Shigehiro Kitamura

Acute onset of nephrotic syndrome during interferon-α retreatment for chronic active hepatitis C

A 57-year-old woman was scheduled to receive recombinant interferon-α retreatment for chronic active hepatitis C. During the course of therapy, the patient showed rapid onset of oliguria, dizziness, edema, and a pre-shock state. She was subsequently admitted to hospital and was diagnosed as having nephrotic syndrome. After admission, albumin-dominant proteinuria persisted despite the discontinuation of interferon therapy. Light microscopy of a renal needle biopsy specimen showed interstitial lymphoid cell infiltration, but no marked changes of the glomeruli and no staining for immunoglobulin or complement. Electron microscopy showed diffuse effacement of the glomerular epithelial foot processes, leading to a diagnosis of minimal change nephrotic syndrome with interstitial nephritis. Proteinuria resolved after the initiation of oral prednisolone therapy (1 mg/kg per day). The number of patients with chronic hepatitis C requiring interferon retreatment is increasing rapidly. We herein report this rare case of acute onset of nephrotic syndrome during interferon-α retreatment.

公衆浴場での溺水後発症したLegionella pneumophila serogroup 6による劇症肺炎の1例

A 57-year-old male was admitted to our hospital because of high fever, productive cough and dyspnea. Six days prior to admission he had an episode of drowning in a public bath. On admission chest X-ray showed wide-spread pneumonia causing severe respiratory distress for which mechanical ventilatory support was started. Despite chemotherapy including erythromycin and rifampicin his condition continued to deteriorate. Chemistry showed marked elevation of CPK and findings of acute renal failure. He eventually passed away with septic shock. During the course Legionellae remained negative with culture of broncho-alveolar lavage fluid. L. pneumophila serogroup 1 (SG1) antigen in the urine was not detected, and no elevation of serum antibody titer was noted. Culture of the material obtained from the lung abscess at autopsy revealed L. pneumophila SG6 and serum antibody titer against SG6 also was found to be extremely high. With this evidence we concluded that this case of pneumonia was caused by L. pneumophila SG6. We believe this is the first reported case of the SG6 pneumonia in Japan. Another remarkable feature of this case was massive rhabdomyolysis pathologically confirmed after autopsy. Although the pathogenesis of this process has not been clarified, there are several case reports of rhabdomyolysis complicated with Legionnairs disease in the past. Therefore, we should bear in mind and pay careful attention while coping with this disease.

Arteriovenous hemangioma in the middle mediastinum: Report of a case

Hemangiomas in the mediastinum are uncommon, and the majority of these tumors are located in the anterior mediastinum. The present report describes an extremely rare case of a hemangioma in the middle mediastinum. A 40-year-old male patient presented with a mass in the left lower lung field on radiographic screening. Chest computed tomography and magnetic resonance imaging revealed a mass measuring 2.5 cm in diameter, which was close to the descending aorta in the left middle mediastinum. The tumor resection was performed by video-assisted thoracic surgery. The thoracoscopic findings revealed a blackish brown mass with a capsule, which had not invaded the descending aorta or cardiac sac. Histologically, the tumor was composed of typical thick-walled vessels and was therefore classified as an arteriovenous hemangioma.

Benign clear cell “sugar” tumor of the lung in a patient with Birt-Hogg-Dubé syndrome: a case report

BackgroundBirt-Hogg-Dubé (BHD) syndrome is a rare inherited autosomal genodermatosis and caused by germline mutation of the folliculin (FLCN) gene, a tumor suppressor gene of which protein product is involved in mechanistic target of rapamycin (mTOR) signaling pathway regulating cell growth and metabolism. Clinical manifestations in BHD syndrome is characterized by fibrofolliculomas of the skin, pulmonary cysts with or without spontaneous pneumothorax, and renal neoplasms. There has been no pulmonary neoplasm reported in BHD syndrome, although the condition is due to deleterious sequence variants in a tumor suppressor gene. Here we report, for the first time to our knowledge, a patient with BHD syndrome who was complicated with a clear cell “sugar” tumor (CCST) of the lung, a benign tumor belonging to perivascular epithelioid cell tumors (PEComas) with frequent causative relation to tuberous sclerosis complex 1 (TSC1) or 2 (TSC2) gene.Case presentationIn a 38-year-old Asian woman, two well-circumscribed nodules in the left lung and multiple thin-walled, irregularly shaped cysts on the basal and medial area of the lungs were disclosed by chest roentgenogram and computer-assisted tomography (CT) during a preoperative survey for a bilateral faucial tonsillectomy. Analysis of the resected tumor showed large polygonal cells with clear cytoplasm proliferating in a solid pattern. Immunohistochemistry revealed that these tumor cells were positive for microphthalmia-transcription factor, S100, and CD1a but negative for HMB45, indicating that the tumor was a CCST. Genetic testing indicated that the patient had a germline mutation on exon 12 of the FLCN gene, i.e., insertion of 7 nucleotides (CCACCCT) (c.1347_1353dupCCACCCT). Direct sequencing of the FLCN exon 12 using genomic DNA obtained from her microdissected CCST cells clearly revealed loss of the wild-type FLCN sequence, which confirmed complete functional loss of the FLCN gene. On the other hand, no loss of heterozygosity around TCS1- or TSC2-associated genetic region was demonstrated.ConclusionTo our knowledge, this is the first report of CCST of the lung in a patient with BHDS, indicating that CCST should be added to the spectrum of pulmonary manifestations of BHDS.

Drug-induced MPO-ANCA-positive necrotizing crescentic glomerulonephritis preceded by granulomatous hepatitis

A 67-year-old man being treated with allopurinol, furosemide, digoxin, and tamocapril for congestive heart failure and paroxysmal atrial fibrillation was admitted to our hospital because of intermittent fever, general fatigue, and liver dysfunction. On admission, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was positive, and the titer was high. Discontinuation of all four drugs led to improvement of the symptoms, but the patients renal function deteriorated. Liver biopsy showed granulomatous hepatitis (GH), and renal biopsy findings led to a diagnosis of pauci-immune necrotizing crescentic glomerulonephritis (CGN). The patients renal function spontaneously improved without immunosuppressive therapy, and the MPO-ANCA titer decreased. We speculate that some common pathway induced by the drugs, possibly allopurinol, led to the two different clinical diseases, GH and CGN.