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A Case of Spontaneous Hemoperitoneum Presenting as the Initial Manifestation of Systemic Lupus Erythematosus.

The Korean Journal of Internal Medicine.2010;25(4):458-460. doi:10.3904/kjim.2010.25.4.458

Thrombocytopenia is included in the classification criteria for systemic lupus erythematosus (SLE). However, severe thrombocytopenia causing spontaneous bleeding is rare. Here, we describe a 22-year-old woman who presented with spontaneous hemoperitoneum as the first manifestation of SLE. Laboratory findings compatible with SLE included positive antinuclear antibody and a false-positive venereal disease research laboratory. Symptoms suggesting the disease were not prominent early after admission, but headache and seizures that developed on the 3rd day of admission led to the diagnosis of SLE. The brain magnetic resonance imaging and angiography findings were compatible with the neuropsychiatric manifestations of SLE. High-dose steroid and monthly intravenous cyclophosphamide pulse therapy were effective at improving the headache and seizure, as well as the hemoperitoneum.
Adult ; Female ; Hemoperitoneum/*etiology ; Humans ; Lupus Erythematosus, Systemic/*complications ; Thrombocytopenia/etiology

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Postoperative High-Dose Intravenous Iron Sucrose with Low Dose Erythropoietin Therapy after Total Hip Replacement.

Jiyeol YOON ; Sungmin KIM ; Soo Chan LEE ; Hongsub LIM

The Korean Journal of Internal Medicine.2010;25(4):454-457. doi:10.3904/kjim.2010.25.4.454

Erythropoietin combined with parenteral iron sucrose therapy is an alternative to blood transfusion in anemic patients. It was shown to be effective in surgical patients in several previous studies when used in conjunction with other methods. However, there are no guidelines about safety limits in dosage amounts or intervals. In this study, we report a case of significant postoperative hemorrhage managed with high dose parenteral iron sucrose, low dose erythropoietin, vitamin B12, vitamin C, and folic acid. An 80-year-old female patient presented for severe anemia after a total hip arthroplasty and refused an allogenic blood transfusion as treatment. The preoperative hemoglobin of 12.2 g/dL decreased to 5.3 g/dL postoperatively. She received the aforementioned combination of iron sucrose, erythropoietin, and vitamins. A total of 1,500 mg of intravenous iron sucrose was given postoperatively for 6 consecutive days. Erythropoietin was also administered at 2,000 IU every other day for a total of 12,000 IU. The patient was discharged in good condition on the twelfth postoperative day with a hemoglobin of 8.5 g/dL. Her hemoglobin was at 11.2 g/dL on the twentieth postoperative day.
Aged ; Aged, 80 and over ; Anemia/*drug therapy ; Arthroplasty, Replacement, Hip/*adverse effects ; Blood Transfusion ; Drug Therapy, Combination ; Erythropoietin/*administration & dosage ; Female ; Ferric Compounds/*administration & dosage ; Humans

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Anaplastic Thyroid Carcinoma Initially Presented with Abdominal Cutaneous Mass and Hyperthyroidism.

Kyu Hyoung LIM ; Keun Wook LEE ; Jee Hyun KIM ; So Yeon PARK ; Sung Hee CHOI ; Jong Seok LEE

The Korean Journal of Internal Medicine.2010;25(4):450-453. doi:10.3904/kjim.2010.25.4.450

A 63-year-old female was admitted to our hospital with a tender abdominal wall mass about 15 cm in diameter, which she had for 1 month. About 1 week earlier, the patient had also perceived a mass in the neck area. Computed tomography revealed huge thyroid and periumbilical masses. The thyroid hormone levels were consistent with a hyperthyroid state. Pathological examination of the thyroid mass was compatible with anaplastic thyroid carcinoma (ATC) and the abdominal cutaneous mass was shown to be metastatic ATC. Despite palliative radiotherapy and chemotherapy, the patient died of respiratory failure on her 63rd day of hospitalization. This case demonstrates that abdominal cutaneous metastasis and hyperthyroidism can occur as initial manifestations of ATC. To our knowledge, this is the first reported case.
Abdomen ; Female ; Humans ; Hyperthyroidism/*etiology ; Middle Aged ; Skin Neoplasms/*secondary ; Thyroid Neoplasms/pathology

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Thalidomide Induced Nonspecific Interstitial Pneumonia in Patient with Relapsed Multiple Myeloma.

Myung Hee KANG ; Ji Hyun JU ; Hoon Gu KIM ; Jung Hun KANG ; Kyung Nyeo JEON ; Ho Cheol KIM ; Gyeong Won LEE

The Korean Journal of Internal Medicine.2010;25(4):447-449. doi:10.3904/kjim.2010.25.4.447

A 63-year-old female diagnosed with relapsed multiple myeloma visited our hospital complaining of a persistent cough. Since July 2006, she had been taking 100 mg thalidomide daily and gradually developed shortness of breath and a persistent dry cough. A chest X-ray and computed tomography showed ground glass opacities in both lungs. An open lung biopsy of the right middle lobe under general anesthesia revealed chronic peribronchial inflammation, mild interstitial fibrosis, and intra-alveolar macrophage infiltration, with some hemosiderin features, compatible with non-specific interstitial pneumonia (NSIP). After discontinuing the thalidomide, the patient's symptoms did not deteriorate, although the radiographs did not improve. The patient is alive and well with regular outpatient follow-up without progression of the NSIP.
Female ; Humans ; Lung Diseases, Interstitial/*chemically induced ; Middle Aged ; Multiple Myeloma/*drug therapy ; Thalidomide/*adverse effects/therapeutic use

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A Comparison of Three Different Guidelines for Osteoporosis Treatment in Patients with Rheumatoid Arthritis in Korea.

Jiyeol YOON ; Seong Ryul KWON ; Mie Jin LIM ; Kowoon JOO ; Chang Gi MOON ; Jihun JANG ; Won PARK

The Korean Journal of Internal Medicine.2010;25(4):436-446. doi:10.3904/kjim.2010.25.4.436

BACKGROUND/AIMS: Osteoporotic fractures are an important comorbidity with rheumatoid arthritis (RA). We determined the overall fracture risk as assessed by the World Health Organization (WHO)'s FRAX(R) tool in Korean patients with seropositive RA. Additionally, we compared treatment eligibility according to the criteria of the Korean Health Insurance Review Agency (HIRA), FRAX, and the National Osteoporosis Foundation (NOF). METHODS: Postmenopausal women and men > or = 50 years of age with seropositive RA were recruited from one rheumatism center in Korea. The FRAX score was estimated using the Japanese model. Patients were classified as eligible for treatment using the HIRA, NOF, and FRAX thresholds for intervention. RESULTS: The study of 234 patients included 40 men (17%). The mean age was 60 +/- 9 years, and 121 (52%) patients had osteoporosis according to the WHO criteria. The overall median 10-year fracture risk was 13% for major osteoporotic fractures and 3.5% for hip fractures. HIRA guidelines identified 130 patients (56%) eligible for treatment, FRAX included 126 patients (54%), and 151 patients (65%) were included according to NOF guidelines. Older patients with a greater number of risk factors were included by FRAX compared to HIRA. The overall concordance between HIRA and FRAX, expressed as the kappa index, was 0.67, but was as low as 0.44 when limited to patients > or = 60 years of age. CONCLUSIONS: One-half of the patients had osteoporosis requiring treatment. RA patients have a high risk of fracture, and the adoption of a risk-scoring system should be considered.
Aged ; Arthritis, Rheumatoid/*complications ; Bone Density ; Cross-Sectional Studies ; Female ; Hip Fractures/epidemiology ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Osteoporosis/epidemiology/*therapy ; Osteoporotic Fractures/epidemiology ; Practice Guidelines as Topic ; Prevalence

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Engagement of Toll-Like Receptor 3 Induces Vascular Endothelial Growth Factor and Interleukin-8 in Human Rheumatoid Synovial Fibroblasts.

Su Jin MOON ; Mi Kyung PARK ; Hye Jwa OH ; Seon Yeong LEE ; Seung Ki KWOK ; Mi La CHO ; Ji Hyeon JU ; Kyung Su PARK ; Ho Youn KIM ; Sung Hwan PARK

The Korean Journal of Internal Medicine.2010;25(4):429-435. doi:10.3904/kjim.2010.25.4.429

BACKGROUND/AIMS: Angiogenesis, which is a critical step in the initiation and progression of rheumatoid arthritis (RA), involves pro-angiogenic factors, including interleukin (IL)-8 and vascular endothelial growth factor (VEGF). We investigated the role of Toll-like receptor 3 (TLR3) in the regulation of pro-angiogenic factors in RA fibroblast-like synoviocytes (FLS). METHODS: FLS were isolated from RA synovial tissues and stimulated with the TLR3 ligand, poly (I:C). The levels of VEGF and IL-8 in the culture supernatants were measured using enzyme-linked immunosorbent assays, and the mRNA levels were assessed by semiquantitative reverse transcription-polymerase chain reaction. The expression patterns of VEGF and IL-8 in the RA synovium and osteoarthritis (OA) synovium were compared using immunohistochemistry. RESULTS: The expression levels of TLR3, VEGF, and IL-8 were significantly higher in the RA synovium than in the OA synovium. VEGF and IL-8 production were increased in the culture supernatants of RA FLS stimulated with poly (I:C), and the genes for these proteins were up-regulated at the transcriptional level after poly (I:C) treatment. Treatment with inhibitors of nuclear factor-kappaB (NF-kappaB), i.e., pyrrolidine dithiocarbamate and parthenolide, abrogated the stimulatory effect of poly (I:C) on the production of VEGF and IL-8 in RA FLS. CONCLUSIONS: Our results suggest that the activation of TLR3 in RA FLS promotes the production of proangiogenic factors, in a process that is mediated by the NF-kappaB signaling pathway. Therefore, targeting the TLR3 pathway may be a promising approach to preventing pathologic angiogenesis in RA.
Arthritis, Rheumatoid/drug therapy/*etiology/metabolism ; Cells, Cultured ; Fibroblasts/metabolism ; Humans ; Interleukin-8/analysis/*biosynthesis/genetics ; NF-kappa B/physiology ; Neovascularization, Pathologic/etiology ; RNA, Messenger/analysis ; Synovial Membrane/cytology/*metabolism ; Toll-Like Receptor 3/analysis/*physiology ; Vascular Endothelial Growth Factor A/analysis/*biosynthesis/genetics

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Association of the Superoxide Dismutase (V16A) and Catalase (C262T) Genetic Polymorphisms with the Clinical Outcome of Patients with Acute Paraquat Intoxication.

Joong Rock HONG ; Su Jin SEOK ; Du Shin JEONG ; Sang Gon LEE ; Hyo Wook GIL ; Jong Oh YANG ; Eun Young LEE ; Sae Yong HONG

The Korean Journal of Internal Medicine.2010;25(4):422-428. doi:10.3904/kjim.2010.25.4.422

BACKGROUND/AIMS: Many patients with acute paraquat (PQ) intoxication die even at low PQ concentrations, whereas others with similar concentrations recover. Therefore, it is possible that individual differences in antioxidant capacity are responsible for the variable clinical outcome in patients with acute PQ intoxication. METHODS: We investigated whether there was a relationship between the genetic polymorphisms of SOD (V16A), catalase (C262T), and GPX1 (C593T) in 62 patients with acute PQ intoxication and the clinical outcomes of these patients. RESULTS: The frequency of the Mn-SOD V/V, V/A, and A/A genotypes were 56.3, 43.5, and 0% in survivors and 86.9, 13.1, and 0% in non-survivors (p > 0.05). The GPX1 C/C, C/T, and T/T genotypes were present in 100, 0, and 0% of all subjects. The catalase C/C, C/T, and T/T genotypes were present in 100, 0, and 0% of survivors, and in 82.6, 17.4, and 0% of non-survivors. Neither erythrocyte SOD activity nor catalase activity were significantly different between survivors and non-survivors. CONCLUSIONS: No association was found between clinical outcome of acute PQ intoxication and the genetic polymorphism of GPX1 (C593T) or the genetic polymorphisms or enzyme activity of superoxide dismutase (V16A) or catalase (C262T).
Acute Disease ; Adult ; Aged ; Catalase/*genetics ; Female ; Genotype ; Glutathione Peroxidase/genetics ; Humans ; Male ; Middle Aged ; Paraquat/*poisoning ; Poisoning/mortality ; *Polymorphism, Genetic ; Superoxide Dismutase/*genetics

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Serum Globotriaosylceramide Assay as a Screening Test for Fabry Disease in Patients with ESRD on Maintenance Dialysis in Korea.

Jeong Yup KIM ; Young Youl HYUN ; Ji Eun LEE ; Hye Ran YOON ; Gu Hwan KIM ; Han Wook YOO ; Seong Tae CHO ; No Won CHUN ; Byoung Chunn JEOUNG ; Hwa Jung KIM ; Keong Wook KIM ; Seong Nam KIM ; Yung A KIM ; Hyun Ah LEE ; Jong Young LEE ; Yung Chun LEE ; Hun Kwan LIM ; Keong Sik OH ; Seong Hwan SON ; Beong Hee YU ; Kyeong So WEE ; Eun Jong LEE ; Young Ki LEE ; Jung Woo NOH ; Seung Jung KIM ; Kyu Bok CHOI ; Suk Hee YU ; Heui Jung PYO ; Young Joo KWON

The Korean Journal of Internal Medicine.2010;25(4):415-421. doi:10.3904/kjim.2010.25.4.415

BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Adult ; Aged ; Fabry Disease/blood/*diagnosis ; Female ; Humans ; Kidney Failure, Chronic/blood/*therapy ; Male ; Middle Aged ; *Renal Dialysis ; Trihexosylceramides/*blood ; alpha-Galactosidase/genetics/metabolism

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Clinical Significance of Observation without Repeated Radioiodine Therapy in Differentiated Thyroid Carcinoma Patients with Positive Surveillance Whole-Body Scans and Negative Thyroglobulin.

Dong Jun LIM ; Joo Hyun O ; Min Hee KIM ; Ji Hyun KIM ; Hyuk Sang KWON ; Sung Hoon KIM ; Moo Il KANG ; Bong Yun CHA ; Kwang Woo LEE ; Ho Young SON

The Korean Journal of Internal Medicine.2010;25(4):408-414. doi:10.3904/kjim.2010.25.4.408

BACKGROUND/AIMS: Currently, there is no consensus on the necessity of repeated radioiodine therapy (RAI) in patients who show iodine uptake in the thyroid bed on a diagnostic whole-body scan (DxWBS) despite undetectable thyroglobulin (Tg) levels after remnant ablation. The present study investigated the clinical outcomes of scan-positive, Tg-negative patients (WBS+Tg-) who did or did not receive additional RAI. METHODS: We retrospectively reviewed 389 differentiated thyroid carcinoma patients who underwent a total thyroidectomy and received high-dose RAI from January 2003 through December 2005. The patients were classified according to surveillance DxWBS findings and TSH-stimulated Tg levels 6 to 12 months after the initial RAI. RESULTS: Forty-four of the 389 patients (11.3%) showed thyroid bed uptake on a DxWBS despite negative Tg levels (WBS+Tg-). There was no difference in clinical and pathological parameters between WBS+Tg- and WBS-Tg- patients, except for an increased frequency of thyroiditis in the WBS+Tg- group. Among the 44 WBS+Tg- patients, 27 subjects were treated with additional RAI; 25 subjects showed no uptake in subsequent DxWBS. Two patients were evaluated only by ultrasonography (US) and displayed no persistent/recurrent disease. The other 17 patients received no further RAI; Eight patients and two patients showed no uptake and persistent uptake, respectively, on subsequent DxWBS. Six patients presented negative subsequent US findings, and one was lost to follow-up. Over the course of 53.2 +/- 10.1 months, recurrence/persistence was suspicious in two patients in the treatment group. CONCLUSIONS: There were no remarkable differences in clinical outcomes between observation and treatment groups of WBS+Tg- patients. Observation without repeated RAI may be an alternative management option for WBS+Tg- patients.
Adult ; Aged ; Female ; Humans ; Iodine Radioisotopes/pharmacokinetics/*therapeutic use ; Male ; Middle Aged ; Thyroglobulin/*blood ; Thyroid Neoplasms/blood/radionuclide imaging/*radiotherapy ; *Whole Body Imaging

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Microarray Analysis of Papillary Thyroid Cancers in Korean.

Hyun Sook KIM ; Do Hyung KIM ; Ji Yeon KIM ; Nam Ho JEOUNG ; In Kyu LEE ; Jin Gu BONG ; Eui Dal JUNG

The Korean Journal of Internal Medicine.2010;25(4):399-407. doi:10.3904/kjim.2010.25.4.399

BACKGROUND/AIMS: Papillary thyroid cancer (PTC) is the most common malignancy of the thyroid gland. It involves several molecular mechanisms. The BRAF V600E mutation has been identified as the most common genetic abnormality in PTC. Moreover, it is known to be more prevalent in Korean PTC patients than in patients from other countries. We investigated distinct genetic profiles in Korean PTC through cDNA microarray analysis. METHODS: Transcriptional profiles of five PTC samples and five paired normal thyroid tissue samples were generated using cDNA microarrays. The tumors were genotyped for BRAF mutations. The results of the cDNA microarray gene expression analysis were confirmed by real-time PCR and immunohistochemistry analysis of 35 PTC patients. RESULTS: Four of the five patients whose PTC tissues were subjected to microarray analysis were found to carry the BRAF V600E mutation. Microarrays analysis of the five PTC tissue samples showed the expression of 96 genes to be increased and that of 16 genes decreased. Real-time reverse transcription-polymerase chain reaction (RT-PCR) confirmed increased expression of SLC34A2, TM7SF4, COMP, KLK7, and KCNJ2 and decreased expression of FOXA2, SLC4A4, LYVE-1, and TFCP2L1 in PTC compared with normal tissue. Of these genes, TFCP2L1, LYVE-1, and KLK7 were previously unidentified in PTC microarray analysis. Notably, Foxa2 activity in PTC was reduced, as shown by its cytoplasmic localization, in immunohistochemical analyses. CONCLUSIONS: These findings demonstrate both similarities and differences between our results and previous reports. In Korean cases of PTC, Foxa2 activity was reduced with its cytoplasmic accumulation. Further studies are needed to confirm the relationship between FOXA2 and BRAF mutations in Korean cases of PTC.
Adult ; Aged ; Carcinoma, Papillary/*genetics ; Female ; *Gene Expression Profiling ; Hepatocyte Nuclear Factor 3-beta/analysis/genetics ; Humans ; Immunohistochemistry ; Kallikreins/analysis/genetics ; Korea ; Male ; Middle Aged ; *Mutation ; Oligonucleotide Array Sequence Analysis/*methods ; Polymerase Chain Reaction ; Proto-Oncogene Proteins B-raf/*genetics ; Thyroid Neoplasms/*genetics ; Vesicular Transport Proteins/analysis/genetics

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