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Parathyroid hormone resistance and B cell lymphopenia in propionic acidemia
T A Griffin1, R W Hostoffer, K Y Tserng
1Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Acta Paediatrica (Oslo, Norway : 1992)
|July 1, 1996
Summary
Propionic acidemia can cause severe hypocalcemia and B cell lymphopenia due to organic acid buildup affecting parathyroid hormone function and immune cell development. Treatment resolved these serious complications.
Area of Science:
- Biochemistry
- Immunology
- Pediatrics
Background:
- Propionic acidemia (PA) is a metabolic disorder caused by propionyl-CoA carboxylase (PCC) deficiency.
- The mechanisms behind hypocalcemia, recurrent infections, and hypogammaglobulinemia in PA decompensation are not well understood.