Patients with chronic kidney disease (CKD) have an increased risk for death from cardiovascular disease (CVD). They have multiple metabolic abnormalities that may accelerate atherosclerosis, such as hypertension, insulin resistance, and dyslipidemia, along with other CKD-related risk factors. In addition, a considerable proportion of patients with advanced stages of CKD are malnourished, presenting “metabolic syndrome with malnutrition”. The presence of malnutrition/inflammation dramatically changes the apparent relationship between CVD death risk and some risk factors. For example, in stage 5 CKD patients on hemodialysis, a higher body mass index and a higher plasma cholesterol are predictors of better survival. To understand the paradoxic epidemiology, we should recognize risk factors for occurrence of CVD events and risk factors of fatality after an event. In this article, we review the unique situation of CKD, emphasizing the need of more strict control of both types of risk factors to improve survival of CKD patients.

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Objective We report a risk of worsening of encephalopathy by glycerol infusion when this osmotic agent is used for the treatment of brain edema in patients with adult-onset type II citrullinemia (CTLN2) caused by citrin deficiency.
Patients and Methods We performed a retrospective investigation of 3 patients with CTLN2 treated for brain edema at our institute: a 31-year-old male patient and a 40-year-old female patient received treatment for encephalopathy-related brain edema with 10% glycerol infusion and 20% D-mannitol, and a 40-year-old male patient received only 20% D-mannitol infusion. In addition, we also performed a retrospective study in 11 CTLN2 patients reported previously (8 patients treated with 10% glycerol, 2 treated with 10% glycerol and 20% mannitol, and 1 treated with 20% mannitol).
Results The 12 patients treated with 10% glycerol, including 2 of our patients, died due to rapidly deteriorating encephalopathy and brain edema. On the other hand, the 2 patients who received only 20% D-mannitol, including one of our patients, recovered with the disappearance of brain edema.
Conclusion In CTLN2 patients, glycerol infusion seems to be associated with exacerbation of encephalopathy itself and only mannitol should be used for the treatment of brain edema in patients with this disorder.

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Objective To investigate whether or not the newly revised classification of the severity of idiopathic interstitial pneumonia (IIP) is appropriate with respect to quality of life (QOL).
Methods The association between the subscale of Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and pulmonary function or serum marker was analyzed using Pearson′s correlation coefficient. The association between the subscale of SF-36 and the previous or newly revised classification of the severity of IIP was analyzed using Spearman′s rank correlation test.
Patients Forty patients with idiopathic pulmonary fibrosis (IPF) were enrolled.
Results The mean deviation value scores for 7 items, excluding bodily pain (BP) in SF-36 were below the national reference values. % vital capacity (VC) was correlated with the 7 items excluding BP. However, neither serum LDH nor KL-6 values were correlated with any item in SF-36. According to the new or previous classification of the severity, severity was correlated with physical function, limitation of role functioning related physical problems and general health (GH); the correlation coefficient with the new one was slightly higher than the previous one. Based on these results, we established a unique draft on the classification of the severity. %VC <70% was added as an item for the newly revised classification in our draft. In our draft, there was rank correlation between the 7 items, excluding BP, in SF-36 and severity.
Conclusion With respect to QOL, the newly revised classification of the severity of IIP was not satisfactory, but the hypoxemia during exercise in patients with resting PaO₂ >80 Torr and reduction of VC were found to be important factors.

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Objective In the current studies, we investigated the clinical effects of long-term macrolide antibiotic therapy for patients with chronic small airway disease (CAD) that clinically and radiologically mimics but is pathologically distinct from diffuse panbronchiolitis (DPB).
Patients and Methods Twenty-one Japanese patients were selected on the basis of clinical criteria for DPB and were categorized as DPB or CAD following histological evaluation of surgical lung biopsies. All patients received long-term macrolide therapy, and therapeutic results were compared for the DPB and CAD groups.
Results Clinical, laboratory, radiological, and bacterial features, as well as neutrophilia in bronchoalveolar lavage fluid were strikingly similar in both groups. Long-term treatment with macrolides improved the clinical symptoms and PaO₂ in both groups. There was a significant improvement in forced expiratory volume in one second (FEV₁), vital capacity (VC), and %VC in patients with DPB but not in patients with CAD. Neutrophilia in bronchoalveolar lavage fluid was also reduced following therapy in DPB patients but was refractory in CAD patients.
Conclusion Based on the different responses to macrolides, CAD might be associated with conditions distinct from those of DPB. Nevertheless, low-dose macrolide therapy may be applied in CAD to achieve clinical improvement, such as in respiratory symptoms and PaO₂.

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The true incidence of hepatocellular carcinoma (HCC) in patients with primary biliary cirrhosis (PBC) remains undetermined due to limited epidemiological studies and some conflicting results. Some studies indicated that in PBC, male gender, cirrhosis, hepatitis C virus (HCV) superinfection, and history of blood transfusion are associated with the development of HCC, and the occurrence of HCC in the early stage of PBC is rare. We present herein a 75-year-old male patient with stage I PBC who developed oropharyngeal squamous cell carcinoma, followed by HCC and duodenal adenocarcinoma without hepatitis B or C virus infection. While it could be argued that the concurrence of HCC and stage I-PBC in our patient was coincidental, patients with early stage PBC should be strictly followed up as cirrhotic patients with PBC by monitoring the serum concentration of tumor markers for HCC and appropriate imaging methods.

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We treated a 66-year-old woman with hepatic encephalopathy secondarily induced by an intrahepatic portosystemic venous shunt (IPSVS). In serial observations, the volume of the liver became smaller and encephalopathy could not be controlled with conservative therapy. We occluded the IPSVS successfully using percutaneous transcatheter embolization with micro coils. Following embolization, encephalopathy disappeared and blood flow of all branches of portal vein improved. In cases with an IPSVS without liver cirrhosis, blood flow in the portal vein and liver volume must be followed carefully, and interventional radiology may be considered effective in those who do not show a satisfactory response to conservative therapy.

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We describe a 76-year-old man in whom a gastric tube was used for esophageal reconstruction via the anterior mediastinum after esophagectomy for esophageal cancer. Stenosis of the tube resulted in fluid accumulation, which directly compressed the heart and caused angina-like chest pain associated with ST-segment depression in lead V_2-3 on the electrocardiogram (ECG). Coronary angiography revealed no stenosis. Drainage of the fluid in the gastric tube resulted in immediate relief of symptoms and normalization of ECG. Angina-like chest pain associated with ST segment changes were caused by expansion of the gastric tube and compression of the heart.

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We report a case with pheochromocytoma presenting as recurrent syncope due to hypotension. A 71-year-old man was admitted because of recurrent syncope and paroxysmal hypotension. He was diagnosed as having pheochromocytoma. In spite of a large volume of infusion and insulin therapy, syncope and paroxysmal hypotension continued. We speculated that hypotension was mainly due to vasodilatation caused by excess plasma epinephrine and prescribed a non-selective beta-adrenergic blocker. It stabilized blood pressure and syncope disappeared. The right adrenal tumor was excised and he was discharged in good condition. This case report provides some implications for the management of pheochromocytoma complicated with hypotension.

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Ampulla cardiomyopathy is named after the echocardiographic abnormalities occurring in this condition, characterized by extensive akinesis (ballooning ) of the apical region with hypercontraction of the basal segment of the ventricle. We describe 3 young female anorexia nervosa patients showing evidence of this cardiac complication after hypoglycemia. One case was complicated by echocardiographically confirmed ampulla cardiomyopathy while the other 2 patients showed increases in myocardial enzymes and transient electrocardiographic abnormalities consistent with this complication. The precipitating event for all three patients was hypoglycemic coma, and this is the first case report in which this factor lead to the complication of ampulla cardiomyopathy in anorexia nervosa patients.

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A 69-year-old woman caught a cold resulting in nausea, vomiting, diarrhea and severe anorexia. Then she suffered progressively from dyspnea and leg edema, and finally became delirious. On admission severe hypoglycemia, hypothermia, marked tachycardia, generalized edema, mild jaundice and cachexy were noted. EKG showed atrial fibrillation. A chest X-ray, chest CT and echocardiography showed congestive heart failure. Therapeutic use of diuretics induced shock leading to serious liver dysfunction and disseminated intravascular coagulation. However, combined therapy by intravenous glucose, digitalis, diuretics, anti-fibrinolytic drug and hydrocortisone were effective. Addition of antithyroid therapy brought a further favorable outcome.

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We present a 79-year-old woman with severe hyponatremia secondary to resumption of treatment with paroxetine, a selective serotonin-reuptake inhibitor antidepressant. Confusion and fatigue followed re-initiation of paroxetine after a 3-month hiatus. Hyponatremia, serum hypoosmolality, and urine hyperosmolality strongly suggested the syndrome of inappropriate secretion of antidiuretic hormone. Hyponatremia was quickly resolved after discontinuation of paroxetine and initiation of intravenous normal saline infusion together with oral fluid restriction. This case underscores the importance of monitoring serum sodium in elderly patients taking paroxetine, whether this represents a new prescription or reintroduction of the drug.

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Paradoxical embolism may occur in patients with acute pulmonary thromboembolism, when patent foramen ovale (PFO) coexists with pulmonary hypertension (right-left shunt). There have been few case reports of paradoxical embolism in peripheral arteries coincident with acute pulmonary thromboembolism. Here, we describe a case of paradoxical peripheral embolism associated with PFO complicated by acute pulmonary thromboembolism. The patient had severe peripheral ischemia due to a massive thrombus and was treated successfully by peripheral thrombectomy, thrombolysis, implantation of a permanent inferior vena cava filter and anticoagulation.

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A 66-year-old woman who had undergone one year′s treatment for pulmonary nontuberculous mycobacterial disease due to Mycobacterium avium (rifampicin, ethambutol, clarithromycin, streptomycin→levofloxacin) five years earlier was admitted to our hospital because of continuous fever and a newly detected abnormal chest shadow, which was like a fungus ball in the right upper lobe on chest computed tomography in the giant cavitary lesion caused by pulmonary Mycobacterium-avium complex (MAC) disease. A diagnosis of chronic necrotizing pulmonary aspergillosis (CNPA) complicated by pulmonary MAC disease was made because Aspergillus niger was isolated from several sputum specimens, anti-aspergillus antibody was positive, and clinical symptoms such as fever, were disclosed with the radiological finding of a fungus ball-like shadow and an infiltration shadow around the cavity. The patient had received various forms of antifungal chemotherapy, but the clinical effect had been poor. Since then, she had been slowly worsening. Although mycetomas, with the typical appearance of a fungus ball on a chest radiograph, have been reported to easily form in cavitary lesions caused by previous pulmonary tuberculosis, we believe, as illustrated by the present case, that they could also form in such lesions caused by pulmonary MAC disease, since the frequency of pulmonary nontuberculous mycobacterial disease has recently been increasing in comparison with that of pulmonary tuberculosis.

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Recently, a cardiac disorder characterized by ballooning and hypokinesis at the apex has been described as takotsubo (ampulla-shaped) cardiomyopathy. We encountered a patient with a rare case of takotsubo cardiomyopathy associated with microscopic polyangiitis. A 70-year-old woman suddenly presented with ventricular dysfunction during the active phase of microscopic polyangiitis. The findings on echocardiograms and electrocardiograms were consistent with those of takotsubo cardiomyopathy. The ventricular dysfunction completely resolved after treatment with 40 mg/day of prednisolone and methylprednisolone pulse therapy. This unique type of cardiomyopathy can be a complication of microscopic polyangiitis.

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A 15-year-old Japanese man was referred for evaluation of heart failure. Conventional heart failure therapy had little effect, and severe left ventricular dysfunction as well as elevated erythrocyte sedimentation rate persisted. Magnetic resonance angiography showed aortic dilatation with wall thickening characteristic of Takayasu′s arteritis. An endomyocardial biopsy specimen revealed infiltration of natural killer cells and γδ T lymphocytes, which play major roles in vascular injury of Takayasu′s arteritis. Prednisolone administration provided great benefits to cardiac function. These findings suggest that autoimmune cytotoxic mechanisms similar to those in arterial tissue may contribute to cardiac impairment in Takayasu′s arteritis.

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Familial Mediterranean fever (FMF) is an inherited inflammatory disease occurring mainly in Mediterranean and Middle Eastern populations. FMF is caused by mutations in the MEFV gene that encodes pyrin/marenostrin. Here, we report a Japanese female FMF patient with heterozygosity for the compound pyrin E148Q/M694I showing recurrent fever, serositis or delay in skin wound healing. Her father and elder sister were heterozygous for pyrin variant M694I alone and sometimes suffered from mild fever or delay in wound healing, but her mother was heterozygous for pyrin variant E148Q alone and had no symptoms. This suggested that the inheritance of FMF occurred not only in an autosomal recessive manner but also in an autosomal dominant manner in this Japanese family, and the severity of the disease differed among the family members in relation to the mutation. In the treatment of FMF, colchicine, reserpine or prazosin hydrochloride have been reported to prevent the attacks, but, in our patient such drugs were ineffective or caused side effects, and only the anti-allergic drug azelastine was of benefit in relieving the attacks.

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The patient, a 30-year-old housewife, visited a nearby doctor in mid August 2002 because of weight loss and neck swelling. HIV tests done at the hospital were positive. She was referred to and admitted to our hospital on October 2 for detailed examination and treatment of the neck tumor. A coat of epithelial debris extended from the oral cavity to the pharynx and an abscess and a fistula were found in the left tonsil. After hospitalization, an abscess culture revealed the presence of acid-fast bacteria, which was identified as Mycobacterium peregrinum. Treatment with imipenem and clarithromycin resulted in the normalization of CRP (0.1 mg/dl), on day 5 of treatment. The patient was discharged from the hospital after treatment for 2 weeks with imipenem and clarithromycin. Thereafter, the patient received continuous treatment with faropenem and clarithromycin for 4 more weeks, and has shown no signs of recurrence for 11 months to date. Only a few cases of infection with this bacterial strain have been reported. This infection is difficult to treat because most antituberculosis agents are not effective against it and there is limited availability of effective antibiotics. Medical treatment of infection caused by Mycobacterium peregrinum may be useful in such cases.

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