Unilateral diaphragmatic palsy and pleuropericarditis in a patient with granulomatosis with polyangiitis: a case report.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis predominantly affecting upper and lower respiratory tract and kidneys. Unilateral diaphragmatic palsy could be a rare manifestation of GPA. Here we report a case of GPA in a 45-year-old male with unilateral diaphragmatic palsy with pleuropericarditis. CASE PRESENTATION: We report a case of a 45-year-old Khas male who presented with acute onset chest pain and shortness of breath who had elevated right hemidiaphragm, bilateral pleural effusion and pericardial effusion who was later diagnosed as GPA. CONCLUSIONS: GPA should be suspected in all patients with diaphragmatic palsy and pleuropericarditis with appropriate clinical and laboratory picture.

Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting.

Anti-Melanoma Differentiation-Associated gene 5 (Anti-MDA-5) dermatomyositis is a rare subtype of inflammatory myopathy characterized by unique skin lesions, rapidly progressive interstitial lung disease, and skeletal muscle inflammation. It has a high mortality rate in the absence of early treatment. However, diagnosis of this entity is challenging in a country like Nepal because of various constraints such as lack of expert rheumatologists and resource limitations. Here we describe a case of one patient who had presented to us with generalized weakness, cough and shortness of breath who was finally diagnosed as anti-MDA-5 dermatomyositis. He responded to combination of immunosuppressives and is currently doing well. This case highlights the diagnostic and therapeutic challenges in managing such cases in a resource-limited setting.

Importance of clinical history in the diagnosis of psittacosis: A case report.

Introduction: Psittacosis, caused by the bacteria Chlamydia psittaci, is primarily a disease of birds that can be transmitted to humans. The clinical manifestations of the disease are wide, ranging from asymptomatic illness to fulminant psittacosis with multi-organ failure. The organism gets attached to the upper respiratory mucosa after inhalation and the majority remain asymptomatic. However, some people may develop symptoms of atypical pneumonia. Case presentation: Psittacosis usually presents with sudden onset fever with chills and rigor, headache, and myalgia. Here we present a case of a 35 years old female with a history of close contact with parrots who presented to the ER with complaints of high-grade fever and headache for 2 weeks which started 2 days after her parrots died. Discussion: The disease usually manifests as flu-like symptoms or pneumonia and is included in the differential diagnosis of community-acquired pneumonia. Investigations reveal neutrophilia, raised erythrocyte sedimentation rate, C-reactive protein, and elevated liver enzymes which were consistent with the findings of our patient. Chest X-ray showed ill-defined consolidation in the right middle and lower lobes which were inconclusive. Hence, a CT chest was done which revealed patchy ground glass opacities with surrounding consolidation giving a reverse halo sign. Due to her contact with birds and CT findings which were suggestive of psittacosis, she was started on doxycycline and her condition improved thereafter. Conclusion: We highlight the importance of proper history taking and awareness on zoonotic diseases to the general public to prevent, diagnose and treat the disease effectively.

A multidisciplinary approach to accidental inhalational ammonia injury: A case report.

Introduction: Ammonia is a highly toxic irritant gas, and its toxicity usually occurs from occupational exposure. Most are unintentional toxicity. It causes tissue damage via exothermic reaction with body tissues causing liquefactive necrosis. Presentation depends on the level and duration of exposure. Management is supportive and its antidote is not available yet. Presentation of case: We report a case of a 22-year-old male with accidental workplace exposure from coolant damage and leakage. He had altered mental status with visible injury to the nasal cavity, and ulcerated laryngeal mucosa with asymmetric vocal cords on bronchoscopy. Computed tomography showed right lower lobe consolidations. A muti-disciplinary approach and prompt management with respiratory support along with inhaled steroids and bronchodilators, antibiotics, electrolyte replacement, analgesics, and supportive eye and oral treatments were initiated. He had to be managed with a high-flow nasal cannula with subsequent tapering of the oxygen supplementation with titration. Discussion: Inhalation injury due to ammonia toxicity can have varied presentations from mild to severe life-threatening respiratory distress and neurological deterioration. Complications can range from upper airway edema to pulmonary edema. Most of these are usual findings except for the presence of pneumothorax. It could be due to the pre-existing lung status or oxygen therapy but the literature is inadequate. Conclusion: Ammonia inhalational injury may be associated with pneumothorax. It is important to have a multi-disciplinary approach and in-hospital management of ammonia toxicity as well as to raise awareness regarding the work environment and timely recognition of the possible occupational health hazards.

Ruptured pulmonary hydatid cyst presenting as hemoptysis in TB endemic country: A case report.

Introduction: Hydatid disease is a major zoonotic disease. After the liver, the lungs are the common site of involvement. Clinical manifestations of the disease depend on the site and size of the cysts as well as the presence of complications. The majority of the cases remain asymptomatic. Complicated pulmonary cysts can present as chest pain, cough, shortness of breath, and hemoptysis. Sometimes, imaging is not sufficient for diagnosis and histopathological evaluation can provide the confirmatory diagnosis. Case presentation: A 32-year-old female presented with the complaints of episodic hemoptysis for the duration of two years. Radiological imaging could not provide a confirmatory diagnosis. Bronchoscopy was planned further. An endobronchial biopsy was taken for histopathological evaluation after seeing the whitish membranous structure within the right middle lobe bronchus. Hence, the diagnosis of ruptured cystic pulmonary hydatidosis was made. Clinical discussion: Echinococcus granulosus is the cause of cystic pulmonary hydatid disease which is transmitted through contamination by the infected definitive host, mostly dogs. Most cases remain asymptomatic for a long period. Hydatid disease has many clinical and radiological forms which should be recognized and included in the differential diagnosis of many pulmonary problems. Sometimes, imaging and serological studies may not confirm the diagnosis, histopathological evaluation may be required. Conclusion: Uncomplicated hydatid cysts are most commonly diagnosed incidentally in the adult population. Complicated hydatid cyst can present with various clinical manifestations. Episodic hemoptysis is one of the manifestations in our case. Clinicians should be aware of the typical as well as atypical manifestations of pulmonary hydatid disease.

Multiple tracheobronchial diverticula in a post-TB patient: A case report.

The presence of multiple tracheal and bronchial diverticula is a rare condition. We present a case of a 22-years old non-smoker male with a history of pulmonary tuberculosis, having multiple tracheal and bronchial diverticula along with other common sequelae such as stenosed and collapsed upper lobe bronchi.

Case Report: Adult Onset Still's Disease after vaccination against Covid-19.

Vaccination against the virus responsible for COVID-19 has become key in preventing mortality and morbidity related to the infection. Studies have shown that the benefits of vaccination outweigh the risks. However, there are concerns regarding serious adverse events of some vaccines, although they are fortunately rare. Here, we report a case of a 47-year-old female from Kathmandu who presented with high grade fever, dry cough and erythematous rash a week after exposure to the Oxford-AstraZeneca vaccine. She had hepatosplenomegaly, persistent leucocytosis, anaemia and thrombocytosis along with markedly raised inflammatory markers. Her tests for infectious causes and haematological malignancies were negative and she showed no response to multiple antibiotics. Finally, she had a dramatic response to steroids with disappearance of fever and normalization of other laboratory parameters. Hence, she was diagnosed with Adult-onset Still's Disease (AOSD). She was under methotrexate and prednisolone tapering dose and doing well as of the time of writing. The trigger for the disease was hypothesized to be the vaccine because of the strong temporal association.

Pneumomediastinum in COVID-19 Patients with Acute Respiratory Distress Syndrome.

BACKGROUND: Pneumomediastinum, an uncommon entity, has been on the rise with increasing cases of COVID-Acute respiratory distress syndrome. It has been unclear whether this entity represents an indicator of poor clinical outcome or not. The aims of this study were to find out the incidence of pneumomediastinum in COVID-Acute respiratory distress syndrome patients, describe their clinical characteristics and try to explain its plausible mechanisms.. METHODS: A descriptive, cross-sectional study was carried out in the ICU of our hospital among 280 patients admitted with COVID-Acute respiratory distress syndrome over a period of 6 months. Demographics along with various clinical, laboratory, and radiological parameters were analyzed. Relevant statistical analyses were done to summarize our findings. RESULTS: The incidence of pneumomediastinum in COVID-ARDS patients was 2.8%. All patients were male, none had pulmonary co-morbidities and six of them (60%) were on invasive mechanical ventilation. All intubated patients were on lung protective mechanical ventilation. The median PEEP, peak airway pressure and plateau pressure were 10 (IQR: 4), 29 (IQR: 8) and 28 (IQR: 4) respectively. The mean CT severity score was 22.7 (SD: 1.64). Five patients died after 6.8 days (SD: 4.8) of diagnosis. The average hospital stay was of 34 days. CONCLUSIONS: Pneumomediastinum is a possible complication of COVID-Acute respiratory distress syndrome which could signify the disease severity and vice versa. Furthermore, it could be an indicator of relatively poor prognosis and therefore requires larger studies to establish the association.

Guidelines for diagnostic flexible bronchoscopy in adults: Joint Indian Chest Society/National College of chest physicians (I)/Indian association for bronchology recommendations.

Flexible bronchoscopy (FB) is commonly performed by respiratory physicians for diagnostic as well as therapeutic purposes. However, bronchoscopy practices vary widely across India and worldwide. The three major respiratory organizations of the country supported a national-level expert group that formulated a comprehensive guideline document for FB based on a detailed appraisal of available evidence. These guidelines are an attempt to provide the bronchoscopist with the most scientifically sound as well as practical approach of bronchoscopy. It involved framing appropriate questions, review and critical appraisal of the relevant literature and reaching a recommendation by the expert groups. The guidelines cover major areas in basic bronchoscopy including (but not limited to), indications for procedure, patient preparation, various sampling procedures, bronchoscopy in the ICU setting, equipment care, and training issues. The target audience is respiratory physicians working in India and well as other parts of the world. It is hoped that this document would serve as a complete reference guide for all pulmonary physicians performing or desiring to learn the technique of flexible bronchoscopy.

A 44-year-old man with hemoptysis.

A 44-year-old man with background history of diffuse cutaneous systemic sclerosis and dilated cardiomyopathy receiving immunosuppressive medications, presented with a 2-month history of cough and streaky hemoptysis. Clinicoradiological features were consistent with an endotracheal mass. Subsequently, the patient developed nodular skin lesions and the tracheal mass increased in size causing central airway obstruction. This clinicopathologic conference discusses the clinical and radiological differential diagnoses of such a clinical presentation and their management options.

Impact of a structured ICU training programme in resource-limited settings in Asia.

OBJECTIVE: To assess the impact on ICU performance of a modular training program in three resource-limited general adult ICUs in India, Bangladesh, and Nepal. METHOD: A modular ICU training programme was evaluated using performance indicators from June 2009 to June 2012 using an interrupted time series design with an 8 to 15 month pre-intervention and 18 to 24 month post-intervention period. ICU physicians and nurses trained in Europe and the USA provided training for ICU doctors and nurses. The training program consisted of six modules on basic intensive care practices of 2-3 weeks each over 20 months. The performance indicators consisting of ICU mortality, time to ICU discharge, rate at which patients were discharged alive from the ICU, discontinuation of mechanical ventilation or vasoactive drugs and duration of antibiotic use were extracted. Stepwise changes and changes in trends associated with the intervention were analysed. RESULTS: Pre-Training ICU mortality in Rourkela (India), and Patan (Nepal) Chittagong (Bangladesh), was 28%, 41% and 62%, respectively, compared to 30%, 18% and 51% post-intervention. The intervention was associated with a stepwise reduction in cumulative incidence of in-ICU mortality in Chittagong (adjusted subdistribution hazard ratio [aSHR] (95% CI): 0.62 (0.40, 0.97), p = 0.03) and Patan (aSHR 0.16 (0.06, 0.41), p<0.001), but not in Rourkela (aSHR: 1.17 (0.75, 1.82), p = 0.49). The intervention was associated with earlier discontinuation of vasoactive drugs at Rourkela (adjusted hazard ratio for weekly change [aHR] 1.08 (1.03, 1.14), earlier discontinuation of mechanical ventilation in Chittagong (aHR 2.97 (1.24, 7.14), p = 0.02), and earlier ICU discharge in Patan (aHR 1.87 (1.02, 3.43), p = 0.04). CONCLUSION: This structured training program was associated with a decrease in ICU mortality in two of three sites and improvement of other performance indicators. A larger cluster randomised study assessing process outcomes and longer-term indicators is warranted.

Management of pulmonary alveolar proteinosis with whole lung lavage using extracorporeal membrane oxygenation support in a postrenal transplant patient with graft failure.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of excessive lung surfactant in the alveoli leading to restrictive lung functions and impaired gas exchange. Whole lung lavage (WLL) is the treatment modality of choice, which is usually performed using double lumen endobronchial tube insertion under general anesthesia and alternating unilateral lung ventilation and washing with normal saline. It may be difficult to perform WLL in patients with severe hypoxemia wherein patients do not tolerate single lung ventilation. Extracorporeal membrane oxygenation support (ECMO) has been used in such patients. We report a patient with autoimmune PAP following renal transplant who presented with marked hypoxemia and was managed by WLL under ECMO support.

Validation of the St. George's Respiratory Questionnaire in Nepal.

The St. George's Respiratory Questionnaire (SGRQ) is a standardized questionnaire for measuring impaired health and perceived well-being in chronic airway disease, but it is not available in the Nepali language. We translated the original SGRQ into Nepali and validated its use in 150 individuals aged 40 to 80 years with and without COPD.We also examined if the SGRQ could be used as a screening tool to identify individuals at risk for COPD. We translated the SGRQ following a standard protocol. The validation study was then conducted in both community and hospital-based settings in Nepal. We enrolled 100 participants from a community setting who were not actively seeking medical care, 50 of which met criteria for chronic obstructive pulmonary disease (COPD) (post-bronchodilator forced expiratory volume in 1 second [FEV1]/ forced vital capacity [FVC]<70%) and 50 who did not. We also enrolled 50 participants with an established diagnosis of COPD who attended outpatient pulmonary clinics. All participants completed the questionnaire. We used linear regressions to compare average SGRQ scores by disease status categories and by lung function values, adjusted for age, sex, height and body mass index (BMI).All 150 participants (mean age 59.8 years, 48% male, mean BMI 20.5 kg/m2) completed the SGRQ. In multivariable regression, the average SGRQ total score was 23.9 points higher in established cases of COPD and 18.1 points higher in community cases of COPD when compared to participants without COPD living in the community (all p<0.001). The SGRQ total score also increased by an average of 2.1 points for each 100 mL decrease in post-FEV1 (p<0.001). The area-under-the-curve for the SGRQ total score as a predictor of COPD was 0.77 (95% confidence interval [CI] 0.68 to 0.85) and the optimal cutoff to identify COPD was 33 points.We developed a Nepali-validated version of SGRQ, which correlated well with both disease status and severity.