ROLE tonsillectomy in PFAPA syndrome
Kevin K. Wong, MD; Jane C. Finlay, MD, FRCPC; J. Paul Moxham, MD, FRCSC
Objective: To test the efficacy (potency) tonsillectomy in improving symptoms and preventing episode recurrence in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis).
Design: Retrospective case series.
Background: a tertiary care hospital of children.
Patients: Patients treated in teaching hospitals in Vancouver, British Columbia, Canada between 2000 and 2004 with a diagnosis of PFAPA syndrome or patients who have been diagnosed earlier in the consultation.
Intervention: Tonsillectomy.
Main Outcome Measurements: Resolution of symptoms in the 3rd, 12th, and 24th after tonsillectomy.
Results: Eight of the nine patients achieved complete remission within 3 months. The rest, the frequency of episodes of recurrence was reduced from every two weeks to once every 3 to 4 months. This patient ultimately had resolution of symptoms at 2 years after tonsillectomy. There are no complications caused by tonsillectomy.
Conclusion: Tonsillectomy is a good treatment for patients with PFAPA syndrome.
PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) was first described in 1987 by Marshall et al. This clinical syndrome usually affects children less than 5 years. Periodic fevers (temperature> 39 ° C) lasts 3 to 6 days and occur at intervals of 3 to 6 weeks. In addition aphtosa stomatitis, pharyngitis and cervical adenitis, less common symptoms may be found, such as malaise, headache, abdominal pain, vomiting, hepatosplenomegaly, and arthralgia. Usually improves the situation of children in between episodes of recurrence. The condition of an average of 4.5 years.
The exact cause of PFAPA syndrome remains unknown. Causes of infection (bacterial and viral) is the cause of most can be removed by culture, serology and skin tests. Theory of autoimmune and rheumatologic disorders are also not agreed. Plus also no geographic or ethnic predilection for patients. Diagnosis of clinical circumstances because there is no definitive laboratory test that can yield positive results in the PFAPA syndrome. During an acute episode, WBC and ESR has consistently increased.
PFAPA syndrome treatment is based on case series or retrospective review of medical. Treatment is usually given oral kortikoseroid. In one study found that there was a 76% improvement in symptoms on corticosteroids for patients with PFAPA syndrome. However, prednisolone can not prevent further episodes and short intervals antarepisode usually results in some patients. Cimetidine is also used to prevent relapse and reduce the severity of the case, although keberhasilaanya still limited. Several case reports have been published in the last decade is to explain patients PFAPA syndrome. Based on these limited data, tonsillectomy may improve symptoms. Herein, has described 9 patients with PFAPA syndrome who ditonsilektomi.
METHOD
We have conducted a retrospective case series analysis on all patients admitted to the British Columbia Children's Hospital between January 1 2000-31 December 2004 with the diagnosis of PFAPA syndrome or patients who have been previously diagnosed at the initial consultation. Patients included in the above criteria tonsillectomy performed by 1 surgeon. The patient's parents agreed and granted penjelasanmengenai procedure to be performed. All patients were observed at least 24 months after tonsillectomy. Do records of disease recurrence in the interval between episodes (if there is no complete remission), and some complications from the surgical procedure. Ethics approval for this study was obtained from the study of ethics University of British Columbia.
RESULTS
Total patients with PFAPA syndrome is 9 patients, 5 men and 4 women. The mean age of 4.1 years (range 3-5 years of age). The mean fever indicated in patients was 4.1 weeks and occurs within 2-6 weeks of the onset of illness. No patients who received previous treatment. The nine patients minmal observed 24 months after tonsillectomy. Obtained complete remission in 8 of 9 patients in 3 months after tonsillectomy. The patient's symptoms usually disappear at 24 months postoperatively. No complications (minor or major) caused by tonsillectomy.
Opinion
PFAPA syndrome symptoms, particularly fever and pharyngitis observed with the background of otolaryngology. Marshal et al noted that periodic fever was first explained at the beginning of the 1940s, but new PFAPA syndrome is found at the end of the second decade. As a result, the introduction of PFAPA syndrome cases also experienced misdiagnosis as a syndrome or other disease symptoms and signs is almost the same. For sindom PFAPA diagnosis, a physician must have knowledge and a clear understanding of the differential diagnosis of PFAPA syndrome, ie cyclic neutropenia, fever hereditary and Behcet's disease. These diseases should be ruled out before the establishment of the diagnosis of PFAPA syndrome correctly. How to make the diagnosis sidrom PFAPA is the exclusion dagnosis way.
Cases of cyclic neutropenia is less common than PFAPA syndrome, but the two conditions are indistinguishable. At cyclic neutropenia, the episode raised approximately every 21 days (range 14-35 days) and are often associated with aphthous ulcers, gingivitis, cervical lymphadenopathy, and fever. Neutropenia occurs due to oscillation neutrophil production in the bone marrow. During the period of neutropenia, generally decreased PMN <200 cells / microliters for 3-5 days but it can be returned to normal levels quickly. During the symptomatic period, the number of PMN may have on the level of the norm. So as to establish the diagnosis of blood needed hiting checks twice a week for 6 weeks or at least 2 weeks before the expected febrile episode. On histopathologic examination of bone maturation obtained mielosit stop on the stage. Treatment is usually given granulocyte colony stimulating factor.
Hereditary periodic fever is generally classified in familial Mediterranean fever (FMF), with hyper-IgD syndrome and Hibernian fever. FMF is autoimflamasi disease characterized by periodic attacks of fever and serositis. FMF is an autosomal recessive disease that occurs mainly in the Turkish, Armenian, Arab, and Jewish descent. The incidence of febrile attacks associated with the severity of abdominal pain, arthritis, and pleurisy or chest pain that is characterized by an increased acute phase reactants. FMF diagnosis can be considered in individuals with different ethnic backgrounds who have experienced episodes of febrile illness. Colchicine merupaka primary treatment, because it can control the attack and prevent progression to amloidosis.
Same with PFAPA syndrome, hyper-IgD syndrome usually first arises at a very young age (median onset age: 6 months) and includes periodic fever. Characteristics fever is for 3-7 days, and the attacks usually occur every 4-8 weeks. Berkuang frequency and severity of attacks in line with the increasing age of the patient. However, in contrast to the fever episode PFAPA syndrome. PFAPA syndrome, fever occurs in the whole life of the patient. Other symptoms include chills, lymphadenopathy, abdominal pain, vomiting, diarrhea and headache. During the attack, the acute response phase that occurs leukocytosis, Neutrophilia, and increased ESR. Increased serum IgD and IgA (> 100U / mL) is a distinctive sign, but not all there. It takes a retrial during this period. Causes of this disease are believed MVK gene mutation that encodes mevalonate kinase. The main treatment is only with supportive therapy. Recent studies are testing the drugs etanercep, TNF receptor Fc fusion protein, which is expected to generate a combined effective results.
Behcet's disease is a disease multiorgan characterized by oral apthae and at least 2 of the following: (1) genital apthae, (2) synovitis, (3) posterior uveitis, (4) vasculitis pustulasn cutaneous, (5) meningoencephalitis, (6) ulcer recurrence genital, and (7) uveitis in the absence of inflammatory bowel disease and collagen vascular disease. Same with PFAPA syndrome, there is no specific laboratory tests, but the clinical krieia can be helpful in diagnosis. Behccet disease usually no febrile episodes, and oral ulcers are usually more severe than PFAPA syndrome. Characteristics of these symptoms can help in memmbedakannya with PFAPA syndrome.
The literature that uses tonslektomi in the treatment of patients with PFAPA syndrome showed explanation article 6 of 41 cases. A substantial decrease episodes of PFAPA syndrome occurs more than 84% of patients (37 of 44). Most authors who examine tonsillectomy in PFAPA syndrome concluded that tonsillectomy is one treatment option that can be considered. Hereditary problems with the current literature solely based on retrospective case series study (EBM fifth level). Leong et al found PFAPA syndrome patients may have recurrent infections and will complete resolution after the procedure, tonsillectomy. Paa our case, 89% of patients experienced resolution of symptoms (8 of 9) similar to the literature. There is one patient in our series of cases which did not experience resolution of symptoms completely, but reduced the frequency of relapse episodes. Just after 2 years after tonsillectomy, these patients experienced resolution of symptoms perfectly. Is this resolution is due to the direct influence of tonsillectomy or for healing itself naturally disease, is still unknown.
Although the PFAPA syndrome can eventually heal itself, many authors recommend the treatment to be done properly at the time of diagnosis. Medical management (such as steroids and cimetidine) are the most widely prescribed treatment. Tonsillectomy is still not considered in the flow of treatment, but both medical and surgical treatment, both demonstrated success in meresolusi symptoms. Current management support the delivery of steroid treatment. A wide variety of oral prednisone dose has been proposed, with a dose of 1-2 mg / kg single doses is the most common dose is given. Another approach to expand doses for 7 days or replace potency steroid (eg, prednisone given every day administration of 2 mg / kg / day and betamethasone 0.3 mg / kg / day on other days). Two kai cimetidine dose of 150 mg daily for 6 months pral yelah also used successfully. The H2 antagonist cimetidine is meninhibisi chemotaxis and activation of T cells Thomas and Edwards found 8 of 28 patients treated with cimetidine remission of symptoms perfectly.
NSAID use showed results that failed to satisfy in controlling the symptoms of PFAPA syndrome. Acetaminophen and ibuprofen reduce fever as much as 6% and 33% of patients (respectively), but when the drug's effect is lost once the fever again. Other medical drugs such as antibiotics, acyclovir, and colchicine have minimal effect in relieving symptoms. Complications administration of a single dose of steroids is very rare in children, naumn risks that arise must be described in oang parents.
Tonsillectomy is a surgical option that can improve the symptoms of patients with PFAPA syndrome. Based on our knowledge, no studies using neoadjuvant or adjuvant medical treatment for tonsillectomy in patients with PFAPA syndrome. A definite role that the tonsillectomy role in resolution of symptoms remains unclear, but the syndrome may be caused by a general immune response in tonsil parenchyma. There is no difference in the appearance of the patient is done or not done adennoidektomi on tonsillectomy. However, adenoidectomy alone can not meresolusi symptoms. In our study, no complications arise. Based on the literature, we did not encounter complications were reported on the actions of tonsillectomy in patients with PFAPA syndrome. No reason was stated that the complications of tonsillectomy in PFAPA syndrome is higher than in other patients.
In conclusion, the PFAPA syndrome is a condition that is not fair and pendiagnosisnya way is with a diagnosis of exclusion. In addition to tonsillectomy, the other main therapy is steroid therapy. Treatment options that can be used is prednisone, cimetidine, or tonsillectomy who have demonstrated success in reducing or eliminating the symptoms completely. PFAPA syndrome treatment is based on the theory that the cause of PFAPA syndrome is a dysregulation of the immune response. Based on this theory, if the deviation of immune response is reduced, then the symptoms will disappear. We get good observations with tonsillectomy. There are 8 of 9 patients in our study who showed complete remission of their symptoms after tonsillectomy. The remaining patients experienced a decrease in the frequency of attacks is farcical and experienced resolution of symptoms after 24 months.
Because PFAPA syndrome is a relatively newly recognized in the clinic, it takes more research was done to determine the maximum treatment. Based on our experience, we found that the children who qualify for surgery, tonsillectomy is a good treatment option for PFAPA syndrome.
Kevin K. Wong, MD; Jane C. Finlay, MD, FRCPC; J. Paul Moxham, MD, FRCSC
Objective: To test the efficacy (potency) tonsillectomy in improving symptoms and preventing episode recurrence in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis).
Design: Retrospective case series.
Background: a tertiary care hospital of children.
Patients: Patients treated in teaching hospitals in Vancouver, British Columbia, Canada between 2000 and 2004 with a diagnosis of PFAPA syndrome or patients who have been diagnosed earlier in the consultation.
Intervention: Tonsillectomy.
Main Outcome Measurements: Resolution of symptoms in the 3rd, 12th, and 24th after tonsillectomy.
Results: Eight of the nine patients achieved complete remission within 3 months. The rest, the frequency of episodes of recurrence was reduced from every two weeks to once every 3 to 4 months. This patient ultimately had resolution of symptoms at 2 years after tonsillectomy. There are no complications caused by tonsillectomy.
Conclusion: Tonsillectomy is a good treatment for patients with PFAPA syndrome.
PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) was first described in 1987 by Marshall et al. This clinical syndrome usually affects children less than 5 years. Periodic fevers (temperature> 39 ° C) lasts 3 to 6 days and occur at intervals of 3 to 6 weeks. In addition aphtosa stomatitis, pharyngitis and cervical adenitis, less common symptoms may be found, such as malaise, headache, abdominal pain, vomiting, hepatosplenomegaly, and arthralgia. Usually improves the situation of children in between episodes of recurrence. The condition of an average of 4.5 years.
The exact cause of PFAPA syndrome remains unknown. Causes of infection (bacterial and viral) is the cause of most can be removed by culture, serology and skin tests. Theory of autoimmune and rheumatologic disorders are also not agreed. Plus also no geographic or ethnic predilection for patients. Diagnosis of clinical circumstances because there is no definitive laboratory test that can yield positive results in the PFAPA syndrome. During an acute episode, WBC and ESR has consistently increased.
PFAPA syndrome treatment is based on case series or retrospective review of medical. Treatment is usually given oral kortikoseroid. In one study found that there was a 76% improvement in symptoms on corticosteroids for patients with PFAPA syndrome. However, prednisolone can not prevent further episodes and short intervals antarepisode usually results in some patients. Cimetidine is also used to prevent relapse and reduce the severity of the case, although keberhasilaanya still limited. Several case reports have been published in the last decade is to explain patients PFAPA syndrome. Based on these limited data, tonsillectomy may improve symptoms. Herein, has described 9 patients with PFAPA syndrome who ditonsilektomi.
METHOD
We have conducted a retrospective case series analysis on all patients admitted to the British Columbia Children's Hospital between January 1 2000-31 December 2004 with the diagnosis of PFAPA syndrome or patients who have been previously diagnosed at the initial consultation. Patients included in the above criteria tonsillectomy performed by 1 surgeon. The patient's parents agreed and granted penjelasanmengenai procedure to be performed. All patients were observed at least 24 months after tonsillectomy. Do records of disease recurrence in the interval between episodes (if there is no complete remission), and some complications from the surgical procedure. Ethics approval for this study was obtained from the study of ethics University of British Columbia.
RESULTS
Total patients with PFAPA syndrome is 9 patients, 5 men and 4 women. The mean age of 4.1 years (range 3-5 years of age). The mean fever indicated in patients was 4.1 weeks and occurs within 2-6 weeks of the onset of illness. No patients who received previous treatment. The nine patients minmal observed 24 months after tonsillectomy. Obtained complete remission in 8 of 9 patients in 3 months after tonsillectomy. The patient's symptoms usually disappear at 24 months postoperatively. No complications (minor or major) caused by tonsillectomy.
Opinion
PFAPA syndrome symptoms, particularly fever and pharyngitis observed with the background of otolaryngology. Marshal et al noted that periodic fever was first explained at the beginning of the 1940s, but new PFAPA syndrome is found at the end of the second decade. As a result, the introduction of PFAPA syndrome cases also experienced misdiagnosis as a syndrome or other disease symptoms and signs is almost the same. For sindom PFAPA diagnosis, a physician must have knowledge and a clear understanding of the differential diagnosis of PFAPA syndrome, ie cyclic neutropenia, fever hereditary and Behcet's disease. These diseases should be ruled out before the establishment of the diagnosis of PFAPA syndrome correctly. How to make the diagnosis sidrom PFAPA is the exclusion dagnosis way.
Cases of cyclic neutropenia is less common than PFAPA syndrome, but the two conditions are indistinguishable. At cyclic neutropenia, the episode raised approximately every 21 days (range 14-35 days) and are often associated with aphthous ulcers, gingivitis, cervical lymphadenopathy, and fever. Neutropenia occurs due to oscillation neutrophil production in the bone marrow. During the period of neutropenia, generally decreased PMN <200 cells / microliters for 3-5 days but it can be returned to normal levels quickly. During the symptomatic period, the number of PMN may have on the level of the norm. So as to establish the diagnosis of blood needed hiting checks twice a week for 6 weeks or at least 2 weeks before the expected febrile episode. On histopathologic examination of bone maturation obtained mielosit stop on the stage. Treatment is usually given granulocyte colony stimulating factor.
Hereditary periodic fever is generally classified in familial Mediterranean fever (FMF), with hyper-IgD syndrome and Hibernian fever. FMF is autoimflamasi disease characterized by periodic attacks of fever and serositis. FMF is an autosomal recessive disease that occurs mainly in the Turkish, Armenian, Arab, and Jewish descent. The incidence of febrile attacks associated with the severity of abdominal pain, arthritis, and pleurisy or chest pain that is characterized by an increased acute phase reactants. FMF diagnosis can be considered in individuals with different ethnic backgrounds who have experienced episodes of febrile illness. Colchicine merupaka primary treatment, because it can control the attack and prevent progression to amloidosis.
Same with PFAPA syndrome, hyper-IgD syndrome usually first arises at a very young age (median onset age: 6 months) and includes periodic fever. Characteristics fever is for 3-7 days, and the attacks usually occur every 4-8 weeks. Berkuang frequency and severity of attacks in line with the increasing age of the patient. However, in contrast to the fever episode PFAPA syndrome. PFAPA syndrome, fever occurs in the whole life of the patient. Other symptoms include chills, lymphadenopathy, abdominal pain, vomiting, diarrhea and headache. During the attack, the acute response phase that occurs leukocytosis, Neutrophilia, and increased ESR. Increased serum IgD and IgA (> 100U / mL) is a distinctive sign, but not all there. It takes a retrial during this period. Causes of this disease are believed MVK gene mutation that encodes mevalonate kinase. The main treatment is only with supportive therapy. Recent studies are testing the drugs etanercep, TNF receptor Fc fusion protein, which is expected to generate a combined effective results.
Behcet's disease is a disease multiorgan characterized by oral apthae and at least 2 of the following: (1) genital apthae, (2) synovitis, (3) posterior uveitis, (4) vasculitis pustulasn cutaneous, (5) meningoencephalitis, (6) ulcer recurrence genital, and (7) uveitis in the absence of inflammatory bowel disease and collagen vascular disease. Same with PFAPA syndrome, there is no specific laboratory tests, but the clinical krieia can be helpful in diagnosis. Behccet disease usually no febrile episodes, and oral ulcers are usually more severe than PFAPA syndrome. Characteristics of these symptoms can help in memmbedakannya with PFAPA syndrome.
The literature that uses tonslektomi in the treatment of patients with PFAPA syndrome showed explanation article 6 of 41 cases. A substantial decrease episodes of PFAPA syndrome occurs more than 84% of patients (37 of 44). Most authors who examine tonsillectomy in PFAPA syndrome concluded that tonsillectomy is one treatment option that can be considered. Hereditary problems with the current literature solely based on retrospective case series study (EBM fifth level). Leong et al found PFAPA syndrome patients may have recurrent infections and will complete resolution after the procedure, tonsillectomy. Paa our case, 89% of patients experienced resolution of symptoms (8 of 9) similar to the literature. There is one patient in our series of cases which did not experience resolution of symptoms completely, but reduced the frequency of relapse episodes. Just after 2 years after tonsillectomy, these patients experienced resolution of symptoms perfectly. Is this resolution is due to the direct influence of tonsillectomy or for healing itself naturally disease, is still unknown.
Although the PFAPA syndrome can eventually heal itself, many authors recommend the treatment to be done properly at the time of diagnosis. Medical management (such as steroids and cimetidine) are the most widely prescribed treatment. Tonsillectomy is still not considered in the flow of treatment, but both medical and surgical treatment, both demonstrated success in meresolusi symptoms. Current management support the delivery of steroid treatment. A wide variety of oral prednisone dose has been proposed, with a dose of 1-2 mg / kg single doses is the most common dose is given. Another approach to expand doses for 7 days or replace potency steroid (eg, prednisone given every day administration of 2 mg / kg / day and betamethasone 0.3 mg / kg / day on other days). Two kai cimetidine dose of 150 mg daily for 6 months pral yelah also used successfully. The H2 antagonist cimetidine is meninhibisi chemotaxis and activation of T cells Thomas and Edwards found 8 of 28 patients treated with cimetidine remission of symptoms perfectly.
NSAID use showed results that failed to satisfy in controlling the symptoms of PFAPA syndrome. Acetaminophen and ibuprofen reduce fever as much as 6% and 33% of patients (respectively), but when the drug's effect is lost once the fever again. Other medical drugs such as antibiotics, acyclovir, and colchicine have minimal effect in relieving symptoms. Complications administration of a single dose of steroids is very rare in children, naumn risks that arise must be described in oang parents.
Tonsillectomy is a surgical option that can improve the symptoms of patients with PFAPA syndrome. Based on our knowledge, no studies using neoadjuvant or adjuvant medical treatment for tonsillectomy in patients with PFAPA syndrome. A definite role that the tonsillectomy role in resolution of symptoms remains unclear, but the syndrome may be caused by a general immune response in tonsil parenchyma. There is no difference in the appearance of the patient is done or not done adennoidektomi on tonsillectomy. However, adenoidectomy alone can not meresolusi symptoms. In our study, no complications arise. Based on the literature, we did not encounter complications were reported on the actions of tonsillectomy in patients with PFAPA syndrome. No reason was stated that the complications of tonsillectomy in PFAPA syndrome is higher than in other patients.
In conclusion, the PFAPA syndrome is a condition that is not fair and pendiagnosisnya way is with a diagnosis of exclusion. In addition to tonsillectomy, the other main therapy is steroid therapy. Treatment options that can be used is prednisone, cimetidine, or tonsillectomy who have demonstrated success in reducing or eliminating the symptoms completely. PFAPA syndrome treatment is based on the theory that the cause of PFAPA syndrome is a dysregulation of the immune response. Based on this theory, if the deviation of immune response is reduced, then the symptoms will disappear. We get good observations with tonsillectomy. There are 8 of 9 patients in our study who showed complete remission of their symptoms after tonsillectomy. The remaining patients experienced a decrease in the frequency of attacks is farcical and experienced resolution of symptoms after 24 months.
Because PFAPA syndrome is a relatively newly recognized in the clinic, it takes more research was done to determine the maximum treatment. Based on our experience, we found that the children who qualify for surgery, tonsillectomy is a good treatment option for PFAPA syndrome.
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