Informatics Publishing Limited

Sabita Upreti ¹, Mrudula Giri ², Arjan Aryal ²

Affiliations
1 Pharmacology Depatment, Krupanidhi College of Pharmacy, Bengaluru, IN
2 Department of Pharmacology and Pharmacy Practice, Krupanidhi College of Pharmacy,Bengaluru, IN

Abstract

Beta-thalassemia is a genetic haematological disorders characterized by abnormalities in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals.

The prevalence of this disorder is estimated to be 1 in /100,000 of total world population. It is most common in people of African, Mediterranean and Southeast Asian descent. Major complications associated with Beta thalassemia are hemolytic anemia, poor body growth and skeletal abnormalities during infancy as a result regular lifelong blood transfusion is required. The minor complication is the carrier of genetic trait for beta thalassemia and do not experience major health problem except mild anaemia.

Diagnosis of thalassemia is based on hematologic and molecular genetic testing. Differential diagnosis is usually upfront but may include genetic sideroblastic anemias, congenital dyserythropoietic anemias, and other conditions with high levels of Haemoglobin - F (such as juvenile myelomonocytic leukemia and aplastic anemia). Regular RBC transfusions, iron chelation ,management of complications of iron overdose are some treatment approaches for thalassemia major.

While, Bone marrow transplantation is the definite cure currently available. Individuals with thalassemia intermedia may require splenectomy, folic acid supplementation, treatment of extramedullary erythropoietic masses and leg ulcers, prevention and therapy of thromboembolic events.

Prognosis for individuals with beta-thalassemia has improved substantially in the last 20 years. However, cardiac disease remains the main cause of death in patients with iron overload. Induced pluripotent stem cells, prenatal diagnosis, oral sodium phenyl butyrate therapy,drugs such as Deferoxamine , Deferasirox (ICL670), Deferiprone which are clinical trials are new approaches to treat beta thalassemia.

Keywords

Beta Thalassemia, Bone Marrow Transplant, Induced Pluripotent Stem Cells.

Full Text

   

Homesh Choudhary ¹, Rajeswari Ramasamy ¹, Arjan Aryal ¹, Abino Denny ¹, Kalyani Kattakam ¹

Affiliations
1 Department of Pharmacy Practice, Krupanidhi College of Pharmacy, Bangalore-35, IN

Abstract

Guillain-Barre syndrome is an autoimmune disorder which is caused by the body's immune system resulting in the damaging of peripheral nervous system. However immune system starts to destroy the myelin sheath (which surrounds the axon of many peripheral nerve), and will not able to transmit the signal effectively after getting damaged that may result in painful sensation leading to muscle weakness. GBS can also probably be an outcome of viral (Cytomegalovirus) or bacterial infection (Campylobacter jejuni). The virus and bacteria are likely to change the nature of the nervous system in such a way that the immune system cannot recognize the cells as its own and can allow some immune cells (lymphocytes and macrophages) to attack myelin and leading to destruction of the myelin sheath.

Here a case of GBS in 50 year old women with past medical history of paresthesia was reported .According to medical literature the incidence of GBS is very rare syndrome. The patient was diagnosed with GBS with lumbar puncture that indicated albuminocytogical dissociation, a characteristic of GBS. The patient was managed with anticoagulant, anticonvulsant, anti-arrhythmic, digestive enzyme, nitrogen mustard- alkylating agent , chemoprotectant, antiemetic, PPI, gastro protectative agent along with plasmapheresis. The importance of correct diagnosis by the physician and the subsequent management is reviewed.

Keywords

Guillain Barre Syndrome, Plasmapheresis, Cyclophosphamide, Intravenous Immunoglobulin.

Full Text