PMID- 30519970
OWN - NLM
STAT- MEDLINE
DCOM- 20190408
LR  - 20210109
IS  - 1432-1076 (Electronic)
IS  - 0340-6199 (Print)
IS  - 0340-6199 (Linking)
VI  - 178
IP  - 3
DP  - 2019 Mar
TI  - Growth of children with biliary atresia living with native livers: impact of 
      corticoid therapy after portoenterostomy.
PG  - 341-349
LID - 10.1007/s00431-018-3302-z [doi]
AB  - We addressed growth of biliary atresia (BA) patients living with native livers 
      between ages 0-6 and effects of post-surgical corticosteroid treatment on growth. 
      Growth charts of 28 BA patients born in Finland between 1987 and 2017 were 
      retrospectively evaluated. Dosage and length of corticosteroid treatment and 
      hydrocortisone substitution were reviewed. At birth, BA patients were shorter 
      (median height - 0.6 (interquartile range (IQR) - 1.3 to - 0.1) SDS, n = 28, 
      P < 0.001) than general population. Height remained stable during early childhood 
      (median height - 0.6 (IQR - 1.4 to 0.1) SDS for girls and - 0.4 (IQR - 1.6 to 
      0.2) SDS for boys at 6 years of age). Patients were of normal height adjusted 
      weight at 6 years with a median age and sex-adjusted body mass index (ISO-BMI) of 
      20.9 (IQR 19.3 to 25.0) for girls and 22.1 (IQR 20.7 to 25.6) for boys. Higher 
      (>/= 50 mg/kg) cumulative post-portoenterostomy prednisolone dosage resulted in 
      0.18 SDS lower height per treatment week (beta - 0.18, SE 0.04, P < 0.001) compared 
      to lower dosage (< 50 mg/kg).Conclusion: BA patients grow normally during early 
      childhood. As high postoperative corticosteroid dosage has a short-term negative 
      effect on height, very high dosages should be avoided. What Is Known: * Growth of 
      biliary atresia patients has mostly been shown to be within normal limits * 
      Corticosteroids may decrease growth rate What Is New: * Biliary atresia patients 
      surviving with their native livers are shorter than general population and their 
      mid-parental target height at birth * A high (> 50 mg/kg) cumulative prednisolone 
      dosage has a negative transitory impact on height gain after portoenterostomy.
FAU - Ruuska, Satu Maria
AU  - Ruuska SM
AUID- ORCID: 0000-0002-2768-855X
AD  - Department of Gastroenterology, Children's Hospital, Helsinki University 
      Hospital, PL 347, 00029 HUS, Helsinki, Finland. satu.ruuska@hus.fi.
AD  - Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University 
      Hospital, Helsinki, Finland. satu.ruuska@hus.fi.
FAU - Laaperi, Mitja Tapani
AU  - Laaperi MT
AD  - Pediatric Research Center, Children's Hospital, Helsinki University Hospital, 
      Helsinki, Finland.
FAU - Hukkinen, Maria
AU  - Hukkinen M
AD  - Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University 
      Hospital, Helsinki, Finland.
AD  - Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, 
      Helsinki, Finland.
FAU - Jalanko, Hannu
AU  - Jalanko H
AD  - Department of Pediatric Nephrology and Transplantation, Children's Hospital, 
      Helsinki University Hospital, Helsinki, Finland.
FAU - Kolho, Kaija-Leena
AU  - Kolho KL
AD  - University of Tampere, Tampere, Finland.
AD  - Tampere University Hospital, Tampere, Finland.
FAU - Pakarinen, Mikko P
AU  - Pakarinen MP
AD  - Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University 
      Hospital, Helsinki, Finland.
AD  - Pediatric Research Center, Children's Hospital, Helsinki University Hospital, 
      Helsinki, Finland.
AD  - Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, 
      Helsinki, Finland.
LA  - eng
GR  - 1234/Sigrid Juseliuksen Saatio/
GR  - 1234/Lastentautien Tutkimussaatio/
GR  - 1234/Helsingin ja Uudenmaan Sairaanhoitopiiri/
PT  - Journal Article
DEP - 20181205
PL  - Germany
TA  - Eur J Pediatr
JT  - European journal of pediatrics
JID - 7603873
RN  - 0 (Glucocorticoids)
SB  - IM
MH  - Biliary Atresia/*drug therapy/physiopathology/surgery
MH  - Body Height/*drug effects
MH  - Child
MH  - Child, Preschool
MH  - Combined Modality Therapy
MH  - Dose-Response Relationship, Drug
MH  - Drug Administration Schedule
MH  - Female
MH  - Follow-Up Studies
MH  - Glucocorticoids/*pharmacology/therapeutic use
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - *Portoenterostomy, Hepatic
MH  - Retrospective Studies
MH  - Treatment Outcome
PMC - PMC6437119
OTO - NOTNLM
OT  - Age and sex-adjusted body mass index (ISO-BMI)
OT  - Biliary atresia (BA)
OT  - Corticosteroids
OT  - Portoenterostomy
COIS- CONFLICT OF INTEREST: The authors declare that they have no conflict of interest. 
      ETHICAL APPROVAL: This study was approved by the Ethical Committee of Helsinki 
      University Hospital and in accordance with the 1964 Helsinki declaration and its 
      later amendments or comparable ethical standards. INFORMED CONSENT: For this type 
      of study formal consent is not required.
EDAT- 2018/12/07 06:00
MHDA- 2019/04/09 06:00
PMCR- 2018/12/05
CRDT- 2018/12/07 06:00
PHST- 2018/10/10 00:00 [received]
PHST- 2018/11/27 00:00 [accepted]
PHST- 2018/11/05 00:00 [revised]
PHST- 2018/12/07 06:00 [pubmed]
PHST- 2019/04/09 06:00 [medline]
PHST- 2018/12/07 06:00 [entrez]
PHST- 2018/12/05 00:00 [pmc-release]
AID - 10.1007/s00431-018-3302-z [pii]
AID - 3302 [pii]
AID - 10.1007/s00431-018-3302-z [doi]
PST - ppublish
SO  - Eur J Pediatr. 2019 Mar;178(3):341-349. doi: 10.1007/s00431-018-3302-z. Epub 2018 
      Dec 5.