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osteoporosis and thalassemia minor

K Lampropoulou-Adamidou, S Tournis, I K Triantafyllopoulos
There are numerous studies presenting the beneficial effect of bisphosphonates (BPs) on bone disease of patients suffering from beta-thalassemia major (TM). Although BPs have been widely used, adverse events have been described including atypical femoral fractures (AFF). In the present case, a male adult patient suffering from TM sustained an AFF fulfilling all major and two minor criteria. Before AFF, the patient had been treated with zoledronic acid for three years and remained another one year without osteoporosis therapy...
March 2016: Journal of Musculoskeletal & Neuronal Interactions
Renzo Galanello, Raffaella Origa
Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor. Individuals with thalassemia major usually present within the first two years of life with severe anemia, requiring regular red blood cell (RBC) transfusions...
May 21, 2010: Orphanet Journal of Rare Diseases
R Di Matteo, F Liuzza, F Pezzillo, L Gerardino, G Maccauro
Beta-Thalassemia Major (TM), firstly described by Cooley, is and inherited blood disorder that leads to anaemia due to an imbalanced globin chain synthesis effecting erythroid maturation and red cells survival and requests regular life-long blood transfusions and iron-chelating therapy. Among all the complications and organ diseases, osteopenia and osteoporosis, as progressive diseases are getting important cause of morbidity in a population whose longevity is increasing. The authors describe a case of a 26-year-old female with Beta-Thalassemia Major affected by a subtrochanteric fracture of the right femur on minor trauma...
September 2007: La Clinica Terapeutica
Turker Cetin, Cagatay Oktenli, Taner Ozgurtas, Mujdat Yenicesu, S Yavuz Sanisoglu, Yusuf Oguz, Oguzhan Yildiz, Ismail Kurt, Ugur Musabak, Fatih Bulucu, Ismail H Kocar
BACKGROUND: Persons with beta-thalassemia minor usually are symptomless. However, we previously reported renal tubular dysfunction in a patient with beta-thalassemia minor. The aim of this study is to investigate renal function in patients with beta-thalassemia minor. METHODS: Forty-one subjects with beta-thalassemia minor and 20 sex- and age-matched healthy subjects were enrolled in the study. For analysis, patients were divided into 2 groups: group A, all patients with anemia (n = 19), and group B, patients without anemia (n = 22)...
December 2003: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
M Angastiniotis, N Pavlides, K Aristidou, A Kanakas, M Yerakaris, E Eracleous, T Posporis
An increasing number of adult thalassaemics have been complaining of aches and pains of varying degrees of severity. In a minority the pains are debilitating and there is stiffness in movement. This study is an attempt to understand the osteoporosis of thalassaemia using DEXA and MRI as the main investigative tools. 122 patients with homozygous beta-thalassaemia were examined by DEXA. It was found that almost half had BMD below two standard deviations from the mean for the normal population, especially in the lumbar spine...
1998: Journal of Pediatric Endocrinology & Metabolism: JPEM
J Kalef-Ezra, A Challa, N Chaliasos, I Hatzikonstantinou, I Papaefstathiou, V Cholevas, D Glaros, P Lapatsanis
Homozygous beta-thalassemia is a severe hereditary disorder associated with osteopenia. Recently it was suggested that thalassemia minor may be a risk factor for osteoporosis. The purpose of the present study was to investigate this suggestion. Bone mineral status was assessed in 22 premenopausal women and 21 men with beta-thalassemia minor. In vivo neutron activation analysis was applied to measure hand-bone phosphorus (HBP), single-photon absorptiometry to measure forearm bone mineral content (BMC), and dual-energy X-ray absorptiometry to measure spinal bone mineral density (BMD)...
June 1995: Bone
L Rioja, R Girot, M Garabédian, G Cournot-Witmer
The histological features of thalassemic bone are imperfectly known, and the roles of bone marrow hyperactivity, iron overload or vitamin D deficiency in the pathogenesis of the disease are not clearly identified. In this study we examined iliac crest biopsies from 17 transfusion-dependent children with homozygous beta-thalassemia and severe radiological skeletal thalassemic changes, including widening of medullary spaces and osteoporosis. Rachitic lesions were not observed. Serum ferritin concentrations were increased in all but one subject...
January 1990: Bone and Mineral
H J Chiou, C M Tiu, C Y Chang, S S Chen, T Chang
There were altogether 68 patients suffered from beta-thalassemia in the Veterans General Hospital from 1979 to 1986. However only 18 patients had abnormal roentgenologic findings. They were 7 males and 11 females. Their ages ranged from 8 months to 47 years with an average of 13 years. Clinically beta-thalassemia was divided into 3 types: 1) thalassemia major, 2) thalassemia intermediate, 3) thalassemia minor. The osteoporosis, hepatosplenomegaly, and extramedullary hematopoiesis with pseudo-tumor formation...
March 1990: Zhonghua Minguo Xiao Er Ke Yi Xue Hui za Zhi [Journal]
N Gómez Rodríguez, J M Sánchez Bursón, A Atanes Sandoval, J Graña Gil, J de Toro Santos, F Galdo Fernández
We present the case of a 48-year-old premenopausal woman with right lumbosciatalgia secondary to osteoporosic vertebral collapses. Two of her three offsprings, a 31-year-old man and a 16-year-old woman, had chronic dorsolumbalgia, the study of which suggested the diagnosis of minor beta-thalassemia. As opposed to thalassemic osteoarthropathy, frequently observed in the forms of "major" and "minor" beta-thalassemia, rheumatic manifestations have almost never been described in the "minor" forms. We highlight the possibility of symptomatic osteoporosis as the first manifestation and we review the locomotive disorders associated to this hemoglobinopathy...
June 1992: Anales de Medicina Interna: Organo Oficial de la Sociedad Española de Medicina Interna
N Greep, A L Anderson, J C Gallagher
A case report of a 53 year old postmenopausal woman with severe osteoporosis occurring in association with beta thalassemia trait is presented. She was found to have marked axial osteopenia with lumbar spine bone mineral density that was more than three standard deviations below the mean, but had normal bone mineral density of the proximal radius. A biopsy of the iliac crest showed evidence of high turnover osteoporosis. Bone densitometry of the spine and hip in a consecutive series of eleven asymptomatic individuals with thalassemia trait also is reported...
January 1992: Bone and Mineral
U Schlumpf, N Gerber, H Bünzli, U Elsässer, A Pestalozzi, A Böni
Seven cases with the syndrome of thalassemia minor and pauciarticular, non-erosive, seronegative arthritis are described. Other known causes of seronegative arthritis had been excluded. There was a predilection for middlesized joints such as ankles, wrists and elbows. Usually the synovitis was asymmetrical from onset, with one to eight joints affected. Finger or toe joint were usually spared. Extraarticular synovitis such as tenosynovitis or bursitis were not observed, nor were nodules, signs of vasculitis or visceral involvement...
August 20, 1977: Schweizerische Medizinische Wochenschrift
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