Prevalence and Incidence of Endocrine Disorders in Children: Results of a Survey in Baden-Wuerttemberg and Bavaria (EndoPrIn BB) 2000–2001

Abstract

Background: Sparse data is available on the incidence of endocrine disorders among children in Germany. Aim: A pioneer study was established to analyse, in the German states of Baden-Wuerttemberg (BW) and Bavaria (BY), the incidence and prevalence of congenital adrenal hyperplasia (AGS; CAH), precocious puberty (PP), primary congenital hypothyreosis (PCH), Graves disease (MB), and growth disorders related to the Ullrich-Turner syndrome (UTS) and growth hormone deficiency (GHD).

Methods: Participation in the study involved each paediatric hospital in BW and BY (n=63), and all regional paediatricians belonging to the Association of Statutory Health Insurance Physicians (SHI) practising in these states (n=1 443). Data collection was done from January 1, 2000, to December 31, 2001, and included all patients in the 0-<18 age range.

Results: Completeness of data was 81% for CAH and 55% for UTS (capture-mark-recapture method). The incidence rate (IR, per 100 000/year) versus prevalence rate (per 100 000 at the time point December 31, 2001) was: CAH 0.64 vs. 9.60; PP 2.42 vs. 10,85; PCH 1.88 vs. 14.97; MB 0.89 vs. 3.25; UTS 2.15 vs. 29.07; and GHD 3.47 (IR). Among neonates, the incidence of CAH was 1/7 794; PCH 1/2 629 and UTS 1/2 300.

Conclusions: A pioneer study has been established in Germany for investigating the frequency of AGS (CAH), PP, PCH, MB, UTS, and GHD among children and adolescents. Our data shows that these disorders occur in approx. 2 700 children per year in total Germany, and about 12 000 of these children need to be treated in specialized paediatric endocrinological centres.

Zusammenfassung

Hintergrund: Zahlen zur Häufigkeit endokrinologischer Erkrankungen bei Kindern liegen für Deutschland nur in sehr begrenztem Umfang vor. Ziel: Erstmalige Erfassung bei Kindern von Inzidenz und Prävalenz des Adrenogenitalen Syndroms (AGS), der Pubertas praecox (PP), der primären kongenitalen Hypothyreose (PCH), des Morbus Basedow (MB) und der durch das Ullrich-Turner-Syndrom (UTS) und einen Wachstumshormonmangel (GHD) bedingten Wachstumsstörungen in Baden-Württemberg (BW) und Bayern (BY).

Methoden: Alle baden-württembergischen und bayerischen Kinder- und Jugendkliniken (n=63) sowie die bei den kassenärztlichen Vereinigungen beider Länder gemeldeten niedergelassenen Kinder- und Jugendärzte (n=1 443) waren an der Erhebung beteiligt. In die Untersuchung eingeschlossen wurden alle 0- bis <18-jährigen Kinder mit AGS, PP, PCH, MB, UTS, GHD (Gesamterfassungszeitraum: 01.01.2000–31.12.2001). Die Erfassung erfolgte mittels Meldekarten.

Ergebnisse: Die Erfassungsvollständigkeit des AGS betrug 81% und die des UTS 55% (Capture-Mark-Recapture-Methode). Inzidenz- (IR, pro 100 000 pro Jahr) bzw. Prävalenzraten (pro 100 000 zum Zeitpunkt 31.12.2001): AGS 0,64 bzw. 9,60, PP 2,42 bzw. 10,85, PCH 1,88 bzw. 14,97, MB 0,89 bzw. 3,25, UTS 2,15 bzw. 29,07 und GHD 3,47 (nur IR). Bei Neugeborenen (Nenner: Lebendgeborene in 2000/2001) betrugen die Neuerkrankungsraten für das AGS 1/7 794, für die PCH 1/2 629 und für das UTS 1/2 300 (nur Mädchen).

Schlussfolgerungen: Erstmals wurden Daten zur Häufigkeit von AGS, PP, PCH, MB, UTS und GHD bei Kindern und Jugendlichen in Deutschland erhoben. Hochgerechnet auf Deutschland erkranken daran jährlich ca. 2 700 Kinder neu. Rund 12 000 Kinder mit diesen Erkrankungen benötigen eine oft lebenslange Betreuung in spezialisierten pädiatrisch endokrinologischen Zentren.

Keywords

incidence - prevalence - children - endocrine disorders

Schlüsselwörter

Inzidenz - Prävalenz - Kinder - endokrinologische Erkrankungen

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