A Case of Hemochromatosis / 대한내과학회지

Idiopathic hemochromatosis is hereditary hemochromatosis without secondary cause. Hereditary hemochromatosis is a common disorder of iron metabolism with a prevalence of 1 case per 250-300 persons. Affected individuals absorb excessive amounts of dietary iron for a long time and tissue iron deposition results in skin discoloration, arthropathy, hepatic cirrhosis, heart cirrhosis, heart failure, diabetes mellitus and impotence. Early diagnosis and institution of phlebotomy treatment will prevent these manifestations and normalize life expectancy. We report a case of hemochromatosis in 65-year-old male of no transfusion and iron ingestion history who was anti-HCV(+), chronic alcoholic, hepatic iron index>2.24.

Clinical Efficacy of Carvedilol in Patients with Moderate to Severe Congestive Heart Failure

BACKGROUND: Clinical trials have shown that b-adrenergic blocking drugs are effective and well tolerated in patients with mild to moderate congestive heart failure. Carvedilol is a mild b1-selective adrenergic blocking agent with vasodilating properties due to a blocker and antioxidant and anti-proliferative properties. This study assessed the efficacy and safety of carvedilol in patients with moderate to severe congestive heart failure caused by idiopathic dilated cardiomyopathy. METHODS: We enrolled 27 patients with moderate to severe congestive heart failure with a left ventricular ejection fraction of 35% by MUGA scan. Each patient was randomly assigned to either control (n-9) or carvedilol (n-18, target dose 25 mg bid) for 6 months while background therapy with digoxin, diuretics, and ACE inhibitor remained constant. RESULTS: Compared to the control group, patients in the carvedilol group showed significant increase of left ventricular ejection fraction (p<0.05). In addition, patients in the carvedilol group had a tendency to show a decrease in left ventricular end-diastolic dimension and heart rate. Also, the carvedilol group had a greater frequency of symptomatic improvement than the control group. There was neither serious side effects nor hospitalization. CONCLUSION: These finding indicate that carvedilol produces important clinical benefits in patients with moderate to severe heart failure treated with digoxin, diuretics, and ACE inhibitor without serious side effects.

The Degree of TGF-beta1 and alpha-SMA Expression in Liver Tissues with its Prognostic Value for the Therapeutic Efficacy of Interferon in Chronic Hepatitis B / 대한간학회지

BACKGROUND/AIMS: The aim of this study was to evaluate the effect of alpha-interferon (IFN) on liver histology as well as on activation of hepatic stellate cell ( HSC) and trans for ming growth fact or beta-1 (TGF beta-1) expression. We had also investigated the clinical usefulness of TGFbeta-1 and alpha-smooth muscle actin (alpha-SMA) expression in liver tissue for predicting a response to alpha-IFN therapy in chronic hepat it is B. METHODS: We studied the expression of TGFbeta-1 and alpha-SMA in liver biopsys pecimens from 51 chronic hepatitis B pat ients. Using immunohistochemical staining and a semiquant it ative scoring met hod, we also evaluated TGF-beta1 and alpha-SMA expression in liver stellate cells before and after alpha-IFN therapy in liver tissue from rebiopsys pecimen of the 12 chronic hepatitis B pat ients. Recombinant IFN alpha-2b (Intron A) in doses of 6 MU/ d was given to patients intramus cularly three times per week for 6 months (total doses , 432 MU). The patients were divided into two groups according to serum alanine aminotransferase levels as well as HBV- DNA and HBeAg s eroconversion stat e. Histological grading and staging scores were according to modified Histological Activity Index (HAI) grading systems of Ishak (1995). RESULTS: The index of portal inflammation and total scores of HAI grading significantly decreased in biopsies after alpha-IFN treatment, but the scores of fibrosis staging showed no significant change in biopsies after IFN treatment. A significant decrease in alpha-SMA expression, especially in periportal area, was found, but the change of TGFbeta-1 expression was not significant. The immunoreactivity of alpha-SMA was significantly lower in responders than in non-responders, whereas the diffference of immunoreactivity of TGF-beta1 between these two groups was not found. CONCLUSIONS: These findings suggest that alpha-IFN therapy may reduce the necroinflammatory activity in liver tissues of chronic B viral hepatitis and that the degree of alpha-SMA expression before treatment may be employed as a pottent predicting indicator for the therapeutic efficacy of IFN-alpha.

A Case of Pulmonary Siderosis / 대한내과학회지

Pulmonary siderosis is one kind of pneumoconiosis caused by the long term inhalation of iron dust. Iron is deposited in the lungs, usually in the form of iron oxides. Iron oxides are relatively inert particles with a minimal fibrotic response. It occurs in a number of occupations including welding, steel rolling and grinding, casting, iron ore mining and oxyacetylene cutters. We have experienced the first case of pulmonary siderosis in Korea. A 42-year-old woman who had engaged in a metalware manufacturing factory as a manager for 7 years was admitted because of dyspnea on exertion. A pulmonary function test disclosed a mildly obstructive ventilatory defect. Open lung biosy revealed deposition of iron-laden macrophages in most alveolar spaces by which confirmed the diagnosis of pulmonary siderosis. We report a case of pulmonary siderosis with a review of the literature.

Clinical Report of Effects of Pre and Post-partum Thyroiditis (PPT) / 대한내분비학회지

BACKGROUND: Excessive iodine intake increases the occurrence of autoimmune thyroid disorders by enhancing immunogenecity of iodine-rich thyroglobulin, In Korea, most of postpartum women take a large amount of iodine-rich seaweed. Although the excessive iodine intake may affect the thyroid function, only a few reports were available concering iodine intake, especially on postpartum period. METHODS: A prospective study was undertaken in 146 of normal delivered postpartum women. Dietary intake and urinary excretion of iodine, serum T3, T4, TSH, anti-TPO Ab and anti-Tg Ab were measured before and 1, 6, 12 and 24 weeks after delivery. Iodine intake was analyzed by one-to-one interview using 24hr recall and food frequency questionnaire. RESULTS: 1. PPT was occurred in 6 (10.3%) postparturn women, It presented as hypothyroidism alone in 1 (16.7%), transient thyrotoxicosis followed by hypothyroidisrn in 3 (50.0%), and thyrotoxicosis alone in 2 (33.3%) of the follwed-up patients. 2. During pregnancy, no difference was found in age, serum T3, T4 and TSH between PPT and normal thyroid function group. 3. In PPT group, anti-TPO and anti-Tg Ab were significantly higher than those of normal thyroid function group during pregnancy, and their sensitivity for PPT was 40% and 33%, respectively. But there was no correlation between dietary iodine intake and the titer of thyroid auto-antibodies. 4. There was no correlation between pre and post-partum dietary iodine intake and occurrence of PPT. CONCLUSION: In Korea, the incidence of PPT was slightly higher than other nations. The sensitivity of thyroid auto-antibodies was too low to use for prediction of PPT. Pre and post-partum iodine intake had no effect on the occurrence of PPT and post-partum thyroid function.