Background. The UAE reported its first cluster of COVID 2019 in a group of returned travellers from Wuhan in January 2020. Various comorbidities are associated with worse disease prognosis. Understanding the impact of ethnicity on the disease outcome is an important public health issue but data from our region is lacking. Aim. We aim to identify comorbidities among patients hospitalized for COVID-19 that are associated with inhospital death. Also, to assess if ethnicity is correlated with increased risk of death. Patients and Method. The study is a single-centre, observational study in Shaikh Shakhbout Medical City, Abu Dhabi. Patients admitted with COVID-19, between 1st of March and the end of May, were enrolled. Records were studied for demography, comorbidity, and ethnicity. Ethnicity was divided into Arabs (Gulf, North Africa, and the Levant), South Asia (India, Pakistan, Bangladesh, Nepal, and Afghanistan), Africans, the Philippines, and others. The study was approved by the Department of Health of Abu Dhabi. Results. 1075 patients (972 males) were enrolled. There were 24 nationalities under 5 ethnicity groups. Mean (average) age was 51 years (20–81). 101 (9.4%) died with deceased patients being significantly older. Death risk was not significantly influenced by sex. Duration of hospitalization among survivors was 6.2 days (0.2–40.4) with older patients and men staying longer ( P < 0.01 ). Comorbidities of diabetes, hypertension, cardiovascular disease, chronic renal disease, liver disease, and malignancy were associated with higher risk of mortality univariate, but only liver disease reached statistical significance after adjustment for age. The highest percentage of death was seen in Arab Levant (21.2) followed by the Asian Afghan (18.8); however, differences among ethnicities did not reach statistical significance ( P = 0.086 ). Conclusion. COVID-19 outcome was worse in older people and those with comorbidities. Men and older patients required longer hospitalization. Ethnicity is not seen to impact the risk of mortality.
LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency caused by a mutation in the LRBA gene. Affected individuals present with a variety of clinical symptoms including hypogammaglobulinemia, recurrent infections, splenomegaly, hepatomegaly, and autoimmune cytopenias. Except for hypogammaglobulinemia, the remaining features resemble autoimmune lymphoproliferative syndrome (ALPS). Here, we report the case of a 14-year-old boy with the ALPS phenotype, eventually diagnosed with LRBA deficiency. He presented with lymphadenopathy and hepatosplenomegaly, along with autoimmune cytopenia. Due to recurrent infections and worsening gastrointestinal symptoms, whole-exome sequencing was conducted and revealed a novel homozygous pathogenic variant in the LRBA gene (c.534del; p.9Asp179IIef*16). The patient recently suffered from clinical deterioration due to SARS-COV-2 which appears to have triggered an acute worsening of his existing Cytomegalovirus colitis leading to an eventual demise. A literature search for reported LRBA deficient patients with ALPS-like phenotype revealed 11 patients. The most common clinical presentations in LRBA patients with ALPS-like phenotype included autoimmunity (100%), splenomegaly (91%), lymphadenopathy (36.4%), and respiratory tract infections (63.6%). LRBA deficiency is unique in the fact that it encompasses immune deficiency, autoimmunity, and lymphoproliferation. In children with multiple symptoms related to these domains, a genetic diagnosis is necessary to ensure tailored and precise medical therapy.
Background. COVID-19 patients can present with neurological manifestations in the form of headache, dizziness, hyposmia, myalgia, peripheral neuropathy, acute cerebrovascular disease, and encephalopathy. Neurological involvement could be due to virus-induced brain hypoxia, brain infection, or immune reaction. We aim to describe the neurological presentation of COVID-19 patients and study their neuroimaging findings and disease outcome. Method. The study is a single-centre, retrospective, observational study in Sheikh Shakhbout Medical City (SSMC), Abu Dhabi, UAE. Patients diagnosed with COVID-19 between March and May 2020 who presented with neuropathological features with or without respiratory manifestations of COVID-19 were enrolled. Electronic records were studied for age, sex, duration of hospitalization, detailed neurological presentation, history or documented concomitant fever and respiratory features of COVID-19, inflammatory markers, neuroimaging, progress, and disease outcome. Results. Thirty-three patients of 10 nationalities presented with neurological manifestations. Mean (range) age was 51.4 (21–86) years. Twenty-four had comorbidities, and 18 had no prior or concomitant respiratory symptoms. Ten patients presented with encephalopathy and exhibited altered behavior/sensorium: 7 presented with myositis, 8 with stroke, and 4 with seizures, and 4 had peripheral and cranial nerve involvement. The mean (average) duration of hospital stay was 11.4 days (1-38) with the longest observed in stroke patients. Fifteen patients (45%) died and 3 (9%) had residual weakness. Serum ferritin, CRP, and procalcitonin were higher in the severe disease group and correlated with risk of death. Twelve of 22 brain images showed abnormalities including haemorrhage, infarcts, small vessel ischemia, and oedema. Risk of death was higher in older age but did not differ based on the underlying neuropathology. Conclusion. COVID-19 patients who present with neurological involvement have a higher risk of mortality which is aggravated by older age and higher inflammatory markers. The type of neurological pathology does not seem to influence the risk of mortality.
Adenovirus is a common cause of upper and lower respiratory tract infections. Rarely, neurological manifestations may occur, ranging from mild aseptic meningitis to potentially fatal acute necrotising encephalopathy (ANE). Very little is known in regards to the exact pathogenesis of ANE in association with adenovirus. This report describes the presentation of a previously healthy 14-month-old girl diagnosed with adenovirus-induced ANE. Herein, we highlight the clinicoradiological manifestation of this uncommon association with adenovirus in order to maintain a high index of suspicion for early diagnosis and a better outcome.
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