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A Rare Childhood Illness That Affects Hormones, Swallowing, and the Brain

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Allgrove syndrome with early neurodegeneration in a child A case report from Syria. DOI 10.1097MD.0000000000047238

Allgrove syndrome, also called “Triple A” syndrome, is a rare condition that affects only about 1 in a million people. It causes three main problems: no tears (alacrima), trouble swallowing (achalasia), and a serious hormone issue called adrenal insufficiency, which means the body can’t handle stress well.

This case report shares the story of a young girl from Syria who showed signs of the disease early in life. At just four years old, she had vomiting, weak muscles, difficulty swallowing, dry eyes, and darker skin patches. Tests showed her body was not making enough cortisol, a hormone needed for survival, especially during stress or illness.

She was diagnosed with Addison’s disease (a type of adrenal insufficiency) and achalasia after special X-ray tests. Doctors also found that her brain was affected—something called early neurodegeneration. She had seizures and brain scans showed shrinkage in parts of her brain.With medication and later surgery to help her eat better, her condition improved somewhat, but nerve and brain symptoms remained. This shows how quickly Allgrove syndrome can get worse, especially in children.Catching the disease early is important. If not treated, it can lead to life-threatening problems like adrenal crisis. Children with unusual symptoms like dry eyes, feeding problems, or fainting should be checked for hormone issues. Regular care from different specialists helps manage the symptoms and supports growth and development.

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