Diffuse alveolar haemorrhage in the setting of eosinophilic pneumonia

A 65-year-old woman who presented with a constellation of symptoms, including cough with haemoptysis, fever, chills and hypoxia along with weight loss, was found to have diffuse alveolar haemorrhage. After a myriad of investigations returned normal, an open lung biopsy was performed, which revealed the diagnosis to be subacute eosinophilic pneumonia. This is one of its kind of rare presentations where eosinophilic pneumonia presents as diffuse alveolar haemorrhage and has been reported only five times prior to this.

Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum

A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.

Seronegative Goodpasture’s syndrome associated with organising pneumonia

Goodpasture’s syndrome is a rare vasculitis associated with anti-glomerular basement membrane (anti-GBM) autoantibodies that target type IV collagen found in the basement membranes of glomeruli and alveoli. We present a case of a 79-year-old man with seronegative Goodpasture’s syndrome with predominant respiratory symptoms and mild acute kidney injury that initially improved. Final diagnosis was made by immunofluorescent staining on open lung biopsy which also revealed concomitant organising pneumonia. The patient underwent treatment with corticosteroids, cyclophosphamide, haemodialysis and plasmapheresis. This was an atypical presentation wherein the patient only exhibited pulmonary symptoms early in the course of illness in the setting of negative anti-GBM antibody serum testing, which made diagnosis challenging. With this case, we emphasise that clinicians should have a high suspicion for Goodpasture’s syndrome in the setting of unexplained severe pulmonary or renal disease despite negative anti-GBM antibody testing.

What Open-Lung Biopsy Teaches Us about ARDS in COVID-19 Patients: Mechanisms, Pathology, and Therapeutic Implications

Difficulties have risen while managing Acute Respiratory Distress Syndrome (ARDS) caused by COVID-19, although it meets the Berlin definition. Severe hypoxemia with near-normal compliance was noted along with coagulopathy. Understanding the precise pathophysiology of this atypical ARDS will assist researchers and physicians in improving their therapeutic approach. Previous work is limited to postmortem studies, while our report addresses patients under protective lung mechanical ventilation. An open-lung minithoracotomy was performed in 3 patients who developed ARDS related to COVID-19 and were admitted to the intensive care unit to carry out a pathological and microbiological analysis on lung tissue biopsy. Diffused alveolar damage with hyaline membranes was found, as well as plurifocal fibrin microthrombi and vascular congestion in all patients’ specimens. Microbiological cultures were negative, whereas qualitative Reversed Transcriptase Polymerase Chain Reaction (RT-PCR) detected SARS-CoV-2 in the pulmonary parenchyma and pleural fluid in two patients. COVID-19 causes progressive ARDS with onset of severe hypoxemia, underlying a dual mechanism: shunt effect through diffused alveolar damage and dead space effect through thrombotic injuries in microvascular beds. It seems reasonable to manage this ventilation-perfusion ratio mismatch using a high dose of anticoagulant combined with glucocorticoids.

Diagnostic approach in parenchymal lung diseases: transbronchial lung biopsy or cryobiopsy?

Background/aim: Diagnosis of interstitial lung diseases requires a multidisciplinary approach, and a gold standard for histological diagnosis is open lung biopsy. Transbronchial lung biopsy (TBLB) and in recent years an alternative method, cryobiopsy (TBLC), are used for the diagnosis of parenchymal lung lesions. The aim of this study is to compare the efficacy of concomitant conventional TBLB and TBLC.Materials and methods: A total of 82 patients who underwent TBLC for diagnosis of diffuse parenchymal lung diseases at Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital between 2015 and 2018 were screened retrospectively and included in the study. Of the patients, 53.7% (n: 44) were male, and 46.4% (n:38) of them were female. The mean age was 58.37 (±9.33) years. First TBLB and then TBLC were performed to all patients in the same session and their diagnostic performances were compared.Results: Although both procedures were done in the same session, 45 patients (54.9%) were diagnosed with TBLB and 75 patients (91.5%) were diagnosed with TBLC (P ˂ 0.001). Hemorrhage was observed in 39 patients (47.6%), but only one had a massive hemorrhage. Pneumothorax was observed in 6 patients (7.3%) and none of them required tube drainage.Conclusion: Transbronchial lung cryobiopsy is a promising technique for the diagnosis of parenchymal lung diseases compared to transbronchial lung biopsy.

A CASE OF BURNT OUT LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS POST-PARTUM HYPOPITUITARISM

Objective: Hypopituitarism in post-partum women is usually attributed to lymphocytic hypophysitis (LH), but can also be secondary to systemic pathology. We discuss a case of a lady presenting with central hypogonadism and diabetes insipidus, who further developed persistent cough, leading to an unexpected diagnosis of burnt-out Langerhans’ cell histiocytosis (LCH). Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) and open-lung biopsy results are discussed. Results: A 28 year old lady, presented at 10 months postpartum with polydipsia, polyuria and amenorrhoea for 3 months. Her results showed prolactin of 25 (reference,<23.5) μg/L , oestrogen of 24.8 (reference,30–49), follicular stimulating hormone 6 (reference, 2–20) IU/L and luteinizing hormone 6 (reference, 2–70) IU/L. Water deprivation test demonstrated sodium 148 (reference, 135–145) mEq/l, serum osmolality 310 (reference, 275–295) mmol/kg and urine osmolality 107 (reference,50–1450) mmol/kg improving to 142 mEq/L, 295mEq/L and 535 mEq/L respectively, after desmopressin. Gadolinium enhanced pituitary MRI demonstrated a markedly thickened stalk with uniform enhancement. Chest HRCT confirmed bilateral upper zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis (PLAM) or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as PLAM exacerbates with oestrogen therapy and pregnancy which she was able to successfully pursue without disease exacerbation. Conclusion: The patient’s initial presentation was considered as LH, but subsequent cystic changes on HRCT led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.

Pulmonary Infectious Complications in Children with Hematologic Malignancies and Chemotherapy-Induced Neutropenia

Infections frequently complicate the treatment course in children with hematologic malignancies undergoing chemotherapy. Febrile neutropenia (FN) remains a major cause of hospital admissions in this population, and respiratory tract is often proven to be the site of infection even without respiratory signs and symptoms. Clinical presentation may be subtle due to impaired inflammatory response. Common respiratory viruses and bacteria are widely identified in these patients, while fungi and, less commonly, bacteria are the causative agents in more severe cases. A detailed history, thorough clinical and basic laboratory examination along with a chest radiograph are the first steps in the evaluation of a child presenting signs of a pulmonary infection. After stratifying patient’s risk, prompt initiation of the appropriate empirical antimicrobial treatment is crucial and efficient for the majority of the patients. High-risk children should be treated with an intravenous antipseudomonal beta lactam agent, unless there is suspicion of multi-drug resistance when an antibiotic combination should be used. In unresponsive cases, more invasive procedures, including bronchoalveolar lavage (BAL), computed tomography (CT)-guided fine-needle aspiration or open lung biopsy (OLB), are recommended. Overall mortality rate can reach 20% with higher rates seen in cases unresponsive to initial therapy and those under mechanical ventilation.

Investigating the Role of Open Lung Biopsy in Diagnosing the Type of Chronic Lung Disease in Children

Objective: This study was conducted for the practical use of biopsy in the diagnosis of chronic lung disease and the guidance of risks and benefits. Design of Study: We studied 64 children with chronic lung disease who underwent open lung biopsy in 5 years at the Children's Medical Center . Results: Biopsy results were diagnostic in 57 cases (89.1%) and non-diagnostic in 7 cases (10.9%). The biopsy determined the type of mass in all cases where a possible diagnosis of lung mass or thoracic wall was made. In 37 cases (57.8%) the diagnosis was changed and the exact diagnosis was determined. The main side effects (including pneumothorax, hemothorax, pyothorax, and pleural effusion) were 50% (32 cases), the most common of which were pneumothorax and pleural effusion, with a total of more than 87% of these major complications. 22 patients (34.4%) required intubation. 24 patients (37.5%) were admitted to the ICU after surgery. The death occurred in only one case, who was a 3-month-old boy with a disorder of INR and suffering from acute respiratory distress syndrome. There was no mortality that could be directly related to surgery. Conclusion: Open lung biopsy is a gold standard for the histological diagnosis of chronic pulmonary disease in children and plays an important role in the treatment of children with chronic pulmonary diseases. However, serious and common side effects of this method should be considered