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Issue 150 Winter 2023

Endocrinologist > Winter 2023 > Hot topics


| Hot topics

A new review by Gibbons et al. showcases why multidisciplinary approaches are crucial in furthering our understanding of endocrine conditions. They highlight lymphangioleiomyomatosis (LAM), a rare chronic disease where the lungs of affected individuals (almost exclusively genetically female) will progressively develop large cysts that replace normal alveolar airspace. This results in an inevitable decline in lung function that, in some extreme circumstances, can result in a need for lung transplantation.

Despite recent advances in understanding the pathogenesis, there remains only one viable treatment option to delay the loss of function. This involves inhibition of mTORC1 (mechanistic target of rapamycin complex 1) with medications such as sirolimus, but this is not curative, only preventative.

The article provides the background of LAM; it links the disease and its progression to oestrogen, concisely describes the melanocytic markers and introduces the role of the immune environment in LAM disease progression. It then brings all the strands together and pushes forward to what we need to know in order to attempt to successfully treat this disease and give patients much better outcomes.

Ultimately, this review highlights why lateral and collaborative thinking are essential in tackling some of endocrinology’s biggest issues.

Read the full article in Endocrine-Related Cancer 30 e230102

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