This report of a Harlequin foetus surviving is due essentially to two factors. a) the intensive care provided to the erythrodermic infant, looking after its nutrition, temperature control and controls of infection and b) the use of a new synthetic Vitamin A acid--Etretinate in the management of dyskeratinisation syndromes. As this is of autosomal recessive inheritance, besides genetic counselling, Singapore has to develop its prenatal diagnostic techniques viz-amniocentesis and ultrasound studies of the skin, to detect dermatological problems.
A 10 month old girl with Henoch-Schönlein purpura (HSP) developed a rash predominantly on the anterior aspect of the knees. The authors speculate that this distribution of the rash was due to the pressure effects consequent to crawling and propose that the distribution of the rash in HSP should be described as pressure-dependent rather than merely gravity-dependent. The therapeutic implications of pressure-dependency in HSP are discussed.
The obesity-Cerebral-Ocular Syndrome is an uncommon disorder which closely resembles the Laurence-Moon-Biedl-Syndrome. It is also one of several disorders in which obesity, mental retardation and ocular abnormalities are associated. The article presents a case study followed up over 18 years and emphasises the importance of ophthalmic review in preserving visual function.
Early identification and rehabilitation of hearing impairment is important for development of language in affected children. Behavioural audiological tests in children are unreliable. Brainstem auditory evoked response (BAER) although a reliable objective test, is difficult to perform. Otoacoustic emissions (OAE) is now thought to be a more practicable screening modality. A study to compare the use of OAE and BAER in the paediatric population was thus undertaken. 100 children underwent OAE and BAER screening in SGH from August 1991 to February 1992. OAE was equally effective when compared with the BAER in testing for passed or failed subjects (p < 0.05, X2 = 4.9). The sensitivity and specificity of OAE with respect to BAER was 95% and 93% respectively. Mean test time was 3.1 minutes for OAE and 28.6 minutes for BAER. Success rate for OAE was 100%. We therefore conclude that OAE is a feasible alternative to the BAER as a hearing screening modality.
3 neonates admitted into our intensive care unit had their arterial blood gases analyzed. A total of 64, 67 and 73 arterial blood samples were analyzed for the 3 neonates respectively. The relationship between PaO2 and the corresponding SaO2 were plotted in an oxygen hemoglobin dissociation curve. PaO2 of 50mmHg corresponded to SaO2 of 80%, 84% and 92% for the 3 babies respectively, reflecting the fact that SaO2 can differ considerably. PaO2 of 50mmHg or SaO2 of 87% by themselves are inadequate as indicators of lower limit of arterial oxygenation. Indeed a consideration of other factors such as oxygen consumption, cardiac output, hemoglobin concentration are integral to this issue. PaO2 of 70mmHg corresponded to SaO2 of 92%, 93% and 97% respectively. However, at the upper limit of arterial oxygenation, the oxygen hemoglobin dissociation curve flattens out and PaO2 is a more sensitive indicator of the upper limit of arterial oxygenation as compared to SaO2.
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