Validation and psychometric properties of the Turkish version of Neuromuscular disease Swallowing Status Scale (NdSSS) in patients with oro-pharygo-esophageal dysphagia in neuromuscular disorders.

Objective: Dysphagia is one of the most disabling conditions arising from neuromuscular disorders(NMD). There is no specific methods to use in the evaluation of dysphagia in NMD patients. We aimed both to evaluate the applicability of the Neuromuscular Disease Swallowing Status Scale (NdSSS) for dysphagia in all phases of swallowing in various NMD patients and to investigate psychometric properties of this scale. Methods: Patients with NMD were enrolled. Functional Oral Intake Scale (FOIS), Fiberoptic Endoscopic Evaluation of Swallowing (FEES), NdSSS and High-Resolution Esophageal Manometry (HRM) were performed on all subjects within 72 hours. While the convergent and concurrent validities were used as validation method, Cohen's kappa and Cronbach's alpha coefficient were calculated for inter-rater reliability. The correlation between FOIS, PAS and HRM diagnosis according to Chicago version 3.0 (CCv3) were analyzed. Results: 115 NMD patients were included. There was good correlation between NdSSS and FOIS and PAS scores (Spearman's rank correlation coefficient (r):0.927, r:0.927 and r:-0.836, r:0.841, respectively). Also, there was a positive good correlation between NdSSS and CCv3 evaluating disorders of esophageal peristalsis (r:0.677-0.679, p=0.001). When evaluated separately, there were good correlation between NdSSS levels; and PAS (r:-0.648-0.656); and CCv3 (r:0.514-0.573) levels for ALS. For Myasthenia gravis there was a good correlation between NdSSS levels; and CCv3 (r:0.577-0.622); FOIS (r:0.508-0.521); and PAS (r:-0.504-0.519) scores. Also, for myopathy; a very good(CCv3(0.976-0.982)) and good(FOIS (0.511-0.581) and (PAS (-0.516-0.550)) correlations were defined for myopathy. Conclusion: The NdSSS was found applicable to detect both oropharyngeal and esophageal dysphagia risk in patients with NMD and is a valid and reliable swallowing screening tool that can evaluate oro-pharyngo-esophageal dysphagia in NMD patients.

Evaluation of pentraxin-3 in familial Mediterranean fever patients during attack and attack-free periods.

INTRODUCTION: Pentraxin-3 (PTX-3) is a prototype of pentraxin proteins that have been shown to be involved in acute phase response. In this study, we aimed to investigate the relationship between PTX-3 levels and familial Mediterranean fever (FMF) disease, and to evaluate PTX-3 as a novel diagnostic marker of FMF. METHOD: Forty-three male patients diagnosed with FMF and 42 healthy individuals were included in the study. Patients with other inflammatory diseases and patients who used drugs having anti-inflammatory properties were excluded from the research. Blood samples were obtained during both attack and attack-free periods. RESULTS: Patient attack periods were confirmed by combining physical examination and elevation of acute phase reactants. Acute phase reactants were significantly higher in attack versus attack-free periods (p < 0.01), however PTX-3 levels were not significantly different between the two periods. Additionally, PTX-3 levels in FMF patients were higher than in controls in both attack (917.29 ± 725.29 vs 451.83 ± 291.95, p < 0.01) and attack-free periods (748.23 ± 487.53 vs 451.83 ± 291.95, p < 0.01). CONCLUSION: In this study, we showed that PTX-3 levels, in both FMF attack and attack-free periods, were significantly higher than in the control group. Finally, PTX-3 may be a promising biomarker for FMF diagnosis and may predict FMF attacks (Tab. 2, Fig. 2, Ref. 18).

Efficiency of rifampicin in emergency treatment of severe hyperbilirubinemia : report of two cases and review of literature.

We showed in this study that rifampicin therapy is more effective than plasmapheresis and steroid treatment in diseases associated with severe hyperbilirubinemia. In our opinion, rifampicin treatment may suitable especially for patients with persistent hyperbilirubinemia, and it would be appropriate to use rifampicin as a challenge therapy to patients with severe hyperbilirubinemia, but liver function tests in these patients must be monitored closely.

The effect of FAAH, MAGL, and Dual FAAH/MAGL inhibition on inflammatory and colorectal distension-induced visceral pain models in Rodents.

BACKGROUND: Recent studies showed that the pharmacological inhibition of endocannabinoid degrading enzymes such as fatty acid amide hydrolase (FAAH) and monoacyl glycerol lipase (MAGL) elicit promising analgesic effects in a variety of nociceptive models without serious side effects. However, the full spectrum of activities is not observed upon inhibition of either FAAH or MAGL enzymes alone and thus dual FAAH and MAGL inhibitors have been described. Visceral pain is strongly associated with inflammation and distension of the gut. Thus, we explored the comparable effects of FAAH, MAGL, and dual FAAH/MAGL inhibitors on inflammatory and mechanically evoked visceral pain models. METHODS: Visceral inflammatory and distension-induced pain were assessed with the 0.6% acetic acid writhing test in mice and colorectal distension (CRD) test in rats, respectively. The selective FAAH inhibitor PF 3845, MAGL inhibitor JZL 184, dual inhibitor JZL 195, and the cannabis analog CP 55,940 were given systemically 30 min prior to nociceptive testing. KEY RESULTS: PF 3845 (5, 10, and 20 mg/kg), JZL 184 (5, 10, and 20 mg/kg), and JZL 195 (5, 10, and 20 mg/kg) elicit dose-dependent antinociceptive in the acetic acid writhing test. In the CRD model, while JZL 195 (5, 10, or 20 mg/kg) and PF3845 (10, 20, and 40 mg/kg) produced dose-dependent antinociceptive effects comparable to those of CP 55,940 (0.1, 0.3, or 1 mg/kg), JZL 184 (10, 20, and 40 mg/kg) alone did not alter the visceromotor response (VMR). CONCLUSIONS & INFERENCES: The selective FAAH inhibitor and dual FAAH/MAGL inhibitors were effective in both inflammatory and mechanically evoked visceral pain, while the MAGL inhibitor elicited an analgesic effect in inflammatory, but not in distension-induced, visceral pain.