Outcome, Clinical Profile and Risk Factors for Mortality of Neonates Necessitating Mechanical Ventilation

Background: Assisted ventilation has turn out to be an essential part of the neonatal intensive care unit (NICU). It is one of the main methods of support in the ICU and undoubtedly influences the survival of sick newborns. Aims: 1. To investigate common indications for mechanical ventilation in newborns 2. To investigate factors influencing the outcome. Method: It is a descriptive study of 60 infants admitted to the Department of Pediatric Medicine in the ICU over a one-year period in the department of Paediatrics, Abbasi Shaheed Hospital. The information was gathered and analysed in a pre-designed format. Results: Of a total of 60 infants, 46 survived, 14 died, and one infant was discharged despite medical advice. 36 children were born vaginally, 20 were born via LSCS, and 4 via assisted delivery. Postnatal asphyxia was the most common ventilation indication in full-term newborns, while HMD was present in preterm infants. The best results were obtained in ventilated infants with MAS, with 100% survival, followed by apnoea in premature infants, perinatal asphyxia, and HMD. Pulmonary haemorrhage (48.3%) was the most common complication among deceased infants, followed by sepsis (28.3%) and shock (23.4%) with a significant p <0.05. There were no complications in 76.7% of the surviving infants. Conclusions: Among the many widely available variables studied in this study, maximum and mean peak inspiratory pressure (PIP or (PEEP), maximum respiratory rate, maximum mean airway pressure (MAP) and average ventilation demand was much greater among non-survivals in comparison to the survivors. Bicarbonate, PH and excess base have been found to be important determinants of mortality in ventilated newborns. Keywords: Indications, mechanical ventilation and Results

Anti-glomerular basement membrane disease in children: a brief overview

Anti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Evidence of anti-GBM antibodies in serum or histologically are required for diagnosis. Treatment in children is based on very limited adult data and often involves the use of acute apheresis to rapidly remove circulating factors coupled with intensive immunosuppression such as cyclophosphamide and intravenous corticosteroids. There is also an emerging role for the use of biologic agents such as B cell depletion. The evidence base in children with anti-GBM disease is extremely limited. Multi-centre international collaboration is required to provide insight into this disease, better describe its prognosis and work towards improving outcomes. This review article summarises the key features of this disease in children, highlights treatment options and considers areas of unmet need.

P20 Scleritis as initial presenting feature in ANCA-associated vasculitis

Case report - Introduction ANCA-associated vasculitis (AAV) encompasses the clinical entities of GPA, MPA, renal-limited vasculitis and eGPA. Even though well recognised and described in the medical literature, ocular manifestations in AAV are relatively uncommon (&lt;20%) and may precede, present concomitantly with, or follow systemic manifestations. Our patient developed scleritis as the first manifestation of AAV and presented to the ophthalmology department. Within a few days, he developed systemic symptoms and subsequently severe and potentially life-threatening pulmonary haemorrhage. With collaborative working, he received appropriate treatments and made a good recovery. Case report - Case description Our patient is a 36-year-old Indian gentleman, who presented to Broomfield Hospital ophthalmology department in February 2021 with a 1-week history of pain and redness involving the left eye. Diagnosis of anterior scleritis was made and he received dexamethasone 0.1% eye drops and later switched to prednisolone 60mg/day. Investigations are shown below (Table 1). Diagnosis of AAV was made and he came under the care of the rheumatology. By this time, he noticed fleeting but severe arthralgia. He received three pulses of I.V. methyl prednisolone and received first dose of 1000mg of rituximab and one pulse of IV cyclophosphamide. His haemoglobin dropped with reduced oxygen saturation of 88% on air. Repeat CT chest showed extensive pulmonary haemorrhage and he was admitted to ITU. He did not need intubation and was transferred to University College London Hospital. Following five plasma exchanges and high-dose prednisolone, CRP fell from 113 to 6.6 and saturation improved to 98% on air. He completed four rituximab infusions. By June 2021 his chest X-ray returned to normal. 1. Table of Investigations Case report - Discussion This patient’s story highlights multiple clinical aspects of AAV. Published literature states that ocular manifestations as “initial” presentation of AAV are very uncommon (about 6%). The ophthalmologist requested the correct investigations including ANCA which helped to establish the diagnosis. Within a few weeks, the patient went on to develop other systemic manifestations which necessitated stepping-up the immune therapy. Rituximab was chosen for remission induction as it is now established as an alternative to cyclophosphamide. We discussed the case at virtual MDT of ENRAD (Eastern Network for Rare Autoimmune Disease) and got swift approval to use rituximab. Unfortunately, his clinical course was complicated with development of pulmonary haemorrhage which is potentially life-threatening. The PEXIVAS trial (Walsh et al NEJM 382; 622-31 (2020)) compared groups randomised to plasma exchange or no plasma exchange in addition to corticosteroids and either rituximab or cyclophosphamide. Outcomes were not different between the groups. However, Kronbichler et al (Nephrol Dial Transplantation 36; 227-31 [2021]) have argued that there was a trend towards better outcomes in a subgroup with alveolar haemorrhage and that plasma exchange may still have a role in such patients. Following five cycles of plasma exchange our patient made an excellent recovery from pulmonary haemorrhage which is very rewarding. Case report - Key learning points Scleritis is an uncommon presenting feature of AAV and should prompt the physician to look for systemic symptoms and check for ANCA serology. To recognise pulmonary haemorrhage as a potential life-threatening manifestation in a patient with AAV who drops haemoglobin. Despite lack of strong clinical trial evidence, plasma exchange can be a very useful therapeutic tool. Early recognition and initiation of immune therapy is crucial to induce remission. Collaborative working with clinicians from different medical specialities is the key for improved patient outcomes.

Diffuse alveolar haemorrhage and myocardial infarction: life-threatening effects from self-injected hyaluronic acid dermal filler

Hyaluronic acid (HA) is a widely used dermal filler for soft tissue augmentation. We described a case of a 38-year-old transwoman who presented with sudden onset of severe respiratory distress following self-injection of HA dermal filler. She developed multiple episodes of pulmonary haemorrhage, and her chest X-ray showed diffuse ground-glass opacities consistent with diffuse alveolar haemorrhage (DAH). There were no relevant drugs or past medical histories. Anti-nuclear antibodies and rheumatoid factor were negative. Initially, the pulmonary haemorrhage episodes and ventilation requirement improved with systemic steroid, however she subsequently developed acute myocardial infarction with progressive clinical deterioration leading to death. To the best of our knowledge, this is the first HA-related DAH with myocardial infarction reported with a fatal outcome. This case highlights the importance of awareness and the necessity of having a high suspicion of DAH in patients with history of illicit HA dermal filler use.

Good pasture’s syndrome diagnosed in postpartum period: acute kidney injury severe enough to warrant renal transplant

Objective: Goodpasture's syndrome (GPS) is the association of pulmonary haemorrhage with acute kidney injury (AKI) resulting from injury by auto-antibodies. Its de novo occurrence in pregnancy is extremely rare with only few cases reported. High risk of mortality and lack of consensus in treatment warrants its reporting. Case report: A 24 year old primigravida, with no history suggestive of renal disease, presented to us in her third trimester with anuria. She was initially managed as sepsis or preeclampsia related AKI. However, even after delivery there was no improvement in kidney function with hemodialysis and she developed hemoptysis. Renal biopsy made a diagnosis of Anti-Glomerular Basement Membrane disease. With careful multi-disciplinary treatment, she delivered a live born baby and was discharged under stable condition on hemodialysis, currently awaiting a kidney transplant. Conclusion: This case highlights that the current management for GPS should be revised to improve the outcome of AKI. Also, it determines how important it is for obstetricians to consider whether a pregnancy should be terminated to improve the outcome of AKI in pregnant patients with GPS

Haemoptysis: just another case of endocarditis? A case report

Background Pulmonary arteriovenous malformations (PAVM) are rare, and most cases are congenital. They require prompt recognition and management particularly in patients presenting with hypoxia and haemoptysis. We describe a unique case of recurrent endocarditis causing pulmonary artery aneurysms (PAAs) and formation of PAVM. Case summary A 60-year-old woman presented with dyspnoea, haemoptysis, and severe hypoxia. Her background was significant for previous pacemaker lead infection, refractory heart failure secondary to severe tricuspid valve distortion by her pacemaker lead, tricuspid and mitral valve replacements complicated by recurrent endocarditis over several years. Two years prior to her current presentation computed tomography (CT) scanning revealed new small PAAs thought possibly to be mycotic in origin. After her current presentation, prompt high-resolution CT scanning of her chest with contrast revealed significant pulmonary haemorrhage and new clusters of PAVM. Urgent pulmonary angiography confirmed PAVM and was successfully treated with coil embolization. Her dyspnoea, pulmonary haemorrhage, and hypoxia resolved. Discussion Acquired causes account for a very small percentage of PAVM and the mechanism of their development is unknown. As she had recurrent right-sided endocarditis and her PAAs developed following this, with new PAVM developing 2 years later; we hypothesize that they were causally related. We believe this is the first case of recurrent left- and right-sided endocarditis leading to formation of PAAs and development of PAVM presenting with significant hypoxia and haemoptysis requiring prompt intervention.

Diagnosis and endovascular management of pulmonary arteriovenous malformations

Pulmonary arteriovenous malformations (PAVM) are abnormal communication of a branch of the pulmonary artery and pulmonary vein circumventing the intervening pulmonary capillaries. This results in a right-to-left (R-L) shunt and its related manifestations, which include hampered gas exchange leading to hypoxaemia, dyspnoea, paradoxical emboli leading to stroke, cerebral abscess, myocardial infarction and pulmonary haemorrhage due to rupture of the PAVM. Endovascular transcatheter embolization of the feeding vessels with coils or occlusion devices is the current standard care of treatment and preferred treatment modality. The articles aim to provide insights into the current trends in diagnosis, the current recommendations, approach and management options for patients with PAVM.

Leptospirosis with pulmonary haemorrhage and multiple organ failure: a case report and literature review

Pulmonary haemorrhage is an important complication of leptospirosis. We herein report an uncommon case of severe pulmonary haemorrhage and multiple organ failure caused by leptospirosis in a 49-year-old man who was previously healthy. He was a farm worker who was admitted to the hospital because of haemoptysis. He had worked in a paddy field 4 days prior to admission. Chest computed tomography revealed pulmonary haemorrhage, which rapidly deteriorated into haemorrhagic shock and multiple organ failure. Based on the patient’s possible history of contact with contaminated water and the DNA sequence of Leptospira detected in his bronchoalveolar lavage fluid, the patient was diagnosed with pulmonary haemorrhagic leptospirosis. Despite the administration of a fluid bolus, norepinephrine, broad-spectrum antibiotics, and haemostatics, and even with administration of a blood transfusion and extracorporeal life support, the pulmonary haemorrhage could not be controlled effectively. The patient eventually died of haemorrhagic shock. Leptospirosis can be a life-threatening disease despite aggressive treatment, even with extracorporeal life support. Next-generation sequencing can provide important diagnostic clues for patients with atypical leptospirotic symptoms.