Cases reported "Autoimmune Diseases"

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1/9. risperidone in the treatment of choreiform movements and aggressiveness in a child with "PANDAS".

    The acronym 'PANDAS' (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) is used to describe neuropsychiatric symptoms putatively resulting from autoimmune responses to streptococcal infection in vulnerable children. A case of 'PANDAS' is presented to increase physician awareness of this disorder and to document effectiveness of risperidone in chorea and treatment-resistant disruptive behavior associated with this syndrome. To our knowledge, this is the first case report on risperidone in pediatric chorea, although studies on effectiveness of this agent in Tourette's disorders have been previously published.
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2/9. Autoerythrocyte sensitization (Gardner-diamond) syndrome mimicking compartment syndrome.

    We report a case with an unusual manifestation of autoerythrocyte sensitization syndrome (Gardner-diamond syndrome). The patient had the clinical signs of compartment syndrome of the forearm requiring fasciotomy. Dermatological signs identical to Gardner-diamond syndrome should alert the physician to avoid unnecessary fasciotomy, as these patients respond well to psychiatric treatment as in the present case.
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3/9. Clinical holistic medicine: chronic infections and autoimmune diseases.

    The consciousness-based (holistic) medical toolbox might be useful in general practice and in cases of recurrent infections and chronic infection or inflammation. From our clinical experiences, there is hope for improvement from a number of diseases caused by disorders affecting the regulation of the immune system when the physician includes the holistic medical approach. Our scientific understanding of the connection between consciousness and cellular order is still limited. consciousness-based holistic medicine removes (as explained by the holistic process theory of healing) the "blockages" in the tissues of the body and facilitates function and informational exchange of the cells of the body. Many blockages and repressed feelings in an area would imply "noise and disturbances" on the level of intercellular communications, which in turn means major difficulties for the cells of the immune system. For this they are totally dependent on the body information system, which the holistic treatment aims to recover. Processing the blockages increases the coherence of the cells and organism, thus increasing the intercellular flow of information in the area and thus strengthening the immune defense and healing the disease. The area of clinical holistic medicine is going through a rapid development and the toolbox of consciousness-based medicine is available for dealing with many diseases arising from disturbances in the regulation of the immune system. Holistic medicine has yet to be better explained scientifically and our proposed holistic cures have yet to be documented clinically. We invite the medical community to cooperate on this important challenge.
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4/9. A case of autoimmune progesterone dermatitis in an adolescent female.

    BACKGROUND: progesterone-induced dermatitis is a rare disorder. It typically occurs in females due to an autoimmune phenomenon to endogenous progesterone production, but can also be caused by exogenous intake of a synthetic progestin. Here, we present a case of autoimmune progesterone dermatitis (AIPD) seen in an adolescent female. CASE: The patient is a 15-year-old Caucasian female with no significant past medical history and no prior exogenous hormone use, who presented to her primary care physician complaining of cyclic skin eruptions. She noted that her dermatologic symptoms occurred monthly, just prior to her menses. An intradermal skin test using 0.1 cc of progesterone was performed. The patient immediately developed a wheal, confirming the diagnosis of AIPD. The patient was begun on a continuous regimen of an oral contraceptive pill with 30 micrograms of ethinyl estradiol and 0.15 mg of levonorgestrel. The skin eruptions have not returned since the initiation of this therapy. CONCLUSION: Autoimmune progesterone dermatitis manifests via the occurrence of cyclic skin eruptions. women with the disorder commonly present with dermatologic lesions in the luteal phase of the menstrual cycle. diagnosis of AIPD is confirmed by performing a skin allergen test using progesterone. Due to its rarity, AIPD should be considered a diagnosis of exclusion. In cases believed to be due to an endogenous production of progesterone, several methods of therapy have been attempted. The ultimate goal of therapy is the suppression of ovulation, which will prevent endogenous hormone production as progesterone is only produced in ovulatory cycles. Currently, the first-line choice of therapy is a combination oral contraceptive. If this treatment is ineffective, patients have been treated with danazol, gonadotropin releasing hormone analogs, tamoxifen, and oophorectomy with varying success.
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5/9. Open-label study of clarithromycin in patients with undifferentiated connective tissue disease.

    OBJECTIVE: The macrolide family of antibiotics (erythromycin, clarithromycin, and others), have both antimicrobial and immunomodulatory effects. This study explored the effect of clarithromycin on the clinical course of patients with undifferentiated connective tissue disease (UCTD) in a 12-week open-label study. methods: The diagnosis of UCTD was based on symptoms/signs of connective tissue disease, and the presence of 1 or more positive autoimmune disease tests, but with insufficient criteria to make a definitive diagnosis. Screening and monthly follow-up visits over 12 weeks included the following: history and physical examination; concurrent medications; the 68/66 tender/swollen joint count; visual analog scores 0 to 100 mm for patient and physician global assessment of disease activity, and patient pain; antinuclear antibody panel, rheumatoid factor, erythrocyte sedimentation rate, c-reactive protein, and blood chemistry. RESULTS: Seven patients with rheumatic disease were treated with clarithromycin; 6 of 7 had symptomatic relief. Two subjects treated empirically before the decision to perform an open-label study responded favorably. Four of 5 patients who completed the prospective open-label study had mean maximal improvements from baseline of 78, 75, and 79% in patient pain, patient global, and investigator global assessments, respectively. pain relief occurred as early as 1 week. Drug withdrawal with rechallenge in 2 patients resulted in flare followed by recapture of symptomatic relief. CONCLUSIONS: clarithromycin, a macrolide antibiotic, led to clinical improvement in patients with UCTD. Efficacy and safety data support further investigation of macrolide antibiotic use as a primary or adjunctive treatment in various connective tissue diseases.
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6/9. Undiagnosed purpura: a case of autoerythrocyte sensitization syndrome associated with dermatitis artefacta and pseudo-ainhum.

    A 23-year-old young woman presented with recurrent episodes of painful bruising along with linear erosions on the accessible areas of the body of nine years duration with a pseudo-ainhum of her left nipple for the past three months. Her case history included repeated visits to various physicians at different centers and an extensive investigative profile. A diagnosis of autoerythrocyte sensitization was made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any organic cause for her ailment. She was placed on psychiatric management and has remained symptom-free after six months follow-up. The case is reported for its rarity, as well as for the association of autoerythrocyte sensitization syndrome with frank dermatitis artefacta and pseudo-ainhum, which to the best of our knowledge has not yet been reported in the literature.
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7/9. Autoimmune disorders complicating adolescent Hodgkin's disease.

    Four adolescents with Hodgkin's disease also developed autoimmune diseases. There were two idiopathic thrombocytopenic purpura (ITP), one polymyositis, and one scleroderma. The first two patients developed ITP in the absence of a spleen, and with their Hodgkin's disease in remission. The first patient with Hodgkin's disease has been continuously free of cancer for over five years. The second patient was a 17-year-old male whoe Hodgkin's disease recurred, but whose disease was in remission at the time the ITP occurred. The polymyositis occurred in an 18-year-old youth when he was in his initial remission for his Hodgkin's disease, but his disease subsequently recurred two years later. This youth presented with Coombs positive autoimmune hemolytic anemia. The polymyositis did not respond to therapy, and he is left with severe muscle wasting and weakness; however, the polymyositis is now quiescent. The scleroderma occurred in an 18-year-old female who had been continuously free of Hodgkin's disease for eight years. The scleroderma did not respond to drug therapy and she now has moderate skin changes, but remains in continuous remission of her Hodgkin's disease. Although there are a few reports of Hodgkin's disease and concurrent autoimmune disorders, physicians dealing with cancer in adolescents should be aware of this association.
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8/9. cogan syndrome: autoimmune-mediated audiovestibular symptoms and ocular inflammation.

    BACKGROUND: cogan syndrome is an uncommon disorder that presents with symptoms involving the eyes and ears. At its onset, it can mimic many common entities. family physicians should be aware of cogan syndrome because it can be successfully treated if it is recognized early in its course. It is one of the few treatable causes of deafness. methods: medline files were searched from 1982 to the present for "Cogan's syndrome." Additional references were obtained by cross-referencing bibliographies from available articles. RESULTS AND CONCLUSIONS: As first defined in 1945, cogan syndrome includes nonsyphilitic interstitial keratitis and attacks of vertigo, tinnitus, and hearing loss. Although it usually begins with only one symptom, most patients have both auditory and ocular findings within 1 year of the onset. If untreated, most patients become deaf within 36 months. blindness occurs in about 5 percent of patients, but ocular symptoms relapse during a period of years. The disease eventually involves other organs with clinical and pathologic findings that suggest vasculitis. Aortic insufficiency, the most serious complication, develops in 15 percent of patients. The cause of cogan syndrome remains unknown, but several studies suggest an autoimmune-mediated process. Many reports document an improvement in symptoms with immunosuppressive therapy, particularly if started early in the course of the illness. family physicians should include cogan syndrome in their differential diagnosis when a young adult seeks care with audiovestibular symptoms or ocular inflammation.
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9/9. incidence of autoimmune disease in patients after breast reconstruction with silicone gel implants versus autogenous tissue: a preliminary report.

    OBJECTIVE: To test the hypothesis that there is a higher incidence of autoimmune disorders in patients who have undergone breast reconstruction with silicone gel implants rather than autogenous tissue. DESIGN: Prospective study. SETTING: Tertiary referral center dealing exclusively with cancer. patients: All female breast cancer patients who underwent breast reconstruction between January 1986 and March 1992. patients were nonrandomly assigned to breast reconstruction with one of the following four methods: (1) silicone gel implant only, (2) latissimus dorsi flap with implant, (3) latissimus dorsi flap without implant, and (4) transverse rectus abdominis flap. The first two groups made up the implant cohort and the last two groups the autogenous tissue cohort. Selection of reconstructive method was made on clinical grounds and was based on both physician and patient preference. MAIN OUTCOME MEASURES: Documented diagnosis of autoimmune disorder by Board-certified rheumatologist. Results: Three hundred eight implants were used in 250 patients, and 408 reconstructions with tissue were performed on 353 patients. The two groups were similar in age and tumor stage. The two groups contributed 615.8 and 663.4 person-years of follow-up, respectively. One patient from each group (< 0.5%) had a documented occurrence of an autoimmune syndrome requiring therapy. Both cases were considered mild, and after initial low-dose steroid therapy, both patients are now off steroids. CONCLUSION: The incidence of autoimmune disease in mastectomy patients receiving silicone gel implants is not different than in patients who had reconstruction with autogenous tissue.
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