Intracranial calcifications in childhood medulloblastoma: relation to nevoid basal cell carcinoma syndrome.
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BACKGROUND AND PURPOSE: Medulloblastoma is one of the most common posterior fossa tumors to occur in children. Our purpose was to document the frequency, location, and time of occurrence of intracranial calcifications in cranial CT studies of children with medulloblastoma. METHODS: We retrospectively reviewed cranial CT studies of 56 patients diagnosed with medulloblastoma from 1983 through 1997 for the presence of intracranial calcifications. The findings were compared with 159 cranial CT studies of patients who were evaluated in the emergency department (control group). Thirty-two patients with medulloblastoma without shunts were compared with 118 patients from the control group without shunts. Similarly, 24 patients with medulloblastoma with shunts were compared with 41 patients from the control group with shunts. RESULTS: Overall, three (9%) patients with medulloblastoma without shunts, four (16%) patients with medulloblastoma with shunts, and four (10%) patients from the control group with shunts had falx calcification. Only the two children carrying the diagnoses of medulloblastoma and nevoid basal cell carcinoma syndrome, however, had calcification of the falx cerebri shown on the cranial CT scans obtained during the peridiagnostic period. Both were diagnosed with medulloblastoma before the age of 3 years and later developed jaw cysts and multiple basal cell carcinomas in the radiation field. CONCLUSION: Previous studies have shown that falx calcification is a major component of nevoid basal cell carcinoma syndrome. Our two cases illustrate the importance of considering the diagnosis of nevoid basal cell carcinoma syndrome when falx calcification is present in young patients with medulloblastoma. If the concomitant diagnosis of nevoid basal cell carcinoma syndrome is made, alternative types of therapy should be sought to minimize radiation therapy sequelae. (+info)
Pulmonary calcifications: a review.
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Pulmonary calcification is a common asymptomatic finding, usually discovered on routine chest X-ray or at autopsy. Pulmonary calcifications are caused mainly by two mechanisms: the dystrophic form and the metastatic form (1). Despite the different aetiologies, the pulmonary function and clinical manifestations are quite similar in both forms. We present a review of the clinical and radiology findings of the different aspects of pulmonary calcifications according to its pathogenesis and its anatomic distribution: parenchymal, lymphe node and pleural. (+info)
Secondary anterior crocodile shagreen of Vogt.
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The clincopathological features and pathogenesis of secondary mosaic degeneration of the cornea (anterior crocodile shagreen of Vogt) are described. The structural basis for the normal anterior corneal mosaic pattern seems to lie in the particular arrangement of many prominent collagen lamellae of the anterior stroma that thake an oblique course to gain insertion into Bowman's layer. Since, at normal intraocular pressure, Bowman's layer is under tension, when viewed from the anterior surface the cornea appears smooth. By releasing the tension, however, a reproducible polygonal ridge pattern becomes manifest. It is suggested that a prolonged phthisical state of the eye is one condition wherein the mosaic pattern may become permanent and that, as a secondary event, this is followed by irregular calcification of Bowman's layer which particularly involves the ridges projecting into the epithelium. Biomicroscopically these ridges corresponded to the branching reticular arrangement of the mosaic opacities. (+info)
A British family with herediatary pancreatitis.
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A family with hereditary pancreatitis is described. Nine family members definitely have had pancreatitis, whilst 15 more are suspected of having the disease. The condition presents as recurrent attacks of epigastric or central abdominal pain, sometimes radiating to the back, often associated with vomiting. The attacks of pain usually last three to four days. The inheritance fits well with an autosomal dominant pattern with limited penetrance, as it does in other families described in the literature. There is no aminoaciduria as has been described in some previously reported families. The attacks of pain start in childhood or young adult life (mean age of onset inthis family is 12-6 years) and appear to cease in this family by the age of 40 years. The diagnosis of pancreatitis in members of the family who have had confirmed pancreatitis was made by finding a raised serum amylase concentration in four cases, at laparotomy in four cases, and by pancreatic calcification seen on radiography in one case, The literature on the condition is reviewed, and it is speculated that the condition may have been underdiagnosed in Britain. (+info)
Effects of massive doses of ergocalciferol plus cholesterol on pregnant rats and their offspring.
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Ergocalciferol (320,000 or 480,000 IU/kg) plus cholesterol (60 mg/kg) in olive oil solution was administered daily on 1, 2, or 4 consecutive days to pregnant rats from 9,10, 14, or 18 of gestation. The control animals received only olive oil. Disseminated lesions of metastic calcinosis were found in various tissues, in the coronary arteries and myocardium, in the media of the abnormal aorta, in the lung and pleura, in the gastoinstestinal tract, and in the kidney. This is in contrast to the atherosclerosis described in nonpregnant rats fed a similiar diet. A significant decline in maternal weight as well as a high rate of morbidity and mortality was observed. In mothers killed on day 22 of pregnancy, fetal and placental growths appeared significantly retarded suggesting a direct effect of the steroid or its more active metabolite, 1,25-dihydroxycholecalciferol, on the fetus or the trophoblastic tissue. Fetal bone lesionsassociated with a generalized retardation of ossification, placental edema, or calcification accompanied by a loss of the normal structure of the placenta and degenerative manifestation at this level were observed. Moreover, we noted a striking alteration of the fetal face in 33-39% of experimental fetuses, called by us carnival fetuses. (+info)
Radiological abnormalities in temporal lobe epilepsy with clinicopathological correlations.
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In 73 patients with drug-resistant temporal lobe epilepsy submitted to an unilateral anterior temporal lobectomy the radiographs were studied to see if there were any correlation with the pathology subsequently found and with the outcome of the operation. A small middle cranial fossa, focal calcification, and temporal horn displacement are often better indices of the underlying pathology than temporal horn dilatation alone. In a small number of cases, however, radiological changes were seen on the side opposite to an unilateral EEG focus, thus suggesting bilateral disease. (+info)
Coronary-artery calcification in young adults with end-stage renal disease who are undergoing dialysis.
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BACKGROUND: Cardiovascular disease is common in older adults with end-stage renal disease who are undergoing regular dialysis, but little is known about the prevalence and extent of cardiovascular disease in children and young adults with end-stage renal disease. METHODS: We used electron-beam computed tomography (CT) to screen for coronary-artery calcification in 39 young patients with end-stage renal disease who were undergoing dialysis (mean [+/-SD] age, 19+/-7 years; range, 7 to 30) and 60 normal subjects 20 to 30 years of age. In those with evidence of calcification on CT scanning, we determined its extent. The results were correlated with the patients' clinical characteristics, serum calcium and phosphorus concentrations, and other biochemical variables. RESULTS: None of the 23 patients who were younger than 20 years of age had evidence of coronary-artery calcification, but it was present in 14 of the 16 patients who were 20 to 30 years old. Among those with calcification, the mean calcification score was 1157+/-1996, and the median score was 297. By contrast, only 3 of the 60 normal subjects had calcification. As compared with the patients without coronary-artery calcification, those with calcification were older (26+/-3 vs. 15+/-5 years, P<0.001) and had been undergoing dialysis for a longer period (14+/-5 vs. 4+/-4 years, P< 0.001). The mean serum phosphorus concentration, the mean calcium-phosphorus ion product in serum, and the daily intake of calcium were higher among the patients with coronary-artery calcification. Among 10 patients with calcification who underwent follow-up CT scanning, the calcification score nearly doubled (from 125+/-104 to 249+/-216, P=0.02) over a mean period of 20+/-3 months. CONCLUSIONS: Coronary-artery calcification is common and progressive in young adults with end-stage renal disease who are undergoing dialysis. (+info)
Evaluation of ocular tumors with technetium-99m-MIBI: planar pinhole technique or SPECT?
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OBJECTIVE: This study compares 2 imaging protocols, planar pinhole technique (PPHT) and SPECT, for evaluating ocular masses with 99mTc-MIBI. METHODS: Sixteen patients with ocular lesions were studied. Planar images were acquired 10 min after the injection of 740 MBq 99mTc-MIBI with an LFOV camera fitted with a pinhole collimator (5.0 mm). A SPECT study was performed immediately after the planar study, using a 360 degrees orbit, 64 steps, 20 s/stop, a 128 x 128 matrix, and a low-energy high-resolution (LEHR) collimator. Twelve lesions (9.5-18.0 mm) proved to be malignant: 8 primary tumors (ocular melanoma); 3 local relapses of different tumors of the conjunctiva; and 1 ocular metastasis from breast cancer. The remaining 4 lesions (10.0-16.0 mm) were benign: 1 inflammatory lesion; 1 benign intraocular calcification; and 2 naevi. RESULTS: SPECT images showed 11 of 12 malignant lesions (91.6%), whereas the planar technique demonstrated only 4 of the 12 lesions (33.3%). One false-positive result, the inflammatory lesion, was visualized by both techniques. The remaining benign lesions were not detected with either method. CONCLUSION: Technetium-99m-MIBI SPECT is a sensitive technique for detecting malignant ocular tumors. SPECT imaging is a better alternative to planar imaging for ocular tumors. (+info)