Primary trabeculodysgenesis in association with neonatal Marfan syndrome

We present the clinical and ophthalmological findings in two infants with neonatal Marfan syndrome (nMFS) and primary trabeculodysgenesis (PT). Fibrillin 1 (FBN1) mutations were confirmed in both cases. Numerous eye anomalies have been recognized in infants with nMFS, but PT has not been reported previously. Our report expands the phenotype of nMFS, and highlights the importance of early and careful ophthalmological assessment of these infants. (C) 2004 Wiley-Liss, Inc.

Frequency of insomnia report in patients with obstructive sleep apnoea hypopnea syndrome (OSAHS)

Background and purpose: Insomnia and Obstructive Sleep Apnoea Hypopnea Syndrome (OSAHS) are the two most common sleep disorders, and both have significant associated health costs. Despite this, relatively little is known about the prevalence or impact of insomnia in those with OSAHS, although a recent study suggested there may be substantial comorbidity between these disorders [Chest 120 (2001) 1923-9]. The primary aim of this study was to further explore the prevalence of insomnia in OSAHS. A secondary aim was to assess the effect of factors that may impact on both conditions, including mood and sleep-beliefs. Patients and methods: Consecutive patients referred to an accredited Sleep Investigations Unit (n = 105) completed a brief standardized battery of validated questionnaires assessing sleep-related variables and mood. Results: Results showed a high rate of prevalence of clinical insomnia in this OSAHS population, and a strong positive correlation between OSAHS and insomnia symptom severity. Further, OSAHS patients with comorbid insomnia had increased levels of depression, anxiety and stress compared to patients with OSAHS-only, and both patient groups reported similar and significant levels of dysfunctional beliefs about sleep. Findings in relation to habitual sleep, assessed using subjective (diary) and objective criteria (polysomnogram), were mixed but generally showed greater sleep disturbance among those with OSAHS-insomnia compared to those with OSAHS-only. Conclusions: Overall these findings suggest that comorbidity of insomnia in OSAHS patients may lead to increased OSAHS severity and that patients with both conditions may experience more symptoms relating to depression, anxiety and stress. These findings underscore the need for insomnia assessment and management services, even in clinics that primarily service patients with OSAHS. (C) 2004 Elsevier B.V. All rights reserved.

Response of crayfish, Procambarus clarkii, haemocytes infected by white spot syndrome virus

White spot syndrome virus ( WSSV) is a serious pathogen of aquatic crustaceans. Little is known about its transmission in vivo and the immune reaction of its hosts. In this study, the circulating haemocytes of crayfish, Procambarus clarkii, infected by WSSV, and primary haemocyte cultures inoculated with WSSV, were collected and observed by transmission electron microscopy and light microscopy following in situ hybridization. In ultrathin sections of infected haemocytes, the enveloped virions were seen to be phagocytosed in the cytoplasm and no viral particles were observed in the nuclei. In situ hybridization with WSSV-specific probes also demonstrated that there were no specific positive signals present in the haemocytes. Conversely, strong specific positive signals showed that WSSV replicated in the nuclei of gill cells. As a control, the lymphoid organ of shrimp, Penaeus monodon, infected by WSSV was examined by in situ hybridization which showed that WSSV did not replicate within the tubules of the lymphoid organ. In contrast to previous studies, it is concluded that neither shrimp nor crayfish haemocytes support WSSV replication.White spot syndrome virus (WSSV) is a serious pathogen of aquatic crustaceans. Little is known about its transmission in vivo and the immune reaction of its hosts. In this study, the circulating haemocytes of crayfish, Procambarus clarkii, infected by WSSV, and primary haemocyte cultures inoculated with WSSV, were collected and observed by transmission electron microscopy and light microscopy following in situ hybridization. In ultra-thin sections of infected haemocytes, the enveloped virions were seen to be phagocytosed in the cytoplasm and no viral particles were observed in the nuclei. In situ hybridization with WSSV-specific probes also demonstrated that there were no specific positive signals present in the haemocytes. Conversely, strong specific positive signals showed that WSSV replicated in the nuclei of gill cells. As a control, the lymphoid organ of shrimp, Penaeus monodon, infected by WSSV was examined by in situ hybridization which showed that WSSV did not replicate within the tubules of the lymphoid organ. In contrast to previous studies, it is concluded that neither shrimp nor crayfish haemocytes support WSSV replication.