Orodental, ear and eye anomalies in Egyptian Brachmann de Lange syndrome cases

The Brachmann de Lange syndrome (BDLS), first described in its full clinical presentation by Brachmann (1916) and Cornelia de Lange (1933) is a multi-system syndrome involving congenital malformations, growth retardation and neurodevelopmental delay. We describe here twelve Egyptian cases with this syndrome with emphasis on the orodental, ear and eye abnormalities and their relation to the severity of expreseion of the disorder. The crodental anomalies were high arched palate, Iong philtrum, micrognathia, macrostomia hypoplasia of upper anterior teceth, fissured tongue macroglossia. Ear malformations were low-set large ears. Otoscopy revealed normal drum appearance. Audiogram revealed conductive, sensorineural or mixed hearing loss. Ocular manifestations were nystagmus, convergentt squint, enophthalmos, myopia 8 blue scleca. The study emphasizes certain eye, ear and crodental anomalies as diagnostic features of the BDLS which correlate with the severity of expression of the syndrome. None of the cases had chromosomal aberrations and the parental consanguinity rate was not increased thus supporting dominant mutations or minor chromosomal etiology.     

Supervisors matter more than you think: components of a mission-centered organizational climate

A study was conducted in a medical center among a diverse sample of employees to examine whether components of organizational climate related to workers' knowledge of the organization's mission and mission-centered values. Findings supported a mediated relationship between supervisor behaviors, mission knowledge, and customer service orientation (the organization's key mission value). Employee perceptions of coworker and organizational support and knowledge of their own performance expectations also related positively to customer service orientation. Results suggest that supervisors are in an ideal position to disseminate a mission-centered climate. Practical applications of these findings for management wishing to develop mission-centered climates in health care organizations are discussed.     

Erythrina alkaloids: isolation and characterisation of alkaloids from seven erythrina species

The alkaloids present in the seeds of seven ERYTHRINA species, E. FUSCA Lour, E. COSTARICENCIS M. Micheli, E. LEPTORHIZA A. DC., E. SPECIOSA Andrews, E. VARIEGATA L., E.MELANACANTHA Taubert ex Harms, and E. BERTEROANA Urban, have been screened by gas chromatography-mass spectrometry (GC/MS). The novel alkaloid erythrartine N-oxide has been isolated from E. BERTEROANA and characterized as an N-oxide of theknown alkaloid, erythrartine.     

Influencing prescribing practices and associated outcomes utilizing the drug use evaluation process

Influencing prescribing practices and the implementation of pharmaceutical care utilizing the drug use evaluation (DUE) process at a 891-bed teaching hospital are described. The DUE program has been structured to provide for significant pharmacy involvement while maintaining medical staff responsibility as outlined by the Joint Commission on Accreditation of Healthcare Organizations. A multidisciplinary approach is used to identify problems and develop prescribing criteria and educational initiatives. Pharmacists provide drug therapy monitoring and engage in clinical interventions and documentation of outcomes on a daily basis. DUE program pharmacists help target possible interventions, assure monitoring and outcome documentation, and compile results of all initiatives for reporting purposes to the medical staff and quality assessment program. Specific performance compliance and problems are identified and incorporated into the credentialing process. Corrective measures are determined by the Pharmacy and Therapeutics (P&T) Subcommittee with subsequent actions carried out by peer physicians. The net result is a positive influence on prescribing practices that improves the appropriate and effective use of drugs and improves patient outcomes.     

Pharmacokinetics of Nisoldipine Coat--Core Formulation in Subjects with Liver Cirrhosis

The pharmacokinetics of a controlled-release formulation (coat--core) of the calcium channel blocker nisoldipine was investigated in eight subjects with biopsy-proved liver cirrhosis and eight healthy subjects. In Stage I, subjects received a single 10-mg dose to determine if this dose would be safely tolerated in the subjects with cirrhosis. Because all subjects in both groups tolerated the dose without difficulty, all were continued to Stage II. In Stage II, subjects received a once-daily dose of 10-mg coat-core tablets for 7 days. Serial plasma samples were assayed for nisoldipine in both stages. The C(max) and AUC of nisoldipine were approximately fourfold to fivefold higher (p < 0.01) in subjects with cirrhosis as compared to healthy subjects; however, there was overlap in the range of pharmacokinetic parameters between the two groups. The accumulation factor following multiple dosing was similar in both groups. Results suggest that nisoldipine dose should be optimized by monitoring of a pharmacodynamic end point, such as effect on blood pressure. It is likely that dose requirements for patients with liver disease will be lower.     

Self-Directed Treatment of Intermittent Heartburn: A Randomized, Multicenter, Double-Blind, Placebo-Controlled Evaluation of Antacid and Low Doses of an H(2)-Receptor Antagonist (Famotidine)

BACKGROUND: Heartburn, a common symptom, is self-treated with oral antacids. Efficacy of antacids has not been demonstrated for individual, spontaneous heartburn episodes. METHODS: We conducted a double-blind, randomized, placebo-controlled, parallel-group study of self-directed treatment for episodic heartburn comparing famotidine (FAM) 5, 10, or 20 mg and antacid (11 mEq ANC) to placebo (PBO) during a 4-week period. Twenty-nine US investigators enrolled a total of 565 outpatients, ages 18--81 years (mean 44.1 years) with heartburn but not seeking care for heartburn. Treatment of spontaneous heartburn episodes was permitted as needed, up to twice daily, with self-administered test drug. An open-label, backup antacid was provided to use if test drug did not provide adequate relief. Patients assessed heartburn relief hourly and recorded use of backup antacid. Relief was defined as complete relief of symptoms without the use of backup antacid. RESULTS: The media proportion of episodes relieved was: PBO, 41%; FAM 5 mg, 59%, 0.05 less-than-or-equal p < 0.10; FAM 10 mg, 70%, p < 0.001; FAM 20 mg, 69%, p < 0.001; antacid, 62%, p < 0.05 (p-values versus PBO). Supplemental analyses incorporating time to relief confirmed that famotidine and antacid provided more rapid and more frequent relief than placebo (odds ratio for relief relative to PBO: FAM 5 mg, 1.55, p = 0.003; FAM 10 mg, 1.94, p < 0.001; FAM 20 mg, 2.13, p < 0.001; antacid 1.57, p = 0.003). The tolerability profile was similar with famotidine, antacid, and placebo. CONCLUSIONS: The positive results with antacid demonstrated for the first time the efficacy of antacid in self-treatment of individual heartburn episodes and provided internal validation of this study paradigm. Patients in this study self-medicated effectively using low doses of famotidine on an as needed basis for spontaneous episodes of heartburn.     

Clinical neurological evaluation

The importance of the neurological evaluation for PLDD procedures is discussed. Elements of the basic examination are outlined and the reason for specific methods of testing are offered. The physician should pay attention to patient complaints, mechanical signs, and patient capabilities.