The congenital absence of the pectoralis muscle is usually a manifestation of Poland syndrome. However, a nonsyndromic congenital absence of this muscle is rare, and such absences are usually partial and unilateral. A complete or bilateral absence is even rarer. Two young men presented to our outpatient clinic with incidentally noted unilateral flat chest walls. By chest computed tomography, they were diagnosed with a congenital unilateral absence of the pectoralis muscles. They did not show any functional disability of the arms. As the congenital absence of the pectoralis muscles is often associated with leukemia and genitourinary anomalies, it is advised that hematological testing and renal ultrasonography be performed, even in nonsyndromic cases.

Objective: This study aimed to investigate whether interactions between multiple serum cytokines may be implicated in the mechanism of action (MOA) of sublingual immunotherapy (SLIT) for Japanese cedar pollinosis. Methods: A Tokyo Metropolitan Bureau of Social Welfare and Public Health-initiated clinical study of active SLIT involving 202 patients with Japanese cedar pollinosis was jointly conducted by Tokyo Metropolitan Institute of Medical Science and Nippon Medical School between 2006 and 2008. Fifty target cytokines were quantified in serum samples collected at 6 times from baseline to the end of the study, for 300 cytokine measurements in total, using Bio-Plex Pro Human Cytokine Group I/II Panels. Therapeutic outcome was assessed based on nasal symptom scores and quality-of-life questionnaire results. Results: Fifty-five percent of patients were free of symptoms or reported symptomatic improvements by 2 grades or greater after 2 years of SLIT treatment, while 27% showed no improvement or worsening of symptoms. Thirty-eight patients who benefited the most from treatment (responders) as well as 37 patients who benefited the least from treatment (non-responders) were identified and their serum cytokine profiles were compared. Cluster analysis of the 300 cytokine measurements identified 6 cytokine clusters that were strongly correlated with a positive response to treatment, and this correlation was consistent throughout the treatment. Conclusion: Certain cytokine clusters are strongly correlated with a positive therapeutic outcome, suggesting they have a role in the MOA of immunotherapy.

Background: The physical condition of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis (RPGN) is at times too critical for physicians to undertake a renal biopsy. In such cases, physicians need to start treatments without determining the pathological features of the disease. However, the prevalent clinical practice guidelines for ANCA-associated RPGN in Japan do not necessitate the pathological findings in a renal biopsy for determining first-line treatments. The aim of this study was to assess the correlation between the pathological findings and the clinical guideline-based treatments of ANCA-associated RPGN. Methods: We investigated clinical and pathological features of patients who were admitted to our hospital to undergo a percutaneous renal biopsy for the diagnosis of ANCA-associated RPGN and were treated per clinical practice guidelines formulated by the Japanese Society of Nephrology. We divided patients into the following three groups according to their treatments: (a) group A, oral intake of prednisolone (PSL) only; (b) group B, methylprednisolone (mPSL) pulse therapy followed by oral intake of PSL; and (c) group C, mPSL pulse therapy followed by oral intake of PSL and the administration of immunosuppressive agents. We compared the crescent formation ratio (CFR) in glomeruli in each treatment group. Results: The median CFR (%; ±standard deviation [SD]) in group A (8.7%±7.7%) was significantly lower than that in groups B (30.2%±15.7%) and C (71.3%±25.5%); group A vs. group B, P<0.05; group A vs. group C, P<0.0001. In addition, the median CFR in group B was significantly lower than that in group C (P<0.01). The median serum creatinine levels (mg/dL; ±SD) in both groups A (2.03±0.96 mg/dL) and B (1.90±0.86 mg/dL) were significantly lower than that in group C (4.30±1.63 mg/dL); group A vs. group C, P<0.01; group B vs. group C, P<0.01. No significant difference was observed in the serum levels of C-reactive protein and ANCA in each treatment group. Although the pathological features of renal biopsy are not included in the factors in the clinical scores described in the guidelines, guideline-based treatments reflected the severity of the crescent formation in each treatment group. Conclusions: This study suggests that the clinical practice guidelines for ANCA-associated RPGN used in Japan facilitate physicians to determine disease management in patients presenting difficulties in undergoing a renal biopsy.

Objectives: Macrolide therapy is an important conservative therapy for chronic rhinosinusitis, especially in Japan. The mechanism underlying this therapy involves anti-inflammatory and not antimicrobial activity. However, the administration of long-term low-dose macrolides (LTLMs) causes an increase in the number of antibiotic-resistant bacteria. EM900 is a derivative of erythromycin (EM), with anti-inflammatory but not antibacterial effects. It does not induce macrolide-resistant bacteria as shown by LTLM. In the present study, we analyzed the inhibitory effects of EM900 in comparison with those of clarithromycin (CAM) on inflammatory cytokine production in human nasal epithelial cells (HNEpCs). Methods: After HNEpCs were cultured for 4 days, CAM or EM900 was added into the culture, followed by stimulation with tumor necrosis factor (TNF)-α. Interleukin (IL)-8 and vascular endothelial growth factor (VEGF) levels were measured using real-time polymerase chain reaction (RT-PCR) and an enzyme-linked immunosorbent assay (ELISA). Results: Both the ELISA and RT-PCR showed that EM900 and CAM significantly inhibited IL-8 production in HNEpCs. In contrast, EM900 and CAM did not suppress the increased VEGF production when HNEpCs were stimulated with TNF-α. Conclusion: EM900 showed an anti-inflammatory effect, such as that of CAM, due to the inhibitory effect on IL-8 production in HNEpCs.

Background: Many patients with bone metastases whose diagnoses came only after they had suffered aggravated conditions are still frequently encountered. However, there have been few studies regarding the early diagnosis of such metastases. We retrospectively reviewed the clinical courses of cases we experienced between 2004 and 2014 to clarify the practical situation of diagnosis of such bone metastases. Methods: We undertook a retrospective review of 56 of our patients with bone metastasis who had no history of malignancy at their first visit, who visited our departments between 2004 and 2014. The initial diagnoses at the first visit to any clinic, the period from the first visit to any clinic to diagnosis of bone metastasis, the process to make the diagnosis, the frequency of severe skeletal-related events at diagnosis, and the examination serving as the basis for diagnosis were evaluated. Results: The diagnosis of bone metastasis was made at the first visit in only 6 of the 56 patients. Pathological fractures, paralysis and/or calcemia were seen in 62.5% of the patients at diagnosis of bone metastasis. The median period from the first visit to any clinic to diagnosis was 7.0 weeks. Typically, the diagnosis of bone metastasis was made only after aggravation. The most frequent examination to serve as the basis of diagnosis was magnetic resonance imaging. Conclusions: Diagnosis of bone metastasis is challenging in patients without a history of malignancy at their first visit. For early diagnosis, it is important to recognize this challenge and to keep it in mind together with ongoing observation.

We report two cases of granular cell tumors (GCTs) arising on rare sites, namely, the nape and umbilicus. While GCTs have a very characteristic histology, their clinical signs and symptoms are non-specific. Therefore, it is extremely difficult to make a diagnosis of GCT on the basis of clinical findings only. The two cases reported here were clinically similar to hypertrophic scars and keloids, respectively. Skin surgeons should remember that GCTs are an important differential diagnosis from hypertrophic scars and keloids.

Sclerotic fibromas of the skin (SFSs) have a very characteristic histology but the clinical signs are non-specific. Consequently, it is extremely difficult to make a diagnosis of SFS on the basis of the clinical findings only. We report here two cases of SFS arising on the right scapular region and the right lower leg, respectively. Both cases were clinically similar to keloids. Skin surgeons should remember that SFSs are an important differential diagnosis from keloids.

Background: Polycystic ovary syndrome (PCOS) is a common ovulatory disorder that can be induced by sodium valproate (VPA). Patient: We report a case of PCOS that developed in a 15-year-old girl with idiopathic epilepsy after she took VPA. VPA administration stopped her seizures, but it also led to weight gain and amenorrhea, and the patient was diagnosed with PCOS on the basis of diagnostic imaging and serological examination results. Cessation of VPA administration led to reduced weight gain and restored menstruation. Conclusions: The risk of PCOS developing in patients with epilepsy is known to be high, and the association of VPA with PCOS is well established, so if physicians feel this is the best drug to prescribe for female patients with epilepsy, they should carefully monitor the patients' weight and menstruation, and immediately perform ovarian imaging and hormonal examinations if any abnormalities are observed.

A 69-year-old woman was diagnosed with sarcoidosis, which was not treated with corticosteroid therapy. Her levels of angiotensin converting enzyme decreased significantly over 4 years and a mass lesion was detected near the lower part of her left main bronchus, and diagnosed as small cell lung cancer (SCLC). Treatment of the SCLC with a series of chemotherapeutic agents produced excellent results. The pulmonary sarcoidosis did not show any deterioration despite the frequent use of amrubicin, which is known to be a cause of interstitial pneumonia. This is a case report of SCLC complicated with sarcoidosis in a stage of spontaneous remission, possibly suggesting an association between sarcoidosis and tumor immunity, since recent reports have suggested that immune checkpoint inhibitors might be involved in the development of sarcoidosis.

Congenital mesoblastic nephroma (CMN) is a rare tumor of infancy. CMNs can be histologically divided into classic, cellular, and mixed subtypes. Cellular CMNs are difficult to differentiate from Wilms tumors. Herein, a neonate with cellular CMN accompanied by macroscopic hematuria, is described. The clinical, pathological, and imaging features of the disease are discussed.