Pulmonary arterial hypertension in systemic sclerosis: a national inpatient analysis.

OBJECTIVES: We aim to study the prevalence and epidemiology of pulmonary arterial hypertension in SS, and the impact of PAH on SSc hospitalizations in the United States population. METHODS: We utilized the National Inpatient Sample (NIS) from 2016-2019 to obtain adult hospitalizations with the primary/secondary diagnosis of SSc and coexistent PAH (SSc-PAH). Epidemiological variables, mortality rates, and secondary outcomes were studied including pulmonary embolism, atrial flutter, atrial and ventricular fibrillation, pneumonia, sepsis, cardiac arrest and cardiac & renal failure, and ventilator requirement. Healthcare burden was estimated from total hospital charges (THC) and length of stay (LOS). Statistical analysis was performed on STATA 16.1, using linear and logistic regression analyses. RESULTS: Out of 126,685 adult systemic sclerosis hospitalizations, 16.89% had PAH (SSc-PAH). The SSc-PAH group had significantly more females (85.4 % vs. 83.8%) and higher mean age (64.85±13.29 vs. 62.56±14.51). More African Americans were in this group than in the control group (19.5% vs. 14.6, p-value<0.001) while Whites (61.3% vs. 65.6%, p<0.001) and Asians (18.0 % vs. 2.8%, p<0.001) were less common. Charlson comorbidity index was higher for the SSc-PAH population (3.42 vs. 2.94, p-value<0.001). SSc-PAH group had a higher adjusted odds ratio (aOR) for mortality (aOR: 1.39, p<0.001), increased LOS (6.64 vs. 6.0 days, p<0.001) increased THC ($83,813 vs. $71,016, p <0.001). For the SSc-PAH group, there were also significantly higher odds of cardiac failure (aOR 3.13), ventilator requirement (aOR 2.15), cardiac arrest (aOR 1.39), kidney failure (aOR 1.63), pulmonary embolism (aOR 1.84), atrial flutter (aOR 1.86) atrial fibrillation (aOR1.56) and pneumonia (aOR 1.22). No significant difference in ventricular fibrillation, sepsis, or respiratory failure was noted. CONCLUSION: Pulmonary arterial hypertension in SSc is associated with worse outcomes in terms of mortality and morbidity, and higher healthcare burden compared to SSc without PAH. Also, PAH disproportionately affects White, African American & Asian populations. There remains a pressing need to continue efforts for early diagnosis and management of PAH in SSc patients.

IgG4-related disease and isolated retroperitoneal fibrosis: A narrative review.

Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically.

Neurocysticercosis presenting with oculomotor nerve palsy: Case report and literature review.

Neurocysticercosis (NCC), a central nervous system infection caused by the cystic larvae of Taenia Solium, is endemic in many low-to-middle income countries. NCC is known to have a variety of presentations depending on the size and site of involvement including chronic headaches, seizures, hydrocephalus, and ischemic insults. NCC has also been rarely associated with cranial nerve palsies. We report the case of a 26-year-old Nepalese lady who presented with isolated left-sided oculomotor nerve palsy and was found to have midbrain NCC. She was treated with anthelminthic agents and corticosteroids which led to clinical improvement. NCC can present with a variety of focal neurological syndromes. To the best of our knowledge, this is the first case report of NCC presenting with third cranial nerve palsy in the state of Qatar and the middle east. We also review the literature for other cases of NCC which presented with isolated oculomotor nerve palsy.

HIV and AIDS-defining opportunistic illnesses in the state of Qatar: A cohort population-based retrospective study covering 17 years (2000-2016).

Background and introduction: Human immune deficiency virus (HIV) infection remains a major health problem since discovery of the virus in 1981. Globally, since introduction of antiretroviral therapy, AIDS-related mortality was reduced by 47% since 2010. Also, HIV-related opportunistic infections (OIs) became less common, especially with use of prophylaxis to prevent such infections. In this study, we aim to assess the incidence of HIV infection and related OIs in Qatar for 17-year period, and to assess the spectrum of these infections, risk factors and treatment outcomes. Methods: This is a retrospective cohort study for all HIV infected patients registered in the state of Qatar from 2000 to 2016. Incidence of HIV infection and related opportunistic illness was calculated per 100,000 population. Demographic and Clinical characteristic were compared between two groups of patients with and without opportunistic illness. Results: of 167 cases with HIV infection 54 (32.3%) had opportunistic illness. The average incidence rate of HIV infection over 17 years is 0.69 per 100,000 population, and the incidence rate for opportunistic illness is 0.27 per 100,000 population. The most common opportunistic illness is pneumocystis jirovecii pneumonia (PCP) which constituted 25% of cases, followed by cytomegalovirus (CMV) retinitis 7.2%, Tuberculosis 5.4%, Toxoplasmosis 4.2% and, less than 2% for each of Kaposi sarcoma, lymphoma and cryptococcal infection.The outcome of treatment of cases with opportunistic illness showed cure rates of 59.3%, one year relapse rates of 8.76% and overall, 90-day mortality of 3.7% however, 33.4% of patients left the country before completion of therapy.Most of our patients in both groups were of young age, majority males, and almost half of them were Qatari. The CD4 count, CD4%, CD4/CD8 ratio and viral load were statistically significant risk factors in cases with opportunistic illness with a p value < 0.05, however presence of comorbidities was lower in patients with opportunistic illness P value of 0.032. Conclusion: Qatar has a low prevalence rate for HIV infection and related opportunistic illness. Early diagnosis and use of antiretroviral therapy are important measures to decrease the rate of opportunistic illness.

A 5-year retrospective study of Actinomyces odontolyticus bacteremia in the state of Qatar, case series.

Introduction and importance: Manifestations of infection by A. odontolyticus include thoracic, abdominal, pelvic, and central nervous system disease. In the four decades following its isolation, more than 20 cases of invasive infections were reported in multiple geographic locations including the United States and Europe. As such, A. odontolyticus is an emerging bacterium and related research is encouraged for further characterization of its prevalence and clinical significance. Our case series represents the first case series about A. odontolyticus bacteremia in the state of Qatar. Methods: We are reporting 15 cases with isolated A. odontolyticus positive blood cultures at Hamad Medical Corporation, State of Qatar from 1/Jan/2016 to 1/Nov/2020. Electronic health records (EHR) of patients who were identified to have positive blood cultures were accessed and the demographics and other clinically related data were collected and mentioned in this manuscript, after obtaining the appropriate approval from the Corporation Medical Research Council (MRC). Outcomes: We are reporting 15 cases with isolated A. odontolyticus positive blood cultures at Hamad Medical Corporation, State of Qatar from 1/Jan/2016 to 1/Nov/2020. Conclusion: 12 of the 15 reported cases were considered significant and received a complete course of antimicrobial therapy. The patients presented with a wide variety of clinical pictures and were of variable age.

Sodium-glucose co-transporter 2 inhibitors induced euglycemic diabetic ketoacidosis within four days of initiation.

Euglycemic diabetic ketoacidosis (EDKA) is a well-known complication of sodium-glucose co-transporter 2 inhibitors, and many cases with variable onset following the initiation of these agents are reported before, with a median onset of approximately 2 wk. This letter discusses a 45-year-old lady who initially presented with ischemic stroke but developed EDKA 4 d after starting empagliflozin, a rare occurrence. The patient had severe metabolic acidosis that necessitated admission into the intensive care unit. Prompt discontinuation of empagliflozin and DKA management resulted in clinical recovery.

Post-COVID-19 vaccine medium-vessel vasculitis and acute anterior uveitis, causation vs temporal relation; case report and literature review.

INTRODUCTION: and importance: Multiple immunologic phenomena were reported following the administration of COVID-19 vaccines. However, the important point is that their possible association with medium-vessel vasculitis involving the celiac trunk and its branches with acute anterior uveitis in the same patient has not been reported before. CASE PRESENTATION: In this manuscript, we are reporting a case of a middle-aged gentleman who developed vasculitis involving the celiac trunk and its branches, and acute anterior uveitis one week and three weeks after the second dose of Pfizer BioNTech COVID-19 vaccine, respectively. The patient showed significant clinical and radiographic improvement after receiving corticosteroids and azathioprine. CLINICAL DISCUSSION: Previously reported cases of vasculitis following COVID-19 vaccines included both renal-limited and more generalized vasculitis with some being positive and others negative for ANCA (anti-neutrophil cytoplasmic antibodies). Nevertheless, it is worth mentioning that most cases responded to immunosuppressive treatment. Post-COVID-19 vaccine uveitis was reported in patients with different age spans including both anterior and posterior uveitis, with remission being achieved after the use of corticosteroids. CONCLUSIONS: Multiple cases of vasculitis and acute anterior uveitis were reported following COVID-19 vaccines; however, it is important to mention that more research is needed to establish an association between the COVID-19 vaccine and both vasculitis and acute anterior uveitis. In our opinion, the benefits of the COIVID-19 vaccine largely outweigh the expected risks.

Actinomyces odontolyticus bacteraemia associated with cervical and mediastinal abscesses in an immunocompetent patient: First reported case in Qatar.

The Actinomyces bacteria are associated with cervicothoracic disease in immunocompromised patients; however, Actinomyces odontolyticus cervical infection with extensive spread to the mediastinum in a previously healthy patient was not reported before in Qatar. The patient underwent drainage of collections in synchrony with intravenous antibiotics and recovered with an excellent outcome.

Salmonella-induced pulmonary and pericardial abscesses in a patient presenting with subacute cough.

The non-typhoid Salmonella (NTS) species are commonly associated with gastroenteritis and other forms of intestinal disease. Thoraco-pulmonary infections are less commonly reported. We describe the case of a 66-year-old Qatari lady who presented with subacute cough. Chest imaging revealed multiple pulmonary and a pericardial cavitary lesion with air fluid levels. Bronchoalveolar lavage culture grew Salmonella species group D. The patient was treated with 4 weeks of appropriate antibiotics. Clinical and radiological improvement were documented on subsequent follow up. To our knowledge, this is the first reported case of pulmonary and pericardial salmonella abscesses in the state of Qatar.

Prognostic tools and candidate drugs based on plasma proteomics of patients with severe COVID-19 complications.

COVID-19 complications still present a huge burden on healthcare systems and warrant predictive risk models to triage patients and inform early intervention. Here, we profile 893 plasma proteins from 50 severe and 50 mild-moderate COVID-19 patients, and 50 healthy controls, and show that 375 proteins are differentially expressed in the plasma of severe COVID-19 patients. These differentially expressed plasma proteins are implicated in the pathogenesis of COVID-19 and present targets for candidate drugs to prevent or treat severe complications. Based on the plasma proteomics and clinical lab tests, we also report a 12-plasma protein signature and a model of seven routine clinical tests that validate in an independent cohort as early risk predictors of COVID-19 severity and patient survival. The risk predictors and candidate drugs described in our study can be used and developed for personalized management of SARS-CoV-2 infected patients.

Leukocyte chemotactic factor 2-related amyloidosis presenting with severe jaundice and hepatic encephalopathy.

Amyloidosis is a well-known disease with various types and subtypes. One of the most recently identified types is leukocyte chemotactic factor 2 amyloidosis (LECT 2), which was found to be common in certain ethnic backgrounds. It is suggested that the diagnosis of this type is vital to prevent any therapy-related complications when it is erroneously diagnosed as AL amyloidosis. The clinical presentation is usually slowly progressive kidney disease and mild hepatic impairment. We report a case of LECT2 amyloidosis, which presented with severe painless cholestasis and hepatic encephalopathy alongside progressive kidney disease.

Post-COVID-19 vaccine Guillain-Barré syndrome; first reported case from Qatar.

INTRODUCTION: Guillain-Barré syndrome (GBS) is an immune-mediated peripheral neuropathy that was reported following meningococcus, polio, influenza and rabies vaccines. However, an association with the COVID-19 vaccine is yet to be established. PRESENTATION OF CASE: We present the case of an elderly gentleman with no history of SARS-CoV-2 infection or any recent viral or bacterial illnesses who presented with GBS 20 days after the second dose of COVID-19 vaccination. The diagnosis was established based on physical examination, magnetic resonance imaging (MRI) of the spine, cerebrospinal fluid (CSF) analysis and electromyography (EMG). DISCUSSION: Due to the occurrence of GBS after certain types of infections, molecular mimicry has become widely acceptable as the underlying pathophysiology. The reported cases of GBS following vaccination further supported this theory, however proving a causal relationship between vaccines and GBS on the molecular level remains a challenge. CONCLUSIONS: To the best of our knowledge this is the first reported case in the state of Qatar. It is important to mention that more research is needed to establish an association between COVID-19 vaccine and GBS. In our opinion, the benefits of COIVID-19 vaccine largely outweigh its risks.

SGLT-2 inhibitors associated euglycemic and hyperglycemic DKA in a multicentric cohort.

Euglycemic diabetic ketoacidosis (EuDKA) secondary to Sodium-glucose co-transporter-2 inhibitors (SGLT2i) in type 2 diabetes mellitus (T2D) is a rare but increasingly reported phenomenon. Not much is known about the burden of EuDKA in patients on SGLT2i or the associated factors. This retrospective cohort study tries to delineate the differences in factors associated with the development of EuDKA as compared to hyperglycemic DKA. We conducted a multicentre, retrospective study across three tertiary care centers under Weill Cornell affiliated-Hamad Medical Corporation, Qatar. The cohort comprised of T2D patients on SGLT2i who developed DKA between January 2015 to December 2020. The differences between the subjects who developed EuDKA or hyperglycaemic DKA (hDKA) were analyzed. A total of 9940 T2D patients were on SGLT2i during 2015-2020, out of which 43 developed DKA (0.43%). 25 developed EuKDA, whereas 18 had hDKA. The point prevalence of EuDKA in our cohort was 58.1%. EuDKA was most common in patients using canagliflozin, followed by empagliflozin and Dapagliflozin (100%, 77%, and 48.3%, respectively). Overall, infection (32.6%) was the most common trigger for DKA, followed by insulin non-compliance (13.7%). Infection was the only risk factor with a significant point estimate between the two groups, being more common in hDKA patients (p-value 0.006, RR 2.53, 95% CI 1.07-5.98). Canagliflozin had the strongest association with the development of EuDKA and was associated with the highest medical intensive care unit (MICU) admission rates (66.6%). In T2D patients on SGLT2i, infection is probably associated with an increased risk of developing EuDKA. The differential role of individual SGLT2i analogs is less clear and will need exploration by more extensive prospective studies.

IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report.

INTRODUCTION AND IMPORTANCE: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and inflammation. We present the case of a 40-years-old gentleman with RPF whose presenting complaints were bilateral flank pain and weight loss and was found to have IgG4 related disease. To the best of our knowledge, IgG4-related disease with isolated retroperitoneal involvement is a very rare occurrence. CASE PRESENTATION: The diagnosis of IgG4-Related retroperitoneal fibrosis was made based on clinical, radiological and histopathological criteria. Imaging revealed isolated retroperitoneal involvement and the patient was started on oral steroids with a good clinical response after ten days. Repeated imaging months later showed significant regression in the fibrosis. CLINICAL DISCUSSION: RPF can occur due to many etiologies and is categorized to idiopathic and secondary. Factors associated with secondary RPF include medications, autoimmune disease, malignancy and IgG4 related disease. Almost all the reported cases of IgG4 related disease had evidence of multifocal involvement such as the pancreas, aorta and kidneys. Our patient was diagnosed with isolated RPF due to IgG4 disease. He met all the suggested diagnostic criteria, was started on oral steroids and had an excellent clinical outcome. CONCLUSION: IgG4 related disease can present with isolated retroperitoneal fibrosis without involvement of other organ systems. The diagnosis should be based on specific criteria. Treatment with corticosteroids can lead to remission both clinically and radiographically.

Primary mediastinal hydatidosis successfully treated with combined medical therapy and VATS (Video-assisted thoracoscopic surgery).

Mediastinal involvement of hydatidosis is rare even in endemic areas. Isolated mediastinal without lung or liver involvement is even less commonly reported. We present the case of a young gentleman who was diagnosed with primary mediastinal hydatidosis based on clinical, radiological and pathological criteria. He underwent successful resection of the lesion by VATS (Video-assisted thoracoscopic surgery) preceded by two weeks of medical treatment with albendazole and had an excellent outcome. To the best of our knowledge, this is the first reported case in the state of Qatar.

Cerastes cerastes snakebite complicated by coagulopathy and cardiotoxicity with electrocardiographic changes.

Snakebites occur worldwide with varying frequency. Outcomes associated with snakebite are varied and depend on the type of snake and the general health of the patient. We report a case of snakebite by Cerastes cerastes or desert horned viper. Consumptive coagulopathy and cardiotoxicity with electrocardiographic changes complicated the clinical course of the patient. To the best of our knowledge, this is the first reported case in the state of Qatar.

Hypotension: an unusual presentation of vitamin B12 deficiency, with complete recovery following cyanocobalamin therapy.

Vitamin B12 deficiency and its sequelae are well described and reported, especially in vegetarians. However, its association with haemodynamic instability is not well identified. We report a case of a young man, previously healthy, presenting with fever, hypotension requiring vasopressors and pancytopenia. Extensive workup was unrevealing for possible infective, inflammatory or endocrine causes except for vitamin B12 deficiency. Fever and haematological parameters stabilised after adequate supplementation of cyanocobalamin (vitamin B12).