Short stature and low IGF-1 and IGFBP-3 despite normal growth hormone secretion in a 4 year-old girl with primary empty sella syndrome. 1996

J Dötsch, and W F Blum, and W Rascher, and J Kreuder, and W Kiess
Department of Pediatrics, University of Giessen, Germany.

A 4.1 year-old girl presented with short stature, cleft lip and additional upper incisor. Magnetic resonance imaging showed an empty sella due to a hypoplastic anterior pituitary. IGF-I and IGFBP-3 were at and below the 1st percentile, respectively. In contrast, normal spontaneous growth hormone (GH) secretion (5.5-h nocturnal sampling) was observed and pharmacological provocation raised (GH to levels between 15.4 and 53 micrograms/l. GH-binding protein levels were normal (210 pM). GH therapy led to an increase of growth velocity from 4.5 to 10.8 cm/year and a normalization of IGF-I and IGFBP-3 levels. The findings may imply an abnormal GH secretion pattern or a bioinactive GH in our patient. The data indicate that measurements of IGF-1 and IGFBP-3 may be a more sensitive test for integrative GH activity than GH testing itself.

UI MeSH Term Description Entries
D007334 Insulin-Like Growth Factor I A well-characterized basic peptide believed to be secreted by the liver and to circulate in the blood. It has growth-regulating, insulin-like, and mitogenic activities. This growth factor has a major, but not absolute, dependence on GROWTH HORMONE. It is believed to be mainly active in adults in contrast to INSULIN-LIKE GROWTH FACTOR II, which is a major fetal growth factor. IGF-I,Somatomedin C,IGF-1,IGF-I-SmC,Insulin Like Growth Factor I,Insulin-Like Somatomedin Peptide I,Insulin Like Somatomedin Peptide I
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D001827 Body Height The distance from the sole to the crown of the head with body standing on a flat surface and fully extended. Body Heights,Height, Body,Heights, Body
D002352 Carrier Proteins Proteins that bind or transport specific substances in the blood, within the cell, or across cell membranes. Binding Proteins,Carrier Protein,Transport Protein,Transport Proteins,Binding Protein,Protein, Carrier,Proteins, Carrier
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002971 Cleft Lip Congenital defect in the upper lip where the maxillary prominence fails to merge with the merged medial nasal prominences. It is thought to be caused by faulty migration of the mesoderm in the head region. Harelip,Cleft Lips,Harelips,Lip, Cleft,Lips, Cleft
D004652 Empty Sella Syndrome A condition when the SELLA TURCICA is not filled with pituitary tissue. The pituitary gland is either compressed, atrophied, or removed. There are two types: (1) primary empty sella is due a defect in the sella diaphragm leading to arachnoid herniation into the sellar space; (2) secondary empty sella is associated with the removal or treatment of PITUITARY NEOPLASMS. Empty Sella Syndrome, Primary,Empty Sella Syndrome, Secondary,Empty Sella Turcica,Empty Sella Turcica Syndrome,Primary Empty Sella Syndrome,Secondary Empty Sella Syndrome,Empty Sella Syndromes,Empty Sella Turcicas,Sella Turcica, Empty,Sella Turcicas, Empty
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014096 Tooth, Supernumerary An extra tooth, erupted or unerupted, resembling or unlike the other teeth in the group to which it belongs. Its presence may cause malposition of adjacent teeth or prevent their eruption. Molar, Fourth,Teeth, Supernumerary,Fourth Molar,Fourth Molars,Molars, Fourth,Supernumerary Teeth,Supernumerary Tooth

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