The polyglandular autoimmune syndrome designates the dysfunction of endocrine and nonendocrine system involving two or more organs on the basis of an autoimmune mechanism. The autoimmune nature of these diseases has been based on the presence of lymphocytic infiltration in the affected gland, organ specific autoantibodies in the serum, cellular immune defects and an association with the HLA DR/DQ genes or immune response genes. This syndrome is usually classified into three classes and their etiology or pathogenesis is still not completely understood. A 28-year-old woman developed vitiligo and insulin dependent diabetes mellitus during the treatment of Graves' disease with antithyroid drug. She had a tendency of spontaneous ketonemia and serum c-peptide levels were low(0.21, 0.16ng/mL: fasting and glucagon stimulated). Thyrotrophin binding inhibitor immunoglobulin and pancreas iIslet cell cytoplasmic antibody were positive. We report here a case of polyglandular autoimmune syndrome, type III manifesting Graves' disease, vitiligo, and insulin dependent diabetes mellitus.
Adult ; Autoantibodies ; Bone Diseases ; C-Peptide ; Cytoplasm ; Diabetes Mellitus ; Fasting ; Female ; Genes, MHC Class II ; Giant Cells ; Glucagon ; Granuloma ; Graves Disease ; Humans ; Immunoglobulins ; Insulin ; Ketosis ; Pancreas ; Pedigree ; Thyrotropin ; Vitiligo

BACKGROUND: Pulmonary aspergilloma usually results from the ingrowth of the colonized Aspergillus in the damaged bronchial tree, pulmonary cyst, or cavities of patients with underlying lung diseases. We analyzed the clinical features, diagnostic methods, and managements of 36 patients with pulmonary aspergilloma. METHODS: From February 1988 to February 2000, medical records of 36 patients who were diagnosed as pulmonary aspergilloma at Chung Ang university hospital were reviewed retrospectively. RESULTS: The mean age (mean+/-SD) was 53.3+/-11.8 years, The male to female ratio was 2.36:1. The most frequent symptom was hemoptysis which occured in 24 patients (65%). The most common underlying disease was pulmonary tuberculosis (81%). The upper lobe was most commonly involved area. In 9 patients, chest CT was taken with prone position and 7 of them showed movability of the fungus ball. Eleven patients showed positive test for precipitin antibody of Aspergillus species. As for the treatment, 20 patients underwent surgical resection. Post-operative complications were reported in 7 cases. The post-operative mortality was 5.6% (2/36). CONCLUSION: Pulmonary aspergillomas usually develop in patients with underlying respiratory diseases. Resectional lung surgery is considered to be the mainstay of therapy for pulmonary aspergilloma. However, operation is associated with significant complication rate and even deaths. Therefore, it is necessary to develop a reasonable criteria for the selection of suitable candidates with aspergilloma for the surgery.
Aspergillus ; Colon ; Female ; Fungi ; Hemoptysis ; Humans ; Lung ; Lung Diseases ; Male ; Medical Records ; Mortality ; Prone Position ; Retrospective Studies ; Tomography, X-Ray Computed ; Tuberculosis ; Tuberculosis, Pulmonary

BACKGROUND: Pulmonary aspergilloma usually results from the ingrowth of colonized Aspergillus from a damaged bronchial tree, a pulmonary cyst, or from the cavities of patients with underlying lung diseases. In the present study, we analyzed the clinical features, diagnostic methods, and managements of 36 patients with pulmonary aspergilloma. METHODS: Thirty-six patients were diagnosed as having pulmonary aspergilloma at Chung-Ang University Hospital between February 1988 and February 2000. Their medical records were reviewed retrospectively. RESULTS: The age of patients (median +/- SD) was 53.3 +/- 11.8 years, the male to female ratio was 2.36: 1, and the most frequent symptom was hemoptysis, which occurred in 24 patients (65%). The most common underlying disease was pulmonary tuberculosis (81%), and the upper lobes of both lungs were the most frequently involved sites. Nine patients received a chest CT in the prone position and seven of these showed a movable fungus ball. Eleven patients were positive for the precipitin antibody to A. fumigatus. Twenty patients underwent surgical resection, and post-operative complications were reported in seven cases. The post-operative mortality was 5.6% (2/36). CONCLUSION: Pulmonary aspergilloma usually develops in the patients with underlying lung diseases. Resectional lung surgery is considered the mainstay of therapy for pulmonary aspergilloma. However, this operation is associated with significant complications and death in some cases. Therefore, it is necessary to develop reasonable criteria for selection of candidates for such surgery.
Adult ; Aged ; Aspergillosis/complications/*diagnosis/therapy ; Bronchiectasis/complications ; Diagnosis, Differential ; Female ; Forced Expiratory Volume ; Hemoptysis/etiology ; Human ; Lung Diseases, Fungal/complications/*diagnosis/therapy ; Male ; Middle Aged ; Postoperative Complications/mortality ; Retrospective Studies ; Treatment Outcome ; Tuberculosis, Pulmonary/*complications

BACKGROUND: Anthracofibrosis, a descriptive term for multiple black pigmentation with fibrosis on bronchoscopic examination, has a close relationship with active tuberculosis (TB). However, TB activity is determined in the later stage by the TB culture results in some cases of anthracofibrosis. Therefore, it is necessary to identify early markers of TB activity in anthracofibrosis. There have been several reports investigating the serum levels of IL-2 sRalpha, IFN-gamma and TBGL antibody for the evaluation of TB activity. In the present study, we tried to measure the above mentioned serologic markers for the evaluation of TB activity in patients with anthracofibrosis. METHODS: Anthracofibrosis was defined when there was deep pigmentation (in more than two lobar bronchi) and fibrotic stenosis of the bronchi on bronchoscopic examination. The serum of patients with anthracofibrosis was collected and stored under refrigeration before the start of anti-TB medication. The serum of healthy volunteers (N=16), patients with active TB prior to (N=22), and after (N=13), 6 month-medication was also collected and stored. Serum IL-2 sRalpha and IFN-gamma were measured with ELISA kit (R&D system, USA) and serum TBGL antibody was measured with TBGL EIA kit (Kyowa Inc, Japan). RESULTS: Serum levels of IL-2 sRalpha in healthy volunteers, active TB patients before and after medication, and patients with anthracofibrosis were 640+/-174, 1,611+/-2,423, 953+/-562, and 863+/-401 pg/ml, respectively. The serum IFN-gamma levels were 0, 8.16+/-17.34, 0.70+/-2.53, and 2.33+/-6.67 pg/ml, and TBGL antibody levels were 0.83+/-0.80, 5.91+/-6.71, 6.86+/-6.85, and 3.22+/-2.59 U/ml, respectively. The serum level of TBGL antibody was lower than that of other groups (p<0.05). There was no significant difference of serum IL-2 sRalpha and IFN-gamma levels among the four groups. CONCLUSION: The serum levels of IL-2 sRalpha, IFN-gamma and TBGL antibody were not useful in the evaluation of TB activity in patients with anthracofibrosis. More useful ways need to be developed for the differentiation of active TB in patients with anthracofibrosis.
Bronchi ; Constriction, Pathologic ; Enzyme-Linked Immunosorbent Assay ; Fibrosis ; Healthy Volunteers ; Humans ; Interleukin-2* ; Pigmentation ; Refrigeration ; Tuberculosis* ; Tuberculosis, Pulmonary

BACKGROUND: The therapeutic effects of surfactant on acute lung injury derive not only from its recruiting action on collapsed alveoli but also from its anti-inflammatory effects. Pro-apoptotic action on alveolar neutrophils represents one of the important anti-inflammatory mechanisms of surfactant. In the present study, we evaluated the effects of surfactant on the apoptosis of human peripheral and rat alveolar neutrophils. METHODS: In the (Ed- the article is not definitely needed but it helps to separate the two prepositions 'in') in vitro study, human neutrophils were collected from healthy volunteers. An equal number of neutrophils (1X10(6)) (Ed-confirm) was treated with LPS (10, 100, 1000ng/ml), surfactant (10, 100, 1000micro gram/ml), or a combination of LPS (1000ng/ml) and surfactant (10, 100, 1000micro gram/ml). After incubation for 24 hours, the apoptosis of neutrophils was evaluated by Annexin V method. In the in vivo study, induction of acute lung injury in SD rats by intra-tracheal instillation of LPS (5mg/kg) was followed by intra-tracheal administration of either surfactant (30mg/kg) or normal saline (5ml/kg). Twenty-four hours after LPS instillation, alveolar neutrophils were collected and the apoptotic rate was evaluated by Annexin V method. In addition, changes of the respiratory mechanics of rats (respiratory rate, tidal volume, and airway resistance) were evaluated with one chamber body plethysmography before, and 23 hours after, LPS instillation. RESULTS: In the in vitro study, LPS treatment decreased the apoptosis of human peripheral blood neutrophils (control; 47.4+/-5.0%, LPS 10ng/ml; 30.6+/-10.8%, LPS 100ng/ml; 27.5+/-9.5%, LPS 1000ng/ml; 24.4+/-7.7%). The combination of low to moderate doses of surfactant with LPS promoted apoptosis (LPS 1000ng/ml + Surf 10micro gram/ml; 36.6+/-11.3%, LPS 1000ng/ml + Surf 100micro gram/ml; 41.3+/-11.2%). The high dose of surfactant (1000micro gram/ml) decreased apoptosis (24.4+/-7.7%) and augmented the anti-apoptotic effect of LPS (LPS 1000ng/ml + Surf 1000micro gram/ml; 19.8+/-5.4%). In the in vivo study, the apoptotic rate of alveolar neutrophils of surfactant-treated rats was higher than that of normal saline-treated rats (6.03+/-3.36% vs. 2.95+/-0.58%). The airway resistance (represented by Penh) of surfactant-treated rats was lower than that of normal saline-treated rats at 23 hours after LPS injury (2.64+/-0.69 vs. 4.51+/-2.24, p<0.05). CONCLUSIONS: Surfactant promotes the apoptosis of human peripheral blood and rat alveolar neutrophils. Pro-apoptotic action on neutrophils represents one of the important anti-inflammatory mechanisms of surfactant.
Acute Lung Injury* ; Airway Resistance ; Animals ; Annexin A5 ; Apoptosis* ; Healthy Volunteers ; Humans ; Neutrophils* ; Plethysmography ; Rats* ; Respiratory Mechanics ; Tidal Volume

BACKGROUND AND OBJECTIVES: Although the standard management of an abdominal aortic aneurysm (AAA) is surgery, endovascular stent-graft treatment is more attractive for patients with significant co-morbid conditions. We evaluated the immediate and mid-term outcomes for the endovascular treatment of AAA. SUBJECTS AND METHODS: Between November 1996 and August 2004, 59 patients with an AAA (53 males, mean age 68.0+/-9.6 years, 3 cases with ruptured AAA) underwent an endovascular stent-graft repair at our institute. All patients were evaluated by an angiography, taken just after the completion of the procedure and at followed up with computed tomography (CT) at 1, 3, 6 and 12 months, and yearly thereafter. RESULTS: Technical success was achieved in 54 of the 59 patients (91.5%). The periprocedural mortality rate was 3.4% (2 of the 59 patients). A primary endoleak was found in 12 patients (20.3%)(type I; 9 patients, type II; 2 patients, type III; 1 patient), 2 of which required subsequent surgical conversion. Spontaneous resolution of an endoleak was seen in 4 patients (33.3%). The average follow-up period of 57 patients was 27.5 months (range from 72 days to 2581 days). In 8 patients (14.0%), a newly developed secondary endoleak was documented. A total 14 patients (23.7%) died during the follow-up period (rupture; 3, operation-related sepsis; 3, unrelated causes; 3, cardiac arrest; 1, unknown causes; 4). The cumulative survival rates at 30 days and at 1 and 2 years were found to be 93.0, and 85.7 and 76.3%, respectively, using Kaplan-Meier methods. Secondary intervention was required in 12 patients (21.8%), and surgical conversion in 4 (6.8%), with 2 (3.4%) requiring conversion to open surgery immediately after the intervention. In those with technical success, without endoleaks and graft failure, the survival rate during follow-up was higher (97.1%; rate with the exception of unrelated cause of death) than that of all the patients. CONCLUSION: The immediate and mid-term results suggest that the endovascular treatment of an AAA is technically feasible and effective. There was higher mortality and morbidity in primary and newly developed endoleak cases; therefore, proper selection of cases, according to the anatomical and clinical criteria, is essential, with meticulous regular follow-ups being critical for the optimal endovascular treatment of an AAA.
Angiography ; Aortic Aneurysm, Abdominal* ; Conversion to Open Surgery ; Endoleak ; Follow-Up Studies ; Heart Arrest ; Humans ; Male ; Mortality ; Sepsis ; Survival Rate ; Transplants ; Treatment Outcome

Rhabdomyolysis is a syndrome involving the breakdown of skeletal muscle causing myoglobin and other intracellular proteins and electrolytes to leak into the circulation. There are various causes of acute rhabdomyolysis in childhood, such as direct trauma to muscle, muscle necrosis from ischemia, inflammation in muscle, or exposure to drugs and toxins. The most-important complication of this disorder is acute renal failure (ARF). However, the contributing factors to the development of ARF in children with rhabdomyolysis remain obscure. We report two cases of rhabdomyolysis after excessive exercise.
Acute Kidney Injury ; Child ; Electrolytes ; Humans ; Inflammation ; Ischemia ; Muscle, Skeletal ; Muscles ; Myoglobin ; Necrosis ; Proteins ; Rhabdomyolysis

Diabetes mellitus(DM) is a metabolic syndrome caused by deficiency of insulin secretion and a consequence of insulin resistance. Poor glycemic control is a common finding in children with Type 1 DM(T1DM). Approximately 60% of the young patients with T1DM develop abnormalities in the eyes and 15-20% in the kidney. Diabetic nephropathy (DN) is a serious metabolic complication of T1DM that leads to renal failure. Some clinical studies report that the duration of prepubertal diabetes may contribute less to the development of microvascular complications than pubertal and postpubertal duration. There have been few cases of DN in prepubertal patients with T1DM in Korea. Thus we report a case of a 12-year-old female with T1DM who had poor glycemic control and was diagnosed as DN in a prepubertal period. It was proven by renal biopsy after microscopic hematuria and proteinuria were detected through the mass school urinary screening program.
Biopsy ; Child ; Diabetic Nephropathies ; Eye ; Female ; Hematuria ; Humans ; Insulin ; Insulin Resistance ; Kidney ; Korea ; Mass Screening ; Proteinuria ; Renal Insufficiency

Peritonitis is one of the major complications of CAPD(continuous ambulatory peritoneal dialysis). Recently, multidrug-resistant organisms, such as vancomycin-resistant enterococcus (VRE) have been rarely reported by the pathogen as of CAPD-associated peritonitis. But, there is limited information on choices of effective therapy for VRE peritonitis in patients undergoing CAPD. We present a pediatric case of successful treatment of CAPD-associated peritonitis due to VRE with linezolid, and review of the literature.
Acetamides ; Enterococcus ; Humans ; Oxazolidinones ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis

BACKGROUND: Strategic infarct dementia (SID) is characterized by focal ischemic lesions involving specific sites that are critical for higher cortical functions. However, the mechanisms of SID are not well understood. We evaluated lesion sites, neuropsychiatric symptoms, neuroimaging and neuropsychological findings in patients with SID and have come up with suggestions of the mechanism behind SID. METHODS: Eleven patients with SID according to the NINDSAIREN criteria for vascular dementia were included. All patients were given a neurologic examination, brain MRI with MRA and brain perfusion SPECT using Tc-99m HMPAO. We evaluated neuropsychiatric symptoms and neuropsychological status using a Korean-version of the Neuropsychiatric Inventory(K-NPI) and Seoul Neuropsychological Screening Battery. RESULTS: Various sites were responsible for SID; the thalamus (n=5), genu of internal capsule (n=2), temporooccipital lobe (n=2), medial temporal lobe (n=1), medial frontal lobe (n=1). The most common neuropsychiatric symptom was apathy according to the K-NPI. Brain perfusion SPECT revealed ipsilateral cortical hypoperfusion, mainly in the frontal and temporal areas. In several cases, there were some degrees of cortical hypoperfusion in the contralateral areas of the lesion. On neuropsychological assessment, cognitive deficits on attention and frontal executive function were prominent. CONCLUSIONS: The thalamus, genu of internal capsule, and temporooccipital area were the most common sites responsible for SID. Based on the results that there were cortical hypoperfusion ipsilateral to subcortical strategic infarct and prominent cognitive deficits on attention and frontal executive function, it is suggested that disruption of the frontal-subcortical circuit may play an important role in patients with SID.
Apathy ; Brain ; Cerebral Infarction ; Dementia* ; Dementia, Vascular ; Executive Function ; Frontal Lobe ; Humans ; Internal Capsule ; Magnetic Resonance Imaging ; Mass Screening ; Neuroimaging* ; Neurologic Examination ; Neuropsychological Tests ; Perfusion ; Seoul ; Sudden Infant Death ; Technetium Tc 99m Exametazime ; Temporal Lobe ; Thalamus ; Tomography, Emission-Computed, Single-Photon