Managmentof Thallasemia patient of dental surgery

Management of Thalassemic

Patient of Dental Surgery

DR SYED MUHAMMAD ALIMS ORAL SURGERY RESIDENT

UNDER SUPERVISION OF Prof DR ,SYEDMAHMOOD HAIDERVICE PRINCIPAL HEAD OF DENTISTRY ,HEAD OF OMFS

DEPARTMENT KARACHI MEDICAL AND DENTAL COLLEGE KARACHI PAKISTAN

[email protected]

THALASSEMIA

Word Thalassemia is derived from the Greek word Thalass ("sea") and -emia ("blood").

The Disease is very common in peoples living in Mediterranean geographical region, but found all over the world including Pakistan.

It is a autosomal, single gene recessive blood disease It is characterized by reduced or absent amounts of

hemoglobin (Cooley & Lee 1925; Rund & Rachmilewitz, 2005; Verma et al., 2011)

KINDS OF THALASSEMIA

There are two basic groups of thalassemia disorders:

α thalassemia β thalassemia.

ALPHA (α) THALASSEMIA

The α-thalassemia involve the genes HBA1 and HBA2, inherited in a Mendalian fashion.

There are two gene loci and so four alleles

It is also connected to the deletion of the chromosome 16.

α Thalassemia result in decreased alpha-globin production, therefore fewer alpha-globin chains are produced, resulting in an excess of β chains in adults and excess γ chains in newborns.

BETA (β)THALASSEMIA

Beta(β)thalassemia are due to mutations in the HBB gene on chromosome 11, inherited in an autosomal recessive fashion.

The severity of the Disease depends on the nature of the mutation.

Mutations are characterized as either βo or β

Thalassemia major :- No formation of β chains, the most severe form of β thalassemia.

Management before the Thalassemic Disease occur Management after the Thalassemic Disease occur

Management of Thalassemia

Control or stop consanguineous marriages among the carrier

Stop birth of Thalassemic child

Management before the Thalassemic Disease occur

Thalassemia is a inherited autosomal recessive blood disease

Population screening identification of carrier and genetic counseling are necessary for controlling the disease

Prenatal diagnosis can be done by relative rate of globin biosynthesis by fetoscopy and fetal blood sampling around 16 to18 week of intrauterine life or by analysis of fetal DNA by ultrasound guided chorionic villous biopsy at around 8-9 week of gestation or fetal aminocytes by aminocentesis if both parent are carriers

Antenatal diagnosis and genetic counseling

The following method are being used to managed and minimize the harmful effect

Bone marrow transplantation Neocyte Transfusion Genetic engineering Blood transfusion

Management after the Disease

Test of Thalassemia.

A complete blood count (CBC) reveals anemiaRed blood cells will appear small and abnormally shaped when looked at under a microscope..A test called hemoglobin electrophoresis shows the presence of an abnormal form of hemoglobin.A test called mutational analysis can help detect alpha thalassemia that cannot be seen with hemoglobin electrophoresis.

When a patient of thalassemia came for Dental Treatment, it is necessary that maximum information about patient has to be taken.

Detailed history Examination (systemic and Oral

examination Medical Diagnosis of Thalassemia Life expectancy Condition of Patient Coagulation and vitamin k Deficiency

Management of Dental Surgery

The medical diagnosis of the specific type of thalassemia is very necessary for best treatment.

planning of dental treatment differs according to whether the patient is of thalassemia major or intermediate and is always depend upon the present medical condition of the patient and future life expectancy.

In patients with thalassemia intermediate, more complex treatments can be considered.

Management of Dental Surgery

• characteristics with thalassemia intermediate and other less severe variants like thalassemia minor patients are clearly the possible oro facial deformities and malocclusions problems in patients may be dealt with surgically.

• However, such treatments are not indicated in patients with thalassemia major, particularly if blood transfusion and chelator therapy has not been properly carried out

Thalassemia patients can present generally mild liver alternations., In extreme cases may involve strongly diminished coagulation factor production, intense vitamin K absorption deficiencies due to continuous antibiotic coverage provided, or previously undetected and progressing hepatitis.

It is also important to exercise caution when using hepatotoxic drugs of common use in dental practiceThe liver function test and coagulation tests(PT,APTT,INR) must be carried out before treatment. In thalassemia , several transfusions was given to secure coagulation, especially as a result of the administered platelet fraction, coagulation tests must be also carried out before starting dental treatment

• If thalassemia patient is diabetic than strictly preventive measures in dental treatment has to be observed. periodontitis, gingivitis and splenectomy etc., makes the situation more dangerous Cutando (2002)

• If thalassemia patient with cardiac problem come for simple dental treatments, and be careful for, pericarditis and congestive heart failure as iron over load due to serial transfusion causes deposition of extra iron in heart Cutando (2002)

•

• It should also be in consideration that dental treatment of thalassemia patients drugs that have depressive effects upon the central nervous system and hence also upon respiration, should be used with caution.

• It is also important to taken into consideration that the liver physiology and renal excretory capacity of these patients are depressed.

Therefore depressant drugs should be avoided or used with care

• The absence of hypersplenism , without leucopenia (a reduction in the number of white cells in the blood) or thrombocytopenia (deficiency of platelets in the blood) facilities dental treatment of both thalassemia major and thalassemia intermediate.

• In contrast the presence of hypersplenism with leucopenia and

thrombocytopenia requires the provision of antibiotic coverage and platelet concentrates before dental procedures can be carried out.

• In the case of splenectomy patients, the risk of sepsis (the presence in tissues of harmful bacteria and their toxins, typically through infection of a wound) is high and the oral cavity must be prevented from constituting a source of bacterial spread.

• This may require extreme care and caution in dental treatments The increase in platelet count resulting from splenectomy implies a greater risk of thrombosis (local coagulation or clotting of the blood in a part of the circulatory system. );

• The administration of antiplatelet drugs in such cases requires monitoring of bleeding time or consultation with a hematologist

• Antibiotics prophylaxis

• Cutando (2002) Terezhalmy and Hall (198) Suggested oral anti microbial regimen for aspleenic (refers to the absence of normal spleen function) patients undergoing dental or oral surgical treatments.

• Penicillin V 2 G., 30 minutes- 2 hour before procedure, then 500 mg. post operative q6th for 8 doses.

• Erythromycin 1G., 30 minutes to 2hour before procedure, then 500 mg post operative q6th for 8 doses.

• If the patients are receiving oral deferiprone, the side effects of the drug should be taken into account, since they can affect already impair organs such as the liver and immune system.

In Pakistan no work oral and maxillofacial manifestation has been carried out But from 2012 Dr, Syed Muhammad Ali Started to work 0n Clinical and Radiological studies of oral and maxillofacial manifestation of Thalassemia Under The Supervision of Prof, Dr Syed Mahmood Haider.This is the First step in this field from Pakistan

Oral and Maxillofacial Manifestation

1. Cutando, A. Thalassemia and their dental implications. 2000 . Medicina Oral. 7 : 36-45.

2. Terezhalmy, G. T., E.H., Hall.. 1984.The asplenic patient : A consideration for antimicrobial prophylaxis. Oral Surg. 57 (1) :114-117. 

Ali, S.M., S. M. Haider, A., Sufyan S. G.Hassan,  S. H. Jaffry.2016. Oral and maxillofacial manifestation in 50 β –thalassemic patients from Karachi- a clinical study. Pakistan Oral & Dental Journal 36: (2): 179-183

HAIDER,S.M., and S.M.,ALI. (2015), Review of clinical and radiological studies of oral and maxillo-facial manifestation in thalassemia from Pakistan FUUAST J. BIOL., 5(1): 175-178

References

Federal Govt,of Pakistan has passed a bill in National Assembly,2013 that screening of thalassemia test is compulsory before marriage Governor of Sindh in 2014 also passed an ordinance in this connection.