Bilateral microtia, absent patellae, short stature, poor weight gain, and characteristic facial features are described in two female sibs. Other skeletal anomalies included complete habitual dislocation of the elbow, slender ribs and long bones, abnormal modelling of the glenoid fossae with hooked clavicles, and clinodactyly. Bone age was significantly delayed and there was flattening of the epiphyses. This unusual combination of features has many similarities to the syndrome described by Hurst et a].In 1988 Hurst et all described two unrelated males with small ears, short stature, absent patellae, characteristic facial features, and skeletal anomalies. The boys were matched through the London Dysmorphology Database 'unknowns' group. Both boys had in addition unusual and similar radiographic findings. In particular, there were flat, abnormally modelled glenoid fossae with dislocation of the radial heads and hooked clavicles. Bone age was markedly delayed with abnormal epiphyses in both children. The two boys differed in that the second boy died at 4 days of age and was found to have an atrial septal defect, small adrenal glands, and bilateral hydronephrosis, while the other remained well aged 4j years but was found to have an IQ of 60 and craniosynostosis.

We describe the occurrence of an adrenocortical adenoma causing Cushing's syndrome in a 13-year-old girl with neurofibromatosis (NF). Three further cases of adrenocortical tumours in patients with NF have been reported previously, suggesting that there may be an association between NF and tumours of the adrenal cortex.

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digitalization, during which they used an arbitrary fixed dosage schedule. They also give no information on the severity of the cardiac failure in their series of 43 children and the only comment on the clinical response is that "no toxic symptoms were noted on this regimen, and heart rate and failure were well controlled."Digoxin was administered to all their patients by mouth as an elixir. Are they suggesting that intramuscular digoxin is unnecessary in cardiac failure or is the implication that in all their patients the failure was mild enough to permit oral therapy?If the authors could provide the supplementary data on the clinical aspects of their cases, this would help considerably in the evaluation of their suggested dosage regimen.