Clinical and Electrophysiologic Features of Peripheral Neuropathy in Churg-Strauss Syndrome

BACKGROUND: Churg-Strauss syndrome (CSS) is characterized by disseminated vasculitis and eosinophilia in patients with asthma. This study was performed to delineate the characteristics of peripheral neuropathy and to identify the factors associated with the severity of neurologic disability. METHODS: We reviewed the medical records of 12 consecutive patients with Churg-Strauss syndrome who had been diagnosed at Samsung Medical Center. The diagnosis of CSS was based on the American college of Rheumatology 1990 criteria. RESULTS: Of the 12 patients, 9 had neurologic involvement. Six had neurologic symptoms as presenting manifestations. All neurologic manifestations were peripheral neuropathy; 5 had multiple mononeuropathy, 2 had distal symmetric polyneuropathy, and 2 had mononeuropathy. Neurologic disability score depended on the rapidity of steroid treatment. CONCLUSIONS: Neurologic involvement is common and develops early in patients with CSS, usually manifesting as peripheral neuropathy. Neurologic disability score showed that early steroid therapy was related with less neurologic disability.

A case of intestinal perforation in Churg-Strauss syndrome patient / 대한내과학회지

Churg-Strauss syndrome (CSS) is a disorder characterized by systemic small-to-medium vessel vasculitis, extravascular granulomas and hypereosinophilia. Gastrointestinal manifestations can occur in 37~62% of CSS patients but intestinal perforation is very rarely demonstrated. We report a case of 34-year-old man with CSS, who presented with intestinal perforation three months after diagnosed as CSS. Emergency laparotomy was performed and jejunal ulcers with perforation were found. Partial jejunectomy was performed. Pathologic findings of the resected specimen were interpretated as mucosal ulcerations which are considered to be caused by ischemia secondary to the vasculitis and eosinophilic infiltration. Since the operation, the patient has been asymptomatic.

Ever wheeze as a predictor of cough variant asthma / 천식및알레르기

BACKGROUND: Objective tests including the methacholine bronchial provocation test and the induced sputum examination are now crucial in the detection of causes of chronic cough. On the other hand, carefully taken history of characteristics, complications, or duration of cough are known not useful in diagnosing causes of chronic cough. OBJECTIVES: To determine whether ever wheeze history can be an important clue in the diagnosis of cough variant asthma. METHODS: Patients with a history of cough lasting more than 3 weeks were enrolled. Patients who were current smokers, who had been receiving an angiotensin-converting enzyme inhibitor, who had basal FEV1 less than 70% of predicted value, who had adventitial sound on auscultation, or who had experienced resting dyspnea were all excluded. We divided the patients into two groups according to the ever wheeze history. The methacholine test (reference value of PC20 = 25mg/ml) and the induced sputum eosinophil percentile (reference value = 3%) were compared between the two groups. All patients were evaluated by an anatomic diagnostic protocol, and the prevalence of cough variant asthma was compared. RESULTS: Three hundred eighty-two patients (138 males and 244 females) with mean age of 48.2 years met the inclusion criteria. The mean duration of cough was 30.4 months (range, 3 weeks to 600 months). Ever wheezers were 154 (40.3%), and non-wheezers were 228 (59.7%). Ever wheeze group showed higher positive methacholine test (37.7% vs. 14.5%, p<0.001) and increased sputum eosinophils (43.5% vs 11.4%, P<.001) compared to non-wheeze group. The prevalence of cough variant asthma was significantly higher in ever wheeze group (33.8% vs. 6.1%, P<.001), and that of eosinophilic bronchitis showed similar result (13.6% vs. 4.8%, P<.01). The positive predictive value of the methacholine test was lower in non-wheeze group compared to ever wheeze group (42.4% vs 86.2%, P<.01). CONCLUSION: Ever wheeze history might be very important in suspecting cough variant asthma as a cause of chronic cough. In chronic cough patients without ever wheeze, prevalence of cough variant asthma and positive predictive value of methacholine test were low.

A case of eosinophilic cellulitis associated with angioedema and eosinophilia / 천식및알레르기

Eosinophilic cellulitis was first described by Wells, which is characterized by cellulitis like skin lesion, eosinophilic infiltration of dermis and subcutaneous fat with characteristic flame figure appearance of chollagen bundles. Peripheral eosinophilia is not necessary for the diagnosis of eosinophilic cellulitis, but association with hypereosinophilic syndrome was reported. Episodic angioedema associated with eosinophilia was first described by Gleich et al. as recurrent attack of angioedema; peripheral eosinophilia; and body weight gain; they described this as separate disease entity rather than variant of the hypereosinophilic syndrome. Both angioedema associated with eosinophilia and eosinophilic cellulitis were reportedly associated with hypereosinophilic syndrome, and we believe both diseases are variants of idiopathic hypereosinophilic syndrome. We experienced a patient with eosinophilic cellulitis who has expressed skin lesion resembling angioedema associated with eosinophilia, and who also has involvement of pleura and gastrointestinal tract. We speculate that this patient represents an overlap syndrome of idiopathic hypereosinophilic syndrome, eosinophilic cellulitis and angioedema associated with eosinophilia.

Correlation between serum prolactin levels and immunocytochemical findings of pituitary adenomas in patients with acromegaly / 대한내과학회지

BACKGROUND: Acromegaly occurs by excessive secretion of growth hormone and more than 99% of cases are caused by a growth hormone-secreting pituitary adenoma. Pituitary adenomas expressing multiple immunoreactivities are common. We assumed that the pituitary adenomas which is immunochemically detected growth hormone and prolactin are responsible for it and reviewed 28 patients with acromagaly to determine the correlation between serum hormonal level and immunocytochemical finding. METHODS: Twenty-eight patients with acromegaly who underwent surgery of pituitary adenoma in Samsung Medical Center from October 1998 to may 2001 were included. Baseline hormonal evaluations and several endocine tests were performed. Immunocytochemical stain was done. RESULTS: According to the extent of hormonal stain, the adenoma was divided into two groups. The adenoma showing immunoreactivity over 50% to growth hormone was 100%, to prolactin was 71.4% and to FSH was 25.0%. The extent of other hormonal stain was less than 20%. There were no significant differences in age, sex, the ratio of macroadenoma and microadenoma, the basal serum GH level, serum IGF-1 level, and the response to TRH, somatostatin and bromocriptine suppression test between the two groups divided by the the extent of prolactin stain. But the serum prolactin level was 55.0+/-63.4 ng/mL, and 19.9+/-12.2 ng/mL each in two groups which was siginificantly increased in the adenoma showing immunoreactivity over 50% to prolactin. CONCLUSION: Acromegaly patients with higher expression of prolactin on immunocytochemical studies showed higher serum prolactin levels and patients with hyperprolactinemia showed higher serum IGF-1.

Occupational asthma induced by cephalosporin precursor / 천식및알레르기

A 41-year-old male pharmaceutical worker developed dyspnea and wheezing 10 years after beginning the work. He had handled powder of cephalosporin precursor, 7-aminocephalosporanic acid (7-ACA). He had suffered from dyspnea, chest tightness and wheezing in the workplace for the last three years. Recently, these symptoms were aggravated. His pulmonary function test showed severe obstructive pattern and PC(20) was 0.06 mg/ml on MBPT(methacholine bronchial provocation test). Skin prick test and intradermal test showed positive reactions to ceftriaxone, cefazolin, etc. During the skin test, he suffered from an asthmatic attack and anaphylactic shock. Bronchial provocation test with cefotiam elicited a dual asthmatic reaction. We presumed that IgE-mediated mechanism may be mainly involved in the pathogenesis of cephalosporin induced occupational asthma, in addition to other immunologic or non-immunologic mechanisms. Further studies will be necessary to investigate the pathogenesis of this disease.

A Case of Prosthetic Valve Endocarditis Caused by Listeria monocytogenes / 감염

Listerial endocarditis is rare disease with about 60 reported cases in the literatures. Although the clinical and laboratory features of listerial endocarditis are similar to those of subacute bacterial endocarditis caused by other pathogens, the incidence of complications and mortality rates are high. Early diagnosis, adequate treatment, and timely surgery are important for the better outcome. We report a case of a 62 year-old male with prosthetic valve endocarditis caused by Listeria monocytogenes, who was successfully treated with antibiotics and surgery (aortic valve and mitral valve re-replacement).

A Case of Malignant External Otitis with Pichia ohmeri / 감염

Pichia ohmeri is a rare fungal pathogen for human infection. To date, only 5 cases of P. ohmeri infection have been reported including fungemia, disseminated infection and peritonitis. We report the first case of malignant external otitis and mastoid osteomyelitis caused by P. ohmeri. The patient had a long history of diabetes, hypertension and arrrhythmia. The patient was admitted to the hospital because of otorrhea, otalgia and hearing difficulty for 3 months. Internal auditory canal MRI showed osteomyelitis of left mastoid bone and skull base. Culture of ear discharge and bone biopsy specimen grew P. ohmeri. The patient had been treated with amphotericin-B followed by fluconazole.

Successful treatment of refractory hemolytic uremic syndrome with intravenous gamma-immunoglobulin therapy / 대한내과학회지

The hemolytic uremic syndrome (HUS) is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure with or without a clinically apparent etiology. The conventional treatment of choice is plasmapheresis as a first-line therapy. Most patients respond to plasmapheresis whereas some patients are refractory to the therapy. The second-line therapy is not well established although various therapies such as steroid, vincristine, intravenous immunoglobulin have been suggested. The intravenous immunoglobulin therapy in refractory hemolytic-uremic syndrome have rarely been successful in complete remission. We report a case of refractory HUS in a 48 year-old man who developed hemolytic anemia, thrombocytopenia, acute renal failure and ischemic retinopathy. The patient was refractory to plasmapheresis as a first-line therapy. The patient received intravenous immunoglobulin therapy as a second-line therapy after 8 days of plasmapheresis, which subsequently resulted in a complete remission of refractory HUS. The complete remission using immunoglobulin in HUS has not been previously reported in Korea.