Multiple neurosurgical treatments for different members of the same family with Currarino syndrome

Introduction

Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons.

A successful chronic care program in Al Ain-United Arab Emirates

Background

The cost effective provision of quality care for chronic diseases is a major challenge for health care systems. We describe a project to improve the care of patients with the highly prevalent disorders of diabetes and hypertension, conducted in one of the major cities of the United Arab Emirates.

Settings and Methods

The project, using the principles of quality assurance cycles, was conducted in 4 stages. The assessment stage consisted of a community survey and an audit of the health care system, with particular emphasis on chronic disease care. The information gleaned from this stage provided feedback to the staff of participating health centers. In the second stage, deficiencies in health care were identified and interventions were developed for improvements, including topics for continuing professional development. In the third stage, these strategies were piloted in a single health centre for one year and the outcomes evaluated. In the still ongoing fourth stage, the project was rolled out to all the health centers in the area, with continuing evaluation. The intervention consisted of changes to establish a structured care model based on the predicted needs of this group of patients utilizing dedicated chronic disease clinics inside the existing primary health care system. These clinics incorporated decision-making tools, including evidence-based guidelines, patient education and ongoing professional education.

Results

The intervention was successfully implemented in all the health centers. The health care quality indicators that showed the greatest improvement were the documentation of patient history (e.g. smoking status and physical activity); improvement in recording physical signs (e.g. body mass index (BMI)); and an improvement in the requesting of appropriate investigations, such as HbA1c and microalbuminurea. There was also improvement in those parameters reflecting outcomes of care, which included HbA1c, blood pressure and lipid profiles. Indicators related to lifestyle changes, such as smoking cessation and BMI, failed to improve.

Conclusion

Chronic disease care is a joint commitment by health care providers and patients. This combined approach proved successful in most areas of the project, but the area of patient self management requires further improvement.     

Cost-effectiveness of Oral Versus Intravenous Ibuprofen Therapy in Preterm Infants With Patent Ductus Arteriosus in the Neonatal Intensive Care Setting: A Cohort-based Study

PurposeUse of ibuprofen for the patent ductus arteriosus (PDA) has become increasingly common. This study aimed to evaluate the clinical and economic impact of oral ibuprofen versus intravenous ibuprofen for PDA among preterm infants.MethodsThis retrospective, cohort-based pilot study examined the clinical and economic associations of oral versus intravenous ibuprofen for PDA. A decision-analytic model was constructed, from the hospital perspective, to follow the oral versus intravenous administrations of ibuprofen for PDA and their clinical and economic consequences. The course regimen of either formulation was an initial 10 mg/kg followed by 5 mg/kg at 24- and 48-h intervals. Clinical and resource utilization data were extracted from Cerner medical database, from 2014 through 2018, at the tertiary neonatal intensive care unit setting in Qatar. The primary outcome measures were the rate of successful closure based on the ductal diameter measure after the first course of treatment and the overall direct medical cost of PDA management. A population of 118 neonates was required for results with 80% power and 0.05 significance. Sensitivity analyses involving unit costs and a subgroup analysis based on gestational age and birth weight, added to a second-order probabilistic analysis of all model inputs, were performed.FindingsForty infants were available for inclusion in the oral ibuprofen study group, not achieving the desired sample size, with successful PDA closure reported in 64% of cases compared with a reduced success of 36% with intravenous ibuprofen (n = 59) (risk ratio = 0.56; 95% CI, 0.32–0.97; P = 0.04), which was associated with economic advantage to oral ibuprofen. The probabilistic analysis illustrated that oral ibuprofen costs less than intravenous ibuprofen in 72% of patient cases, with QAR 48,751 (US $13,356) (95% CI, QAR 47,500–50,000, US $13,014–$13,699) in mean savings. Sensitivity analyses confirmed the robustness of study conclusions and found that the rate of closure success versus failure was the most influential on results, followed by the occurrence of adverse drug events with both intravenous and oral ibuprofen. Although both ibuprofen formulations had similar safety profiles (P = 0.16), the intravenous formulation was associated with a larger number of adverse drug effects.ImplicationsThis is the first cost-effectiveness evaluation of oral versus intravenous formulations of ibuprofen among infants with PDA. The oral ibuprofen might be associated with an enhanced ductal closure at a considerably lower cost. The study results support recent trends in neonatal intensive care unit practices in favor of the oral administration of ibuprofen.     

Incidence of community‐acquired methicillin‐resistant Staphylococcus aureus carrying Pantone‐Valentine leucocidin gene at a referral hospital in United Arab Emirates

Community‐acquired methicillin‐resistant Staphylococcus aureus (CA‐MRSA) is an emerging pathogen in hospitalized patients worldwide. The present study was undertaken to identify CA‐MRSA in hospitalized patients in a 350‐bed tertiary care hospital in Sharjah, UAE over a 2‐year period from January 2011 to December 2012. CA‐MRSA was defined based on identification within first 48 h of admission in the hospital. Staphylococcal cassette chromosome (SCC) mec typing of the CA‐MRSA isolates was carried out by multiplex polymerase chain reaction (PCR). Detection of PVL and mecA genes was done by PCR using the GenoType^® MRSA test system (Hain Lifescience). Patient's clinical data and antimicrobial susceptibility pattern of the CA‐MRSA isolates were also evaluated. Fifty seven of the 187 MRSA isolates were identified as CA‐MRSA. All the CA‐MRSA strains in our study belonged to SCCmecIV type and were positive for both PVL and mecA genes. The patients with CA‐MRSA infections were young (median age, 32 years) and the majority of infections involved the skin and soft tissue (36%). Antimicrobial susceptibility pattern of the CA‐MRSA isolates showed a better susceptibility profile to the non‐beta‐lactam antimicrobials with the exception of ciprofloxacin having 28% resistance. This study evidently strengthens the recent observation of an increase in CA‐MRSA emergence among hospitalized patients in the UAE.