Studies of marginal zinc deprivation in rhesus monkeys. V. Fetal and infant skeletal effects

Skeletal maturation was evaluated in newborn and infant rhesus monkeys that had been subjected to a marginally zinc-deficient diet (4 ppm zinc) from conception through 12 months of postnatal life. Serial radiographic assessment of skeletal development was performed and compared to both ad libitum and pair-fed controls. Radiographs were obtained at birth and at 1, 3, 9, and 12 months of age. In each age group a maturation indicator was selected to identify individuals with abnormal skeletal maturation defined on the basis of presence of epiphyseal ossification centers. Animals were compared only within a given sex group. Additionally, to evaluate endochondral bone mineralization, the appearance of the zone of provisional calcification on the metaphyseal side of the growth plate and the width of the growth plate were assessed. A marginal level of zinc deprivation during gestation and during the 1st yr of life was found to be associated with significantly delayed skeletal maturation and defective mineralization. This abnormality of bone mineralization has many features similar to human rachitic syndromes and suggests that zinc plays an important role in endochondral bone formation.     

Cerebral Hereditary Haemorrbagic Telangiectasia Demonstrated Angiographically

The findings on cerebral angiography in a patient with hereditary haemorrhagic telangiectasia are described. These consisted of a telangiectatic vascular lesion with pooling of contrast and early venous filling. It is suggested that cerebral angiography should be carried out in a patient with Osier's disease and cerebral signs to exclude such a lesion.     

Evaluation and management of renal tumors in the Birt-Hogg-Dubé syndrome

PURPOSE: Herein we describe the evaluation and management of renal tumors in Birt-Hogg-Dubé (BHD), an autosomal dominant disorder predisposing to cutaneous fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax and renal tumors. MATERIALS AND METHODS: A total of 124 affected individuals underwent comprehensive clinical evaluation, including body computerized tomography, to determine cutaneous, pulmonary and renal manifestations of BHD. Of these individuals 14 had their renal tumors managed at our institution. RESULTS: Of the 124 BHD affected individuals 34 (27%) had renal tumors of various histologies, most commonly hybrid oncocytic tumor and chromophobe renal carcinoma. Average age at renal tumor detection was 50.4 years and multiple tumors were found in a majority of patients. Some patients with renal tumors were identified that did not have the characteristic cutaneous hallmarks of BHD. In 4 of the 14 patients treated at our institution small (less than 3 cm) renal tumors were observed, while 10 others underwent a total of 12 renal procedures, including 4 radical and 8 partial nephrectomies. At a median of 38 months of followup 5 of these 10 patients remained free of disease, 3 had small renal tumors and 2 died of metastatic renal cancer. CONCLUSIONS: Patients with BHD are at risk for multiple renal tumors that are often malignant and can metastasize. Individuals at risk or affected by BHD should be radiographically screened for renal tumors at periodic intervals and they are best treated with nephron sparing surgical approaches. Genetic testing for this syndrome is now available.     

Radiation-Induced Soft-Tissue Fibrosarcoma: Surgical Therapy and Salvage

Soft-tissue fibrosarcomas of the chest wall that occur after radiation therapy for breast carcinoma are rare and usually lethal. The histories of the 10 previously reported patients emphasize the dismal prognosis for untreated patients and suggest that surgical intervention offers the only hope of reasonable palliation. We report another patient, now alive and free from disease 3½ years after radical resection. The cases of these 11 patients are reviewed, with emphasis on the clinical presentation, the difficulty of making the diagnosis, and the significant palliation that may be achieved with radical chest wall resection.