The Tübingen Palatal Plate Approach to Robin sequence: Summary of Current Evidence
We present a narrative review of our data on the Tübingen palatal plate (TPP), which show the following: a) in a randomized trial, the TPP was superior to a sham procedure in alleviating UAO; b) children treated with the TPP in infancy showed an intellectual development within the reference range; c) prone positioning is no alternative, as it is ineffective and associated with an increased risk of sudden death; d) the TPP reduces the mixed-obstructive apnea index to near-normal values, both in isolated and most (83%) syndromic RS; e) of 443 infants (129 syndromic) treated with the TPP in our center, 23 ultimately received a tracheostomy (all with syndromic RS); f) recent data suggest that the TPP may induce mandibular catch-up growth; g) the TPP may also help in reducing respiratory complications following cleft closure in RS; and h) TPP treatment is applied by various centers around the world, although it is unclear whether its effectiveness is invariably controlled by endoscopy and sleep studies. Given these data from peer-reviewed studies, it may be questioned whether the “First do no harm” principle is always adhered to when subjecting RS infants to more invasive procedures such as mandibular distraction osteogenesis or tongue-lip adhesion.
Conclusion: MTLE is a heterogeneous syndrome. Establishing the factors responsible for, and associated with, drug resistance is important for optimal management and treatment, as early identification of drug resistance should then ensure a timely referral for surgical treatment is made. This prospective study shows that sleep activation on EEG, ictal automatisms, occurrence of focal to bilateral tonic-clonic seizures, and increased number of tried AEDs are negative prognostic factors. PMID: 31513436 [PubMed - as supplied by publisher]
Abstract Obstructive sleep apnoea (OSA) is a highly prevalent disease, and there is an increased demand for OSA diagnosis and treatment. However, resources are limited compared with the growing needs for OSA diagnosis and management, and alternative strategies need to be developed to optimise the OSA clinical pathway. In this review, we propose a management strategy for OSA, and in general for sleep-disordered breathing, to be implemented from diagnosis to follow-up. For this purpose, the best current options seem to be: 1) networking at different levels of care, from primary physicians to specialised sleep labora...
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ConclusionsThe International Classification of Sleep Disorders, Third Edition criteria are adequate for a reliable diagnosis of disorders of arousal. Further validation studies, confirming DOA diagnosis with video polysomnography, are needed to investigate the predictive value of ICSD-3 criteria.
Genes, Vol. 10, Pages 713: The Global Prader–Willi Syndrome Registry: Development, Launch, and Early Demographics Genes doi: 10.3390/genes10090713 Authors: Jessica Bohonowych Jennifer Miller Shawn E. McCandless Theresa V. Strong Advances in technologies offer new opportunities to collect and integrate data from a broad range of sources to advance the understanding of rare diseases and support the development of new treatments. Prader–Willi syndrome (PWS) is a rare, complex neurodevelopmental disorder, which has a variable and incompletely understood natural history. PWS is characterized by ear...
llo Persico Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease worldwide and its incidence is definitely increasing. NAFLD is a metabolic disease with extensive multi-organ involvement, whose extra-hepatic manifestations include type 2 diabetes mellitus, cardiovascular disease, obstructive sleep apnea, chronic kidney disease, osteoporosis, and polycystic ovarian syndrome. Recently, further evidence has given attention to pathological correlations not strictly related to metabolic disease, also incorporating in this broad spectrum of systemic involvement hypothyroidism, psoriasis,...
Fibromyalgia (FM) is a chronic widespread musculoskeletal pain condition that predominantly affects women and interferes with individuals ’ daily functioning.58,80 Repeated activity interference can substantially limit one's successful adjustment to chronic pain through the development of negative self-schema and reduction of rewards that stem from engaging in meaningful and enjoyable activities.23,26 Hence, a central target of chro nic pain management is helping individuals increase their engagement in and performance of important daily activities.
ConclusionAutonomic functions should be questioned in detail as well as motor and sensory symptoms of RLS, and care should be taken especially on cardiac dysfunction.
Kirsch DB. Disruption in health care (and sleep medicine):“it’s the end of the world as we know it…and I feel fine.”.J Clin Sleep Med. 2019;15(9):1185–1188.