Search results for "Phenocopy"

showing 3 items of 3 documents

Search for a gene responsible for Floating-Harbor syndrome on chromosome 12q15q21.1.

2012

International audience; Floating-Harbor syndrome (FHS) is characterized by characteristic facial dysmorphism, short stature with delayed bone age, and expressive language delay. To date, the gene(s) responsible for FHS is (are) unknown and the diagnosis is only made on the basis of the clinical phenotype. The majority of cases appeared to be sporadic but rare cases following autosomal dominant inheritance have been reported. We identified a 4.7 Mb de novo 12q15-q21.1 microdeletion in a patient with FHS and intellectual deficiency. Pangenomic 244K array-CGH performed in a series of 12 patients with FHS failed to identify overlapping deletions. We hypothesized that FHS is caused by haploinsuf…

AdultHeart Septal Defects VentricularMaleCandidate geneFloating Harbor syndrome[SDV.GEN] Life Sciences [q-bio]/GeneticsHaploinsufficiencyBiologyBioinformaticsShort statureCraniofacial Abnormalities03 medical and health sciences12q15q21.1 microdeletion[SDV.BDD] Life Sciences [q-bio]/Development BiologyGeneticsmedicineHumansAbnormalities MultipleGenetic Predisposition to Disease[ SDV.BDD ] Life Sciences [q-bio]/Development BiologyChild[SDV.BDD]Life Sciences [q-bio]/Development BiologyGenetics (clinical)Growth Disorders030304 developmental biologySequence DeletionPhenocopyGenetics0303 health sciencesComparative Genomic Hybridization[SDV.GEN]Life Sciences [q-bio]/GeneticsChromosomes Human Pair 12Genetic heterogeneity030305 genetics & heredityChromosomeHigh-Throughput Nucleotide Sequencinghigh-throughput sequencingmedicine.disease3. Good healthPhenotypeFloating–Harbor syndromeChild PreschoolMutation (genetic algorithm)Femalemedicine.symptomHaploinsufficiency[ SDV.GEN ] Life Sciences [q-bio]/Genetics
researchProduct

Brugada phenocopy in diabetic ketoacidosis, the importance of the diagnostic approach

2020

Abstract Brugada phenocopies (BrP) are clinical entities that present with identical ECG patterns to those of true Brugada Syndrome (BrS) but are elicited by various other clinical circumstances. Our manuscript shows an interesting case of a type-1 Class A BrP in a young patient with diabetic ketoacidosis and hyperkalemia.

Phenocopycongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyHyperkalemiaDiabetic ketoacidosisbusiness.industry030204 cardiovascular system & hematologymedicine.disease03 medical and health sciences0302 clinical medicineBrugada phenocopy Brugada syndrome Diabetic ketoacidosis Differential diagnosis Hyperkalemia Treatment030225 pediatricsInternal medicinePediatrics Perinatology and Child HealthCardiologyMedicinecardiovascular diseasesmedicine.symptomCardiology and Cardiovascular MedicinebusinessBrugada syndrome
researchProduct

BRUGADA PATTERN IN HEROIN ADDICTION: SYNDROME OR PHENOCOPY?

2020

Brugada phenocopies (BrPs) are clinical entities that show an electrocardiogram (ECG) pattern similar to what is observed in Brugada syndrome (BrS). They are caused by different clinical conditions. We describe a case of BrP in a man that developed acute kidney failure secondary to rhabdomyolysis, after heroin addiction. His initial ECG showed Brugada type 1 pattern resolved after hemodialytic treatment. A provocative test with ajmaline, which resulted negative, was performed to confirm the diagnosis. BrPs can mimic a true BrS and a fast recognition of these clinical and electrocardiographic findings may avoid diagnostic mistakes thus preventing unnecessary or inaccurate treatmen

Settore MED/14 - NefrologiaBrugada Phenocopy heroin addiction rhabdomyolysis hyperkalemia.Settore MED/11 - Malattie Dell'Apparato Cardiovascolare
researchProduct