aili ali asgharian

Selection of iron chelator medication for thalassemic patients depends on clinical and laboratory characteristics such as age, serum ferritin and severity of different organ damage. Patient choice is also important because the compliance to treatment is essential. Policy makers should decide to import brands versus to facilitate making medicationsin the country. Insurance companies also should decide for coverage the costs. In this paper we report the current situation of iron chelator usage for patients with beta thalassemia major in our center.
We reviewed data the medical records of patients in November 2015.
There were 463 patients on iron chelator medications. Three hundred and thirty patients (71.2%) had regular blood transfusions and reminders were not on regular blood transfusion. Two hundred and fifty one (54%), and 212(46%) were using one or two iron chelators, respectively. Two hundred and two (44%) patients were using a combination of a parenteral and one oral medication. Sixty seven (14%) patients were using only parenteral iron chelator. The most common used medication was Osveral (made in Iran by Osveh Pharmaceutical) in about one third of patients. All non-transfusion dependent patients were using Osveral. The parenteral medication (Desferrioxamine) was distributed in two brands. One imported brand as Desferal (Novartis) and the other as Desfonak (made in Iran by Ronak Daru).Deferiprone(made in Avesina, Iran) was used in combination with desferrioxamine in 141 patients and as the only treatment in 11 patients.
Availability of different types of medications makes the opportunity to select the best protocol for patients.

Context: Vitamin D deficiency is a common nutritional disorder in Iran. Vitamin D is an essential health factor from birth onward. This study was conducted to summarize epidemiologic researches regarding vitamin D deficiency in different parts of the country and to conclude if food fortification is necessary.Evidence Acquisition: The study was designed in Thalassemia Research Center, Sari, Iran. It was a narrative review on the current situation of vitamin D deficiency in Iran. Related literature of the studies, in Farsi and English, conducted in the recent decade were explored. Data source of the study was Medline, SID, PubMed, Scopus, Request, Web-of-knowledge, Springer, Ovid, and Google Scholar. Twenty five cross-sectional researches were found regarding vitamin D status in Iran from 2003 to 2013. There was also a Meta-analysis conducted in 2008. The total amount of 25(OH) D3 was measured mostly by radio immune assay (RIA) method. Most of the studies were done on adults. Definition of vitamin D deficiency was based on cut off of the kit in most studies; however in some studies the serum parathyroid hormone (PTH) was measured and cut off for diagnosis based on increased PTH. Maximum rate of sever vitamin D deficiency was reported as 47% in 2011. Vitamin D deficiency is very frequent in Iran. Dealing with a national important nutritional problem is important. One way is to fortify suitable foods or edible products such as milk or cooking oil. Increasing public awareness about the problem and motivating people to do something on their expense is another option. In the current case taking vitamin D supplements on a regular basis, daily, weekly, or any other routines which could be available and cost effective may solve the problem. For people above one year old it is recommended to take 300’000 IU (as intramuscular injection or oral dose) of vitamin D as a starting dose, then 50,000 IU oral dose every three months.

Extramedullary hematopoiesis in the kidney and adrenal are rarely reported in medical literature and are usually found as incidentaloma. It usually occurs in patients with hematologic disorder such as thalassemia. The patient was a 23-year-old Iranian man with beta thalassemia who was admitted with a suprarenal mass. Adrenal mass was detected by ultrasonography and computed tomography. Results of biochemical evaluations were insignificant. The patient underwent right adrenalectomy. Gross and microscopic histopathologic examination demonstrated extramedullary hematopoiesis without any adrenal tissue. To the best of our knowledge, we document the first reported case of adrenal extramedullary hematopoiesis in Iran, which seems to be rare and remarkable.

While thalassemia major (TM) used to be a prevalent genetic disease in the past, however, Phenylketonuria (PKU) is quite rare in spite of consanquiness marriage rate of about 40% in the region. Preventive efforts for TM started >20 years ago but neonatal screening for PKU started since 2007. This is the first report of co-existence of thalassemia and PKU in Middle East and in consideration of the prevalence of each genes, this chance association is a very unusual event. We report a case of having PKU and TM.