[Spondyloepiphyseal dysplasia tarda with progressive arthropathy: 2 siblings affected]. / Displasia espóndilo epifisiaria tarda con artropatía progresiva: 2 hermanos afectados.

We described a Mexican family whose parents were consanguineous. Therefore two children were affected by a progressive arthropathy with deformity in all finger joints, restricted joint mobility and broad major joints. This condition was diagnosed like atypical juvenile rheumatoid arthritis (JRA) because the test for serum rheumatoid factors and antibodies were negative and failed to respond to anti-rheumatoid treatment. However their radiographic studies showed the spine with universal platyspondyly, enlargement epiphyses of the hands, the absence of destructive and the presence of the dysplastic bone changes. These manifestations permit us to do the diagnosis of spondyloepiphyseal dysplasia tarda with progressive arthropathy. In this report we suggest that a complete radiologic study of the patient will allowed to diagnosis this hereditary autosomal recessive entity; likewise it will let us differ of JRA and others polyarticular conditions of childhood.

[Ultrasonographic findings in hepatoblastoma]. / Hallazgos ultrasonográficos en hepatoblastoma.

At de National Institute of Pediatrics in Mexico City, the ultrasonographic findings of hepatoblastomas were analyzed in order to establish the echographic pattern of this malignant liver tumor. The ultrasonic images of patient's livers dating back six years were retrospectively reviewed. The hyperrecognisance of the hepatoblastoma as a solid, poorly limited mass, with or without the presence of calcifications, with the displacement of the portal vein and frequently found in the right lobe, were considered as the most relevant echographic characteristics of the hepatoblastoma. The echographic characteristics found of the hepatoblastoma can aid in the sonographic diagnosis of the disease. The leukemic liver infiltrations, the primary liver lymphoma and the metastasis, due to their hyperrecognisance, can be confused with the diagnosis of hepatoblastoma.

[Surgical treatment of pleuropulmonary infection in children]. / Tratamiento quirúrgico de la infección pleuropulmonar en el niño

Pleural empyema is most frequently seen in surgical pathology of the chest at the Hospital del Niño IMAN. Decortication has been necessary, with or without pulmonary resection, in 58% of the cases, contrasting with the limited number of cases in series published in countries with a high standard of life and where a conservative criterion for treatment is held by the authors and some national pediatric groups. Thus, a retrospective analysis is made with 118 patients who underwent surgery between 1971 and 1976 with ages ranging from 3 months to 17 years. The strict management procedure followed in all cases in mentioned, as well as the criterion to perform thoracotomy, to establish the irreversible nature of the anatomical lesions and to evaluate the results. 114 patients were discharged following recovery; 11 of these showed complications such as bronchopleural fistula, atelectasis and postoperative fever, which were treated with pleural drainage and bronchoscopy. Four patients died: one from upper gastrointestinal bleeding and three from sepsis. With the clinical and radiological, as well as the bronchoscopic evaluation, the destructive and irreversible nature of the lesions was determined. This was confirmed by the histopathological study of the resected parts. It is concluded that there should be no doubts or disagreements when surgical treatment is indicated.

[Intraspinal tumors in children. Review of 24 cases]. / Tumores intraespinales en los niños. Revisión de 24 casos

The clinical cases of 24 patients with intraspinal neoplasms seen at the Neurology and Neurosurgery ward of the Hospital del Nino IMAN, in a period of four and a half years are reviewed. The majority of our cases were diagnosed as suffering from tumors of embryological origin like teratomas, dermoid cysts and neuroblastomas. The signs and symptoms were reviewed, noting the importance of weakness of the lower extremities, the alteration in reflexes and sensitivity as well as back pain. We emphasize the great help of the X ray examination of the spinal colum, both plain and with contrast media. The treatment that our patients underwent was reviewed and the results were evaluated, pointing out the fact that life prognosis is related to malignancy of the lesions and the neurological sequelae are intimatly related with the early diagnosis and treatment of this type of lesions.