Introduction: Major Beta Thalassemia (MBT) is a common hereditary condition in Algeria (2%) and in North Africa it requires repeated transfusions. Life expectancy has improved significantly thanks to advances in chelating treatments that will delay endocrine complications associated with secondary hemochromatosis. The hypoparathyroidism (HPT) continues to be seen (520%), its relation with the iron overload is not clearly elucidated.
Patients and methods: It is about 20 BTM patients of mean age 29 years (2046) (10 Males/10 Females) hospitalized in hematology at the Pierre Marie Curie Center, Algiers. In whom an infra-clinical HPT has been researched in cases of hypocalcemia, hyperphosphoremia or high alkaline phosphatase.
Results: The prevalence of asymptomatic HPT is 25%, normo-calcemic in 40%. Vitamin D < 10 ng/ml exists in 50% of MBT. Among patient with HPT, 3 patients are diabetics. 2 patients had infra clinical hypothyroidism, only one BTM with HPT has a growth delay less than 2 deviation, and all cases have central hypogonadism except one patient. All HPT patients are under Defirasirox, the 5 cases benefited from biological check up of magnesium level and bone densitometry.
|Patients||Age (years)/Sex||Calcium/phosphorus||PAL ui/l||Parathormon pg/ml||Vit D ng/ml||Feritin ng/ml|
|Case 1||25/Female (F)||84/43 mg/l (miligrams/liter)||106||15.41||8.2||9177|
|Case 2||35/Male (M)||69/42 mg/l||144||16.07||8.1||1500|
|Case 3||26/F||57/81 mg/l||113||4.81||8.1||8620|
|Case 4||31/M||77/48 mg/l||Not available||22.34||12.9||5736|
|Case 5||28/M||86/54 mg/l||170||22.77||8.1||4193|
Discussion: An annual screening of HPT after the start of the second decade is required. Its coexistence with a frequent hyper-calciuria in the BTM make that the conventional preventive treatment by living D and calcium in the long course must be reviewed because of the risk of nephrocalcinose worsening the renal prognosis already altered by the renal hemosiderosis. Other therapeutic weapons such as recombinant Parathormon prove to be more effective both renally and on bone capital already altered by other endocrinopathies such as central hypogonadism. Exception for infra clinical HPT, in which wait and see seems to be more adequate management.
19 May 2018 - 22 May 2018