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First presentation of LPIN1 acute rhabdomyolysis in adolescence and adulthood.
Pizzamiglio, Chiara; Lahiri, Nayana; Nirmalananthan, Niranjanan; Sood, Bhrigu; Somalanka, Subash; Ostrowski, Philip; Phadke, Rahul; O'Donovan, Dominic Gerard; Muntoni, Francesco; Quinlivan, Rosaline.
Afiliação
  • Pizzamiglio C; MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom. Electronic address: c.pizzamiglio@ucl.ac.uk.
  • Lahiri N; Clinical Genetics Department, St George's University Hospitals NHS Foundation Trust, London, United Kingdom.
  • Nirmalananthan N; Departments of Neurology and Neuroradiology, Atkinson Morley Regional Neurosciences Centre, St George's Hospital, London, United Kingdom.
  • Sood B; South West Thames Renal and Transplantation Unit and South West Thames Institute for Renal Research, Saint Helier Hospital, Carshalton, Surrey, United Kingdom.
  • Somalanka S; South West Thames Renal and Transplantation Unit and South West Thames Institute for Renal Research, Saint Helier Hospital, Carshalton, Surrey, United Kingdom.
  • Ostrowski P; South West Thames Regional Genetics Service, St George's University NHS Foundation Trust, London, United Kingdom.
  • Phadke R; Division of Neuropathology, Dubowitz Neuromuscular Centre, UCL Great Ormond Street Hospital for Children, United Kingdom; Division of Neuropathology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
  • O'Donovan DG; Neuropathology, Department of Histopathology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
  • Muntoni F; Paediatric Neurology, Dubowitz Neuromuscular Centre, UCL Institute of Child Health and Great Ormond Street Hospital for Children, London, United Kingdom.
  • Quinlivan R; MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Neuromuscul Disord ; 30(7): 566-571, 2020 07.
Article em En | MEDLINE | ID: mdl-32522502
LPIN1 mutations are a known common cause of autosomal recessive, recurrent and life-threatening acute rhabdomyolysis of childhood-onset. The first episode of rhabdomyolysis usually happens in nearly all cases before the age of 5 and death is observed in 1/3 of patients. Here we present two cases of acute rhabdomyolysis with a milder phenotype caused by LPIN1 mutation presenting in adolescence (11 years old) and adulthood (40 years old) after Parvovirus infection and metabolic stress, respectively. In our opinion, the mutation types, epigenetic factors, the environment exposition to triggers or the existence of proteins with a similar structure of LPIN1, may have a role in modulating the onset of rhabdomyolysis. LPIN1 should be included on a panel of genes analysed in the investigation of adult individuals with rhabdomyolysis. Metabolic and viral stressors should be included in the list of possible rhabdomyolysis precipitant.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Rabdomiólise / Fosfatidato Fosfatase Idioma: En Ano de publicação: 2020 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Rabdomiólise / Fosfatidato Fosfatase Idioma: En Ano de publicação: 2020 Tipo de documento: Article