G2-lymphocyte chromosomal radiosensitivity in patients with LPS responsive beige-like anchor protein (LRBA) deficiency

Abstract

Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency is an autosomal recessive primary immunodeficiency disease characterized by a CVID-like phenotype, particularly severe autoimmunity and inflammatory bowel disease. This study was undertaken to evaluate radiation sensitivity in 11 LRBA-deficient patients. Therefore, stimulated lymphocytes of the studied subjects were exposed to a low dose γ-radiation (100 cGy) in the G₂ phase of the cell cycle and chromosomal aberrations were scored. Lymphocytes of age-sex matched healthy individuals used in the same way as controls. Based on the G₂-assay, six (54.5%) of the patients had higher radiosensitivity score comparing to the healthy control group, forming the radiosensitive LRBA-deficient patients. This chromosomal radiosensitivity showed that these patients are predisposed to autoimmunity and/or malignancy, and should be protected from unnecessary diagnostic and therapeutic procedures using ionizing radiation and exposure to other DNA damaging agents.     

Silver nanoparticles as active ingredient used for alcohol-free mouthwash

We developed an effective and non-irritant mouthwash that is alcohol-free and has a low concentration of silver nanoparticles (SNP) in order to be used for preventing oral cavity infections in immunocompromised oncologic patients. We studied antimicrobial effects of silver nanoparticles (SNP) in the range of (50–0.024 µg/ml) and 3% of ethanol (30,000 µg/ml) in mouthwash. Antimicrobial effects of two treatments were studied by doing challenge test on microorganisms such as Streptococcus mutans, Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa, Candida albicans and measuring MIC and MBC (MFC) values of SNP toward mentioned microorganisms. These values of SNP respectively were in the range of (0.78–3.12) and (1.56–12.5 µg/ml). Results showed that SNP in the MIC and the lower concentrations killed all of the used microorganisms. No difference was observed between the antimicrobial effect of ethanol-free mouthwash containing SNP and mouthwash containing SNP and ethanol (30,000 µg/ml). SNP has high antimicrobial effects at low concentrations and it can be a good alternative for ethanol (30,000 µg/ml) because ethanol is also irritating, especially to sensitive or inflamed mucosa.     

Comparison of the Effect of Fentanyl,Sufentanil, Alfentanil and Remifentanil on Cardiovascular Response to Tracheal Intubation in Children

Objective

Laryngoscopy and tracheal intubation may cause significant cerebral and systemic hemodynamic responses. Many drugs have been shown to be effective in modifying these hemodynamic responses, including fentanyl, sufentanil, alfentanil and remifentanil. The purpose of the current study was to compare the efficacy of fentanyl, sufentanil, alfentanil and remifentanil on blunting cardiovascular changes during laryngoscopy and intubation in children.

Methods

Eighty children, 1–6 years old, classified as American Society of Anesthesiologists physical status I and II who were scheduled for elective surgery with general anesthesia and orotracheal intubation, were enrolled in this randomized and double-blinded study. Patients were randomly assigned into four groups of 20 patients. Group F received fentanyl 1µg/kg⁻¹, group S received sufentanil 0.1 µg/kg⁻¹, group A received alfentanil 10 µg/kg⁻¹ and group R received remifentanil 1 µg/kg⁻¹ intravenously. After establishment of neuromuscular blockade confirmed with a nerve stimulator, laryngoscopy and orotracheal intubation were performed 3 min after induction. Hemodynamic variables including systolic and diastolic blood pressure (SAP, DAP) and heart rate (HR) were recorded at base line (before opioid administration), before laryngoscopy and one minute after orotracheal intubation.

Findings

The patients'' characteristics and laryngoscopy grade were similar in all groups. There was no significant difference in the mean values of SAP, DAP and HR at each measured time between the four groups. There was significant difference in the mean values of SAP, DAP and HR measured over time in each group.

Conclusion

The intravenous fentanyl attenuated laryngoscopy-induced SAP, DAP and HR increases better than sufentanil, alfentanil or remifentanil and hemodynamic stability is better preserved with fentanyl.     

Prevalence of Celiac Disease in Iranian Children with Recurrent Abdominal Pain Referred to a Pediatric Referral Center

Objective

Clinical features of Iranian children with celiac disease (CD) are still unknown and there is scant information about atypical presentation of celiac disease from Iran. The aim of this study was to determine prevalence of CD in Iranian children presenting with functional abdominal pain (FAP).

Methods

In this cross-sectional study, 301 children affected by FAP were screened for CD by anti-tissue transglutaminase antibody (tTG IgA). IgA antibody was also measured to exclude IgA deficiency. The antibodies were measured by enzyme linked immunosorbent assay. Diagnosis of CD was confirmed by duodenal biopsy that was scored according to the Marsh classification in cases with abnormal titer of tTG antibody.

Findings

A total of 301 children (138 males, 163 females) with FAP were studied. Endoscopic duodenal biopsy was taken for patients with positive and borderline tTG test. Two out of 301 cases were IgA deficient and celiac disease was suspected for one of them based on histological findings. Four out of 299 patients with normal IgA had abnormal tTG titer; intermediate ranges (16–23 U/ml) were detected in 1 and positive ranges (≥24 U/ml) in 3 cases. CD was suggested in all patients with abnormal titer of tTG (1.33%) based on histological findings.

Conclusion

The prevalence of celiac disease in children with FAP is estimated 1.3% (nearly 2 times higher than in normal population) in Iran.     

Comparison of mild and microdose GnRH agonist flare protocols on IVF outcome in poor responders

Purpose  

To compare the IVF outcome of clomiphene citrate/gonadotropin/antagonist (mild protocol) and microdose GnRH agonist flare protocols for poor responders undergoing in vitro fertilization.     

A randomized trial of ovulation induction with two different doses of Letrozole in women with PCOS

Objective  

To compare the effects of either a 5 or 7.5 mg daily dose of Letrozole in PCOS women undergoing ovulation induction and timed intercourse.     

Correlation of myometrial thickness and the latency interval of women with preterm premature rupture of the membranes

Objective  

To verify the hypothesis that a correlation exists between myometrial thickness (MT) and latency interval (LI) in patients with preterm premature rupture of membrane (PPROM).     

An unusual presentation of anetoderma: a case report

Background  

Anetoderma is a benign condition with focal loss of dermal elastic tissue resulting in localized areas of flaccid or herniated saclike skin. Currently, anetoderma is classified as either primary (idiopathic), or secondary anetoderma (which is associated with a variety of skin conditions, penicillamine use, or neonatal prematurity). Lesions appear on the upper arms, trunk, and thighs.     

Normal adrenal function in an infant following a pregnancy complicated by maternal adrenal cortical carcinoma and mitotane exposure

Maternal adrenal cortical carcinoma in pregnancy is rare. We report a case of an infant born to a mother with a history of adrenal cortical carcinoma. The pregnancy was complicated by fetal exposure to mitotane and dexamethasone. Despite the potential teratogenic exposures, there was no evidence of adrenal dysfunction in the infant. Growth and development at 12 months of age are normal and prognosis appears favorable. The long-term impact of fetal exposure to mitotane and glucocorticoid requires further investigation.