Trisomy 21 in childhood acute lymphoblastic leukemia: a Pediatric Oncology Group study (8602)

Of 1,036 children with newly diagnosed non-T, non-B acute lymphoblastic leukemia (ALL) and a demonstrated cytogenetic abnormality treated on the frontline Pediatric Oncology Group (POG) therapeutic trial 8602, there were 33 patients with trisomy 21 as the sole abnormality. Of these 33, 14 had Down syndrome (DS). Although the non-DS (NDS) trisomy 21 cases tended to be older than the DS cases, there were no other significant differences in clinicobiologic features nor in treatment outcomes between the DS and NDS groups, nor between the entire trisomy 21 group and the other chromosome abnormality group. Among NDS patients with +21 and one additional abnormality, +X, +16, -20, and structural abnormalities involving 6q or 12p were common findings. Kaplan-Meier event-free survival (EFS) curves showed a 4-year EFS of 80% (SE, 12%) in NDS trisomy 21 cases, 71% (SE, 22%) in DS cases with trisomy 21 as the sole abnormality, and 69% (SE, 2%) in cases with other chromosome abnormalities. Trisomy 21 as a sole acquired abnormality in NDS patients suggests a good prognosis.     

Preoperative albumin as a predictor of one-year mortality in patients with fractured neck of femur

A simple measure to determine one-year mortality following hip fractures has its benefits. Where there is controversy over implant selection, such a scoring system can facilitate the decision-making process. We undertook a retrospective analysis of one-year postoperative mortality of our hip fracture patients and established their admission serum albumin levels to see if there was any correlation between this and one-year mortality. Our results showed one-year mortality was significantly higher (p=0.0049) for those patients with a serum albumin of <35g/dl. Of the patients with low albumin, we found that there was no statistical significance between one-year mortality and source of admission (p=0.0789). Prefracture serum albumin can be used as a simple predictor of one-year mortality in patients presenting with a fractured neck of femur, thereby aiding operative planning and implant selection with a view to likely survival and possible need for revision.     

Recurrence of unexpected infant death

Families which had experienced two or more unexpected infant deaths were the subject of detailed confidential enquiries, including necropsy examination. Cases were derived from two main sources: first, deaths occurring during a nationwide programme of support for families with a subsequent baby (8 families) plus 2 families from a scries of confidential enquiries in Sheffield, and second, direct referrals from paediatricians (17 families). Fifty-seven deaths were studied. Twenty-four families had experienced 2 and three had experienced 3 deaths: 11 deaths (19%) were found to be adequately explained by history or post-mortem findings; 7 (12%) were probably accidental; 31 (55%) were most probably due to an action by one of the parents (filicide); only 5 (9%) were considered to be true or idiopathic sudden infant death syndrome; in 3 (5%) cases there was insufficient information to draw a conclusion. Five (18%) of the families lived in circumstances of serious social deprivation. A history of psychiatric illness was present in one or both parents in 18 (67%) of the families.     

Cellular adaptation in migraineurs with chronic daily headache

Ergotamine and analgesic misuse are now recognized as causes of chronic daily headache and the condition responds well to drug withdrawal with reduced headache frequency. In this study, we have investigated whether medication misuse is associated with an alteration in membrane transduction which is sensitive to drug withdrawal. This was carried out by assay of the thrombin-stimulated generation of inositol phosphates in platelets from 12 migraine patients with chronic daily headache and analgesic misuse, 7 migraine patients with chronic daily headache and ergotamine misuse and 7 control subjects. After drug withdrawal, a significant decrease in headache frequency was seen at one month in both patient groups. Withdrawal of analgesics produced a significant decrease in thrombin-stimulated inositol phosphate production at one month; this was further decreased a month later with a reduction in B_max of 60% and no significant change in K_D. A similar pattern was obtained in ergot misuse patients, with the K_D value decreasing by 56% one month after drug withdrawal. These results provide evidence of an adaptation in transduction with misuse of analgesics and ergotamine which correlates with headache frequency.     

Psoralen–ultraviolet A vs. narrow-band ultraviolet B phototherapy for the treatment of vitiligo

BACKGROUND: Although many treatment modalities have been tried for the treatment of vitiligo, none is uniformly effective. Psoralen phototherapy (psoralen ultraviolet A (PUVA)) is established as efficacious treatment for vitiligo. Recently, narrow-band UVB (NBUVB) has been reported to be an effective and safe therapeutic option in patients with vitiligo. OBJECTIVE: To compare the efficacy of PUVA and NBUVB in the treatment of vitiligo. DESIGN AND SETTING: Retrospective analysis of 69 patients with vitiligo who were treated either with PUVA or NBUVB at the pigmentary clinic of the Dermatology Department of the Postgraduate Institute of Medical Education and Research, Chandigarh, India. OUTCOME MEASURES: The following variables were compared between the two groups of patients: repigmentation status, number of treatments for marked to complete repigmentation in existing lesions, appearance of new lesions or increase in size of existing lesions, adverse effect of therapy, stability of repigmentation and colour match. RESULTS: In PUVA-treated group, 9 patients showed marked to complete repigmentation (23.6%) and 14 patients showed moderate improvement (36.8%), whereas in NBUVB-treated group, 13 patients showed marked to complete repigmentation (41.9%) and 10 patients showed moderate improvement (32.2%). A statistically significantly better stability and colour match of repigmentation with surrounding skin was seen in NBUVB-treated patients. CONCLUSION: We showed that NBUVB is more effective than PUVA and repigmentation induced with NBUVB is statistically significantly more stable.     

Superficial granulomatous pyoderma: an idiopathic granulomatous cutaneous ulceration

A 70-year-old male with a superficial granulomatous ulcer is reported. Histopathological findings were the same as those described for superficial granulomatous pyoderma, a recognized variant of classic pyoderma gangrenosum. The differences between pyoderma gangrenosum and its variant superficial granulomatous pyoderma are highlighted.