Mizue Nakajima

Hematopoietic capacity of preterm cord blood hematopoietic stem/progenitor cells

Full-term cord blood (TCB) hematopoietic stem/progenitor cells (HSC/HPCs) are used for stem cell transplantation and are well characterized. However, the properties of preterm cord blood (PCB) HSC/HPCs remain unclear. In the present study, we compared HSC/HPCs from TCB and PCB with respect to their expression of surface markers, homing capacity and ability to repopulate HSCs in the NOD/Shi-scid mice bone marrow. The proportion of CD34+CD38- cells was significantly higher in PCB. On the other hand, the engraftment rate of TCB CD34+ cells into NOD/Shi-scid mice was significantly higher than PCB CD34+ cells. The expression of VLA4 was stronger among TCB CD34+ cells than PCB CD34+ cells. Moreover, there was a positive correlation between the proportion of CD34+CXCR4+ cells and gestational age. These data suggest that the homing ability of HSCs increases during gestation, so that TCB may be a better source of HSCs for transplantation than PCB.

Prenatal diagnosis of isolated congenitally corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac defect characterized by the atria connecting with anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries, which allows hemodynamic compensation. Most patients with ccTGA have associated intracardiac anomalies, which could be a diagnostic clue, whereas isolated forms are infrequently diagnosed during the neonatal period and in utero. We describe a fetus that was diagnosed with ccTGA and without additional cardiac anomalies at 25 weeks of gestation. The parallel course of the great arteries discovered during a routine obstetric scan indicated this rare cardiac anomaly. Further detailed examination of the ventricular morphology helped to confirm the diagnosis. Despite hemodynamic compensation, the long-term prognosis of ccTGA is uncertain because of the possible development of arrhythmias or heart failure later in life. Our findings showed that fetal echocardiography can detect prenatal ccTGA.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome in a 14-year-old boy: an immunohistochemical study of infiltrating lymphocytes in acneous skin regions.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome was proposed as a clinico-radiological entity combining skin, bone and joint manifestations [1]. Here, we report a 14-year-old boy with SAPHO syndrome, in whom we investigated the pathology of acneous skin regions with aberrant T-cell lymphocyte infiltration in the dermal area. A 14-year-old boy was admitted to our hospital exhibiting persistent high-grade fever with acne conglobata, in addition to chest, left knee joint and lumbar pain. Physical examination revealed swelling of the left knee joint and severe facial acne (Fig. 1). Haematological and biochemical investigations were all normal; however, C-reactive protein was 8.58 mg/dl and the ESR was 54 mm/h. Urinalysis showed normal findings. The purified protein derivative of tuberculosis reaction was unchanged from the previous year. Blood, urine, stool and pharyngeal cultures were all negative. Compliment, ferritin, and serum immunoglobulin level were normal. LE test, antibodies related to collagen diseases and tumour markers were all negative. Gallium scintigraphy showed abnormal accumulation in the left iliosacral joint, bilateral parotid glands, and acne lesions of the forehead (Fig. 2). CT scans of the pelvis showed increased space in the left iliosacral joint, thus suggesting swelling of the joint cartilage, but no solid-type periosteal reaction [6] were observed (Fig. 3). After admission, we treated the patient with intravenous antibiotics, but the clinical symptoms remained unchanged. For differential diagnosis, we performed a skin biopsy and found pyoderma with focal foreign body reaction and aberrant lymphoid cell infiltration (Fig. 4). We then started to administer oral ibuprofen. Clinical symptoms and laboratory findings improved rapidly after ibuprofen administration. Two years after discharge, his course has been uneventful with continued oral administration of ibuprofen. Because our case has not been refractory, we did not use pamidronate [4].