Risk factors for decreased total body and regional bone mineral density in hemodialysis patients with severe secondary hyperparathyroidism.

Hyperparathyroidism contributes significantly to decreased bone mineral density (BMD) in end-stage renal disease patients, but this negative influence is not homogeneous throughout the skeleton. We studied the BMD by dual-energy X-ray absorptiometry on total body and on different regions of the skeleton in 42 patients with severe hyperparathyroidism on hemodialysis. We also evaluated the relationship between different risk factors and BMD found on the regions examined in these patients. The legs and other sites where cortical bone predominate were mostly affected, whereas trabecular bone was relatively preserved. This is probably the result of the different effects of hyperparathyroidism on cortical and trabecular bone, but we cannot rule out the interference of ectopic calcifications and sclerotic lesions of vertebral end-plates falsely increasing lumbar spine BMD. The main determinants of low total-body BMD were, in order of importance, immobility, high intact parathyroid hormone levels, low body mass index, and low albumin. Eleven patients presented with pathologic fractures, mainly in the legs, and BMD was lower in this group than in patients without fractures. In conclusion, our study makes clear that hyperparathyroidism is a great threat to bone density in hemodialysis patients, mainly in the legs, the site mostly affected by fragility fractures in our patients. Physicians must worry not only with high parathyroid hormone levels, but also with the nutritional state of these patients.     

A new method for the correction of secondary unilateral cleft lip nose

Summary In 1982, Dibbell described a procedure to correct this distortion by rotating and advancing the nostril medially and superiorly. We used this method in our department for several years, but as Dibbell himself experienced, we didn't always obtain satisfactory results. Like many authors, I agree that the best approach for proper correction of the secondary unilateral cleft lip nose deformity is the external approach using transcolumellar incisions, because the lower lateral cartilage exposure is inadequate with Dibbell's method. I added the transcolumellar incision similar to Bardach's to solve this problem. In this paper, this combination technique will be presented and contrasted with Dibbell's and Bardach's techniques.     

Distribution of α-integrin subunits in fetal polycystic kidney diseases

An alteration in cell/matrix interactions is one of the suggested mechanisms leading to cyst formation in polycystic kidney diseases. Most of these interactions are mediated by β1-integrins, a subfamily of integrin receptors, formed by the association of the β1-chain with different α-subunits. To date, no study on α-integrin subunit distribution during the early stages of cyst development has been reported. Using immunofluorescence, we analyzed the distribution of α-integrin subunits (α1, α2, α3, α5, and α6) and basement membrane proteins in kidneys of fetuses with autosomal dominant (ADPKD) or autosomal recessive polycystic kidney disease (ARPKD). The distribution was compared with that observed in normal fetal and post-natal kidneys, and in fetal cystic dysplasia and Meckel syndrome. Marked increase in α1-integrin staining was observed in normal and cystic collecting duct cells of both polycystic diseases (PKD), compared with normal and cystic controls. The distribution of integrin subunits α2, α3, and α6 was irregular in cyst epithelial cells of PKD and cystic controls. The increased expression of the α1-subunit specifically observed in PKD collecting duct cells may be an early consequence of the genetic defect in ARPKD. In ADPKD it parallels the reported expression of polycystin, the protein product of PKD1. The irregular expression of α2, α3, and α6 integrin subunits observed in all types of cysts suggests that cell/matrix interactions are altered early and may participate in the development of cysts, perhaps by contributing to the deregulation of cell survival in cystic diseases. Received May 28, 1996; received in revised form October 2, 1996; accepted October 25, 1996     

A fluid level in an acute extradural haematoma

We report a fluid level in an acute extradural haematoma developing after placement of a ventriculoperitoneal shunt for hydrocephalus. This fluid level was thought to be due to a mixture of blood and cerebrospinal fluid.     

ASSESSMENT OF THE PREVALENCE INDEX ON SIGNS OF COMBINATION SYNDROME IN PATIENTS TREATED AT BAURU SCHOOL OF DENTISTRY,UNIVERSITY OF SAO PAULO

A group of destructive changes occurring in jaws in patients with maxillary complete dentures and mandibular removable partial dentures (bilaterally) has been described in the literature as the combination syndrome. However, this condition is not clinically observed in all patients. The aim of this study was to establish the prevalence index on signs of combination syndrome and to verify whether these changes also occurred in patients rehabilitated with a mandibular removable partial denture (unilaterally). Sample was composed of 44 patients, completely edentulous in the maxilla. Thirty-two patients had a Kennedy Class I removable partial denture and 12 a Kennedy Class II. Three major alterations were observed in 20.5% of the studied population. Nevertheless, these changes were present only in 25% of patients with Kennedy Class I removable partial denture. Based on the findings of this study, it can be concluded that patients with Kennedy Class II removable partial denture do not have similar signs that lead to the combination syndrome’s condition.     

Laryngeal pleomorphic rhabdomyosarcoma

A case of an extremely unusual tumor of the larynx, pleomorphic rhabdomyosarcoma, is presented with a review of literature. This is the fifth case of this malignancy described in the larynx in the English language literature. A histopathological diagnosis was made with immunohistochemistry and electron microscopy. In contrast to other reported cases, the tumor in the present case had a very aggressive behavior. Despite radical surgery involving total laryngectomy and neck dissection followed by radiation therapy, the patient died of disease 8 months following treatment. Received: 14 March 1997 / Accepted: 23 January 1998