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Background: Traditionally, hypermetabolism is described in sickle cell disease (SCD). Despite this, few studies have compared rest energy expenditure (REE) with estimated by predictive equations (EEE) in the assessment of adults with SCD. Aims: To compare REE values determined by indirect calorimetry (IC) with that EEE in adults with SCD. Subjects and Methods: A cross-sectional observational study was performed with 46 individuals over 34 years old in the treatment from two reference centers for SCD located in the city of Rio de Janeiro, Brazil. The dual-energy X-ray absorptiometry (DXA) and IC were used to assess BC and REE, respectively. Blood levels were measured to assess hemolytic and protein markers. Pearson’s correlation test was used in the univariate correlation. The Intraclass Correlation Coefficient (ICC) and the Bland-Altman analysis were used in the comparison between EEE and IC. P-values ≤ 0.05 were considered statistically significant. Results: Most participants were from the female sex, Hb SS genotype (80.4%) and with black color (52.2%). The mean age was 50 years old. Weight (r= 0.469; p=0,001), LM (r = 0.631; p=0.000), BMC (r = 0.508; p=0.000) and CRP(r=0.319; p = 0.002) correlated positively with the REE. There was no linear correlation between makers of hemolysis with REE. The REE was overestimated in the EEE when compared to IC (p

Background: Historically, malnutrition is described in individuals with SCD. However, more recent studies have shown a change in the profile of the nutritional status and distribution of body composition of SCD patients, mainly adult individuals. Aims: To assess the body composition (BC), resting energy expenditure (REE), and the biomarkers of hemolysis in adults with sickle cell disease (SCD). Subjects and Methods: A cross-sectional observational study was performed with 64 individuals over 39 years old in the treatment from two reference centers for SCD located in the city of Rio de Janeiro, Brazil. The dual-energy X-ray absorptiometry (DXA) and indirect calorimetry were used to assess BC and REE, respectively. Blood levels of hemoglobin, reticulocytes, lactate dehydrogenase (LDH), leukocytes, platelets, total and direct bilirubin, total protein, and albumin were measured to assess the hemolysis and protein status. The descriptive and inferential analysis was composed of the different methods (one-way ANOVA with the multiple comparison test of Tukey, Student t-test, and Pearson's correlation coefficient). Were considered statistically significant when the p-values were ≤ 0.05. Results: Most participants with SCD were female sex, colored (brown/black), and mean age of 51.2 years old. The obesity prevalence was 70.7% according to the body fat (BF%), with a major mean among women (p < 0.0001). Men had a higher mean of lean mass (LM) (p=0.0005) and fat-free mass (FFM) (p=0.0007). There was no difference for REE in comparing the genotypes (p= 0.53), and genders (p=0.075). The hemolysis markers (LDH, reticulocytes, and TB) correlated inversely with BMI (p=0.013), FM (p=0.022), and FFM (p=0.034). Conclusions: The important change observed in body composition in people with sickle cell disease was characterized by a high percentage of fat body and a decrease in lean mass. The hemolysis markers LDH, reticulocytes, and BT correlated inversely with BMI, FM, and FFM indicating that high levels of hemolysis may affect nutritional status, without influencing the REE. Keywords: sickle cell disease, body composition, fat mass, fat-free mass, energy expenditure, hemolysis.

Sarcopenia is a generalized loss of muscle mass affecting muscle function and strength with age. The cause of sarcopenia is multifactorial and physiopathological mechanisms, including the decline of neuron numbers, changes in muscle metabolism, oxidative damage, reduced response to nutrients, and inflammation throughout proinflammatory cytokines that increase myofibrillar protein degradation and decrease protein synthesis. This chapter presents two case reports about sarcopenia and its associated multimorbidity impact on elderly patients. Sarcopenia in the elderly, when it advances to generalized muscle wasting, is mainly associated with multimorbidity which becomes a diagnostic, therapeutic, and palliative challenge to the physician; in these cases, a multidisciplinary approach is best for appropriate diagnosis and management results in a high-quality patient care setting.

Leg ulcers (LU) are one of the most frequent complications in individuals with sickle cell disease (SCD). Old reports and unofficial documents found at the Nutrition Outpatient Clinic in a reference center for hematological diseases (NOC/RCHD) describe the use of oral supplement (OS) hypercaloric, hyper protein and arginine-rich (OS/HHA) for the healing of leg ulcers. The aim is to describe a rare case of a patient with sickle cell disease and leg ulcer submitted a dietetics orientations and intervention with OS/HHA. A 31-year-old male patient had been performing clinical treatment of sickle cell disease genotype SS for eight years at the RCHD of Rio de Janeiro. He had a unilateral ulcer on his right ankle for about six years, performing dressings by a Nursing team. He started follow-up at the NOC, being classified as grade III malnutrition. 13 nutrition consultations were performed during the study and 10 nutritional intervention consultations were carried out offering an OS/HHA containing 6 g/day of arginine, twice daily for 80 days. For ethical reasons, the participant was informed about the case report, agreeing and signing the consent form and the authorized image use term. Based on the analysis of food intake during the intervention period, energy consumption was on average 2.706 Kcal, including OS/HHA. Leg ulcers were found with regular margins and macerated in some areas, an ulcerated lesion with adherent and purulent exudate with 80% adherent thickness and in large quantities on the 1st day of supplementation. After eighty days, the lesion had regular margins and epithelial tissue, an ulcerative bed with 100% granulation, tissue and scarce exudate, showing improvement in healing. The patient presented an improvement in the nutritional status and leg ulcer healing. It is necessary to perform experimental research to corroborate or not the results observed in this rare case report.

OBJETIVO: Avaliar o estado nutricional antropométrico de crianças e adolescentes com doença falciforme, atendidas em um hospital de pediatria no município do Rio de Janeiro. MÉTODOS: Trata-se de estudo descritivo transversal, com dados extraídos dos prontuários. Foram coletadas variáveis antropométricas, sociodemográficas, clínicas e hematológicas referentes à última consulta da criança no ano de 2006. RESULTADO: A amostra foi composta por 161 crianças e adolescentes. Dentre elas, 15,5% apresentaram baixa estatura (estatura/idade

OBJETIVO: Relatar estudo de caso de atendimento nutricional a criança com diagnóstico de anemia falciforme. DESCRIÇÃO DO CASO: Realizaram-se quatro atendimentos nutricionais pela equipe do Internato de Nutrição da Universidade do Estado do Rio de Janeiro (Uerj) no período de julho a setembro de 2005 a criança de 1 a 4 meses, feminina, negra, com anemia falciforme. Na avaliação do estado nutricional e do ganho de peso, empregaram-se os seguintes indicadores antropométricos: comprimento/idade, peso/idade e peso/comprimento, e um indicador de impacto nutricional. A análise dietética compreendeu a avaliação da ingestão energética e de macronutrientes observada na primeira consulta após 30 dias de intervenção. COMENTÁRIOS: Ao longo do período analisado, a baixa estatura para a idade (z=−1,32) evoluiu para adequação (z=0,87), enquanto o peso em relação ao comprimento manteve-se inadequado (z=−2,53). O ganho de peso foi 50% inferior ao incremento esperado. O consumo energético inicialmente inadequado (60% das recomendações) alcançou, após 30 dias, 117%. A Estratégia em Saúde da Família vem sendo recomendada como importante ferramenta para monitorar as condições nutricionais, bem como para melhorar a atenção prestada. Entretanto, considerando o modelo de atenção primária local, observa-se haver necessidade de capacitação, especialmente no que tange às peculiaridades inerentes à condição de nutrição e de saúde dos portadores de anemia falciforme.OBJECTIVE: To report the nutritional follow-up of a black baby girl, one year and four months old, with homozygous sickle cell anemia. CASE DESCRIPTION: From July-September 2005, the infant attended four nutritional appointments at the Nutrition Internship Program from the State University of Rio de Janeiro, Rio de Janeiro, Brazil. The nutritional status was evaluated by the anthropometric indexes: length/age, weight/age and weight/length, and by one indicator of nutritional impact. Analysis of the energy dietary intakes and macronutrients ingestion was performed in two opportunities: at the first nutritional evaluation and 30 days after the first intervention. COMMENTS: At the first nutritional evaluation, the child presented low stature/age (z=−1,32), that became adequate during the follow-up (z=0,87). The low weight/length (z=−2,53) was similar at the first and final evaluations. The weight gain during the follow-up period was only 50% of the expected increment. The energy dietary intake was 66% of the recommended energy intake at the first appointment and 117% after 30 days. The Family Health Strategy is recommended as an important tool to monitor nutritional status and improve nutritional care. There is a need of developing professional skills regarding nutritional care of patients with sickle cell anemia.

Objetivo: Relatar estudo de caso de atendimento nutricional a crianca com diagnostico de anemia falciforme. Descricao do caso: Realizaram-se quatro atendimentos nutricionais pela equipe do Internato de Nutricao da Universidade do Estado do Rio de Janeiro (Uerj) no periodo de julho a setembro de 2005 a crianca de 1 a 4 meses, feminina, negra, com anemia falciforme. Na avaliacao do estado nutricional e do ganho de peso, empregaram-se os seguintes indicadores antropometricos: comprimento/idade, peso/idade e peso/comprimento, e um indicador de impacto nutricional. A analise dietetica compreendeu a avaliacao da ingestao energetica e de macronutrientes observada na primeira consulta apos 30 dias de intervencao. Comentarios: Ao longo do periodo analisado, a baixa estatura para a idade (z=−1,32) evoluiu para adequacao (z=0,87), enquanto o peso em relacao ao comprimento manteve-se inadequado (z=−2,53). O ganho de peso foi 50% inferior ao incremento esperado. O consumo energetico inicialmente inadequado (60% das recomendacoes) alcancou, apos 30 dias, 117%. A Estrategia em Saude da Familia vem sendo recomendada como importante ferramenta para monitorar as condicoes nutricionais, bem como para melhorar a atencao prestada. Entretanto, considerando o modelo de atencao primaria local, observa-se haver necessidade de capacitacao, especialmente no que tange as peculiaridades inerentes a condicao de nutricao e de saude dos portadores de anemia falciforme. Palavras-chave: anemia falciforme; estado nutricional; crescimento; ganho de peso; insuficiencia de crescimento.

OBJETIVO: Relatar estudo de caso de atendimento nutricional a criança com diagnóstico de anemia falciforme. DESCRIÇÃO DO CASO: Realizaram-se quatro atendimentos nutricionais pela equipe do Internato de Nutrição da Universidade do Estado do Rio de Janeiro (Uerj) no período de julho a setembro de 2005 a criança de 1 a 4 meses, feminina, negra, com anemia falciforme. Na avaliação do estado nutricional e do ganho de peso, empregaram-se os seguintes indicadores antropométricos: comprimento/idade, peso/idade e peso/comprimento, e um indicador de impacto nutricional. A análise dietética compreendeu a avaliação da ingestão energética e de macronutrientes observada na primeira consulta após 30 dias de intervenção. COMENTÁRIOS: Ao longo do período analisado, a baixa estatura para a idade (z=−1,32) evoluiu para adequação (z=0,87), enquanto o peso em relação ao comprimento manteve-se inadequado (z=−2,53). O ganho de peso foi 50% inferior ao incremento esperado. O consumo energético inicialmente inadequado (60% das recomendações) alcançou, após 30 dias, 117%. A Estratégia em Saúde da Família vem sendo recomendada como importante ferramenta para monitorar as condições nutricionais, bem como para melhorar a atenção prestada. Entretanto, considerando o modelo de atenção primária local, observa-se haver necessidade de capacitação, especialmente no que tange às peculiaridades inerentes à condição de nutrição e de saúde dos portadores de anemia falciforme. OBJECTIVE: To report the nutritional follow-up of a black baby girl, one year and four months old, with homozygous sickle cell anemia. CASE DESCRIPTION: From July-September 2005, the infant attended four nutritional appointments at the Nutrition Internship Program from the State University of Rio de Janeiro, Rio de Janeiro, Brazil. The nutritional status was evaluated by the anthropometric indexes: length/age, weight/age and weight/length, and by one indicator of nutritional impact. Analysis of the energy dietary intakes and macronutrients ingestion was performed in two opportunities: at the first nutritional evaluation and 30 days after the first intervention. COMMENTS: At the first nutritional evaluation, the child presented low stature/age (z=−1,32), that became adequate during the follow-up (z=0,87). The low weight/length (z=−2,53) was similar at the first and final evaluations. The weight gain during the follow-up period was only 50% of the expected increment. The energy dietary intake was 66% of the recommended energy intake at the first appointment and 117% after 30 days. The Family Health Strategy is recommended as an important tool to monitor nutritional status and improve nutritional care. There is a need of developing professional skills regarding nutritional care of patients with sickle cell anemia.