Abdul Rahim Wong

Arterial compliance and vitamin E blood levels with a self emulsifying preparation of tocotrienol rich vitamin E

The tocotrienol vitamin E has potent antioxidant property, however absorption is low due to high lipid solubility. A self emulsifying preparation of tocotrienol rich vitamin E (SF-TRE) had been reported to increase their bioavailability. This randomized, placebo controlled, blinded end point clinical study aimed to determine the effects of 50, 100 and 200 mg daily of SF-TRE and placebo for two months on arterial compliance and vitamin E blood levels. Assessment of arterial compliance by carotid femoral pulse wave velocity (PWV) and augmentation index (AI), plasma vitamin E, serum total cholesterol and low density lipoprotein cholesterol were taken before and after 2 months’ treatment in 36 healthy males. Un-supplemented tocotrienol levels were low, after treatment, all SF-TRE treated groups had significantly higher plasma α, δ and δ tocotrienol concentrations compared to placebo. Augmentation index change from baseline to end of treatment for groups placebo, 50, 100, and 200 mg were 2.22±1.54, −6.59±2.84, −8.72±3.77, and −6.27±2.67% respectively (p=0.049, 0.049, and 0.047 respectively). Groups 100 and 200 mg showed significant improvement after treatment with pulse wave velocity reductions of 0.77 m/s and 0.65 m/s respectively (p=0.007 and p=0.002). There was no effect of SF-TRE on serum lipids. We conclude that there was a trend towards improvement in arterial compliance with 2 months’ of SF-TRE.

Sildenafil as treatment for human immunodeficiency virus-related pulmonary hypertension in a child

Abstract:  Human Immunodeficiency Virus (HIV)‐related pulmonary hypertension is a relatively rare disease that can affect HIV sufferers. This is almost always associated with a poor outcome and death. An 18 month‐old girl, probably the youngest on record, was diagnosed to have pulmonary hypertension (PHT) and retrospectively found to have HIV infection. Sildenafil was used to control her PHT and she remains alive even after 2 years.

Hansenula anomala infection in a neonate.

Abstract: We present an unusual neonatal fungal infection, Hansenula anomala in a very low birthweight infant who underwent abdominal surgery for an omphalocele. Despite treatment with adequate doses of amphotericin B, the yeast continued to grow from the blood culture, and was only eradicated with the use of oral ketoconazole.

Device Closure of Patent Ductus Arteriosus in Marfan Patients: Safety and Effect on the Aortic Root Diameter

OBJECTIVES There is little evidence in the medical literature about safety of PDA device closure and its effects on aortic root diameter in Marfan patients. We reported on nine Marfan patients whose ducts were occluded by coil or Amplatzer Duct Occluder. PATIENTS AND INTERVENTIONS Two patients had aneurismal type E ducts which were closed by a single coil, respectively. The others had conical ducts for which even Amplatzer occluder, stiff, or double coils were used. RESULTS No untoward consequence was found during a median follow up period of 18.5 months (range 3 months to 6.33 years). Two patients had small residual shunts. The observed change in the mean ratio of aortic root diameter to its estimated value was statistically insignificant in seven patients who had complete data on their aortic root diameter before PDA closure and during the follow up. CONCLUSIONS We concluded that PDA device closure in Marfan patients is safe. Risk of residual shunt is higher even after closure of small ducts.

Prevalence of β-2 adrenergic receptor (β2AR) polymorphisms and its influence on a model used to assess endothelial function using pulse wave analysis (PWA)

BACKGROUND Pulse wave analysis (PWA) combined with beta(2)-agonist challenge has recently been used to assess endothelial function. beta-2 adrenergic receptor (beta(2)AR) polymorphisms may affect response to beta(2)-agonist. We determined whether beta(2)AR polymorphisms influence endothelial response in our model using PWA and salbutamol. METHODS 388 healthy Malay subjects (177 males, 211 females) were genotyped for 5 functionally important single nucleotide polymorphisms (SNPs) of beta(2)AR; 298 subjects proceeded with assessment of endothelial function. The parameter augmentation index (AIx) was recorded non-invasively using SphygmoCor. Recording of AIx at baseline was followed by administration of 500 microg sublingual glyceryl trinitrate (GTN). AIx recordings were repeated at 3, 5, 10, 15 and 20 min post-GTN. Subjects then inhaled 400 microg of salbutamol before AIx recordings at 5 min intervals up to 20 min. Maximum changes in AIx after GTN and salbutamol represented endothelium independent and endothelium dependent vasodilatation (EDV) respectively. RESULTS Allele frequencies of mutated Gly16, Glu27, Ile164, -20C and -47C were 47%, 6.8%, 0%, 30% and 9.3% respectively. No significant differences in EDV were noted between genotype groups of each studied SNPs. CONCLUSIONS Assessment of endothelial function using PWA and salbutamol was not influenced by beta(2)AR polymorphisms.

Arrhythmogenic right ventricular cardiomyopathy in an 11-year-old girl and typical echocardiographic features.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare entity that affects children as well as adults. This is a genetic disorder that can be inherited and whose hallmark is fibrofatty replacement of ventricular muscle, especially of the right side, with ventricular arrhythmias and progressive heart failure. We describe a case of an 11-year-old Saudi girl with ARVC and the echocardiographic characteristics that had been highlighted by the Taskforce for ARVC, and we suggest that a concurrent criterion be added to aid further echo identification in children.

Type B Patent Ductus Arteriosus

Knowledge of the different types of ductus arteriosus and their anatomical shape, length, and narrowest diameter aids in the decision to close the patent ductus arteriosus (PDA) either by transcatheter or surgical methods. Krichenko et al. [1] published an angiographic classification of PDAs in 1989. Type B PDAs are best described as window-like PDAs with a short length, with the narrowest end at the aorta and an ampulla at the pulmonary end. In that series, type B PDAs represented approximately 17% of all PDAs. A series from India did not find type B PDAs in their group [2]. We present an angiographic picture depicting a type B PDAwith a relatively long segment, making it conical in shape with the narrowest end at the aortic segment (Fig. 1). This was closed via three detachable coils (flipper coils, Cook, Bloomington, IN, USA)—8 mm with four loops, followed by a 6.5 mm with four loops and a 3 mm with five loops—delivered retrogradely via the aorta with only a single loop in the aortic end (Fig. 2). References