Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Phenotype details: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial: initial diagnosis, before molecular testing
Diagnosis/Definite: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
- Unknown
- Familial
- Familial, autosomal dominant
- Familial, autosomal recessive
- Familial, X-linked
- Familial, X-linked dominant
- Familial, X-linked dominant, male sparing
- Familial, X-linked recessive
- Paternal, Y-linked
- Maternal, mitochondrial
- Isolated (sporadic)
- Di-genic
- Complex
- - = Not applicable
Age/Examination: age at which the individual was examined.
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Age/Diagnosis: age diagnosis was confirmed
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Age/Onset: Age first symptoms disease appeared in individual:
- 35y = 35 years
- 04y08m = 4 years and 8 months
- 00y00m01d12h = 1 day and 12 hours
- 18y? = around 18 years
- 30y-40y = between 30 and 40 years
- >54y = older than 54
- ? = unknown
Phenotype/Onset: individual's phenotype at Age/Onset described using HPO
Protein: result from protein staining

 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000025682 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00029663 |
0000027815 |
large increase glycerol elimination urine |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034419 |
0000027816 |
large increase glycerol elimination urine |
- |
- |
Familial |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034420 |
0000027817 |
large increase glycerol elimination urine |
- |
- |
Familial |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034421 |
0000028015 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034410 |
0000028017 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034409 |
0000028020 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034408 |
0000028023 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034411 |
0000028026 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034412 |
0000028029 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034413 |
0000028032 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034414 |
0000028035 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034415 |
0000028038 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034404 |
0000028041 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034407 |
0000035809 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034418 |
0000035812 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034423 |
0000035821 |
large increase glycerol elimination urine |
- |
- |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00049064 |
0000158938 |
chronic pancreatis; glycerol serum 8.19mM, urine 210mM, leukocyte 0% GK |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
61y |
refractory hypertriglyceridemia |
- |
Johan den Dunnen |
00210373 |
0000158939 |
retarded growth/psychomotor development, fractures, abnormal teeth, seizures; glycerol serum 4.5mM, urine 283mM, fibroblast 4% GK |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
2y |
- |
- |
Johan den Dunnen |
00210374 |
0000158940 |
proximal weakness/atrophy scapular-humeral/pelvic-femoral; hand atrophy, lower leg weakness; no retractions/skeletal deformities; no ptosis, no dysphagia; no respiratory insuficiency; no dizziness ataxia; Vignos scale 4 (4 30y); Brook scale 2; CPK 810; IQ70 |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
3y |
- |
- |
Johan den Dunnen |
00210375 |
0000158941 |
mental retardation; glycerol serum 8.6mM, urine 93.6mM, fibroblast 10% GK |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
7y |
MR |
- |
Johan den Dunnen |
00210376 |
0000158942 |
1w-metabolic acidosis; small gestational age, lethargy preceded by fever/vomiting/diarrhoea; 7m-mild convergent strabismus, right inguinal hernia; glycerol plasma 5.2mM, urine 48mM, fibroblast 0.1% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
20m |
gastrointestinal symptoms, ketoacidosis |
- |
Johan den Dunnen |
00210377 |
0000158943 |
single episode hypoglycaemia (birht), small gestational age, birth weight 2450g, facial dysmorphism (square face, frontal bossing, low set mildly dysmorphic ears), hypoplastic right thumb cryptorchidism; glycerol plasma 6.4mM, urine 38mM, fibroblast 0.1% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
1d |
hypoglycaemia |
- |
Johan den Dunnen |
00210378 |
0000158944 |
1.5y, birth weight 3450g, no hypoglycaemic episodes; glycerol plasma 4.8mM, urine 60mM, fibroblast 0.4% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210379 |
0000158945 |
62y healthy maternal grandfather, never experienced clinical episode of hypoglycaemia; glycerol plasma 5.5mM, urine 32mM, fibroblast 0.1% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
none |
- |
Johan den Dunnen |
00210380 |
0000158946 |
mild psychomotor retardation, attention deficit disorder; glycerol plasma 1.3mM, urine 47mM, fibroblast 0.2% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
3y |
vomiting, ketoacidosis |
- |
Johan den Dunnen |
00210381 |
0000158947 |
delayed psychomotor development; glycerol plasma 3.6mM, urine 66mM, fibroblast 0.3% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
10m |
generalised seizures |
- |
Johan den Dunnen |
00210382 |
0000158948 |
never experienced clinical episode of hypoglycaemia, normal development; glycerol plasma 5.4mM, urine 41mM, fibroblast 0.3% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210383 |
0000158949 |
never experienced clinical episode of hypoglycaemia, normal development; glycerol plasma 5.1mM, urine 24mM, fibroblast 0.3% glycerol incorporation |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210384 |
0000158950 |
pseudo-hypertriglyceridemia, chronic alcoholic on hemodialysis for essential hypertension; fibroblast GK activity 32% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
36y |
pseudohypertriglyceridemia |
- |
Johan den Dunnen |
00210385 |
0000158951 |
glycerol males 4.0mM, carriers 0.54mM |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210386 |
0000158952 |
biochemically confirmed GKD |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
fasting hypoglycaemic coma |
- |
Johan den Dunnen |
00210387 |
0000158953 |
plasma glycerol 4.6 mmol, fibroblast GK 12% |
glycerol kinase deficiency |
GKD |
Familial |
- |
- |
- |
fasting hypoglycaemic coma |
- |
Johan den Dunnen |
00210388 |
0000158954 |
biochemically confirmed GKD |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
fasting hypoglycaemic coma |
- |
Johan den Dunnen |
00210389 |
0000158955 |
seizures; biochemically confirmed GKD |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
seizures |
- |
Johan den Dunnen |
00210390 |
0000158957 |
- |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
- |
neonatal seizures |
- |
Johan den Dunnen |
00210392 |
0000158959 |
hypotonia, developmental delay; 14m-febrile seizure; fibroblast GK 5.55% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
5d |
glyceroluria |
- |
Johan den Dunnen |
00210394 |
0000158962 |
3.5y-omiting, lethargy, hypoglycemiaand seizures, development mildly delayed, ketonuric and clinically asymptomatic; fibroblast GK 11.6% ; IQ84; walk-18m |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
2d |
seizures |
- |
Johan den Dunnen |
00210397 |
0000158966 |
15d-persisten high TG; 4y-developmental dealy; glycerol serum 4.7mmol, urine 3730mmol, almost no GK activity; intellectual disability |
glycerol kinase deficiency, adrenal hypoplasia; mental retardation |
GKD;AHC;MR |
Unknown |
- |
- |
<14d |
failure to thrive, salt wating, convulsions |
- |
Johan den Dunnen |
00210401 |
0000158967 |
high urine glycerol; glycerol serum 2.5mmol, urine 1750mmol, slightly reduced GK activity |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
2y |
seizures, developmental delay |
- |
Johan den Dunnen |
00210402 |
0000158968 |
glycerol serum 5.9mmol, urine 5410mmol |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
64y |
persistent hypertriglyceridemia |
- |
Johan den Dunnen |
00210403 |
0000158969 |
glycerol serum 6.5mmol, urine 6584mmol |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
11y |
persistent hypertriglyceridemia |
- |
Johan den Dunnen |
00210404 |
0000158970 |
glycerol serum 6.1mmol, urine 6983mmol |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
61y |
persistent hypertriglyceridemia |
- |
Johan den Dunnen |
00210405 |
0000158971 |
glycerol serum 4.3mmol, urine 1105mmol |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
58y |
persistent hypertriglyceridemia |
- |
Johan den Dunnen |
00210406 |
0000158972 |
4.5y-mild asymmetric spasticity, convergent strabismus, myopia, mild facial dysmorphism, pseudohypertriglyceridemia; glycerol plasma 2.78mmol, urine 170mmol, GK activity 3%; mild intellectual disability |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
2y |
episodic vomiting, dysmorphic, mild mental retardation, hyperactivity |
- |
Johan den Dunnen |
00210407 |
0000158973 |
normal growth and development; glycerol plasma 4.1mmol, urine 58.2mmol, GK activity 22% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
2y |
ketoacidosis, somnolence after vomiting, altered consciousness during gastrointestinal episodes |
- |
Johan den Dunnen |
00210408 |
0000158974 |
born 34w, birth weight 1950g, severe asphyxia, suspected AHX; 5d deceased of circulatory collapse; glycerol plasma 12mmol, urine 31mmol, GK activity 16% |
glycerol kinase deficiency, adrenal hypoplasia |
GKD;AHC |
Unknown |
- |
- |
1d |
small, respiratory distress sysndrome, adrenal glands imbalance |
- |
Johan den Dunnen |
00210409 |
0000158975 |
2-year-old boy was born 37w, birth weight 2760g, complicated pregnancy (premature rupture membrane), neonatal meningitis; glycerol plasma 0.08mmol, urine 6.9mmol, GK activity 85% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
3y |
delayed development, hypotonia, positive GowerÃs sign, persistent unexplained microcytic anemia |
- |
Johan den Dunnen |
00210410 |
0000158976 |
metabolic acidosis, recovered rapidly intravenous glucose therapy, did not tolerate physical exercise as well as friends, excessive physical activity elicited vomiting, hypoglycaemic symptoms, unconsciousness; glycerol serum 2.1-8.4mmol, urinary 43-280, fibroblast GK activity 1% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
4y6m |
pale, somnolent, hyperventilating, complaining severe abdominal pains |
- |
Johan den Dunnen |
00210411 |
0000158977 |
born full term, birthweight 3730g, normal growth rate/mental development; glycerol serum 3.4-8.0mmol, urinary 128-390, fibroblast GK activity 3% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
1y6m |
seizures, unconscious |
- |
Johan den Dunnen |
00210412 |
0000158979 |
GK activity 8.3% |
glycerol kinase deficiency |
GKD |
Unknown |
- |
- |
14d |
hypertriglyceridemia |
- |
Johan den Dunnen |
00210414 |