Comparison of the Exposure Obtained by Endoscope and Microscope in the Extended Trans-Sphenoidal Approach

Objective: Trans-sphenoidal surgery is often combined with other approaches for the treatment of middle cranial base tumors. By combining a maxillotomy with trans-sphenoidal approach, significantly wider exposure to these regions is gained. However, endoscope-assisted techniques have also been used for sellar and parasellar and upper clival regions. Methods: An extended trans-sphenoidal approach was performed on 10 cadaver heads using the operating microscope and was repeated with a 0-degree endoscope. The mean horizontal and vertical distances were measured and pictured for each technique, and both distances were compared using a parametric paired Student's t-test. Results: The mean horizontal distances in the 10 specimens were 19.5 ± 1.8 mm by microscope and 27.5 ± 2.2 mm by endoscope, and the mean vertical distances were 25.8 ± 1.9 mm by the microscope and 34.5 ± 3.5 mm by the endoscope. Conclusion: The aim of this study was to quantify the amount of exposure obtained with an extended trans-sphenoidal approach and to compare both endoscopic and microscopic techniques. Using the endoscope in conjunction with the operating microscope may provide additional exposure and better access in skull base surgery.     

Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

Molecular Characterization of a Full Genome Turkish Hepatitis C Virus 1b Isolate (HCV-TR1): A Predominant Viral Form in Turkey

Based on direct sequencing information from 5UTR and NS5B regions, we identified subtype 1b as a predominant hepatitis C virus genome in Turkey, which affected more than 91% of 79 patients studied. Next, the full genome sequence of a Turkish 1b isolate was obtained by the cloning of polypeptide-encoding region into 7 overlapping fragments. Turkish 1b isolate, which was named HCV-TR1, comprises 9361 nucleotides, including 306 nucleotides of 5UTR, a single long open reading frame of 9033 nucleotides, and 22 nucleotides of 3UTR. When compared to HCV 1b polypeptide sequences available at GenBank, the predicted polypeptide displayed a total of 36 amino acid substitutions, of which 16 was specific for HCV-TR1 isolate. Despite these changes, major structural and functional motifs of HCV proteins were maintained in HCV-TR1. In contrast, HCV-TR1 displayed amino acid substitutions in 6 out of 9 major cytotoxic T-cell epitopes. These data suggest that HCV-TR1 encodes functionally intact viral proteins, but it also encodes altered viral epitopes, which may affect host immune-response.     

Memory impairment in patients with cirrhosis

BACKGROUND AND AIM: Subclinical hepatic encephalopathy (HE) in cirrhotic patients is usually characterized by memory impairment and psychomotor slowing. Our aim was to investigate memory status in cirrhotic patients with and without clinically overt HE. MATERIAL AND METHODS: Thirty-two cirrhotic patients (10 female and 22 male) aged 49 +/- 17 years and 20 healthy subjects (six female and 14 male) aged 46 +/- 12 years were included in the study. Memory status was defined by Wechsler Memory Scale, verbal memory process and complex memory process tests. RESULTS: Grade-1 HE was detected in 7 (22%) patients with cirrhosis. We detected 36 to 92% decrement in various memory tests in cirrhotic patients without HE as compared to healthy subjects. The scores for all psychometric testing results were significantly lower in cirrhotic patients without HE as compared to healthy subjects. We detected 42.9 to 100% decrement in various memory tests in cirrhotic patients with HE than cirrhotic patients without HE. However, there was no statistical significant difference between cirrhotic patients with and without HE. There was no statistical significant difference in cirrhotic patients with Child-Pugh A, B, and C. CONCLUSION: In conclusion, memory status was influenced in which patients with cirrhosis yet has a normal mental and neurological status to routine clinical examination (subclinical HE). Occasionally, decreased memory performance may adversely affect the satisfaction and lifestyle of these patients. Therefore, subclinical HE is an important social problem.     

Erratum to: Evaluation of cardiac functions in juvenile systemic lupus erythematosus with two-dimensional speckle tracking echocardiography

Clinical Rheumatology -     

Hepatocellular carcinoma: Where there is unmet need

Hepatocellular carcinoma (HCC) is a complex and heterogeneous tumor most commonly associated with underlying chronic liver disease, especially hepatitis. It is a growing problem in the United States and worldwide. There are two potential ways to prevent HCC. Primary prevention which is based on vaccination or secondary prevention involving agents that slow down carcinogenesis. Several pathways have been thought to play a role in the development of HCC; specifically, those involving vascular endothelial growth factor (VEGF)‐mediated angiogenesis, WNT, phosphatidylinositol 3‐kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR), AMP‐activated protein kinase (AMPK), and c‐MET. Currently, there are only a limited number of drugs which have been proven as effective treatment options for HCC and several clinical trials are testing drugs which target aberrations in the pathways mentioned above. In this review, we discuss currently approved therapies, monotherapies and combination therapy for the treatment of HCC.     

MRI quantification techniques in fatty liver: the diagnostic performance of hepatic T1, T2, and stiffness measurements in relation to the proton density fat fraction

PURPOSENonalcoholic fatty liver disease (NAFLD) can progress to liver cirrhosis and is predicted to become the most frequent indication for liver transplantation in the near future. Noninvasive assessment of NAFLD is important for diagnosis and patient management. This study aims to prospectively determine the liver stiffness and T1 and T2 values in patients with NAFLD and to compare the diagnostic performance of magnetic resonance elastography (MRE) and mapping techniques in relation to the proton density fat fraction (PDFF).METHODSEighty-three patients with NAFLD and 26 participants with normal livers were imaged with a 1.5 T scanner. PDFF measurements obtained from the multiecho Dixon technique were used to quantify the liver fat. MRE, native T1 mapping (modified Look-Locker inversion recovery [MOLLI] schemes 5(3)3, 3(3)3(3)5, and 3(2)3(2)5 and the B1-corrected variable flip angle [VFA] method), and T2 mapping values were correlated with PDFF. The diagnostic performance of MRE and the mapping techniques were analyzed and compared.RESULTST1 values measured with the MOLLI schemes and the B1-corrected VFA (p < 0.001), and the stiffness values from MRE (p = 0.047) were significantly higher in the NAFLD group. No significant difference was found between the groups in terms of T2 values (p = 0.127). In differentiation of the NAFLD and control groups, the B1-corrected VFA technique had slightly higher accuracy and area under the curve (AUC) than the MOLLI schemes. In the NAFLD group, there was a good correlation between the PDFF, MOLLI 3(3)3(3)5 and 3(2)3(2)5, and VFA T1 measurements (r=0.732; r=0.735; r=0.716, p < 0.001, respectively).CONCLUSIONLiver T1 mapping techniques have the potential to distinguish steatotic from nonsteatotic livers, and T1 values seem to have a strong correlation with the liver fat content.

Nonalcoholic fatty liver disease (NAFLD) is one of the most common causes of chronic liver disease, with an estimated worldwide prevalence of around 25% (1). It may range from simple steatosis, which is considered a benign condition, to nonalcoholic steatohepatitis (NASH), which can progress to fibrosis, cirrhosis, liver failure, and hepatocellular carcinoma. NAFLD is also known to be associated with metabolic syndrome, which is a risk factor for cardiovascular disease and type II diabetes mellitus (2–4).The gold standard method for diagnosing NAFLD and distinguishing its different patterns is a liver biopsy which has considerable limitations, including sampling errors, its invasive nature and associated complication risks, small sample size, and inter- and intraobserver variability (5, 6). These drawbacks constrain its utility for clinical monitoring and make it unsuitable as a screening method. Therefore, there is an urgent clinical need for an accurate noninvasive approach in the assessment of NAFLD. Accordingly, both the European Association for the Study of the Liver and the American Association for the Study of Liver Disease propose magnetic resonance imaging (MRI) as a noninvasive diagnostic tool for NAFLD (7, 8). Proton density fat fraction (PDFF)-based MRI and magnetic resonance spectroscopy (MRS) techniques are considered the most accurate noninvasive methods for the quantification of liver fat (9–12). The PDFF is accepted as a standardized biomarker of hepatic steatosis. Studies suggest that this biomarker is equivalent to the hepatic “signal fat fraction” (FF) after correcting all the confounding factors (13). However, PDFF measurement is not suitable for the assessment of any inflammation or fibrosis in NAFLD (13). On the other hand, recent studies have shown that other quantitative MRI techniques such as magnetic resonance elastography (MRE) and T1–T2 mapping can be useful in detecting hepatic inflammatory and fibrotic changes (14, 15–21). Thus, the application of a multiparametric MRI protocol might be helpful in liver tissue characterization and thereby in the risk stratification and therapeutic management of patients with NAFLD.In this prospective study, we aimed to determine liver stiffness and T1 and T2 values in patients with NAFLD and nonsteatotic subjects and compare the diagnostic performance of MRE and mapping techniques in relation to the FF.