Showing papers by "Princess Margaret Hospital for Children published in 1976"

Metatropic Dwarfism and its Variants (Report of six cases)

Abstract: Summary Six cases of METATROPIC DWARFISM or its VARIANTS–four which are the first Australian cases and one which is the first Indonesian case–are reported. Four of the cases had characteristic clinical and radiographic appearances whereas two, who died in early infancy, showed some variability in radiographic features. In one of the variants microscopy showed histologic features consistent with metatropic dwarfism. Microscopic, ultrastructural and biochemical studies of cartilage should be performed in all the cases of bone dysplasias in which the radiographic diagnosis is unknown or uncertain.

The influence of dietary protein deficiency on haemopoietic cells in the mouse

Abstract: These experiments examined the effect of a diet limited only in protein (4% by weight) on haemopoietic stem cells in mice. This diet places severe restrictions on growth and cell proliferation and this was reflected in lower numbers of colony forming units (CFUs) and in vitro colony forming cells (CFCs). Differences were apparent in the response of different organs to this stress; for instance, the incidence of spleen CFUs fell sharply from around 40/mg spleen tissue to 1 -4/mg spleen tissue after 3 weeks on a low protein diet. This selective loss did not occur in bone marrow where total CFUs remained proportional to cellular content. Yet a third pattern was shown by thymus CFUs–although the numbers were low these increased from 16/thymus in normal mice to 132/thymus in deprived mice. This was the only organ examined which showed an increase. The effects of a return to a high protein (18 %) diet showed that the spleen was the most responsive organ. By day 5 after the return to 18% protein the spleen contained as many CFUs per million cells as the bone marrow. During this time the content of CFU in the spleen had increased some 50-fold whereas bone marrow CFUs only doubled. The spleen assumes the major reconstitutive role during the refeeding process.

Anomalous left pulmonary artery with an unusual barium swallow

Abstract: An atypical finding on barium swallow in a child with an anomalous left pulmonary artery causing obstructive emphysema in the right lung is described. The value of bronchography and need for early surgical intervention is stressed.

Epiphyseo-metaphyseal cupping following infantile scurvy

Abstract: A five year old girl presented with short femora, cupped distal metaphyses and flexion deformity of the knees suggesting previous epiphyseo-metaphysea damage. The actiology was suggested by finding a history of previous infantile scurvy with separated distal femoral epiphyses. The rarity of this complication and possible contributing factors are discussed.

Kerosene pneumonia--its incidence in Perth and case history of a recent fatality.

Abstract: SUMMARY The incidence of admissions to the Princess Margaret Hospital for Children due to ingestion of kerosene in the years 1968/69 and 1973/74 has been ascertained. The chest x-rays of the patients admitted have been reviewed and the radiological changes assessed. A recent fatal case of kerosene pneumonitis is reported.

The long-term management of cystinuria.

Abstract: Macdonald, W. B., and Selvarajah, K. (1976).Aust. paediat. J., 12, 102–112. The long-term management of cystinuria. The maintenance of effective treatment for cystinuria is difficult in early childhood, for practical reasons. Older children and adults who have had renal colic or surgery are motivated to persist with tedious and inconvenient treatment as part of their life-style. Experiences with the management of a selected sample of five children with cystinuria are recorded. The group includes one patient who has been successfully treated with D-penicillamine for over 10 years, one of the longest treatment spans recorded for this drug in the treatment of cystinuria.