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Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Severe or recurrent bleeding: Epistaxis (often in childhood) is a severe recurring problem.Upper or lower GI and liver hemorrhage (in later adult years).Hematuria.
Hepatorenal tyrosinemia/fumarylacetoacetate hydrolase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
One of our patients presented with bleeding and was investigated as a problem in coagulation before chemical evidence of hepatic disease was identified. Patients may present with epistaxis or intestinal bleeding [31]. Elevated levels of PT and PTT may be found even in asymptomatic infants discovered by newborn screening. An infant presenting with liver disease and hypoglycemia may be thought to have Reye syndrome. Between acute crises the liver is enlarged.
Epistaxis
Published in S. Musheer Hussain, Paul White, Kim W Ah-See, Patrick Spielmann, Mary-Louise Montague, ENT Head & Neck Emergencies, 2018
Epistaxis is defined as bleeding from the nose and is one of the commonest emergencies dealt with by the otolaryngologist. The overall incidence of epistaxis in the general population is difficult to determine because most cases are unreported, minor, self-limiting episodes or those controlled with simple first-aid measures. Fewer than 10% of patients seek medical attention for epistaxis and fewer than 10% of those requiring hospitalisation require surgical intervention for control of bleeding.
Safety of intranasal corticosteroids for allergic rhinitis in children
Published in Expert Opinion on Drug Safety, 2022
Min Zhang, Jing-Zi Ni, Lei Cheng
Epistaxis, with a prevalence of 5–10%, is always mild and self-limiting [38]. Evidence from clinical trials has shown a slightly higher incidence of epistaxis in INCS therapy group than in placebo group, which may be explained by the dryness and thinness of the nasal mucosa with INCSs [28]. Additionally, a meta-analysis has reported that the overall relative risk of INCS-induced epistaxis is 1.48 (95% confidence interval [CI], 1.32–1.67). Beclomethasone hydrofluoroalkane (HFA), FF, MF, and FP, but not aqueous BDP and CIC, are associated with a relatively high risk of epistaxis. The associations of epistaxis with BUD and TAA have been just analyzed in small-size samples and related studies show a high heterogeneity, making it difficult to establish definite conclusions about these associations [38]. The most common INCS-related adverse effect is epistaxis, which may be severe enough to discontinue the use of INCSs [39–41]. Local adverse effects, such as nasal atrophy, septal ulcer or perforation, rarely occur [1,28].
Safety review of current systemic treatments for severe chronic rhinosinusitis with nasal polyps and future directions
Published in Expert Opinion on Drug Safety, 2021
The exhalation delivery system has been shown to improve drug delivery to the posterior and superior sinonasal spaces while reducing deposition in the non-ciliated nasal vestibule [31]. During exhalation, the soft palate elevates due to positive pressure in the oropharynx, which in turn isolates the nasal cavity from the rest of the respiratory system [32]. In theory, this should allow good nasal and sinus distribution while limiting systemic absorption. A 3-month double-blinded RCT reported fluticasone to be well tolerated when delivered through an exhalation device [8]. A 12-month single-arm study evaluating the safety and efficacy of the exhalation system with fluticasone found a number of local adverse events, including epistaxis (11.2%), nasal erythema (17.5%) and acute sinusitis (13.9%) [33]. Ocular examination, including the intraocular pressure, was all normal with no evidence of glaucoma or subcapsular cataracts [33]. A similar 12-month single-arm study suggested a similar safety profile with nasal mucosal disorders (10.2%) and epistaxis (6.8%) noted as the only adverse events [34].
Low-intensity diode laser combined with nasal glucocorticoids in the treatment of recurrent epistaxis in children: a randomized controlled trail
Published in Acta Oto-Laryngologica, 2023
Shuyue Wang, Haiyao Zheng, Tao Liao
Epistaxis is prevalent in the Ear, Nose, and Throat (ENT) clinic and emergency room. Mild epistaxis cases present with a small amount of blood nasal mucus; however, severe cases can cause hemorrhagic shock [1]. According to the location of bleeding, it can be divided into anterior bleeding and posterior bleeding. Childhood idiopathic epistaxis routinely originates from the Kiesselbach plexus (also called Little’s area) in the anterior septum. It is associated with diverse factors such as allergic inflammation, infection, dry air exposure, hemorrhagic diathesis, and local chemical irritation, etc. [2]. Among the various risk factors, AR (allergic rhinitis) is ubiquitous in pediatric epistaxis, whose pathogenesis is Type I allergic inflammation of the nasal mucosa mediated by IgE [3].