Bone Metabolism in Thalassemia<sup>a</sup>

Garofalo, Franco; Piga, Antonio; Lala, Roberto; Chiabotto, S.; Stefano, Marco Di; Isaia, G.C.

doi:10.1111/j.1749-6632.1998.tb10526.x

Cited by 20 publications

(25 citation statements)

References 19 publications

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“…Vogiatzi et al hanno segnalato in adolescenti T una DMO bassa (Z score ≤ -2) nel 61,3 % dei casi e ridotta (Z score compreso tra -1 e -2) nel 22,6%; il progredire dell'età, inoltre, correla strettamente con un decremento della DMO (38). La riduzione della DMO sembra essere simile nei due sessi, così come le fratture ossee (12,13). Una bassa DMO è associata, nei T, alla cardiomiopatia, al diabete, all'epatite cronica, all'incremento delle transaminasi, all'amenorrea (31) ed alla mancata terapia ormonale sostitutiva nei casi di ipogonadismo ipogonadotropo (29)(30)(31).…”

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Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Trotta¹,

Corrado²,

Cantatore³

2011

Reumatismo

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cesso di catene globiniche libere nel citosol degli eritroblasti ed eritropoiesi inefficace (morte intramidollare dell'eritroblasto) (4). Sulla base della catena mancante si distinguono diverse forme di ST; in Italia prevale nettamente la b-talassemia (BT) (5). Tra le BT ricordiamo: la talassemia major (Morbo di Cooley) (TM) nella quale la malattia trova piena espressione clinica (anemia grave post-natale, espansione midollare, fabbisogno trasfusionale sistematico, interessamento multi-organo con eritropoiesi extra-midollare a livello splenico ed epatico) (5); la talassemia intermedia (TI) con espressione clinica meno spiccata (esordio tardivo, assenza per lunghi periodi di fabbisogno trasfusionale) (6). INTRODUZIONEL e sindromi talassemiche (ST), descritte da Cooley e Lee nel 1935 (1), sono patologie genetiche in cui si ha l'abolizione/riduzione della sintesi di una o più catene globiniche, costituendo i più frequenti disordini monogenici al mondo (2, 3). Le ST, oltre alla riduzione della sintesi emoglobinica con conseguente anemia, manifestano un ec- Reumatismo, 2010; 62(2):119-126 SUMMARY Transfusion program and chelating therapy treatment has extended the life expectancy of thalassaemic patient; osteoporosis is considered an important cause of morbidity in adult patients who display increased fracture risk. This is a case report is about a thalassaemic young female with multiple spine fractures (D11, D12 e L2) and lumbar spine DEXA -Tscore = -3,1 and femoral = -3,4. This was in spite of therapy with alendronate 70 mg/week from January 2006 to September 2007. The patient was subsequentently treated for 18 months with 1-34 recombinant human parathyroid hormone and colecalciferol (100.000 U/monthly). After 4 months of therapy, the patient showed a decrease in spinal pain (Roland and Morris

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Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Trotta¹,

Corrado²,

Cantatore³

2011

Reumatismo

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“…This is because they still had normal endocrine function. 9 A group with bone age difference >36 months had higher serum ferritin level compared with that with bone age difference <36 months. A study by Brook 14 found that patients with bone age difference >36 months had poor prognosis compared with patients with bone age difference <36 months.…”

Section: Discussionmentioning

confidence: 97%

“…20 Properly treated thalassemia patients can grow normally until 10-12 years, but a growth impairment can still occurs thereafter. 9 This might be due to direct toxicity of iron overload to osteoblast. 9 All subjects had bone age disturbance.…”

Section: Discussionmentioning

confidence: 99%

“…9 This might be due to direct toxicity of iron overload to osteoblast. 9 All subjects had bone age disturbance. This might be due to inadequate transfusion methods they got.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with thalassemia can grow normally until 10-12 years, but after that, some of them can have growth impairment. 9 We performed physical examination, serum ferritin measurement, and bone age examination. A child was diagnosed as short stature if the height was below third percentile on National Center for Chronic Disease Prevention and Health Promotion (CDC) 2000 curve.…”

Section: Methodsmentioning

confidence: 99%

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Association between age and serum ferritin level with bone age deficit in children with thalassemia major

Andayani¹,

Sekarwana²,

Fadil³

2008

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Background Multiple blood transfusions in thalassemia patientslead to iron overload in bone tissue. Iron overload can bedetermined by serum ferritin measurement. Several studies haveevaluated association between serum ferritin level and growth,but without bone age examination.Objective To determine the association between age and serumferritin level with bone age in children with thalassemia major.Methods This study was conducted at Hasan Sadikin HospitalBandung during March-May 2007. We performed physicalexamination, serum feritin measurement, and bone ageexamination. Data were analyzed with x 2 to determine associationbetween variables. The association between age and serum ferritinlevel with bone age deficit was analyzed with regression logisticmodel.Results Subjects consisted of 49 patients with thalassemia major.All subjects had bone age deficit. Most boys were in age group of>10 years and had bone age difference >36 months, while mostgirls were <10 years and had bone age difference <36 months.Subjects with bone age difference <36 months mostly had serumferritin level <5,000 ng/dL, while most subjects with bone agedifference >36 months had serum ferritin level =5,000 ng/dL.This was statistically significant (x 2 =4.573, P=0.032). There wasassociation between age and bone age deficit (OR=13.461, 95%CI 3.199;56.640), but not with serum ferritin level (OR= 2.199,95% CI 0.532;9.095).Conclusion In thalassemic children, bone age deficit is associatedwith age, but not with serum feritin level.

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Bone health in pediatric transfusion‐dependent beta‐thalassemia: Circulating osteoprotegerin and RANKL system

Youssry

Saad

Madboly

et al. 2021

Pediatric Blood & Cancer

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Introduction While the mechanism of bone disease in thalassemia is multifactorial and still under investigation, the receptor activator of nuclear factor kappa B (RANK), receptor activator of nuclear factor kappa B ligand (RANKL), and osteoprotegerin (OPG) have pivotal roles in regulating bone metabolism. This study aimed to measure RANKL and OPG serum levels, and to detect the incidence of RANKL rs9533156, OPG rs2073618, and OPG rs2073617 genotypes in pediatric β‐thalassemia patients and to assess their relation to bone mineral density. Methods Sixty patients with transfusion‐dependent β‐thalassemia (TBT) patients ages 5 to 14 years were included, and 60 healthy, age‐ and sex‐matched volunteers contributed as a control group. The patients were scanned for bone mineral density. Results The mean of spine dual‐energy X‐ray absorptiometry (DXA) Z‐score in patients was −1.66 ± 1.02 standard deviation (SD). Twenty‐four of them had low spine DXA Z‐scores. The patients showed significantly lower OPG levels and OPG/RANKLs ratios than the control group (3.28 ± 9.11 ng/ml and 11.38 ± 14.93 ng/ml, and 0.01 ± 0.03 and 0.07 ± 0.09, respectively). The RANKL SNP rs9533156 TC heterozygous genotype was detected more with statistical significance in patients than controls. The incidence of OPG rs2073618 and OPG rs2073617 genotypes were 2.3 times and 1.9 times more frequent in patients than controls, respectively. Conclusion The RANK/RANKL/OPG system may have an important role in regulating bone metabolism in TBT patients, although further studies are needed to clarify its role.

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Bone Metabolism in Thalassemia^a

Cited by 20 publications

References 19 publications

Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Association between age and serum ferritin level with bone age deficit in children with thalassemia major

Bone health in pediatric transfusion‐dependent beta‐thalassemia: Circulating osteoprotegerin and RANKL system

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Bone Metabolism in Thalassemiaa

Cited by 20 publications

References 19 publications

Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

Association between age and serum ferritin level with bone age deficit in children with thalassemia major

Bone health in pediatric transfusion‐dependent beta‐thalassemia: Circulating osteoprotegerin and RANKL system

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Bone Metabolism in Thalassemia^a