Pediatric obstructive sleep apnea (OSA) is a broad-spectrum disorder that needs timely diagnosis and management.It is mainly caused by anatomical factors.Adenotonsillar hypertrophy is an important correctable cause.We describe a narrative review of this disease in children.Although uncommon in adults, it is an important disorder causing mortality and morbidity in children which warrants timely diagnosis and management.

Background: Pulmonary hypertension (PH) is a disorder of the pulmonary vasculature caused due to vasoconstriction, vaso-destruction, or vaso-obliteration as a complication of a varied spectrum of diseases referred to a pulmonologist. We conducted this study to contemplate the profile of PH in a tertiary care hospital in Mumbai. Methodology: This was an observational study undertaken in the department of pulmonary medicine at a tertiary care center after internal ethical committee approval. Patients with respiratory symptoms referred to us with PH as estimated pulmonary artery systolic pressure (PASP) of ≥40 mmHg by transthoracic two-dimensional echocardiography (corresponding to mean pulmonary artery pressure of ≥25 mmHg) were included in the study. The profile of these patients was studied on basis of parameters such as demography, etiology, symptomatology, radiological features, 6-min walk distance (6-MWD), and spirometry. Data were analyzed using Microsoft Excel software. Results: Among 347 patients, 54% were men. Majority of the patients (53.5%) were aged between 45 and 65 years. The most common symptom was dyspnea (86%). The most common examination finding was loud pulmonary component of second heart sound (62%). The common etiologies of PH were postinfectious obliterative bronchiolitis (OB) 30%, interstitial lung diseases 26%, chronic obstructive pulmonary disease 24%, and obstructive sleep apnea 14%. There was a negative correlation between 6-MWD, forced vital capacity % predicted, forced expiratory volume in 1st s % predicted, and PASP. Conclusion: Airway disease is the most common etiology of PH in patients presenting to a pulmonologist in India, out of which postinfectious OB forms the major bulk.

Introduction: Thoracocentesis and pleural biopsy are recommended for the evaluation of undiagnosed exudative pleural effusion.There are multiple etiologies associated with them, out of which malignancy is one of them.Hence, the diagnosis of malignant pleural effusion (MPE) has been proposed in recent perspectives.We aimed to find the profile of MPE, efficacy of percutaneous closed needle pleural biopsy (PCNPB) in diagnosing MPE, overall yield, and complication rate to evaluate the continued relevance of this traditional procedure.Methods: This was a prospective study carried out on consecutive consenting patients at the Department of Pulmonary Medicine at a tertiary care hospital from July 2016 to May 2018.The diagnosis was based on cytobiochemical, microbiological, and histopathological results along with clinical history.Data were analyzed with respect to pleural fluid assessment in terms of cytobiochemical and microbiological evaluation; while pleural biopsy was studied histopathologically.Results: Two hundred and fifty patients with exudative pleural effusion were enrolled.Tuberculosis (218, 87.2%) was the most common etiology followed by malignancy (22, 8.8%).The most common presenting complaint was chest pain (100%) followed by dyspnea (90.47%).Metastatic adenocarcinoma was found in 81.81% followed by mesothelioma in 18.18%.The sensitivity of pleural biopsy for malignancy was found to be 63.63% (p < 0.003, odds ratio [OR]: 2.01), and those fulfilling Leung's criteria, sensitivity was found to be 90.90%(p < 0.001, OR: 3.67).The sensitivity of pleural fluid for malignancy was 18.18% (p < 0.05, OR: 1.51).All cases of mesothelioma have asbestos exposure.The complication in the form of mild post-pleural biopsy pain was encountered in 10%, which required mild analgesics.Other complications in the form of selfresolving pneumothorax were seen in 6%, which increased hospital stay to 2-3 days and self-resolving hematoma (3%). Conclusion:In this modern era, PCNPB still holds high sensitivity, efficacy rate, and relevance for diagnosing MPE with less complication rate, less hospital stay, and can be done on a daycare basis.Also, we have very less research and paperwork regarding this topic.

Connective tissue disorders (CTDs) are one of the common causes of interstitial lung diseases (ILDs).This prospective observational study included around 51 patients of CTD-ILDs, and their demographic, clinical, radiological, and laboratory profiles were studied.The most common type of CTD-ILD in our study is rheumatoid arthritis-related ILD.On high-resolution computed tomography (HRCT) thorax, nonspecific interstitial pneumonia (NSIP) was the most common pattern seen in 30 patients (59%), followed by usual interstitial pneumonia (UIP) seen in 20 patients (39%).Even though CTD-ILDs are similar to other idiopathic ILDs in clinical and radiological presentation, patients with CTDs have to be evaluated clinically and radiologically for early diagnosis.Early treatment initiation and pulmonary rehabilitation help in delaying the progression of disease.Among all ILDs, CTD-ILDs are associated with better prognosis and survival.

Chromosomal breakage syndromes are a group of genetic disorders that are ascribable to the autosomal recessive mode of inheritance. Xeroderma pigmentosum is one of the chromosomal breakage syndromes which is due to the involvement of deformity in the deoxyribonucleic acid's nucleotide excision repair. Xeroderma pigmentosum is a genetic disorder, which is autosomal recessive, heterogeneous, and more common in cases of consanguinity, caused by mutations in at least 10 genes and 9 complementation groups. The disorder is very rare. Patients experience photophobia and extreme photosensitivity and have pigmentary changes in ultraviolet light-exposed areas of the body with freckling, premalignant, and malignant skin lesions arising in keratinocytes soon after the fleeting exposure to sunlight. Patients are also oversensitive to environmental mutagens such as cigarette smoke and possibly to the widely used agricultural insecticide, diazinon. Progressive neurological abnormalities along with some rare complications are also noticed among these patients. Symptoms and thoracic high-resolution computed tomography are considered for diagnosis. Only corticosteroids can be given to limit the progression of the disease. Xeroderma pigmentosum-related interstitial lung disease is one of the rarest forms and we thereby report an interesting case.

A 15-year-old boy presented with bilateral spontaneous pneumothorax and diabetes insipidus.

Bronchiectasis is a disease hallmarked by abnormal permanent enlargement of large and medium size airways. The clinical presentation could be variable and it exhibits a considerable amount of overlap with other airway disorders. The etiology of bronchiectasis is heterogenous and constitutes of inherited and acquired causes pertaining to airway obstruction or defects in host immunity.

The QT interval is an electrocardiographical measurement that denotes the time interval between the commencement and completion of the cardiac ventricular contraction process.Alterations in its value indicate abnormal cardiac rhythm and herald the risk of torsades de pointes; a fatal ventricular arrhythmia.Causes leading to a prolonged QT interval encompass a heterogeneous gamut including genetic conditions, electrolyte imbalances, hormonal imbalances, and drugs.A wide range of drugs can lead to a prolonged QT interval and these include certain crucial drugs which are routinely prescribed by a pulmonologist for infectious as well as non-infectious pulmonary indications.This becomes particularly relevant in this decade which has witnessed an excrescence in drug-resistant tuberculosis cases.Certain vital drugs employed in its management prolong QT interval significantly.In these situations, the clinician faces the predicament of cautiously prescribing these drugs to eradicate the disease microbiologically whilst balancing the risk of sudden cardiac death due to torsades de pointes.We summarise the basics of QT interval which every pulmonologist presently needs to know.

Abstract Background Birt-Hogg-Dubé syndrome (BHDS) is a rare monogenic condition mostly associated with germline mutations at FLCN. It is characterized by either one or more manifestations of primary spontaneous pneumothorax (PSP), skin fibrofolliculomas and renal carcinoma (chromophobe). Here, we comprehensively studied the mutational background of 31 clinically diagnosed BHDS patients and their 74 asymptomatic related members from 15 Indian families. Results Targeted amplicon next-generation sequencing (NGS) and Sanger sequencing of FLCN in patients and asymptomatic members revealed a total of 76 variants. Among these variants, six different types of pathogenic FLCN mutations were detected in 26 patients and some asymptomatic family members. Two of the variants were novel mutations: an 11-nucleotide deletion (c.1150_1160delGTCCAGTCAGC) and a splice acceptor mutation (c.1301-1G > A). Two variants were Clinvar reported pathogenic mutations: a stop-gain (c.634C > T) and a 4-nucleotide duplication (c.1329_1332dupAGCC). Two known variants were: hotspot deletion (c.1285delC) and a splice donor mutation (c.1300 + 1G > A). FLCN mutations could not be detected in patients and asymptomatic members from 5 families. All these mutations greatly affected the protein stability and FLCN-FNIP2 interaction as observed by molecular docking method. Family-based association study inferred pathogenic FLCN mutations are significantly associated with BHDS. Conclusion Six pathogenic FLCN mutations were detected in patients from 10 families out of 15 families in the cohort. Therefore, genetic screening is necessary to validate the clinical diagnosis. The pathogenic mutations at FLCN affects the protein–protein interaction, which plays key roles in various metabolic pathways. Since, pathogenic mutations could not be detected in exonic regions of FLCN in 5 families, whole genome sequencing is necessary to detect all mutations at FLCN and/or any undescribed gene/s that may also be implicated in BHDS.

Background: Drug regimens for the treatment of drug-resistant tuberculosis (DR-TB) are composed of salvage drugs to which a patient has never been exposed to previously.Methods: A retrospective observational study was conducted in a DR-TB Center attached to a medical college in a metropolitan city using the database of category V patients (n = 100) who were prediagnosed and referred.The clinical records of the patients were reviewed for demographic data, history, sputum examinations, co-morbidities, and adverse drug reactions (ADRs).The therapy outcomes were assessed as per Revised National Tuberculosis Control Programme (RNTCP) guidelines.Results: Their mean age was 29.1 years; there were 57 males.Mean body weight was 41.8 kg.Pediatric patients (age 12-17) constituted 13%.All the patients had pulmonary TB.Of the 100 cases, 80 were Category IV failure; 5% were defaulters of Category IV; and 15% were treated with second-line drugs adequately in private.Durg-susceptibility test (DST) showed extensively drug-resistant TB (XDR-TB) in 63 and pre-XDR-TB in 37 patients.The outcomes of Category V treatment were cure (7%), died (33%), failed on therapy (4%), transferred out (16%), lost to follow-up (2%), and still on the therapy (35%).Various comorbidities were present in 25% patients.ADRs were seen in 44%, and peripheral neuropathy (18%) was the most commonly observed ADR.Conclusions: DR-TB patients were younger and males were more affected.Mortality of Category V regimen was high (33%).Most common comorbidities were anemia and hypothyroidism.Adverse reactions were common (44%); ADR peripheral neuropathy being the most common.

Pleuroparenchymal fibroelastosis (PPFE) is a rare variant of interstitial lung disease (ILD).PPFE is characterizsed by peculiar clinical, radiological and histological features.Initially considered an idiopathic entity, this disease is now classified in the rare category of the recent classification of idiopathic interstitial pneumonias (IIPs).However, it largely continues to be underdiagnosed owing to the lack of awareness about this condition.PPFE may be misdiagnosed as the other commonly diagnosed ILDs, especially IIP.The diagnosis is clinched with lung biopsy.We report a case of PPFE diagnosed by transbronchial lung biopsy.

The differential diagnosis of mediastinal masses encompasses a vast spectrum ranging from benign tumors to notorious malignancies. Mediastinal lipoma is a very rarely encountered mediastinal mass. It is usually an incidentally unearthed finding. It has a classical radiographic countenance. Knowledge of its existence and presentation aids early diagnosis and can avert unnecessary invasive investigations for alternative diagnosis. The radiographic picture of cardiophrenic obliteration may be seen in a number of heterogeneous conditions such as loculated pleural effusion, pericardial effusion, diaphragmatic hernias, or in certain mediastinal masses. However, mediastinal masses are rarely kept as a differential of this radiological presentation due to a lack of awareness. This leads to performance of invasive tests like thoracocentesis. We herein report an interesting case of mediastinal lipoma incidentally picked up during a chest radiograph done as a part of preoperative evaluation for an unrelated diagnosis. The chest radiograph exhibited a lesion with an obliteration of the right cardiophrenic angle.

InvestigationForeign body aspiration is common in children and notorious for an innocuous presentation in adolescents and adults.An 18-year-old girl presented with clinico-radiological presentation suggestive of post-obstructive consolidation with bronchostenosis.On fiberoptic bronchoscopy, a foreign body was visualised in the right lower lobe bronchus to which the patient admitted the accidental swallowing of the tip of a pen cap at the age of 11 years retrospectively.Foreign body aspiration presents with similar symptoms in adults and children, with the exception of the delay in diagnosis is common in adults.Bronchostenosis is a known complication of foreign body, however, our patient presented as masquerading bronchostenosis due to the characteristic lodgment of the pen cap.

Hereditary haemorrhagic telangiectasia (HHT), synonymously known as Osler-Weber-Rendu syndrome, is a rare autosomal inherited disorder characterised by abnormal blood vessel formation in the skin, mucous membranes, and organs including the lungs, liver and central nervous system.Straight back syndrome is characterised by the loss of normal upper thoracic spinal curvature, i.e. thoracic kyphosis, resulting in reduced antero-posterior diameter of thorax, often associated with cardiac murmurs on auscultaion and cardiomegaly on chest radiograph.The association of these abnormalities occurring simultaneously has been rarely reported.We present a case of HHT and mitral valve prolapse (MVP) associated with straight back syndrome.The patient also had respiratory failure.

Sarcoidosis is a multisystemic granulomatous disease most commonly involving the pulmonary system and having a myriad of manifestations. However literature is scanty pertaining to the profile and scoring system in sarcoidosis. This study was undertaken to understand the profile of sarcoidosis and an endeavor to assess the functional status with a simplified scoring system. This was an observational study undertaken in the department of Pulmonary Medicine at a tertiary care. The profile of these patients was studied in terms of clinical features, radiological findings, the New Modified Criteria Clinical Radiological Physiological (TNMC CRP) score, six-minute walk distance (6MWD), spirometry, arterial blood gas parameters, serum angiotensin converting enzyme (ACE) levels and tissue biopsy histopathology. The 68 patients included 41 women and 27 men with a mean age of 42.7 years. They comprised of 18 (27%), 39 (57%), 4 (6%), 7 (10%) cases of stage 1, 2, 3, 4 sarcoidosis respectively. Most common presenting symptom and sign was progressive dyspnea 49 (72%), and peripheral lymphadenopathy 15 (22%). Serum ACE was elevated in 57 (83%). The average 6MWD was 360 meters. Most common high resolution computed tomography (HRCT) finding was mediastinal lymphadenopathy and peri-bronchovascular nodules. Spirometry was restrictive abnormality in 48 (96%) patients. Evidence of pulmonary hypertension (PH) was present in 32 (47%) patients. Tissue diagnosis revealed granulomatous inflammation in 51 biopsies with a transbronchial lung biopsy (TBLB) yield of 62%. The average TNMC CRP score was 5. There was a positive correlation between this score and 6MWD which was statistically significant. The score correlated with the functional status. Diagnosis of sarcoidosis warrants a comprehensive and multimodality approach. HRCT and tissue biopsy are the most important diagnostic armamentariums. Modified simplified scores help assess the functional status of the disease.

Abstract Background: The Coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) has led to a global health care crisis. We report profiles of cases admitted to our hospital. Methods: We conducted this study at the pulmonology unit of a Dedicated COVID Hospital (DCH) of Western India. This is a prospective observational study which analysed the demographical data, clinical parameters, comorbidities, complications and laboratory and outcome parameters. Results: Our study included 101 patients. The mean age was 43 years; 64 (63.3%) were men and 37 (36.7%) women. Out of 101, 6 (6%) had mild, 30 (30%) had moderate and 65 (64%) had severe COVID-19 disease. Severity increased with age and comorbidities. Ninety-four (94%) had pneumonia. Of these 94, 65 (69%) patients had acute respiratory distress syndrome (ARDS). Twenty-one (21%) were mild, 20 (31%) moderate and 24 (37%) severe ARDS. ARDS severity increased with age and in men. Thirty-six (36%) required oxygenation only and 29 (29%) required additional ventilatory management – mostly non-invasive ventilation (NIV). The laboratory values analysis revealed elevation of D-dimers level in 66 (65.3%) and IL6 in 68 (67.3%). Diabetes mellitus (DM) and hypertension (HT) were the common comorbidities. Totally three (2.97%) patients died due to COVID-19 infection. Mortality was associated with HT and myocarditis. Mean duration of hospital stay was 15 days, and it increased with increasing severity of disease and ARDS. Conclusion: COVID-19 is common in the adult with male preponderance. The majority recovered with a good outcome. Comorbidities affected outcome adversely.

Pulmonary hypertension (PH) is a progressive disorder of the pulmonary vasculature caused due to vasoconstriction, vasodestruction, or vaso-obliteration. PH results from heterogeneous pathophysiologic mechanisms culminating in elevation of mean pulmonary arterial pressure. Patients may either present with symptoms or with chest radiographic, electrocardiographic, or echocardiographic findings done as a part of diagnostic evaluation for the primary disease or as a part of preoperative assessment. And hence, the clinician resorted to could be a family physician, pulmonologist, cardiologist, surgeon, or anesthesiologist. Hence, there is a necessity of a basic awareness about its clinical presentations, diagnostic modalities, and the available treatment options. The diagnosis can be perplexing and warrants a stepwise evaluation. Echocardiography (ECHO) is the preliminary diagnostic modality and provides a fairly accurate judgment of the pulmonary artery pressure; however, some patients may require an invasive evaluation by right heart catheterization. The definitive management of PH depends on the underlying etiology. Pulmonary arterial hypertension has a specific set of targeted pharmacotherapy, while for other categories of PH, treatment majorly focuses on counseling, management of the underlying disorder, and cardiopulmonary rehabilitation. The 6th World Symposium on PH has catered to all these issues and has incorporated manifold pertinent modifications in all these areas. Hence, we conducted an online survey to gauge the awareness about the entity and its facets among pulmonologists and penned down this review based the current concepts about PH to bridge the gaps in the existing knowledge.

Birt-Hogg-Dubé syndrome (BHDS) is an uncommon autosomal dominant syndrome. It is also known as Hornstein-Knickenberg syndrome. It is an inherited disorder culminating in mutations in folliculin coding gene (FLCN). The clinical exhibitions of the syn-drome are multi-systemic, comprising of a constellation of pulmonary, dermatologic and renal system manifestations. The most common presentations include fibrofolliculomas, renal cell carcinomas, lung cysts and spontaneous pneumothorax. The treatment is conservative with regular monitoring of the renal and lung parameters. Fibrofolliculomas may require surgical excision and recurrent events of pneumothorax may warrant pleurodesis. We reported a case series of 2 patients presenting with symptoms of progressive breathlessness along with dermatological manifestations and subsequently showing radiological manifestations of Birt-Hogg-Dubé syndrome in the form of lung cysts.

INTRODUCTION Spirometry with flow volume loop (FVL) is an easy-to-use bed side test to determine upper airway obstruction (UAO). The study aimed to find the prevalence of UAO and the ability of visual and quantitative criteria to detect UAO. METHODS This was retrospective study conducted for a period of two years at the pulmonary medicine department of a tertiary care center to find the proportion of UAO cases among patients undergoing spirometry, to study the profile of UAO, and to investigated the utility of spirometric indices described for UAO. The demographic data, diagnosis, relevant imaging and bronchoscopy reports of patients were noted. These comprised of Empey's index, ratio of the flow at the mid-point of the forced expiratory manoeuvre to the flow at the mid-point of the forced inspiratory manoeuvre (FEF50/FIF50), FIF50 <100 L/min and qualitative features like flattening of inspiratory or expiratory loops, box-shaped loop and oscillations on the loop. RESULTS Mean age of the population was 43 years. Prevalence of UAO was 24 (3.1%). The most common cause of UAO was multinodular goiter (MNG) seen in 10 (39%). The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for Empey's index were 62.5%, 89.4%, 15.7% and 98.6%, respectively. The sensitivity, specificity, PPV and NPV for qualitative criteria of flow volume loop (FVL) visual abnormalities were 95.6%, 99.7%, 91.6% and 99.8%, respectively. The sensitivity, specificity, PPV and NPV of FIF50 <100 L/min were 79.1%, 80.4%, 11.5% and 99.1%, respectively. Variable extrathoracic UAO was seen in 17, variable intrathoracic UAO in 4, while 3 cases had fixed UAO. CONCLUSIONS Spirometry with FVL is a simple and useful screening test for UAO.