Successful reduced-intensity SCT from unrelated cord blood in three patients with X-linked SCID

We describe three males with X-linked SCID (X-SCID) who were successfully treated by reduced-intensity SCT from unrelated cord blood (CB). Mean age at transplant was 5.7 months (range, 3–9 months). Pre-transplant conditioning for all patients consisted of fludarabine (FLU) (30 mg/m2 per day) from day −7 to day −2 (total dose 180 mg/m2) and BU 4 mg/kg per day from day −3 to day −2 (total dose 8 mg/kg). All CB units were serologically matched at HLA-A, B and DR loci. Although two patients had suffered from fungal or bacterial pneumonia before transplantation, there were no other infectious complications during transplantation. All patients engrafted and achieved 100% donor chimerism. We also confirmed full donor chimerism of both T and B cells. Only one patient developed acute GVHD grade III, which was resolved by increasing the dose of oral corticosteroid. None of the patients has developed chronic GVHD during follow up for 21–77 months. None of the patient received i.v. Ig replacement post transplant, or showed delay in psychomotor development. Reduced-intensity conditioning consisting of FLU and BU and transplantation from unrelated CB was an effective and safe treatment for these patients with X-SCID

The Chemokine Receptor CXCR4 Strongly Promotes Neuroblastoma Primary Tumour and Metastatic Growth, but not Invasion

Neuroblastoma (NB) is a heterogeneous, and particularly malignant childhood neoplasm in its higher stages, with a propensity to form metastasis in selected organs, in particular liver and bone marrow, and for which there is still no efficient treatment available beyond surgery. Recent evidence indicates that the CXCR4/CXCL12 chemokine/receptor axis may be involved in promoting NB invasion and metastasis. In this study, we explored the potential role of CXCR4 in the malignant behaviour of NB, using a combination of in vitro functional analyses and in vivo growth and metastasis assessment in an orthotopic NB mouse model. We show here that CXCR4 overexpression in non-metastatic CXCR4-negative NB cells IGR-NB8 and in moderately metastatic, CXCR4 expressing NB cells IGR-N91, strongly increased tumour growth of primary tumours and liver metastases, without altering the frequency or the pattern of metastasis. Moreover shRNA-mediated knock-down experiments confirmed our observations by showing that silencing CXCR4 in NB cells impairs in vitro and almost abrogates in vivo growth. High levels of CXCL12 were detected in the mouse adrenal gland (the primary tumour site), and in the liver suggesting a paracrine effect of host-derived CXCL12 on NB growth. In conclusion, this study reveals a yet unreported NB-specific predominant growth and survival-promoting role of CXCR4, which warrants a critical reconsideration of the role of CXCR4 in the malignant behaviour of NB and other cancers

Clinical Anatomy for the Innervated Pattern and Boundary of the Subdeltoid Bursa

The aim of this study was to accurately identify the distribution of sensory nerve branches running to bursa with mesoscopic dissection and boundaries following the injection of gelatin into the bursa. Eighteen shoulders of 11 Korean soft cadavers (average age, 65 years; age range, 43 - 88 years) were dissected. The most prominent point of greater tubercle of the humerus (GT) was used as a reference point. The horizontal line passing through GT was used as the x-axis while the vertical line passing through the GT was used as the y-axis. Average distances of the anterior, posterior, superior, and inferior from the GT were 1.9±0.6, 2.4±1.3, 2.1±0.7, and 3.2±1.5 cm, respectively. In 15 cases of 18 shoulders, the anterior branch of the axillary nerve was distributed to the subdeltoid bursa that was running posteriorly. The muscular branch of the anterior and middle parts of the deltoid was distributed to the branch of nerve that was running into the subdeltoid bursa. A branch of the posterior cord of brachial plexus was distributed to the subdeltoid bursa that was running anteriorly in three cases. Most of the branches of the axillary nerve were distributed into the posterolateral area. The branches of the posterior cord of brachial plexus were distributed in the anterolateral area. These results might be useful for preventing residual pain on the anterior shoulder region following an injection for the relief of shoulder pain