Treatment of short stature M. Hashemipour MD Pediatric Endocrinologist Isfahan University of Medical Sciences 1.

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Presentation transcript:

Treatment of short stature M. Hashemipour MD Pediatric Endocrinologist Isfahan University of Medical Sciences 1

Treatment of short stature

Short stature Height velocity can be a sensitive indicator of disease

The height of an individual Height of an individual Depends on Genetics Nutritional status Hormonal milieu various environmental factors

The indications for of GH treatment Turner syndrome idiopathic short stature intrauterine growth retardation Prader-Willi syndrome Down syndrome Chondrodysplasia Chronic renal failure Height Deficit at Puberty

The indications for of GH Treatment GH improves growth and clinical status in children with cystic fibrosis

Bone Mass Development and Bone Metabolism in Juvenile Idiopathic Arthritis: Treatment with Growth Hormone for 4 YearsBone Mass Development and Bone Metabolism in Juvenile Idiopathic Arthritis: Treatment with Growth Hormone for 4 Years The indications for of GH Treatment

X-linked hypophosphatemic rickets. AIDS wasting or cachexia Short-bowel syndrome

Goals of the Treatment   Achieve normal height during childhood   Attain normal adult height

The growth response Maximal in the first year of treatment 200% of the pre-treatment velocity After several years, averages 150% of the baseline

DURATION OF THERAPY GH therapy should be continued at least until growth ceases Many young adults who had GHD in childhood have normal GH In contrast, children with multiple pituitary hormone deficiencies (usually organic) rarely recover the ability to secrete growth hormone as an adult.

Adverse reactions hypertension hair loss exacerbation of psoriasis seizures Otitis media Glucosuria Rhinitis Flu-like syndrome

slipped capital epiphysis of femur Pseudo tumor cerebri Leukemia INS resistance Recurrence risk of CNS tumors Hypothyroidism Transient gynecomastia Scoliosis Adverse reactions

carpal tunnel syndrome increase in the growth and pigmentation of nevi, local lipoatrophy or lipodystrophy Swelling of hands and feet type 2 diabetes mellitus Acute pancreatitis Retinal changes

Adverse reactions Mild transient edema Fever, malaise, dizziness hyperlipidemia vomiting, nausea, abdominal pain pain in hip, knee, and back, scoliosis, myalgia Musculoskeletal pain, swelling, stiffness, arthralgia

Idiopathic Short Stature defined by height more than 2.25 standard deviations below the mean for age and sex Height velocity<25th Diagnostic evaluation excludes causes of short stature

Idiopathic Short Stature Growth hormone therapy is approved by FDA Whether is due to correction of abnormality in GH secretion or to the effect of pharmacologic doses of GH is not known Multiple years of this therapy may add a few centimeters to adult height but it is difficult to predict which children will respond IU/kg weekly divided into daily doses

ISS& GH Therapy ISS& GH Therapy FDA in 2003  Height <2.25 SD mean value  Epiphyses are not closed  Delayed BA  Impaired or attenuated pubertal growth spurt  Expected adult height is less than 160 cm for boys 160 cm for boys 150 cm for girls 150 cm for girls

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