A Case of Duodenal Crohn's Disease Associated with Ampullar Insufficiency and Cholangitis

Abstract: A 67-year-old Japanese woman with duodenal Crohn's disease developed cholangitis. Radiographic and endoscopic examinations revealed a marked stenosis of the third portion and a dilatation of the second portion of the duodenum. The endoscopic and histopathological observations showed this disease affected the Vater's papilla. Duodenoscopic manometry indicated a reduced pressure of the phasic sphincter of Oddi, indicating ampullar insufficiency. E. coli was found in the bile culture as the infecting organism. The authors suggest that the cholangitis in this patient was caused by the reflux of duodenal contents into the biliary tract.     

Onset of Aortic Dissection Complicated with Cushing's Disease: A Case Report and Review of the Literature

Cushing''s syndrome and Cushing''s disease cause various metabolic disorders associated with high cortisol levels. Some reports have shown that Cushing''s syndrome is complicated with dissecting aortic aneurysm and aortic dissection after long-term exposure to high cortisol levels. We herein report a rare case of aortic dissection complicated with Cushing''s disease. Aortic dissection may occur even under relatively short periods of high cortisol conditions. This case suggests that hypercortisolemia should be treated as soon as possible in order to prevent aortic dissection in subjects with Cushing''s disease.     

Aortic and Mitral Valve Stenosis with Regurgitation: Not Due to Rheumatic Heart Disease

The patient is a 13‐year‐old Mexican–American immigrant who had no previous medical care. Upon arrival to the United States she was diagnosed with severe mitral valve stenosis and regurgitation. In addition she had severe aortic stenosis with mild to moderate aortic valve regurgitation, which was thought to be rheumatic heart disease. On the basis of the clinical and echocardiographic findings she was taken to the operating room for both mitral and aortic valve replacement. Her operation was complicated by the discovery that her aorta was completely calcified, as were her coronary arteries, mitral valve and aortic valve. She underwent aortic and mitral valve replacement as well as replacement of her aortic arch. Her coronary arteries were patch augmented and reimplanted into the newly created ascending aorta. Based on the pathologic examination of the surgical tissue a diagnosis of Gaucher disease was made. (Echocardiography 2011;28:E24‐E27)     

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.     

Aortic valve perforation in the setting of Cogan's syndrome

Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio‐vestibular manifestations. Systemic manifestations are quite unusual with pan‐vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56‐year‐old woman presented with ocular, audio‐vestibular, and systemic manifestations with medium‐sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.     

HLA B27: A prognostic factor in juvenile chronic arthritis

This study was performed to assess the frequency of HLA B27 in patients with juvenile chronic arthritis (JCA) of varying severity and outcome by studying three patient categories: those in whom cytostatic treatment with azathioprine had been started, those with secondary amyloidosis, and those with arthroplasty of the knee or hip joints. In the first category the frequency of the HLA B27 allele was compared between those who had attained remission and those who had not. In the second and third categories the rate at which amyloidosis developed and the timing for the need of arthroplasty, were compared for HLA B27-positive and-negative patients. A control group consisted of 37 patients with uncomplicated seronegative polyarthritis. Ten of the 37 patients in the control group (27%) were HLA B27 positive as opposed to 84 out of 190 (44%) in the three study groups. Of the 101 patients treated with azathioprine, two out of 15 in remission were HLA B27 positive, whereas as many as 41 out of 86 with still active disease were HLA B27 positive (p=0.013). Of the secondary amyloidosis patients, 29 out of 51 carried HLA B27. The HLA B27-positive patients contracted amyloidosis on average 5.9 (median 6.7) years earlier than the HLA B27-negative patients (p=0.038). Of the arthroplasty patients, 39 out of 91 carried HLA B27. The HLA B27-positive patients underwent arthroplasty on average 2.9 (median 3.5) years earlier than the HLA B27-negative patients (p=0.050). We conclude that HLA B27-positive cases are accumulated among the most severe cases of JCA.     

Reactive arthritis after pharyngeal infection: report of two siblings

Abstract

?In this report we describe the cases of two siblings with reactive arthritis (ReA) induced by pharyngeal infections. The patients were a man and his sister living with their parents. He developed arthritis in August 1997, and his younger sister developed similar symptoms in September 1998. Their disease conditions were both severe and required hospitalization. Their conditions improved with the administration of nonsteroidal anti-inflammatory drugs together with antibiotics, and both fully recovered within 1–2 weeks. Rheumatic fever was ruled out since streptococcal infections were not demonstrated with antistreptolysin O (ASO) or antistreptokinase (ASK) titers, or with pharyngeal culture. The sister suffered from a rash which was similar to erythema nodosum on her lower extremities, but neither chorea nor carditis was observed. Both human leukocyte antigen (HLA) typing analyses revealed positive results for HLA-B40 and -B39 for the brother and sister, respectively. Both HLA-B40 and -B39 are considered to be related to HLA-B27-negative ReA, most likely poststreptococcal reactive arthritis (PSRA). Therefore, the two patients were tentatively diagnosed as suffering from PSRA.     

Aortic regurgitation secondary to diastolic prolapse of a tubular intimal flap into the left ventricle in a patient with anuloaortic ectasia

A 32-year-old man with distal skeletal manifestations of Marfan's syndrome had experienced shortness of breath and orthopnea for one month. Physical examination showed the presence of severe aortic regurgitation. Both noninvasive and invasive studies revealed that the aortic regurgitation was induced by previously undescribed peculiar and unusual etiology: diastolic prolapse of a circumferentially dissected tubular intimal flap into the left ventricle. The patient underwent surgical repair with striking clinical improvement.     

Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve,Bicuspid Aortic Valve,and Aortic Coarctation

Atypical Shone''s complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.     

A Case of Turner's Syndrome with Crohn's Disease

Abstract: A case of Crohn's disease that developed during the follow-up of Turner's syndrome is presented in this paper, together with references to the literature. This case had a mosaic pattern (45X46Xi (Xq)) in chromosomal analysis and BW52, DR2 in HLA typing with autoantibodies.     

Reactive arthritis after pharyngeal infection: report of two siblings

 In this report we describe the cases of two siblings with reactive arthritis (ReA) induced by pharyngeal infections. The patients were a man and his sister living with their parents. He developed arthritis in August 1997, and his younger sister developed similar symptoms in September 1998. Their disease conditions were both severe and required hospitalization. Their conditions improved with the administration of nonsteroidal anti-inflammatory drugs together with antibiotics, and both fully recovered within 1–2 weeks. Rheumatic fever was ruled out since streptococcal infections were not demonstrated with antistreptolysin O (ASO) or antistreptokinase (ASK) titers, or with pharyngeal culture. The sister suffered from a rash which was similar to erythema nodosum on her lower extremities, but neither chorea nor carditis was observed. Both human leukocyte antigen (HLA) typing analyses revealed positive results for HLA-B40 and -B39 for the brother and sister, respectively. Both HLA-B40 and -B39 are considered to be related to HLA-B27-negative ReA, most likely poststreptococcal reactive arthritis (PSRA). Therefore, the two patients were tentatively diagnosed as suffering from PSRA. Received: July 3, 2001 / Accepted: October 10, 2001     

B Cells and Immunoglobulins Dependent Mechanisms in Rheumatoid Arthritis: A Possible Rationale of the Extracorporeal Immunomodulation for Rheumatoid Arthritis

Abstract: Patients with rheumatoid arthritis (RA) have several options for treatment nowadays, although we do not know what types of therapies are effective for these patients because RA is a very heterogenous disease. We discuss several possible mechanisms of RA in this review and explain one possible scenario of autoantibodies dependent arthritis confirmed by anti‐glucose‐6‐phosphate isomerase antibodies. We also propose several efficacious treatments for treating these patients as made‐to‐order therapies.     

Real Time Triplane Echocardiography in Aortic Valve Stenosis: Validation,Reliability, and Feasibility of a New Method for Valve Area Quantification

Aims: The aim of the study was to validate a novel formula for aortic valve area (AVA) based on the principle of continuity equation, that substitutes Doppler‐derived stroke volume (SV) by SV directly measured with real time simultaneous triplane three‐dimensional echocardiography (RT3P). RT3P has proved accuracy for left ventricular volume calculation. So far, however, neither this potential has been applied to hemodynamic assessment, nor RT3P has succeeded in the evaluation of aortic valve disease. Methods and results: AVA was measured in 21 patients with aortic stenosis using Gorlin's equation, Doppler continuity equation (two‐dimensional echocardiography), the novel RT3P method, and by substituting Doppler‐derived SV by SV measured with two‐dimensional stroke volume (2DSV). RT3P has the best linear association (R²= 0.61) and the best correlation with Gorlin of all noninvasive methods (even if not statistically significant). RT3P carries significantly lower mean differences with catheterization, as compared with 2D and 2DSV (Table 4). Standard deviations of mean differences between RT3P and catheterization and between the other echocardiographic methods are not statistically different, even if RT3P seems to be nearer to catheterization. Inter‐ and intraobserver variability were, respectively, 0.03 ± 0.11 cm² and 0.02 ± 0.03 cm², better than 2D and 2DSV. Conclusions: RT3P has revealed to be more accurate than two‐dimensional method in AVA quantification, with a better intraobserver agreement. In addition, it allows simple and fast image acquisition. (Echocardiography 2010;27:644‐650)