Thoracic endometrial syndrome with unilateral exudative pleural effusion.

A woman in her 40s presented with exertional dyspnoea with an absence of haemoptysis, cough, fever and weight loss. The patient had a medical history of extensive endometriosis. Investigations revealed a large right-sided pleural effusion. The effusion was aspirated and was exudative in nature.A contrast-enhanced CT thorax was performed to help exclude dual pathology. The only positive finding was bilateral breast nodules, subsequently found to be benign fibroadenomas on histological analysis of biopsy samples.After malignancy was ruled out as a cause, the patient was referred for medical thoracoscopy for a biopsy and other investigations. Histology demonstrated the presence of endometrial tissue in the pleura and thereby confirmed the diagnosis of thoracic endometrial syndrome.Video-assisted thoracoscopic surgery repair of diaphragm and talc pleurodesis was carried out in an uncomplicated procedure and the patient was discharged with good recovery.

Raised FGF23 Correlates to Increased Mortality in Critical Illness, Independent of Vitamin D.

BACKGROUND: Fibroblast Growth Factor (FGF23) is an endocrine hormone classically associated with the homeostasis of vitamin D, phosphate, and calcium. Elevated serum FGF23 is a known independent risk factor for mortality in chronic kidney disease (CKD) patients. We aimed to determine if there was a similar relationship between FGF23 levels and mortality in critically ill patients. METHODS: Plasma FGF23 levels were measured by ELISA in two separate cohorts of patients receiving vitamin D supplementation: critical illness patients (VITdAL-ICU trial, n = 475) and elective oesophagectomy patients (VINDALOO trial, n = 76). Mortality data were recorded at 30 and 180 days or at two years, respectively. FGF23 levels in a healthy control cohort were also measured (n = 27). RESULTS: Elevated FGF23 (quartile 4 vs. quartiles 1-3) was associated with increased short-term (30 and 180 day) mortality in critical illness patients (p < 0.001) and long-term (two-year) mortality in oesophagectomy patients (p = 0.0149). Patients who died had significantly higher FGF23 levels than those who survived: In the critical illness cohort, those who died had 1194.6 pg/mL (range 0-14,000), while those who survived had 120.4 pg/mL (range = 15-14,000) (p = 0.0462). In the oesophagectomy cohort, those who died had 1304 pg/mL (range = 154-77,800), while those who survived had 644 pg/mL (range = 179-54,894) (p < 0.001). This was found to be independent of vitamin D or CKD status (critical illness p = 0.3507; oesophagectomy p = 0.3800). FGF23 levels in healthy controls were similar to those seen in oesophagectomy patients (p = 0.4802). CONCLUSIONS: Elevated baseline serum FGF23 is correlated with increased mortality in both the post-oesophagectomy cohort and the cohort of patients with critical illness requiring intensive care admission. This was independent of vitamin D status, supplementation, or CKD status, which suggests the presence of vitamin D-independent mechanisms of FGF23 action during the acute and convalescent stages of critical illness, warranting further investigation.

De novo pancytopaenia in an older adult with severe COVID-19 infection.

During the COVID-19 pandemic, it was recognised that SARS-CoV-2 can cause multisystem illness. Non-respiratory complications observed early in the pandemic were haematological in nature. A rare but serious haematological complication of COVID-19 infection is pancytopaenia. We describe a case of an older adult without pre-existing haematological disease or risk factors for cell dyscrasia with severe pancytopaenia induced by COVID-19, who developed critical illness requiring respiratory support in intensive care and died. Our case report highlights that de novo pancytopaenia may only present with mild dermatological manifestations and may indicate severe COVID-19 infection. Management is primarily supportive and early involvement of haematology should be sought.

Pneumocystis pneumonia causing cavitating lung nodules in an immunocompetent individual.

Pneumocystis jirovecii pneumonia (PCP) is a potential life-threatening pulmonary infection which commonly manifests in immunosuppressed patients especially with HIV, with underlying malignancies, severe malnutrition as well as those on immunosuppressive treatments. There have been case reports of symptomatic PCP in individuals with a normally functioning immune system with typical clinical features and radiologic findings of bilateral and diffuse interstitial opacities. However, PCP in immunocompetent individuals presenting with lung nodules had been rarely reported. We report a 53-year-old immunocompetent gentleman who presented with subacute cough, progressive shortness of breath and radiographic findings of multiple lung nodules with central cavitation. The diagnosis of PCP was made by detection of PCP DNA PCR in bronchoalveolar lavage sample following fibreoptic bronchoscopy. This case also highlights the atypical radiographic findings of multiple cavitating lung nodules as a presentation of PCP in an immunocompetent patient.

Pneumomediastinum in patients with SARS-CoV-2 treated with non-invasive ventilation.

SARS-CoV-2, causing the pandemic COVID-19, has rapidly spread, overwhelming healthcare systems. Non-invasive positive pressure ventilation (NIV) can be used as a bridging therapy to delay invasive mechanical ventilation or as a standalone therapy. Spontaneous pneumomediastinum is rare and self-limiting, but there is an increased incidence documented in COVID-19.Here we document two cases of pneumomediastinum-related prolonged NIV therapy in severe COVID-19. Patient 1, a 64-year-old man, who developed symptoms after NIV therapy was weaned and survived. Patient 2, an 82-year-old woman, failed to improve despite NIV therapy, on investigation was found to have a pneumomediastinum. After review, the patient was placed on best supportive care and died 3 days later.We highlight the importance of recognising less common causes of deterioration in severe COVID-19 treated with NIV. In addition, pneumomediastinum in these cases may not always lead to poor outcomes.

Metabolic and Endocrine Challenges.

This review aims to provide an overview of metabolic and endocrine challenges in the setting of intensive care medicine. These are a group of heterogeneous clinical conditions with a high degree of overlap, as well as nonspecific signs and symptoms. Several diseases involve multiple organ systems, potentially causing catastrophic dysfunction and death. In the majority of cases, endocrine challenges accompany other organ failures or manifest as a complication of prolonged intensive care unit stay and malnutrition. However, when endocrine disorders present as an isolated syndrome, they are a rare and extreme manifestation. As they are uncommon, these can typically challenge both with diagnosis and management. Acute exacerbations may be elicited by triggers such as infections, trauma, surgery, and hemorrhage. In this complex scenario, early diagnosis and prompt treatment require knowledge of the specific endocrine syndrome. Here, we review diabetic coma, hyponatremia, hypercalcemia, thyroid emergencies, pituitary insufficiency, adrenal crisis, and vitamin D deficiency, highlighting diagnostic tools and tricks, and management pathways through defining common clinical presentations.

Mepolizumab rescue therapy for acute pneumonitis secondary to DRESS.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome represents a severe adverse drug reaction driven by eosinophilia. Treatment is focused on withdrawal of medication, supportive care and immunosuppression such as high-dose corticosteroid therapy. Here we report a 56-year-old male patient who initially presented with breathlessness and eosinophilia, subsequent development of respiratory failure and admission to ITU for non-invasive ventilation. The patient continued to deteriorate despite high-dose prednisolone and methylprednisolone. Other causes of hypereosinophilia were normal. He was diagnosed with DRESS syndrome secondary to pregabalin and was treated with subcutaneous mepolizumab. We observed the rapid resolution of eosinophilia and clinical improvement; the patient was discharged home within a month of administration. This represents the successful use of mepolizumab in the acute setting of pulmonary failure secondary to DRESS. A similar approach could be adopted in other acute conditions with refractory eosinophilic inflammation where standard steroid therapy has failed.

Oxygen prescription: improving compliance using methods from BMJ Open Quality journal.

Oxygen is an important drug frequently used in the management of acutely unwell hospital patients. However, oxygen overuse can have fatal side effects particularly for those patients at risk of iatrogenic hypercapnia. British Thoracic Society Guidelines state that oxygen must be prescribed for all patients, with target saturations stipulated on the prescription for patient safety. A quality improvement project was undertaken with the aim to improve the oxygen prescription rate across the respiratory ward at a district general hospital, over a period of 3 months. Quality improvement methods were implemented based on data analysis at each stage, following discussion with senior doctors and specialist nurses, and after reviewing previous quality improvement projects published on BMJ Open Quality. The initial interventions of poster reminders and multidisciplinary team education failed to significantly improve the rates of oxygen prescription. Use of a targeted intervention where stickers were placed above oxygen taps significantly improved prescription rate from 20% in the non-targeted group to 60% in the targeted group. This was based on a BMJ Open Quality published improvement method. The current guidelines from the British Thoracic Society, and hospital's own guidelines, advise good oxygen prescribing. However, these recommendations alone are ineffective at achieving compliance among prescribers. Further targeted interventions have shown improvements in oxygen prescriptions and could lead to better clinical practice, patient care and safety.

Pyrexia of unknown origin: inferior vena cava agenesis.

A 26-year-old woman presented with a 5-day history of fever after returning from Bali. She denied sexual contact abroad. On examination, there was suprapubic tenderness and a widespread maculopapular rash. Malaria serology was negative and blood tests were normal except for an elevated C reactive protein. Treatment was initially with ceftriaxone, metronidazole and doxycycline, but her symptoms failed to improve. A CT pelvis suggested a possible tubo-ovarian abscess, a suspected inferior vena cava (IVC) anomaly and left internal iliac/femoral venous thrombosis. A gynaecology review demonstrated left tubo-ovarian tenderness and fullness. An MRI suggested pelvic inflammatory disease and thrombophlebitis affecting the pelvic veins; deep vein thrombosis (DVT) treatment was commenced. Further family history revealed thrombosis throughout multiple generations. Further imaging analysis demonstrated agenesis of the IVC with compensatory dilation of pelvic collaterals and an acute DVT of the deep pelvic venous system. The patient was discharged with direct oral anticoagulant therapy.

A novel 506kb deletion causing ÎµÎ³Î´ß thalassemia.

We describe a novel deletion causing ÎµÎ³Î´ß thalassemia in a Pakistani family. The Pakistani deletion is 506kb in length, and the second largest ÎµÎ³Î´ß thalassemia deletion reported to date. It removes the entire ß globin gene (HBB) cluster, extending from 431kb upstream to 75kb downstream of the ε globin gene (HBE). The breakpoint junction occurred within a 160bp palindrome embedded in LINE/LTR repeats, and contained a short (9bp) region of direct homology which may have contributed to the recombination event. Characterization of the deletion breakpoints has been particularly challenging due to the complexity of DNA deletion, insertion and inversion, involving a multitude of methodologies, mirroring the changing DNA analysis technologies.