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Dental management of patients with hereditary angioedema: report of case
radiated patient. Spec Care Dent 1(4):166-173,1981. 7. Carl, W. Dental m anagem ent of head and neck cancer patients. J Surg Oncol 15(3):265-281, 1980. 8. Murray, C.'G., and others. Radiation necrosis of the mandible: a 10 year study, part I. Factors influenc ing the onset of necrosis. Int J Radiat Oncol Biol Phys 6(5):543-548, 1980. 9. Morrish, R.B., and others. Osteonecrosis in pa tients irradiated for head and neck carcinoma. Cancer 47(8):1980-1983, 1981.
10. Beumer, J., and others. Postradiation dental ex tractions: a review of the literature and a report of 72 - episodes. Head Neck Surg 6(l):581-586, 1983. 11. Bedwinek, J.M., and others. Ostennecrosis in patients treated w ith definitive radiotherapy for squamous cell carcinomas of the oral cavity and nasoand oropharynx. Radiology 119(3):665-667, 1976. 12. Rahn, A.O., and Drone, J.B. Dental aspects of the problems, care, and treatm ent of the irradiated oral cancer patient. JADA 74(5):957-966, 1967.
13. Hayward, J.R., and others. The m anagem ent of teeth related to the treatm ent of oral cancer. CA 19(2):98-106, 1970. 14. D a lits c h , W .W . D e n ta l e x t r a c t i o n in hem ophilia. JADA 21(10):1804-1811, 1934. 15. Niebel, H.N., and others. Removal of teeth from irradiated tissue. J Oral Surg 15(4):313-319, 1957. 16. Gianelly, A.A., and Goldman, H.M. Biologic basis of orthodontics, Philadelphia, Lea & Febiger, 1971, pp 44-111, 119-202.
Dental management of patients with hereditary angioedema: report of case Hans S. Malmstrom, Dr O dont Janet M. Hock, BDS, PhD Collin Sanford, DMD
The causes, diagnostic findings, and management of hereditary angioneurotic edema are reviewed, and a case report that describes dental treatment of a patient with hereditary angioneurotic edema in an outpatient clinic is presented.
T T -M. JL e re d ita ry a n g io e d e m a , first d e scribed by Q uinche in 1882,1 is a rare disease w ith clinical significance for the d e n ta l p ra c titio n e r. D o m in an t in h e r itance of the disease was show n in 1888 by Osier.2 After reviewing the literature, D onaldson3 and Frank and others,4 con cluded that hereditary angioedem a may affect both males and females and diverse ethnic groups. In susceptible patients, he reditary angioedem a may be a serious sequela of traum a associated w ith dental trea tm en t involving th e oral m ucosa, tooth extractions, or oral surgery.4’6 The case report presented in this paper de scribes the m anagem ent and treatm ent of a patient w ith hereditary angioedem a in a dental outpatient clinic. The cardinal sign of h ered itary a n gioedem a is edem a of the extrem ities, face, airw ay , a n d a b d o m e n .4-7' 9 T he characteristic edem a first becomes evi dent during adolescence. However, onset of the disease has been reported in pre school children and infants. W ith the ex ception of abdom inal pain, the edem a is n e ith e r p a in fu l nor p ru ritic .4,7 T h irty m inutes to several hours before the sw ell ing occurs, patients report a tingling or tightness at th e site of the edema. The edem a may last from 4 hours to 1 week.4
The most serious site affected by heredi tary angioedem a is the respiratory tract, w h e re la ry n g e o d e m a m ay be fa ta l. L anderm ann10 reported a 30% m ortality am o n g p a tie n ts w ith h e re d ita ry a n gioedema. Donaldson and Evans11 and Rosen and others12 showed that the onset of heredi tary angioedem a was a result of changes in com plem ent 1 inhibitor activity and suggested that synthesis of the inhibitor was either decreased or deficient. As a consequence, prem ature activation of the com plem ent cascade can occur, resulting in massive, uncontrolled edema of the af fected organ.
Report of case This case report involves a 37-year-old w hite m ale w ith a confirmed history of hereditary angioedem a. His first sym ptom s occurred at the age of 15, in 1962, w hen he had an attack of abdom inal pain. He was adm itted into a hospi tal, and the pain was diagnosed as appendicitis but was not treated. During the next 4 years, the attacks becam e more frequent and severe. He w as hospitalized several times, and finally, in 1978, at the age of 31, during hospitalization for an abdom inal attack, his sym ptom s were diagnosed as hereditary angioedem a. Since 1979, his disease has been m onitored by a physician. Long-term, preventive treatm ent, danazol, 400 mgm/day, has been prescribed. To treat acute attacks, e-am inocaproic acid and dexam ethasone were prescribed. The patient reported that the m edications reduced the se verity and frequency of his attacks. Despite this therapy, he still has approxim ately one attack per month. Since his disease w as diagnosed, a diagnosis of hereditary angioedem a w as con firm ed in his m other, sister, and daughter. The patient has had a 15-year history of alcohol abuse. Since 1982, he has lim ited drinking to weekends. The patient has received dental care on an irregular basis, m ostly because of the difficulty in m anaging the m edical condition. Most of the present restorative work w as done w ith the patient in 1972 under general anesthesia. At his last dental visit, in 1982, the m andibular
right first molar was extracted w ithout com pli cations, using local anesthesia. He also was seen tw o tim es by a den tal h y g ie n ist w ho treated h im successfully using nitrous oxide analgesia. The p atient contacted the health cen te r in A p ril 1983, an d req u e sted com prehensive care. D uring the exam ination, his dental problem s w ere identified as generalized m oderate to localized severe p eriodontitis, re current enam el caries, and defective restora tions. B ecause of lim ited finances, h e w as treated on an outpatien t basis. Prior to the p atien t’s initial visit, he was tak ing danazol as p rescrib ed by th e p a tie n t’s physician. A few hours after the exam ination to evaluate the extent of periodontal disease and caries, an attack of hereditary angioedem a occurred. The p atient w as h ospitalized for 3 days. After discussions w ith th e patient and consultations w ith the physician, th e decision was m ade to continue treatm ent on an outpa tien t basis. T reatm ent w as provided in three sessions. Based on our evaluation of inform a tio n from th e literature, the recom m endations of the p atien t’s physician, an d the inform ed consent of the patient, the follow ing drug reg im en was im plem ented. The p atien t continued to take danazol, 200 mgm tw ice a day. For 3 days before an d 3 days after each dental ap pointm ent, €-am inocaproic acid, 10 gm/day, was prescribed. The evening before each den tal visit, the patient was given tw o u n its of fresh-frozen plasm a by intravenous infusion. At each visit, the patient w as sedated using intravenous adm inistration of diazepam 20 to 30 m gm infused for 3 to 3 Vi hours, and inhala tion of 50% nitrous oxide at a flow rate of 6 L/hr. His vital signs w ere m onitored frequently and regularly. A m edical em ergency care tray, in cluding an intubation set and epinephrine, d a n a z o l, e - a m in o c a p r o ic a c id , d i p h e n hydram ine, and hydrocortisone w ere prepared for each visit. A rrangem ents w ere m ade w ith appropriate m edical personnel to han dle an emergency if one arose. During the first session, m andibular teeth were root planed, am algam restorations were placed in three m andibular teeth, an d a tem po rary crow n w as placed on the m andibular left first m olar, w hich was prepared for a gold crown. During the second session, m axillary teeth w ere treated; treatm ent in clu d ed peri odontal therapy, six com posite restorations in anterior teeth, one am algam restoration, and
Malmstrom—Hock—Sanford : MANAGEMENT OF PATIENTS WITH ANGIOEDEMA ■ 957
extraction of three molars. The gold crow n was placed on the m andibular left first molar. The third session consisted of an evaluation of dis ease control and com pletion of periodontal and restorative treatm ent done during the first tw o sessions. Oral hygiene evaluation and in structions w ere given at the start of each ap pointm ent and were review ed at subsequent health m aintenance visits. After each visit, the patient was observed in the clinic for 4 to 5 hours before being discharged. He continued taking danazol, 400 mgm/day, for 2 days. He reported one m ild attack of abdom inal p ain 2 days after the second session. The p a tie n t’s recovery w as otherw ise uncom plicated. The treatm ent plan includes m onitoring the p atient for disease control at 3-month inter vals, using analgesic sedation w ith nitrous oxide or diazepam (or both), and following prem edication w ith danazol, 400 mgm/day.
reg im en has been ach iev ed by using either danazol or e-aminocaproic acid in com bination w ith transfusion of freshfro z e n p la s m a .5'613,15 D a n a z o l a n d e-am inocaproic acid are used 2 to 3 days before the operation and continued for a few days after the operation. Based on the hypothesis that fresh-frozen plasm a will provide com plem ent 1 inhibitor,16 freshfrozen plasm a should be transfused 12 hours before the operation. Transfusion of fresh-frozen plasm a is not w ithout risk as u r t i c a r i a , i n f e c t i o u s h e p a t i t i s , a n a p h y la c tic shock, and in tra v e n o u s hem olysis may occur.17 However, Frank13 and other researchers5-6' 1518'19 recom m end the use of fresh-frozen plasm a, as, in their experience, patients who receive
Dental management and treatm ent of a patient with hereditary angioedema can be accomplished in an outpatient setting safely and satisfactorily.
D isc u ssio n
plasm a before a surgical or dental proce dure do not have significant angioedem a. Currently, there are no drugs that w ill control acute attacks of hereditary angio edema adequately. The response of the edema to epinephrine, antihistam ines, or glucocorticoids is poor and may result in rapid airway blockage. One experim ental approach has been to treat acute attacks by adm inistering partially purified com plem ent 1 inhibitor.20 This therapy needs further study to confirm its efficacy. Cur rently, depending on the severity of the attack, F rank13 and P ruet and others5 s u g g e s t th a t e p in e p h r in e , d i p h e n h y d ram in e hydrochloride, hy d ro co rti sone, or e-am inocaproic acid could be prescribed. Dental care in an outpatient clinic was selected for the patient, partly because of the need to lim it the cost of treatm ent and partly to im prove the p atien t’s u n d er standing of the rationale and lim itations of dental care and to im prove his m otiva tion to m aintain oral health. Compliance w ith recall visits to prevent progression of dental diseases is probably the most im portant and most difficult com ponent of dental treatm ent for patients w ith he reditary angioedem a, as their lives may be endangered by dental treatment.
M e d ic a l m a n a g e m e n t of h e r e d ita r y angioedem a consists of long-term p re vention therapy, short-term treatm ent to prevent anticipated attacks, and dealing w ith acute attacks.13 Unless the attacks significantly affect life-style, long-term treatm ent should not be used in patients who have less than one attack of heredi tary an g io e d e m a each m o n th .13 C ur rently, androgens and their derivatives, or an tifib rin o ly tic drugs, are u sed for lo n g -te rm p r e v e n tiv e m a n a g e m e n t. H osea an d o th e rs 14 an d F ra n k 13 co n clu d ed that the m ost successful drug available at the tim e of their studies was an attenuated androgen, danazol. In clin ical trials, this drug prevented attacks of hereditary angioedem a in most of the pa tients tested .13'14 Danazol has a relatively low toxicity and is the least m asculiniz ing of available androgens. It is thought that the androgens overcome inhibition of com plem ent 1 by stim ulating effector sites in the liver and inducing synthesis of com plem ent 1 inhibitor. The second major group of drugs used in the long term treatm ent of hereditary angioedem a is th e antifibrinolytic group of w hich e-am inocaproic acid is the m ost com m only used. A lthough these drugs are po tent inhibitors of plasm in, they are con S u m m ary sidered less effective than the androgens Dental m anagem ent and treatm ent of a and potentially m ore toxic.13 In the short-term m anagem ent of h ered patient w ith hereditary angioedem a can itary angioedem a, a successful preventive be accom plished safely and satisfactorily 958 ■ JADA, Vol. I l l , D ecem ber 1985
in an outpatient setting by a dentist in collaboration w ith a physician. However, as acute attacks can occur as a com plica tion of treatm ent, we recom m end that dental treatm ent be provided in a hospital e n v iro n m e n t w ith a d e q u a te m ed ic al emergency resources. «vr»*
Dr. M almstrom was resident, division of family dentistry, School of Dental Medicine, U niversity ol C onnecticut H ealth Center, Farm ington, CT, at the tim e the research for this paper was being done, anc currently is assistant professor, departm ent of com m unity dentistry, Emory University School of Den tistry, 1462 Clifton Rd, NE, Atlanta. Dr. Hock is as sociate professor; and Dr. Sanford is assistant profes sor and director, division of fam ily dentistry, School of Dental Medicine, University of Connecticut Health Center, Farm ington, CT 06032. A ddress requests foi reprints to Dr. Hock.
1. Q uinche, H. Uber Akutes Um schriebene. Auto odem M onatsschr Prakt Dermatol 1:129-131, 1882. 2. Osler, W. Hereditary angioneurotic oedem a. An J Med Sei 95:362-367, 1888. 3. D onaldson, V.H. T h erap y of th e “neurotic edem a.” N Engl J M ed 286:835-836, 1972. 4. Frank, M.M.; Gelfand, J.A.; and Atkinson, J.P H ereditary angioedem a: the clinical syndrom e and it; m anagem ent. Ann Intern Med 84:580-593, 1976. 5. Pruet, C.W., and others. M anagem ent of the air way in patients w ith hereditary angioedem a. Laryn goscope 93(6):749-755, 1983. 6. Mauro, J.V., and others. Hereditary angioneuro tic edem a: clinical m anagem ent and case report JADA 104(5):641-643, 1982. 7. Donaldson, V.H., and Rosen, F.S. Hereditary angioneurotic edema: a clinical survey. Pediatric 37:1017-1027, 1966. 8. Heft, M.W., and Flynn, P.M. Hereditary angio edema: review of literature and dental treatm ent JADA 95(5):986-990, 1977. 9. Ohela, K. H ereditary angioneurotic edema ii Finland. Acta Med Scand 201:415-427, 1977. 10. Landerm an, N.S. H ereditary angioneuroti< edem a. J Allergy 33:316-329, 1962. 11. Donaldson, V.H., and Evans, R.R. A biochem i cal abnorm ality in hereditary angioneurotic edem a Am J Med 35:37-44, 1963. 12. R osen, F .S ., a n d o th e rs. H e re d ita ry an gioneurotic edem a: two genetic variants. Scienci 148:957-958, 1965. 13. F ra n k , M .M . H e re d ita ry a n g io e d e m a . Ir L ichtenstein, L.M., and Fauci, A.S., eds. C urren therapy in allergy and imm unology. St. Louis, C. V Mosby Co, 1983, pp 54-57. 14. Hosea, S.W., and others. Long term therapy o hereditary angioedem a w ith danazol. A nn Interr Med 93:809-812, 1980. 15. H urley, J.E., and Rayden, M.R. Hereditary angio-edem a in relation to dentistry. Br J Oral Surj 16:26-30, 1978. 16. Jaffe, C.J., and others. Hereditary angioedema: the use of fresh frozen plasm a for prophylaxis in pa tients undergoing oral surgery. J Allergy Clin Im m unol 55:386-393, 1975. 17. Miller, R.D., and Brzica, S.M. Blood, blooc com ponent, colloid and autotransfusion therapy. Ii Miller, R.D., ed. A nesthesia, vol 2, chap 28. New York Churchill Livingstone, 1981, pp 885-922. 18. Delfino, J.J., and others. M anagem ent of pa tients w ith hereditary angioneurotic edem a. J Ora Surg 36:890-892, 1978. 19. Albright, B.W., and Taylor, C.G. Hereditary angioneurotic edema: report of a case. J Oral Surj 37:888-890, 1979. 20. Gadek, J.E., and others. Replacem ent therapy in hereditary angioedem a: a successful treatm ent o acute episodes of angioedem a w ith partly purifiec com plem ent 1 inhibitor. N Engl J Med 302:542-546 1980.
10. Beumer, J., and others. Postradiation dental ex tractions: a review of the literature and a report of 72 - episodes. Head Neck Surg 6(l):581-586, 1983. 11. Bedwinek, J.M., and others. Ostennecrosis in patients treated w ith definitive radiotherapy for squamous cell carcinomas of the oral cavity and nasoand oropharynx. Radiology 119(3):665-667, 1976. 12. Rahn, A.O., and Drone, J.B. Dental aspects of the problems, care, and treatm ent of the irradiated oral cancer patient. JADA 74(5):957-966, 1967.
13. Hayward, J.R., and others. The m anagem ent of teeth related to the treatm ent of oral cancer. CA 19(2):98-106, 1970. 14. D a lits c h , W .W . D e n ta l e x t r a c t i o n in hem ophilia. JADA 21(10):1804-1811, 1934. 15. Niebel, H.N., and others. Removal of teeth from irradiated tissue. J Oral Surg 15(4):313-319, 1957. 16. Gianelly, A.A., and Goldman, H.M. Biologic basis of orthodontics, Philadelphia, Lea & Febiger, 1971, pp 44-111, 119-202.
Dental management of patients with hereditary angioedema: report of case Hans S. Malmstrom, Dr O dont Janet M. Hock, BDS, PhD Collin Sanford, DMD
The causes, diagnostic findings, and management of hereditary angioneurotic edema are reviewed, and a case report that describes dental treatment of a patient with hereditary angioneurotic edema in an outpatient clinic is presented.
T T -M. JL e re d ita ry a n g io e d e m a , first d e scribed by Q uinche in 1882,1 is a rare disease w ith clinical significance for the d e n ta l p ra c titio n e r. D o m in an t in h e r itance of the disease was show n in 1888 by Osier.2 After reviewing the literature, D onaldson3 and Frank and others,4 con cluded that hereditary angioedem a may affect both males and females and diverse ethnic groups. In susceptible patients, he reditary angioedem a may be a serious sequela of traum a associated w ith dental trea tm en t involving th e oral m ucosa, tooth extractions, or oral surgery.4’6 The case report presented in this paper de scribes the m anagem ent and treatm ent of a patient w ith hereditary angioedem a in a dental outpatient clinic. The cardinal sign of h ered itary a n gioedem a is edem a of the extrem ities, face, airw ay , a n d a b d o m e n .4-7' 9 T he characteristic edem a first becomes evi dent during adolescence. However, onset of the disease has been reported in pre school children and infants. W ith the ex ception of abdom inal pain, the edem a is n e ith e r p a in fu l nor p ru ritic .4,7 T h irty m inutes to several hours before the sw ell ing occurs, patients report a tingling or tightness at th e site of the edema. The edem a may last from 4 hours to 1 week.4
The most serious site affected by heredi tary angioedem a is the respiratory tract, w h e re la ry n g e o d e m a m ay be fa ta l. L anderm ann10 reported a 30% m ortality am o n g p a tie n ts w ith h e re d ita ry a n gioedema. Donaldson and Evans11 and Rosen and others12 showed that the onset of heredi tary angioedem a was a result of changes in com plem ent 1 inhibitor activity and suggested that synthesis of the inhibitor was either decreased or deficient. As a consequence, prem ature activation of the com plem ent cascade can occur, resulting in massive, uncontrolled edema of the af fected organ.
Report of case This case report involves a 37-year-old w hite m ale w ith a confirmed history of hereditary angioedem a. His first sym ptom s occurred at the age of 15, in 1962, w hen he had an attack of abdom inal pain. He was adm itted into a hospi tal, and the pain was diagnosed as appendicitis but was not treated. During the next 4 years, the attacks becam e more frequent and severe. He w as hospitalized several times, and finally, in 1978, at the age of 31, during hospitalization for an abdom inal attack, his sym ptom s were diagnosed as hereditary angioedem a. Since 1979, his disease has been m onitored by a physician. Long-term, preventive treatm ent, danazol, 400 mgm/day, has been prescribed. To treat acute attacks, e-am inocaproic acid and dexam ethasone were prescribed. The patient reported that the m edications reduced the se verity and frequency of his attacks. Despite this therapy, he still has approxim ately one attack per month. Since his disease w as diagnosed, a diagnosis of hereditary angioedem a w as con firm ed in his m other, sister, and daughter. The patient has had a 15-year history of alcohol abuse. Since 1982, he has lim ited drinking to weekends. The patient has received dental care on an irregular basis, m ostly because of the difficulty in m anaging the m edical condition. Most of the present restorative work w as done w ith the patient in 1972 under general anesthesia. At his last dental visit, in 1982, the m andibular
right first molar was extracted w ithout com pli cations, using local anesthesia. He also was seen tw o tim es by a den tal h y g ie n ist w ho treated h im successfully using nitrous oxide analgesia. The p atient contacted the health cen te r in A p ril 1983, an d req u e sted com prehensive care. D uring the exam ination, his dental problem s w ere identified as generalized m oderate to localized severe p eriodontitis, re current enam el caries, and defective restora tions. B ecause of lim ited finances, h e w as treated on an outpatien t basis. Prior to the p atien t’s initial visit, he was tak ing danazol as p rescrib ed by th e p a tie n t’s physician. A few hours after the exam ination to evaluate the extent of periodontal disease and caries, an attack of hereditary angioedem a occurred. The p atient w as h ospitalized for 3 days. After discussions w ith th e patient and consultations w ith the physician, th e decision was m ade to continue treatm ent on an outpa tien t basis. T reatm ent w as provided in three sessions. Based on our evaluation of inform a tio n from th e literature, the recom m endations of the p atien t’s physician, an d the inform ed consent of the patient, the follow ing drug reg im en was im plem ented. The p atien t continued to take danazol, 200 mgm tw ice a day. For 3 days before an d 3 days after each dental ap pointm ent, €-am inocaproic acid, 10 gm/day, was prescribed. The evening before each den tal visit, the patient was given tw o u n its of fresh-frozen plasm a by intravenous infusion. At each visit, the patient w as sedated using intravenous adm inistration of diazepam 20 to 30 m gm infused for 3 to 3 Vi hours, and inhala tion of 50% nitrous oxide at a flow rate of 6 L/hr. His vital signs w ere m onitored frequently and regularly. A m edical em ergency care tray, in cluding an intubation set and epinephrine, d a n a z o l, e - a m in o c a p r o ic a c id , d i p h e n hydram ine, and hydrocortisone w ere prepared for each visit. A rrangem ents w ere m ade w ith appropriate m edical personnel to han dle an emergency if one arose. During the first session, m andibular teeth were root planed, am algam restorations were placed in three m andibular teeth, an d a tem po rary crow n w as placed on the m andibular left first m olar, w hich was prepared for a gold crown. During the second session, m axillary teeth w ere treated; treatm ent in clu d ed peri odontal therapy, six com posite restorations in anterior teeth, one am algam restoration, and
Malmstrom—Hock—Sanford : MANAGEMENT OF PATIENTS WITH ANGIOEDEMA ■ 957
extraction of three molars. The gold crow n was placed on the m andibular left first molar. The third session consisted of an evaluation of dis ease control and com pletion of periodontal and restorative treatm ent done during the first tw o sessions. Oral hygiene evaluation and in structions w ere given at the start of each ap pointm ent and were review ed at subsequent health m aintenance visits. After each visit, the patient was observed in the clinic for 4 to 5 hours before being discharged. He continued taking danazol, 400 mgm/day, for 2 days. He reported one m ild attack of abdom inal p ain 2 days after the second session. The p a tie n t’s recovery w as otherw ise uncom plicated. The treatm ent plan includes m onitoring the p atient for disease control at 3-month inter vals, using analgesic sedation w ith nitrous oxide or diazepam (or both), and following prem edication w ith danazol, 400 mgm/day.
reg im en has been ach iev ed by using either danazol or e-aminocaproic acid in com bination w ith transfusion of freshfro z e n p la s m a .5'613,15 D a n a z o l a n d e-am inocaproic acid are used 2 to 3 days before the operation and continued for a few days after the operation. Based on the hypothesis that fresh-frozen plasm a will provide com plem ent 1 inhibitor,16 freshfrozen plasm a should be transfused 12 hours before the operation. Transfusion of fresh-frozen plasm a is not w ithout risk as u r t i c a r i a , i n f e c t i o u s h e p a t i t i s , a n a p h y la c tic shock, and in tra v e n o u s hem olysis may occur.17 However, Frank13 and other researchers5-6' 1518'19 recom m end the use of fresh-frozen plasm a, as, in their experience, patients who receive
Dental management and treatm ent of a patient with hereditary angioedema can be accomplished in an outpatient setting safely and satisfactorily.
D isc u ssio n
plasm a before a surgical or dental proce dure do not have significant angioedem a. Currently, there are no drugs that w ill control acute attacks of hereditary angio edema adequately. The response of the edema to epinephrine, antihistam ines, or glucocorticoids is poor and may result in rapid airway blockage. One experim ental approach has been to treat acute attacks by adm inistering partially purified com plem ent 1 inhibitor.20 This therapy needs further study to confirm its efficacy. Cur rently, depending on the severity of the attack, F rank13 and P ruet and others5 s u g g e s t th a t e p in e p h r in e , d i p h e n h y d ram in e hydrochloride, hy d ro co rti sone, or e-am inocaproic acid could be prescribed. Dental care in an outpatient clinic was selected for the patient, partly because of the need to lim it the cost of treatm ent and partly to im prove the p atien t’s u n d er standing of the rationale and lim itations of dental care and to im prove his m otiva tion to m aintain oral health. Compliance w ith recall visits to prevent progression of dental diseases is probably the most im portant and most difficult com ponent of dental treatm ent for patients w ith he reditary angioedem a, as their lives may be endangered by dental treatment.
M e d ic a l m a n a g e m e n t of h e r e d ita r y angioedem a consists of long-term p re vention therapy, short-term treatm ent to prevent anticipated attacks, and dealing w ith acute attacks.13 Unless the attacks significantly affect life-style, long-term treatm ent should not be used in patients who have less than one attack of heredi tary an g io e d e m a each m o n th .13 C ur rently, androgens and their derivatives, or an tifib rin o ly tic drugs, are u sed for lo n g -te rm p r e v e n tiv e m a n a g e m e n t. H osea an d o th e rs 14 an d F ra n k 13 co n clu d ed that the m ost successful drug available at the tim e of their studies was an attenuated androgen, danazol. In clin ical trials, this drug prevented attacks of hereditary angioedem a in most of the pa tients tested .13'14 Danazol has a relatively low toxicity and is the least m asculiniz ing of available androgens. It is thought that the androgens overcome inhibition of com plem ent 1 by stim ulating effector sites in the liver and inducing synthesis of com plem ent 1 inhibitor. The second major group of drugs used in the long term treatm ent of hereditary angioedem a is th e antifibrinolytic group of w hich e-am inocaproic acid is the m ost com m only used. A lthough these drugs are po tent inhibitors of plasm in, they are con S u m m ary sidered less effective than the androgens Dental m anagem ent and treatm ent of a and potentially m ore toxic.13 In the short-term m anagem ent of h ered patient w ith hereditary angioedem a can itary angioedem a, a successful preventive be accom plished safely and satisfactorily 958 ■ JADA, Vol. I l l , D ecem ber 1985
in an outpatient setting by a dentist in collaboration w ith a physician. However, as acute attacks can occur as a com plica tion of treatm ent, we recom m end that dental treatm ent be provided in a hospital e n v iro n m e n t w ith a d e q u a te m ed ic al emergency resources. «vr»*
Dr. M almstrom was resident, division of family dentistry, School of Dental Medicine, U niversity ol C onnecticut H ealth Center, Farm ington, CT, at the tim e the research for this paper was being done, anc currently is assistant professor, departm ent of com m unity dentistry, Emory University School of Den tistry, 1462 Clifton Rd, NE, Atlanta. Dr. Hock is as sociate professor; and Dr. Sanford is assistant profes sor and director, division of fam ily dentistry, School of Dental Medicine, University of Connecticut Health Center, Farm ington, CT 06032. A ddress requests foi reprints to Dr. Hock.
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