Internal medicine journal
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Internal medicine journal · Feb 2023
ReviewNon-ischaemic Dilated Cardiomyopathy: Recognising the Genetic Links.
The landscape of genetically related cardiac disease continues to evolve. Heritable genetic variants can be a primary cause of familial or sporadic dilated cardiomyopathy (DCM). There is also increasing recognition that genetic variation is an important determinant of susceptibility to acquired causes of DCM. ⋯ Identifying patients who are most likely to benefit from genetic testing is paramount. The objective of this review is to highlight the importance of recognising genetic DCM, key genotype-phenotype correlations and the value of genetic testing in clinical management for both the individual and their family. This is likely to become more relevant as management strategies continue to be refined with genotype-specific recommendations and disease-modifying therapies.
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Internal medicine journal · Jan 2023
ReviewMethamphetamine-associated cardiomyopathy: an addiction medicine perspective.
Methamphetamine-associated cardiomyopathy (MaCM) is an increasingly recognised serious complication from methamphetamine (MA) use. It is characterised as the development of otherwise unexplained heart failure in the context of MA use. MaCM predominantly affects a young and vulnerable population with high morbidity and mortality. ⋯ Abstinence is difficult for most patients to achieve; all clinicians require an understanding of MaCM and how to educate patients on the risks of ongoing use. Where available, referral to addiction medicine specialists to assist with treatment of MUD is recommended. This review aims to: (i) explain the proposed pathologic mechanisms of MaCM; (ii) summarise recent recommendations of the screening and treatment of MaCM; and (iii) highlight the role of addiction medicine in the management of patient with MaCM.
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Internal medicine journal · Jan 2023
ReviewPulmonary Hypertension in Remote and Disadvantaged Population: Overcoming Unique Challenges for Improved Outcomes.
Pulmonary hypertension (PH) is a common and debilitating medical condition with high mortality. PH research has traditionally focused on pulmonary arterial hypertension and its management in expert PH centres. Other forms of PH such as PH associated with cardiac or respiratory disease are more common, less well-understood and associated with higher mortality. ⋯ We propose a simple diagnostic approach, ideally suited to regions where resource allocation is scarce, using clinical skills, echocardiography, and an escalation algorithm. The brief history, epidemiology and current literature on PH are summarised to inform the busy clinicians. We highlight two case examples from the Top End to illustrate the challenges and potential solutions.
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Internal medicine journal · Dec 2022
ReviewCurrent approaches to the diagnosis and management of amyloidosis.
Amyloidosis is a collection of diseases caused by the misfolding of proteins that aggregate into insoluble amyloid fibrils and deposit in tissues. While these fibrils may aggregate to form insignificant localised deposits, they can also accumulate in multiple organs to the extent that amyloidosis can be an immediately life-threatening disease, requiring urgent treatment. Recent advances in diagnostic techniques and therapies are dramatically changing the disease landscape and patient prognosis. ⋯ Technetium scintigraphy has made noninvasive diagnosis much easier, and ATTR is now recognised as the most common type of amyloidosis because of the increased identification of age-related ATTR. There are emerging ATTR treatments that slow disease progression, decrease patient hospitalisations and improve patient quality of life and survival. This review aims to update physicians on recent developments in amyloidosis diagnosis and management and to provide a diagnostic and treatment framework to improve the management of patients with all forms of amyloidosis.
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Internal medicine journal · Dec 2022
ReviewGuidance for transfusion management in patients receiving magrolimab therapy (anti-CD47 monoclonal antibody).
Magrolimab (Hu5F9-G4) is a first-in-class anti-CD47 IgG4 monoclonal antibody, with potential applications in several malignancies including myelodysplastic syndrome. CD47 blockade in malignancy has been shown to promote antitumour effects. However, the ubiquity of CD47 on red blood cells can result in interference in pretransfusion immunohaematology investigations and hinder timely provision of red blood cell units, with potential to mask clinically significant alloantibodies. ⋯ Antibody screening using an antihuman globulin reagent that does not detect human IgG4 subclass may distinguish magrolimab interference from an underlying alloantibody in patient plasma. Clear and consistent protocols for laboratories and close communication with clinicians are paramount to facilitate timely and safe transfusion support for patients receiving magrolimab therapy. As local transfusion laboratories gain experience with magrolimab, this will assist in our understanding and comfort in managing these patients.