A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency

A 14-year-old girl presented with acute glomerulonephritis. Tests revealed hypocomplementemia and elevated Antistreptolysin-O titers, and renal biopsy revealed endocapillary and mesangial proliferative glomerulonephritis with double contours of the glomerular basement membrane (GBM). Despite methylprednisolone pulse therapy and the administration of oral prednisolone, overt proteinuria and hypocomplementemia persisted. A second renal biopsy 6 months later confirmed the initial diagnosis of dense deposit disease (DDD) based on electron-dense deposits in the GBM. C3 nephritic factor (C3NeF) and a deficiency of complement factor H (CFH) were not evident. A nephritis-associated plasmin receptor (NAPlr), nephritogenic group A streptococcal antigen, and the plasmin activity by in situ zymography were been in both the first and second biopsy specimens. The patient received combined immunomodulatory therapy with prednisolone and mizoribine, and the urinary protein decreased to a mild level at 27 months after disease onset. These findings suggest that persistent glomerular NAPlr deposition may be associated with the pathogenesis of DDD in some patients without the involvement of C3NeF or CFH mutation and that DDD patients of this type may respond to immunomodulatory treatment.     

Successful treatment of pediatric endocarditis and pericarditis due to MRSA with linezolid

The linezolid treatment for methicillin-resistant Staphylococcus aureus (MRSA) infection was sporadically reported in children. Here we describe a case of a 6 month-old patient underwent mediastinal drainage and artificial conduit removement caused by MRSA infection. After that, linezolid treatment was started and bacteremia was resolved after 14 days of treatment. At discharge, he was no febrile, his blood culture were sterile. We report a case of a patient with MRSA endocarditis treatment with linezolid.     

Thermal coronary angiography in pediatric coronary artery bypass grafting

A 2-year-old boy was referred to our institution for treatment of coronary artery obstruction after an arterial switch operation (ASO). We performed a pediatric coronary artery bypass graft (CABG) with skeletonized left internal thoracic artery (LITA). The patient was 88 cm tall and weighed 11 kg. The left anterior descending (LAD) coronary artery was also identified about 1 mm in diameter. Anastomotic failure of imposed stricture was detected with an infrared camera (IRIS-V, VERITAS, Tokyo, Japan). In the situation of CABG, thermal coronary angiography (TCA) is very useful to reveal satisfactory blood flow into the left coronary system during this procedure.     

Serial brain imaging analysis of stroke-like episodes in MELAS

We report 2 patients of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and consider the pathophysiology of stroke-like lesions, using magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI) on MRI, perfusion imaging on MRI, and 1H magnetic resonance spectroscopy (1H-MRS). In Patient 1, T2-weighted imaging (T2-WI) on MRI at onset and even at 44 days after onset of the stroke-like episode showed high intensity in left parietal, temporal, and occipital lobe lesions. In the temporal lobe lesion, the apparent diffusion coefficient (ADC) at 44 days after onset was higher (average: 1.219x10(-3)mm2/s) than that in a normal region (average: 0.796x10(-3)mm2/s). (1)H-MRS of the left parietal lobe lesion at the same day showed a decrease in N-acetylaspartate/(creatine+phosphocreatine) (NAA/Cr) (0.43) and a peak in lactate. 1H-MRS of the contralateral side at the same day showed NAA/Cr (1.57) and no peak in lactate. Thereafter, ADC gradually decreased and NAA/Cr gradually increased, and the peak in lactate disappeared in the lesion. In Patient 2, T2-WI at onset showed high intensity in bilateral occipital lobe lesions. In the left occipital lobe lesion, ADC at the same day was higher (1.082x10(-3)mm2/s) than that in a normal region (average: 0.841x10(-3)mm2/s). (1)H-MRS of the left occipital lobe lesion at the same day showed a decrease of NAA (3.0mM) and a peak in lactate (13.1mM) (measured by LCModel). In 1H-MRS of the normal left parietooccipital lobe at 4 months before onset, NAA was 7.6mM and there was no peak in lactate (0mM). Perfusion imaging at onset showed high intensity in bilateral occipital lobes, which indicated hyperperfusion in stroke-like lesions. Thereafter, ADC gradually decreased and the peak in lactate partially decreased, and the low concentration of NAA persisted (regardless of the partial recovery) in the lesion. These results suggest that the stroke-like episodes is related to vasogenic edema, hyperperfusion, and neuronal damage. Acute oxidative phosphorylation defect may have a crucial role in the pathophysiology of stroke-like episodes.     

Monitoring neutrophil engraftment in allogeneic stem cell transplantation by flow cytometric analysis of neutrophil-specific antigens NA1 and NA2

Neutrophil-specific antigen (NA) expression on neutrophils was analysed in 18 Japanese children before and after allogeneic stem cell transplantation (allo-SCT) with myeloablative regimen. Donor-recipient NA-incompatibility was present in one of eight NA1/NA2 heterozygous patients and eight of 10 NA1/NA1 or NA2/NA2 homozygous patients. After allo-SCTs from NA-incompatible donors, a neutrophil recipient-to-donor conversion was confirmed in all cases. Conversion to donor NA type was complete before the absolute neutrophil count reached 0.1 x 10(9)/l. These observations indicate that flow cytometric analysis of NA antigens is a simple and useful method for monitoring neutrophil engraftment in NA-incompatible allo-SCT.     

Evaluation of the quality of life between inpatients and outpatients receiving cancer chemotherapy in Japan

A quality of life (QOL) assessment has become increasingly common in cancer clinical trials. Seventy-four consecutive patients treated for cancer between August 2005 and January 2006 at the Cancer Chemotherapy Center in the University of Occupational and Environmental Health, Japan, were examined. The 8-Short form health survey (SF-8) was utilized as a comprehensive scale and quality of life questionnaire for cancer patients treated with anticancer drugs (QOL-ACD) as disease specific scale for the QOL evaluation. The QOL for outpatients was investigated in comparison with that for inpatients. All questionnaires were collected and baseline questionnaires were filled in by 98.1% of the subjects. The physical comprehensive score (PCS) of SF-8 for the outpatients was higher than that for the inpatients. The physical condition of the outpatients was better than that of inpatients. There was no difference in the baseline scores of the QOL-ACD scales in daily activity, psychological condition, social attitude, and face scale of the analyzed domains between the two groups. Furthermore, a longitudinal study from admission to outpatient was carried out on 27 patients who were treated on an outpatient basis in our clinic. No difference in the baseline scores of the SF-8 and QOL-ACD scales were observed in any of the analyzed domains. These data suggest that the present QOL study has a sufficient feasibility for the outpatients evaluated in our study, and QOL of outpatients after discharge is equal to that of inpatients receiving cancer chemotherapy.