Long-term prognosis of Kawasaki disease patients with coronary artery obstruction

Summary The prognosis of coronary artery obstruction was studied in patients with Kawasaki disease. Between May 1973 and December 1987, coronary artery obstruction was diagnosed by coronary angiography in 30 patients (21 males, 9 females), of whom, only 8 (26.7%) had clinical symptoms. One patient died after 9 years of illness. Two complained of frequent chest pain, which disappeared after bypass surgery in one case and spontaneously in the other. Five had symptomatic myocardial infarction. Myocardial ischemia was diagnosed in 31.8% by treadmill stress testing, but was well demonstrated in 85.7% by thallium-201 myocardial tomography. Frequent ventricular premature beats, Wenckebachtype atrioventricular block, and ST-segment depression accompanied by chest pain were recognized by 24-h Holter monitoring. In the past, the methods used to determine the prognosis of Kawasaki disease patients with coronary artery obstruction were not adequate. However, the examinations used in this study revealed an improved ability to determine the prognosis in this disease. Myocardial tomography, in particular, provided a more accurate evaluation of myocardial damage. Ventricular arrhythmias seem to be a serious problem in these patients. Therefore, careful observation using these tests, especially myocardial tomography and Holter monitoring, should be done even if the patients are free of symptoms.     

Immunohistochemical analysis of hepatic interferon alpha-beta receptor level: relationship between receptor expression and response to interferon therapy in patients with chronic hepatitis C

Background/Aims: This study aimed to determine the expression level of interferon alpha/beta (IFN-α/β) receptor in the liver immunohistochemically and evaluate its usefulness in predicting the outcome to IFN therapy in patients with chronic hepatitis C.Methods: The level of IFN-α/β receptor expression was determined in immunoperoxidase-stained pretreatment sections of 55 chronic hepatitis C patients later treated with IFN. We used liver biopsy specimens and mouse monoclonal anti-human IFN-α/β receptor antibody. Quantitative analysis of immunostaining was performed by image analysis software. The level of IFN-α/β receptor was expressed as Unit (U). Sustained responders were patients who showed persistent disappearance of serum HCV-RNA during the 6-month period after treatment, while non-responders showed persistence of viremia after therapy.Results: Positive immunostaining was observed in the cytoplasm of hepatocytes. The mean expression level of hepatic IFN-α/β receptor in sustained responders (2.65±1.11 U, n=15) was significantly (p<0.001) higher than in non-responders (1.61±1.05 U, n=40). A significant decrease in IFN-α/β receptor expression level was observed in patients with advanced liver fibrosis. In patients with low level viremia (pretreatment serum HCV-RNA <1 Meq/ml, n=18), the level of IFN-α/β receptor in sustained responders (2.89±1.12 U, n=11) was significantly (p<0.01) higher than in non-responders (0.93±0.33 U, n=7).Conclusions: Our results suggest that measurement of the level of hepatic IFN-α/β receptor in patients with chronic hepatitis C might be useful for predicting the response to IFN therapy. Resistance to IFN therapy in patients with chronic hepatitis C might be due to low levels of hepatic IFN-α/β receptor.     

Acylated ghrelin level in patients with OSA before and after nasal CPAP treatment

Background and objective: Patients with newly diagnosed OSA have been reported to have recent weight gain prior to diagnosis. Ghrelin stimulates food intake and increases weight gain. Plasma ghrelin is decreased in obese and increased in lean individuals. Of the two circulating forms of ghrelin, acylated and unacylated, the former is thought to be essential for the biological activity of ghrelin. Methods: The plasma levels of the two forms of ghrelin were measured in 21 OSA patients (with a mean of 46.2 sleep‐disordered events/h) before and after 1 month of nasal CPAP (nCPAP) treatment, and were compared with those in 14 untreated OSA patients and 13 individuals without OSA. Results: The BMI was significantly higher in the 21 OSA patients than in the non‐OSA group as were the baseline acylated (11.4 ± 5.86 vs 7.19 ± 3.80 fmol/mL, P = 0.03) and unacylated (84.2 ± 50.6 vs 48.3 ± 23.2 fmol/mL, P = 0.02) ghrelin levels. The total ghrelin level was positively correlated with the number of sleep‐disordered breathings (P = 0.002). After 1 month of nCPAP treatment, the acylated ghrelin level significantly decreased (P = 0.02) while the unacylated ghrelin level did not (P = 0.09). Conclusions: Treatment of OSA may play an important role in the management of obesity in these patients by reducing the acylated ghrelin level.     

Liver transplantation for hepatocellular carcinoma: the Japanese experience

Despite the wide spectrum of selection criteria used by living donor liver transplantation (LDLT) centers in Japan, LDLT for hepatocellular carcinoma (HCC) can achieve an acceptable outcome comparable to the outcome for deceased donor liver transplantation (DDLT) for HCC. One of the most crucial considerations in liver transplantation for HCC is the advent of expanded criteria that allow more patients with HCC to receive the organs and offer similar or even better results compared to the Milan of UCSF criteria. Expanded criteria for HCC are proposed from three single-center and one multicenter study in Japan. These criteria are based on the independent predictors for outcome derived from the analyses of the pretransplant factors and explant pathology. The beneficial effect of those proposed criteria can be predicted by the inclusion rates of the patients compared to the Milan or UCSF criteria in the same cohort and the outcome for those included patients. While application of the UCSF criteria increases the inclusion rate compared to the Milan criteria by 5–10%, these proposed criteria increase the inclusion rates by 5–54% compared to the Milan criteria. The higher inclusion rates compared to the application of the Milan criteria are achieved by criteria including tumor markers, either AFP or PIVKA II or both. Inclusion of tumor markers in addition to parameters of tumor morphology might be the key to establish the best criteria for liver transplantation for HCC.     

Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011

Background

IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.

Methods

Two IgG4-RD study groups, the Umehara and Okazaki teams, were organized by the Ministry of Health, Labor and Welfare Japan. As IgG4-RD comprises a wide variety of diseases, these groups consist of physicians and researchers in various disciplines, including rheumatology, hematology, gastroenterology, nephrology, pulmonology, ophthalmology, odontology, pathology, statistics, and basic and molecular immunology throughout Japan, with 66 and 56 members of the Umehara and Okazaki teams, respectively. Collaborations of the two study groups involved detailed analyses of clinical symptoms, laboratory results, and biopsy specimens of patients with IgG4-RD, resulting in the establishment of comprehensive diagnostic criteria for IgG4-RD.

Results

Although many patients with IgG4-RD have lesions in several organs, either synchronously or metachronously, and the pathological features of each organ differ, consensus has been reached on two diagnostic criteria for IgG4RD: (1) serum IgG4 concentration >135?mg/dl, and (2) >40% of IgG+ plasma cells being IgG4+ and >10?cells/high powered field of biopsy sample. Although the comprehensive diagnostic criteria are not sufficiently sensitive for the diagnosis of type 1 IgG4-related autoimmune pancreatitis (IgG4-related AIP), they are adequately sensitive for IgG4-related Mikulicz??s disease (MD) and kidney disease (KD). In addition, the comprehensive diagnostic criteria, combined with organ-specific diagnostic criteria, have increased the sensitivity of diagnosis to 100% for IgG4-related MD, KD, and AIP.

Conclusion

Our comprehensive diagnostic criteria for IgG4-RD are practically useful for general physicians and nonspecialists.     

Seven‐action approaches for the management of hypertension in Asia – The HOPE Asia network

Asia is a large continent and there is significant diversity between countries and regions. Over the last 30 years, absolute blood pressure (BP) levels in Asia have increased to a greater extent than those in other regions. In diverse Asia‐Pacific populations, for choosing an Asia‐specific approach to hypertension management is important to prevent target organ damage and cardiovascular diseases. In this consensus document of HOPE Asia Network, we introduce seven action approaches for management of hypertension in Asia.     

Multiple floppy valves with all cardiac valves prolapsing: Clinical course and treatment

Summary Two cases with prolapse of all four cardiac valves are described and compared with two similar ones previously reported. The severity and progression of regurgitation of each of the valves differed by case, despite having similar echocardiographic findings consistent with the diagnosis of multiple floppy valves. Two of the four patients had their aortic valve replaced because of severe regurgitation: the excised valves revealed myxomatous degeneration. None of the patients had any stigmata of Marfan or Ehlers-Danlos syndrome, except for the presence of hyperextensive joints. There may be an unknown collagen disorder that caused floppiness in all the valves.     

Nonsecretory primary plasma cell leukemia successfully treated with VAD and MP therapy

A 78-year-old woman was admitted to our hospital because of lumbago and appetite loss. Blood analysis revealed anemia, hypercalcemia and circulating plasma cells. Bone marrow aspiration showed an elevated ratio (43%) of plasma cells, which expressed CD38 in the absence of CD19 and CD56 expression. Spinal MR imaging revealed multiple compression fractures and suggested diffuse invasion of plasma cells into the spinal bodies. No M-protein was detectable in serum or urine by immunoelectrophoresis and immunofixation, but cytoplasmic M-protein (IgG-kappa) was detected by enzyme antibody staining. On the basis of the history and data, nonsecretory primary plasma cell leukemia was diagnosed. First, the patient was given modified VAD therapy (vincristine, doxorubicin, and prednisolone) and complete remission was obtained. Then MP therapy (melphalan and prednisolone) was instituted, and remission has since been maintained for 11 months. Like many other cases of primary plasma cell leukemia, this case suggests that CD56 may act as an adhesion molecule between neoplastic plasma cells and bone marrow stromal cells. Our experience with this exceedingly rare case suggests the superiority of combination chemotherapy as an induction therapy and the effectiveness of MP therapy as maintenance therapy for this disease.     

Hot spring bath and Legionella pneumonia: an association confirmed by genomic identification

A 59-year-old man developed pneumonia 9 days after bathing in a hot spring spa. Bilateral shadows on his chest radiograph rapidly progressed after admission. He was successfully treated with erythromycin and rifampicin. Legionella pneumophila serogroup 6 was recovered from an intratrachial specimen and a significant elevation was observed in a paired indirect fluorescent antibody to Legionella. Persistent slight fever and chest rentogenographic shadows resolved after administering low-dose prednisolone to treat organizing pneumonia shown by transbronchial lung biopsy. The same serotype of Legionella was recovered from the water of the hot spring spa where the man had bathed. When the extracted DNA of these two strains showed identical restriction fragments by pulsed-field gel electrophoresis, we had direct evidence that hot spring spas can be a source of Legionella pneumonia.