Email:
riteekabhimani@gmail.com
Address : Flat no. PH1, 7th floor, Al medina center, D street,
Upper Gizri, Karachi, Pakistan
Institute : Shaheed Mohtarma Benazir Bhutto Medical College,
Karachi, Pakistan
ORCID: 0009-0003-5775-0777
Article type: Letter to the Editor
Word Count: 289
Key Words: Hemoglobinopathies, Sickle cell anemia, Hemolytic
Anemia
Tables: None
Figures: None
Conflict of interest : None
Declaration: None
Acknowledgement: None
To The Editor ,
We have read the article “Pulmonary hypertension screening in Children
with sickle cell disease” by Kok Hoe Chan et al.1 with great interest
and the work done by the authors is highly commendable. The article’s
concluding point is that in children with SCD, PHT symptoms are
inconsistent with ECHO, NT-proBNP, or BNP outcomes. Children using
hydroxyurea have a low incidence of PHT based on TRV, hence screening
may not be recommended for this demographic. However, it would be an
honor to draw your attention to a few concerns that we possess.
Firstly, the author refrained from mentioning the impact of certain
genes on pulmonary hypertension in sickle cell disease as mentioned in a
study conducted in 2016 2 which shows a decreased expression of the
MAPK8 gene contributes to the pathogenic progression of precapillary
pulmonary hypertension in SCD patients, and single nucleotide
polymorphisms in ADRB1, ACVRL1, and BMP6 associated with PHT in patients
with SCD.3
Second, the essential elements of the pathophysiology underlying
vaso-oclusive crises and pulmonary hypertension in SCD patients are
hemolysis and nitric oxide deficiency, both of which are significant in
disrupting endothelium, endothelial proliferation, and platelet
activation, they have not been highlighted in this
study.3
Moreover, considering the fact that the author has highlighted the
crucial PHT biomarkers NT-proBNP, BNP, and ECHO, a study conducted in
2020 reveals additional potential biomarkers, such as Factor-15
(GDF-15), a cytokine overexpressed in hypoxic conditions including PHT,
and superoxide dismutase, a mitochondrial enzyme also upregulated in
PHT, can also be utilized for diagnosing PHT in patients with
SCD.4
Lastly, the administration of vasodilators, oxygen therapy, iron
chelation, and stem cell transplant are among the treatments for PHT
that the author could have discussed for a better understanding of the
topic.5