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1.
Arch Pediatr ; 21(5): 521-8, 2014 May.
Artigo em Francês | MEDLINE | ID: mdl-24686038

RESUMO

Very early in life, sodium intake correlates with blood pressure level. This warrants limiting the consumption of sodium by children. However, evidence regarding exact sodium requirements in that age range is lacking. This article focuses on the desirable sodium intake according to age as suggested by various groups of experts, on the levels of sodium intake recorded in consumption surveys, and on the public health strategies implemented to reduce salt consumption in the pediatric population. Practical recommendations are given by the Committee on nutrition of the French Society of Pediatrics in order to limit salt intake in children.


Assuntos
Hipertensão/etiologia , Hipertensão/prevenção & controle , Necessidades Nutricionais , Cloreto de Sódio na Dieta/administração & dosagem , Cloreto de Sódio na Dieta/efeitos adversos , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Feminino , França , Humanos , Lactente , Recém-Nascido , Masculino , Inquéritos Nutricionais , Valores de Referência , Estatística como Assunto
2.
Arch Pediatr ; 21(4): 424-38, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24636590

RESUMO

Lipids are an important source of energy for young children and play a major role in the development and functioning of nervous tissue. Essential fatty acids and their long-chain derivatives also fulfill multiple metabolic functions and play a role in the regulation of numerous genes. The Food and Agriculture Organization of the United Nations (FAO), the World Health Organization (WHO), and the French Agency for Food, Environmental and Occupational Health & Safety (Agence nationale de sécurité sanitaire de l'alimentation, de l'environnement et du travail [ANSES]) have recently recommended a minimum daily intake in preformed long-chain polyunsaturated fatty acids (LC-PUFAs): arachidonic acid (ARA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA). Mother's milk remains the only reference, but the large variability in its DHA content does not guarantee that breastfed children receive an optimal DHA intake if the mother's intake is insufficient. For children fed with infant formulas, ARA and DHA intake is often below the recommended intake because only one-third of infant formulas available on the market in France are enriched in LC-PUFAs. For all children, linoleic acid (LA) intake is on average higher than the minimal recommended values. The consequences of these differences between intake and recommended values are uncertain. A cautious attitude is to come close to the current recommendations and to advise sufficient consumption of DHA in breastfeeding women. For bottle-fed children, infant formulas enriched in LC-PUFAs and with moderate levels of LA should be preferred. LC-PUFA-rich fish should be consumed during breastfeeding, and adapted vegetable oils when complementary foods are introduced.


Assuntos
Ingestão de Energia , Lipídeos , Pediatria , Recomendações Nutricionais , Ácido Araquidônico/administração & dosagem , Fenômenos Fisiológicos da Nutrição Infantil , Pré-Escolar , Ácidos Docosa-Hexaenoicos/administração & dosagem , Ácido Eicosapentaenoico/administração & dosagem , Ácidos Graxos Insaturados/administração & dosagem , França , Humanos , Lipídeos/administração & dosagem , Estado Nutricional , Sociedades Médicas , Organização Mundial da Saúde
3.
Prenat Diagn ; 34(5): 424-30, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24395195

RESUMO

OBJECTIVE: The 22q11.2 deletion (del22q11.2) is one of the most common microdeletions. We performed a collaborative, retrospective analysis in France of prenatal diagnoses and outcomes of fetuses carrying the del22q11.2. METHODS: A total of 272 fetuses were included. Data on prenatal diagnosis, ultrasound findings, pathological features, outcomes and inheritance were analyzed. RESULTS: The mean time of prenatal diagnosis was 25.6 ± 6 weeks of gestation. Most of the diagnoses (86.8%) were prompted by abnormal ultrasound findings [heart defects (HDs), in 83.8% of cases]. On fetal autopsy, HDs were again the most common disease feature, but thymus, kidney abnormalities and facial dysmorphism were also described. The deletion was inherited in 27% of cases. Termination of pregnancy (TOP) occurred in 68.9% of cases and did not appear to depend on the inheritance status. However, early diagnosis was associated with a higher TOP rate. CONCLUSION: This is the largest cohort of prenatal del22q11.2 diagnoses. As in postnatally diagnosed cases, HDs were the most frequently observed abnormalities. However, thymus and kidney abnormalities and polyhydramnios should also be screened for in the prenatal diagnosis of del22q11.2. Only the time of diagnosis appeared to be strongly associated with the pregnancy outcome: the earlier the diagnosis, the higher the TOP rate.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Síndrome de DiGeorge/diagnóstico , Resultado da Gravidez , Ultrassonografia Pré-Natal , Adolescente , Adulto , Autopsia , Síndrome de DiGeorge/epidemiologia , Feminino , Feto , França , Inquéritos Epidemiológicos , Humanos , Pessoa de Meia-Idade , Gravidez , Estudos Retrospectivos , Adulto Jovem
4.
Arch Pediatr ; 20(5): 523-32, 2013 May.
Artigo em Francês | MEDLINE | ID: mdl-23562320

RESUMO

Processed baby foods designed for infants (4-12 months) and toddlers (12-36 months) (excluding infant formula, follow-on formula, the so-called growing-up milks, and cereal-based foods for infants), which are referred to as baby foods, are specific products defined by a European regulation (Directive 2006/125/CE). According to this Directive, such foods have a composition adapted to the nutritional needs of children of this age and should comply with specifications related to food safety in terms of ingredients, production processes, and prevention of infectious and toxicological hazards. Hence, they differ from ordinary foods and from non-specific processed foods. This market segment includes the full range of foods that can be part of children's diet: dairy products (dairy desserts, yoghurts, and fresh cheese), sweet products (nondairy desserts, fruit, and drinks), and salty products (soups, vegetable-based foods, meat, fish, and full dishes). This market amounted to 89,666 MT in France in 2011 and 83,055 MT in 2010 (a total of 325,524 MT in the 27 countries of the European Union in 2010, including 90,438 MT in Germany, 49,144 MT in Spain, and 40,438 MT in Italy). The consumption of baby foods in France varies with infant age and parental choice. Baby foods account for 7 % of total energy intake at 4-5 months, 28 % at 6-7 months, 27 % at 8-11 months, 17 % at 1-17 months, and 11 % at 18-24 months. Among parents, 24 % never offer their children any baby foods, 13 % do so 1-3 days/week and 63 % 4-7 days/week. Among consumers, 55 % of children eat more than 250 g/day of baby foods. As baby foods only account for a minor fraction of overall food intake, their impact on the quality of young children's diet is much less than that of growing-up milks, particularly for preventing insufficient iron and vitamin D intake. Their consumption, however, has an indirect benefit on the nutritional quality of the diet and on food safety, particularly regarding toxicological hazards, as it postpones the introduction of non-specific processed foods, which are inadequate for this age group owing to both their nutritional composition and lower food safety control. Baby foods represent a family of products meeting parents' expectations and adapted to infants and young children. They are clearly beneficial in terms of food safety, but the nutritional benefit to be expected from their consumption is minimal: their main advantage is postponing or decreasing the consumption of non-specific industrially processed foods.


Assuntos
Grão Comestível/normas , Alimentos Infantis/normas , Fórmulas Infantis/normas , Transtornos da Nutrição do Lactente/prevenção & controle , Necessidades Nutricionais , Pré-Escolar , Feminino , Inocuidade dos Alimentos , França , Humanos , Lactente , Transtornos da Nutrição do Lactente/etiologia , Masculino , Valor Nutritivo , Pediatria , Sociedades Médicas
5.
Arch Pediatr ; 20 Suppl 2: S29-48, 2013 Nov.
Artigo em Francês | MEDLINE | ID: mdl-25063312

RESUMO

The prevalence of breastfeeding in France is one of the lowest in Europe: 65% of infants born in France in 2010 were breastfed when leaving the maternity ward. Exclusive breastfeeding allows normal growth until at least 6 months of age, and can be prolonged until the age of 2 years or more, provided that complementary feeding is started after 6 months. Breast milk contains hormones, growth factors, cytokines, immunocompetent cells, etc., and has many biological properties. The composition of breast milk is influenced by gestational and postnatal age, as well as by the moment of the feed. Breastfeeding is associated with slightly enhanced performance on tests of cognitive development. Exclusive breastfeeding for at least 3 months is associated with a lower incidence and severity of diarrhoea, otitis media and respiratory infection. Exclusive breastfeeding for at least 4 months is associated with a lower incidence of allergic disease (asthma, atopic dermatitis) during the first 2 to 3 years of life in at-risk infants (infants with at least one first-degree relative presenting with allergy). Breastfeeding is also associated with a lower incidence of obesity during childhood and adolescence, as well as with a lower blood pressure and cholesterolemia in adulthood. However, no beneficial effect of breastfeeding on cardiovascular morbidity and mortality has been shown. Maternal infection with hepatitis B and C virus is not a contraindication to breastfeeding, as opposed to HIV infection and galactosemia. A supplementation with vitamin D and K is necessary in the breastfed infant. Very few medications contraindicate breastfeeding. Premature babies can be breastfed and/or receive mother's milk and/or bank milk, provided they receive energy, protein and mineral supplements. Return to prepregnancy weight is earlier in breastfeeding mothers during the 6 months following delivery. Breastfeeding is also associated with a decreased risk of breast and ovarian cancer in the premenopausal period, and of osteoporosis in the postmenopausal period.


Assuntos
Aleitamento Materno , Desenvolvimento Infantil , Diabetes Mellitus Tipo 1/prevenção & controle , Hipersensibilidade/prevenção & controle , Transtornos da Nutrição do Lactente/prevenção & controle , Relações Mãe-Filho , Mães/estatística & dados numéricos , Obesidade/prevenção & controle , Adulto , Asma/prevenção & controle , Índice de Massa Corporal , Aleitamento Materno/estatística & dados numéricos , Cognição , Depressão Pós-Parto/prevenção & controle , Dermatite Atópica/prevenção & controle , Suplementos Nutricionais , Medicina Baseada em Evidências , Feminino , França/epidemiologia , Promoção da Saúde , Inquéritos Epidemiológicos , Humanos , Lactente , Prevalência , Fatores de Risco , Organização Mundial da Saúde
6.
Br J Nutr ; 108(9): 1714-20, 2012 Nov 14.
Artigo em Inglês | MEDLINE | ID: mdl-22947201

RESUMO

Improving health through better nutrition of the population may contribute to enhanced efficiency and sustainability of healthcare systems. A recent expert meeting investigated in detail a number of methodological aspects related to the discipline of nutrition economics. The role of nutrition in health maintenance and in the prevention of non-communicable diseases is now generally recognised. However, the main scope of those seeking to contain healthcare expenditures tends to focus on the management of existing chronic diseases. Identifying additional relevant dimensions to measure and the context of use will become increasingly important in selecting and developing outcome measurements for nutrition interventions. The translation of nutrition-related research data into public health guidance raises the challenging issue of carrying out more pragmatic trials in many areas where these would generate the most useful evidence for health policy decision-making. Nutrition exemplifies all the types of interventions and policy which need evaluating across the health field. There is a need to start actively engaging key stakeholders in order to collect data and to widen health technology assessment approaches for achieving a policy shift from evidence-based medicine to evidence-based decision-making in the field of nutrition.


Assuntos
Ensaios Clínicos como Assunto/economia , Dieta/economia , Distúrbios Nutricionais/prevenção & controle , Tecnologia Biomédica/economia , Custos e Análise de Custo/métodos , Medicina Baseada em Evidências/economia , Humanos , Distúrbios Nutricionais/economia , Política Nutricional
7.
Arch Pediatr ; 19(10): 1110-7, 2012 Oct.
Artigo em Francês | MEDLINE | ID: mdl-22959889

RESUMO

Protein energy malnutrition (PEM) occurs when energy and protein intake do not meet requirements. It has a functional and structural impact and increases both morbidity and mortality of a given disease. The Nutrition Committee of the French Pediatric Society recommends weighing and measuring any child when hospitalized or seen in consultation. The body mass index (BMI) must be calculated and analyzed according to references any time growth kinetics cannot be analyzed. Any child with a BMI below the third centile or -2 standard deviations for age and sex needs to be examined looking for clinical signs of malnutrition and signs orienting toward an etiology and requires having his BMI and height dynamics plotted on a chart. PEM warrants drawing up a nutritional strategy along with the overall care plan. A target weight needs to be determined as well as the quantitative and qualitative nutritional care including its implementation. This plan must be evaluated afterwards in order to adapt the nutritional therapy.


Assuntos
Desnutrição Proteico-Calórica/diagnóstico , Índice de Massa Corporal , Criança , Humanos , Programas de Rastreamento , Guias de Prática Clínica como Assunto , Prevalência , Valores de Referência
8.
Arch Pediatr ; 19(3): 295-304, 2012 Mar.
Artigo em Francês | MEDLINE | ID: mdl-22310020

RESUMO

Iron deficiency is the most frequent cause of hypochromic microcytic anemia in children, but other causes, some of them requiring specific management, may be involved. Checking the iron-status is absolutely mandatory. When iron-status parameters are low, inadequate intake, malabsorption, blood loss, and abnormal iron utilization must be tested. In absence of iron deficiency, α- and ß-globin and heme biosynthetic gene status must be checked. Assessing the iron stock level is difficult, because there is an overlap between the values observed in iron-replete and iron-deprived patients, so that at least 2 iron-status parameters must be below normal for diagnosing iron deficiency. Furthermore, inflammation may also mimic some characteristics of iron deficiency. Diagnosing iron deficiency leads to prescribing iron supplementation with follow-up at the end and 3 months after cessation of treatment. When iron stores are not replete at the end of treatment, compliance and dosage must be reevaluated and occult bleeding sought. The latter is also required when the iron store decreases 3 months after cessation of iron replacement.


Assuntos
Anemia Hipocrômica/diagnóstico , Adolescente , Anemia Hipocrômica/etiologia , Anemia Hipocrômica/terapia , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/etiologia , Anemia Ferropriva/terapia , Criança , Pré-Escolar , Feminino , Seguimentos , Heme/genética , Humanos , Lactente , Ferro/administração & dosagem , Ferro/sangue , Masculino , alfa-Globinas/genética , Globinas beta/genética
9.
Arch Pediatr ; 19(3): 316-28, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22284232

RESUMO

The aims of the present position paper by the Committee on Nutrition of the French Society of Paediatrics were to summarize the recently published data on vitamin D in infants, children and adolescents, i.e., on metabolism, physiological effects, and requirements and to make recommendations on supplementation after careful review of the evidence. Scientific evidence indicates that calcium and vitamin D play key roles in bone health. The current evidence, limited to observational studies, however, does not support other benefits for vitamin D. More targeted research should continue, especially interventional studies. In the absence of any underlying risk of vitamin D deficiency, the recommendations are as follows: pregnant women: a single dose of 80,000 to 100,000 IU at the beginning of the 7th month of pregnancy; breastfed infants: 1000 to 1200 IU/day; children less than 18 months of age, receiving milk supplemented with vitamin D: an additional daily dose of 600 to 800 IU; children less than 18 months of age receiving milk not supplemented with vitamin D: daily dose of 1000 to 1200 IU; children from 18 months to 5 years of age: 2 doses of 80,000 to 100,000 IU every winter (November and February). In the presence of an underlying risk of vitamin D deficiency (dark skin; lack of exposure of the skin to ultraviolet B [UVB] radiation from sunshine in summer; skin disease responsible for decreased exposure of the skin to UVB radiation from sunshine in summer; wearing skin-covering clothes in summer; intestinal malabsorption or maldigestion; cholestasis; renal insufficiency; nephrotic syndrome; drugs [rifampicin; antiepileptic treatment: phenobarbital, phenytoin]; obesity; vegan diet), it may be justified to start vitamin D supplementation in winter in children 5 to 10 years of age as well as to maintain supplementation of vitamin D every 3 months all year long in children 1 to 10 years of age and in adolescents. In some pathological conditions, doses of vitamin D can be increased. If necessary, the determination of 25(OH) vitamin D serum concentration will help determine the level of vitamin D supplementation.


Assuntos
Cálcio/administração & dosagem , Pediatria , Sociedades Médicas , Vitamina D/administração & dosagem , Vitamina D/fisiologia , Adolescente , Adulto , Fatores Etários , Desenvolvimento Ósseo/fisiologia , Cálcio/fisiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Política Nutricional , Necessidades Nutricionais , Gravidez , Valores de Referência , Estações do Ano , Deficiência de Vitamina D/tratamento farmacológico , Deficiência de Vitamina D/etiologia
10.
Br J Nutr ; 107(3): 325-38, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22115523

RESUMO

The diagnosis of cows' milk protein allergy (CMPA) requires first the suspicion of diagnosis based on symptoms described in the medical history, and, second, the elimination of cows' milk proteins (CMP) from the infant's diet. Without such rigorous analysis, the elimination of CMP is unjustified, and sometimes harmful. The elimination diet should be strictly followed, at least until 9-12 months of age. If the child is not breast fed or the mother cannot or no longer wishes to breast feed, the first choice is an extensively hydrolysed formula (eHF) of CMP, the efficacy of which has been demonstrated by scientifically sound studies. If it is not tolerated, an amino acid-based formula is warranted. A rice protein-based eHF can be an alternative to a CMP-based eHF. Soya protein-based infant formulae are also a suitable alternative for infants >6 months, after establishing tolerance to soya protein by clinical challenge. CMPA usually resolves during the first 2-3 years. However, the age of recovery varies depending on the child and the type of CMPA, especially whether it is IgE-mediated or not, with the former being more persistent. Once the child reaches the age of 9-12 months, an oral food challenge is carried out in the hospital ward to assess the development of tolerance and, if possible, to allow for the continued reintroduction of CMP at home. Some children with CMPA will tolerate only a limited daily amount of CMP. The current therapeutic options are designed to accelerate the acquisition of tolerance thereof, which seems to be facilitated by repeated exposure to CMP.


Assuntos
Aleitamento Materno , Fórmulas Infantis/química , Hipersensibilidade a Leite/dietoterapia , Proteínas do Leite/efeitos adversos , Aminoácidos/uso terapêutico , Criança , Pré-Escolar , Árvores de Decisões , União Europeia , França , Humanos , Tolerância Imunológica , Lactente , Alimentos Infantis/efeitos adversos , Hipersensibilidade a Leite/imunologia , Valor Nutritivo , Proteínas de Plantas/uso terapêutico , Hidrolisados de Proteína/uso terapêutico , Remissão Espontânea
12.
Arch Pediatr ; 18(1): 79-94, 2011 Jan.
Artigo em Francês | MEDLINE | ID: mdl-21115329

RESUMO

New data on food allergy has recently changed the management of children with cow's milk protein allergy (CMPA). The diagnosis of CMPA first requires the elimination of cow's milk proteins and then an oral provocation test following a standard diagnostic procedure for food allergy, without which the elimination diet is unjustified and sometimes harmful. Once the diagnosis is made, the elimination diet is strict, at least until the age of 9-12 months. If the child is not breastfed or the mother cannot or no longer wishes to breastfeed, the first choice is a formula based on extensive hydrolyzate of cow's milk (eHF), provided that its effectiveness has been demonstrated. When eHF fails, a formula based on amino acids is warranted. eHF based on rice protein hydrolysates is an alternative to cow's milk eHF. Infant formulas based on soy protein can be used after the age of 6 months, after verification of good clinical tolerance to soy. Most commonly, CMPA disappears within 2 or 3 years of life. However, the age of recovery varies depending on the child and the type of CMPA, and whether or not it is IgE-mediated, the first being more sustainable. When the child grows, a hospital oral provocation test evaluates the development of tolerance and, if possible, authorizes continuing the reintroduction of milk proteins at home. Some children with CMPA will tolerate only a limited daily amount of cow's milk proteins. The current therapeutic options are designed to accelerate the acquisition of tolerance, which seems facilitated by regular exposure to cow's milk proteins.


Assuntos
Hipersensibilidade a Leite/dietoterapia , Hipersensibilidade a Leite/etiologia , Proteínas do Leite/efeitos adversos , Humanos , Lactente
13.
Clin Pharmacol Ther ; 88(1): 101-8, 2010 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-20505667

RESUMO

Carnitine palmitoyltransferase 2 (CPT2) deficiency is a rare mitochondrial fatty acid oxidation (FAO) disorder characterized by myalgia, exercise intolerance, and rhabdomyolysis. We evaluate the efficacy of bezafibrate (BZ), a hypolipidemic drug, as a treatment for this form of CPT2 deficiency. A pilot trial was conducted with BZ in six patients for 6 months. There was a follow-up period of 3 years. The oxidation rates of the long-chain fatty acid derivative palmitoyl-CoA, measured in the mitochondria of the patients' muscles, were markedly lower than normal before treatment and increased significantly (+39 to +206%; P = 0.028) in all patients after BZ treatment. The evaluation of the therapeutic effects by the patients themselves (using the Short Form Health Survey (SF-36)), as well as by the physicians, indicated an improvement in the condition of the patients; there was an increase in physical activity and a decline in muscular pain. The results suggest that BZ has a therapeutic effect in the muscular form of CPT2 deficiency.


Assuntos
Bezafibrato/uso terapêutico , Carnitina O-Palmitoiltransferase/biossíntese , Carnitina O-Palmitoiltransferase/deficiência , Hipolipemiantes/uso terapêutico , Doenças Musculares/tratamento farmacológico , Doenças Musculares/etiologia , Atividades Cotidianas , Acil-CoA Desidrogenase de Cadeia Longa/biossíntese , Acil-CoA Desidrogenase de Cadeia Longa/genética , Adulto , Carnitina O-Palmitoiltransferase/genética , Teste de Esforço , Feminino , Seguimentos , Regulação Enzimológica da Expressão Gênica/efeitos dos fármacos , Humanos , Linfócitos/efeitos dos fármacos , Linfócitos/enzimologia , Masculino , Pessoa de Meia-Idade , Mitocôndrias Musculares/enzimologia , Músculo Esquelético/efeitos dos fármacos , Músculo Esquelético/enzimologia , Doenças Musculares/genética , Oxirredução , Consumo de Oxigênio/efeitos dos fármacos , Dor/epidemiologia , Dor/etiologia , Palmitoil Coenzima A/metabolismo , Projetos Piloto , Rabdomiólise/tratamento farmacológico , Rabdomiólise/enzimologia , Resultado do Tratamento , Adulto Jovem
15.
Arch Pediatr ; 17(1): 51-9, 2010 Jan.
Artigo em Francês | MEDLINE | ID: mdl-19944575

RESUMO

Atherosclerosis begins during childhood. From childhood, a strong relation has been shown between the prevalence and extent of the asymptomatic atherosclerosis lesions and cardiovascular risk factors such as elevation in body mass index, blood pressure and plasma lipid concentrations. These risk factors depend not only on the subjects' genetic predisposition, but also on environmental parameters, particularly diet. The Committee on Nutrition reviewed the scientific basis of dietary recommendations for children that could reduce the risk factors and thereby, reduce the risk of coronary heart disease in later life: the effects of prenatal nutrition; the beneficial consequences of breast-feeding on later levels of cholesterolemia, blood pressure and corpulence; the role of dietary lipids on plasma lipid concentration, of salt and potassium on blood pressure, and of lifestyle on corpulence.


Assuntos
Transtornos da Nutrição Infantil/etiologia , Doença da Artéria Coronariana/etiologia , Dieta Aterogênica , Comportamento Alimentar , Adulto , Pressão Sanguínea , Índice de Massa Corporal , Aleitamento Materno , Criança , Transtornos da Nutrição Infantil/sangue , Transtornos da Nutrição Infantil/prevenção & controle , Pré-Escolar , Colesterol/sangue , Doença da Artéria Coronariana/sangue , Doença da Artéria Coronariana/prevenção & controle , Feminino , Humanos , Hipercolesterolemia/sangue , Hipercolesterolemia/etiologia , Hipercolesterolemia/prevenção & controle , Lactente , Recém-Nascido , Obesidade/sangue , Obesidade/etiologia , Obesidade/prevenção & controle , Gravidez , Fatores de Risco
16.
Eur J Haematol ; 83(6): 572-8, 2009 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-19682065

RESUMO

OBJECTIVES: To assess the prevalence in children with sickle cell disease of low bone mineral density (BMD), a feature found in up to 82% of adults but not well known in children. METHODS: In 53 children (45 SS, 4 SC, 4 Sbeta-thalassemia) with a mean age of 12.8 +/- 2.4 years, we assessed height; weight; sexual maturation; number of hospitalizations, painful crises, and transfusions in the last 3 years; calcium intake; steady-state hemoglobin and leukocyte count; calcaemia, phosphataemia, and calciuria/creatinuria; serum 25-(OH)D and PTH concentrations; and osteocalcin, urinary deoxypyridinoline, and the C-terminal component of pro-collagen type I. BMD was assessed using dual X-ray absorptiometry. RESULTS: Mean lumbar spine Z-score was -1.1 +/- 1.3 (-3.9 to +1.8). The Z score was significantly lower in girls than in boys in the prepubertal subgroup (-1.74 +/- 0.27 vs. -0.53 +/- 0.31) (P = 0.0169), but not in the pubertal group (-1.15 +/- 0.41 vs. -1.33 +/- 0.70). BMD was not associated with any of the disease-severity markers in girls but was unexpectedly associated with fewer vaso-occlusive crises and hospitalizations in boys. BMD did not correlate with hemoglobin or leukocyte counts. Vitamin D deficiency [25-(OH)D < 12 ng/mL] was found in 76% of patients and secondary hyperparathyroidism (PTH > 46 pg/mL) in 38%. BMD was not related to calcium intake, vitamin D status, osteocalcin, or bone resorption markers. CONCLUSION: A slight BMD decrease was found in SCD children, starting before puberty and being more marked in females. The decrease was unrelated to disease severity, vitamin D deficiency, or bone hyperresorption, suggesting abnormal bone formation as the underlying mechanism.


Assuntos
Anemia Falciforme/complicações , Doenças Ósseas Metabólicas/epidemiologia , Deficiência de Vitamina D/epidemiologia , Adolescente , Anemia Falciforme/sangue , Anemia Falciforme/terapia , Antropometria , Biomarcadores , Densidade Óssea , Doenças Ósseas Metabólicas/sangue , Doenças Ósseas Metabólicas/etiologia , Reabsorção Óssea/urina , Cálcio/metabolismo , Cálcio da Dieta/análise , Criança , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Vértebras Lombares/química , Masculino , Osteocalcina/sangue , Osteogênese/fisiologia , Hormônio Paratireóideo/sangue , Prevalência , Puberdade , Deficiência de Vitamina D/sangue , Deficiência de Vitamina D/etiologia
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