Sleep disturbances in 22q11.2 deletion syndrome: a case with obstructive and central sleep apnea.

The 22q11.2 deletion syndrome is characterized by wide phenotypic variability, frequently involving characteristic craniofacial features, cardiac malformations, and learning difficulties. Skeletal anomalies are also common and include an obtuse angle of the cranial base, retrognathia, and cervical spine abnormalities. Despite these anomalies, sleep-disturbed breathing is not reported frequently in patients with 22q11.2 deletion syndrome. We describe a patient with an obstructive sleep disturbance that was successfully treated with a tonsillectomy followed by mandibular distraction osteogenesis. She also had central sleep apnea, initially attributed to spinal cord impingement from cervical instability. Posterior cervical fusion was associated with a decrease in the number of central apneic events.     

New scaphocephaly severity indices of sagittal craniosynostosis: a comparative study with cranial index quantifications.

OBJECTIVE: To describe a novel set of scaphocephaly severity indices (SSIs) for predicting and quantifying head- and skull-shape deformity in children diagnosed with isolated sagittal synostosis (ISS) and compare their sensitivity and specificity with those of the traditional cranial index (CI). METHODS: Computed tomography head scans were obtained from 60 patients diagnosed with ISS and 41 age-matched control patients. Volumetric reformations of the skull and overlying skin were used to trace two-dimensional planes defined in terms of skull-base plane and internal or surface landmarks. For each patient, novel SSIs were computed as the ratio of head width and length as measured on each of these planes. A traditional CI was also calculated and a receiver operating characteristic curve analysis was applied to compare the sensitivity and specificity of the proposed indices with those of CI. RESULTS: Although the CI is a sensitive measure of scaphocephaly, it is not specific and therefore not a suitable predictor of ISS in many practical applications. The SSI-A provides a specificity of 95% at a sensitivity level of 98%, in contrast with the 68% of CI. On average, the sensitivity and specificity of all proposed indices are superior to those of CI. CONCLUSIONS: Measurements of cranial width and length derived from planes that are defined in terms of internal or surface landmarks and skull-base plane produce SSIs that outperform traditional CI measurements.     

Management of acute intra-abdominal hemorrhage in ruptured hepatoblastoma with transarterial embolization using calibrated gelfoam particles

This brief report aims to evaluate the treatment outcome of transarterial embolization in ruptured hepatoblastoma complicated with acute intra-abdominal hemorrhage. Three children (mean age 6 years) with high-risk hepatoblastoma presented with rupture and acute intra-abdominal hemorrhage. In addition to aggressive fluid resuscitation and blood product support, super-selective embolization of the arteries with active bleeding or pseudoaneurysm was performed using calibrated gelfoam particles, with a technical success rate of 100%. Hemodynamic status and hemoglobin level were normalized in all patients within 2 days postembolization. The 30-day survival rate was 100%. No major complication was detected apart from mild elevation of alanine transaminase.     

Overestimation of Peer Smoking Prevalence Predicts Smoking Initiation Among Primary School Students in Hong Kong

PurposeTo investigate the relationship between perceived prevalence of smoking and smoking initiation among Hong Kong primary second- to fourth-grade-students.MethodsA cohort of 2,171 students was surveyed in 2006 and again in 2008. Students who perceived ever-smoking prevalence in peers as “none” or “some” were considered as correct (reference group), whereas those who perceived it as “half” (overestimation) or “most/all” (gross overestimation) were considered as incorrect.ResultsAt baseline, overestimation was found to be cross-sectionally associated with ever-smoking (p < .01). At follow-up, 7.2% of never-smoking students with incorrect estimation at baseline had started smoking, which was 79% (95% confidence interval: 3%–213%), greater than that of 3.7% for those with correct estimation. Among the never-smoking students with incorrect estimation, subsequent correct estimation was associated with 70% (95% confidence interval: 47%–83%) lower risk of smoking initiation compared with persistent incorrect estimation.ConclusionOverestimation of the prevalence of peer smoking predicted smoking initiation among children. Interventions should be carried out to evaluate whether correcting children's overestimation of peer smoking could reduce smoking initiation.     

Potent, orally active heterocycle-based combretastatin A-4 analogues: synthesis, structure-activity relationship, pharmacokinetics, and in vivo antitumor activity evaluation

The synthesis and structure-activity relationship study of a series of compounds with heterocycles in place of the cis double bond in combretastatin A-4 (CA-4) are described. Substituted tosylmethyl isocyanides were found to be the key intermediates in construction of the heterocycles. Cytotoxicities of the heterocycle-based CA-4 analogues were evaluated against NCI-H460 and HCT-15 cancer cell lines. 3-Amino-4-methoxyphenyl and N-methyl-indol-5-yl were the best replacements for the 3-hydroxy-4-methoxyphenyl in CA-4. 4,5-Disubstituted imidazole was found to be the best for the replacement of the cis double bond in CA-4. Medicinal chemistry efforts led to the discovery of compounds 24h and 25f that were found to be 32 and 82% bioavailable, respectively, in rat. Evaluation of 24h and 25f against murine M5076 reticulum sarcoma in mice revealed that both compounds were orally efficacious with an increase in life span of 38.5 and 40.5%, respectively.     

A retrospective drug utilization evaluation of vancomycin usage in paediatric patients

Objective: To evaluate the appropriateness of use of vancomycin in paediatric patients at KK Women's and Children's Hospital, the major paediatric hospital in Singapore to identify potential problems in prescribing practices that may necessitate intervention to optimize vancomycin usage. Methods: A retrospective drug utilization evaluation was performed for paediatric patients who received intravenous vancomycin from 1 June 1998 to 31 June 1999. The outcome measures were consistency of vancomycin indication with recommended guidelines, dosing regimens, microbiological data, monitoring of serum drug levels, renal function, clinical outcomes and adverse drug reactions (ADRs). Results: A total of 96 cases was available for evaluation. Sixty‐two (64·6%) courses of vancomycin were consistent with guidelines for indication of therapy. Eighty‐six (89·6%) of the dosing regimen were consistent. All infusion times that were recorded (56·3%) were consistent with criteria. Of the patients treated with vancomycin for more than 1 day, peak and/or trough serum vancomycin levels were ordered for 70 cases. Of the 56 cases with paired levels ordered, 46 cases had at least one level that fell outside the therapeutic range. Nineteen (19·8%) cases of ADRs were documented. Fifty‐eight (60·4%) cases received concurrent nephrotoxic drugs. However, a substantial portion of vancomycin courses were apparently not prescribed for appropriate indications, and there was poor recording of vancomycin administration information and sampling time. Conclusion: The majority of dosing regimens of vancomycin was consistent with guideline criteria. The most evident problem was the sub‐optimal use of the monitoring of vancomycin serum levels. The information derived from this study may be used as a for further study and for the development of strategies for optimize vancomycin usage.     

Peri-operative risk factors for acute lung injury after elective oesophagectomy

Acute lung injury after oesophagectomy is well recognized butthe risk factors associated with its development are poorlydefined. We analysed retrospectively the effect of a numberof pre-, peri- and post-operative risk factors on the developmentof lung injury in 168 patients after elective oesophagectomyperformed at a single centre. The acute respiratory distresssyndrome (ARDS) developed in 14.5% of patients and acute lunginjury in 23.8%. Mortality in patients developing ARDS was 50%compared with 3.5% in the remainder. Features associated withthe development of ARDS included a low pre-operative body massindex, a history of cigarette smoking, the experience of thesurgeon, the duration of both the operation and of one-lungventilation, and the occurrence of a post-operative anastomoticleak. Peri-operative cardiorespiratory instability (measuredby peri-operative hypoxaemia, hypotension, fluid and blood requirementsand the need for inotropic support) was also associated withARDS. Acute lung injury after elective oesophagectomy is associatedwith intraoperative cardiorespiratory instability. Br J Anaesth 2001; 86: 633–8     

Steroid-induced avascular necrosis of the hip in neurosurgical patients: epidemiological study

BACKGROUND: Avascular necrosis (AVN) of the femoral head is perceived to be a rare complication of short-term steroid therapy for neurosurgical conditions but its precise risk is unknown. METHODS: Retrospective review of hospital records between 1994 and 2001. RESULTS: The risk of developing AVN of the femoral head is 0.3% with an incidence of one per one thousand patients per year. CONCLUSIONS: It would be advisable to minimize both the dosage and the duration of steroid treatment where possible.