A Rare Case of Lung Hypoplasia, Cardiac Anomalies and Ovarian Tumour in a Patient with Mayer-Rokitansky-Küster-Hauser Syndrome.

Hypoplasia of the lung is an uncommon congenital abnormality of the respiratory system in contrast to pulmonary agenesis. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the congenital absence of the upper two-thirds of the vagina and uterus with normal secondary sexual characteristics, ovary and normal karyotype. We report a 31-year-old female patient who presented in 2022 with cough with expectoration, left-side chest pain and breathlessness for 4 years to tertiary hospital, Puducherry, India. She was evaluated for amenorrhoea and diagnosed as MRKH syndrome and the patient underwent right-side oophorectomy for right ovarian torsion with a tumour. Computed tomography pulmonary angiogram and fiberoptic endoscopy were suggestive of left lung hypoplasia and the patient was advised symptomatic treatment for lung hypoplasia and planned for vaginoplasty for which she refused.

Nil Intervention is at Times the Best Intervention: Benign emptying of pneumonectomy space.

A sudden drop of air-fluid level in the pneumonectomy space in the absence of a bronchopleural fistula and pleural infection is termed benign emptying of the pneumonectomy space (BEPS). We report a 28-year-old female patient who presented to a tertiary care referral centre, in Pondicherry, India in 2020 with multiple episodes of vomiting. Subsequent to a left-sided pneumonectomy due to tuberculosis, she was diagnosed with BEPS. Generally, patients with BEPS are clinically stable, afebrile with no fluid expectoration and have a normal white blood cell count. Bronchoscopy reveals an intact bronchial stump and pleural fluid cultures are often sterile. In terms of management, close monitoring and early detection of a bronchopleural fistula are the key points. BEPS should be a differential diagnosis in case of a drop in the air-fluid level of the post-pneumonectomy space. Awareness of this entity is crucial as it helps prevent unnecessary and morbid surgical interventions.

Cocaine-Induced Steroid Resistant Organising Pneumonia in a Young Male: The Lows of Getting High.

Organizing pneumonia (OP) is a diffuse parenchymal lung disease occurring due to injury to the alveoli leading to typical histopathological features. Infections, connective tissue disorders, and medications are common aetiologies of OP. Cocaine-induced OP is uncommon. The patient had a fever and sore throat for two days corresponding to crack inhalation, followed by breathlessness that rapidly progressed to acute hypoxemic respiratory failure within one week. Radiology showed bilateral consolidation and ground glass opacities but did not respond to empiric treatment with antibiotics. After a multidisciplinary discussion, he was provisionally diagnosed as OP and treated with an intravenous methylprednisolone pulse dosage followed by oral prednisolone. OP was confirmed by surgical lung biopsy with the detection of Masson bodies. In view of progressive respiratory failure, steroid-resistant OP was diagnosed, and rituximab was administered as a second-line agent, but unfortunately, succumbed to respiratory failure. OP should be considered a differential in patients with consolidation who are non-responsive to initial conventional treatment. Multidisciplinary discussion and early lung biopsy to initiate immunosuppressants in the inflammatory stage of OP are emphasized for a possible better response.

Overcoming the challenges of a misdiagnosed rare lung disease - Idiopathic pleuroparenchymal fibroelastosis.

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by pleural and subpleural lung fibroelastosis with an upper lobe predominance. We present the third case of idiopathic pleuroparenchymal fibroelastosis (IPPFE) from India, as well as the second antemortem diagnosis. A 27-year-old man presented with a one-year history of mMRC class II dry cough and shortness of breath. He described a fifteen-kilogram weight loss. After a clinico-radiological diagnosis, he was given anti-tubercular treatment and referred because he showed no improvement. A high-resolution computed tomography of the chest revealed bilateral upper lobe bullae, parenchymal and subpleural fibrosis, and irregular pleural thickening. PPFE was found in surgical lung and pleural biopsies. He was given systemic glucocorticoids but did not respond clinically or radiologically. Pirfenidone and a lung transplant were out of reach for him. He died nine months after being diagnosed with his condition. Finally, IPPFE is an extremely rare entity, with only three cases reported from our subcontinent. As a result, it is easily underdiagnosed or misdiagnosed; clinician awareness of this condition is critical for better diagnosis and management.

An unexpected and unusual cause of pulmonary hypertension in a patient with hypersensitivity pneumonitis: a partial anomalous pulmonary venous connection causing pulmonary artery hypertension.

Partial anomalous pulmonary venous connection (PAPVC) occurs when any pulmonary vein, but not all, drains directly into the right atrium or its venous tributaries. PAPVC can very rarely present as an individual cause of pulmonary artery hypertension. Here we are presenting the case of a 41-year-old farmer with a history of exertional dyspnea for the past 3 years, which increased over 6 months. Chest high-resolution computed tomography was suggestive of non-fibrotic hypersensitivity pneumonitis. Hence, the patient was started on systemic steroids, with which the patient's oxygen saturation improved. On 2D echocardiography, the right ventricle systolic pressure was 48 + right atrial pressure. Right heart catheterization showed a mean pulmonary artery pressure of 73 mmHg and pulmonary vascular resistance of 8.7. On further evaluation, a computed tomography pulmonary angiogram was done, which surprisingly revealed the left superior pulmonary vein draining into the left brachiocephalic vein.

Comparison of efficacy of autologous blood patch pleurodesis versus doxycycline pleurodesis in the management of persistent air leak in patients with secondary spontaneous pneumothorax. A randomized control trial.

Secondary spontaneous pneumothorax (SSP) patients sometimes have a persistent air leak (PAL) that leads to increased morbidity and increased hospital stay. Further, these patients also need pleurodesis to prevent a recurrence. Autologous blood patch pleurodesis (ABPP) has been successfully tried in post lobectomy air leak cases. However, its utility in SSP patients with PAL has not been widely established. In this context, we performed a non-blinded randomized control study to compare the efficacy of ABPP vs doxycycline pleurodesis in the closure of air leak in SSP patients. This study was a non-blinded randomized control study done in 38 SSP patients with PAL. They were randomized into doxycycline arm and ABPP arm. Post pleurodesis air leak was assessed every 12 hours for cessation. Success was considered when there was a complete cessation of air leak. Post-procedural complications were noted and patients were followed up for 28 days to look for recurrence. The median time for closure was 24 hours [interquartile range (12.24)] for ABPP and 36 hours [interquartile range (24.72)] for doxycycline pleurodesis. Success rate of ABPP vs doxycycline pleurodesis is 94.7% vs 84.2% (p=0.6). Post-procedure pain was more in doxycycline group (15% vs 73%) (p<0.01). On visual analogue scale, ABPP had a lesser pain as compared to doxycycline pleurodesis (average visual analogue scale value of 2 vs scale of 7). The recurrence rate was equal in both groups (5%). Though ABPP and doxycycline have similar success rates, ABPP has an early closure of air leak and greater patient tolerability. ABPP can be tried as an alternative to doxycycline pleurodesis in SSP patients with persistent air leaks.

Endocrine paraneoplastic syndromes in lung cancer: a respiratory physician's perspective.

Lung malignancy is known to be one of the leading causes of cancer-related mortality. Endocrine paraneoplastic syndromes in lung cancer are common. These are due to secretion of various substances and not because of direct tumour invasion or me-tastasis. These syndromes have also been associated with lung cancer prognosis. This review describes the many endocrine paraneoplastic syndromes seen in lung cancer and narrates their incidence, biology, clinical features, diagnosis, and management.

New twist to an old problem: COVID-19 and idiopathic pulmonary fibrosis.

Iidiopathic pulmonary fibrosis (IPF) diagnosis and treatment during this COVID-19 pandemic have been affected. COVID-19 has not only impacted the prognosis of these patients but also the approach to these patients. Pulmonary function tests (PFT) and lung biopsies are less encouraged now. Traditional antifibrotics used in IPF should be encouraged irrespective of patient lung function and in those with non-definite usual interstitial pneumonia in high resolution computed tomography.

Hospital treatment of severe acute exacerbation of chronic obstructive pulmonary disease in COVID-19 situation: back to basics.

Patients of chronic obstructive pulmonary disease (COPD) during COVID-19 pandemic have higher morbidity. Treatment of these patients require aerosolization procedures like nebulization and noninvasive modalities for ventilation like non-invasive ventilation (NIV) and high flow nasal cannula (HFNC). Role of these procedures in corona virus transmission when treating a case of acute exacerbation of chronic obstructive pulmonary disease should be further studied.

When attacked by a new enemy, do not forget the old ones. A tale of 2 diseases: tuberculosis and COVID-19.

e two diseases together.

Joint Indian Chest Society-National College of Chest Physicians (India) guidelines for spirometry.

Although a simple and useful pulmonary function test, spirometry remains underutilized in India. The Indian Chest Society and National College of Chest Physicians (India) jointly supported an expert group to provide recommendations for spirometry in India. Based on a scientific grading of available published evidence, as well as other international recommendations, we propose a consensus statement for planning, performing and interpreting spirometry in a systematic manner across all levels of healthcare in India. We stress the use of standard equipment, and the need for quality control, to optimize testing. Important technical requirements for patient selection, and proper conduct of the vital capacity maneuver, are outlined. A brief algorithm to interpret and report spirometric data using minimal and most important variables is presented. The use of statistically valid lower limits of normality during interpretation is emphasized, and a listing of Indian reference equations is provided for this purpose. Other important issues such as peak expiratory flow, bronchodilator reversibility testing, and technician training are also discussed. We hope that this document will improve use of spirometry in a standardized fashion across diverse settings in India.