A case of spontaneous intracerebral hemorrhage in neurofibromatosis type 1 associated with pheochromocytoma / 대한내과학회지

Spontaneous intracerebral hemorrhage is very rare in patients with neurofibromatosis type 1. A 30-year-old woman was referred to our hospital for evaluation of headaches, nausea, and vomiting. She was diagnosed with neurofibromatosis type 1 and a spontaneous intracerebral hemorrhage. She was treated with conservative management, including antihypertensive medications for 32 months. However, because of sustained hypertension, we performed screening tests for a pheochromocytoma. The 24 hr urine VMA and urine metanephrines were elevated. Abdominal CT showed a right adrenal mass, 7.5 x 5.8 cm in size.. After a successful resection of the tumor, the hypertension resolved without medication for > 1 year. This case illustrates that delayed diagnosis and treatment of pheochromocytoma can cause serious complications from hypertension, such as cerebrovascular hemorrhage. Thus, in patients with neurofibromatosis and hypertension, screening for pheochromocytoma is important for the early detection of an adrenal tumor.

Prognostic Significance of the Lown Grades and Late Potentials in Patients after Myocardial Infarction

BACKGROUND AND OBJECTIVES: The aims of this study were to assess the long term overall survival of patients after an acute myocardial infarction (AMI), and to determine the association of survival with the occurrence of ventricular arrhythmia, as recorded by Holter electrocardiography (ECG) and signal-averaged electrocardiography (SAECG). SUBJECTS AND METHODS: One hundred fifty two patients with an AMI were enrolled between January 2000 and August 2006. SAECG and Holter ECG were performed before hospital discharge (at range of 2-10 day). The grading system of Lown was used to evaluate the ventricular premature beats on Holter ECG. Three groups of patients were identified based on the seriousness of the ventricular arrhythmia, as identified by the Holter ECG: Lown grade 0, Lown grades 1, 2 and Lown grades 3, 4, 5. SAECG was performed with a high pass frequency of 25 Hz and 40 Hz. The presence of late potentials (LPs) recorded on SAECG was evaluated. The predictors for survival were assessed using Cox's proportional hazard model and Kaplan-Meier analysis. RESULTS: The mean duration of follow-up was 45.8+/-25.5 months. Twenty four patients (15.8%) died during follow-up. The multivariate predictors of all cause death included age [hazard ratio (HR)=1.25, 95% confidence interval (CI)=1.08-1.47, p=0.003] and Lown grades 3, 4 and 5 (HR=19.17, 95% CI=1.25-290.80, p=0.034). Survival analysis did not show a significant relationship between LPs and overall patient survival. The only predictors for overall mortality were age and the Lown grade. CONCLUSION: SAECG did not predict mortality for the patient with AMI. The ventricular arrhythmias recorded by conventional Holter before hospital discharge may be a useful noninvasive prognostic test after an AMI.

Lack of Association Between Osteoarthritis of the Knee and Gene Polymorphisms of VDR in Korean Postmenopausal Women / 대한류마티스학회지

OBJECTIVE: To determine whether polymorphisms of the Vitamin D receptor (VDR)gene,known to be associated with osteoporosis and/or osteoarthritis (OA) in Caucasians,might also relate to the risk of OA and osteoporosis in Korean postmenopausal women METHODS: A population of 130 postmenopausal women,including 76 healthy controls and 54 knee OA patients,were studied using anteroposterior radiographs of the knee,which were graded for OA according to the Kellgren classification system.The VDR genotype was determined by using polymerase chain reaction and by digestion with the three restriction enzymes Taq I,Bsm I,and Apa I.Femoral neck bone mineral density (BMD)was assessed in all participants by dual energy X-ray absorptiometry . RESULTS: VDR genotype frequency distributions in Koreans were much different from Caucasian's both in the OA group and the control group.Especially, "t t", "B B" and "A A" genotype were very rare,prominently differentiating from Caucasians.But within Koreans,no significant differences in VDR genotype frequencies were observed between OA cases and controls.VDR genotype was not significantly associated with the radiographic grades of OA.And there were no significant relationships of VDR genotype with BMD scores in each group. CONCLUSION: In Korean postmenoposal women,the VDR gene polymorphisms do not significantly contribute to an increased prevalence of knee OA or to differences in BMD.VDR genotype analysis would not be helpful for assessing the risk of knee OA in Koreans because :(1)there is no correlation of VDR genotypes with the radiographic severity of OA ;and (2)there is a more skewed distribution of VDR genotypes in Korean population compared to the Caucasian's .

A Case of Malignant Lymphoma during Treatment with Methotrexate for Rheumatoid Arthritis / 대한류마티스학회지

Rheumatoid arthritis (RA)is a multisystemic inflammatory disease with a prevalance of approximately 1%of the population.The use of disease modifying anti-rheumatic drug (DMARD)is an essential regimen for the treatment of RA. Among DMARDs,methotrexate (MTX)is used worldwide with confirmed effectiveness.However,cytopenia,pulmonary injury,and hepatic toxicity are a few side effects limiting its use.In addition,although the oncogenic potential of MTX is low,several cases have been reported.Recently an increased risk of developing lymphoproliferative disorders has been reported in patients with RA. The incidence is higher in elderly individuals with severe longstanding seropositive RA,those with Sjogren's or Felty's syndrome,and in patients with RA treated with prolonged low dose MTX.We describe a case of RA who developed non-Hodgkin's lymphoma during low dose MTX therapy.

A Case of the Membranous Glomerulonephritis Associated with Squamous Cell Lung Cancer / 결핵

The nephrotic syndrome that occurs in the absence of renal vein thrombosis, amyloidosis, neoplastic infiltration of the kidneys is an unusual but a well recognized paraneoplastic syndrome. The most frequently reported neoplasms associated with nephrotic syndrome are Hodgkin's disease and various carcinomas. The most common renal lesions are membranous glomerulonephritis(MGN) associated with carcinomas and minimal change lesions associated with Hodgkin's disease. Approximately 40% to 45% of patients clinically manifest the MGN symptoms prior to the diagnosis of the tumor, 40% simultaneously with the tumor and the remaining 15% to 20% following the tumor. Therefore, evaluating the underlying malignancy in patients with MGN is important. Here we report a patient with squamous cell lung cancer, which was detected 12 months after a MGN had been diagnosed, with a review of the relevant literature.

Predictable Factors for Paradoxical Reactions in Conscious Sedation with Midazolam During ERCP / 대한소화기내시경학회지

BACKGROUND/AIMS: Paradoxical reaction after midazolam administration is relatively uncommon and can obstruct the performance of ERCP. But it can not be predicted before drug administration. We investigate the difference in occurrence of paradoxical reaction according to personal characteristics and clinical status of patients. METHODS: During 155 ERCP procedures, we injected midazolam and meperidine intravenously for conscious sedation until deep sleep occurred. Among 155 patients, 108 patients did not showed paradoxical reaction (group I) and 47 patients (30.3%) showed gross behavioral disturbance and/or agitation (group II). Paradoxical agitation was seen in 9 (7.1%) procedures. RESULTS: Type A-like personality (p=0.002), sleep-talking habit (p=0.026) and presence of pain at the beginning of ERCP (p=0.036) and during ERCP (p=0.021) were seen more frequently in group II. Duration of ERCP was longer (p=0.034) and dosage of midazolam was larger (p=0.009) in group II. In multivariate analysis, having sleep-talking (OR, 5.5), type A-like personality (OR 3.9) and dosage of midazolam (OR 1.3) were risk factors of paradoxical reaction. CONCLUSIONS: Paradoxical agitation after midazolam administration was uncommon and can be managed with flumazenil. Paradoxical reaction can be predicted more often in patients with type A-like personality, sleep-talking habit, complaining pain before ERCP, and in patients injected large dosage of midazolam.

A Case of Collapsing Glomerulopathy Discovered in End Stage Renal Disease(ESRD) with Normal Sized Kidney / 대한신장학회잡지

Collapsing glomerulopathy is recently described as the disease which causes rapidly progressive renal failure. Clinically, the disease starts with constitutional symptoms, and then nephrotic syndrome with marked proteinuria and hypertension follows. Eventually the disease rapidly progresses to the ESRD within several weeks to months. Its typical renal biopsy findings are extensive glomerular capillary collapse, visceral epithelial cell hypertrophy and hyperplasia, and variable degree of tubulointerstitial inflammation. Such findings closely resemble those of HIV associated nephropathy(HIVAN) except endothelial inclusion body in HIVAN. Here we present, for the first time in Korea, a 16 yrs-old female patient with ESRD in a normal sized kidney. Nephrotic syndrome and hypertension were also accompanied. Serologically, she had no evidence of HIV infection. Though her symptom duration was somewhat shorter than that previously reported, her renal biopsy findings were those of collapsing glomerulopathy as previously described. Her renal function did not return to normal, and now she is on regular hemodialysis, waiting for renal transplantation.