Volume CXIV, Number 4, November 7, 1996

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespa

Could Eosinophilia predict clinical severity in nasal polyps?

Abstract Background Although nasal polyps are one of the most frequent diseases, their etiopathogenesis remains unclear.Since eosinophils are the main inflammatory cells in the substantial proportion of nasal polyp tissues, they are considered potentially responsible for the etiopathogenesis and prognosis of the disease. Aim of this study was to investigate the relation between mucosal and peripheral eosinophilia and their relation with disease severity in nasal polyps. Methods The study included 53 patients with nasal polyps who underwent endoscopic sinus surgery. Preoperative Lund-MacKay computed tomography (CT) scores and the Lund-Kennedy endoscopic scores of the patients were recorded. Nasal polyp tissues were stained with hematoxylin and eosin, eosinophil counts were performed using high-power field (HPF, 400×) under the light microscope, and the patients were grouped as those with high mucosal eosinophil count and those with low mucosal eosinophil count. Results The mean Lund-MacKay CT score and the mean Lund-Kennedy endoscopic score were higher in the patients with high mucosal eosinophil count than in those with low mucosal eosinophil count. Likewise, the mean Lund-MacKay CT score and the mean Lund-Kennedy endoscopic scores were significantly higher in the patients with high peripheral eosinophil count than in those with low peripheral eosinophil count (p < 0.05 for both). Moreover, the mean peripheral eosinophil count was significantly higher in the patients with high mucosal eosinophil count than in those with low mucosal eosinophil count (p < 0.05). Conclusion Mucosal and peripheral eosinophilia can be used as a marker to predict disease severity in nasal polyps

The role of p53, Ki-67 and laminin expression in the differential diagnosis of keratoacanthoma and well-differentiated SCC

Introduction: We have aimed to evaluate the difference between the expression of p53, Ki-67, and laminin in keratoacanthoma and well-differentiated SCC (SCC) and to determine its importance in differential diagnosis. Methods: This study totally included 46 cases consisting of 23 cases with keratoacanthoma and 23 with SCC. As well as age, gender, localization, and diameter of the lesion, the expression of p53, Ki-67 and laminin was evaluated. Results: No statistically significant difference was found between KA and well-differentiated SCC in terms of diameter, age, and localization. There was a statistically significant difference between KA and well-differentiated SCC in terms of p53 and Ki-67 staining (P < 0.001). Increased expression of p53 and Ki-67 was found in well-differentiated SCC. A statistically significant correlation was present between the expression of p53 and Ki-67 in KA. A statistically significant difference was detected between KA and well-differentiated SCC in terms of laminin staining (P = 0.018). Increased laminin expression was determined in well-differentiated SCC. Conclusion: We have determined in this study that p53, Ki-67 and laminin may be used as adjuvant immunohistochemical markers in differential diagnosis of KA and well-differentiated SCC

An alternative treatment in hypertriglyceridemia-induced acute pancreatitis in pregnancy: Plasmapheresis

Hormonal influences during pregnancy can compromise otherwise controlled lipid levels in women with familial hypertriglyceridemia and predispose to pancreatitis leading to increased morbidity in both mother and fetus. Both cholesterol and triglyceride levels in serum increase progressively during pregnancy. The mainstay of treatment includes dietary restriction of fatty meal and lipid-lowering medications. Experiences with plasmapheresis are limited. We report two cases of hypertriglyceridemia-induced acute pancreatitis during pregnancy, which were successfully treated by plasmapheresis

O efeito da "sedação multifásica" no exame de tomografia computadorizada e ressonância magnética em crianças, pais e anestesiologistas

JUSTIFICATIVA E OBJETIVOS: O nosso objetivo foi investigar o efeito da "sedação multifásica" em crianças submetidas à Tomografia Computadorizada (TC) ou Ressonância Magnética (RM), em seus pais e nos anestesiologistas responsáveis. "Sedação multifásica" foi definida como "o nível de sedação pretendido obtido com um ou mais agentes através da mesma via ou vias diferentes com mais de uma administração". MATERIAL E MÉTODOS: Cem crianças e seus respectivos pais foram randomicamente designados para um dos dois grupos de estudo. Na fase 1, os pacientes do Grupo I receberam midazolam (0,5 mg.kg-1) em 5 mL de suco de frutas e os pacientes do Grupo II (grupo controle) receberam apenas suco de frutas. Na fase 2, após a canulação intravenosa (iv), bolus de propofol foi administrado para alcançar a sedação adequada para realização do exame de imagem. Os escores de ansiedade das crianças e de seus pais foram registrados usando a escala de Oucher e o IDATE, respectivamente, e a satisfação dos pais foi avaliada pela escala analógica visual (EAV). O número de tentativas para canulação iv, tempo de preparação e quantidade de hipnóticos foram registrados. RESULTADOS: O estado de ansiedade das crianças foi semelhante entre os grupos antes da pré-medicação, porém mais tarde esse nível foi menor no Grupo I. Antes do procedimento, o escore dos pais no IDATE foi semelhante, mas depois foi menor no Grupo I. A satisfação dos pais no Grupo I foi maior que no Grupo II. O número de tentativas de canulação iv e a dose necessária de propofol foi menor no Grupo I. CONCLUSÃO: O procedimento de "sedação multifásica" diminui a dor e a ansiedade das crianças e a ansiedade dos pais, aumentando a sua satisfação. Ele fornece uma sedação confortável e segura, pois possui um processo de preparação curto e sem problemas também para o anestesiologista responsável

Monitorization of the effects of spinal anaesthesia on cerebral oxygen saturation in elder patients using near-infrared spectroscopy

OBJECTIVE: Central blockage provided by spinal anaesthesia enables realization of many surgical procedures, whereas hemodynamic and respiratory changes influence systemic oxygen delivery leading to the potential development of series of problems such as cerebral ischemia, myocardial infarction and acute renal failure. This study was intended to detect potentially adverse effects of hemodynamic and respiratory changes on systemic oxygen delivery using cerebral oxymetric methods in patients who underwent spinal anaesthesia. METHODS: Twenty-five ASA I-II Group patients aged 65-80 years scheduled for unilateral inguinal hernia repair under spinal anaesthesia were included in the study. Following standard monitorization baseline cerebral oxygen levels were measured using cerebral oximetric methods. Standardized Mini Mental Test (SMMT) was applied before and after the operation so as to determine the level of cognitive functioning of the cases. Using a standard technique and equal amounts of a local anaesthetic drug (15 mg bupivacaine 5%) intratechal blockade was performed. Mean blood pressure (MBP), maximum heart rate (MHR), peripheral oxygen saturation (SpO2) and cerebral oxygen levels (rSO2) were preoperatively monitored for 60 min. Pre- and postoperative haemoglobin levels were measured. The variations in data obtained and their correlations with the cerebral oxygen levels were investigated. RESULTS: Significant changes in pre- and postoperative measurements of haemoglobin levels and SMMT scores and intraoperative SpO2 levels were not observed. However, significant variations were observed in intraoperative MBP, MHR and rSO2 levels. Besides, a correlation between variations in rSO2, MBP and MHR was determined. CONCLUSION: Evaluation of the data obtained in the study demonstrated that post-spinal decline in blood pressure and also heart rate decreases systemic oxygen delivery and adversely effects cerebral oxygen levels. However, this downward change did not result in deterioration of cognitive functioning

Can we use magnesium for sedation in the intensive care unit for critically ill patients: Is it as effective as other sedatives?

Objectives: The aim of this prospective, randomized study was to investigate the effect of magnesium added to midazolam on the hemodynamics, transition time to a T-piece, mechanical ventilation duration, additional sedative-analgesic requirement using bispectral index (BIS) monitorization and sedation scales