Growth hormone treatment in cartilage-hair hypoplasia: Effects on growth and the immune system

被引:0
作者
Bocca, G
Weemaes, CM
van der Burgt, I
Otten, BJ
机构
[1] Univ Med Ctr St Radboud, Dept Pediat Endocrinol, NL-6500 HB Nijmegen, Netherlands
[2] Univ Med Ctr St Radboud, Dept Pediat, NL-6500 HB Nijmegen, Netherlands
[3] Univ Med Ctr St Radboud, Dept Pediat Immunol, NL-6500 HB Nijmegen, Netherlands
[4] Univ Med Ctr St Radboud, Dept Human Genet, NL-6500 HB Nijmegen, Netherlands
[5] Univ Med Ctr St Radboud, Dept Pediat Endocrinol, NL-6500 HB Nijmegen, Netherlands
关键词
cartilage-hair hypoplasia; growth hormone; immune function;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disorder characterized by metaphyseal chondrodysplasia with severe growth retardation and impaired immunity. We studied the effects of growth hormone treatment on growth parameters and the immune system in four children with CHH. The effects of growth hormone on growth parameters are the most prominent in patients with the mildest growth retardation. However, the effects are temporary and last only for 1 year. There is no gain in final height. Serum immunoglobulins did not change during growth hormone treatment. We conclude that growth hormone treatment is not beneficial in children with CHH.
引用
收藏
页码:47 / 54
页数:8
相关论文
共 50 条
  • [31] Outcomes of 42 pregnancies in 14 women with cartilage-hair hypoplasia: a retrospective cohort study
    Elina Holopainen
    Svetlana Vakkilainen
    Outi Mäkitie
    Orphanet Journal of Rare Diseases, 15
  • [32] Oral findings in patients with cartilage-hair hypoplasia - cross-sectional observational study
    Heidi Arponen
    Svetlana Vakkilainen
    Jaana Rautava
    Outi Mäkitie
    Orphanet Journal of Rare Diseases, 18
  • [33] Marked variability in the radiographic features of cartilage-hair hypoplasia: Case report and review of the literature
    Kwan, Andrea
    Manning, M. A.
    Zollars, Linda K.
    Hoyme, H. Eugene
    AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2012, 158A (11) : 2911 - 2916
  • [34] EARLY PRENATAL-DIAGNOSIS OF CARTILAGE-HAIR HYPOPLASIA (CHH) WITH POLYMORPHIC DNA MARKERS
    SULISALO, T
    SILLENCE, D
    WILSON, M
    RYYNANEN, M
    KAITILA, I
    PRENATAL DIAGNOSIS, 1995, 15 (02) : 135 - 140
  • [35] Identification of novel RMRP mutations and specific founder haplotypes in Japanese patients with cartilage-hair hypoplasia
    Yuichiro Hirose
    Eiji Nakashima
    Hirofumi Ohashi
    Hiroshi Mochizuki
    Yuki Bando
    Tsutomu Ogata
    Masanori Adachi
    Emi Toba
    Gen Nishimura
    Shiro Ikegawa
    Journal of Human Genetics, 2006, 51 : 706 - 710
  • [36] Treatment with recombinant IL-2 for recurrent respiratory infection in a case of cartilage-hair hypoplasia with autoimmune hemolytic anemia
    Matsumoto, S
    Ozono, K
    Yamamoto, T
    Yamaoka, K
    Okamura, T
    Hara, J
    Shima, M
    Okada, S
    JOURNAL OF BONE AND MINERAL METABOLISM, 2000, 18 (01) : 36 - 40
  • [37] Identification of novel RMRP mutations and specific founder haplotypes in Japanese patients with cartilage-hair hypoplasia
    Hirose, Yuichiro
    Nakashima, Eiji
    Ohashi, Hirofumi
    Mochizuki, Hiroshi
    Bando, Yuki
    Ogata, Tsutomu
    Adachi, Masanori
    Toba, Emi
    Nishimura, Gen
    Ikegawa, Shiro
    JOURNAL OF HUMAN GENETICS, 2006, 51 (08) : 706 - 710
  • [38] Granulocyte colony-stimulating factor-responsive chronic neutropenia in cartilage-hair hypoplasia
    Ammann, RA
    Duppenthaler, A
    Bux, J
    Aebi, C
    JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, 2004, 26 (06) : 379 - 381
  • [39] Craniofacial and Craniocervical Features in Cartilage-Hair Hypoplasia: A Radiological Study of 17 Patients and 34 Controls
    Arponen, Heidi
    Evaelahti, Marjut
    Maekitie, Outi
    FRONTIERS IN ENDOCRINOLOGY, 2021, 12
  • [40] Altered oral microbiome, but normal human papilloma virus prevalence in cartilage-hair hypoplasia patients
    Arponen, Heidi
    Vakkilainen, Svetlana
    Tomnikov, Natalie
    Kallonen, Teemu
    Silling, Steffi
    Maekitie, Outi
    Rautava, Jaana
    ORPHANET JOURNAL OF RARE DISEASES, 2024, 19 (01)