In 1976, Gonzales-Crussi et al. (Am. J. Dis. Child. 130:1015–18, 1976) introduced the first case of bridging bronchus as a rare bronchial branching anomaly; since then, only 14 worldwide cases was described. We suggest our case might be number 15 and could be the first case of type six of this bronchial anomaly. We present a case of a 10-month-old infant with bridging bronchus, congenital tracheal stenosis, and double outlet right ventricle who underwent major laparoscopic surgery for repair of gastrointestinal anomalies to raise awareness of this rare underdiagnosed congenital anomaly and a thorough discussion of the tracheobronchial anomalies and its clinical implications.
Background
Schwartz–Jampel syndrome (SJS) is a very rare inherited disorder characterized by multiple skeletal deformities, limited joint mobility, micrognathia, blepharophimosis, myotonia, and growth retardation. SJS is caused by mutations in the gene encoding perlecan (heparan sulfate proteoglycan). Anesthetic management of these patients is challenging. The use of spinal anesthesia in these patients has not been reported.
Case presentation
A 14-year-old boy was scheduled for inguinal hernia and hydrocele repair. The diagnosis of SJS was based on his dysmorphic features, electromyographic (EMG) pattern and genetic testing. General anesthesia may encounter difficult airway management, resistance to muscle relaxants, or possibility of malignant hyperthermia. Regional anesthesia may be difficult or even harmful due to skeletal deformities. We report successful management of spinal anesthesia and surgery was done. The patient had an uneventful recovery and was discharged home. We describe the special precautions against pitfalls for using this technique in patients with SJS.
Conclusion
Spinal anesthesia may be an effective and safe technique for patients with SJS and it may
Background
The use of hydroxychloroquine in coronavirus disease (COVID-19) pandemic raised significant concerns as regards safety and efficacy in hospitalized patients. The objective was to examine the effect of hydroxychloroquine on clinical improvement and mortality among hospitalized patients with COVID-19.
Methods
A prospective cohort study was conducted at four general hospitals in the Western region, Saudi Arabia. Patients who had absolute or relative contraindication for using hydroxychloroquine were excluded. Patients concomitantly receiving other medications including azithromycin, antivirals, and supportive treatment were not excluded.
Results
A total 267 patients were included in the current analysis; 185 (69.3%) on hydroxychloroquine and 82 (30.7%) on non-hydroxychloroquine treatments. The average age was 46.0±13.3 years and 78.3% of the patients were males. Approximately 95.9% of the patients were symptomatic with mild (50.6%), moderate (32.6%), severe (8.2%), or ARDS symptoms (4.5%). Compared with no hydroxychloroquine, those on hydroxychloroquine had significantly longer length of stay (11.5±7.1 versus 7.8±4.3 days, p<0.001), more ICU admission (22.7% versus 9.8%, p=0.012), and more intubation (12.4% versus 3.7%, p=0.026). Improvement of symptoms (84.3% versus 81.7%, p=0.595) and hospitalization death (7.0% versus 1.2%, p=0.071) were not significantly different between groups. With exception of length of stay, the association of hydroxychloroquine with the above negative outcomes disappeared after adjustment for several factors including disease severity and concomitant use of azithromycin.
Conclusions
Hydroxychloroquine is not associated with better improvement of symptoms compared with other treatments. Moreover, it is associated with longer length of stay but not mortality or ICU admission in adjusted analysis.
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