langerhans cell histiocytosis: growth hormone treatment over 3 years in kigs simon j howell kigs 10...
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Langerhans Cell Histiocytosis:Growth Hormone Treatment
over 3 years in KIGS
Simon J Howell
KIGS 10 Year Book, 1999
S J Howell - KIGS 10 Year Book, 1999
Baseline characteristics of 82 KIGS patients withLangerhans cell histiocytosis
TSH, thyroid-stimulating hormone; ACTH, adrenocorticotrophic hormone; LH, luteinising hormone;FSH, follicle-stimulating hormone; DI, diabetes insipidus
Variable Cross-sectional data
Boys (n=55; 67.1%) Girls (n=27; 32.9%)
Patients Percentile Patients Percentile
(n) (median) (10th) (90th) (n) (median) (10th) (90th)
Mid-parental height (SD) 52 -0.3 -1.2 0.8 24 0.0 -1.7 1.5
Birth weight (SDS) 49 -0.4 -2.1 1.2 26 -0.2 -1.1 0.6
Age at diagnosis of LCH(years)
33 2.7 0.5 11.3 17 3.0 0.8 7.2
Age at start of GH (years) 55 9.2 4.8 14.4 27 8.8 -6.0 15.7
Height (SDS) 55 -2.0 -3.6 -1.0 27 -2.0 -3.8 -0.8
Height – mid-parentalheight (SDS)
52 -1.8 -2.9 -0.8 24 -1.8 -3.6 0.2
Height velocity (cm/year) 32 3.6 1.6 -5.5 15 4.1 0.1 6.4
GH dose (IU/kg/week) 55 0.50 0.34 0.72 27 0.50 0.42 0.82
Hormon deficits All patients (n)
TSH (n=79) 14 (17.7%)
ACTH (n=78) 8 (10.3%)
LH/FSH (n=57) 6 (10.5%)
DI (n=79) 53 (67.1%)
S J Howell - KIGS 10 Year Book, 1999
Response to GH treatment of patients with LCH(cross-sectional data for 3 years)
Variable Cross-sectional data
Boys GirlsPatients Percentile Patients Percentile
(n) (median) (10th) (90th) (n) (median) (10th) (90th)
At start
Age (years) 55 9.2 4.8 14.4 27 8.8 6.0 15.7
Height (SDS) 55 -2.0 -3.6 -1.0 27 -2.0 -3.8 -0.8
Height – mid-parentalHeight (SDS)
52 -1.8 -2.9 -0.8 24 -1.8 -3.6 0.2
Height velocity (cm/year) 32 3.6 1.6 5.5 15 4.1 0.1 6.4
(1/4)
S J Howell - KIGS 10 Year Book, 1999
Response to GH treatment of patients with LCH(cross-sectional data for 3 years)
Variable Cross-sectional data
Boys GirlsPatients Percentile Patients Percentile
(n) (median) (10th
) (90th
) (n) (median) (10th
) (90th
)
First year
Age (years) 39 9.8 6.3 15.7 22 9.7 7.0 16.7
Height (SDS) 39 -1.8 -3.4 -0.4 22 -1.5 -3.2 -0.1
Height – mid-parentalHeight (SDS)
37 -1.5 -2.5 -0.3 14 -1.2 -3.3 0.6
Height velocity (cm/year) 39 8.6 4.1 12.0 22 9.3 1.5 12.0
(2/4)
S J Howell - KIGS 10 Year Book, 1999
Response to GH treatment of patients with LCH(cross-sectional data for 3 years)
Variable Cross-sectional data
Boys GirlsPatients Percentile Patients Percentile
(n) (median) (10th) (90th) (n) (median) (10th) (90th)
2nd year
Age (years) 28 10.0 6.6 14.4 12 10.6 8.6 13.6
Height (SDS) 28 -1.8 -3.2 -0.2 12 -0.9 -2.2 0.1
Height – mid-parentalHeight (SDS)
26 -1.4 -2.7 0.2 10 -0.9 -2.5 0.2
Height velocity (cm/year) 28 6.8 4.0 8.8 12 9.2 7.8 9.7
(3/4)
S J Howell - KIGS 10 Year Book, 1999
Response to GH treatment of patients with LCH(cross-sectional data for 3 years)
Variable Cross-sectional data
Boys GirlsPatients Percentile Patients Percentile
(n) (median) (10th) (90th) (n) (median) (10th) (90th)
3rd year
Age (years) 20 11.0 7.4 14.3 10 11.7 9.8 14.8
Height (SDS) 20 -1.0 -2.7 0.4 10 -0.6 -1.5 0.4
Height – mid-parentalHeight (SDS)
18 -1.2 -2.5 0.6 8 -0.9 -2.0 0.4
Height velocity (cm/year) 20 6.7 4.1 8.6 10 8.8 4.6 10.1
(4/4)
Response to GH treatment of patients with LCH(longitudinal data for 3 years)
S J Howell - KIGS 10 Year Book, 1999
Variable Longitudinal data(boys, n=13, girls, n=4)
Patients Percentile
(n) (median) (10th
) (90th
)
At start
Chronological (years) 17 7.3 4.2 10.5
Height (SDS) 17 -2.0 -4.4 -1.1
Height – MPH (SDS) 15 -2.4 -3.9 -1.0
Height velocity (cm/year) 11 4.5 0.9 6.5
MPH, mid-parental height
(1/4)
Response to GH treatment of patients with LCH(longitudinal data for 3 years)
S J Howell - KIGS 10 Year Book, 1999
MPH, mid-parental height
Variable Longitudinal data(boys, n=13, girls, n=4)
Patients Percentile
(n) (median) (10th
) (90th
)
1st
year
Chronological (years) 17 8.3 5.0 11.7
Height (SDS) 17 -1.6 -3.2 -0.4
Height – MPH (SDS) 15 -2.1 -3.3 -0.4
Height velocity (cm/year) 17 8.9 5.6 13.1
(2/4)
Response to GH treatment of patients with LCH(longitudinal data for 3 years)
S J Howell - KIGS 10 Year Book, 1999
MPH, mid-parental height
Variable Longitudinal data(boys, n=13, girls, n=4)
Patients Percentile
(n) (median) (10th
) (90th
)
2nd
year
Chronological (years) 17 9.2 6.0 12.6
Height (SDS) 17 -1.1 -2.5 0.2
Height – MPH (SDS) 15 -1.7 -2.6 0.1
Height velocity (cm/year) 17 7.3 3.7 9.9
(3/4)
Response to GH treatment of patients with LCH(longitudinal data for 3 years)
S J Howell - KIGS 10 Year Book, 1999
MPH, mid-parental height
Variable Longitudinal data(boys, n=13, girls, n=4)
Patients Percentile
(n) (median) (10th
) (90th
)
3rd
year
Chronological (years) 17 10.1 7.0 13.6
Height (SDS) 17 -0.8 -2.6 0.5
Height – MPH (SDS) 15 -1.5 -2.5 0.6
Height velocity (cm/year) 17 7.3 4.2 9.3
(4/4)
S J Howell - KIGS 10 Year Book, 1999
Response to GH replacement; comparison of patientswith idiopathic GHD and with GHD associated with LCH
n/a, not available(Ranke and Guilbaud 1991)
Variable Idiopathic GHD Langerhans cell histiocytosis
Cross-sectional data Longitudinal data(n=289) (n=82) (n=17)
Percentile Percentile Percentile(median) (10th) (90th) (median) (10th) (90th) (median) (10th) (90th)
Pretreatment
Age (years) 7.0 3.6 10.6 9.0 5.2 14.7 7.3 4.2 10.5
Height (SDS) -2.8 -4.4 -1.9 -2.0 -3.5 -0.9 -2.0 -4.1 -1.1
Height velocity(cm/year)
n/a 3.6 0.9 6.4 4.5 0.9 6.5
(1/3)
S J Howell - KIGS 10 Year Book, 1999
n/a, not available(Ranke and Guilbaud 1991)
Response to GH replacement; comparison of patientswith idiopathic GHD and with GHD associated with LCH
Variable Idiopathic GHD Langerhans cell histiocytosis
Cross-sectional data Longitudinal data(n=289) (n=82) (n=17)
Percentile Percentile Percentile(median) (10th) (90th) (median) (10th) (90th) (median) (10th) (90th)
1st year
Age (years) 8.0 4.6 11.6 9.8 6.6 15.7 8.3 5.0 11.7
Height (SDS) -2.2 -3.4 -1.1 -1.8 -3.2 -0.1 -1.6 -3.2 -0.4
Height velocity(cm/year)
8.8 5.8 12.9 8.8 3.8 12.0 8.9 5.6 13.1
(2/3)
S J Howell - KIGS 10 Year Book, 1999
n/a, not available(Ranke and Guilbaud 1991)
Response to GH replacement; comparison of patientswith idiopathic GHD and with GHD associated with LCH
Variable Idiopathic GHD Langerhans cell histiocytosis
Cross-sectional data Longitudinal data(n=289) (n=82) (n=17)
Percentile Percentile Percentile(median) (10th) (90th) (median) (10th) (90th) (median) (10th) (90th)
2nd year
Age (years) 9.0 5.6 12.6 10.3 7.2 13.6 9.2 6.0 12.6
Height (SDS) n/a -1.5 -3.0 0.1 -1.1 -2.5 0.2
Height velocity(cm/year)
6.7 4.7 9.4 7.3 4.4 9.7 7.3 3.7 9.9
(3/3)
S J Howell - KIGS 10 Year Book, 1999
aCorresponds to the start of the first year of GH treatmentbGH doseand frequency of injections are median values based on the whole of the first year of GH therapycThe range quoted for pubertal patients in the cross-sectional group is the percentage who had entered puberty at the start and at the end of the first yeardData from only 76 patientseData from only 15 patients
Factors influencing first-year height velocity during GH therapy
Idiopathic GHD Langerhans cell histiocytosis
Cross-sectional data(n=82)
Longitudinal data(n=17)
Chronological age (yrs)a 7.0 9.0 7.3
Target height SDS – height SDSa 2.4 1.8d 2.4e
GH dose (IU/kg/week)b 0.60 0.51 0.58
Frequency of injections (n/week)b 5 6 6
Pubertal (%)c 0 13-24 0