1/4. reflex sympathetic dystrophy arising in a patient with familial mediterranean fever.A 14-year-old girl with familial mediterranean fever (FMF) had had acute attacks of fever, abdominal pain, and arthritis for 4 years. Her last arthritis attack was protracted, leading to reflex sympathetic dystrophy (RSD) in her right lower extremity. Physical therapy along with sympathetic ganglion block and corticosteroid therapy was used for the treatment. To our knowledge, this is the first reported case of RSD arising in a patient with FMF. Early recognition of RSD in FMF patients is important, and physical therapy should be applied along with medical treatment.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/4. Amyloid goiter in familial mediterranean fever (FMF): a clinicopathologic study of 10 cases.FMF amyloidosis is an important etiological factor of end stage renal disease (ESRD) in Mediterranean Countries. Apart from major target organs as cardiovascular, respiratory and gastrointestinal system, endocrine organs can also be involved. We planned to investigate the thyroid involvement in our amyloidosis group. The aim of this study was to determine clinical characteristics of amyloid goiter in FMF patients and the abnormalities of thyroid function, as well as to identify pathologic characteristics. Twenty-two hemodialysis patients (mean age 34.1 /- 14 years, range 17-68) whose ESRD secondary to FMF amyloidosis were evaluated with physical examination, serum levels of thyroid hormones, ultrasound examination of thyroid glands, thyroid syntigraphic studies. goiter was found in 10 patients (4 male, 6 female) having enlarged neck mass (mean age 35 /- 14 years, range 23-64). The serum levels of thyroid hormones and TSH were normal in 4 patients. Other four cases had euthyroid sick syndrome. Only one patient developed tender enlarged neck mass with subacute thyroiditis symptoms and one had primary hyperthyroidism. Ultrasound examination showed; hypoecoic nodules in 6 patients diffuse multinodular enlargement in 4 patients. Thyroid syntigraphic studies revealed hypoactive nodules in 7 patients and hyperactive nodules in 3 patients. After the laboratory tests were completed, in 10 patients diagnosis were made with fine needle aspiration biopsy. Of 10 patients 5 underwent subtotal thyroidectomy. Immunohistochemical evaluation demonstrated the presence of amyloid AA immunoreactivity in all cases. In conclusion fine needle aspiration from the thyroid when enlarged is useful in the diagnosis of suspected amyloidosis, especially since it is a safe, easily performed procedure. With the help of amyloid goiter diagnosis the patient's prognosis on hemodialysis and with renal transplantation can be predicted. Amyloid goiter must be searched in hemodialysis patients especially in Mediterranean Countries.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/4. Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature.Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is a recently described pediatric periodic disease characterized by recurrent febrile episodes associated with head and neck symptoms. The origin of this syndrome is unknown, and it can last for several years. During healthy periods, patients grow normally. The differential diagnosis includes other diseases characterized by periodic fevers, such as familial mediterranean fever, familial Hibernian fever, hyperglobulinemia D syndrome, Behcet's disease, cyclic neutropenia, juvenile rheumatoid arthritis, and several infectious diseases. Many treatments have been used, with various results, including antibiotics, nonsteroidal anti-inflammatory drugs, acetylsalicylic acid, colchicine, antiviral medicines, steroids, cimetidine, and tonsillectomy. We describe 5 new patients affected by PFAPA syndrome who were observed at the Department of Pediatric Otorhinolaryngology, Spedali Civili, Brescia, italy, from November 2000 to August 2001. All children underwent physical examination, bacterial, fungal, and viral cultures, chest radiography, and several laboratory studies. The patients were treated by successful tonsillectomy, and after a mean follow-up of 10 months, no recurrence was observed. An analysis of the literature is also presented with particular emphasis on the differential diagnosis of this rare illness and the results of the different therapeutic options.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/4. PFAPA syndrome mimicking familial mediterranean fever: report of a Turkish child.The PFAPA (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitidis) syndrome is characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. Herein, we present a Turkish child with PFAPA syndrome mimicking familial mediterranean fever because of a rare presentation. A 9-year-old boy was admitted with recurrent fever, aphthous stomatitis, sore throat, headache, and general body pains, lasting 2 to 3 days since 3.5 years of age. He was completely symptom-free between the attacks. He was diagnosed as having familial mediterranean fever according to the clinical findings when he was 6 years of age and colchicum tablet was administrated. Despite colchicines therapy for 8 months, his attacks did not subside; therefore, the drug was discontinued. He had high fever, a painful cervical lymphadenopathy, aphthous stomatitis, and tonsillo-pharyngitis. The patient was then diagnosed as having PFAPA syndrome. He was given a single dose of prednisolone (0.35 mg/kg/dose). His complaints dramatically and completely disappeared 3 h after administration of the drug. During the 8th month of follow-up, a similar febrile attack lasting only 1 day was noted and it was controlled with a single dose of prednisolone (0.5 mg/kg/day). At this writing the patient is in the 12th month of follow-up, and there have been no symptoms after the second attack. In conclusion, our patient shows that PFAPA syndrome can be confused with familial mediterranean fever. We also would like to emphasize that the typical PFAPA syndrome can be easily diagnosed by detailed history-taking and physical findings.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |