Plasma exchange for the patients with dilated cardiomyopathy in children is safe and effective in improving both cardiac function and daily activities.

Autoantibodies against cardiac proteins play an important role in the development of dilated cardiomyopathy (DCM). The efficacy and safety of apheresis such as immunoadsorption (IA) or plasma exchange (PE) to remove such antibodies have been reported in adult DCM patients. However, apheresis for pediatric DCM has not been performed because of technical difficulty due to relatively low blood volume and instability of hemodynamics. As we have experiences of preforming apheresis on hemodynamically unstable children, we have preformed ten courses of PE on seven child DCM patients including both patients in chronic and acute phase to assess the safety and efficacy to PE. Under general anesthesia, the patients were administered PE three times during 3 days as 1 course. Simultaneously, continuous hemodiafiltration (CHDF) was performed in series with the PE circuit to stabilize hemodynamic status and to minimize the adverse effects of PE. The changes in LVEF, CTR, mBP, the dosage of furosemide and NYHA were assessed before and after the procedure of PE. There were no severe adverse effects such as systemic bleeding or refractory hypotension due to apheresis. Echocardiography showed that mean baseline LVEF was 24.3 ± 7.8%. Mean LVEF significantly increased 1 week after PE to 30.5 ± 12.5%. CTR significantly decreased after PE. Mean BP significantly increased 1 month after PE (54.5 ± 10.7 to 60.7 ± 9.8 mmHg). NYHA improved after PE significantly (NYHA; 3.4 ± 1.1 to 2.5 ± 1.1). PE is safe and effective in improving both cardiac function and daily activities.

A first case of meningitis/encephalitis associated with SARS-Coronavirus-2.

Novel coronavirus (SARS-Coronavirus-2:SARS-CoV-2) which emerged in Wuhan, China, has spread to multiple countries rapidly. We report the first case of meningitis associated with SARS-CoV-2 who was brought in by ambulance due to a convulsion accompanied by unconsciousness. He had never been to any foreign countries. He felt generalized fatigue and fever (day 1). He saw doctors nearby twice (day 2 and 5) and was prescribed Laninamivir and antipyretic agents, His family visited his home and found that he was unconsciousness and lying on the floor in his vomit. He was immediately transported to this hospital by ambulance (day 9). Under emergency transport, he had transient generalized seizures that lasted about a minute. He had obvious neck stiffness. The specific SARS-CoV-2 RNA was not detected in the nasopharyngeal swab but was detected in a CSF. Anti- HSV 1 and varicella-zoster IgM antibodies were not detected in serum samples. A brain MRI showed hyperintensity along the wall of right lateral ventricle and hyperintense signal changes in the right mesial temporal lobe and hippocampus, suggesting the possibility of SARS-CoV-2 meningitis. This case warns the physicians of patients who have CNS symptoms.

Involvement of epidermal growth factor receptor and downstream molecules in bone and soft tissue tumors.

Correlations among epidermal growth factor receptor (EGFR) gene amplification, mutation, and overexpression/activation of proteins were investigated in 39 cases of bone/soft tissue tumors (BSTTs). By immunohistochemistry, EGFR overexpression was found in 22.6% of sarcomas, but not in benign lesions. By immunoblotting, among sarcoma cases showing upregulation of EGFR, 47.4% showed EGFR activation. In 2 cases of malignant fibrous histiocytoma with high level of EGFR gene copy numbers, EGFR expression and phosphorylation levels were significantly higher; and signal transducer and activator of transcription 3 (Stat-3) was activated. Point mutations were detected in 4 cases, 3 of which were missense mutations. In these 3 cases, activation of EGFR and Stat-3 was found in 2 cases. In the cases without gene aberrations, upregulation of the EGFR was found in both sarcomas and benign lesions; but activation was found only in sarcomas. However, EGFR activation did not specifically correlate with activation of particular downstream molecules. Among the 3 downstream cascades, Akt pathway was more frequently activated than those of Stat-3 or extracellular signal-related protein kinase 1/2, and Stat-3 was activated in tumors exhibiting an epithelial nature, including synovial sarcoma and chordoma. These results suggest that persistent Stat-3 activation may be a critical event downstream of overexpressed EGFR by high level of EGFR gene copy numbers. In contrast, tumors harboring EGFR mutation may not necessarily activate EGFR or specific downstream cascades. Finally, in BSTTs, Akt functions as a predominant molecule. These overall results could provide novel insights into the involvement of EGFR and downstream molecules and suggest that EGFR-mediated cascades are candidates for molecular targeting therapy in defined subsets of BSTTs.

A case of cerebral tuberculoma mimicking neurocysticercosis.

Case: A 42-year-old Peruvian woman residing in Japan for 11 years with a family history of neurocysticercosis presented to our intensive care unit with fever and intense headache.Computed tomography indicated multiple micronodular lesions in the brain parenchyma, and cerebral tuberculoma and neurocysticercosis were considered in the differential diagnosis. Neurocysticercosis was initially suspected, and oral praziquantel was initiated. However, because of a high adenosine deaminase level in the cerebrospinal fluid and positive peripheral blood interferon gamma release test result, cerebral tuberculoma was subsequently considered. Outcome: Antituberculous drugs with steroids were initiated on day 10, after which the symptoms gradually resolved; the patient was discharged on day 29. Gadolinium-contrast magnetic resonance imaging 8 months later showed reduced nodular shadows, confirming cerebral tuberculoma. Conclusion: Immediate diagnosis and treatment are imperative for cerebral tuberculoma, a lethal infection. Considering the recent increases in immigration worldwide, increased cases of tuberculoma mimicking neurocysticercosis are expected.

Comparison of efficacy between continuous hemodiafiltration with a PMMA high-performance membrane dialyzer and a PAN membrane hemofilter in the treatment of septic shock patients with acute renal failure.

The aim of this study was to investigate whether continuous hemodiafiltration (CHDF) with a high-performance membrane dialyzer made of polymethylmethacrylate (PMMA-CHDF) in the treatment of septic shock patients with acute renal failure (ARF) is clinically relevant. 30 patients were treated with PMMA-CHDF. 13 patients treated with CHDF used a hemofilter made of polyacrylonitrile membrane (PAN-CHDF). Systolic blood pressure significantly increased in the PMMA-CHDF group following 24 h of treatment (p < 0.01), whereas it did not improve in the PAN-CHDF group. Urine volume significantly increased in the PMMA-CHDF group following 24 h of treatment which was more than in the PAN-CHDF group (p < 0.05). 28-day survival was 83.3% in the PMMA-CHDF group and 30.8% in the PAN-CHDF group, respectively (p < 0.01). We can assume that PMMA-CHDF in the treatment of septic shock patients with ARF is clinically relevant.