Congenital xerocytosis

Hereditary xerocytosis is a genetic disease inherited as an autosomal dominant trait and is a rare cause of hemolytic anemia. It is caused by abnormal erythrocyte membrane permeability: monovalent cation pump activity is increased and the Na/K pump cannot compensate for the K lost. As a consequence, xerocytes dehydrate, becoming rigid and sensitive to metabolic stress and oxidation. Morbility depends on the severity of the hemolytic anemia. Periodic episodes of jaundice are common during mild infections; most patients remain asymptomatic but experience mild-to-moderate hemolytic anemia, which is generally well compensated. The diagnostic clues are a markedly increased flow through the Na/K pump with a decrease in total intracellular cation content and subsequent red cell dehydration. Treatment is based on monitoring for eventual complications and careful observation during infections, which may worsen the anemia. Splenectomy is not useful and for some authors may even be contraindicated. The prognosis is generally very good. We report the case of a patient with episodes of hemolytic anemia during intercurrent infections and positive diagnostic tests for hereditary xerocytosis.     

The relationships between dental age, chronological age and bone age in Turkish adolescents with constitutional delay of growth

The aim of this study was to investigate the relationships between dental age and bone age in Turkish adolescents with constitutional delay of growth and compare them with a group of normal, healthy adolescents. Left hand and wrist radiographs and dental panaromic radiographs of 33 adolescents (25 boys and 8 girls) aged between 10 and 16 years with constitutionally delayed growth were assessed. The control group comprised 41 healthy adolescents (24 boys, 17 girls) aged between 10 and 16 years. Bone age was determined according to Greulich and Pyle; dental age was assessed using the Demirjian method. In the control group, no statistical difference was found between chronological, bone and dental ages. In the group of adolescents with constitutional growth delay, there was no significant difference between chronological age and dental age, but the differences between dental age and bone age and between chronological age and bone age were found to be statistically significant. It was found that Demirjian's dental age assessment is a valid method for scoring dental age in Turkish adolescents. Adolescents with constitutional delay of growth had dental maturation appropriate for chronological age, but not for bone age.     

The effect of endometrial injury on implantation and clinical pregnancy rates

Aim: To assess the effect of endometrial scratching (ES) on in vitro fertilization-embryo transfer outcome (IVF-ET)

Materials and methods: Retrospective matched case control study including all fresh IVF cycles performed between January 2006 and December 2012 at an academic IVF center. ES with an endometrial biopsy catheter was performed in the cycle preceding the index IVF cycle. Patients (n?=?238) were pair matched with controls according to age, number of previous failed IVF cycles and number of embryos transferred.

Results: Demographic and cycle characteristics were comparable in all of the following: age, number of previous cycles, number of collected oocyte, number of embryos transferred and quality of transferred embryos. Implantation, clinical and ongoing pregnancy rates were comparable in both groups (28%, 34% and 18.4% vs 30%, 40.3% and 29%, in ES group and controls, respectively). Logistic regression analysis found no significant association between ES and pregnancy rate.

Conclusions: Mechanical endometrial stimulation did not improve implantation and pregnancy rates. Furthermore, no factors that may predict which patients could benefit from ES were identified. Further prospective studies are warranted to evaluate possible benefits in different subsets of patients such as patients with recurrent implantation failures.     

Protective effect of L-arginine on gentamicin-induced nephrotoxicity in rats

Introduction:

L-arginine has a protective effect on gentamicin-induced renal failure and it may decrease the tubular reabsorption of another cationic substance, gentamicin due to its cationic structure. The aim of this study is to compare the possible protective effects of L-arginine and its inactive isomer D-arginine on gentamicin-induced nephrotoxicity in rats.

Materials and Methods:

Wistar albino rats were housed in metabolic cages and assigned to six groups as: control group, gentamicin (100 mg/kg), gentamicin + L-arginine (2 g/l), gentamicin + D-arginine (2 g/l), gentamicin + L-arginine + Nv-nitro-L-arginine methyl ester (L-NAME) (100 mg/l) and gentamicin + D-arginine + L-NAME. Gentamicin was administered by subcutaneous injections and the other drugs were added in drinking water for seven consecutive days. The animals were killed by decapitation and intracardiac blood and urine samples were obtained on the seventh day. Blood urea nitrogen, serum creatinine, sodium, potassium, urine gamma glutamyl transferase, creatinine, sodium, potassium and gentamicin levels were measured using High Performance Liquid Chromatography (HPLC) technique.

Results:

Gentamicin treated group had significant increase in blood urea nitrogen, serum creatinine, fractional Na excretion and urine gamma glutamyl transferase levels, and significant decrease in creatinine clearance compared to the control group. L-arginine and D-arginine reversed these findings. L-NAME abolished the nephroprotective effect of L-arginine. The urinary levels of gentamicin were significantly increased in rats treated with L-arginine or D-arginine compared to those treated with gentamicin. L-arginine and D-arginine reversed the advanced degenerative changes due to gentamicin administration in histopathological examination.

Conclusion:

Our study revealed the protective effect of L-arginine on gentamicin-induced nephrotoxicity, the contribution of the cationic feature of L-arginine, and the major role of NO in this protective effect.KEY WORDS: D-arginine, gentamicin, HPLC, L-arginine, nephrotoxicity     

Initial Oxygenation Response to Inhaled Nitric Oxide Predicts Improved Outcome in Congenital Diaphragmatic Hernia

Background

Pulmonary hypertension (PH) is the most important complication of congenital diaphragmatic hernia (CDH) and still has a high mortality rate. The aim of this study was to evaluate the effectiveness of inhaled nitric oxide therapy in PH due to CDH.

Methods

Hospital records of children who had undergone inhaled nitric oxide therapy for PH due to CDH between June 2009 and December 2011 were reviewed.

Results

Twenty-nine patients had a diagnosis of CDH at the time of study, and eight of these patients underwent nitric oxide therapy because of failure of conventional ventilation techniques, which was successful in five of these patients. Patients who had a good overall outcome of nitric oxide therapy experienced rapid improvement (pretreatment, mean PaO₂ = 44.8 mmHg; after the first hour of therapy, mean PaO₂ = 96.8 mmHg), whereas patients with no response did not have a similar course (pretreatment, PaO₂ = 37 mmHg; after the first hour, PaO₂ = 54.6 mmHg).

Conclusion

Inhaled nitric oxide therapy seems to increase survival in PH due to CDH. No predictive parameters to orient patient selection could be identified; however, the early response seemed to predict the overall outcome. Good results in our series were attributed to routine use of sildenafil and dopamine, along with the nitric oxide inhalation.     

The potential antiepileptic activity of astaxanthin in epileptic rats treated with valproic acid

ObjectivesEpilepsy is a neurological disease characterized by sudden, abnormal, and hyper- discharges in the central nervous system (CNS). Valproic acid (VPA) is commonly used as a broad-spectrum antiepileptic therapeutic. However, in many cases, patients develop resistance to VPA treatment due to overwhelming oxidative stress, which in turn might be a major catalyst for disease progression. Therefore, antioxidants can potentially become therapeutic agents by counteracting reactive oxygen species (ROS)-mediated damage. The present study is aimed to evaluate the potential antiepileptic effect of astaxanthin (ASTA) in pentylenetetrazol (PTZ) induced epileptic model rats that are chronically treated with VPA for 8 weeks.MethodFifty-male Wistar rats were randomly divided into five groups: Non-PTZ group, PTZ, PTZ/VPA, PTZ/ASTA, and PTZ/VPA/ASTA treated groups.ResultsPTZ/VPA treated group showed a neuroprotective effect with improvement in antioxidant levels, behavioral test, and histopathological changes induced by PTZ. VPA also exhibited an anti-inflammatory effect as its treatment resulted in the reduction of tumor necrosis factor-α (TNF-α). ASTA exhibited an anticonvulsant effect and enhanced anti-inflammatory effect as compared to VPA. During the combined therapy, ASTA potentiated the antiepileptic effect of the VPA by reducing the oxidative stress and TNF-α as well as increased the glutathione (GSH) levels. Also, there were substantial improvements in the behavioral and histopathological changes in the VPA/ASTA treated group as compared to the VPA treated group.ConclusionASTA could have an antiepileptic and anti-inflammatory effect by reducing ROS generation. Therefore, co-administration of both the therapeutics (VPA/ASTA) has a synergistic effect in treating epilepsy and could potentially minimize recurrence and/or exacerbation of seizures.     

Efficacy of levobupivacaine wound infiltration with and without intravenous lornoxicam for post-varicocoele analgesia: a randomized, double-blind study

BACKGROUND AND OBJECTIVE: The oxicam NSAID lornoxicam is a potent analgesic with excellent anti-inflammatory properties in a range of painful and/or inflammatory conditions, including postoperative pain. Levobupivacaine, the S-(-)-isomer of bupivacaine, is a long-acting local anaesthetic that can be infiltrated into wounds for management of postoperative pain. We assessed the analgesic efficacy of lornoxicam when administered as an adjuvant to levobupivacaine wound infiltration after varicocoele operation. METHODS: Sixty patients who underwent varicocoele surgery were randomly assigned to three different treatment groups. Before skin closure, patients received the following treatments: group I (n=20) patients received normal saline 20 mL wound infiltration and intravenous lornoxicam (Xefo, Nycomed Pharma AS, Roskilde, Denmark) 2 mL (8 mg); group II (n=20) patients received 0.25% levobupivacaine (Chirocaine, Abbott Scandinavia AB, Solna, Sweden) 10mL with normal saline 10 mL wound infiltration and intravenous normal saline 2 mL; group III (n=20) patients received 0.25% levobupivacaine 10 mL with normal saline 10 mL wound infiltration and intravenous lornoxicam 2 mL (8 mg). Pain scores and total pethidine (meperidine) consumption were measured at 1, 2, 4, 6, 12 and 24 hours postoperatively. Time to first analgesic requirement and patient satisfaction were also compared post-surgery. RESULTS: Pain scores during the first 6 hours postoperatively were significantly lower in group III than in group I and group II (p<0.01). Total pethidine consumption was significantly lower in group III (34.0+/-28.0 mg) than in group I (74.0+/-25 mg) and group II (76.0+/-29 mg) [p<0.01]. Time to first analgesic was also significantly longer in group III (14.8+/-8.4 hours) than in group I (6.2+/-5.2 hours) and group II (5.8+/-7.1 hours) [p<0.01]. The incidence of postoperative nausea and vomiting was significantly lower in group III than in group I and group II (p<0.05). More patients in group III described their analgesia as good or excellent than in group I or group II (p<0.01). CONCLUSION: In this study, levobupivacaine wound infiltration with adjuvant intravenous lornoxicam administration was associated with better postoperative analgesia during the early postoperative hours after varicocoele surgery than that induced by lornoxicam alone or levobupivacaine wound infiltration alone.     

Gender Comparisons of Unhealthy Weight-control Behaviors Among Sixth-Graders

ObjectiveTo examine gender differences in unhealthy weight-control behaviors (UWCB) and identify key psychosocial and demographic correlates of UWCB among sixth-graders.MethodsA cross-sectional survey was completed by 146 boys and 153 girls at a middle school. Secondary data analyses included bivariate tests and multivariable logistic regression.ResultsForty-seven percent of participants reported 1 or more UWCB, with no differences by gender (P = .75). Factors common to boys and girls included: lower global self-esteem; lower body-esteem; and greater negative parental modeling among participants who engaged in UWCB compared to those who did not. However, multivariable models revealed gender differences. Among boys, body mass index, negative parental modeling, and global self-esteem retained statistically significant associations with UWCB after controlling for other variables in the model, whereas race and weight-related body-esteem remained significant for girls.Conclusions and ImplicationsThis research highlights the need for gender-specific UWCB prevention programs implemented in late childhood and early adolescence.     

New procedure for treatment of atrial fibrillation in patients with valvular heart disease

Patients with valvular heart disease suffer from atrial fibrillation for more than 12 months after valve surgery and have a low probability of remaining in sinus rhythm. We performed an intra-operative procedure similar to surgical maze ??? procedure for conversion of this arrhythmia to sinus rhythm. We did this study to evaluate the efficacy of this procedure to restore the sinus rhythm in patients with valvular heart disease. 28 patients with valvular heart disease and chronic persistent atrial fibrillation underwent different combinations of valve surgery and concomitant reduction of left and right atrial size and resection of both atrial auricles in Shahid Madani cardiothoracic center from September 2004 to October 2008. The procedure for atrial fibrillation treatment was performed with cardiopulmonary bypass and after mitral valve replacement. There was one in-hospital death postoperatively because of respiratory failure, but no other complication till 6 months after the operation. Out of 28 patients, 23 were in sinus rhythm one week after the operation, one patient had junctional rhythm after the operation that restored to sinus rhythm and 4 patients had persistent atrial fibrillation. During the 12-month follow up, atrial fibrillation was corrected in 82.14%. Doppler echocardiography in these patients with sinus rhythm demonstrated good atrial contractility. This procedure on both atria is effective and less invasive than the original maze procedure to eliminate the atrial fibrillation, and can be performed in patients with valvular heart disease without increasing the risk of operation.