An Unusual Case of Left Ventricular Outflow Tract Obstruction after Aortic Valve Replacement.

Surgical valve replacement improves the symptoms and prognosis of patients with valvular heart diseases. Aortic regurgitation elicits volume overload that causes enlargement of the left ventricle (LV), while the LV size often shrinks to near normal after aortic valve replacement (AVR), which is referred to as "reverse remodeling". We experienced a case in which LV outflow tract (LVOT) obstruction became apparent after AVR, resulting in worsening of heart failure. A 65-year-old man who had undergone surgical AVR for aortic valve regurgitation 15 months previously exhibited dyspnea on effort accompanied with severe LVOT obstruction. With double pressure catheters, we directly recorded an augmented pressure gradient in the LVOT and rapid relief of the obstruction by intravenous administration of the anti-arrhythmic drug cibenzoline. Since the considerable LV hypertrophy had been indicated by an electrocardiogram and echocardiography before AVR, we suspected that dilation of the LV chamber due to aortic valve regurgitation could have masked the subclinical LVOT obstruction, which became clinically evident after LV size reduction due to reverse remodeling after AVR.

Chronic Thromboembolic Pulmonary Hypertension Complicated by Giant Pulmonary Artery Aneurysm.

A pulmonary artery (PA) aneurysm is an extremely rare condition that can be idiopathic or secondary. Only a few reports on giant PA aneurysms associated with chronic thromboembolic pulmonary hypertension (CTEPH) are available in the literature. Here, we present a case of CTEPH associated with a secondary giant PA aneurysm detected by autopsy. A 68-year-old woman was diagnosed with pulmonary hypertension (PH) and a PA aneurysm with a diameter of 7.5 cm 7 years before admission. CTEPH was suspected as the cause of PH; however, she refused to undergo surgical treatment. Although her condition improved temporarily with pulmonary vasodilators, she had recurrence of heart failure and died because of the deterioration of her general condition. An autopsy revealed a giant PA aneurysm without medial degeneration, suggesting a secondary PA aneurysm associated with PH. Histological findings indicated multiple organized thrombi with recanalization in the PA bilaterally, and CTEPH was diagnosed as the cause of PH. Although rare, when a PA aneurysm is detected, it is important to consider that CTEPH might be associated with a giant PA aneurysm. A better understanding of this condition is necessary to improve the therapeutic strategy.

Food allergy in nursery children of Kawasaki city, Japan.

BACKGROUND: Food allergies are often life threatening. In order to establish appropriate food allergy measures in nursery children, it is important to analyze local epidemiological data on the food allergy prevalence in nursery children. However, no such data are currently available for the city of Kawasaki, Japan. OBJECTIVE: The present study retrospectively evaluated food allergy prevalence among nursery children in Kawasaki city. METHODS: Data from children with food allergies requiring food avoidance in the nurseries of Kawasaki city between 2007 and 2016 were evaluated. RESULTS: From 2007 to 2016, the prevalence of food allergies among nursery children in Kawasaki city increased from 2.7% to 5.3%. The increase of food allergy prevalence was higher in 2-5 year-old children than in 0-1 year-old children (2.0% to 4.7% vs. 5.3% to 7.0%, respectively). The top five most common food allergies were hen's egg (73.0%), cow's milk (29.3%), nuts (9.7%), soy (8.9%), and wheat (6.5%). Hen's egg was consistently identified as a causative food of food allergy in more than 70% (73.0-89.1%) of food avoidance cases over the 10 year period. The increase of egg allergy prevalence was higher in 2-5 year-old children than in 0-1 year-old children (1.7% to 3.2% vs. 5.2% to 6.0%, respectively). CONCLUSIONS: Food allergies, to hen's egg in particular, have increased considerably among nursery children in the city of Kawasaki, Japan, and that increase was higher among older children.

Various Cardiac Abnormalities Caused by Bacterial Myocarditis.

A 69-year-old woman without any past disease history was hospitalized for heart failure. After hospitalization, she showed myocardial infarction, atrioventricular dissociation, and cardiac dysfunction, and finally she passed away despite intensive care. Autopsy revealed that the cardiac abnormalities were due to bacterial myocarditis possibly resulting from urinary tract infection by E. coli. Although bacterial myocarditis is rare in developed countries, we should consider its possibility when patients show various cardiac abnormalities with bacterial infection.

Adrenal insufficiency causes life-threatening arrhythmia with prolongation of QT interval.

A 63-year-old woman who had hypopituitarism was re-admitted to our hospital because of fever, diarrhea and disturbance of consciousness with life-threatening arrhythmia due to prolongation of the QT interval. She has been treated with hydrocortisone consequently, and has shown few ventricular arrhythmias with normalization of the QT interval. There have been several reports showing the case of prolonged QT interval with adrenal insufficiency, but there are few reports of isolated adrenocorticotropic hormone deficiency without any electrolytes imbalance that showed polymorphic ventricular tachycardia associated with QT prolongation. We discuss some possible mechanisms of how adrenal insufficiency causes life-threatening arrhythmia. Since lack of glucocorticoid hormone might induce prolongation of the QT interval, patients with adrenal insufficiency should be paid attention as candidates of lethal arrhythmias particularly when exposed to excessive stresses.

Measures for food allergy emergency in nurseries.

Background: As food allergy potentially can induce life-threatening anaphylaxis, measures for food allergy are required at nurseries caring for food allergy children, but a large-scale factual investigation has not been carried out. Objective: We evaluated measures for food allergy emergency in nurseries. Methods: A questionnaire survey regarding emergency measures in all authorized nurseries (411 facilities including 20,586 children) was conducted in Kawasaki city, Japan. Results: The recovery rate of the questionnaire was 46.5%, which include 14,343 children of 191 facilities in total. A total of 637 children (4.4%) in 157 facilities (82.2%) requires elimination diets that were suggested by physicians. Among them, 22 children had been suggested to undergo the use of epinephrine auto-injection kit for emergency. 161 facilities (84.3%) had set a specific manual for emergency of food allergy. Emergency cases over the past one year were 4 cases and there was no case that had been suggested to use epinephrine auto-injection kit. All were anaphylaxis and the causes of these included 2 accidental digestion of culprit foods and the causes of other 2 cases were unknown. A case who required no elimination diet showed first episode of anaphylaxis. All cases were recovered. Conclusion: High percentage of nurseries in Kawasaki city has cared for food allergy children. While many children with food allergy have been in nurseries, only several cases of anaphylaxis have been reported for a 1 year. Among 4 cases of anaphylaxis, no specific cause has been recognized in 2 cases and 1 case has been the first episode of anaphylaxis. While most of nurseries have set specific measures for emergency of food allergy, there is certain possibility that nursery staffs can encounter the first episode of anaphylaxis even if there is no food allergy child. For all nurseries, emergency measures for food allergy are vital.

Autopsy case of Dubin-Johnson syndrome with pneumonia and abetalipoproteinemia-like lipid profile.

We report the autopsy of a 79-year-old Japanese woman with Dubin-Johnson syndrome accompanied by pneumonia, an abetalipoproteinemia-like lipid profile and acanthocytosis. On admission, physical examination of the patient revealed malnutrition. Blood tests revealed marked inflammatory changes and mild liver dysfunction. Chest X-ray indicated bilateral pneumonia. Total cholesterol, low-density lipoprotein (LDL) cholesterol and triglyceride levels were 89 mg/dL, 5 mg/dL and 6 mg/dL, respectively. Peripheral blood smears revealed numerous acanthocytes. Despite the administration of antibiotics and nutritional support, the patient died. Autopsy revealed a black liver, atrophy of fat tissue on the mesentery, and pneumonia with bilateral pleural effusion. We believe that the abetalipoproteinemia-like lipid profiles in this case were caused by malnutrition and the inflammatory changes rather than the direct effects of Dubin-Johnson syndrome. We base this conclusion on the following three findings: 1) the patient's lipid profile before hospitalization was in the normal range, 2) her serum LDL cholesterol and triglyceride levels gradually increased after nutritional support began, and 3) blood tests revealed marked inflammatory changes (C-reactive protein 9.0 mg/dL; interleukin-6 16.4 pg/mL). This case provides important information that enhances our understanding of lipid metabolism under conditions of malnutrition and inflammation.

Impact of Oral Treatment on Physical Function in Older Patients Hospitalized for Heart Failure: A Randomized Clinical Trial.

BACKGROUND: Frailty is a characteristic of older patients with heart failure, who undergo functional decline during hospitalization. At present, continuous intravenous infusion of diuretics is widely used for the treatment of hospitalized patients with heart failure. In this prospective, randomized, open-label controlled trial, we tested whether an early switch from continuous intravenous infusion therapy to oral treatment with diuretics prevents functional decline in patients hospitalized for heart failure. METHODS: A total of 59 patients hospitalized for heart failure were randomized to either continuous intravenous infusion (n = 30) or oral medication (n = 29) within 48 h of admission. The primary outcome was the Barthel index, a universally utilized scale to assess the functional status of patients in their activities of daily living, assessed at 10 days. Secondary outcomes included the number of daily steps counted using pedometers and average hospital costs. RESULTS: Barthel index scores were significantly higher in the oral medication group than in the intravenous group (78.1 ± 20.8 vs. 59.6 ± 34.2, P = 0.029). The number of daily steps was significantly higher in the oral treatment group relative to the intravenous group (P < 0.001), and the average hospital costs were similar between the randomized groups. Multivariate analysis revealed that oral medication was a significant independent predictor of Barthel index score at day 10, and the number of daily steps was significantly associated with the patient's functional outcome. CONCLUSIONS: This trial showed that, in patients hospitalized for heart failure, oral medication increased functional independence during hospitalization compared with sustained continuous intravenous infusion, most likely because the release from the infusion line enabled the patients to be more mobile. Notably, these beneficial effects were achieved without increasing hospital costs.

Association of change in the type of job with prevalence of components of the metabolic syndrome-special reference to job stress.

OBJECTIVE: It is well established that job stress is a leading cause of cardiovascular disease. The relationship with the metabolic syndrome, however, has received only limited attention. The present study was designed to investigate associations between change of the type of job and the prevalence of metabolic syndrome components from the aspect of on-the-job stress and alteration in life style. METHODS: Thirty-six male workers of the manufacturing department were transferred to the carsales department at the same automobile company in 1992 to 1993. These same workers were transferred back to the manufacturing department after two years. We compared the first health-check data before the transfer in 1992 (Term A), a second set of data two years after transfer in 1994-95 (Term B) and a third set of data two years following transfer back to the manufacturing department in 1996-1998 (Term C). The workers were requested to provide information about drinking and smoking habits, and answer Karasek's questionnaire and a simple stress questionnaire in order to clarify the possibility of job stress in occurrence of the metabolic syndrome, defined in terms of obesity, hypertension, dyslipidemia, and impaired glucose tolerance as components. RESULTS: Five workers had two or more components of the metabolic syndrome before the transfer to the car-sales department (Group I). One demonstrated improvement, three no change, and one increase in symptoms from A to B. Seven workers had more than two components after the transfer to car-sales department (Group II), and six of them exhibited decrease two years following transfer back to the manufacturing department. Five of them also showed elevated liver enzymes in serum with the appearance of the components, and three of them demonstrated recover. Three workers had two components of the metabolic syndrome only at time point C (Group III), while the remaining 21 workers had 0 to one component throughout the observation period (Group IV). Amount of drinking and smoking increased significantly when working in the sales department but these items returned to the previous values after rejoining manufacturing, though differences were not observed between workers with (Group II) and without (Group IV) components of the metabolic syndrome. Body mass index (BMI) and alanine aminotransferase (ALT) increased significantly when workers moved to the sales department and that was significant in Group II as compared to Group IV. Three components of Karasek's JCQ changed significantly during job transfer, though differences were not observed between the workers with (Group II) and without (Group IV) components of the metabolic syndrome. Logistic regression analysis with age, lifestyle, Karasek's JCQ, and ALT revealed that elevation of ALT value was associated with having two or more components of metabolic syndrome, while hours of sleep demonstrated an inverse association. CONCLUSION: Elevated ALT and reduction of sleep hours may be associated with development of the metabolic syndrome in workers who change their type of job.

Heparin-induced thrombocytopenia and thrombosis in a patient with polycythemia vera.

A 75-year-old Japanese woman with polycythemia vera was admitted to our hospital in January 2003 with suspected pulmonary thromboembolism. After administration of heparin, platelet count decreased from 1,694 x 10(9)/l on admission to 60 x 10(9)/l on hospital day 14. The patient developed acute limb embolism and transient cerebral ischemic attack on days 17 and 25, respectively. Signs and symptoms mimicked those of disseminated intravascular coagulation. Antibodies against heparin and platelet factor 4 complexes were detected in serum, and a diagnosis of heparin-induced thrombocytopenia and thrombosis was made. Argatroban treatment improved thrombocytopenia and hypercoagulable state.