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Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by Adrenocorticotropic hormone resistant adrenal insufficiency, Alacrmia, Achalasia of the oesophageal cardia, progressive neurological degeneration and occasionally autonomic instability (making it 4A syndrome). Reported neurological abnormalities included developmental delay, ataxia and polyneuropathy with sensory, motor and autonomic components, long-tract degeneration, parkinsonism and mild dementia. In this paper we report a 13 year old boy with Allgrove syndrome presenting with muscular weakness that was confirmed by EMG studies. To our knowledge, muscle disease in Allogrove syndrome was not reported before. [ABSTRACT FROM AUTHOR]

Introduction: Untreated children with growth hormone deficiency (GHD) have typical somatic features, including short stature, acromicria and distinctive craniofacial features including small head circumference. Patients and Methods: By using a cross sectional study design, we investigated the effect of GHD on craniofacial growth with photographic facial morphometrics & various anthropometric measurements, in 20 children with GHD compared with 20 healthy children and normal first degree relatives of the same age and sex group. Results: Untreated children with GHD had retarded facial height & width (p<0.01) compared with the control group. Moreover all anthropometric measurements (weight, height, head circumference, sitting height, arm span and sub-ischial leg length) were reduced in GHD children in comparison to controls (p<0.01) except ear length which was above 3rd percentile. Also small head circumference for chronologic age and for height age was observed in GHD children (p<0.01). In addition small hands and feet for age (below 3rd percentile) were found in untreated GHD children when compared with normal controls. This report validates & quantifies the clinical impression of foreshortened facies in GHD children. [ABSTRACT FROM AUTHOR]