Turnerdemant2036
t time of presentation in few patients, missing modified Rankin Scale score at discharge.
None.
None.
Consanguinity is a commonly recognized practice among marriages in the Middle East and may lead to an increase in the prevalence of inherited disorders. Autosomal recessive deafness is the most common form of inherited congenital hearing loss (CHL).
Determine the association of consanguineous marriages with congenital sensorineural hearing loss (SNHL) and auditory neuropathy.
Descriptive and analytical cross-sectional study.
Ear specialist hospital.
Children with severe-to-profound congenital SNHL, who had been referred to the specialist hospital for cochlear implant were analyzed. Patients were divided into subgroups based on degree of consanguinity.
The relative risk of having more than one child with SNHL in offspring of a consanguineous marriage.
189 parents and children with CHL.
The parents of 157 children (83.1%) were blood-related. Of those, 48 had more than one child with CHL (31.4%), while only two parents who were not blood-related had more than one child with CHL (6.25%;
=.005). Among the 189 children, 131 (69.3%) parents were direct cousins. Only 39 (20.6%) and 43 (22.8%) children had family histories of CHL on the paternal and maternal sides, respectively. There was no statistically significant difference in the prevalence of auditory neuropathy between the offspring of consanguineous and non-consanguineous marriages (
=.648).
The risk of having more than one child with SNHL in the offspring from a consanguineous marriage is 3.5 times higher than that of a non-consanguineous mating.
The association of hearing loss degree with consanguinity was not studied.
None.
None.
Cochlear implant (CI) recipients with a cochleovestibular malformation (CVM) are at a higher risk of experiencing an intra-operative cerebrospinal fluid (CSF) gusher and, therefore are at greater risk of developing postoperative meningitis than are CI recipients with normal cochlear anatomy. To control CSF gushers, the FORM electrode array was developed.
To assess the ability of the FORM24 electrode array in managing intraoperative CSF gushers and preventing postoperative CSF leakage in a population of CI recipients.
Retrospective.
Tertiary health care center.
All CIs in which a FORM24 was used between January 2014 and March 2018 were reviewed for demographic and safety results.
Safety results were assessed as the intraoperative or postoperative presence of an episode of CSF leakage or meningitis.
177 CI recipients.
Thirty-six (20.3%) had a CVM and 141 had normal anatomy (79.7%). Dolutegravir nmr Of the 36 participants with a CVM, 20 (55.6%) experienced an intraoperative CSF gusher, all of which were resolved. No cases of postoperative leakage or meningitis were recorded after a mean follow-up time of 36 months.
The FORM24 array is able to help surgeons stop intraoperative CSF gushers and prevent postoperative CSF leakage and meningitis in CI recipients with a CVM.
Further studies are needed.
None.
None.
There is limited data from Saudi Arabia on the demographic characteristics, outcomes and effectiveness of different treatment modalities in children with intracranial ependymoma.
Study the characteristics of pediatric ependymoma and outcomes of treatment modalities in Saudi Arabia.
Retrospective.
Tertiary care center.
Children with intracranial ependymoma who were younger than 14 years of age and treated between 2006 and 2015 were included in the study. Patients with prior radiation, chemo-therapy, or surgical resection at other centers were excluded.
Kaplan-Meier survival curves were used to estimate the event-free (EFS) and overall survival (OS) rates of the patients.
22.
Of the 22 children, 4 (18.2%) were less than three years old. All intracranial ependymomas had upfront surgical resection of the primary tumor. Gross total resection was achievable in 9 (42.9%) cases and subtotal resection in another 9 (42.9%). Near-total resection was done in 3 (14.3%) cases. Median time from surgery to start of radiotherapy was 62 days. RT was given to 17 (77.3%) patients. Both mean and median RT dose was 55.8 Gy. Only 5 (22.7%) of the children received chemotherapy. The median duration of follow-up was 5.38 years and the median time for EFS was 2.27 years. The cumulative OS rate of the study was 44.5%. The cumulative EFS survival rate of the study was 18.6%. Among demographic, pathological, radiological features, none had a statistically significant effect on the survival.
The outcomes are comparable to those reported by international investigators for similar populations. Further improvements can be achieved by avoiding delays in radiation therapy and adding molecular staging.
The limited number of cases, retrospective nature, lack of molecular biology and size of the tumors.
None.
None.Double-chambered right ventricles (DCRV) and left ventricles are rare entities on their own. We present two cases with an unusual combination of double-chambered right as well as left ventricles. One was discovered in a 28-year-old female, while the other was found at birth in a female child. The differing nature of both the patient demographics as well as the presentation with a common morphological background is shown on both computed tomography and magnetic resonance imaging. The oldest description of an obstructive muscular band within the right ventricle was given in 1867. In the literature, there is ample description of the long-term prognosis and management guidelines for DCRV, but no inheritance patterns or risk factors have been identified except for associations with septal defects, tetralogy of Fallot and transposition of the great arteries. A combination of both double-sided left as well as right ventricles has been published in literature a few times with little details about management and prognosis. SIMILAR CASES PUBLISHED Although many cases of DCRV and DCLV have been published, to our knowledge only 3 cases of combined DCRV and DCLV have been published in literature.
Venous thromboembolism or extensive thrombosis is relatively common in patients with severe COVID-19 infection and has been associated with increased mortality. During the current COVID-19 pandemic, several prophylactic doses and types of low-molecular-weight heparin (LMWH) are being used worldwide; however, there are no high-quality studies or recommendations for an optimal prophylactic LMWH dose.
Investigate the relationship between coagulation parameters and the LMWH dose, and mortality and ICU admission in hospitalized patients with severe COVID-19 pneumonia.
Retrospective.
Tertiary care hospital.
Data on clinical features, coagulation parameters and anticoagulant medications of inpatients with severe COVID-19 were collected for the period between 11 March 2020 and 31 April 2020.
Mortality and ICU admission for prophylactic dose LMWH (0.5 mg/kg twice daily) and therapeutic dose LMWH (1 mg/kg twice daily).
154 cases.
Ninety-eight (63.6%) patients were treated with the LMWH prophylactic dose and 56 (36.