Nonketotic hyperglycinemia: novel mutation in the aminomethyl transferase gene. Case report

Arch Argent Pediatr. 2016 Jun 1;114(3):e142-6. doi: 10.5546/aap.2016.eng.e142. Epub 2016 Jun 1.
[Article in English, Spanish]

Abstract

Panton-Valentine leukocidin (PVL) is an exotoxin that is produced by many strains of Staphylococcus aureus, and an important virulence factor. A PVL-positive S. aureus infection leads to rapid and severe infections of soft tissue and necrotizing pneumonia in healthy adolescents, and has a high mortality. This case report included a 12-year-old male patient who admitted for fever, respiratory distress and hip pain and was identified with necrotizing pneumonia with septic pulmonary embolism, psoas abscess, cellulitis and osteomyelitis. The PVL positive methicillin-sensitive S. aureus (MSSA) was isolated in the patient blood culture.

La hiperglicinemia no cetósica es un raro trastorno metabólico autosómico recesivo hereditario causado por una deficiencia en el sistema enzimático de división de la glicina mitocondrial. Se desconoce la incidencia general de la hiperglicinemia no cetósica, aunque es mayor en ciertas poblaciones, como las del norte de Finlandia (1/12 000) y de la Columbia Británica (1/63 000). Se sabe que son tres los genes que causan hiper-glicinemia no cetósica: GLDC, AMT y GCSH. Las mutaciones en el gen AMT son responsables del 20% de los casos de hiperglicinemia no cetósica. En este artículo describimos una mutación novedosa del codón de terminación (c.565C>T, p.Q189*) del gen AMT en un niño de cuatro meses de vida con hiperglicinemia no cetósica.

Keywords: AMT gene mutation; Hiccups; Hypotonia; Infant; Seizure.

Publication types

  • Case Reports

MeSH terms

  • Aminomethyltransferase / genetics*
  • Humans
  • Hyperglycinemia, Nonketotic / genetics*
  • Infant
  • Male
  • Mutation*

Substances

  • Aminomethyltransferase