We report an autopsy case of secondary hemochromatosis associated with multiple frequent blood transfusion for the treatment of aplastic anemia. A 23-year-old man had been diagnosed as having aplastic anemia at the age of 13. He received a whole blood transfusion, about 1280 ml, every month during the past 10 years. Recently he developed diabetes mellitus and a congestive heart failure. The autopsy revealed that multiple organs were affected by secondary hemochromatosis, including the liver, heart, pancreas, spleen, bone marrow, stomach, thyroid gland, adrenal glands, and testes. The lungs and liver showed gross and microscopic findings consistent with a congestive heart failure in addition to hemochromatosis. The details are presented. This is a case of rare secondary hemochromatosis occurring in a young man and presenting the classic histopathologic changes indistinguishable from those of primary hemochromatosis.
Adrenal Glands ; Anemia ; Anemia, Aplastic* ; Autopsy* ; Blood Transfusion ; Bone Marrow ; Diabetes Mellitus ; Heart ; Heart Failure ; Hemochromatosis* ; Humans ; Liver ; Lung ; Pancreas ; Spleen ; Stomach ; Testis ; Thyroid Gland ; Young Adult

It is reported that the recurrence rate in intermittent exotropia is 20-50%. In this study, we evaluated whether the recurrence is reduced with asymmetric recession of bilateral lateral recti(LROU) in intermittent exotropia.Twenty-nine patients underwent the asymmetric LROU recession for intermittent exotropia with follow-up of 6-54 months. The amount of recession of lateral rectus muscle in deviating eye was 2mm more than in the fixating eye. Immediate postoperative overcorrection, the rate of orthophotia, and the occurrence of consecutive esotropia(ET) were analysed.The mean amount of immediate postoperative esodeviation was 16.5+/-7.4PD(0-34PD); four patients showed 0-4PD, 18 patients 11-20PD, and 7 patients showed considerable amount of ET as high as 21-30PD. Consecutive EToccurred in 6.8% of patients despite large amounts of postoeprative ET. The outcome of orthophoria was favorable, in 93.1% of patients in follow up of 6 months, 85.1% in 12 months and 78.5% in 24 months.This study suggests that asymmetric LROU recession is an effective procedure for the correction of intermittent exotropia.
Esotropia ; Exotropia* ; Follow-Up Studies ; Humans ; Recurrence

Phellinus strains were collected from different areas in Korea. Of them, the fast mycelial growing strains were artificially cultivated on the oak logs to produce fruiting body. The varieties, Phellinus linteus ASI26099 (Korea Sanghwang) and P. baumii PBJS (Jangsoo Sanghwang) were grown under the same conditions as controls. Their cultivating characteristics including mycelial colonization, pinhead formation, and fruiting body formation rate were investigated on the logs. Basidiocarps of Phellinus strains HN00K9, HN6036, and ASI26099 were concentrically zonate and shallowly sulcate, and dark chestnut showing typical characteristics of Tropicoporus linteus (synonyum: P. linteus, Inonotus linteus, polyporus linteus), which is distinguishably different to PBJS. HN00K9 showed the highest yield of fruiting body among the mushroom strains. The β-glucan content in fruiting bodies of HN00K9 was 20% higher than those of other strains. Bioactive effects of polysaccharide samples from fruiting bodies of Phellinus strains, HN00K9, HN6036, ASI26099, and PBJS were assessed on cell viability and cytokine (IL-6 and TNF-α) inhibition and finally on anticancer to different human cancer cells.

Phellinus strains were collected from different areas in Korea. Of them, the fast mycelial growing strains were artificially cultivated on the oak logs to produce fruiting body. The varieties, Phellinus linteus ASI26099 (Korea Sanghwang) and P. baumii PBJS (Jangsoo Sanghwang) were grown under the same conditions as controls. Their cultivating characteristics including mycelial colonization, pinhead formation, and fruiting body formation rate were investigated on the logs. Basidiocarps of Phellinus strains HN00K9, HN6036, and ASI26099 were concentrically zonate and shallowly sulcate, and dark chestnut showing typical characteristics of Tropicoporus linteus (synonyum: P. linteus, Inonotus linteus, polyporus linteus), which is distinguishably different to PBJS. HN00K9 showed the highest yield of fruiting body among the mushroom strains. The β-glucan content in fruiting bodies of HN00K9 was 20% higher than those of other strains. Bioactive effects of polysaccharide samples from fruiting bodies of Phellinus strains, HN00K9, HN6036, ASI26099, and PBJS were assessed on cell viability and cytokine (IL-6 and TNF-α) inhibition and finally on anticancer to different human cancer cells.

We present an adult female patient who developed irreversible paraplegia and areflexia four days post intrathecal chemotherapy with methotrexate, cytosine arabinoside and hydrocortisone. On magnetic resonance imaging (MRI) of the lumbar spine, diffuse gadolinium enhancement of the anterior spinal nerve roots (ventral roots) was detected. Methylprednisolone was intravenously administered at a daily dose of 30mg/kg for three days. Despite this treatment, flaccid weakness in the lower extremities and urinary retention persisted. Following consolidation chemotherapy, no improvement in neurologic status was noted. Six months later, a follow-up MRI revealed severe atrophy of the thoracic spinal cord.
Adult ; Antineoplastic Agents/administration & dosage/*adverse effects/*therapeutic use ; Female ; Humans ; Magnetic Resonance Imaging ; Paraplegia/*chemically induced/*pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/*drug therapy

BACKGROUND: Follicular patterned thyroid nodules with incomplete nuclear features of papillary thyroid carcinoma (FTN-INPTCs) are difficult to diagnose, and their biological behavior and association with follicular variants of PTC (FVPTCs) have not yet been established. The aim of this study is to determine immunohistochemical and molecular characteristics of FTN-INPTCs. METHODS: We investigated immunohistochemical features (galectin-3, HBME-1, CK19, fibronectin-1, CITED1), BRAF V600E mutation and RASSF1A promoter methylation status in 30 FTN-INPTC cases, along with 26 FVPTCs, 21 follicular adenomas (FAs) and 14 nodular hyperplasias (NHs). RESULTS: Expression of galectin-3, HBME-1, CK19 and CITED1 was significantly higher in FTN-INPTCs than in FAs or NHs, but expression of galectin-3, CK19 and fibronectin-1 was lower in FTN-INPTCs than in FVPTCs. The BRAF V600E mutation was not detected in the benign nodules or FTN-INPTCs, whereas 57% of FVPTCs had the mutation. RASSF1A promoter methylation was higher in FTN-INPTCs than in benign nodules but there was no difference between FTN-INPTCs and FVPTCs. CONCLUSIONS: Our results represent the borderline immunohistochemical and molecular characteristics of FTN-INPTC. We conclude that FTN-INPTC is an intermediate lesion between a benign nodule and a FVPTC, and that it is pathogenetically related to FVPTC.
Adenoma ; Carcinoma ; Carcinoma, Papillary ; Factor IX ; Galectin 3 ; Hyperplasia ; Methylation ; Thyroid Gland ; Thyroid Neoplasms ; Thyroid Nodule

Metformin has been reported to increase the expression of the glucagon-like peptide-1 (GLP-1) receptor in pancreatic beta cells in a peroxisome proliferator-activated receptor (PPAR)-alpha-dependent manner. We investigated whether a PPARalpha agonist, fenofibrate, exhibits an additive or synergistic effect on glucose metabolism, independent of its lipid-lowering effect, when added to metformin. Non-obese diabetic Goto-Kakizaki (GK) rats were divided into four groups and treated for 28 days with metformin, fenofibrate, metformin plus fenofibrate or vehicle. The random blood glucose levels, body weights, food intake and serum lipid profiles were not significantly different among the groups. After 4 weeks, metformin, but not fenofibrate, markedly reduced the blood glucose levels during oral glucose tolerance tests, and this effect was attenuated by adding fenofibrate. Metformin increased the expression of the GLP-1 receptor in pancreatic islets, whereas fenofibrate did not. During the intraperitoneal glucose tolerance tests with the injection of a GLP-1 analog, metformin and/or fenofibrate did not alter the insulin secretory responses. In conclusion, fenofibrate did not confer any beneficial effect on glucose homeostasis but reduced metformin's glucose-lowering activity in GK rats, thus discouraging the addition of fenofibrate to metformin to improve glycemic control.
Animals ; Blood Glucose/metabolism ; Body Weight/drug effects ; Diabetes Mellitus, Experimental/*drug therapy/*metabolism ; Drug Therapy, Combination ; Feeding Behavior/drug effects ; Fenofibrate/*pharmacology/therapeutic use ; Glucagon-Like Peptide 1/agonists/metabolism ; Glucose/*metabolism ; Glucose Tolerance Test ; Homeostasis/*drug effects ; Immunohistochemistry ; Injections, Intraperitoneal ; Insulin-Secreting Cells/drug effects/metabolism/pathology ; Lipid Metabolism/drug effects ; Male ; Metformin/*pharmacology/therapeutic use ; Peptides/administration & dosage/pharmacology ; Rats ; Receptors, Glucagon/metabolism ; Venoms/administration & dosage/pharmacology

Leptin, which is a plasma protein produced by the obese gene, is expressed and secreted by adipocytes. The clearance of lepdn from the circulation is unknown. But, markedly elevated serum leptin concentrations have recently been reported in patients with chronic renal failure. The purpose of the present study was to investigate plasma leptin concentration of patients with chronic renal failure and evaluate the factors affecting plasma leptin levels. Plasma leptin, insulin, and body mass index were determined in 34 patients with chronic renal failure and 55 control subjects. The plasrna leptin concentrations were not significantly different between patients with chronic renal failure and control subjects (9.4+/-11.8 vs 4.9+/-4.2ng/ml, P>0.05). The serum leptin concentrations were not significantly higher in both male and female CRF patients compared with control subjects (3.96+/-5.72 vs 2.48+/-1.65, P=0.1947, 17.07+/-14.02 vs 7.49+/-4.63ng/ml, P=0.07, respectively). And, there was no significant correlation between serum creatinine and plasma leptin. However, there was significant correlation between plasma leptin concentration and insulin level (P<0.05). We fit a multiple linear regre- ssion analysis with plasma leptin level as the dependent variable in CRF. Sex (male vs female) (P< 0.001) and insulin (P=0.004) were independently associated with plasma leptin level in CRF. These results suggested that plasma leptin level was regulated or affected by multiple factors inclu- ding sex and insulin resistance. Additional study is required to evaluate relationship between plasma leptin and insulin resistance in chronic renal failure.
Adipocytes ; Body Mass Index ; Creatinine ; Female ; Humans ; Insulin ; Insulin Resistance ; Kidney Failure, Chronic* ; Leptin* ; Male ; Plasma*

OBJECTIVES: It is widely accepted that estrogen has favorable effects on cardiovascular diseases, especially in the postmenopausal women. Endothelin-1(ET-I), released from the vascular endothelium, is a 21-amino acid peptide with strong vasoconstrictor activity. However, the effect of estrogen on the vasoconstriction to ET-1 has not been extensively studied. METHODS: To investigate the effect of estrogen (175beta-estradiol) on the vascular contraction to ET-1, porcine coronary artery(PCA) rings were suspended in organ chambers(37 degrees C, 95% O2/5% CO2) for measurement of isometric tension change. Endothelium was removed mechanically if necessary. In acute experiments, vascular rings were preincubated for 15minutes with 3different concentrations of 170beta-estradiol(10(-6), 10(-5), 10(-4)M) and concentration-contraction curves to cumulative doses of ET-1 were constructed. In the experiments after a longer exposure to 17beta-estradiol, the vessels with endothelium were exposed in the 5% CO2 incubator to 3different concentrations of 17beta-estradiol(10(-9), 10(-8), 10(-7)M) for 44-50 hours, and then concentrationcontraction curves to ET-1 were obtained. RESULTS: Incubation for 15minutes with 170beta-estradiol(10(-4)M) inhibited ET-1-induced contraction in the vessels with endothelium(area under the curve and maximal contraction, p<0.05 compared with control). This effect persisted regardless of the sex and the presence or absence of the endotheliurn. Incubation of the vessels far a longer time with 170beta-estradiol(44-50 hours) resulted in the inhibition of maximal contraction to ET-1(p<0.05) by a lower concentration of 175beta-estradiol(10(-7)M) than in acute experiments in male PCA rings, but an enhanced contraction to ET-1(area under the curve; p<0.05) by 10M of 175beta-estradiol was observed in female PCA rings. CONCLUSION: Short-time incubation with 17Pbeta-estradiol has an inhibitory effect on the contraction to ET-1 in PCA rings. This effect is independent of the presence of the endothelium and the sex of the pigs. A longer incubation with 17beta-estradiol results in a similar inhibitory effect on male(but not female) PCA rings, suggesting that a sex-related difference may exist concerning the effect of 17beta-estradiol on ET-1-induced contraction.
Cardiovascular Diseases ; Coronary Vessels* ; Endothelin-1* ; Endothelins ; Endothelium ; Endothelium, Vascular ; Estrogens ; Female ; Humans ; Incubators ; Male ; Passive Cutaneous Anaphylaxis ; Swine ; Vasoconstriction

PURPOSE: Intracavernosal self injection therapy is now being widely used to treat patients with erectile dysfunction. However, there is a large number of patients who give up the self injection program due to the fear of injection. Therefore, less invasive route of drug administration is highly recommended. We investigated the efficacy of intraurethral instillation of Prostaglandin El (PGE1 ) solution in the patients who showed full erection with intracavernosal injection of PGE1. MATERIALS AND METHODS: Twenty-nine impotent patients who showed full erection with intracavernosal injection of PGE1 were included in this study. We estimated the grades and durations of the penile erection after intraurethral instillation of PGE1 RESULTS: Sixteen out of 29 patients(55.2%) showed full erection with intraurethral instillation. Two patients(6.9%) showed urethral pain which disappeared within 24 hours. But there was no systemic side effect CONCLUSIONS: Intraurethral instillation of PGE1 appears to be safe, well tolerated, and less invasive treatment modality Thus it can be selected as an alternative treatment of impotence in selected cases.
Alprostadil* ; Erectile Dysfunction* ; Humans ; Male ; Penile Erection