Assuntos
Neoplasias de Tecido Conjuntivo/diagnóstico por imagem , Fluordesoxiglucose F18 , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias de Tecido Conjuntivo/etiologia , Osteomalacia , Síndromes Paraneoplásicas , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Medronato de Tecnécio Tc 99m , Tomografia Computadorizada de Emissão de Fóton Único , Imagem Corporal TotalRESUMO
OBJECTIVE: To evaluate osteopathy in thalassemia by bone mineral densitometry (BMD) and biochemical indices. METHODS: Prospective review analysis with no follow up from 2006 to 2007 of 42 regularly transfused thalassemics aged 10-25 years (27 boys, 15 girls) was done. Anthropometry, pubertal stage and symptomatology were noted. Urinary C-terminal cross-linked telopeptide of type-1 collagen (Crosslaps) by ELISA; serum 25-OH vitamin D and osteocalcin by RIA; parathyroid hormone (PTH) and ferritin by chemiluminescence and IGF-1 by Enzyme immunoassay were evaluated. Dual Energy X-ray Absorptiometry (DEXA) of lumbar spine and femur was done on Lunar prodigy system. Data was entered and analyzed using the SPSS for Windows software. Mean comparisons were done by ANOVA 1 and data was compared using Chi-square test and p value < 0.05 was taken as significant. RESULTS: Of 42 patients, 81% had osteoporosis by Z-score of DEXA. Urinary crosslaps was high in 55%; 36% had increased osteocalcin; 62% had low vitamin D levels; 38% had high parathyroid levels and IGF-1 was low in 52%. Mean serum ferritin level was 5344 ± 2855 ng/dl. There was statistical significance (p = 0.046) between chronological age and BMD. All 42 cases were divided into two groups: Group-1 (Normal DEXA), Group-2 (Abnormal DEXA) and analysis of biochemical indices between two groups showed no significant difference in any of the biochemical parameters. CONCLUSION: This study revealed majority of thalassemics with inadequate chelation have bone resorption with advancing chronological age and BMD should be evaluated regularly for early diagnosis to prevent morbidity.
Assuntos
Osteoporose/epidemiologia , Osteoporose/etiologia , Talassemia/complicações , Talassemia/diagnóstico , Absorciometria de Fóton/métodos , Adolescente , Adulto , Distribuição por Idade , Análise de Variância , Biomarcadores/análise , Transfusão de Sangue/métodos , Densidade Óssea/fisiologia , Distribuição de Qui-Quadrado , Criança , Estudos de Coortes , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Incidência , Índia/epidemiologia , Masculino , Osteocalcina/análise , Osteoporose/fisiopatologia , Hormônio Paratireóideo/análise , Estudos Prospectivos , Radioimunoensaio , Medição de Risco , Distribuição por Sexo , Talassemia/terapia , Vitamina D/sangue , Adulto JovemRESUMO
This study was conducted to determine L-carnitine levels in regularly transfused and chelated beta thalassemia patients (n=40; mean age, 17.5+/-5.0 years). Ten age matched controls were also studied. The mean L-carnitine level in thalassemic patients was 23.71+/- 7.3 microM as compared to control 29.26+/-2.37 microM (P<0.0001). Mean carnitine was significantly lower (P=0.037) in those with ferritin greater than 2000 ng/dL (22.80+/-6.97 microM) in comparison to those with ferritin less than 2000 ng/dL (30.1+/-7.77 microM). Although carnitine levels in non vegetarians was higher (26.91+/-8.4 microM) than in vegetarians (22.34+/-6.55 microM), this difference was not statistically significant (P=0.072). We conclude that L-carnitine levels were found to be lower in thalassemics as compared to age matched controls.