Table 2 The data of the cases with PGGH available in the literature
Author | case | Age (year) | Gender | Ethnicity | Family history | GC exposure | Clinical manifestation | Cortisol (nmol/L) | ACTH (pmol/L) | 24hUFC (nmol/24 h) | Imaging of adrenal gland | Treatment | Prognosis |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Our case | 1 (Patient 1) | 3.6 | M | Chinses | + | + | central obesity, hirsutism, moon face, acne, buffalo hump, fatigue, decreased cognitive ability, hypertension | 0.00–117.27 | 0.00–4.35 | undetectably low | normal | mifepristone | remission |
2 (Patient 2) | 0.55 | M | Chinses | + | + | central obesity, moon face, acne, buffalo hump, hypertension | 11.51–40 | <1.00 | normal | no | death | ||
Russcher et al. [3] | 3 | 13 | F | Netherlander | – | + | obesity, fatigue, growth retardation, violaceous striae, osteopenia | <30 | <3.00 | normal | stop using budesonide | spontaneous remission | |
Newfield et al. [5]. | 4 | 10.8 | F | Americian | – | – | central obesity, moon face, buffalo hump, violaceous striae, osteopenia, learning disability, early puberty | 304.18 ± 15.28 | 6.09 ± 2.35 | normal | normal | mifepristone | remission |
Nicolaides et al. [4]. | 5 | 9 | F | Greek | – | – | central obesity, moon face, buffalo hum, violaceous striae, acanthosis nigricans, hirsutism | 0.45–7.79 | 1.00 | 9.00 | normal | no | spontaneous remission |
Su, et al. [6] | 6 | child | NM | Chinses | – | – | central obesity, moon face, buffalo hum, violaceous striae, hirusm, hypertension, growth retardation, ostalgia | Low | Normal | Low | normal | NM | NM |
Iida et al. [14] | 7 | 54 | M | Japanese | + | – | central obesity, moon face, buffalo hump, DM | 20.00 ± 19.00 | <2.00 | 40.00–50.00 | normal | NM | NM |
Krysiak et al. [21] | 8 | 28 | F | Polish | – | – | Obesity, hypertension, prediabetes, osteopenia | 120.31 | <2.00 | 272.4–317.8 | adrenal gland atrophy | ketoconazole, cabergoline | death because of an accident |
Liu., et al. [22] | 9 | 27 | M | Chinses | + | – | central obesity, moon face, buffalo hum, violaceous striae, osteopenia | 10.75 | <0.22 | 11.95 | adrenal gland atrophy | mifepristone | remission |
10 (father of case 9) | adult | M | Chinses | + | – | hypertension, moon face, buffalo hum, violaceous striae, osteopenia, hyperglycemia | NM | < 0.22 | 23.62 | normal | no | exacerbation | |
Al-Shoumer, et al. [23] | 11 | 32 | F | Kuwaitis | – | + | obesity, moon face, buffalo hum, violaceous striae, DM, renal calculus, myasthenia, hypertension, | 28 | < 1.00 | undetectably low concentration | adrenal myelolipoma | NM | NM |
Zhang, et al. [1] | 12 | 29 | M | Chinses | – | – | central obesity, moon face, buffalo hum, violaceous striae, acne, osteopenia, hypokalemia, impaired glucose tolerance, hypertension | 72.64–216.56 | 5.60–9.56 | 378.12 | normal | no | spontaneous remission |
Santen, et al. [17] | 13 | 46 | F | Americian | + | + | obesity, moon face, fatigue, headache, abdominal pain, nausea, diarrhea, anxiety/depression, muscle and joint aches, hypertension | 63.00–98.00 | NM | 276.00 | NM | no | NM |