Accuracy of intraoperative electrophysiological testing in confirming correct cochlear implant electrode positions

Objective: To investigate the accuracy of intraoperative electrophysiological studies in detecting incorrectly positioned electrodes in cochlear implant surgery.

Study design: A retrospective chart review.

Setting: Tertiary referral centre.

Patients: In total, 104 consecutive patients with a mean age of 5 years underwent cochlear implant surgery at our centre between January 2012 and December 2013. All patients were implanted with Cochlear Nucleus Freedom implants.

Method: A retrospective study to compare intraoperative neural response telemetry (NRT), impedance and electrode position using Stenver’s transorbital plain X-ray view.

Results: Intraoperative electrophysiological tests for patients with Cochlear Nucleus Freedom implants showed 97% sensitivity and 100% specificity compared with postoperative X-ray imaging.

Conclusion: NRT results for the position of cochlear implants were very accurate when checked by X-ray imaging showing that this technique is sufficient in most cases. Stenver’s plain X-ray view is needed in complicated cases with abnormal NRT testing or difficult electrode insertion.     

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.     

Factors affecting nutritional status among pediatric patients with transfusion-dependent beta thalassemia

Malnutrition affects the growth, efficacy of treatments and quality of life in children suffering from thalassemia. This study was conducted to assess the nutritional status of thalassemic patients and to determine the factors involved. Data were obtained from 140 thalassemic patients aged 8–18 years in Mashhad, Iran, on anthropometry, food record and biochemical profile. The prevalence of malnutrition was 44.3 % for boys and 19.6 % for girls, as determined by low body mass index. Furthermore, 44.3 % of boys and 37.7 % of girls were found to be of short stature. Sum of triceps and subscapular skinfold thickness and arm muscle area (AMA) calculation showed the incidence of 7.4 % leanness and 60.7 % wasting among thalassemic children and adolescents. The average of energy intake met 74 % of recommended dietary allowance, although more than 71 % under-reporting was calculated for food records. The intake of energy, macronutrients, zinc, iron and vitamin E was positively correlated (P < 0.05) with anthropometric measures. Age, age at first transfusion, age of starting chelation and serum alkaline phosphatase were considered as positive predictors for nutritional status, whereas puberty, gender and fasting blood sugar as negative predictors. Nutritional status of thalassemic children and adolescents should be monitored, focusing on their nutrition education and supplementation, treatment protocol and control on blood sugar. These may play important roles in enhancing the quality of life in thalassemic children and adolescents.     

The surgical management of lichen sclerosus of the glans penis: our experience and review of the literature

IntroductionLichen sclerosus (LS) of the glans penis is a chronic, progressive, scleroatrophic inflammatory process of unknown etiology affecting the glans penis, prepuce, and urethra and may lead to severe impairment of sexual and urinary function.AimsTo report our experience of surgical management of LS of the glans penis.Main Outcome MeasuresComplications, patients’ satisfaction, cosmesis, resolution of pain and puritus, and postoperative sexual function and were recorded retrospectively.MethodsThe surgical outcome of the 31 patients who have undergone resurfacing of the glans penis with the use of skin grafting for the management of genital LS in our institute is reported.ResultsAfter a median follow‐up of 12.8 months, 26 patients (84%) were fully satisfied with cosmetic and functional results, and 71% of them have resumed sexual activity.ConclusionsResurfacing of the glans penis represents a simple and reproducible technique for the management of LS and yields excellent functional and cosmetic results. Garaffa G, Shabbir M, Christopher N, Minhas S, and Ralph DJ. The surgical management of Lichen Sclerosus of the glans penis: Our experience and review of the literature. J Sex Med 2011;8:1246–1253.     

Congenital Nasopharyngeal Teratoma in a Neonate

Background

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma (NPT) associated with a wide cleft palate.

Case Presentation

A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6×4×3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma (hairy polyp). The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development.

Conclusion

Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction.     

Comparison of Two Phototherapy Methods (Prophylactic vs Therapeutic) for Management of Hyperbilirubinemia in Very Low Birth Weight Newborns

Objective

Preterm and low birth weight (LBW) infants are at greater risk of developing bilirubin-associated brain damage compared with term infants. Certainly, phototherapy, if used appropriately, is capable of controlling the bilirubin levels in LBW infants; but there is not a unique phototherapy treatment strategy in LBW infants. This study was designed to compare the prophylactic phototherapy and late treatment of jaundiced newborns weighing 1000-1500 grams.

Methods

Sixty newborns with birth weight 1000–1500 g were studied. They were divided into two groups: the “Prophylactic” group, in which phototherapy started within six hours after birth and continued for at least 96 hours, and the "Treatment" group, which received phototherapy when indicated according to birth weight and suspended when bilirubin level fell below 50% of bilirubin level for blood exchange. Mean value of daily transcutaneous bilirubin (TCB), duration of phototherapy, the need for blood exchange, and the highest TCB value in both groups were analyzed.

Findings

In the prophylactic group, the highest daily mean rate of TCB was 7.71±1.84 mg/dl, which happened on the third day. In the treatment group, it was 8.74±1.72 mg/dl on the fourth day after birth. The TCB values in prophylactic group were significantly less than those of the treatment group only on the fourth and fifth days after birth (P<0.001). Although the median duration of phototherapy in the treatment group was shorter than that of the prophylactic group (137.60±57.39 vs 168.71±88.01 hours, respectively), this difference was not statistically significant. Only one neonate needed blood exchange in the treatment group.

Conclusion

The prophylactic phototherapy treatment for babies weighing 1000–1500 g significantly decreases bilirubin levels on the fourth and fifth days after birth but the clinical course of hyperbilirubinemia does not alter in LBW infant, as indicated by the non-significant change in the duration of phototherapy.     

Potential Mechanisms Involved in the Anticonvulsant Effect of Walnut Extract on Pentylenetetrazole-Induced Seizure

Objective

It was the aim of this study to determine the potential effect of walnut kernel extract (WKE) on experimentally induced seizures in rats and to evaluate the role of benzodiazepines and ethosuximide (ESM) within these pathways.

Materials and Methods

Male Wistar rats were selected and divided into eight groups. Seizures were evoked by intravenous infusion of pentylenetetrazole (PTZ; 2 mg/ml/min). In combination with PTZ, animals were treated with vehicle or WKE (100 mg/kg i.p.), with or without cotreatment with either flumazenil (FMZ; 5 mg/kg i.p.), ESM (150 mg/kg i.p.) or diazepam (DPZ; 0.5 mg/kg i.p.).

Results

WKE administration significantly increased the PTZ dose needed to induce the first myoclonic jerk (13.09 ± 1.29 vs. 49.71 ± 12.03 mg/kg; p < 0.001), decreased the severity of seizure grades and reduced the mortality rate to 0%. FMZ did not significantly reduce the anticonvulsant effect of WKE. The combination of DPZ and WKE showed a synergic anticonvulsant effect, whereas ESM had no significant influence (p > 0.05) on the WKE effects.

Conclusion

These findings indicated that WKE was effective at reducing seizure severity, at increasing the dose to the first myoclonic jerk and highly efficacious at preventing mortality, because 100% of animals were protected. It seems that this positive effect could apply through signaling pathways other than benzodiazepine-mediated γ-aminobutyric acid receptors and may at least in part be similar to ESM.Key Words: Walnut kernel, Seizure, Rat, Anticonvulsant effect, Pentylenetetrazole     

Effect of human amniotic membrane on anastomosis leakage in dog model

One of the major concerns in rectal cancer surgery and complicated anastomosis is anastomosis breakdown and leakage. For decades, radiation (as neoadjuvant or adjuv ant therapy) was known as a risk factor for anastomosis leakage. Diverting loop ileostomy has been usually employed to divert the fecal flow and protect complicated anastomosis. However, there is a high rate of complications related to diverting loop ileostomy and its closure. This experimental study was carried out to investigate the effect of human amniotic membrane on colonic anastomosis in dogs that underwent preoperative radiation therapy. In this prospective controlled animal trial study, a total of ten male cross-breed dogs aged 6–8 months and weighing 10–15 kg were randomly assigned in to two groups of five dogs. Group 1 with five dogs received 10 Gy of external radiation preoperatively, while the second didn’t receive radiation. After anesthesia and exploration, about 8 cm above the dentate line, the bowel was cut and end-to-end anastomosis was done in two layers hand sewn technique. HAM measuring 2.5 cm × 2.5 cm was wrapped around the anastomosis and sutured with vicryl 3-0. In each group, one of the dogs died. The non-parametric Mann—Whitney test was used to compare the two groups. Two-tailed P value <0.05 was considered statistically significant. Cross sections of the healed anastomosis were scored according to modified scoring system. Histological evaluation of both groups demonstrated ulceration of the mucosa with infiltration of the acute and chronic inflammatory cells as well as granulation tissue formation. The healing starts by fibroblast proliferation and collagen deposition. A thin fibrotic tissue was formed between the serosa and patch graft. (P > 0.05, i.e., healing in both irradiated and non-irradiated groups was the same). Unfavorable effects of radiotherapy and loop ileostomy may be resolved with application of human amniotic membrane, which influences anastomosis healing and eliminates the need for ileostomy insertion.