Novel use of fresh frozen plasma in treating hereditary angioedema: A success story from Pakistan

Hereditary angioedema (HAE) due to a C1-esterase inhibitor(C1-INH) deficiency is a rare and potentially life-threatening disorder. It is characterized by an episodic and self-limiting increase in vascular permeability. The condition manifests itself as recurrent attacks of swelling in any part of the body. The angioedema can cause the involvement of the respiratory tract, skin, and gastrointestinal tract. Laryngeal involvement can make the condition life-threatening. It does not respond well to conventional angioedema therapy of steroids, adrenaline, and antihistamines. The targeted therapy for HAE consists of plasma-derived or recombinant C1-INH, ecallantide, and icatibant or bradykinin receptor antagonist. In the absence of these therapies, it becomes difficult to manage this condition effectively. We present a case of hereditary angioedema, who presented with life-threatening laryngeal edema, causing asphyxia, leading to cardiac arrest. Due to a lack of availability of C1-INH concentrate, he was given fresh frozen plasma (FFP). His condition gradually improved, and he was successfully extubated after three days. This is the first time we are reporting a case from Pakistan in which the patient was successfully treated with FFP for an acute attack of hereditary angioedema

Predictors of mortality in hospitalized patients with influenza: A five-year experience from a tertiary care centre in Pakistan

Influenza outbreaks are associated with significant morbidity. Our aim was to determine the factors associated with increased mortality in hospitalized patients admitted with diagnosis of influenza, at a tertiary care center in Pakistan. This study included all adult patients with an influenza infection, confirmed by realtime reverse-transcriptase polymerase-chain-reaction (RT-PCR) at Aga Khan University Hospital Pakistan. In our study, 112 patients with laboratory-confirmed influenza virus infection were admittedat our hospital from the 1st of January 2013 to the 31st of December 2018. Eighty-nine patients (79.46%) were managed in ward or special care units and 23 patients (20.5%) received treatment in intensive care unit (ICU). The overall mortality in our study was 15/112 (13.4%) with the mortality rate of ICU patients being 47.8% while the mortality rate of patients treated in special care units and wards was only 4.5%. The mean age of patients with influenza infection was 58.1 years (±16.6). Influenza virus type A was found in 87 patients (77.6%), while influenza type B was present in only 25 (22.4%) patients. Out of the 15 non-survivors, 14 had influenza A. Only 17 patients (15.2%) were found to have positive culture of respiratory specimen, out of which 3 were non-survivors and 14 were survivors. Our analysis identified septic shock (odds ratio 45.24; 95%, confidence interval 6.20-330; p\u3c0.001), renal failure (odds ratio 10.88; 95%, confidence interval 1.61-73.52; p=0.01) and ICU stay (odds ratio 17.22; 95%, confidence interval 2.68-110.5; p=0.003) as independent risk factors associated with in-hospital mortality

Subcutaneous emphysema and pneumomediastinum in patients with COVID-19 disease; case series from a tertiary care hospital in Pakistan

Since December 2019, the clinical symptoms of coronavirus disease 2019 (COVID-19) and its complications are evolving. As the number of COVID patients requiring positive pressure ventilation is increasing, so is the incidence of subcutaneous emphysema (SE). We report 10 patients of COVID-19, with SE and pneumomediastinum. The mean age of the patients was 59 ± 8 years (range, 23-75). Majority of them were men (80%), and common symptoms were dyspnoea (100%), fever (80%) and cough (80%). None of them had any underlying lung disorder. All patients had acute respiratory distress syndrome on admission, with a median PaO2/FiO2 ratio of 122.5. Eight out of ten patients had spontaneous pneumomediastinum on their initial chest x-ray in the emergency department. The median duration of assisted ventilation before the development of SE was 5.5 days (interquartile range, 5-10 days). The highest positive end-expiratory pressure (PEEP) was 10 cmH2O for patients recieving invasive mechanical ventilation, while 8 cmH2O was the average PEEP in patients who had developed subcutaneous emphysema on non-invasive ventilation. All patients received corticosteroids while six also received tocilizumab, and seven received convalescent plasma therapy, respectively. Seven patients died during their hospital stay. All patients either survivor or non-survivor had prolonged hospital stay with an average of 14 days (range 8-25 days). Our findings suggest that it is lung damage secondary to inflammatory response due to COVID-19 triggered by the use of positive pressure ventilation which resulted in this complication. We conclude that the development of spontaneous pneumomediastinum and SE whenever present, is associated with poor outcome in critically ill COVID-19 ARDS patients

Epidemiology of sepsis, based on ICD-9 coding, a tertiary care experience from Pakistan

Introduction: There are limited studies on the burden of sepsis, from low to middle income countries (LMIC). We had carried out an analysis of the epidemiology of sepsis, in our hospital, a tertiary care university hospital in Pakistan, based on ICD-9 coding.Materials: Retrospective data from electronic discharge records of all patients aged 17 or above, admitted with sepsis from January 2013–January 2014, at our hospital was taken. A validated method requiring a combination of two ICD-9 codes (international classification of diseases, ninth revision, clinical modification) representing infections and acute organ dysfunction, based on the Angus and Martin methodology, along with ICD-9 codes for sepsis, severe sepsis and septic shock, were used to abstract data.Results: Overall, 8759 patients were identified to have sepsis or severe sepsis, out of total 31,111 admissions between 2013 and 14. Out of these cases, 61.25% (5365) had sepsis while 38.75% (3394) had severe sepsis or septic shock. Out of the total 8759 patients, 58.10% (5089) remained in the ward. 31.93% (2797) utilized the intermediate Care Unit while 9.97% (873) utilized the Intensive Care Unit (ICU). The overall mortality with sepsis, was found to be 9.8% and mortality from septic shock to be around 22.8%. The common comorbidities were diabetes (22.8%), renal disease (14.7%) and COPD (14.7%). The mean length of hospital stay was 3.7 days in mild disease, compared to 7.5 days, in severe sepsis and septic shock group.Conclusions: The first ICD -9 coding-based study signifies a high burden of disease, along with high mortality from septic shock