Phenotypes for disease #02145 (Danon;GSD2B (Danon disease (glycogen storage disease, type IIb (GSD-2B))), OMIM:300257)

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0000043057 - - - Unknown - - - - - Irene Vieitez 00056438
0000067591 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088085
0000067592 - - - Unknown - - - - - Johan den Dunnen 00088086
0000067593 - - - Unknown - - - - - Johan den Dunnen 00088087
0000067594 - - - Unknown - - - - - Johan den Dunnen 00088088
0000067595 - - - Unknown - - - - - Johan den Dunnen 00088089
0000067596 - - - Unknown - - - - - Johan den Dunnen 00088090
0000067597 - - - Unknown - - - - - Johan den Dunnen 00088091
0000067598 - - - Unknown - - - - - Johan den Dunnen 00088092
0000067599 see paper; ... - - Unknown - - - - - Johan den Dunnen 00088093
0000067600 see paper;... - - Unknown - - - - - Johan den Dunnen 00088094
0000067601 see paper; ... - - Unknown - - - - - Johan den Dunnen 00088095
0000067602 see paper; progressive exertional dyspnea, fatigue, abdominal discomfort, motor/mental development normal, general health good, physical function allowed sports involvement, throbbing precordium, systolic murmur left hemithorax, hepatomegaly, ...; mother died at 46y (dilated cardiomyopathy) - - Unknown 19y - - - - Johan den Dunnen 00088096
0000067603 see paper; 14y- admitted to hospital for recurrent episodes of presyncope and severe chest pain on exertion, ... - - Familial, X-linked - - 10y chest pain, palpitations on exertion - Johan den Dunnen 00088097
0000067604 see paper; 13y-unexplained elevation of serum creatinine kinase, proximal weakness, Gower sign, no exertional dyspnea, no cardiac murmur, no hepatomegaly, no muscle atrophy, no hypotonia, normal muscle tone, ... - - Isolated (sporadic) - - 12y incidentally detected high level liver enzymes (ALT, AST)/total bilirubin - Johan den Dunnen 00088098
0000067605 see paper; skeletal myopathy, mental retardation, ophthalmic manifestations, massive hypertrophic obstructive cardiomyopathy necessitating heart transplantation, ... - - Isolated (sporadic) 14y - <09y - - Johan den Dunnen 00088099
0000067606 see paper; 21y-admitted to hospital with exertional dyspnea, ... - - Unknown - - 14y Danon's disease - Johan den Dunnen 00088100
0000067607 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088101
0000067608 see paper; ... - - Unknown - - - - - Johan den Dunnen 00088102
0000067609 see paper;, ..., skeletal muscle weakness, high creatine kinase level, ... - - Isolated (sporadic) - - 12y gastrointestinal - Johan den Dunnen 00088103
0000067610 - - - Unknown - - - - - Johan den Dunnen 00088104
0000067611 - - - Familial, X-linked - - - - - Johan den Dunnen 00088105
0000067612 - - - Familial, X-linked - - - - - Johan den Dunnen 00088106
0000067613 - - - Familial, X-linked - - - - - Johan den Dunnen 00088107
0000067614 - - - Familial, X-linked - - - - - Johan den Dunnen 00088108
0000067615 - - - Familial, X-linked - - - - - Johan den Dunnen 00088109
0000067616 - - - Familial, X-linked - - - - - Johan den Dunnen 00088110
0000067617 - - - Familial, X-linked - - - - - Johan den Dunnen 00088111
0000067618 - - - Familial, X-linked - - - - - Johan den Dunnen 00088112
0000067619 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088113
0000067624 see paper; ... - - Unknown - - - - - Johan den Dunnen 00088151
0000067625 see paper; ..., easy fatigability, anorexia, abdominal pain, progressive diffuse muscle hypotrophy , Wolff-Parkinson-White (WPW) syndrome, heart failure, several syncopal episodes, elevated creatine kinase - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088152
0000067626 see paper; ..., 9y-scleral jaundice, abnormal hepatic laboratory tests, liver biopsy chronic hepatitis with normal serology, 18y-electrocardiography revealed WPW syndrome; 22y-exertion dyspnea, difficulty climbing stairs, several syncopal episodes; ... - - Familial, X-linked dominant - - 09y - - Johan den Dunnen 00088153
0000067627 see paper; ..., 12y-subclinical jaundice, viral hepatitis excluded (serology; 18y-easy fatigability after mild effort; 19y-ECG revealed bradycardia, WPW syndrome; 22y-very low aerobic resistance (tread-mill test); 27y-chest X-ray mild cardiomegaly; women have no raised CK - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088154
0000067628 see paper; ..., infancy mild hypotonia, global developmental delay, elevated serum CK levels, ... - - Familial, X-linked - - - - - Johan den Dunnen 00088155
0000067629 see paper; ..., presented with HCM as teenager, progressed to dilated cardiomyopathy/heart failure, skeletal myopathy, Wolff-Parkinson-White syndrome; teenage carrier sister has HCM, no skeletal myopathy/WPW, ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088156
0000067630 see paper; ..., presented with HCM, Wolff-Parkinson-White syndrome, skeletal myopathy as teenager; carrier mother developed DCM during 40s, ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088157
0000067631 see paper; ..., - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088158
0000067632 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088159
0000067633 see paper; ... - - Unknown - - - - - Johan den Dunnen 00088160
0000067634 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088161
0000067635 see paper; ..., severe skeletal muscular weakness, respiratory failure, history of two OHTs (1st severe HCM, 2nd allograft rejection, myopathy, ... - - Unknown - - 01y heart murmur - Johan den Dunnen 00088162
0000067636 see paper; ..., - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088163
0000067637 12y-HCM; 14y-acute episode heart failure, ECG concentric hypertrophy dilated left ventricle with moderate systolic dysfunction, neurological/EMG normal, increased levels serum creatine kinase (638 U/l); 15y orthotopic heart transplantation - - Isolated (sporadic) - 12y - - - Johan den Dunnen 00088164
0000067638 see paper; ... - - Familial, X-linked dominant - - - - - Johan den Dunnen 00088165
0000067639 severe hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, proximal muscle weakness, chronic painless diarrhea, muscle biopsy autophagic vacuolar myopathy, IHC LAMP2 absence - - Unknown - - - - - Johan den Dunnen 00088166
0000067640 limb-girdle muscle weakness, mild left ventricular diastolic dysfunction, sub-clinical neuropathy, muscle biopsy autophagic vacuolar myopathy, IHC LAMP2 smear - - Unknown - - - - - Johan den Dunnen 00088167
0000068360 glycogen storage disease, type IIb - - Unknown - - - - - Johan den Dunnen 00088956
0000068361 variable clinical presentations, including cardiomyopathy, skeletal muscle pathology, hepatopathy, abnormal mitochondria/LAMP2 immunohistochemistry - - Familial, autosomal dominant - - - - - Johan den Dunnen 00088957
0000068362 Danon disease, cone-rod dystrophy (CRD), visual acuity deteriorated progressively, color vision severely disturbed; ERG reduced photopic/scotopic responses, ... - - Familial, autosomal dominant - - - - - Johan den Dunnen 00088958
0000068363 - - - Unknown - - - - - Johan den Dunnen 00088959
0000068364 - - - Unknown - - - - - Johan den Dunnen 00088960
0000068365 - - - Unknown - - - - - Johan den Dunnen 00088961
0000068366 - - - Unknown - - - - - Johan den Dunnen 00088962
0000068367 - - - Unknown - - - - - Johan den Dunnen 00088963
0000068368 - - - Unknown - - - - - Johan den Dunnen 00088964
0000068369 see paper; cardiomyopathy, mental retardation, myopathy, vacuolar myopathy without acid a-glucosidase deficiency, diffuse chorio-capillary ocular atrophy, successful heart transplantation, ... - - Unknown - - - - - Johan den Dunnen 00088965
0000068370 - - - Unknown - - - - - Johan den Dunnen 00088966
0000068373 see paper; ... - - Isolated (sporadic) - - - - - Johan den Dunnen 00088969
0000068374 see paper; ... - - Familial, X-linked - - - - - Johan den Dunnen 00088970
0000068386 Danon disease - - Unknown - - - - - Peikuan Cong 00088982
0000068387 Danon disease - - Unknown - - - - - Peikuan Cong 00088983
0000068388 Danon disease, hyperCKemia, hypertrophic cardiomyopathy, no muscle weakness, slight mental impairment; muscle biopsy autophagic vacuoles with sarcolemmal features and glycogen storage - - Familial - - - - - Johan den Dunnen 00088984
0000068389 Danon disease - - Unknown - - - - - Peikuan Cong 00088985
0000068390 Danon disease, see paper; progressive hypertrophic cardiomyopathy, death at 16y (before planned heart transplantation); mother developed severe dilated cardiomyopathy, 46y died of complications - - Familial - - 02y06m mild left ventricular hypertrophy, mild myopathy - Johan den Dunnen 00088986
0000068391 Danon disease - - Unknown - - - - - Peikuan Cong 00088987
0000068392 Danon disease - - Unknown - - - - - Peikuan Cong 00088988
0000068393 Danon disease - - Familial - - - - - Peikuan Cong 00088989
0000068396 Danon disease - - Unknown - - - - - Peikuan Cong 00088992
0000068397 Danon disease; cardiomyopathy, hypertrophic (HCM) - - Familial - - - - - Peikuan Cong 00088993
0000068402 Danon disease - - Familial - - - - - Peikuan Cong 00088998
0000068403 Danon disease - - Unknown - - - - - Peikuan Cong 00088999
0000068404 Danon disease - - Unknown - - - - - Peikuan Cong 00089000
0000068406 Danon disease - - Unknown - - - - - Peikuan Cong 00089002
0000068409 Danon disease - - Familial - - - - - Peikuan Cong 00089005
0000068411 Danon disease - - Unknown - - - - - Peikuan Cong 00089007
0000068412 Danon disease - - Familial - - - - - Peikuan Cong 00089008
0000068413 Danon disease - - Familial - - - - - Peikuan Cong 00089009
0000068414 Danon disease - - Unknown - - - - - Peikuan Cong 00089010
0000068415 Danon disease - - Unknown - - - - - Peikuan Cong 00089011
0000068423 see paper; hypertrophic cardiomyopathy, ... - - Familial, X-linked - - - - - Johan den Dunnen 00089021
0000159677 - Danon disease Danon disease Familial, X-linked - - - - - Maria Iascone 00211195
0000334617 see paper; ..., visual impairment secondary to retinitis pigmentosa; uneventful spontaneous pregnancy/delivery; <14m-normal development; 18m-heart murmur, ECG asymptomatic WPW Danon disease Danon disease Familial, X-linked dominant 03y - - - - Johan den Dunnen 00445381
0000334619 see paper; ..., full-time job as storekeeper; 13y-heart palpitations, ECG Wolf–Parkinson–White pattern; 18y-increased echogenicity, borderline increase antero-septal wall thickness; 25y-progressive visual impairment with increased severity dark hours, ERG revealed rods/cones dystrophy; no psychiatric/gastrointestinal/pulmonary complaints; 27y-pacemaker; 42y-atrial fibrillation Danon disease Danon disease Unknown 42y - - - - Johan den Dunnen 00445382
0000343456 The ECG revealed premature atrial beats, premature ventricular beats, and ST-T changes. Echocardiography showed marked thickening of the septum and posterior wall of the left ventricle,reduced wall motion and diastolic dysfunction. Danon disease Danon disease Familial, X-linked dominant 14y 14y 14y precordial discomfort - Chunli Wang 00454846
0000343508 Cardiac enzymes were abnormal, ECG showed incomplete right bundle branch block, high voltage in the left ventricle, ST-T changes in extensive leads, and cardiac ultrasound showed a slight thickening of the left ventricle and septum, and uncoordinated ventricular wall motion. myocarditis Danon disease Familial, X-linked >07y >07y >07y >7y - Chunli Wang 00454847
0000343510 shortness of breath Danon disease Danon disease Familial, X-linked 00y04m 00y04m 00y04m - - Chunli Wang 00454848
0000343511 heart murmur Danon disease Danon disease Familial, X-linked >10y >10y >10y >10y - Chunli Wang 00454849
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