Cerebriform intradermal naevus (a rare form of secondary cutis verticis gyrata)

BACKGROUND: Acquired cerebriform intradermal naevus (CIN) is a rare form of pseudo cutis verticis gyrata. CASE REPORT: A case of acquired CIN of the scalp in a 46-year-old male patient is presented. The clinical and histopathological presentations of CIN are described and the therapeutic possibilities are discussed. CONCLUSIONS: In each individual case the physician must decide whether to do surgery or follow a wait-and-see policy. There is little risk of malignant change of CIN, and surgical excision of such lesions often involves evident mutilation.     

Endotracheal suctioning causes right upper lobe collapse in intubated children

Objective: Right upper lobe collapse is a common radiographic finding in intubated children. We hypothesized that deep suctioning and uncontrolled negative pressures during endotracheal tube suctioning were significant contributory factors. Methods : The incidence of right upper lobe (RUL) collapse in intubated, ventilated children on a paediatric cardiac intensive care unit was determined over a 3-month period ( n = 102). Graduated suction catheters and suction vacuums of < 165 cm H₂O were then introduced. Another prospective audit was carried out 3 months later ( n = 60). Results : We found that 24% developed RUL collapse and 4 developed an apical pneumothorax. Following the introduction of graduated catheters and controlled vacuums pressures, a significant reduction in the incidence of RUL collapse, to 7%, was observed ( p < 0.05). Conclusions : We conclude that high negative pressure and deep-suctioning causes RUL collapse in children. Any lobar collapse not only prolongs the child's stay in intensive care, but can be associated with further morbidity which may have a serious implication. By improving suctioning technique this morbidity can be significantly reduced.     

Septo-optic dysplasia: MR imaging

Septo-optic dysplasia is the diagnosis when optic nerve hypoplasia is seen in conjunction with dysgenesis of the septum pellucidum. Nearly two-thirds of these patients have hypothalamic-pituitary dysfunction, and half have schizencephaly. The disorder is difficult to classify because of the diversity of clinical and pathologic manifestations. Magnetic resonance images of 11 patients with clinical and radiographic evidence of septo-optic dysplasia were reviewed retrospectively. The "syndrome" appears to include two subsets of patients whose abnormalities have different embryogenesis and neuropathologic findings. The existence of these two subsets helps to explain the diversity of the clinical and radiologic findings.     

PJA-BP expression and TCR delta deletion during human T cell differentiation

Recombination of deltaRec to psiJalpha will delete the TCR delta gene, which is thought to play an important role in the bifurcation of the TCR alphabeta versus TCR gammadelta differentiation lineages. We recently detected a DNA-binding protein in human thymocytes, the so- called PJA-BP, which recognizes the psiJalpha gene segment and might be one of the factors involved in the regulation of preferential deltaRec- psiJalpha rearrangements. We now investigate PJA-BP expression and its correlation with TCR delta gene deletion in thymocytes. Our electrophoretic mobility shift assay experiments showed that the PJA-BP is evolutionary conserved in human, murine and simian thymocytes. Using a large series of human hematopoietic malignancies (n = 30), we conclude that PJA-BP expression is thymocyte specific and seems to be restricted to thymocytes committed to the TCR alphabeta lineage. Analysis of seven well-defined human thymocyte subpopulations showed that preferential deltaRec-psiJalpha rearrangements as well as PJA-BP expression can be detected from the immature CD34-/CD1+/CD3- /CD4+/CD8alpha+beta- thymocyte differentiation stage onwards. These experiments indicate that expression of PJA-BP in human thymocytes starts simultaneously with preferential deltaRec-psiJalpha rearrangements, which supports our hypothesis that PJA-BP is one of the factors involved in the preferential recombination of deltaRec to psiJalpha.      

Andrology: Factors influencing the outcome of in-vitro fertilization with epididymal spermatozoa in irreversible obstructive azoospermia

Microsurgical epididymal sperm aspiration (MESA) and in-vitrofertilization (IVF) were found to offer limited opportunityfor fatherhood to 45 men with obstructive azoospermia, due principallyto poor embryo implantation. Adequate sperm preparations wereobtained in 46/50 treatment cycles (92%), with the best motilityfound in the caput epididymis in 89% of cases. The mean fertilizationrate was 11.2% and fertilization occurred in 23 cycles (50%),with embryo transfer arising from 12/26 men with was aplasia(CAV), 4/9 with genital tract obstruction (EV) and 7/11 withirreversible vasectomy (VV). The overall implantation rate waslow, 8.7% per embryo transfer (11.7% per 2-3 embryo transfers)and was not improved by Fallopian transfer. There were two pregnancies(4% per cycle), both in the EV group where embryo formationand implatation (2/4, 50% per cycle) were optimum even thoughsperm preparations were paradoxically inferior to the CAV andVV groups. The spermatozoa retrieved in the two successful EVcycles were appreciably blood contaminated. Analysis of the21 failed embryo transfers showed delayed fertilization in 10cycles, cystic fibrosis (CF) mutation or familial disease in7/12 CAV men and the VV men were older (P<0.001). A pregnancywhich miscarried arose from a case of Young's syndrome, a carrierof CF mutation DF508. Male factors could thus be implicatedin the high embryo wastage of MESA cycles and might also beinfluencing implantation in other IVF procedures. Where feasible,male reconstructive surgery is preferable unless fertilizationcan be improved, possibly by speedier retrieval techniques orby permitting sperm capacitation in vitro, but probably moreeffectively by micro-assisted insemination.