• Journal of Veterinary Clinics
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• v.17 no.2
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• pp.443-449
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• 2000

A three-year-old male jindo with generalized skin lesions (including seborrhea, hyperk- eratosis, alopecia, papules, and ecchymoses), pruritus lymph node enlargement, and fever was brought to Veterinary Teaching Hospital, College of Veterinary Medicine, Chungbuk National Uni- versity. There were no laboratory findings for parasites and fungi in the hair and skin But, the com- plete blood counts (CBC) showed leukocytosis and severe cosinophilia, It was suspected to be an inflammatory and allergic dermatitis. Thus, prednisoIone (0.5 mg/kg PO, BID for 1 week) and ampi- cillin (10 mg/kg PO, BID for 1 week given. One week later, pruritus and ecchymoses were reduced. These treatments were repeated for 7 day again. Three months later, the dog was presented again due to the relapse and exacerbation of the clinical signs. The signs were as follows; severe pru- ritus, vesicobullous skin lesions, anorexia, emaciation, lameness, and welling of carpal joints that showed inflammatory skin lesion and draining of synovia-like fluid. The values of WBC counts were returned to normal ranges. In contrast, eosinophilia was still observed. Coombs test for patient RBC and serum were negative. Hypoalbuminemia (2.5g/dl) was shown by serum chemistry. The uri- nalysis revealed and presence of leukocytes. Luxation finding of right radial carpal joint by polyarthritis was shown in radiography of affected joints.Lupus eryhematosus(LE) cells also appeared in peripheral blood and synovial fluid of affected joints. Definitely, antinuclear antibody (ANA) of patient serum using feline peripheral blood mononuclear cells was detected by all immu- nofluorescence. Based on these findings such as sedum ANA-Positive. major signs (skin disease, non- erosive polyarthritis with soft tissue swelling. and proteinuria), minor signs (fever), and LE cell-pos- itive, a diagnosis of systemic lupus crythematosus (SLE) was mad\ulcorner in this dog. The dog with SLE was administered with Pre(2.0 mg/kg PO, BID for first 4 week and then QOD) to inhibit the production of autoantibodies and with ampicillin (10 mg/kg PO, BID for first 4 weeks and then QOD) to prevent the secondary infection. The condition of this dog was monitored every 2 weeks by physical examinations, radiography, CBC, serum chemistry and urinalyais. At 8th week of treat- ment, the state of SLE evaluated by physical examinations and laboratory findings was markedly improved except for proteinura.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.428-431
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• 2010

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.

• Annals of Clinical Neurophysiology
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• v.9 no.1
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• pp.11-15
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• 2007

Background: The term "overlap syndromes" designates a group of diseases in which polymyositis (PM) or dermatomyositis (DM) is associated with some other disorders of connective tissues. The aim of this study was to delineate the clinical features, laboratory findings, and outcome of treatment of "overlap syndromes" Methods: We analyzed the medical records of 16 patients (PM in 10, DM in 6) with well documented "overlap syndromes" between 1997 and 2004. The diagnosis was made when the criteria for two different disorders were fulfilled. Results: All patients were female. Age of onset ranged from 14 to 52 years (mean 29.8 years) with peak incidence in the third and fourth decades. Systemic lupus erythematosus (SLE) was associated in 10, systemic sclerosis in 7, and rheumatoid arthritis in 3 patients. Four of the patients had two different connective tissue diseases simultaneously. The characteristic clinical features were muscle weakness, arthralgia, Raynaud's phenomenon, and myalgia. In laboratory tests, creatine kinase (CK), lactic dehydrogenase (LDH), and transaminases were usually abnormal. Positive antinuclear antibody (ANA), rheumatoid factor (RF), and cryoglobulin were found in 100%, 69%, and 67% of the patients, respectively. Needle electromyography (EMG) showed abnormal findings compatible with myopathy in 15 patients. The pathology of muscle biopsy from 14 patients revealed findings compatible with inflammatory myopathy. Glucocorticoids were administered to 15 patients. The muscle strength improved in all the treated patients, which was well correlated with repeat CK level and EMG findings. Conclusions: The presence of autoantibodies such as ANA, RF, and cryoglobulin in patients with PM or DM highly suggests the possibility of an overlap syndromes. These syndromes reveal a strong female predominance. The myositis associated with them usually shows a good response to glucocorticoids treatment.

• Clinical and Experimental Reproductive Medicine
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• v.22 no.2
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• pp.211-220
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• 1995

Though the endometriosis is not always related with infertility, endometriosis causes infertility in some patients. There are many treatment modalities of infertile patients who have endometriosis. In recent years, Assisted Reproductive Technology(ART) have been widely accepted as being a useful tool for the treatment of infertile endometriotic patients. The objective of this study was to evaluate the outcome of ART in infertile endometriotic patients who have been carried out IVF-ET from Jan, 1992 to Dec, 1994 and to compare the results between COH/IUI and IVF-ET in the patients with endometriosis stage I. Tubal disease only patients were grouped(308 patient, 956 cycles) as a control. Endometriosis group was subdivided into 4 groups according to American Fertility Society classification; endometriosis stage I (45 patients, 61 cycles), stage II (26 patients, 39 cycles), stage III (26 pateitns, 37 cycles), stage IV (33 patients, 50 cycles). The outcomes of IVF-ET in endometriosis patients were as follows; The oocyte recovery rates were significantly lower in stage III, IV endometriosis. In case of stage III endometriosis, the fertilization rate was significantly lower than other stages of endometriosis. Clinical pregnancy rates per cycle were not different between the tubal group(22%) and the endometriosis group(25%). According to endometriosis stage, the implantation rate and clinical pregnancy rate were significantly lower in stage IV (5.6%, 16%) compared with other stages (I; 10.0%, 26%, II;9.8%, 31%, III;12.6%, 32%). It suggests that some factor like autoantibodies may inhibit implantation of embryos in stage IV endometriosis. To evaluate the possibility that simply increasing the number of gametes at the site of fertilization might account for pregnancies attributed to IVF-ET, the authors retrospectively analyzed the outcome of couples undergoing IUI during hMG cycles and CC cycles between 1992 and 1994 in the women with endometriosis stage 1. In case of stage I endometriosis, though the COH/IUI group showed lower FSH level and lesser age profile than IVF-ET group, IUI group has resulted in lower pregnancy rates(19.2%) compared with the IVF-ET group(26.2%). In conclusion, endometriotic infertile patients can get comparable pregnancy rates with the tubal factor infertility patients during IVF-ET program. Moreover even in stage I endometriosis, IVF-ET may be an more effective treatment modality than COH/IUI.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.45-51
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• 2006

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

• Korean Journal of Psychosomatic Medicine
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• v.29 no.2
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• pp.207-212
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• 2021

Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis is a neuroinflammatory disease mediated by autoantibodies to NMDAR. In the initial clinical stages of anti-NMDAR encephalitis, psychiatric symptoms like delusions, perceptual disturbances, and disorganized speech or behaviors are pronounced even without obvious neurological symptoms. Early treatments like immunotherapy and/or tumor removal are central to good clinical outcomes. Hence, it is important to diagnose early anti-NMDAR encephalitis, distinguishing it from mental disorder. In the present case study, the authors described psychiatric symptoms assessed with Positive and Negative Syndrome Scale (PANSS) of Ms. A, a 26-year-old woman, in the early phase of anti-NMDAR encephalitis. We will discuss the characteristic psychopathology of anti-NMDAR encephalitis toward prompt diagnosis and treatment. Ms. A showed a higher negative subscale score than positive one on the PANSS. Compared with mental disorder, negative symptoms and cognitive impairment would be more prominent in the early stage of anti-NMDAR encephalitis. Rituximab and teratoma removal were effective, and quetiapine showed good tolerability. It is recommended to evaluate anti-NMDAR encephalitis when negative symptoms, cognitive impairment, catatonia, changes in consciousness level, and neurological symptoms are observed, especially in young women.

• The Korean Journal of Blood Transfusion
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• v.29 no.3
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• pp.320-327
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• 2018

A 72-year-old man with general weakness visited the outpatient clinic of the hematology department. The patient had been treated under the diagnosis of autoimmune hemolytic anemia for 2 years. His hemoglobin level at the time of the visit was 6.3 g/dL, and a blood transfusion was requested to treat his anemia. The patient's blood type was A, RhD positive. Antibody screening and identification test showed agglutination in all reagent cells with a positive reaction to autologous red blood cells (RBCs). He had a prior transfusion history with three least incompatible RBCs. The patient returned home after receiving one unit of leukoreduced filtered RBC, which was the least incompatible blood in the crossmatching test. After approximately five hours, however, fever, chills, dyspnea, abdominal pain, and hematuria appeared and the patient returned to the emergency room next day after the transfusion. The $anti-Fy^a$ antibody, which was masked by the autoantibody, was identified after autoadsorption using polyethylene glycol. He was diagnosed with an acute hemolytic transfusion reaction due to $anti-Fy^a$ that had not been detected before the transfusion. In this setting, it is necessary to consider the identification of coexisting alloantibodies in patients with autoantibodies and to become more familiar with the method of autoantibody adsorption.

• Journal of Periodontal and Implant Science
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• v.48 no.6
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• pp.347-359
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• 2018

Purpose: Periodontitis and rheumatoid arthritis (RA) share a similar inflammatory pathogenesis. Porphyromonas gingivalis (Pg) can induce anticyclic-citrullinated peptide autoantibodies (anti-CCP antibodies), a key factor in the development of RA. This study aimed at evaluating the relationships between the 2 diseases and identifying the clinical implications thereof, with a focus on periodontal pathogens in Korean adults. Methods: A total of 260 RA patients and 86 age- and sex-matched control patients without arthritis were enrolled in this prospective cross-sectional study. Periodontal indices and the prevalence and amount of periodontal pathogens were compared between the groups. Correlations between periodontal and RA indices were examined, as were correlations between 9 periodontal pathogens and RA indices. Results: The RA group had significantly higher values than the control group for all investigated periodontal indices (P<0.05) except the number of teeth. The gingival index (GI) was correlated with the disease activity score 28 (DAS28) (r=0.125, P=0.049), RA disease duration (r=0.253, P<0.001), erythrocyte sedimentation rate (ESR) (r=0.162, P=0.010), and anti-CCP antibody titer (r=0.205, P=0.004). Probing pocket depth (PPD) was correlated with ESR (r=0.139, P=0.027) and anti-Pg antibody titer (r=0.203, P=0.001). Bleeding on probing (BOP) was correlated with DAS28 (r=0.137, P=0.030), RA disease duration (r=0.202, P=0.001), ESR (r=0.136, P=0.030), anti-Pg antibody titer (r=0.177, P=0.005), and anti-CCP antibody titer (r=0.188, P=0.007). Clinical attachment level (CAL) and periodontitis severity were correlated with anti-Pg antibody titer (the former r=0.201, P=0.002; the latter r=0.175, P=0.006). The quantity of Pg was positively correlated with the serum anti-Pg antibody titer (r=0.148, P=0.020). Conclusions: The GI, BOP, and PPD showed positive relationships with several RA indices. The anti-Pg antibody titer had positive relationships with PPD, BOP, CAL, and periodontitis severity. Thus, increasing values of periodontal indices could be used as a risk indicator of disease development in RA patients, and an increasing anti-Pg antibody titer could be considered as a warning sign in RA patients suffering with periodontitis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.186-196
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• 2004

Purpose: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are available on autoimmune hepatitis in children. The aim of this study was to evaluate the clinical, biochemical, and histological features, and the long-term outcome of autoimmune hepatitis in Korean children. Methods: We reviewed the medical records of 14 children diagnosed as having autoimmune hepatitis at Seoul National University Children's Hospital from 1990 to 2004, and analyzed clinical, biochemical, and histological features, and clinical outcomes. Results: Mean age at diagnosis was 9 years and 11 of the 14 children were female. Six children presented with acute hepatitis-like manifestations. Jaundice and fatigue were the most common symptoms. Other autoimmune diseases accompanied in 6 children. Anti-nuclear antibody was detected in 13 patients and anti-smooth muscle antibody was positive in 8. All 14 patients were type 1 autoimmune hepatitis. The main histologic findings were interface hepatitis, rosette formation, and cirrhosis. Clinical and biochemical features were improved in six patients treated with ursodeoxycholic acid. Eight patients were treated with corticosteroid alone or in combination with azathioprine and five of them are in biochemical remission. Conclusion: Autoimmune hepatitis is an inflammatory liver disease, which has a favorable long-term outcome if it is diagnosed and treated promptly. Therefore, autoimmune hepatitis should be suspected in children with chronic hepatitis of unknown etiology, especially in female patients who show hypergammaglobulinemia or some clinical features of autoimmune disease.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.205-212
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• 2009

Purpose : Chronic urticaria is a disorder characterized by the appearance of wheals for more than 6 weeks; in most cases, the etiology is unknown. This study was aimed to discover the clinical aspects, the etiologic factors, and the course of chronic urticaria. Methods : 51 children who were diagnosed with chronic urticaria in the past 4 years, and who had had follow-ups more than 6 months after diagnosis in the pediatric department of Soonchunhyang University Hospital in Bucheon, were enrolled in the study. The laboratory findings, clinical aspects, and courses were retrospectively investigated by medical record review and telephone interview. Results : The median age of children with chronic urticaria was 4 years (8 months to 16 years) and the ratio of male to female was 1.4:1. Of the total, 39.2% of patients had a history of atopy. Angioedema occurred concurrently with urticaria in 11.8% of patients, and dermographism was seen in 41.2%. Results of thyroid function tests were normal and thyroid autoantibodies were absent in all cases. Regarding etiology, most cases (74.5%) were forms of idiopathic urticaria. Urticaria was induced by physical factors in 19.6% of patients. Open challenge tests revealed that 3 patients were allergic to food additives (glutamate 2, glutamate, and sulfite 1). In this study, most of the patients reported good response after medication of 1st- or 2nd-generation antihistamines alone. Follow-up at 6 months revealed that 70.6% of patients had experienced remission, and 84.8% of children who had follow-up at 1 year presented remission. Conclusion : Chronic urticaria in most patients was idiopathic. Remission occurred within 1 year of diagnosis, in most cases so chronic urticaria in children seems to have good prognosis.