Congestive heart failure due to an iatrogenic arteriovenous fistula in a patient with neurofibromatosis-1 / 대한내과학회지

A 39-year-old woman, who 3 months prior had undergone sono-guided biopsy for pelvic masses, was admitted to our hospital with dyspnea. After the procedure, a large arteriovenous (AV) fistula was detected. The patient developed dyspnea with pain and swelling of the right leg and subsequently visited our hospital. Multiple cafe-au-lait spots and subcutaneous nodules were noted and a continuous bruit was heard in the right pelvic area. Transthoracic echocardiography revealed marked dilatation of the right atrium, right ventricle, and inferior vena cava. An angiogram showed multiple AV fistulas between the right iliac arteries and veins. The patient underwent aortoiliofemoral bypass surgery. Follow-up echocardiography after surgery showed dramatic improvement in heart function. Molecular analysis revealed a novel variation (c.4270-1G>a) at the 24th intron-exon splicing consensus sequence of the neurofibromin 1 (NF1) gene. This is a rare case of AV fistula that developed after biopsy and caused right-sided heart failure in a patient with neurofibromatosis.

A Case of Erdheim-Chester Disease with periodic fever and knee pain / 대한내과학회지

ECD is a rare, non-Langerhans form of histiocytosis. The first case was reported in 1930 by Chester and the term ECD was first described by Jaffe in 1972. Many authors have suggested that ECD is a part of the spectrum of Langerhans cell histiocytosis(LCH), whereas others believe ECD as a distinct lipoidosis because of the symmetric distribution of the long bone lesions on the roentgenogram and the lack of Langerhans granules and S-100 antibody staining in the foamy histiocytes. We experienced a case of Erdheim-Chester disease (ECD) with periodic night fever, myalgia and both knee pain, the first case reported in Korea.

The Correlation between Coronary Artery Disease and Carotid Atherosclerosis

BACKGROUND: In patients with coronary artery disease (CAD), atherosclerotic changes of carotid arteries (CA) often coexist with CAD itself. If the degree of carotid atherosclerosis can be estimated, it would be very helpful in the management of patients with CAD. METHODS: CA intima-media thickness (IMT) was evaluate by ultrasonography at 12segments (both proximal, middle, distal common CA, bifurcation, internal and external CA-of the extracranial CA) on the 182 subjects whom underwent coronary angiograms. The subjects were divided into 4 groups according to the severity of CAD; control (C, n=23), single vessel disease (1, n=64), two vessel disease (II, n=44), three vessel disease (III, n=51). RESULTS: The means (+/-SD) of maximal IMT, chosen from the 12 segments, of each group were 1.4+/-0.7mm (C), 2.1+/-1.4mm (I), 2.2+/-1.2mm (II), and 2.9+/-1.7mm (II). The 4 groups showed significant differences between each other. The only conparison to yield unsignificant differences was between group I and group II (p=0.02 for C and I, p=0.001 for C and II, p<0.001 for C and III, p=0.01 for I and III, p=0.04 for II and III). When multivariate analysis was used to assess which major risk factors for CAD (age, male sex, smoking, hypertension, diabetes, cholesterol, triglycerides and CAD groups affected CA IMT), group III and increasing age were the most significant variables (p=0.0001 and 0.0035, respectively). CONCLUSIONS:It is necessary to evaluate the status of the extracranial carotid arterial system with ultrasonography in patients with three vessel CAD even if neurologically symptomless. It becomes especially evident in elderly patients.