Literature DB >> 16417848

Multiple cardiac rhabdomyomas as a sole symptom of tuberous sclerosis complex: case report with molecular confirmation.

Sergiusz Jóźwiak1, Dorota Domańska-Pakieła, David J Kwiatkowski, Katarzyna Kotulska.   

Abstract

We report a child in whom multiple cardiac rhabdomyomas were identified on routine fetal ultrasonography. Molecular genetic studies identified the TSC2 gene missense mutation (E36; 4672 G>A, 1558 E>K TSC2). Both general and neurodevelopment of the patient have been normal. When last examined at age 6 years, he had no skin manifestations of tuberous sclerosis complex. Computed tomography of the brain revealed two periventricular calcifications consistent with the molecular diagnosis. This is the first report of molecularly confirmed tuberous sclerosis complex in a child with multiple cardiac rhabdomyomas and no other clinical manifestations of the disease. We propose that all cases of multiple cardiac rhabdomyomas in an infant be given a tentative diagnosis of tuberous sclerosis complex.

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Year:  2005        PMID: 16417848     DOI: 10.1177/08830738050200121101

Source DB:  PubMed          Journal:  J Child Neurol        ISSN: 0883-0738            Impact factor:   1.987


  3 in total

1.  Tuberous sclerosis complex; single center experience.

Authors:  İlknur Erol; Tülin Savaş; Sevda Şekerci; Nalan Yazıcı; Ayşe Erbay; Şenay Demir; Semra Saygı; Özlem Alkan
Journal:  Turk Pediatri Ars       Date:  2015-03-01

2.  Tuberous sclerosis in a term newborn with opisthotonus - The value of ultrasound.

Authors:  C Poggiani; A Laiolo; M Bellini; P Cavalli
Journal:  J Ultrasound       Date:  2007-12-03

3.  Multiple cardiac rhabdomyomas in tuberous sclerosis complex: case report and review of the literature.

Authors:  Paula Frudit; Bruno Kusznir Vitturi; Flavia Cristina Navarro; Ivan Rondelli; Geanete Pozzan
Journal:  Autops Case Rep       Date:  2019-09-30
  3 in total

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