Relationship between regional abnormality of left ventricular rapid filling and coronary microcirculation disturbance in hypertrophic cardiomyopathy

Background and hypothesis: To investigate the mechanism of regional left ventricular diastolic filling disturbance in hypertrophic cardiomyopathy (HCM), we assessed the relationship between abnormalities of regional ventricular rapid filling and regional coronary microcirculation using radionuclide ventriculography and exercise ²⁰¹thallium (²⁰¹Tl) myocardial scintigraphy with sector analysis. Methods: Thirty patients with HCM and 14 patients with atypical chest pain syndrome (controls) were studied. Left ventricular images (left anterior oblique view) were obtained by electrocardiogram-gated ^99mtechnetium radionuclide an-giography and were divided into three sectors radiating from the geometric center. The time-activity curves and their first derivative curves were analyzed, and the peak filling rate (PFR), the ratio of the time-to-peak filling rate per diastolic interval (TPFR/T) were calculated for the global left ventricle and for the lateral and septal sectors. Exercise stress ²⁰¹Tl myocardial scintigraphy was also performed, and early and delayed images were obtained. The regional washout rate (WR) was then evaluated for the lateral and septal sectors. Results: In HCM patients, the regional PFR in the septal sector (corresponding to the region of hypertrophic myocardium) was 285 ± 76%/s, and was significantly lower than that in the controls (398 ± 90%/s, p <0.01). The regional TPFR/T in the septal sector (32 ± 10%) was prolonged compared with the value of 21 ± 5% in the controls (p <0.05). The regional WR in the septal sector was 21 ± 9%, and was significantly lower than that in the controls (43 ± 5%, p <0.01). Moreover, regional WR correlated positively with regional PFR (r = 0.5, p < 0.05) and showed a weak negative relationship with regional TPFR/T (r = -0.4, p <0.07) in the septal sector. Conclusions: These results suggest that regional impairment of rapid filling might be related to a disturbance of the coronary microcirculation in HCM.     

Factors associated with functional limitation in stair climbing in female Japanese patients with knee osteoarthritis

BACKGROUND: Osteoarthritis (OA) of the knee is a common form of arthritis, and affects quality of life. We investigated factors associated with functional limitation in stair climbing among female Japanese patients with knee OA. As weight is a known risk factor for knee OA, we focused on body weight at 40 years of age, and examined the association with present weight, past weight, and weight change. METHODS: Subjects were 360 Japanese women aged 40-92 years who were newly diagnosed with knee OA at 3 university hospitals over a 1-year period. Factors associated with the severity of functional limitation in stair climbing were assessed by calculating odds ratios (OR) using the proportional odds model in logistic regression. RESULTS: Weight at diagnosis showed a positive association with severe functional limitation in stair climbing; however, a negative association was observed for weight change since age 40. Further analysis indicated that the association with weight at age 40 (highest vs. lowest quartile, OR=2.84, 95% confidence interval: 1.03-7.83, trend p=0.071) is stronger than weight at diagnosis. Other significant characteristics were age (70+ vs. 40-59 years, OR=7.37), previous knee pain and/or swelling 12 years or more before diagnosis (OR=2.67), and physical work (OR=1.94). In addition, higher parity was found to be a negatively associated factor (for tripara or more, OR=0.41). CONCLUSIONS: This study identified factors, such as heavy weight at age 40 and physical labor, which are potentially useful for preventing severe functional limitation for female knee OA patients. In addition, higher parity was associated with milder stair climbing limitation.     

Sclerosing cholecystitis associated with autoimmune pancreatitis

AIM: To evaluate the histopathological and radiological findings of the gallbladder in patients with autoimmune pancreatitis (AIP). METHODS: The radiological findings of the gallbladder of 19 AIP patients were retrospectively reviewed. Resected gallbladders of 8 AIP patients were examined histologically and were immunostained with anti- IgG4 antibody. Controls consisted of gallbladders resected for symptomatic gallstones (n = 10) and those removed during pancreatoduodenectomy for pancreatic carcinoma (n = 10), as well as extrahepatic bile ducts and pancreases removed by pancreatoduodenectomy for pancreatic carcinoma (n = 10). RESULTS: Thickening of the gallbladder wall was detected by ultrasound and/or computed tomography in 10 patients with AIP (3 severe and 7 moderate); in these patients severe stenosis of the extrahepatic bile duct was also noted. Histologically, thickening of the gallbladder was detected in 6 of 8 (75%) patients with AIP; 4 cases had transmural lymphoplasmacytic infiltration with fibrosis, and 2 cases had mucosal-based lymphoplasmacytic infiltration. Considerable transmural thickening of the extrahepatic bile duct wall with dense fibrosis and diffuse lymphoplasmacytic infiltration was detected in 7 patients. Immunohistochemically, severe or moderate infiltration of IgG4-positive plasma cells was detected in the gallbladder, bile duct, and pancreas of all 8 patients, but was not detected in controls. CONCLUSION: Gallbladder wall thickening with fibrosis and abundant infiltration of IgG4-positive plasma cells is frequently detected in patients with AIR We propose the use of a new term, sclerosing cholecystitis, for these cases that are induced by the same mechanism as sclerosing pancreatitis or sclerosing cholangitis in AIR     

Consensus of primary care in acute pancreatitis in Japan

The incidence of acute pancreatitis in Japan is increasing and ranges from 187 to 347 cases per million populations. Case fatality was 0.2% for mild to moderate, and 9.0% for severe acute pancreatitis in Japan in 2003. Experts in pancreatitis in Japan made this document focusing on the practical aspects in the early management of patients with acute pancreatitis. The correct diagnosis of acute pancreatitis and severity stratification should be made in all patients using the criteria for the diagnosis of acute pancreatitis and the multifactor scoring system proposed by the Research Committee of Intractable Diseases of the Pancreas as early as possible. All patients diagnosed with acute pancreatitis should be managed in the hospital, Monitoring of blood pressure, pulse and respiratory rate, body temperature, hourly urinary volume, and blood oxygen saturation level is essential in the management of such patients. Early vigorous intravenous hydration is of foremost importance to stabilize circulatory dynamics. Adequate pain relief with opiates is also important. In severe acute pancreatitis, prophylactic intravenous administration of antibiotics at an early stage is recommended. Administration of protease inhibitors should be initiated as soon as the diagnosis of acute pancreatitis is confirmed. A combination of enteral feeding with parenteral nutrition from early stage is recommended if there are no clear signs and symptoms of ileus and gastrointestinal bleeding. Patients with severe acute pancreatitis should be transferred to ICU as early as possible to perform special measures such as continuous regional arterial infusion of protease inhibitors and antibiotics, and continuous hemodiafiltratton. The Japanese Government covers medical care expense for severe acute pancreatitis as one of the projects of Research on Measures for Intractable Diseases.     

Clinicopathologic features of ampullary carcinoma without jaundice

GOALS: To evaluate clinicopathological features of ampullary carcinoma without jaundice. BACKGROUND:: Obstructive jaundice is the most common symptom of patients with ampullary carcinoma. However, some patients with ampullary carcinoma do not have jaundice at the time of diagnosis. STUDY: Clinicopathologic findings of 23 patients with ampullary carcinoma showing no visible jaundice (serum total bilirubin <3.0 mg/dL) and 38 patients with ampullary carcinoma showing jaundice at the time of diagnosis were retrospectively compared. RESULTS: Fifteen of 23 patients with nonjaundiced ampullary carcinoma complained of fever and/or abdominal pain. Five asymptomatic patients were found to have a dilated bile duct on screening ultrasound or to have a tumor-like swelling of the papilla of Vater during routine upper gastrointestinal endoscopy. There was no significant difference in age, sex, size, macroscopic type, histologic type, rates of duodenal invasion, pancreatic invasion, and lymph node metastasis, and prognosis between the two groups. The cumulative 5-year and 10-year survival rates of nonjaundiced patients were 70.2% and 49.0%, compared with 33.6% and 29.4% of jaundiced patients. Ten of the 23 nonjaundiced ampullary carcinomas (43%) were in Stage I, whereas 4 of the 38 jaundiced ampullary carcinomas (11%) were in Stage I (P < 0.01). Mechanisms of nonjaundice in ampullary carcinoma were suspected to be determinant by the infiltrating pattern of the carcinoma to the lower portion of the bile duct. CONCLUSIONS: Mechanisms of nonjaundice in ampullary carcinoma might be determined by the infiltrating pattern of the carcinoma to the lower portion of the bile. As a greater number of nonjaundiced ampullary carcinomas were in an early stage, detection of them may provide an improved clinical outcome.     

The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis

Background

The cholangioscopic features of IgG4-related sclerosing cholangitis (IgG4-SC) remain undefined. The aim of this study was to clarify these endoscopic features using peroral video cholangioscopy (PVCS) in IgG4-SC patients.

Methods

PVCS was performed in 33 patients: IgG4-SC (n = 13); primary sclerosing cholangitis (PSC; n = 5); and cholangiocarcinoma (n = 15), which included hilar cholangiocarcinoma (HCCA; n = 5) and distal cholangiocarcinoma (DCCA; n = 10).

Results

The most frequent findings on PVCS in the IgG4-SC patients were dilated (62 %) and tortuous (69 %) vessels, and absence of partially enlarged vessels. The incidence of dilated and tortuous vessels was significantly higher in IgG4-SC patients than in PSC patients (p = 0.015). Scarring and pseudodiverticula were found significantly more often in PSC patients than in IgG4-SC patients (p = 0.001 and p = 0.0007, respectively). The incidence of partially enlarged vessels was significantly higher in DCCA patients than in IgG4-SC patients (p = 0.004). In contrast, the incidence of dilated vessels was significantly higher in IgG4-SC patients than in HCCA patients (p = 0.015). PVCS performed after corticosteroid therapy showed resolution of bile duct stenosis and dilated, tortuous, or partially enlarged vessels, as well as resolution of friability in all patients with IgG4-SC.

Conclusion

Cholangioscopy was useful in differentiating IgG4-SC from PSC. In addition, monitoring the patterns of proliferative vessels on PVCS may be useful to differentiate IgG4-SC from cholangiocarcinoma.     

When and how does nonstructural mechanical prosthetic heart valve dysfunction occur?

Objective: We diseuss the clinical aspects related to mechanical valve dysfunction based upon 20 years' experience of our cases.Methods: Between January 1982 and December 2001, 21 patients underwent surgical interventions because of mechanical valve dysfunction. Thirteen men and 8 women (mean age 47±20, range 3–75 years-old), were included.Results: Mitral valve dysfunction was observed in 12 patients. Prosthetic valve stenosis occurred in 6 patients between 2 and 224 months, and paravalvular leaks in 4 between 1 and 71 months after surgery. The other 2 patients suffered from hemolytic anemia with mild paravalvular leaks between 102 and 104 months after surgery. Aortic valve dysfunction was observed in 8 patients including 3 stenotic lesions between 48 and 97 months and 5 paravalvular leaks between 3 and 150 months after surgery. Tricuspid regurgitation was seen in 1 patient with a huge right atrium at 42 months after surgery. Early death was seen in 1 patient. Late death occurred in 1 patient, 17 years after operation. Survival, including early death, was 95.2% at 10 and 15 years. One valve-related complication of bioprosthetic valve endocarditis at the mitral and tricuspid positions occurred 12 years after a reoperation. The freedom from complications was 95.2% at 10 years, and 76.2% at 15 years.Conclusions: Paravalvular leaks without overt endocarditis had latency periods of up to 12 years, and pannus formation was observed 20 years after surgery. Early and late clincal results after surgery for mechanical valve dysfunction were excellent.     

Does increased nuchal translucency indicate a fetal abnormality? A retrospective study to clarify the clinical significance of nuchal translucency in Japan

The results of a chromosomal test by genetic amniocentesis in 58 cases with an increased nuchal translucency (NT; > or =3 mm thickness) revealed 47 cases showing a normal karyotype (81%) and 11 cases (19%) showing an abnormal karyotype. However, the cases of a normal karyotype with increased NT also included those with fetal abnormalities. Among the 49 cases in which NT was observed during the first trimester and then subsequently disappeared, chromosomal abnormalities were observed in five, and fetal abnormalities other than chromosomal abnormalities were observed in two. Meanwhile, all nine cases in which an increased NT remained or in which NT continued to increase in size during the second trimester were diagnosed as having cystic hygroma, and chromosomal abnormalities were found in six cases (67%). It should be noted that the shape of increased NT includes NT with a notch (notched NT) and NT without a notch (smooth NT). Among the 20 cases of notched NT, chromosomal abnormalities were observed in eight (40%), and cystic hygroma was observed in nine (45%). On the other hand, among the 38 cases of smooth NT, chromosomal abnormalities were observed in three (7.9%), but no cystic hygroma was observed. Our results confirm that increased NT does not always indicate a fetal abnormality. Whether NT thickness should be measured as a screening tool for fetal abnormalities remains controversial. However, increased NT may be detected by chance, because a maternal-fetal medical examination using ultrasonography is usually performed in Japan. It is therefore considered to be extremely important to establish a system in which cases are referred to obstetricians who are licensed clinical genetic specialists to obtain appropriate genetic counseling whenever increased NT is clinically observed.