Distal ulnar changes in children with thalassemia and deferiprone related arthropathy

Sharma, Rajni; Anand, Rama; Chandra, Jagdish; Seth, Anju; Pemde, Harish; Singh, Varinder

doi:10.1002/pbc.24678

Cited by 16 publications

(4 citation statements)

References 20 publications

(29 reference statements)

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“…Arthropathy of large joints has been recognized as a toxic side effect of deferiprone (DFP) therapy. Bilateral knee involvement is most common, but ankles, hips, shoulders, elbows, wrists and small joints of the hands and feet can also be affected [65][66][67][68][69] . Clinically, the patients complain for musculoskeletal stiffness, joint pain, swelling and effusion, more frequently involving the knees 70 .…”

Section: Deferiprone Related Arthropathymentioning

confidence: 99%

Skeletal radiological findings in thalassemia major

Adamopoulos¹,

Petrocheilou²

2020

JRPMS

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Skeletal changes in beta-thalassemia major (βTM) create a wide spectrum of bone radiographic features. The main pathology is extensive marrow proliferation due to ineffective erythropoiesis but the relative skeletal findings are encountered with a progressively reduced frequency and severity nowadays due to the regular hypertransfusion programs. The resulting hemosiderosis and particularly iron chelation therapy have been associated with dysplastic features which are found more often. Skeletal radiological appearances in βTM relate to a complex multifactorial pathogenesis (i.e. marrow expansion, direct iron and chelation toxicity, endocrine complications) and depend on the severity and duration of the disease, the type and effectiveness of treatment and the toxic effects of chelation therapy. Awareness of these findings is very important since early recognition can be an indicator for therapy adaptation.

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Section: Deferiprone Related Arthropathymentioning

confidence: 99%

Skeletal radiological findings in thalassemia major

Adamopoulos¹,

Petrocheilou²

2020

JRPMS

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“…Sharma et al reported similar radiological features in their study. 24,25 Radiological changes in thalassemia are usually evident by 1 year of age, these changes do occur at an earlier age with severe form in under transfused or non-transfused thalassemic children. Radiological changes are due to ineffective erythropoiesis, anaemia and hypoxia resulting in increased erythropoietin.…”

Section: Discussionmentioning

confidence: 99%

Biochemical indices and radiological examination to evaluate bone health in children with β-thalassemia major

Madhu

Bhagwan

2019

Int J Contemp Pediatr

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Background: β-Thalassemia major is a serious hematological problem requiring regular blood transfusions. In regularly transfused thalassemic patients, metabolic bone disease is an important cause of morbidity. Problems include bone pain, deformities, short stature, osteopenia/osteoporosis, rickets, osteomalacia, spinal deformities, nerve compression and fractures. This study was undertaken to evaluate the status of bone health in children with β-thalassemia major by selected biochemical indices and radiological examination.Methods: Fifty children with β- thalassemia major were enrolled. Detailed history, examination and anthropometry were done. Serum calcium, phosphorus, magnesium, alkaline phosphatase, and vitamin D levels were measured. X-ray of skull, wrist, and knee were studied for radiological changes. Biochemical indices were analysed with appropriate statistical tests.Results: Stunting and wasting was observed in 58% and 44% children respectively. Hypocalcaemia was seen in 22%, hyperphosphatemia in 56% and 24% children had raised alkaline phosphatase levels. Vitamin D levels were severely deficient in 12%, deficient in 50%, and insufficient in 38% of children. There was no significant difference between vitamin D levels of cases and controls. Radiologically almost all children had a range of osteopathy, like thinned out cortex (96%), medullary expansion (54%), loss of density (40%), diffuse osteoporosis (18%) and growth arrest lines were seen in 8% children.Conclusions: Vitamin D abnormalities and radiological features suggestive of decreased bone mineralization are seen in all the children with β-thalassemia major treated with periodic blood transfusion at this institute. No biochemical predictors of bone disease in these children were identified.

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“…Some studies observed that arthropathy is more common in patients with high serum ferritin and DFP doses above 100 mg/kg/d (33,42,54,95), but this could not be confirmed by another study (43). Suspected long-term effects of DFP-related arthropathies are bony dysplasia, deformation and impaired growth of ulnar epiphyses, metaphyses and physes (102).…”

Section: Deferiprone (Dfp)mentioning

confidence: 98%

Safety profiles of iron chelators in young patients with haemoglobinopathies

Botzenhardt

Chan

et al. 2017

European J of Haematology

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Background: This review describes the safety of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX) and combined therapy in young patients less than 25 yr of age with haemoglobinopathies. Methods: Searches in electronic literature databases were performed. Studies reporting adverse events associated with iron chelation therapy were included. Study and reporting quality was assessed using AHRQ Risk of Bias Assessment Tool and McMaster Quality Assessment Scale of Harms. Prospective clinical studies were pooled in a random-effects meta-analysis of proportions. Results: Safety data of 2040 patients from 34 studies were included. Ninety-two case reports of 246 patients were identified. DFX (937 patients) and DFP (667 patients) possess the largest published safety evidence. Fewer studies on combination regimens are available. Increased transaminases were seen in all regimens (3.9-31.3%) and gastrointestinal disorders with DFP and DFX (3.7-18.4% and 5.8-18.8%, respectively). Therapy discontinuations due to adverse events were low (0-4.1%). Reporting quality was selective and poor in most of the studies. Conclusion: Iron chelation therapy is generally safe in young patients, and published data correspond to summary of product characteristics. Each iron chelation regimen has its specific safety risks. DFO seems not to be associated with serious adverse effects in recommended doses. In DFP and DFX, rare, but serious, adverse reactions can occur. Data on combined therapy are scarce, but it seems equally safe compared to monotherapy.

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Distal ulnar changes in children with thalassemia and deferiprone related arthropathy

Abstract: Bony dysplasia, deformation and impaired growth of ulnar epiphyses, metaphyses and physes may be an expression of deferiprone related arthropathy in children with thalassemia major.

Cited by 16 publications

References 20 publications

Skeletal radiological findings in thalassemia major

Skeletal radiological findings in thalassemia major

Biochemical indices and radiological examination to evaluate bone health in children with β-thalassemia major

Safety profiles of iron chelators in young patients with haemoglobinopathies

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