Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022

被引：1

作者：

Tseretopoulou, Xanthippi ^{[1
,2
]}

Ali, Salma R. ^{[1
,2
]}

Bryce, Jillian ^{[2
]}

Amin, Nadia ^{[3
]}

Atapattu, Navoda ^{[4
]}

Bachega, Tania A. S. S. ^{[5
]}

Baronio, Federico ^{[6
]}

Ortolano, Rita ^{[6
]}

Birkebaek, Niels H. ^{[7
]}

Bonfig, Walter ^{[8
,9
]}

Cools, Martine ^{[10
]}

Davies, Justin H. ^{[11
]}

Thomas, Tessy

de Vries, Liat ^{[12
,13
]}

Elsedfy, Heba ^{[14
]}

Amr, Nermine H. ^{[14
]}

Flueck, Christa E. ^{[15
,16
]}

Globa, Evgenia ^{[17
]}

Guran, Tulay ^{[18
]}

Yavas-Abali, Zehra ^{[18
]}

Guven, Ayla ^{[19
]}

Hannema, Sabine E. ^{[20
]}

Iotova, Violeta ^{[21
]}

Konrad, Daniel

Lenherr-Taube, Nina ^{[22
]}

Krone, Nils P. ^{[23
]}

Leka-Emiri, Sofia ^{[24
]}

Vlachopapadopoulou, Elpis

Lichiardopol, Corina ^{[25
]}

Marginean, Otilia ^{[26
]}

Markosyan, Renata ^{[27
]}

Neumann, Uta ^{[28
]}

Niedziela, Marek ^{[29
]}

Banaszak-Ziemska, Magdalena ^{[29
]}

Phan-Hug, Franziska ^{[30
,31
]}

Poyrazoglu, Sukran ^{[32
]}

Probst-Scheidegger, Ursina ^{[33
]}

Randell, Tabitha ^{[34
]}

Russo, Gianni ^{[35
]}

Salerno, Mariacarolina ^{[36
]}

Seneviratne, Sumudu ^{[37
]}

Shnorhavorian, Margarett ^{[38
]}

Thankamony, Ajay ^{[39
]}

Tadokoro-Curraro, Rieko ^{[39
]}

van den Akker, Erica ^{[40
]}

van Eck, Judith ^{[40
]}

Vieites, Ana ^{[41
]}

Wasniewska, Malgorzata ^{[42
]}

Ahmed, S. Faisal ^{[43
]}

机构：

[1] Univ Glasgow, Sch Med Dent & Nursing, Dev Endocrinol Res Grp, Glasgow G51 4TF, Scotland

[2] Royal Hosp Children & Queen Elizabeth Univ Hosp, Off Rare Condit, Glasgow G51 4TF, Scotland

[3] Leeds Teaching Hosp NHS Trust, Dept Paediat Endocrinol, Leeds LS1 3EX, England

[4] Lady Ridgeway Hosp, Dept Paediat Endocrinol, Colombo 00800, Sri Lanka

[5] Univ Sao Paulo, Unidade Endocrinol Desenvolvimento, Lab Hormonios & Genet Mol LIM42, Disciplina Endocrinol & Metabol,Hosp Clin,Fac Med, BR-05508090 Sao Paulo, Brazil

[6] IRCCS Azienda Osped Univ Bologna, Dept Hosp Woman & Child, Pediat Unit, I-40138 Bologna, Italy

[7] Aarhus Univ Hosp, Dept Paediat, DK-8200 Aarhus, Denmark

[8] Tech Univ Munich, Dept Paediat, D-80804 Munich, Germany

[9] Klinikum Wels Grieskirchen, Dept Paediat, A-4600 Wels, Austria

[10] Univ Ghent, Univ Hosp Ghent, Dept Paediat Endocrinol, B-9000 Ghent, Belgium

[11] Univ Hosp Southampton, Dept Paediat Endocrinol, Southampton SO16 6YD, England

[12] Schneider Childrens Med Ctr Israel, Jesse & Sara Lea Shafer Inst Endocrinol & Diabet, IL-4920235 Petah Tiqwa, Israel

[13] Tel Aviv Univ, Fac Med & Hlth Sci, IL-6997801 Tel Aviv, Israel

[14] Ain Shams Univ, Dept Pediat, Cairo 11566, Egypt

[15] Univ Bern, Bern Univ Hosp, Dept Pediat, Pediat Endocrinol Diabetol & Metab,Inselspital, CH-3010 Bern, Switzerland

[16] Univ Bern, Bern Univ Hosp, Dept Biomed Res, Pediat Endocrinol Diabetol & Metab,Inselspital, CH-3010 Bern, Switzerland

[17] Ukrainian Sci & Pract Ctr Endocrine Surg, Transplantat Endocrine Organs & Tissues MOH Ukrain, UA-01021 Kiev, Ukraine

[18] Marmara Univ, Dept Pediat Endocrinol & Diabet, TR-34899 Istanbul, Pendik, Turkiye

[19] Baskent Univ, Istanbul Hosp, Dept Paediat Endocrinol, Med Fac, TR-06790 Istanbul, Turkiye

[20] locat Vrije Univ Amsterdam, Dept Pediat Endocrinol, Amsterdam UMC, NL-1007 MB Amsterdam, Netherlands

[21] Med Univ Varna, Dept Paediat, UMHAT Sv Marina, Varna 9002, Bulgaria

[22] Univ Zurich, Univ Childrens Hosp Zurich, Dept Endocrinol & Diabetol, CH-8032 Zurich, Switzerland

[23] Univ Sheffield, Dept Oncol & Metab, Sheffield S10 2RX, England

[24] PA Kyriakou Childrens Hosp, Dept Endocrinol Growth & Dev, Athens 11527, Greece

[25] Univ Med & Pharm Craiova, Univ Emergency Hosp, Dept Endocrinol, Craiova 200349, Romania

[26] Univ Med & Farm Timisoara, Clin children emergency Hosp Louis Turcanu Timisor, Dept Paediat, Timisoara 300011, Romania

[27] YSMU, Dept Endocrinol, Yerevan 0025, Armenia

[28] Charite Univ Med Berlin, Inst Expt Paediat Endocrinol, Ctr Chron Sick Children, Dept Paediat Endocrinol & Diabetol, D-10117 Berlin, Germany

[29] Poznan Univ Med Sci, Inst Pediat, Dept Pediat Endocrinol & Rheumatol, PL-61701 Poznan, Poland

[30] EHC Morges, Paediat Endocrinol, CH-1110 Morges, Switzerland

[31] Hosp Valais Sion, Paediat Endocrinol, CH-1950 Sion, Switzerland

[32] Istanbul Univ, Istanbul Fac Med, Dept Paediat, Paediat Endocrinol Unit, TR-34093 Istanbul, Turkiye

[33] Kantonsspital Winterthur, Dept Paediat Endocrinol, CH-8401 Zurich, Switzerland

[34] Nottingham Childrens Hosp, Dept Paediat Endocrinol, Nottingham NG7 2UH, England

[35] RCCS Osped San Raffaele, Dept Paediat, Endocrine Unit, I-20132 Milan, Italy

[36] Univ Naples Federico II, Dept Pediat, Unit Immunol, I-80131 Naples, Italy

[37] Univ Colombo, Fac Med, Dept Paediat, Colombo 00700, Sri Lanka

[38] Univ Washington, Seattle Childrens Hosp, Dept Urol, Sch Med, Seattle, WA 98105 USA

[39] Univ Cambridge, Dept Paediat, Cambridge CB2 2QQ, England

[40] Erasmus MC Sophia Childrens Hosp, Dept Pediat, Div Pediat Endocrinol, NL-3015 CN Rotterdam, Netherlands

[41] Hosp Ninos Dr Ricardo Gutierrez, Ctr Invest Endocrinol, Div Endocrinol, RA-1425 Buenos Aires, Argentina

[42] Univ Messina, Dept Human Pathol Adulthood & Childhood, Pediat Unit, I-98166 Messina, Italy

[43] Univ Glasgow, Royal Hosp Children, Dev Endocrinol Res Grp, Sch Med Dent & Nursing, Off Block, Glasgow G51 4TD, Scotland

来源：

JOURNAL OF THE ENDOCRINE SOCIETY | 2024年 / 8卷 / 10期

基金：

英国医学研究理事会;

关键词：

21-hydroxylase deficiency; adrenal insufficiency; adverse events; benchmark; congenital adrenal hyperplasia; sick day episodes; quality improvement; registry; 21-HYDROXYLASE DEFICIENCY; DISEASE; MANAGEMENT; THERAPY; CAH;

D O I：

10.1210/jendso/bvae145

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Background It is unclear whether targeted monitoring of acute adrenal insufficiency (AI) related adverse events (AE) such as sick day episodes (SDEs) and hospitalization rate in congenital adrenal hyperplasia (CAH) is associated with a change in the occurrence of these events. Aim Study temporal trends of AI related AE in the I-CAH Registry. Methods In 2022, data on the occurrence of AI-related AE in children aged <18 years with 21-hydroxylase deficiency CAH were compared to data collected in 2019. Results In 2022, a total of 513 children from 38 centers in 21 countries with a median of 8 children (range 1-58) per center had 2470 visits evaluated over a 3-year period (2019-2022). The median SDE per patient year in 2022 was 0 (0-2.5) compared to 0.3 (0-6) in 2019 (P = .01). Despite adjustment for age, CAH phenotype and duration of study period, a difference in SDE rate was still apparent between the 2 cohorts. Of the 38 centers in the 2022 cohort, 21 had also participated in 2019 and a reduction in SDE rate was noted in 13 (62%), an increase was noted in 3 (14%), and in 5 (24%) the rate remained the same. Of the 474 SDEs reported in the 2022 cohort, 103 (22%) led to hospitalization compared to 299 of 1099 SDEs (27%) in the 2019 cohort (P = .02). Conclusion The I-CAH Registry can be used for targeted monitoring of important clinical benchmarks in CAH. However, changes in reported benchmarks need careful interpretation and longer-term monitoring.

引用

页数：8

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