a finding that is compatible with the expected lifecycle of iron in the body. CONCLUSIONS: Iron deficiency and anemia appear to be an important public health problem among children less than 5 years old at public daycare centers in Recife. Therefore, effective actions aimed at the prevention and control of this deficiency are strongly recommended in this ecological context.

Clinical and Biochemical Parameters of Nutrition to Predict Hepatic Encephalopathy in Cirrhotic Patients / 대한소화기학회지

BACKGROUND/AIMS: Protein-calorie malnutrition is a common complication in cirrhosis. Protein restriction for the treatment of hepatic encephalopathy (HE) may cause disease progression and poor prognosis. Therefore, we evaluated important clinical parameters for nutritional state in cirrhotic patients with or without HE to predict the development of HE. METHODS: Twenty-two cirrhotic patients were divided into two groups; group A-13 patients without HE and group B-9 patients with HE. Clinical and biochemical parameters, serum proteins {serum albumin, insulin-like growth factor-1 (IGF-1), transferrin, leptin, etc}, immunologic parameters and anthropometry were measured. RESULTS: Child-Pugh score and Model for End-stage Liver Disease (MELD) scale were higher in group B (p<0.01). After correction of various factors affecting nutritional assessment, especially of Child-Pugh score and MELD scale, leptin was higher in group B (p<0.05). There was no difference in anthropometric measurements. Transferrin correlated inversely with MELD scale in group A (p<0.01). IGF-1 correlated inversely with total lymphocyte count in group B (p<0.05). Leptin correlated with Child-Pugh scores, total lymphocyte count and mid-arm muscle cirumference in group A (p<0.05, p<0.05 and p<0.05, respectively), and correlated inversely with CD8 in group B (p<0.05). CONCLUSIONS: Leptin level is higher in patients with HE, and further studies for parameters of nutrition to predict HE in many cirrhotic patients will be needed.

Estrategias diagnósticas utilizadas para detectar deficiencias de hierro subclínicas y asociadas a enfermedades crónicas / Diagnostic strategies for detecting subclinical iron deficiencies and associated to chronic diseases

La homeostasis y las variaciones fisiológicas horarias en el metabolismo del hierro se constituyen en verdaderos desafíos para los expertos, quienes intentan diseñar pruebas altamente sensibles y específicas que cuantifiquen los niveles circulantes y de depósito de este elemento, su repercusión en la eritropoyesis,cuantificación de las proteínas, transportadores y receptores involucrados en el proceso, a fin de descartar la presencia de estados carenciales. Las deficiencias de hierro pasan por tres fases; las dos primeras son lasmás difíciles de identificar porque son subclínicas, más aún, cuando pueden cursar simultáneamente con enfermedades crónicas inflamatorias, infecciosas y neoplásicas que de por sí son anemizantes. En este trabajose revisarán las principales pruebas de laboratorio utilizadas para la identificación de deficiencias de hierro, sensibilidad, especificidad, ventajas y limitaciones para su uso.

Correlation between anthropometrics measurements, prealbumin level and transferin serum with Child-Pugh classification in evaluating nutritional status of liver cirrhosis patient.

AIM: to evaluate nutritional status by using anthropometrics and biochemistry examination and to determine the correlation between albumin, prealbumin and transferin serum level with anthropometrics measures in liver cirrhosis patient according to Child-Pugh classification. METHODS: study was conducted on 30 patients with liver cirrhosis Child-Pugh A, B and C. There were 8 patient of Child-Pugh A, 18 Child-Pugh B and 4 Child-Pugh C. Every patient underwent anthropometrics and biochemistry examination including ideal body weight (IBW), body mass index (BMI), triceps skin-fold thickness (TSF), mid-arm circumference (MAC), mid-arm muscle circumference (MAMC), albumin, prealbumin and transferin serum. We used statistical analysis Spearman and Pearson correlation test. RESULTS: by IBW and BMI measurement, most of liver cirrhosis patients indicated normal results. By MAMC measurement, there was no liver cirrhosis patient with good nutritional status. Most of them have slight and moderate malnutrition. There was no significant correlation between anthropometrics measurement with Child-Pugh score, with [p > 0.01]. And also, there was no significant relationship between albumin, prealbumin and serum transferin with anthropometrics measures, but there was significant correlation between prealbumin and transferin level with Child-Pugh score, with [p < 0.01]. CONCLUSION: the conclusion of this study is prealbumin and transferin level may be used to evaluate nutritional status of liver cirrhosis patient, and on the contrary with anthropometrics measures. There was no correlation between anthropometrics measures with albumin, prealbumin and transferin level.