Cholesteryl ester storage disease. Report of a case.

Cholesteryl ester storage disease (CESD) is a rare disorder of familial incidence characterized by the accumulation of cholesteryl ester and triglycerides in the liver, intestine and bone marrow. Until now only 21 cases have been reported in the literature. We present a 9 months old girl presenting with increased abdominal girth. She had normal liver function tests and increased cholesterol and triglycerides serum levels. The liver biopsy showed many cholesterol cristals seen as needle shaped cristals under polarized light. This is the youngest patient being diagnosed clinically in the literature.

Características clínicas e implicações prognósticas do acometimento renal no mieloma múltiplo.

The renal involvement in patients with multiple myeloma has been described as a sign of poor prognosis. The influence of renal insufficiency in the clinical patterns and in the prognosis of patients with multiple myeloma was studied retrospectively in 45 patients. Patients with renal insufficiency, at first visit, more often presented weight loss, proteinuria, hypercalcemia. The means of uricemia, ESR, were higher and the hematocritic mean was lower in patients with renal insufficiency. There was no difference in edema, arterial hypertension, fractures and bone pain. The reversibility of renal insufficiency occurred in 47% of the cases, which happened more often in the first months of the follow up. The creatinine mean was lower in patients with reversible renal insufficiency. The median survival was: patients with renal insufficiency: 11 months; patients with normal renal function: 50 months. Among patients with renal insufficiency those with recuperation of renal function showed a higher median survival (24 months) than those with irreversible renal insufficiency (1 month). The renal involvement then is frequent and often reversible. Patients with impaired renal function showed a worse prognosis; normalization of the renal function was associated with a better outcome.

Immunohistochemical characterisation of probable intravascular haematopoiesis in the vasa rectae of the renal medulla in acute tubular necrosis

Immunohistochemistry was applied to identify the nature of the nucleated cells that accumulate in the vasa rectae of the corticomedullary junction in acute tubular necrosis. In all 6 cases studied, there were intravascular cells that reacted with monoclonal antibodies to erythroblast, macrophages, myeloid cells, T and B lymphocytes and rare megakaryocytes. The findings are consistent with the occurrence of intravascular haematopoiesis in the renal medulia in acute tubular necrosis.

Insuficiência renal aguda. Estudo do quadro clínico e prognóstico.

Acute renal failure (ARF) is a frequent complication in hospitalized patients, and is strongly related to increase of mortality. PURPOSE: To analyze the clinical outcome and the prognostic factors in hospital acquired AFR. METHOD: A prospective study was performed. Data from 200 patients with established ARF admitted during the period of January, 1987 and July, 1990 were collected. RESULTS: The incidence of ARF was 4.9/1000 admissions. Renal ischemia (50%) and nephrotoxic drugs (21%) were the main etiologic factors. The histologic study done in 43 patients showed: acute tubular necrosis (53%), tubular hydrophic degeneration (16%), glomerulopathies (16%) and other lesions (15%). Dialysis therapy was performed in 101 patients and the main indications were: uremia (67%), hypervolemia (22%) and hyperkalemia (9%). The mortality rate was 46.5% and the most important causes of death were: sepsis (38%), respiratory failure (19%) and multiple organs failure (11%). Treatment withdraw was the cause of death in 2 patients. Higher mortality was observed in oliguric patients (62.9%) than non-oliguric (34.5%) (p < 0.05) and in ischemic renal failure (56.7%) when compared to nephrotoxic renal failure (14.7%) (p < 0.05). This difference was maintained when the comparison was done only between dialyzed patients. CONCLUSION: As primary cause of death was not associated to the acute renal failure, we conclude that acute renal failure is an important marker of the gravity of the underlying disease and not the cause of death.

Acute renal failure in renal allograft recipients and patients with native kidneys

In order to evaluate the role of underlying disease in the high mortality observed in acute renal failure (ARF) and risk factors related to the development of oliguric ARF in renal allograft recipients, two groups were selected: 34 patients with native kidneys, aged 16 and 57 years, and presenting ischemic ARF caused by cardiovascular collapse, with no signs of infection at the time of diagnosis; and 34 renal allograft recipients who developed ARF immediately after transplantation, without rejection. ARF was defined either as 30% increase of basal plasmatic creatinine in patients with native kidneys or non-normalization of plasmatic creatinine at day 5 after transplantation in renal allograft recipients; oliguria as diuresis ≤ 400 mL/24 h. There were no differences in age, male frequency, oliguria presence and duration, need for dialysis, and infection episodes for renal allograft recipients and patients with native kidneys. The development of sepsis (3% and 41%) and death rate (3% and 44%) were higher in patients with native kidneys (p < 0.01). The renal allograft recipients with both oliguric (n = 18) and nonoliguric (n = 16) ARF were evaluated and no difference was observed in the recipient's age, donor's age, cold ischemia time, time elapsed until plasmatic creatinine normalization, donor's plasmatic creatinine or urea, and mean arterial pressure. No differences were observed between the groups regarding frequency of infection episodes during ARF and frequency of death. In conclusion, renal allograft recipients presented a lower death rate and were less susceptible to sepsis. Cold ischemia time, age, and hemodynamic characteristics of the donor did not affect the development of oliguria.

The silent period in patients with chronic renal failure undergoing hemodialysis

Silent period was evaluated in 20 adult male patients with chronic renal failure undergoing hemodialysis. Readings were obtained by supramaximal stimulus to the median nerve, during maximum isometric effort of the abductor pollicis brevis muscle against resistance. Two types of abnormalities were observed, motor neuron hypoexcitability with elongated silent period, and motor neuron hyperexcitability with reduction or absence of silent period. Some abnormalities are probably linked with dialysis duration, but show no correlation to presence or absence of peripheral neuropathy. The silent period alterations described in this study could possibly correlate with some other clinical feature frequently seen in patients with chronic renal failure such as hypereflexia of the deep tendon reflexes.

Effects of simvastatin on bone regeneration in the mandibles of ovariectomized rats and on blood cholesterol levels.

The purpose of this study was to evaluate the effects of simvastatin on guided bone regeneration in the mandibles of ovariectomized rats, and to observe their blood cholesterol levels. Seventy female rats were divided into two groups: control and treated, both groups containing normal and ovariectomized rats. A month after ovariectomy a bone defect was created in the mandible, and was covered by a polytetrafluoroethylene membrane. The treated groups received simvastatin orally for 15 or 30 days. The rats were sacrificed 15, 30 or 60 days after surgery, at which time a blood sample was extracted for blood cholesterol level analysis and the mandible was extracted for densitometric, histological and morphometric analysis. All specimens underwent analysis of variance. The ovariectomized animals had higher cholesterol levels than the treated normal animals, and no significant difference was found between the different treatment periods and the sacrifice times. The densitometric, histological and morphometric analysis showed that the treated ovariectomized animals developed more new bone than the control ovariectomized rats, but no significant difference was observed between the treatment periods. It can be concluded that the deficiency of estrogen increased the level of blood cholesterol and that the simvastatin aided new bone formation in the ovariectomized animals.

Chronic renal failure in male and female rats

Gender influences the progression of chronic renal failure (CRF). We studied male (M) and female (F) Wistar rats for 90 days: castrated (CMc, n=7; CFc, n=6) and non castrated controls (CM, n=9; CF, n=6); castrated (CRFMc, n=8; CRFFc, n=6) and non castrated animals submitted to 5/6 nephrectomy (CRFM, n=13; CRFF, n=6). Data are expressed as mean ± SEM. Proteinuria (PTN) was higher in CRFM (554 ± 69mg/24h) compared to CRFMc (277 ± 85 mg/24h), but not in females (CRFF=193 ± 20mg/24h, CRFFc=164 ± 71mg/24h). Mesangial fractional volume increased in all CRF animals. CRF animals showed an increase of glomerular sclerosis index (GSI) and tubulointerstitial damage (TID) but in a smaller proportion in male castrated animals; the opposite occurred with females: castration induced an increase of these parameters. CRF animals showed increased cortical and glomerular fibronectin (FN) rates. Castration decreased glomerular and cortical FN rates in CRFM but not in females. In conclusion, proteinuria was higher in CRFM and probably led to glomerular and interstitial damage, as well as to FN accumulation, castration seems to protect against development of PTN, TID and FN accumulation in males. Castrated female rats presented mesangial expansion, with no changes in PTN, TID and FN rates. It seems that female sex hormones do not protect against renal disease progression, instead, we suggest that male sex hormones lead to acceleration of CRF.

Cocaine associated with onlay bone graft failure: A clinical and histologic report

This patient report presents an unusual onlay bone graft failure following local cocaine application. Three months after the bone grafting procedure performed in the anterior maxilla for bone volume augmentation, the bone graft was totally exposed in the oral cavity as a result of the rubbing of cocaine on the gingival tissue that covered the bone graft. A histologic view of the removed bone fragment presented not only an area of necrosis but also ample spaces filled with necrosis material and resorption areas. Dental practitioners need to be aware of this phenomenon because such patients often do not report the use of drugs, particularly cocaine. Copyright © 2005 by Lippincott Williams & Wilkins.

Efeitos do extrato aquoso de cebola (Allium cepa L.) sobre a função renal e a pressão arterial em ratos Wistar

The vegetal species, Allium cepa, known as onion, is widely used in the folk medicine as diuretic, besides it has been used on the bronchitis, cough, cardiovascular diseases and hypertension treatment. In this study we evaluate the onion aqueous extract (AE) effect on water flow and electrolytes in anesthetized Wistar rats, besides we also evaluate arterial pressure alterations. Two groups were studied: Group 1 (control) - oral tratment with 1.0 mL of distilled water, and Group 2 (experimental) - oral treatment with 1.0 mL of AE 20%. The rats were anesthetized and we canulate the trachea, left carotide artery (for arterial pressure measurement and blood collecting), jugular vein (to execute inulin perfusion - to register glomerular filtration), and urinary bladder (to collect urine). The Group 1 results had shown that the animals had not presented significant alterations (p>0.05) in the analyzed parameters. The animals of Group 2 had a significant reduction (p<0.05) in the arterial pressure (22.0%). However, there were not significant alterations in renal parameters (p>0.05). These results show that the treatment with the AE lead a hypotensor effect in anesthetized Wistar rats, but not followed by renal parameters alterations.

Autogenous bone graft with or without a calcium sulfate barrier in the treatment of class II furcation defects: A histologic and histometric study in dogs

Background: The purpose of this study was to histologically evaluate the healing of surgically created Class II furcation defects treated using an autogenous bone (AB) graft with or without a calcium sulfate (CS) barrier. Methods: The second, third, and fourth mandibular premolars (P2, P3, and P4) of six mongrel dogs were used in this study. Class II furcation defects (5 mm in height × 2 mm in depth) were surgically created and immediately treated. Teeth were randomly divided into three groups: group C (control), in which the defect was filled with blood clot; group AB, in which the defect was filled with AB graft; and group AB/CS, in which the defect was filled with AB graft and covered by a CS barrier. Elaps were repositioned to cover all defects. The animals were euthanized 90 days post-surgery. Mesio-distal serial sections were obtained and stained with either hematoxylin and eosin or Masson's trichrome. Histometric, using image-analysis software, and histologic analyses were performed. Linear and area measurements of periodontal healing were evaluated and calculated as a percentage of the original defect. Percentage data were transformed into arccosine for statistical analysis (analysis of variance; P<0.05). Results: Periodontal regeneration in the three groups was similar. Regeneration of bone and connective tissue in the furcation defects was incomplete in most of the specimens. Statistically significant differences were not found in any of the evaluated parameters among the groups. Conclusion: Periodontal healing was similar using surgical debridement alone, AB graft, or AB graft with a CS barrier in the treatment of Class II furcation defects.

Comparative therapeutic use of Risedronate and Calcarea phosphorica - Allopathy versus homeopathy - In bone repair in castrated rats

Osteoporosis, a disease characterized by progressive bone loss, has been the target of several studies in the past few years. It results in a much higher risk for fractures and might cause slower bone lesion healing. The aim of this work was to study the effects of Risedronate (allopathic medicine) and Calcarea phosphorica 6CH (homeopathic medicine) on the repair of bone lesions in male rats with osteoporosis induced by castration. Eighty-four three-month-old rats were used divided into four groups of twenty-one animals each. Three groups where castrated and one group was submitted to Sham surgery. One month later, cortical lesions were made in all animals' tibiae and, after one day, the different experimental treatments began according to the following groups: CR - castrated/Risedronate (1 mg/kg/day); CCp - castrated/ Calcarea phosphorica 6CH (3 drops/day); CP - castrated/placebo and SP - Sham/placebo. The animals were sacrificed at seven, fourteen and twenty-eight days after the beginning of the treatments and had their tibiae removed. Digital radiographs of the tibiae were taken and analyzed in order to evaluate the optical density of the defect area. Then, they were decalcified and processed for histological and histomorphometrical analysis. The data were submitted to ANOVA, and to the Tukey and Dunnett tests (5%). The allopathic and homeopathic treatments led to different bone formation as regards remodeling and maturation aspects. Further research is necessary to access the resistance and quality of the newly formed bone.

Prosthetic rehabilitation of a bone defect with a teeth-implant supported, removable partial denture

The use of teeth-implant, mucosa-supported removable dentures for rehabilitation of partially edentulous patients involves highly complex biomechanical aspects. This type of prosthesis associates 3 kinds of support that react differently to the functional and parafunctional forces developed in the oral cavity. Although the construction of removable partial dentures may seem paradoxical when osseointegrated implants are placed, in some cases, this option is an excellent alternative to solve difficulties related to the anatomic, biologic, psychomotor, and financial conditions of the patient. This article reports on a case in which a teeth-implant, mucosa-supported removable partial denture was the option of choice for a patient with financial and anatomic limitations, having a large structural loss of the residual alveolar ridge caused by trauma by a gunshot injury at the mandible. The 5-year follow-up did not reveal any type of biomechanical or functional problem. Copyright © 2006 by Lippincott Williams and Wilkins.

Influence of image filters on the reproducibility of measurements of alveolar bone loss

The reproducibility of measurements of alveolar bone loss on radiographs may be a problem on epidemiologic studies, as they are based on comparisons of the diagnosis of various examiners. The aim of the present research paper was to assess the inter- and intra-examiner reproducibility of measurements of the interproximal alveolar bone loss on non-manipulated digital radiographs and after the application of image filters. Five Oral Radiologists measured the distance between the cementoenamel junction (CEJ) to the alveolar crest or to the deepest point of the bony defect on 12 interproximal digital radiographs of molars and bicuspids of a dry human skull. The digital manipulation and the linear measurements were obtained with the Trophy Windows software (Throphy®). For each image, six different versions were created: 1) non-manipulated; 2) bright-contrast adjustment; 3) negative; 4) negative with brightness-contrast adjustment; 5) pseudo-colored; 6) pseudo-colored with brightness-contrast adjustment. In order to prevent interpretation bias because of the repetition of measurements, the examiners measured the radiographs in a random sequence. The two-way ANOVA test at 5% level of significance to compare the means of readings of the same operator with each filter indicated p<0.05 for the majority of operators, while the comparison between the mean values of operators using the same filter indicated p>0.05 for all filters. Based on the results, we concluded that linear measurements of interproximal alveolar bone loss on digital radiographs are highly reproducible among examiners. Nevertheless, the application of image filters significantly influenced the degree of intra-examiner reproducibility. Some filters even reduced the reproducibility of intra-examiner readings.

The influence of ovariectomy, simvastatin and sodium alendronate on alveolar bone in rats

Bisphosphonates are currently used in the treatment of many diseases involving increased bone resorption such as osteoporosis. Statins have been widely used for the treatment of hypercholesterolemia and recent studies have shown that these drugs are also capable of stimulating bone formation. The purpose of this study was to evaluate thel influence of an estrogen deficient state and the effects of simvastatin and sodium alendronate therapies on alveolar bone in female rats. Fifty-four rats were either ovariectomized (OVX) or sham operated. A month later, the animals began to receive a daily dose of simvastatin (SIN - 25 mg/kg), sodium alendronate (ALN - 2 mg/kg) or water (control) orally. Thirty-five days after the beginning of the treatment, the rats were sacrificed and their left hemimandibles were removed and radiographed using digital X-ray equipment. The alveolar radiographic density under the first molar was determined with gray-level scaling and the values were submitted to analysis of variance (α = 5%). Ovariectomized rats gained more weight (mean ± standard deviation: 20.06 ± 6.68%) than did the sham operated animals (12.13 ± 5.63%). Alveolar radiographic density values, expressed as gray levels, were lowest in the OVX-water group (183.49 ± 6.47), and differed significantly from those observed for the groups receiving alendronate (sham-ALN: 193.85 ± 3.81; OVX-ALN: 196.06 ± 5.11) and from those of the sham-water group (193.66 ± 4.36). Other comparisons between groups did not show significant differences. It was concluded that the ovariectomy reduced alveolar bone density and that alendronate was efficient for the treatment of this condition.