|Year : 2011 | Volume
| Issue : 1 | Page : 46-49
Cellulitis on face in a patient with congenital afibrinogenemia
GD Chandan1, AG Annaji2, S Bhatnagar3, U Mohandas1, P Dave1
1 Department of Pedodontics and Preventive Dentistry, VS Dental College and Hospital, Bangalore, India
2 Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bangalore, India
3 Department of Pedodontics and Preventive Dentistry, Jodhpur Dental College, General Hospital, Jodhpur, India
|Date of Web Publication||23-Apr-2011|
G D Chandan
Department of Pedodontics and Preventive Dentistry, VS Dental College and Hospital, VV Puram, KR Road, Bangalore, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Congenital afibrinogenemia is a rare coagulation disorder, with an estimated prevalence of 1 : 1,000,000, characterized by a complete absence to reduced level of circulating fibrinogen. This article presents a case of congenital afibrinogenemia, which presented as cellulitis on the face.
Keywords: Afibrinogenemia, cellulitis, preventive dentistry
|How to cite this article:|
Chandan G D, Annaji A G, Bhatnagar S, Mohandas U, Dave P. Cellulitis on face in a patient with congenital afibrinogenemia. J Indian Soc Pedod Prev Dent 2011;29:46-9
|How to cite this URL:|
Chandan G D, Annaji A G, Bhatnagar S, Mohandas U, Dave P. Cellulitis on face in a patient with congenital afibrinogenemia. J Indian Soc Pedod Prev Dent [serial online] 2011 [cited 2020 Feb 29];29:46-9. Available from: http://www.jisppd.com/text.asp?2011/29/1/46/79933
| Introduction|| |
Fibrinogen or Factor I, is a glycoprotein circulating in the blood in a concentration of 200 - 400 mg/dl, and its half life is two to four days. Fibrinogen is a 340 kDa hexamer, made up of two identical trimers consisting of one alpha, one beta, one gamma chain.  Disorders of fibrinogen (Factor I) are very rare with a prevalence of 1 in 1,000,000.  These disorders are of two types:
Type I (Quantitative) - Afibrinogenemia or Hypofibrinogenemia, and Type II (Qualitative) - Dysfibrinogenemia.
Afibrinogenemia or Hypofibrinogenemia is characterized by a complete absence or reduced level of fibrinogen in the blood. Dysfibrinogenemia is characterized by a normal level, but an altered function of Fibrinogen. The type I disorder (afibrinogenemia or hypofibrinogenemia) is an autosomal recessive disorder, which occurs due to null mutation (deletion, splicing or nonsense mutation) , of either of the three fibrinogens, namely; Alpha FGA(OMIM + 134820), Beta FGB(*134830), or Gamma FGG(*134850), located on chromosome 4q28. ,, Afibrinogenemia occurs in the homozygous state, while hypofibrinogenemia in heterozygous state.  Prevalence of rare disorders is high in cases of consanguinity, therefore, registered cases of such disorders are more in countries like Iran, with high consanguinity, as compared to Europe. ,
Afibrinogenemia is associated with bleeding tendencies and recurrent episodes of spontaneous or traumatic bleeding from the umbilical cord, mucosal surfaces, or GI bleeding, which can be life threatening at times. , The fibrinogen level is less than 100 mg/dl in blood. 
As conversion of fibrinogen to fibrin is the important final step in the coagulation cascade [Graph 1],[Additional file 1] deficiency or absence of fibrinogen will prolong the entire coagulation test including prothrombin time (PT), activated partial thromboplastin time (APTT), Thrombin time, and Reptilase time. 
Routine dental procedures like administering local anesthesia for a nerve block or the extraction of teeth in such patients may sometimes lead to hematoma or uncontrolled bleeding. The management of such complications can be beyond the scope of a normal dental office setup. The purpose of this article is to describe a case of congenital afibrinogenemia and stress the importance of preventive dentistry in children with such disorders.
| Case Report|| |
A 14-year-old female patient reported to the Outpatient Department (OPD) of the Pediatric Dentistry, (Indira Gandhi Institute of Child Health, Bangalore, India.) with a swelling in the lower left region of the face since one week [[Figure 1]a and b]. The patient gave a history of fever and bleeding from the gums since one week, and hemetemesis since one day. The patient was a known case of congenital afibrinogenemia, born of consanguineous marriage. The patient was diagnosed with congenital afibrinogenemia following an episode of uncontrolled bleeding from the umbilical cord. The patient had four siblings; the third sibling had Insulin Dependent Diabetes Mellitus (IDDM).
The extraoral swelling extended from the corner of the mouth to the angle of the mandible, extending into the submandibular region. On intraoral examination, the swelling was seen to extend from the mesial of the second premolar to the pterygomandibular raphe, obliterating the buccal sulcus lower left quadrant. The pericoronal flap over erupting the second permanent molar was inflamed [Figure 2]. There was pus drainage from the gingival sulcus of the second molar. The samples were collected and sent for culture sensitivity. Food debris and calculus were present in the oral cavity.
As the patient had limited mouth opening, an orthopantomograph (OPG) was used for radiographic evaluation. The OPG showed no pathological radiolucencies or any sign of rarefaction or bone loss [Figure 3]. After correlating the clinical and radiographic features the diagnosis of cellulitis of face (secondary to pericoronitis) was made. The patient was admitted to the IGICH (Indira Gandhi Institute of Child Health, Bangalore, India.)
A detailed blood investigation was done. On the first day, hemoglobin was 8 mg/dl. Serum fibrinogen was 54.5 mg/dl (normal values 200 - 350 mg/dl). A coagulation test showed PT was above two minutes (control 15 seconds), APTT was above two minutes. Two units of Fresh frozen plasma were transfused along with packed cells in order to correct the reduced hemoglobin level. Intravenous (IV) Augmentin 625 mg t.i.d and Metrogyl 200 mg t.i.d were administered. On the second day, the blood examination was repeated and hemoglobin was 8.1 gm/dl. The coagulation test showed, PT of 18 seconds and APTT of 45 seconds. IV antibiotics were continued along with fluids and electrolyte. After a week, once the patient was hemodynamically stable, she was referred to the Department of Pedodontics and Preventive Dentistry, V. S. Dental College and Hospital, Bangalore, India.
After taking the pediatrician's consent, careful supragingival ultrasonic scaling was done for the patient, followed by irrigation of the pericoronal flap with Povidone iodine (5% w/v) and the patient was advised to start chlorhexidine mouth rinse (0.2% thrice daily). The patient was recalled after five days. On the second appointment the swelling had reduced considerably [Figure 4] and the mouth opening was normal, so a careful ultrasonic scaling was again done along with subgingival scaling, with hand scalers, followed by fluoride application (APF gel 1.23%). Currently the patient is on a routine follow-up and appropriate preventive measures are taken to maintain the child's oral health to the optimum.
| Discussion|| |
Rarely does a dentist come across a patient with unknown fibrinogen disorders, in other words afibrinogenemia patients are usually diagnosed early in childhood, in the neonatal period, due to uncontrolled bleeding from the umbilical cord or GI bleeding. 
However, the most important aspect of management of dental problems in children with such severe disorders is oral health education and motivation of the patient and parent. It is important to educate the parent regarding the severity of such disorders; they should be registered with the hemophilic society, and should be aware of the medical centers that can provide emergency care, when required. Also, the parents should be advised to report the disorder to the doctor or dentist before starting a new treatment.
A preventive dental program should be tailored, which should include; written material explaining about oral hygiene techniques and home care measures, diet counseling, oral prophylaxis, and fluoride application, and restorative procedures, if needed. For surgical or invasive procedures, a physician's consent should be taken beforehand. A coagulation test should be done. If the coagulation test is prolonged and fibrinogen levels are below 100 mg/dl, prophylactic replacement with Plasma Derived Fibrinogen Concentrate, Cryoprecipitate or Fresh Frozen Plasma (FFP) to maintain levels of fibrinogen level above 100 mg/dl should be considered before surgical or invasive dental procedures. This level should be maintained till the wound healing is complete.  Patients receiving FFP should receive Hepatitis B vaccine or virus inactivated FFP should be used. ,,, The half-life of fibrinogen is two to four days, so treatment should be planned accordingly as recurrent replacement therapy has its complications, which include thromboembolic complications and activation of inhibitors. ,
Antifibrinolytics are useful in conjunction with fibrinogen replacement for the treatment of mucosal bleeding, particularly bleeding involving the dental procedures. Inhibition of local fibrinolysis allows maintenance of the clot and decreases the frequency of rebleeding. Epsilon aminocaproic acid (50 - 60 mg / kg every four to six hours) and tranexamic acid (20 - 25 mg / kg every 8 to 12 hours) can be administered orally or intravenously. 
| Acknowledgments|| |
Indira Gandhi Institute of Child Health
| References|| |
|1.||Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost 2006;4:2115-29. |
|2.||Mannucci PM, Duga S, Peyvandi F: Recessively inherited coagulation disorders. Blood 2004;104:1243 . |
|3.||Duga S, Asselta R, Santagostino E, Zeinali S, Simonic T, Malcovati M, et al. Missense mutations in the human beta fibrinogen gene cause congenital afibrinogenemia by impairing fibrinogen secretion. Blood 2000;95:1336-41. |
|4.||Mosesson MW. Fibrinogen gamma chain functions. J Thromb Haemost 2003;1:231-8. |
|5.||Asselta R, Duga S, Spena S, Santagostino E, Peyvandi F, Piseddu G, et al.Congenital afibrinogenemia: Mutations leading to premature termination codons in fibrinogen Aa-chain gene are not associated with the decay of the mutant mRNAs. Blood 2001;98:3685-92. |
|6.||Neerman-Arbez M. The molecular basis of inherited afibrinogenaemia. Thromb Haemost 2001;86:154-63. |
|7.||Asselta R, Duga S, Simonic T, Malcovati M, Santagostino E, Giangrande PL, et al. Afibrinogenemia: First identification of a splicing mutation in the fibrinogen gamma chain gene leading to a major gamma chain truncation. Blood 2000;96: 2496-500. |
|8.||Corrigan JJ Jr: Blood diseases of infancy and childhood. In: Miller DR, Bachner RL. 6th ed. St. Louis: CV Mosby Company; 1990. p. 866. |
|9.||Peyvandi F, Duga S, Akhavan S, Mannucci PM. Rare coagulation deficiencies. Haemophilia 2002;8:308-21. |
|10.||Acharya SS, Coughlin A, Dimichele DM, North American Rare Bleeding Disorder Study Group. Rare Bleeding Disorder Registry: Deficiencies of factors II, V,VII, X, XIII, fibrinogen and dysfibrinogenemias. J Thromb Haemost 2004;2:248-56. |
|11.||Acharya SS, Dimichele DM. Dimichele. Rare inherited disorders of fibrinogen. Haemophilia 2008;14:1151-8. |
|12.||Kreuz W, Meili E, Peter-Salonen K, Haertel S, Devay J, Krzensk U, et al. Efficacy and tolerability of a pasteurised human fibrinogen concentrate in patients with congenital fibrinogen deficiency. Transfus Apher Sci 2005;32:247-53. |
|13.||Santagostino E, Mancuso ME, Morfini M, Schiavoni M, Tagliaferri A, Barillari G, et al. Solvent/detergent plasma for prevention of bleeding in recessively inherited coagulation disorders: Dosing, pharmacokinetics and clinical efficacy. Haematologica 2006;91:634-9. |
|14.||Klein HG, Dodd RY, Dzik WH, Luban NL, Ness PM, Pisciotto P, et al. Current status of solvent/detergent-treated frozen plasma.Transfusion 1998;38:102-7. |
|15.||Riggert J, Humpe A, Legler TJ, Wolf C, Simson G, Köhler M. Filtration of methylene blue-photooxidized plasma: Influence on coagulation and cellular contamination. Transfusion. 2001;41:82-6. |
|16.||Kreuz W, Meili E, Peter-Salonen K, Dobrkovská A, Devay J, Haertel S, et al. Pharmacokinetic properties of a pasteurised fibrinogen concentrate. Transfus Apher Sci 2005;32:239-46. |
|17.||Ra_anani P, Levi Y, Varon D, Gitel S, Martinowitz U. Congenital afibrinogenemia with bleeding, bone cysts and antibodies to fibrinogen. Harefuah 1991;121:291-3. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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