Clinical_Rounds_in_Endocrinology_Volume_II_-_Pediatric_Endocrinology

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8.2 Stepwise Analysis


Linear growth failure and poor development of secondary sexual characteristics in an
adolescent raise the possibilities of chronic systemic illness, uncontrolled type 1
diabetes, multiple pituitary hormone deficiency, idiopathic growth hormone defi-
ciency, juvenile primary hypothyroidism, and childhood Cushing’s syndrome.
Further, a possibility of Turner syndrome should always be considered in a female
child who presents with short stature and delayed puberty. Though she did not have
any clinical evidence of chronic systemic disease, her weight was more compro-
mised than height (height age > weight age). However, relevant investigations to
exclude the systemic diseases were noncontributory. She had phenotypic abnormali-
ties like multiple pigmented nevi over face, low hairline, and cubitus valgus sugges-
tive of Turner syndrome which was further substantiated by high level of FSH and
karyotype [46Xi(X)(q10)]. Patients of Turner syndrome born-at-term usually (90 %)
have mean birth weight deficit of 600 g, and approximately 20 % of neonates have
low birth weight as well as low birth length(≤2SDS). The index patient had a birth
weight of 2.7 Kg which is within the normal limits. Mean height deficit in an adult
with Turner syndrome is approximately 20 cm, and this is contributed by progressive


c

Fig. 8.1 (continued)


8 Turner Syndrome

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