MetaADEDB: ADE

MetaADEDB 2.0 @ LMMD

Glycogen Storage Disease Type I

(UMLS:C0017920)

Definition:: An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
UMLS ID:: C0017920
MeSH ID:: D005953

Classification 1:

Name:

Congenital, Hereditary, and Neonatal Diseases and Abnormalities
MeSH Tree Number(s):: C16.320.565.202.449.448

Classification 2:

Name:

Nutritional and Metabolic Diseases
MeSH Tree Number(s):: C18.452.648.202.449.448

Synonym(s)

Glycogen Storage Disease Type I

Deficiency of glucose-6-phosphatase

GLUCOSE 6 PHOSPHATASE DEFICIENCY

GLYCOGENOSIS, HEPATORENAL

GLYCOGENOSIS, TYPE I

GSD I

GSD type I

Glucose 6 Phosphatase Deficiency

Glucose-6-phosphatase deficiency

10.

Glycogen storage disease type I

11.

Glycogen storage disease type Ia

12.

Glycogen storage disease, type 1

13.

Glycogen storage disease, type I

14.

HEPATORENAL GLYCOGENOSIS

15.

Hepatorenal glycogen storage disease

16.

Hepatorenal glycogenosis

17.

Liver glycogen disease

18.

VON GIERKE DISEASE

19.

Van Creveld-von Gierke

Associated Drug(s)

	Name	Number of Reports	Data Source
1	Deferasirox	FAERS: 1	US FAERS
2	Diclofenac	FAERS: 1	US FAERS
3	Nelfinavir		OFFSIDES
4	Tenofovir		OFFSIDES
5	Zidovudine		OFFSIDES
6	atovaquone, proguanil drug combination		OFFSIDES

Similar ADE(s)

	Name	Semantic Similarity
1	Glycogen Storage Disease Type V	49.2%
2	Glycogen storage disease type II	29.5%
3	Amino Acid Metabolism, Inborn Errors	28.5%
4	Metal Metabolism, Inborn Errors	28.5%
5	Progeria	28.5%
6	Lysosomal Storage Diseases	28.5%
7	Peroxisomal Disorders	28.5%
8	Galactosemias	28.3%
9	Lactose Intolerance	28.2%
10	Brain Diseases, Metabolic, Inborn	23.1%
11	Leigh Disease	22.5%
12	Cytochrome-c Oxidase Deficiency	20.3%
13	Mucopolysaccharidosis III	20%
14	Smith-Lemli-Opitz Syndrome	19.8%
15	Urea Cycle Disorders, Inborn	18.6%
16	Tyrosinemias	18.6%
17	Werner Syndrome	17.4%
18	Mucopolysaccharidosis II	16.9%
19	Alkaptonuria	16.8%
20	Hypophosphatasia	16.8%
21	Propionic acidemia	16.8%
22	Dihydropyrimidine Dehydrogenase Deficiency	16.8%
23	Cystinosis	16.8%
24	Pseudohypoparathyroidism	16.7%
25	Hyperphosphaturia	16.6%
26	Hypolipoproteinemias	16.3%
27	Hyperhomocysteinemia	15.7%
28	Hyperlipidemia, Familial Combined	15.3%
29	Wasting Syndrome	15.1%
30	Menkes Kinky Hair Syndrome	14.9%
31	Acid-Base Imbalance	14.8%
32	Calcium Metabolism Disorders	14.8%
33	Iron Metabolism Disorders	14.8%
34	Phosphorus Metabolism Disorders	14.8%
35	Water-Electrolyte Imbalance	14.8%
36	Lipid Metabolism Disorders	14.8%
37	Mitochondrial Diseases	14.8%
38	Glucose Metabolism Disorders	14.8%
39	Hyperlipoproteinemia Type III	14.8%
40	Homocystinuria	14.2%
41	Carbamoyl-Phosphate Synthase I Deficiency Disease	13.6%
42	Hepatolenticular Degeneration	13.6%
43	Hyperlipoproteinemia Type IV	13.5%
44	Hyperlipoproteinemia Type V	13.5%
45	Zellweger Syndrome	13.3%
46	Albinism	13.1%
47	Refsum Disease	13%
48	Adrenoleukodystrophy	12.9%
49	Amyloid Neuropathies, Familial	12.4%
50	Hypercalcemia	12.2%
51	Hypocalcemia	12.2%
52	Milk-Alkali Syndrome	12.2%
53	Neuronal Ceroid-Lipofuscinoses	12%
54	Ichthyosis, X-Linked	11.8%
55	Neoplastic Syndromes, Hereditary	11.7%
56	Renal tubular acidosis	11.7%
57	Familial Periodic Paralysis	11.5%
58	Osteopenia	11.3%
59	Renal Aminoacidurias	11.2%
60	Fanconi Syndrome	11.2%
61	Pseudohypoaldosteronism	11.2%
62	Liddle Syndrome	11.2%
63	Brain Diseases, Metabolic	11.2%
64	Multiple Epiphyseal Dysplasia	11.2%
65	Osteochondrodysplasias	11.2%
66	Pelger-Huet Anomaly	11.2%
67	Myasthenic Syndromes, Congenital	11.2%
68	Hypoalphalipoproteinemias	10.8%
69	CHARGE Syndrome	10.6%
70	Gaucher Disease	10.5%
71	Antley-Bixler Syndrome Phenotype	10.3%
72	Cystic Fibrosis	10.3%
73	Cockayne Syndrome	10.2%
74	Fabry Disease	10%
75	Gout	9.9%
76	Lipodystrophy	9.8%
77	Deformity	9.8%
78	Nutrition Disorders	9.8%
79	Dwarfism	9.3%
80	Familial Hypophosphatemic Rickets	9.2%
81	Glycosuria, Renal	9.1%
82	Nail-Patella Syndrome	8.9%
83	Muscular Dystrophy	8.9%
84	Osteogenesis Imperfecta	8.9%
85	Sjogren-Larsson Syndrome	8.8%
86	Aicardi's syndrome	8.7%
87	Rickets	8.5%
88	Porphyrias, Hepatic	8.3%
89	MELAS Syndrome	8.3%
90	Beckwith-Wiedemann Syndrome	8.2%
91	Wolf-Hirschhorn Syndrome	8.2%
92	Anemia, Sickle Cell	8.1%
93	Bloom Syndrome	8.1%
94	Thalassemia	8.1%
95	Muscular Dystrophy, Duchenne	8.1%
96	Acidosis	8.1%
97	Alkalosis	8.1%
98	Amyloidosis	8.1%
99	Calcinosis	8.1%
100	Fanconi Anemia	8.1%
101	Hyperglycemia	8.1%
102	Hyperinsulinism	8.1%
103	Hyperkalemia	8.1%
104	Hypernatremia	8.1%
105	Hypoglycemia	8.1%
106	Hypokalemia	8.1%
107	Hyponatremia	8.1%
108	Familial Mediterranean Fever	8.1%
109	Xanthomatosis	8.1%
110	Hypophosphatemia	8.1%
111	Metabolic acidosis	8.1%
112	Dyslipidemias	8.1%
113	Iron Overload	8.1%
114	Xanthoma	8.1%
115	Niemann-Pick Disease, Type C	8%
116	Canavan Disease	7.9%
117	Alexander Disease	7.9%
118	Ataxia Telangiectasia	7.8%
119	Tay-Sachs Disease	7.8%
120	Autoimmune Lymphoproliferative Syndrome	7.5%
121	Hypokalemic periodic paralysis	7.5%
122	Congenital Hyperinsulinism	7.5%
123	Cystinuria	7.4%
124	Fetal Diseases	7.4%
125	Angioedemas, Hereditary	7.3%
126	Smith-Magenis syndrome	6.9%
127	Fragile X Syndrome	6.9%
128	Cri-du-Chat Syndrome	6.8%
129	Down Syndrome	6.8%
130	Trisomy 21	6.8%
131	Prader-Willi Syndrome	6.5%
132	Marfan Syndrome	6.5%
133	Arthritis, Gouty	6.4%
134	Diabetes Mellitus	6.4%
135	Dehydration	6.2%
136	Lipomatosis	6.2%
137	Water Intoxication	6.2%
138	Wiskott-Aldrich Syndrome	6.1%
139	Achlorhydria	6.1%
140	Osteoporosis	6.1%
141	Bone Demineralization, Pathologic	6.1%
142	Post-Traumatic Osteoporosis	6.1%
143	Celiac Disease	6%
144	Sprue, Tropical	6%
145	Steatorrhea	6%
146	Sprue	6%
147	Optic Atrophy, Hereditary, Leber	5.9%
148	Kallmann Syndrome	5.9%
149	Ectodermal Dysplasia	5.9%
150	Aplasia Cutis Congenita	5.9%
151	Xeroderma Pigmentosum	5.8%
152	Rett Syndrome	5.8%
153	Alstrom Syndrome	5.7%
154	Kartagener Syndrome	5.7%
155	Holoprosencephaly	5.6%
156	Mitochondrial Encephalomyopathies	5.6%
157	Friedreich Ataxia	5.5%
158	Myotonic Dystrophy	5.4%
159	Polycystic Kidney Diseases	5.4%
160	Williams Syndrome	5.4%
161	Primary Ciliary Dyskinesia	5.2%
162	Osteomalacia	5.1%
163	Rubinstein-Taybi Syndrome	5.1%
164	Cutis Laxa	5%
165	Mitochondrial Myopathies	5%
166	Abdominal Cramps	4.9%
167	Abnormalities, Drug-Induced	4.9%
168	Amniotic Band Syndrome	4.9%
169	Asphyxia Neonatorum	4.9%
170	Child Nutrition Disorders	4.9%
171	Hypervitaminosis A	4.9%
172	Infant Nutrition Disorders	4.9%
173	Infant, Premature, Diseases	4.9%
174	Situs Inversus	4.9%
175	Malnutrition	4.9%
176	Infantile Colic	4.9%
177	Retinitis Pigmentosa	4.9%
178	Myelinolysis, Central Pontine	4.9%
179	Pigmentary retinopathy	4.9%
180	Porokeratosis	4.9%
181	Keratoderma, Palmoplantar	4.9%
182	Tuberous Sclerosis	4.9%
183	Glycosuria	4.9%
184	Chronic granulomatous disease	4.9%
185	Reye Syndrome	4.9%
186	Hepatic Encephalopathy	4.8%
187	Variegate Porphyria	4.8%
188	Acute intermittent porphyria	4.8%
189	Porphyria Cutanea Tarda	4.8%
190	Retinal Dysplasia	4.8%
191	Diabetic Ketoacidosis	4.8%
192	Welander Distal Myopathy	4.8%
193	Sickle Cell Trait	4.8%
194	Acidosis, Lactic	4.8%
195	Calciphylaxis	4.8%
196	Hemosiderosis	4.8%
197	Ketosis	4.8%
198	Ketonuria	4.8%
199	Ketoacidosis	4.8%
200	Ketonemia	4.8%
201	Vascular calcification	4.8%
202	Aniridia	4.8%
203	Epidermolysis Bullosa	4.7%
204	HIV Wasting Syndrome	4.6%
205	Nesidioblastosis	4.6%
206	Meconium Aspiration Syndrome	4.3%
207	Duane Retraction Syndrome	4.2%
208	Kernicterus	4.1%
209	Peutz-Jeghers Syndrome	4.1%
210	Myotonia Congenita	4.1%
211	Thrombasthenia	4.1%
212	Antithrombin III Deficiency	4.1%
213	Necrobiosis Lipoidica Diabeticorum	4.1%
214	Protein C Deficiency	4.1%
215	Afibrinogenemia	4.1%
216	Dystonia Musculorum Deformans	4.1%
217	Factor VII Deficiency	4.1%
218	Factor X Deficiency	4.1%
219	Factor XII Deficiency	4.1%
220	Hemophilia A	4.1%
221	Activated Protein C Resistance	4.1%
222	Factor II deficiency	4.1%
223	Factor VIII Deficiency	4.1%
224	Factor V deficiency	4.1%
225	Factor XI Deficiency	4.1%
226	Hypoprothrombinemias	4.1%
227	Lafora Disease	4%
228	Unverricht-Lundborg Syndrome	4%
229	Anemia, Diamond-Blackfan	4%
230	Eye Abnormalities	3.9%
231	Cardiovascular Abnormalities	3.9%
232	Xeroderma	3.9%
233	Mobius Syndrome	3.9%
234	Skin Abnormalities	3.8%
235	Lymphatic Abnormalities	3.8%
236	Congenital Microtia	3.8%
237	Wernicke Encephalopathy	3.8%
238	Anemia, Neonatal	3.7%
239	Persistent Fetal Circulation Syndrome	3.7%
240	Acute Chest Syndrome	3.7%
241	Congenital diaphragmatic hernia	3.7%
242	Pseudoxanthoma Elasticum	3.7%
243	Umbilical hernia	3.7%
244	Acidosis, Respiratory	3.6%
245	Adiposis Dolorosa	3.6%
246	Alkalosis, Respiratory	3.6%
247	Multiple Endocrine Neoplasia	3.6%
248	HIV-Associated Lipodystrophy Syndrome	3.6%
249	Insulin Resistance	3.6%
250	Osteoporosis, Postmenopausal	3.6%
251	Congenital Hypothyroidism	3.5%
252	Dermatitis, Atopic	3.5%
253	Renal Osteodystrophy	3.4%
254	Renal rickets	3.4%
255	Polycystic Kidney, Autosomal Dominant	3.2%
256	Hypercholesterolemia	3.2%
257	Hyperlipoproteinemias	3.2%
258	Hypertriglyceridemia	3.2%
259	Urogenital Abnormalities	3.1%
260	Behcet Syndrome	3.1%
261	Neonatal Abstinence Syndrome	3.1%
262	Gestational Diabetes	3%
263	Latent Autoimmune Diabetes in Adults	3%
264	Fetal Growth Retardation	3%
265	Basal Cell Nevus Syndrome	3%
266	Diabetes Mellitus, Experimental	3%
267	Fetal Hypoxia	3%
268	Congenital nystagmus	3%
269	Fetal Alcohol Spectrum Disorders	3%
270	Toxoplasmosis, Congenital	2.9%
271	Syphilis, Congenital	2.9%
272	Nephrocalcinosis	2.9%
273	Tetany	2.9%
274	Papillon-Lefevre Disease	2.9%
275	Cerebral Amyloid Angiopathy	2.9%
276	Classical Lissencephalies and Subcortical Band Heterotopias	2.8%
277	Kearns-Sayre syndrome	2.8%
278	Hyperkeratosis, Epidermolytic	2.8%
279	Charcot-Marie-Tooth Disease	2.8%
280	Huntington Disease	2.7%
281	Deficiency Diseases	2.7%
282	Prune Belly Syndrome	2.7%
283	Starvation	2.7%
284	Twins, Conjoined	2.7%
285	Refeeding Syndrome	2.7%
286	Waardenburg Syndrome	2.7%
287	Anencephaly	2.6%
288	Chorioamnionitis	2.5%
289	Primary amyloidosis	2.4%
290	Nephroblastoma	2.4%
291	Adenomatous Polyposis Coli	2.2%
292	Maxillofacial Abnormalities	2.2%
293	Dural Arteriovenous Fistula	2.2%
294	Dextrocardia	2.2%
295	Abnormalities, Radiation-Induced	2.1%
296	Amyotrophic Lateral Sclerosis	2.1%
297	Multiple Endocrine Neoplasia Type 1	2.1%
298	Anophthalmos	2.1%
299	Anus, Imperforate	2.1%
300	Hydranencephaly	2.1%
301	Microphthalmos	2.1%
302	Neural Tube Defects	2.1%
303	Paralysis, Obstetric	2.1%
304	Retinopathy of Prematurity	2.1%
305	Tethered Cord Syndrome	2.1%
306	Iniencephaly	2.1%
307	Craniorachischisis	2.1%
308	Limb Deformities, Congenital	2.1%
309	Exencephaly	2.1%
310	Septo-Optic Dysplasia	2.1%
311	Craniofacial Abnormalities	2.1%
312	Cortical Dysplasia	2.1%
313	Malformations of Cortical Development	2.1%
314	Anorectal Malformations	2.1%
315	Bronchopulmonary Dysplasia	2%
316	Hydrops Fetalis	2%
317	Ophthalmia Neonatorum	1.8%
318	CREST Syndrome	1.8%
319	Klinefelter Syndrome	1.8%
320	Scimitar Syndrome	1.7%
321	Blepharophimosis	1.7%
322	Laryngostenosis	1.7%
323	Mouth Abnormalities	1.7%
324	Respiratory Distress Syndrome, Newborn	1.7%
325	Esophageal Atresia	1.7%
326	Intestinal Atresia	1.7%
327	Horseshoe Kidney	1.7%
328	Pectus excavatum	1.7%
329	Klippel-Feil Syndrome	1.6%
330	Gastroschisis	1.6%
331	Synostosis	1.6%
332	Acrodermatitis	1.6%
333	Lymphangiectasis, Intestinal	1.6%
334	Gianotti-Crosti Syndrome	1.6%
335	Turner Syndrome	1.6%
336	Kwashiorkor	1.6%
337	Magnesium Deficiency	1.6%
338	Potassium Deficiency	1.6%
339	Protein Deficiency	1.6%
340	Frontotemporal dementia	1.6%
341	Avitaminosis	1.6%
342	Arthrogryposis	1.4%
343	Choanal Atresia	1.4%
344	Tracheobronchomegaly	1.4%
345	Laryngocele	1.4%
346	Choledochal Cyst	1.4%
347	Hermaphroditism	1.4%
348	Disorders of Sex Development	1.4%
349	Craniosynostosis	1.4%
350	Syndactyly	1.4%
351	Brachycephaly	1.4%
352	Cryptorchidism	1.4%
353	Hypospadias	1.4%
354	Central Nervous System Cysts	1.4%
355	Dandy-Walker Syndrome	1.4%
356	Leukomalacia, Periventricular	1.4%
357	POEMS Syndrome	1.4%
358	Microcephaly	1.3%
359	Macrocephaly	1.3%
360	Obesity	1.3%
361	Denys-Drash Syndrome	1.3%
362	Noonan Syndrome	1.2%
363	Amelia	1.2%
364	Arachnodactyly	1.2%
365	Ectopia Cordis	1.2%
366	Ectromelia	1.2%
367	Hemimelia	1.2%
368	Meningomyelocele	1.2%
369	Phocomelia	1.2%
370	Sirenomelia	1.2%
371	Spina Bifida	1.2%
372	Polydactyly	1.2%
373	Brachydactyly	1.2%
374	Plagiocephaly	1.2%
375	Lower Extremity Deformities, Congenital	1.2%
376	Upper Extremity Deformities, Congenital	1.2%
377	Single umbilical artery	1.2%
378	Laryngomalacia	1.2%
379	Pectus carinatum	1.2%
380	Bladder Exstrophy	1.2%
381	Epispadias	1.2%
382	Multicystic Dysplastic Kidney	1.2%
383	Porencephaly	1.1%
384	Acrocephalosyndactylia	1.1%
385	Ascorbic Acid Deficiency	1.1%
386	Vitamin A Deficiency	1.1%
387	Vitamin D Deficiency	1.1%
388	Vitamin E Deficiency	1.1%
389	Marasmus	1.1%
390	Jaw Abnormalities	1%
391	Aortic coarctation	1%
392	Cor Triatriatum	1%
393	Coronary Vessel Anomalies	1%
394	Dental Enamel Hypoplasia	1%
395	Patent ductus arteriosus	1%
396	Ebstein Anomaly	1%
397	Heart Septal Defects	1%
398	Hyaline Membrane Disease	1%
399	Hypodontia	1%
400	Macrostomia	1%
401	Meningocele	1%
402	Microstomia	1%
403	Tetralogy of Fallot	1%
404	Transposition of Great Vessels	1%
405	Hypoplastic Left Heart Syndrome	1%
406	May-Thurner Syndrome	1%
407	Encephalocele	1%
408	Craniofacial Dysostosis	1%
409	Wolff-Parkinson-White Syndrome	1%
410	Cleft Palate	1%
411	Obesity, Abdominal	1%
412	Poland Syndrome	0.9%
413	Long QT Syndrome	0.8%
414	Platybasia	0.8%
415	Tricuspid Atresia	0.8%
416	Arrhythmogenic Right Ventricular Dysplasia	0.8%
417	Gonadal Dysgenesis	0.8%
418	Bronchomalacia	0.8%
419	Adrenogenital Syndrome	0.8%
420	Tracheomalacia	0.8%
421	Ovotesticular Disorders of Sex Development	0.8%
422	Dermal Sinus	0.8%
423	Spina Bifida Cystica	0.8%
424	Spina Bifida Occulta	0.8%
425	Lissencephaly	0.8%
426	Polymicrogyria	0.8%
427	Pachygyria	0.8%
428	Schizencephaly	0.8%
429	Periventricular Nodular Heterotopia	0.8%
430	Pediatric Obesity	0.8%
431	Folic Acid Deficiency	0.8%
432	Pellagra	0.8%
433	Thiamine Deficiency	0.8%
434	Vitamin B 12 Deficiency	0.8%
435	Vitamin B 6 Deficiency	0.8%
436	Pyridoxine Deficiency	0.8%
437	Micrognathism	0.7%
438	Pierre Robin Syndrome	0.7%
439	Beriberi	0.7%
440	Vitamin K Deficiency	0.6%
441	Aortopulmonary Septal Defect	0.6%
442	Double Outlet Right Ventricle	0.6%
443	Endocardial Cushion Defects	0.6%
444	Hyperandrogenism	0.6%
445	Myocardial bridging	0.6%
446	Aorticopulmonary Septal Defect	0.6%
447	Retrognathia	0.6%
448	Prognathism	0.6%
449	Mandibulofacial Dysostosis	0.5%
450	Romano-Ward Syndrome	0.5%
451	Anemia, Pernicious	0.5%
452	Scurvy	0.5%
453	Truncus Arteriosus, Persistent	0.5%
454	Goldenhar Syndrome	0.5%
455	Arteriovenous fistula	0.4%
456	Talipes	0.4%
457	Congenital clubfoot	0.3%
458	Vertical Talus	0.3%
459	Subacute Combined Degeneration	0.3%

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