Morfoloġija Adrenali Bħala Indikatur tal-Kontroll tal-Mard fit-Tul F'Adulti B'Defiċjenza Klassika ta' 21-Hydroxylase Ⅱ

RIŻULTATI

Karatteristiċi tal-pazjent

Informazzjoni dettaljata dwar il-grupp ta' studju hija miġbura fil-qosor fit-Tabella 1. Erbgħa u erbgħin (48.9%) tal-90 pazjent b'21OHDkienu rġiel. Sitta u erbgħin pazjent (51.1%) kellhom it-tip ta’ ħela ta’ melħ ta’ 21OHD, filwaqt li 44 pazjent (48.9%) kellhom it-tip sempliċi virilizing. Ma kien hemm l-ebda differenzi sinifikanti fil-parametri bijokimiċi u d-dożi ta 'glukokortikojdi bejn it-tipi ta' ħela ta 'melħ u sempliċi virilizing (Tabella 2). Id-90 pazjent kollha ġew ikkurati bi prednisolone, żewġ pazjenti b'hydrocortisone, u 55 bil-fludrocortisone. Il-valuri medjani tad-doża ta’ kuljum ta’ glukokortikojdi/BSA u d-doża ta’ fludrokortisone/BSA kienu 17.5 mg/m2 (medda, 5.2 sa26.7) u 0.06 mg/m2 (medda, 0.03 sa 0.40), rispettivament. Il-livell ACTH u androstenedione/Tproporzjon kienu ogħla b'mod sinifikanti f'pazjenti rġiel milli f'pazjenti nisa (P=0.02 u P<0.001, respectively), while 11β-OHT/T was significantly higher in female than in male patients (P<0.001). Fifty-six out of 90 patients (62.2%) had at least one metabolic morbidity, and the male patients had a significantly higher incidence of metabolic morbidity than female patients (75% vs. 47.8%, P=0.01). The biochemically well-controlled group comprised 26 patients (28.9%, 11 men and 15 women), while the poorly-controlled group contained the remaining 64 patients.

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Sejbiet radjoloġiċi ta' pazjenti Classical 21OHD

Fost id-90 pazjent, 41 (45.6%) kellhom ipertrofija adrenali, 38 (42.2%) kellhom dehra normali, u 11 (12.2%) kellhom ipotrofija adrenali. Fil-grupp ikkontrollat ​​tajjeb, pazjent wieħed wera ipertrofija, filwaqt li 15 u 10 pazjent kellhom dehra normali u ipotrofija tal-glandoli adrenali, rispettivament. Fil-grupp ikkontrollat ​​ħażin, 40, 23, u pazjent 1 wrew ipertrofija, dehra normali, u ipotrofija tal-glandoli adrenali, rispettivament. Tnax-il pazjent (13.3%) kellhom tumuri adrenali, li kienu unilaterali f'disa' pazjenti u bilaterali fi tliet pazjenti. Fosthom, 11-il pazjent urew xaħam gross f'immaġini CT, li kkonfermaw il-preżenza ta 'myelolipoma. Il-pazjent l-ieħor ippreżentaadenoma adrenali. Informazzjoni dettaljata dwar is-sejbiet radjoloġiċi ta' 21 pazjent OHD hija miġbura fil-qosor f'Tabella 3.

Evalwazzjoni volumetrika u lineari tal-glandoli adrenali f'pazjenti 21OHD u paragun ma' kontrolli b'saħħithom

Il-medja u d-devjazzjoni standard tal-volumi adrenali tal-lemin, tax-xellug u totali ta '21 pazjent OHD kienu 8.5±5.5, 9.8±7.3, u 18.2±12.2 mL, rispettivament. Dawn il-valuri kienu kollha akbar b'mod sinifikanti milli fil-kontrolli b'saħħithom (3.5±1.0, 3.6±1.1, 7.1±2.0 mL, P<0.001 for all). The right, left, and average adrenal width were also significantly higher in 21OHD patients than in normal controls (4.7±1.8 mm vs. 3.3±0.5 mm, 4.6±2.0 mm vs. 3.3±0.5 mm, 4.7±1.9 mm vs. 3.3±0.5 mm, P<0.001 for all). All limb width and volume parameters were significantly higher in men than in women among the healthy controls (P<0.001 for all); however, there was no statistically significant difference between men and women with 21OHD (P>{{0}}.05 għal kulħadd). Ksibna żona taħt il-kurva (AUC) ta’ 0.85 għall-klassifikazzjoni ta’ pazjenti b’21OHD (P<0.001) with a sensitivity of 81.1% and specificity of 90% at a cut-off value of 9.1 mL. Table 4 presents detailed information on linear and volumetric parameters.

Analiżi tas-sottogrupp tal-volum adrenali f'pazjenti 21OHD

Il-volum adrenali bejn pazjenti b'21OHD li ħela l-melħ u dawk bit-tip sempliċi virilizing ma kienx differenti b'mod sinifikanti (17.9±13.9 mL vs. 18.4±8.9 mL, P{{10}}.31). Pazjenti b'mestrwazzjoni irregolari wrew volumi adrenali sinifikament akbar minn dawk b'mestrwazzjoni regolari (21.0±12.9 mL vs. 12.7±8.5 mL, P=0.009). Barra minn hekk, pazjenti b'tumuri adrenali kellhom volumi adrenali b'mod sinifikanti akbar minn dawk mingħajr l-ebda mases (31.8±16.1 mL vs. 15.7±9.5 mL, P=0.025).

Analiżi ta 'korrelazzjoni ta' volum adrenali u wisa 'ma' status ormonali

Kemm il-livelli fil-plażma ta' 17-OHP u androstenedione kienu differenti b'mod sinifikanti fost il-gruppi skont il-morfoloġija adrenali (ipertrofija, dehra normali, u ipotrofija; P<0.001 and P<0.02, respectively). Patients with adrenal hyhypertrophy showed the highest 17-OHP and androstenedione levels (median, 49.9 ng/mL [interquartile range (IQR), 22.2 to 108.7] and 2.6 ng/mL [IQR, 1.0 to 5.0], respectively), followed by those with normal adrenal glands (32.1 ng/mL [IQR, 5.1 to 58.3] and 1.1 ng/mL [IQR, 0.5 to 1.6]) and those with adrenal hypotrophy (3.6 ng/mL [IQR, 2.8 to 8.9] and 0.4 ng/mL [IQR, 0.2 to 1.1]) (Fig. 2).

Il-volum adrenali totali kkorrelata b'mod pożittiv ma' ACTH, {{0}}OHP, DHEA-S, androstenedione, 11 -OHT, 11 -OHA, u Preg-S kemm f'pazjenti rġiel kif ukoll f'nisa (r=0.43–0.94, P<0.05 for all). Positive correlations were found for 11β-OHT/T and plasma renin activity in male patients (r=0.49–0.70, P<0.05 for both) and for testosterone in female patients (r=0.70, P<0.001) (Fig. 3). Adrenal width correlated positively with ACTH, 17- OHP, 11β-OHT, 11β-OHT/T, Preg-S, DHEA-S in both male and female patients (r=0.31–0.95, P<0.05 for all). The adrenal width showed positive correlations with androstenedione, 11β-OHA, and plasma renin activity only in males (r=0.43–0.81, P<0.05 for all), and showed a positive correlation with testosterone only in females (r=0.44, P=0.03). Table 5 presents detailed information on the correlation analysis.

Il-volum adrenali totali kkorrelata b'mod pożittiv ma' ACTH, {{0}}OHP, DHEA-S, androstenedione, 11 -OHT, 11 -OHA, u Preg-S kemm f'pazjenti rġiel kif ukoll f'nisa (r=0.43–0.94, P<0.05 for all). Positive correlations were found for 11β-OHT/T and plasma renin activity in male patients (r=0.49–0.70, P<0.05 for both) and for testosterone in female patients (r=0.70, P<0.001) (Fig. 3). Adrenal width correlated positively with ACTH, 17- OHP, 11β-OHT, 11β-OHT/T, Preg-S, DHEA-S in both male and female patients (r=0.31–0.95, P<0.05 for all). The adrenal width showed positive correlations with androstenedione, 11β-OHA, and plasma renin activity only in males (r=0.43–0.81, P<0.05 for all), and showed a positive correlation with testosterone only in females (r=0.44, P=0.03). Table 5 presents detailed information on the correlation analysis.

Id-doża ta' kuljum tal-glukokortikojdi/BSA kkorrelata b'mod negattiv mal-volum adrenali (r{{0}-0.24, P{=0.02), iżda ma wriet l-ebda korrelazzjoni sinifikanti mal-wisa' adrenali ( r=−0.14, P=0.19). Id-doża ta' kuljum ta' fludrocortisone/BSA ma wrietx korrelazzjonijiet sinifikanti

Fig. 2. Livelli fil-plażma ta' (A) 17 -hydroxyprogesterone (17-OHP) u (B) androstenedione skond il-morfoloġija adrenali. Pazjenti b'ipertrofija adrenali urew l-ogħla livelli ta' 17-OHP u androstenedione, segwiti minn dawk bi glandoli adrenali normali u ipotrofija adrenali.

Prestazzjoni dijanjostika tal-volum adrenali u wisa 'għall-pazjenti li jidentifikaw 21OHD bi status ikkontrollat ​​tajjeb u morbidità metabolika

Il-volum u l-wisa' adrenali totali taw AUCs ta' {{0}}.87 (intervall ta' kunfidenza ta' 95% [CI], 0.78 sa 0}.93) u {{1{ {12}}}}.82 (95% CI, 0.72 sa 0.89), rispettivament, għat-tbassir tal-grupp bijokimiku kkontrollat ​​tajjeb (P<0.001 for both) (Fig. 4). We could achieve a sensitivity of 73.1% and specificity of 90.6% using a cut-off value of 10.7 mL of adrenal volume. Using a cutoff value of 4.0 mm of adrenal width, a sensitivity of 80.8% and specificity of 79.7% were obtained. The AUCs of adrenal volume and width showed no significant differences (P=0.24).

It-totalvolum adrenaliirriżulta f'AUC ta' {{0}}.67 (95% CI, 0.56 sa 0.76) għat-tbassir ta' pazjenti b'morbożità metabolika waħda jew aktar (P=0.004) . Bl-użu ta 'valur cut-off ta' 17 mL, inkisbu sensittività ta '55.4% u speċifiċità ta' 76.5%.Wisa adrenalima wrew l-ebda valur dijanjostiku sinifikanti għat-tbassir tal-pazjenti b'morbidità metabolika (AUC, {{0}}.57; 95% CI, 0.46 sa 0.67; P=0.29) . L-AUC tal-volum adrenali kien ogħla b'mod sinifikanti minn dak tal-wisa' adrenali (P=0.03).

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