Nephrotic syndrome is a syndrome that exhibits hypoproteinemia and proteinuria. Richard Bright (1789-1858) in the United Kingdom first showed an association between kidney disease and edema as “Bright′s disease” in 1827 and classified it into three groups. Minimal change nephrotic syndrome is considered to be one of the three groups. In the early 20th century, Friedrich von Müller established the concept of nephrotic (Nephrose) diseases out of “Bright′s disease”. He had already pointed out that the nephrotic syndrome is a syndrome rather than a disease. Although more than 100 years have passed, the cause of nephrotic syndrome remains unknown today. Herein, we introduce the latest findings regarding the treatment and the immunological background for the cause of nephrotic syndrome, especially for Minimal Change Nephrotic Syndrome (MCNS) and Focal Segmental Glomerulosclerosis (FSGS) that are considered to be among the main causes of Nephrotic syndrome among children.

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Background: Several ECG criteria have been proposed to differentiate left from right ventricular outflow tract (OT) premature ventricular contractions (PVC)/ventricular tachycardia (VT); however, differentiating PVC/VT in left bundle branch block (LBBB) remains challenging. Individual patient differences in cardiac rotation, lead positions, and chest wall size may limit the accuracy of the ECG algorithm. We hypothesized that correcting for sinus rhythm (SR) precordial transition would aid in OTVT localization. Methods: We analyzed the surface ECG patterns of 16 patients (8 men, 8 women; age, 55.7 ± 22.2 years) with right ventricular (RV) OTVT and 8 patients (7 men, 8 women; age, 50.1 ± 16.6 years) with left ventricular (LV) OTVT who underwent catheter ablation. SR and VT morphologies were measured with the same 12-lead ECG system. V2 transition ratios were determined by calculating the percentage R wave during VT (R/R+S) VT divided by the percentage R wave in SR (R/R+S) SR. We also determined the V1 and V2 R/S ratios, the time from V2 R-wave onset to its peak (V2Rp), V1- and V2 R-wave durations, S wave in V5 and V6, the precordial transitional zone, and the lead I ECG QRS pattern during PVCs/VT. Results: The V2 transition ratio was significantly greater for LVOT vs. RVOT (1.50 ± 0.69 vs. 0.18 ± 0.11; P = 0.0004). A V2 transition ratio >1.1 predicted an LVOT origin with a sensitivity of 83% and specificity of 88%. Time to V2Rp was significantly greater for LVOT vs. RVOT (82.50 ± 6.12 vs. 41.2 ± 9.90 ms; P = 0.0003). A V2Rp time >70 ms predicted an LVOT origin with a sensitivity of 100% and specificity of 100%. Conclusions: Electrocardiographic measures, the V2 transition ratio, and V2Rp time can reliably distinguish between an RVOT and LVOT origin in patients with LBBB. These measures may be useful in planning the ablation strategy.

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We evaluated the impact of different types of breast cancer screening methods on the ductal carcinoma in situ (DCIS) discovery rate. One was a population-based screening (A group), and the other was an opportunistic screening group (B group). We performed a retrospective investigation of patients who visited our hospital during 2011. Four hundred and sixty-nine patients visited our hospital for breast cancer screening. Of these patients, 30.3% were diagnosed as having breast cancer by histopathology. The discovery rate of breast cancer was significantly higher in group B than in group A. There was no significant difference between group A and group B in the discovery rate of DCIS. Group A used only mammography. However, 82.3% of the patients in group B underwent mammography plus ultrasonography. These results suggested that the addition of ultrasonography to breast cancer screening is useful to detect breast cancer. However, the addition of ultrasonography does not affect the discovery rate of DCIS.

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Combination therapy with gemcitabine hydrochloride and erlotinib hydrochloride was administered to 4 patients with unresectable advanced recurrent pancreatic cancer, and adverse reactions and compliance were investigated. Anorexia was noted as an adverse event in 3 patients. The protocol was continued by dose reduction of erlotinib in 2 patients. One patient survived up to 23 months. These findings suggest that maintenance of drug compliance can be achieved by adjusting the dose of erlotinib and that this influences the antitumor effect.

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We report a case of a 92-year-old woman with obturator hernia. She was admitted to our hospital due to anorexia that had persisted for a few days. On hospital day 3, her blood pressure suddenly decreased. Computed tomography showed intraperitoneal free air and incarceration of the small intestine into the left obturator foramen; thus, perforation peritonitis associated with left obturator hernia was diagnosed. The patient underwent emergency surgery. Operative findings showed perforation of the intestine secondary to the left obturator hernia and right obturator hernia. The manual pulling method was used for removal of the incarcerated small intestine in the obturator foramen, and partial resection of the small intestine was performed. Both obturator foramens were repaired using a simple nonabsorbable suture. The mortality of perforation peritonitis associated with obturator hernia is high, and early diagnosis using a pelvic CT scan is important to reduce mortality.

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Angiosarcoma is a rare and high-grade malignancy lesion that occurs in the skin including scalp and soft tissues. We present a case of a 70-year-old man who exhibited angiosarcoma of the scalp and was revealed to have metastasis to the lung and the pleura by cytological examination after excision of the lesion. Cytological examination of the pleural fluid showed the following atypical cellular features; signet ring-like cells, intracytoplasmic lumina-like or hobnail cell cluster and a whorl-like pattern. Immunohistochemically, the atypical cells were positive for CD31, D2-40 and vimentin, and negative for CD34, TTF-1, calretinin and CEA. The clinical and cytological findings were consistent with metastatic angiosarcoma. Despite treatments, the patient expired due to respiratory failure, 11 months after the first medical examination.

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Pertuzumab was approved as an anti-HER2 agent in Japan in June 2013. In an overseas clinical trial, pertuzumab combination therapy had significantly better efficacy for time to progression and overall survival in HER2-positive metastatic breast cancer. Here, we describe a case of HER2-positive local advanced breast cancer that showed a pathological complete response to therapy with trastuzumab, pertuzumab and docetaxel.

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[in Japanese]

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