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If the patients main issue is appetite or significant visceral fat, Ipamorelin alone may not address the real problem

It inhibits the acetaldehyde dehydrogenase enzyme involved in ethanol metabolism, leading to higher plasma concentrations of acetaldehyde, which induces unpleasant side effects if a patient consumes alcohol while taking this medication, preventing further drinking

Although these patients have extremely low NSP activity, they do not exhibit obvious immunodeficiency symptoms, providing important theoretical support for the safety of DPP1 inhibition therapeutic strategies ( Research indicates that neutrophil function in PLS patients shows specific abnormalities, including: reduced chemotaxis, affecting neutrophil directed migration

Another important direction has been the study of the islet secretome, which provides opportunities for identifying novel biomarkers and potential therapeutic targets (Shapira et al., 2022)