Andersen's syndrome: Potassium‐sensitive periodic paralysis, ventricular ectopy, and dysmorphic features

@article{Tawil1994AndersensSP,
  title={Andersen's syndrome: Potassium‐sensitive periodic paralysis, ventricular ectopy, and dysmorphic features},
  author={Rabi Tawil and Louis J. Pt{\'a}{\vc}ek and Steven G. Pavlakis and Darryl C Devivo and Audrey S. Penn and Coşkun {\"O}zdemir and Robert C Griggs},
  journal={Annals of Neurology},
  year={1994},
  volume={35}
}
Andersen's syndrome is a clinically distinct form of potassium‐sensitive periodic paralysis associated with cardiac dysrhythmias. The subtle nature of the cardiac and dysmorphic features may delay the recognition of this syndrome and its potentially lethal cardiac dysrhythmias. The genetic defect in Andersen's syndrome is not genetically linked to other forms of potassium‐sensitive periodic paralysis and is probably distinct from the long QT syndrome locus. 
Hypokalemic periodic paralysis, facial dysmorphism and ventricular arrhythmia (clinical triad of Andersen-Tawil syndrome).
TLDR
A case of hypokalemic periodic paralysis with dysmorphic facial features and ventricular arrhythmia resembling Andersen-Tawil syndrome is reported.
Two Families of Andersen's Syndrome with Cardiac Arrhythmia, Periodic Paralysis, and KCNJ2 Gene Mutations
TLDR
Two families with genetically confirmed Andersen’s syndrome through clinical and electrophysiological findings are presented, and one of them had a novel mutation c.307G>A (Met307Ile).
KCNJ2 mutation in intractable ventricular arrhythmia with Andersen's syndrome
TLDR
A patient with intractable ventricular arrhythmia and periodic paralysis with heterozygous mutation in KCNJ2 gene, which encodes the cardiac Kir2.1 is reported, who was treated with nicorandil and cardiac pacing with favorable effect.
Andersen's syndrome: A distinct periodic paralysis
TLDR
It is concluded that Andersen's syndrome is a genetically unique channelopathy affecting both cardiac and skeletal membrane excitability, attacks of paralysis may be either hypokalemic or hyperkalemic, a prolonged QT interval is an integral feature of this syndrome, and a prolongedQT interval may be the only sign in an individual from an otherwise typical AS kindred.
Andersen-Tawil Syndrome Presenting with Complete Heart Block.
TLDR
The first case of advanced atrioventricular (AV) block in ATS is reported and clinical factors that may delay diagnosis are highlighted.
Cardiac surgery for a patient with Andersen-Tawil syndrome.
TLDR
A case of successful cardiac surgery in a patient with Andersen-Tawil syndrome, without using cardioplegia is presented.
Andersen syndrome: an association of periodic paralysis, cardiac arrhythmia and dysmorphic abnormalities.
TLDR
The first Brazilian patient presenting AS who also had obesity, obstructive sleep apnea (OSA) and daytime sleepiness is reported, and the R218W mutation in the index patient and her 6-year-old daughter is revealed.
[QTU pattern in a patient with the Anderson-Tawil syndrome].
The Andersen-Tawil syndrome (ATS) is an autosomal dominant or sporadic disorder characterised by periodic paralysis, cardiac arrhythmias and dysmorphic features. Mutations in KCNJ2, which encodes the
Anesthesia for the child with Andersen's Syndrome
  • D. Young
  • Medicine
    Paediatric anaesthesia
  • 2005
TLDR
The anesthetic implications and management of two anesthetics for the same patient, with Andersen’s Syndrome, is described, which may present with syncope and sudden death although many patients remain asymptomatic.
Andersen Cardiodysrhythmic Periodic Paralysis With KCNJ2 Mutations: A Novel Mutation in the Pore Selectivity Filter Residue
TLDR
The clinical and molecular features of 3 cases of Andersen-Tawil syndrome in 2 families are described and one of the mutations (G144D) was located in the pore selectivity filter residue (which is mutated recurrently) and was considered novel.
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