short stature in children

Childhood Short Stature

? Short stature is actually not a disease by itself. It is only a statistically defined height threshold, Medically, it is defined as height less than the 3rd percentile for age on the growth chart derived from local data.
? Physiology of growth
? three phases
? after birth: infancy, childhood and pubertal growth
? .1st year 25cm/year
? 2-3 year 7.5-13cm/year
? 7cm/year at 3-4 year s and
? 6cm/year at 5-6 years. It then remains at 4-6cm/year
? until puberty occurs when their growth will slow to about 4 cm/year
? Girls have their peak height velocity at early puberty, 9cm/year .
? Boys reach their peak height velocity during midpuberty , with growth rate 10cm/year
? In male skeletal growth till 17years while in female till age of 14 years
? .


type of short stature
? A-Genetic short stature/Familial short stature
? the most common type
? a condition characterized by height below the third percentile for age, normal annual growth rate, normal bone age, family history of short stature, expected adult height below the third percentile, normal onset of puberty, and normal result to findings on physical examinationTheir bone age is normal. and
? there is no endocrine abnormalities.,
? Late bloomer))B-Consititutional growth delay
?
? 1- No signs or symptoms of systemic disease
? 2- Bone age delayed up to 2 to 4 years but consistent with height age (age at which
? individual’s height would plot on the 50th percentile)
? 3- Period of poorest growth often occurring between the ages of 18 and 30 months, with steady linear growth thereafter (i.e., normal rate of growth for bone age)
? 4- Parental or sibling history of delayed physical development
? 5-Height prediction consistent with family characteristics
? 6-delayed puberty
How is constitutional delay managed?
? the child is seen once every 3 to 6 months for accurate height measurements
? and determination of growth velocity. A bone age test may be done yearly to assess the
? progression of bony maturation.
? In children who are of mid- to late
? pubertal ages (girls >13 years; boys >14 years) but showing minimal or no `signs of puberty,
? selective use may be made of estrogen or testosterone supplementation to initiate puberty,
? additional assessment may be indicated
? C-Short stature following small for gestational age (SGA)
? It is result from fetal, placental, maternal
? Could be symmetrical (early) Or asymmetrical (late)
? , D-Dysmorphic syndromes
? 1- turner syndrome
? 2- Noonan Syndrome,
? 3-RussellSilver,
? 4- Williams Syndrome
? 5- Down s Syndrome etc
? E-Endocrine disorders
? 1-Growth hormone insufficiency is the most type , congenital, acquired secondary to CNS tumors, head injury or even
? transient due to psychosocial deprivation))common
? 2- severe GH presented with micropenis, hypoglycemia, less than 3year
? 3- Hypothyroidism
? 4-Cushing s Syndrome is another cause of short stature.
? The patient is usually obese and short
?
? Others causes
? 1-skeletal dysplasia(achondrplasia)
? 2-coalic disease
? 3- CHD
? 4- Psychological deprivation
? SLE))5- any chronic illness type
? . Causes of short stature
? Genetic short stature
? Constitutional growth delay
? Combined genetic short stature and constitutional growth delay
? Short stature following small for gestational age
? Dysmorphic syndromes
? Endocrine disorders
? Skeletal dysplasia
? Chronic diseases
? Psychosocial deprivation
? . Assessment of short stature
? Height, weight, height velocity
? Height of parents
? Birth weight, gestation
? History of short stature, past medical history
? Family history of puberty, consanguinity, social history, school
? performance
? Systemic inquiry
? Examination of dysmorphic features
? Systemic examination
? Pubertal development staging
? Blood test if necessary (CBC, TSH, RFT, LFT)
? Radiological examination if necessary (bone age)
? Endocrine assessment (if indicated)
? Isolated GH defeciency DX of
? Random growth hormone levels are of little value because they are generally low in the daytime, even in children of average height. IGF-1 (or somatomedin C) mediates the anabolic effects of growth hormone, and levels correlate well with growth hormone status. However,IGF-1 can also be low in non endocrine conditions (e.g., malnutrition, liver disease), and the assays are somewhat inconsistent from laboratory to laboratory.
? IGFBP-3, which is the major binding protein for IGF-1 in serum, is also regulated by
? growth hormone. IGFBP-3 levels generally indicate growth hormone status and are less affected by nutritional factors than IGF-1. Many endocrinologists now use IGF-1 and IGFBP-3 as their initial screening tests for growth hormone deficiency.
? Definitive diagnosis of GH deficiency traditionally requires demonstration of absent or low levels of GH in response to stimulation. A variety of provocative tests have been devised that rapidly increase the level of GH in normal children. These include administration of insulin, arginine, clonidine, or glucagon. In chronic GH deficiency, the demonstration of poor linear growth, delayed skeletal age, and peak levels of GH (<10 ng/mL) in each of 2 provocative tests, are compatible with GH deficiency.
Treatment if isolated GH D
? The recommended dose of hGH is 0.18–0.3 mg/kg/wk during childhood. Higher doses have been used during puberty. Recombinant GH is administered subcutaneously in 6 or 7 divided doses AT night. Maximal response to GH occurs in the 1st year of treatment
? If growth rate drops below the 25th percentile, compliance should be evaluated before dose is increased
? the Criteria for stopping treatment include a decision by the patient that he or she is tall enough, a growth rate less than 1 inch/yr and a bone age >14 yr in girls and >16 yr in boys
Adverse effect
? 1-Some patients treated with GH have developed leukemia
? 2-pseudotumor cerebri, slipped capital femoral epiphysis, gynecomastia, and worsening of scoliosis.
? 3-Fasting and postprandial insulin levels are characteristically low before treatment, and they normalize during GH replacement. Treatment does not increase risk of type 1 diabetes, but it may increase the risk of type 2 diabetes.
? 4-Use of recombinant GH over the past 2 decades has eliminated the risk for CK(Creutzfeldt-Jakob ) disease and reduced the risk of antibody formation during treatment.