[Radiological aspects of oxalosis (author's transl)].
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Biomedical subjects
Publications and source records attributed to J Sauvegrain.
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A young girl aged 13 years was hospitalized for metaphyseal fractures of both knees, and was found to have idiopathic juvenile osteoporosis which followed a favourable course over a period of one year. The authors recall the difficulties encountered in diagnosing this rare affection as no specific biological changes have been demonstrated. The criteria used are: the age of the patient, the disease usually becoming evident in the prepuberty period, the contrast between the very severe osteoporosis noted on radiological examination and the perfect general condtion of the patient, the negative biological tests results, and finally, the spontaneous repair of the osteoporosis in one to four years.
Radiological abnormalities of the ribs are reported in 5 newborn infants with myotonic dystrophy. In all 5, the ribs appeared very thin, in contrast to the normal appearance of the rest of the skeleton. This slenderness, which is important for diagnosis and prognosis, seems to be caused by hypotonia of the intercostal muscles. However, it is not pathognomonic of myotonic dystropy, for it can be observed in other myopathies.
The neuro-radiological findings in 38 cases of precocious puberty of central origin and 9 cases of hypopituitarism (craniopharyngiomas excepted), are reported. The radiological examination consisted of plain films of the skull and pneumo-encephalography. In the 9 cases with hypopituitarism radiological examination was normal in 4 and localised but quite diverse anomalies were discovered in 5. Out of 38 patients presenting with isosexual precocious puberty, 29 were female and 9 male. Out of the 29 girls, neuro-radiological examination was normal in 20 and showed a hypothalamic anomaly in 9. Out of the 9 boys, 8 had a hypothalamic anomaly, and only one examination was normal. In precocious puberty we found 1 ectopic pinealoma and 2 gliomas of the chiasma. In these three cases the clinical context and radiological examination made the diagnosis obvious. Masses were discovered in 7 (3 spongioblastomas and 4 heterotopias). In 2 cases (spongioblastomas) neurological symptoms were present and made an operation mandatory. In 5 cases (1 spongioblastoma, 4 heterotopias) precocious puberty was an isolated finding. It was not possible to make, on a clinical or radiological bases, a distinction between spongioblastoma and heterotopia. As time has passed the role of surgery has changed. Formerly, surgery aimed at excision of the lesion, but with advances in medical treatment surgical intervention is now directed towards biopsy and the histological study of lesions which may be treated by radiotherapy.
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The authors stress the value of radiological examinations in the diagnosis of interstitial pneumonia in children with immuno-depression secondary to chemotherapy and/or radiotherapy. X-rays play a capital role in the early diagnosis of these conditions and may be of vital interest in some cases, in particular pneumocystosis which is curable. The diagnosis is based upon the discovery of minor signs of radiological interstitial syndrome, which in one third of cases is limited to a decrease in lung volume. This syndrome will be associated sooner or later with signs of alveolar involvement. The authors subsequently describe the underlying background, frequency, immune state, the circumstances of diagnosis and features of the aetiological diagnosis.
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