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ABSTRACT
Medically compromised patients are occasionally dealt with in dentistry. Sometimes patients with
bleeding and clotting disorders may also be encountered in our day to day practice. Hemophilia
being one of those clotting disorder which is commonly seen in males whereas females are carriers of
the disease. The management of such patients in various specialties of dentistry is discussed in this
article.
Keywords: Hemophilia, Tranexamic Acid, Factor VIII
INTRODUCTION
Hemophilia was seen in European royalty and thus
is sometimes known as the royal disease. Queen
Victoria passed the mutation to her son Leopold. The
first written account of hemophilia occurred in the 2nd
century in the Babylonian Talmud. In 1803, Dr. John
Conrad Otto recognized that the disorder was
hereditary and that it affected mostly males and was
passed down by healthy females. 1 The term
hemophilia is derived from the term
hemorrhaphilia which was used in a description of
the condition written by Friedrich Hopff in 1828.
Hemophilia A is the most common and best-known
clotting defect, with a prevalence of about 5 per 100
000 of the population. It is about 10 times as common
as hemophilia B except in some Asians, where
frequencies are almost equal. Inherited as a sex-linked
reces-sive trait, hemophilia affects males.2
Close cooperation is needed between patients
dentist and physician for such cases. So as to manage
these patients education of parents and the patients
Corresponding author:
Sachin Mittal
Senior Lecturer
Department of Oral Medicine & Radiology, Shree
Bankey Bihari Dental College & Research Centre,
Ghaziabad, Uttar Pradesh - 201302,
E-mail: sachin2627@yahoo.co.in
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Orthodontics
There is no contraindication to the movement of
teeth in hemophilia. However, there must be no sharp
edges to appliances, wires etc., which might traumatize
the mucosa.3
Minor surgery
Endotracheal intubation for general anesthe-sia
may cause bleeding from nasal trauma and is
dangerous in unprepared patients, but since
replacement therapy has to be given for the surgical
procedure, intubation can be carried out. An oral latex
cuffed endotracheal tube is recommended to minimize
trauma to the nasal and trachea lining.6
A Factor VIII level of between 50 and 75 per cent is
required for dental extractions. AHF may also need to
be given postoperatively but many patients can be
managed with antifibrinolytic agents given during the
subsequent 10 days. If oral bleeding recurs
postoperatively, Factor VIII must be given. Some
advise the administration of a further single dose of
Factor VIII as a routine on the fourth or fifth
postoperative day. However, this should be
unnecessary if adequate Factor VIII has been given
preoperatively. Antifibrinolytics significantly reduce
Factor VIII requirements.5
Tranexamic acid (Cyklokapron) is used in a dose
of 1 g (30 mg/kg) orally, four times daily starting 24
hours preoperatively. Tranexamic acid used topically
significantly reduces bleeding. 10 ml of a 5 per cent
solution used as a mouth rinse for 2 minutes, four times
daily for 7 days, is recom-mended.6
Desmopressin (deamino-8-D arginine vasopressin:
DDAVP) is a synthetic analogue of vasopressin which
induces the release of Factor VIIIC, von Willebrands
factor (vWF) and tissue plasminogen activator (tPA)
from storage sites in endothelium. Given as an
intra-venous infusion (0.3-0.5 g/kg just before
surgery, and repeated 12 hourly if necessary for up to
4 days), desmopressin can temporarily correct the
haemostatic defect in mild hemophilia.7
It is now avail-able for subcutaneous or intranasal
use when doses of 300 mg appear as effective as 0.2
mg/kg i.v. As desmopressin also causes release of
plasminogen activator, tranexamic acid should also be
given.7
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Prevention of infection
Antimicrobials such as oral penicillin V 250 mg four
times daily should be given postoperatively for a full
course of 7 days to reduce the risk of secondary
haemorrhage. Infection also appears to induce
fibrinolysis. Postoperatively, a diet of cold liquid and
minced solids should be taken for up to 10 days. Care
should be taken to detect hematoma formation which
may manifest itself by swelling, dysphagia or
hoarseness. The patency of the airway must always
be ensured.7
Major surgery
Before major surgery the patient is assessed by
haemostatic screening (AFTT, PT, and platelet count),
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