Metabolic acidosis and abnormal calcium metabolism in patients on maintenance hemodialysis / 대한내과학회지

BACKGROUND: Mild metabolic acidosis is frequently found among the stable patients treated with maintenance hemodialysis. However, its clinical effects have yet to be clarified. This study was undertaken to estimate the prevalence of metabolic acidosis in the patients undergoing chronic hemodialysis and to evaluate the clinical significance of metabolic acidosis, especially in relation to calcium metabolism. METHODS: In 124 patients undergoing maintenance hemodialysis, analysis of arterial blood gas and measurement of various biochemical markers and parathyroid hormone were carried out with predialysis blood obtained from arterial side of arteriovenous fistula. RESULTS: Ninety two patients(74.2%) had metabolic acidosis. Their arterial pH was 7.32+/-0.01, arterial bicarbonate concentration 17.1+/-0.3 mEq/L, and PaCO2 33.1+/-0.5 mmHg. The patients with metabolic acidosis showed a lower calcium(7.90+/-0.16 vs. 8.68+/-0.17 mg/dL, p< 0.05), and higher phosphorus(4.96+/-0.16 vs. 3.68+/-0.39 mg/dL, p< 0.05), alkaline phosphatase(233.6+/-22.7 vs. 145.9+/-13.7 U/L, p< 0.05) and parathyroid hormone(176.5+/-23.7 vs. 52.8+/-14.4 pg/mL, p< 0.05) levels compared to those with normal acid-base balance. In the patients with metabolic acidosis, PaCO2 level showed a positive correlation with arterial bicarbonate concentration(r=0.62, p< 0.001). The lower arterial bicarbonate was, the higher serum potassium(r=-0.24, p< 0.05), phosphorus(r=-0.42, p< 0.001) and anion gap(r=-0.28, p< 0.01) were. When the patients were divided into two groups according to the dialysate buffer used, the lower calcium-acetate group showed lower total calcium(7.28+/-0.25 vs. 7.96+/-0.17 mg/dL, p< 0.05) and ionized calcium(0.85+/-0.05 vs. 1.08+/-0.04 mmol/L, p< 0.05) levels and higher alkaline phosphatase(457.1+/-170.2 vs. 209.4+/-15.9, p< 0.05) and parathyroid hormone (364.4+/-83.7 vs. 155.4+/-23.6 pg/mL, p< 0.05) levels compared to the higher calcium-bicarbonate group. CONCLUSION: Current hemodialytic practice is less than ideal, as evidenced by a high prevalence of metabolic acidosis. The metabolic acidosis in maintenance hemodialysis is associated with abnormal calcium metabolism, suggesting that a more aggressive correction of metabolic acidosis may be required by individualizing dialysis prescription.

Thyroid Disorders in Korean Patients with Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: To define the prevalence of thyroid dysfunction and autoantibodies in rheumatoid arthritis(RA). A prospective controlled study was conducted on 62 RA patients and 55 controls with non-inflammatory rheumatic diseases (3 osteoarthritis, 6 fibromyalgia, 4 carpal tunnel syndrome and 42 patients with nonspecific joint pain). All subjects were tested for T3, T4, TSH, FT4, RAIU, rheumatoid factor and ANA. Anti-thyroglobulin (TG) Ab and anti-microsomal (Mic) Ab were assayed using a more sensitive direct assay of RIA and TSH-R Ab (TRAb) using competitive radioimmunoassay. Thyroid dysfunction was observed in 10 RA patients (16.2%) of which 6 patients had hypothyroidism and 4 patients hyperthyroidism, and was significantly more prevalent in RA (p=0.026). Anti-Mic Ab was significantly more prevalent in RA patients. The frequencies of anti-Mic Ab and anti-TG Ab in RA patients were 69.4% and 58.1% (cut-off value was 0.3U/ml), but were 16.1% and 12.9% when those over 10U/ml were calculated. Thyroid dysfunction was not present when either of anti-TG Ab or anti-Mic Ab was below 0.3U/ml. The prevalence of thyroid dysfunction or autoantibodies were not different according to the presence of RF or ANA. CONCLUSION: The prevalence of thyroid dysfunction and autoantibodies were significantly increased in RA patients and the variance of the latter in other reports might be explained at least partially by difference in cut-off values of different methods. Thyroid dysfunction may not develop when either of anti-Mic Ab or anti-TG Ab is below 0.3 U/ml in RA.

Weber-Christian disease presenting with proptosis: a case report

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.

Weber-Christian disease presenting with proptosis: a case report

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.

Clinical manifestations of amanita subjunquillea poisoning / 대한내과학회지

BACKGROUND: To the best of our knowledge, the report on Amanita subjunquillea poisoning has not been found in the medical literature. We investigated the clinical aspects of Amanita subjunquillea poisoning. METHODS: Sixteen subjects who had ingested the mushroom (A. subjunquillea) were examined for clinical features, laboratory and radiologic findings prospectively. RESULTS: The mean incubation period was 11.5 hours(range: 3 to 17 hours). The initial presentations were gastrointestinal symptoms which persisted for 2 to 4 days. The transaminase levels were elevated in all subjects and peaked on day 3 after ingestion of the mushrooms (mean AST/ALT levels : 3241 IU/L and 3741 IU/L, respectively). Biochemical evidence of pancreatitis and disseminated intravascular coagulation were frequent (83.3% and 62.5%, respectively). Liver ultrasonography and scintigraphy revealed abnormalities in most cases. Massive hepatic necrosis was confirmed by liver biopsy in one subject. The overall mortality was 12.5%. CONCLUSION: Clinical manifestations of A. subjunquillea poisoning were similar to those of other poisonous Amanitaceae intoxication. However, the mortality rate was lower in A. subjunquillea poisoning. Our data showed strong evidence that A. subjunquillea should be classified in the group of poisonous mushrooms.

A Case of Behcet's Disease Complicated with Deep Vein Thrombosis and Cerebral Venous Sinus Thrombosis / 대한류마티스학회지

Behcet's disease is a multisystemic disease, in which vascular involvement occurs in 7. 7-60% of patients. There are three forms of vascular involvement such as venous occlusion, arterial occlusion and arterial aneurysm. Venous lesions are more common than arterial lesions and the common sites of venous thrombosis are inferior vena cava, superior vena cava, and superficial or deep veins of extremities. Thrombosis of cerebral venous sinus is an infrequent complication of Behcet's disease and it seems to be rare that both deep vein thrombosis and cerebral venous sinus thrombosis occured at the same time. We experienced a case of Behcet's disease complicated with thromboses of left common femoral vein and right transverse sinus. A 29-year-old man presented with persistent dull nature headache, nausea, vomiting, blurred vision and left lower leg swelling. Duplex ultrasonography of lower extremity showed thrombus in the left common femoral vein. Magnetic resonance imaging of brain showed isodense signal intensity lesions in right transverse sinus on Tl weighted image. On magnetic resonance angiography, the right transverse, sigmoid sinus and right internal jugular vein were not visualized. The patient showed substantial improvement after treatment with steroid, cyclosporine A and anticoagulants.

Rheumatoid arthritis associated with myelodysplastic syndrome: a case report

Myelodysplastic syndromes (MDS) are a group of refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. There is increasing recognition of immunological abnormalities in patients with MDS, including defective B- and T-cell function, hyper- or hypogammaglobulinemia and monoclonal gammopathy. MDS have been associated with Sjogren's syndrome, polymyalgia rheumatica, relapsing polychondritis and systemic lupus erythematosus. Although there may be various rheumatologic features, including acute arthritis in MDS, chronic inflammatory arthritis is uncommonly combined. There have been a few reports that described cases of rheumatoid arthritis (RA) concurrent with MDS, but advanced rheumatoid arthritis with typical joint deformities has rarely been reported. We report a case of rheumatoid arthritis with atlantoaxial subluxation combined with refractory anemia in a 31-year-old woman.

Two cases of upper airway obstruction masquerading as asthma: One tracheal adenoid cystic carcinoma, the other foreign body aspiration / 천식및알레르기

Reversible airway obstruction and wheezing are typical features of bronchial asthma, but these features may also be observed in other conditions. However, the inclusion of these conditions in the differential diagnosis of asthma-like symptoms in adults is often neglected, resulting in a correct diagnosis being made after months of unsuccessful treatment for asthma. We report two cases of an upper airway obstruction masquerading as asthma. In case one, adenoid cystic carcinoma was identified in the trachea of a patient showing asthma-like symptoms, positive response to allergic skin test and methacholine inhalation challenge. In the other case, aspirated foreign body was misdiagnosed and treated as asthma for more than one year. A high degree of suspicion and a thorough medical evaluation censisting of history, physical examination and pulmonary function test including shapes of spirogram are essential to the differential diagnosis of asthma-like symptoms

A case of occupational asthma induced by terephthaloy1 chloride / 천식및알레르기

Terephthaloyl chloride, a chemical of low molecular weight, is used as an intermediate by a fabric manufacturing industry. It is known to cause gastrointestinal, respiratory and skin irritation. However, it has not been reported as a cause of occupational asthma till now. We report a case of occupational asthma caused by prolonged exposure to terephthaloyl chloride in the workplace. A 38 year-old man visited at the Allergy Clinic because of cough, dyspnea and wheezing for 5 years. He had worked at a factory for 15 years where he was involved in the process of manufacturing fabrics. At presentation, he had no symptoms and showed no abnormality on physical examination. When challenged with vapor of terephthaloyl chloride, he experienced sneezing and paroxysmal cough in a couple of minutes, followed by dyspnea and wheezing at 10 min. He also experienced urticarial rashes on the face and chest. The pulmonary function tests showed an atypical prolonged immediate airway response. PC20 methacholine decreased from 5 mg/ml to 0.79 mg/ml 24 hours after the challenge. Light microscopic examination of bronchial biopsies showed loss of epithelium, thickening of basement membrane, submucosal fibrosis, and increased inflammatory cell infiltration. The immediate drop in FEV1 and urticarial rash to terephthaloyl chloride suggests the possibility of an immediate hypersensitivity immune reaction. Further studies are needed to clarify the exact mechanism of terephthaloyl chloride induced asthma.