High morbidity and mortality in children with untreated congenital deficiency of leptin or its receptor
Leptin
[SDV]Life Sciences [q-bio]
morbidity
age-related changes
mortality
3. Good health
Obesity, Morbid
consanguinity
Cross-Sectional Studies
leptin-signaling deficiency
body growth
Report
oxidative stress
Humans
Morbidity
Child
metabolism
monogenic obesity
Retrospective Studies
DOI:
10.1016/j.xcrm.2023.101187
Publication Date:
2023-09-01T14:27:16Z
AUTHORS (17)
ABSTRACT
The long-term clinical outcomes of severe obesity due to leptin signaling deficiency are unknown. We carry out a retrospective cross-sectional investigation large cohort children with (LEP), LEP receptor (LEPR), or melanocortin 4 (MC4R) (n = 145) evaluate the progression disease. affected individuals undergo physical, clinical, and metabolic evaluations. report very high mortality in (26%) LEPR (9%), mainly pulmonary gastrointestinal infections. In addition, 40% surviving experience life-threatening episodes lung Although precision drugs currently available for deficiencies, as yet, they not accessible Pakistan. An appreciation impact on morbidity early mortality, educational attainment, attendant stigmatization should spur efforts deliver life-saving these matter urgency.
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CITATIONS (14)
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