Challenges in the management of idiopathic pulmonary fibrosis from a low- and middle-income country.

Idiopathic pulmonary fibrosis (IPF) is the most common progressive form of interstitial lung disease (ILD) that leads to gradual deterioration of lung function and ultimately death. Data from low- and middle-income countries (LMIC) on IPF is scarce. In this communication, we report the challenges encountered in managing IPF from Pakistan's largest tertiary care centre. A total of 108 patients with IPF were evaluated at the Aga Khan University Hospital in Karachi, Pakistan from January 2017 to March 2020. A significant concern was that most patients with IPF presented late during their disease. A bigger challenge encountered in clinical practice was the cost and nonavailability of antifibrotic therapy in the country until mid-2020. Successfully addressing these limitations, it is anticipated that better care will be available for the patients suffering from IPF in this part of the world.

Risk Factors Of Idiopathic Pulmonary Fibrosis In Pakistani Population: A Matched Case-Control Studyy.

OBJECTIVE: To evaluate the factors associated with idiopathic pulmonary fibrosisrisk. Methods: The case-controlstudywas conductedfromJanuary 5, 2017,toSeptember 4, 2018, attheprivate-sectorAga Khan University Hospital and the public-sector Jinnah Postgraduate Medical Centre, two large tertiary care centres in Karachi, andcomprisedadultpatientsof eithergenderwithdiagnosedidiopathicpulmonary fibrosis, asdefinedby the IndianChest Registry. Subjects without idiopathic pulmonary fibrosis but registered with the department of pulmonology of the two hospitalswere enrolledas controls.Datawas collectedusinga structuredquestionnaire, andanthropometricmeasurements were noted for each subject. Gastroesophageal reflux disease was assessed using GerdQ. This wasfollowed by serological evaluations and spirometry. Data was analysed using SPSS 21. RESULTS: Of the 459 subjects, 154(33.6%)were cases and305(66.4%)were controls.Amongthe cases, 81(52.6%)were females and 73(47.4%) were males with mean age 66.1±10.9 years. Among the controls, 162(53.1%) were females and 143(46.9%) were males with mean age 64.6±11.1 years(p>0.05.)The most common ethnicity wasUrdu-speaking; 89(58%) among the cases and 150(49%) among the controls (p<0.05). Ethnicity, number of persons in the household per room, and type of house were significantly associated with the risk of developing idiopathic pulmonary fibrosis(p<0.05). CONCLUSIONS: Ethnicity,type of house and the number of personsin a household perroom were found to be the significant risk factorsfor idiopathic pulmonary fibrosisIPF.

An Easy-To-Use Prehospital Severity Scoring Tool To Triage COVID-19 Positive Adults In A Resource-Limited Setting.

Objectives: To develop an easy-to-use severity scoring tool for prehospital triage of patients infected by the coronavirus disease-2019 in resource-limited settings. METHODS: The cohort study was conducted at a tertiary care hospital in Karachi, from August to September 2020, and comprised adult patients of either gender who tested positive for coronavirus disease-2019 on real-time polymerase chain reaction. The scoring system and categorisation were based on validated scales for the detection of pneumonia and opinions from pulmonologists. Data was analysed using SPSS 19. RESULTS: Of the 206 participants, 100(48.5%) were in-patients and 106(51.5%) were out-patients. There were 144(69.9%) males and 62(30.1%) females with an overall mean age of 48.4±16.2 years. After categorisation based on severity, significantly higher number of in-patients were found to be in categories III and IV (p<0.05). CONCLUSIONS: The severity scoring tool could effectively help classify coronavirus disease-2019 patients into mild, moderate and severe cases.

Sarcoidosis: An experience from a tertiary care center in Karachi Pakistan.

OBJECTIVE: To assess the demographic and clinical characteristics and treatment of patients with sarcoidosis in tertiary care settings. METHODS: The descriptive retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data from 2017 to 2019 of patients with established diagnosis of sarcoidosis. Data was collected on a predesigned proforma and it was analysed using SPSS 23. RESULTS: Of the 974 patients evaluated, sarcoidosis was established in 108(11.1%); 58(53.7%) of them being females. The overall mean age was 50.9±12.9 years. Hypertension 49(45.4%) and diabetes mellitus 37(34.3%) were the most frequent co-morbidities. Extra-pulmonary manifestations were found in 27(25%) patients; ophthalmic 9(33.3%), musculoskeletal 8(29.6%) and skin 7(25.9%). Prednisolone was the mainstay of treatment in 93(86.1%) patients. CONCLUSIONS: Sarcoidosis was not found to be rare in Pakistan.

Comparative analysis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis from a tertiary care centre in Pakistan.

OBJECTIVE: To compare the characteristics of connective tissue disease-associated interstitial lung disease with idiopathic pulmonary fibrosis at a tertiary care hospital. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised demographical, clinical and radiological data of patients with interstitial lung disease between October 2016 and October 2017 accessed through the outpatient data registry. Data was compared in terms of characteristics and key features of patients with connective tissue disease-associated interstitial lung disease with those of idiopathic pulmonary fibrosis. Statistical analysis was done using STATA 12. RESULTS: Of the 184 patients, 52(29.3%) had connective tissue disease-associated interstitial lung disease and 62(35%) had idiopathic pulmonary fibrosis. The most prevalent conditions among connective tissue disease-associated interstitial lung disease patients were rheumatoid arthritis 22(42.3%) and scleroderma 13(25%). Compared to patients with idiopathic pulmonary fibrosis, those with connective tissue disease-associated interstitial lung disease were predominantly younger (p<0.001) and female (p<0.001). History of gastroesophageal reflux disease was also significantly lower in connective tissue disease-associated interstitial lung disease (p=0.05). CONCLUSIONS: Connective tissue disease-associated interstitial lung disease patients were found to be younger and predominantly female compared to patients of idiopathic pulmonary fibrosis.

Knowledge about asthma: A cross-sectional survey in 4 major hospitals of Karachi, Pakistan.

OBJECTIVE: To determine knowledge and misconceptions about asthma among the local population.. METHODS: This cross-sectional study was conducted at four tertiary care hospitals; Aga Khan University Hospital, Civil Hospital Karachi, Jinnah Postgraduate Medical Centre and Ojha Institute of Chest Diseases, Karachi, from October to November 2016, and comprised hospital attendants. The questionnaire used in the study comprised 26 questions answered with a true, false or not sure answer.SPSS 20 was used for data analysis. RESULTS: There were 400 participants. The overall mean age was 41.2±14.2 years, and 214(53.5%) of the participants were males. Moreover, 75(19%) participants thought that asthma was a psychological disorder while 181(45%) considered it an infectious disease. Nearly 174(43.5%) believed that inhaled medications had significant side effects. Besides, 264(66%) participants considered steam inhalation to be an effective treatment for asthma, 269(67%) thought that patients with asthma should avoid rice in their diet and 167(42%) considered milk as a common trigger. CONCLUSIONS: Participants' knowledge about asthma was poor and misconceptions were common about the condition.

Spectrum of interstitial lung disease from a tertiary care hospital in Karachi.

OBJECTIVE: To determine the clinical features and patterns of interstitial lung disease. METHODS: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised record of patients diagnosed with interstitial lung disease from January 2005 to December 2015. All patients aged 16 years and above diagnosed with interstitial lung disease on the basis of clinical features, radiological features on high-resolution computed tomography of the chest, and lung biopsies were included. SPSS 19 was used for data analysis. RESULTS: Of the 537 patients, 324(60.3%) of the participants were females. The overall mean age was 60.5±14.9 years. The most common co-morbid condition was diabetes mellitus in 72(13.4%) patients, followed by hypertension in 48(8.9%) and ischaemic heart disease in 21(3.9%). The most common interstitial lung disease was idiopathic pulmonary fibrosis in 217(40.4%) patients, followed by non-specific interstitial pneumonia in 106(19.7%), sarcoidosis in 82(15.3%) and connective tissue disease-related interstitial lung disease in 56(10.4%) patients. CONCLUSIONS: Idiopathic pulmonary fibrosis was found to be the most common interstitial lung disease subtype followed by non-specific interstitial pneumonia, sarcoidosis and connective tissue disease-related-interstitial lung disease.

Case series of rare Interstitial Lung Disease (ILD).

Pulmonary alveolar proteinosis (PAP) is a rare lung disease where periodic acid Schiff (PAS)-positive eosinophilic material accumulates in the alveoli of the lungs. Here we describe two cases of young males who presented with dynpnoea and weight loss. The HRCT scan of the chest in both cases showed the typical "crazy-paving" pattern and lung biopsies confirmed the diagnosis of PAP. They showed remarkable symptomatic improvement with therapeutic whole lung lavage.

Clinical outcomes of pneumocystis pneumonia from a tertiary care centre in Pakistan.

OBJECTIVE: To assess the predisposing immunocompromised states, administration of pneumocystis jirovecii pneumonia prophylaxis, the disease course and outcomes of patients with pneumocystis jirovecii pneumonia. METHODS: The retrospective study was conducted at the Aga Khan University Hospital in Karachi. The medical records of patients diagnosed with pneumocystis jirovecii pneumonia from January 1995 to October 2015 were retrieved. Baseline characteristics, clinical course, treatment, and mortality rates were noted. SPSS 19 was used for data analysis. RESULTS: Of the 37 patients, 24(64.9%) were men and 13(35.1%) were women. The overall mean presenting age was 47.08±16.21 years (range: 19-83 years). Ten (27%) patients were positive for human immunodeficiency virus; 12(32.4%) had an underlying autoimmune disease; 3(8.1%) were transplant recipients; 10(27%) had an underlying malignancy, and 19(51.3%) were on long-term corticosteroid therapy. Only 2(5.4%) patients had received pneumocystis jirovecii pneumonia prophylaxis with trimethoprim-sulfamethoxazole. Moreover, 8(21.6%) patients required intensive care unit admission with a mean stay of 2.03±4.91 days (range: 1-22 days).The overall mortality rate was 7(18.9%). CONCLUSIONS: Pneumonia due to pneumocystis jirovecii was found to be a life-threatening disease in the immunocompromised population. The high mortality burden and resource intensive management of the disease emphasizes the need for PCP prophylaxis in immunosuppressed individuals.

Nutrition in intensive care in adults review of the literature and development of evidence based feeding protocols.

The subject of nutrition in intensive care is broad.Thenutrition support therapy plays a crucial role in the management of critically ill patients. This review was carried out to address the existing controversies and to recognise the current practice guidelines for the management of nutrition in intensive care units (ICUs) in adults. A PubMed search was carried out for clinical trials addressing the current nutrition practice in ICUs, recommendations for calculating energy requirements and efficacy of an algorithmic approach to nutritional delivery in an ICU setting. Algorithms were developed and modified for the practice of nutrition in an adult ICU in a quaternary care hospital in Pakistan.

Tumor necrosis factor -α, interleukin-10, intercellular and vascular adhesion molecules are possible biomarkers of disease severity in complicated Plasmodium vivax isolates from Pakistan.

BACKGROUND: Cytokine-mediated endothelial activation pathway is a known mechanism of pathogenesis employed by Plasmodium falciparum to induce severe disease symptoms in human host. Though considered benign, complicated cases of Plasmodium vivax are being reported worldwide and from Pakistan. It has been hypothesized that P.vivax utilizes similar mechanism of pathogenesis, as that of P.falciparum for manifestations of severe malaria. Therefore, the main objective of this study was to characterize the role of cytokines and endothelial activation markers in complicated Plasmodium vivax isolates from Pakistan. METHODS AND PRINCIPLE FINDINGS: A case control study using plasma samples from well-characterized groups suffering from P.vivax infection including uncomplicated cases (n=100), complicated cases (n=82) and healthy controls (n=100) were investigated. Base line levels of Tumor necrosis factor-α (TNF-α), Interleukin-6 (IL-6), Interleukin-10 (IL-10), Intercellular adhesion molecule-1 (ICAM-1), Vascular adhesion molecule-1(VCAM-1) and E-selectin were measured by ELISA. Correlation of cytokines and endothelial activation markers was done using Spearman's correlation analysis. Furthermore, significance of these biomarkers as indicators of disease severity was also analyzed. The results showed that TNF-α, IL-10, ICAM-1and VCAM-1 were 3-fold, 3.7 fold and 2 fold increased between uncomplicated and complicated cases. Comparison of healthy controls with uncomplicated cases showed no significant difference in TNF-α concentrations while IL-6, IL-10, ICAM-1, VCAM-1 and E-selectin were found to be elevated respectively. In addition, significant positive correlation was observed between TNF-α and IL-10/ ICAM-1, IL-6 and IL-10, ICAM-1 and VCAM-1.A Receiver operating curve (ROC) was generated which showed that TNF-α, IL-10, ICAM-1 and VCAM-1 were the best individual predictors of complicated P.vivax malaria. CONCLUSION: The results suggest that though endothelial adhesion molecules are inducible by pro-inflammatory cytokine TNF-α, however, cytokine-mediated endothelial activation pathway is not clearly demonstrated as a mechanism of pathogenesis in complicated P.vivax malaria cases from Pakistan.

Six-minute walk test performance in healthy adult Pakistani volunteers.

OBJECTIVE: To determine the six-minute walking distance (6MWD) for healthy Pakistanis, identify factors affecting 6MWD, compare published equations with the local data and derive an equation. STUDY DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: Two medical institutes of Karachi, from January to May 2011. METHODOLOGY: Subjects between 15 and 65 years were prospectively enrolled after screening. A standardized 6MWT was administered. SpO2, HR, BP and dyspnoea scores were determined pre- and post-test. RESULTS: Two hundred and eleven (71%) men and 85 (29%) women participated. Mean 6MWD was 469.88 ± 101.24 m: men walked 502.35 ± 92.21 m and women walked 389.28 ± 74.29 m. On univariate analysis, gender, height, weight and age showed a significant relationship with the 6MWD. Gender and age were identified as independent factors in multiple regression analysis, and together explained 33% of the variance. The gender-specific prediction equations were: 6MWD (m) for men = 164.08 + (78.06*1) - (1.90*age in years) + (1.95*height in cms) 6MWD (m) for women = 164.08 - (1.90*age in years) + (1.95*height in cms). CONCLUSION: 6MWDs among the volunteer subjects were shorter than predicted by reference equations in literature. Height, gender and weight combined explained 33% of the variance. The moderate over-estimation of the 6MWD in Pakistani subject. The proposed equation gives predicted (mean) 6MWDs for adult Pakistani naïve to the test when employing standardized protocol.