Developmental and behavioral outcomes of preschool-aged children with biotinidase deficiency identified by newborn screening.

Biotinidase deficiency (BD) may cause neurological symptoms and developmental problems. However, newborn screening of BD and early biotin treatment prevent the manifestation of the majority of symptoms. This study intended to examine the developmental and behavioral outcomes as well as maternal anxiety and depressive symptoms of preschool-aged children with BD and to compare these with the outcomes of healthy preschool-aged children. In total, 49 children with BD and 23 healthy children are included. All children were screened for developmental and behavioral problems. Moreover anxiety and depressive symptomatology of their mothers were evaluated. Despite the high percentage of developmental delay in BD group, the numbers of children screened positive for a developmental delay were statistically similar in children with BD and healthy children. Among patients with BD, children with risk of developmental delay had more unfavorable socio-demographic features compared to typically developing ones. Behavioral problem scores, maternal anxiety, and depressive symptoms scores of children with BD were not higher than the healthy children.Conclusion: Children with BD were not different from their healthy peers in terms of developmental and behavioral outcomes. Developmental problems of children with BD may be related to the unfavorable socio-demographic features, not the BD itself. What is known: • Biotinidase deficiency (BD) may result in neurological symptoms and developmental problems. • Newborn screening and early biotin supplementation prevent the manifestation of the majority of symptoms. What is new: • Preschool-aged children with BD identified by newborn screening are not different from their healthy peers in terms of developmental and behavioral outcomes. • Maternal anxiety and depressive symptoms scores of children with BD are similar to scores of healthy children.

Ovarian-protective effects of clotrimazole on ovarian ischemia/reperfusion injury in a rat ovarian-torsion model.

BACKGROUND/AIMS: To evaluate the ovarian-protective effects of clotrimazole on ovarian ischemia/reperfusion injury in a rat ovarian-torsion model. METHODS: 32 Sprague-Dawley rats were randomly divided into four groups: (1) ischemia group (n = 8) in which only left adnexal torsion was performed for 2 h, but no treatment was given; (2) vehicle group (n = 8) in which left adnexal torsion was performed for 2 h and at the end of 2 h ischemia polyethylene glycol (3% PEG, 1 ml, i.p.) was administered and a 24-hour reperfusion was continued; (3) clotrimazole group (n = 8) in which left adnexal torsion was performed for 2 h and at the end of 2 h ischemia clotrimazole (30 mg/kg, i.p.) was administered and a 24-hour reperfusion was continued, and (4) control group (sham-operated, n = 6) in which no adnexal torsion and no treatment were given. The criteria for ovarian ischemia were follicular cell degeneration, vascular congestion, hemorrhage and infiltration by inflammatory cells. Each specimen was scored for each criterion (0, none; 1, mild; 2, moderate; 3, severe). RESULTS: Clotrimazole significantly decreased plasma levels of serum malondialdehyde, ischemia-modified albumin, and total oxidant status. CONCLUSION: This study showed the ovarian-protective effects of clotrimazole on ovarian ischemia/reperfusion injury.

Curcumin nutrition for the prevention of mesenteric ischemia-reperfusion injury: an experimental rodent model.

BACKGROUND: Curcumin is an anti-oxidant molecule known to be a potent inhibitor of nuclear factor-kappaB (NF-kappaB). It has been shown to attenuate ischemia/reperfusion (I/R) injury in several organ systems. In this study, we sought to investigate the effects of curcumin on the prevention of superior mesenteric artery I/R injury in rats. METHODS: Wistar albino rats were randomly allocated to 3 groups: group I, sham operated (n = 10); group II, I/R injury only (n = 10); group III, curcumin-treated I/R cohort (n = 10). Group I animals underwent laparotomy without I/R injury. After group II animals underwent laparotomy, 60 minutes of superior mesenteric artery ligation were followed by 3 hours of reperfusion. In the curcumin group, 15 days before I/R, curcumin (40 mg/kg) was administered by gastric gavage. All animals were sacrificed at the end of reperfusion. Intestinal tissue samples were obtained to investigate intestinal mucosal injury; in addition we estimated levels of myeloperoxidase (MPO) activity, malondialdehyde (MDA), nitric oxide (NO), glutathione (GSH), interleukin (IL)-6, and tumor necrosis factor (TNF)-alpha. RESULTS: There were statistically significant decreases in GSH levels, along with an increase in intestinal mucosal injury scores, MPO activity, MDA levels, NO, IL-6, and TNF-alpha in group I when compared with groups II and III (P = .01). Curcumin treatment in group III produced a significant increase in GSH levels, as well as a decrease in intestinal mucosal injury scores, MPO activity, MDA, and NO levels when compared with group II (P < .05). CONCLUSION: This study showed that curcumin treatment significantly attenuated reperfusion injury in a superior mesenteric artery I/R model in rats.

Cardioprotective effect of cold-blood cardioplegia enriched with N-acetylcysteine during coronary artery bypass grafting.

BACKGROUND: Cold-blood cardioplegia is a well-known method in coronary artery bypass graft surgery, and several authors have used various agents in the enrichment of cold-blood cardioplegia to decrease ischemia-reperfusion injury seen during surgery. N-acetylcysteine, which can increase glutathione levels, is one of the agents added to cardioplegic solutions to decrease myocardial injury. This study was planned to assess the efficiency of N-acetylcysteine-enriched cold-blood cardioplegia on early reperfusion injury in patients with ischemic heart disease undergoing coronary artery bypass grafting, using measurements of cardiac troponin I and malondialdehyde release. METHODS: Thirty patients (11 women and 19 men) with left ventricular ejection fraction greater than 0.40 scheduled for coronary artery bypass grafting were randomly divided into two groups. We used cold-blood cardioplegia enriched with N-acetylcysteine (50 mg per kilogram of body weight) in the first group and cold-blood cardioplegia alone in the second group. Hemodynamic variables and clinical properties of the patients were preoperatively and postoperatively evaluated. Enzyme releases were measured in the early hours after the operation. RESULTS: In the N-acetylcysteine-enriched group cardiac troponin I levels were lower than in the N-acetylcysteine-free group, and this difference was statistically significant. Cardiac troponin I levels increased in both groups in the 6th and 12th hours postoperatively, but there was a statistically significant difference between the two groups. Malondialdehyde levels were significantly higher in the N-acetylcysteine-free group after the 6th, 12th, 24th, and 48th hours postoperatively when compared with the N-acetylcysteine-enriched group. CONCLUSIONS: N-acetylcysteine-supplemented cold-blood cardioplegia minimizes myocardial injury in the early hours after and during the cardiac surgery.

Effect of ascorbic acid treatment on endothelium-dependent and neurogenic relaxation of corpus cavernosum from middle-aged non-insulin dependent diabetic rats.

AIMS: The purpose of the present study was to investigate functional responses and nitric oxide synthase activity in the corpus cavernosum of young control, middle-aged control and middle-aged non-insulin dependent diabetic rats. METHODS: The animal groups were treated with ascorbic acid. RESULTS: Acetylcholine-mediated endothelium-dependent relaxation of cavernosal tissue was significantly attenuated from a maximum of 58.0 +/- 4.1% (1 mmol, n = 10) in young rats to 44.3 +/- 1.6% in aged-matched controls (P < 0.05) and to 23.3 +/- 2.8% in non-insulin-dependent diabetes mellitus rats (P < 0.01). These deficits in acetylcholine responsiveness were completely prevented by ascorbic acid treatment. Non-adrenergic non-cholinergic relaxations evoked by electrical field stimulation (0.5-64.0 Hz) in the corpus cavernosum of middle-aged control and non-insulin dependent (NID) diabetic rats are blunted and were not restored by ascorbic acid. The histochemical findings demonstrated a decrease of nicotinamide adenine dinucleotide phosphate-diaphorase staining in the cavernosal tissue obtained from middle-aged control rats and middle-aged diabetic rats. CONCLUSION: Partial correction by ascorbic acid may suggest the importance of reactive oxygen species and a therapeutic approach in impotent NID diabetic men.

Effects of Turkish pollen and propolis extracts on respiratory burst for K-562 cell lines.

Bee-collected pollen and propolis are apicultural products which are composed of nutritionally valuable substances and contain considerable amounts of polyphenol substances which may act as potent antioxidants. We wanted to show if respiratory burst within a cancer cell lines could be influenced when incubated with pollen and propolis extracts or not. Pollen and propolis extracts at concentrations of 50, 25, 12.5 and 0 mg/ml were prepared by dimethyl sulfoxide (DMSO). K-562 cell cultures and mononuclear cell (MNC) cultures prepared from a peripheral blood sample to serve as control cells were incubated with extracts for 24 h. Determination of respiratory burst was carried out by intracellular dichlorofluorescein (DCFH) test by using flow-cytometric fluorescence analysis. While about 90% and 66% fluorescence was detected at zero concentrations for both K-562 and MNC cultures, fluorescence positivity decreased (between 3.8% and 11.8%) as concentrations of both propolis and pollen extracts increased for K-562 cell culture, but unchanged (between 20% and 83%) for MNC culture. It was concluded that pollen and propolis extracts inhibit respiratory burst within cancer cell lines probably by their antioxidant potentials.