眼眶硬化性骨髓外造血细胞瘤

骨髓外造血细胞瘤可发生于包括骨髓纤维化、慢性骨髓性门血病和非常少见的红细胞增多症等骨髓增殖紊乱时。当纤维巨细胞增值伴有坚硬肿块形成时，该区域被称为硬化性骨髓外造血细胞瘤（SEMHTs），据我们所知．我们描述的是第一例发生于双侧眼眶SEMHTs的多发性骨髓纤维变中性。     

Intracystic Breast Carcinoma with Giant Axillary Metastasis: A Case Report

Abstract: Cystic breast disease is the most frequent cause of benign breast masses. While breast cysts are common, intracystic lesions, especially intracystic carcinomas, are rare. Furthermore, while breast carcinoma is the most common cancer in women, intracystic breast cancers are rare. Most intracystic breast carcinomas have been described as either papillary or medullary subtypes with infiltrating ductal carcinomas being much less common. The literature has shown their incidence to range from 0.3% to 7% of all breast carcinomas. We describe an interesting case of a premenopausal woman with a large cystic breast carcinoma and review the literature.     

Predictors of smoking initiation and cessation among childhood cancer survivors: a report from the childhood cancer survivor study.

PURPOSE: To examine the determinants of smoking behavior among participants in the Childhood Cancer Survivors Study (CCSS). METHODS: This retrospective cohort survey study was conducted among 9,709 childhood cancer survivors. Main outcomes included smoking initiation and cessation. RESULTS: Twenty-eight percent of patients reported ever smoking and 17% reported being current smokers. Standardized to United States population rates, the observed to expected (O/E) ratios and corresponding 95% confidence limits (95% CL) of cigarette smoking were 0.72 (95% CL, 0.69, 0.75) among all survivors and 0.71 (95% CL, 0.68 to 0.74) and 0.81 (95% CL, 0.70, 0.93) among whites and nonwhites, respectively. Significantly lower O/E ratios were present among both males (O/E, 0.73) and females (O/E, 0.70). Factors independently associated with a statistically significant relative risk of smoking initiation included older age at cancer diagnosis, lower household income, less education, not having had pulmonary-related cancer treatment, and not having had brain radiation. Blacks were less likely to start smoking. Survivors who smoked were significantly more likely to quit (O/E, 1.22; 95% CL, 1.15, 1.30). Among ever-smokers, factors associated with the likelihood of being a current smoker included age less than 13 years at smoking initiation, less education, and having had brain radiation; those age less than 3 years at cancer diagnosis were significantly more likely to be ex-smokers. CONCLUSIONS: Although survivors in the CCSS cohort seem to be smoking at rates below the general population, interventions are needed to prevent smoking initiation and promote cessation in this distinct population.     

Multiple gastrointestinal atresias with cystic dilatation of the biliary duct

A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome. Accepted: 19 April 1999     

Primitive neuroectodermal tumor of the diaphragm: case report

We present a case of primitive neuroectodermal tumor (PNET) arising from the diaphragm in a neonate. PNETs are rare malignant tumors that belong to the group of small, round, blue-cell neoplasms of childhood. To the best of our knowledge, a PNET originating from the diaphragm has not been previously reported. Received: 10 March 2000/Accepted: 11 May 2000     

Leukocytosis in children with Escherichia coli O157:H7 enteritis developing the hemolytic-uremic syndrome

BACKGROUND: Fewer than 10% of children with Escherichia coli O157:H7 enteritis develop hemolytic-uremic syndrome (HUS). OBJECTIVE: To determine whether circulating leukocytes are independent risk markers of developing HUS during E. coli O157:H7 enteritis. METHODS: We reviewed the charts of all children with culture-proved E. coli O157:H7 infections seen at Sainte-Justine Hospital between 1987 and 1997. Epidemiologic data, laboratory indices and circulating leukocytes counts were noted. HUS diagnosis was validated with independent HUS patient lists from the pediatric nephrology services of tertiary care hospitals in the Montreal metropolitan area. The date of onset of enteritis was determined by two independent observers. Leukocyte counts were compared among the following independent groups: (1) uncomplicated O157:H7 enteritis (Group 1); (2) O157:H7 enteritis with the subsequent development of HUS (Group 2); (3) HUS already present at the time of medical consultation (Group 3). RESULTS: There were 369 children with E. coli O157:H7 infection. A complete blood count was not performed in 114 (31%) patients. Observers disagreed on the date of onset of gastroenteritis in 34 (9%) children only (kappa 0.92). The study population thus included 221 patients: Group 1, n = 161; Group 2, n = 27; and Group 3, n = 33. Patients developing HUS (Group 2) presented greater total leukocyte (P < 0.008), polymorphonuclear (P < 0.008) and monocyte (P < 0.07) counts than those with an uncomplicated course (Group 1). Logistic regression analysis showed that young age [odds ratio (OR), 0.98; 95% confidence interval (CI), 0.96 to 0.99], duration of enteric prodrome < or =3 days (OR 4.8, 95% CI 1.13 to 20.7) and initial leukocytosis (OR 1.22, 95% CI, 1.11 to 1.35) were independent predictors of HUS. CONCLUSIONS: Based on the variables identified above, further studies are needed to determine whether the inflammatory response of the host represents only a marker of the severity of gastrointestinal infection or whether, alternatively, it is a pathophysiologic factor that leads to HUS.     

Changes in aromatic characteristics of Loureiro and Alvarinho wines during maturation

Changes in volatiles during maturation in bottles of monovarietal Vinhos Verdes wines from Loureiro and Alvarinho grape varieties, were followed by chemical and sensory analyses. Young wines and wines matured for 8 and 20 months were studied. The volatiles were determined by gas chromatography–mass spectrometry (GC-MS) after extraction on XAD-2 resin. Straight chain fatty acid ethyl esters and acetates of fusel alcohols decreased quicker for Loureiro wine, while the increase in ethyl esters of branched fatty acids was similar for both varieties. Linalool, Ho-trienol, α-terpineol and β-damascenone could be used to differentiate between each variety. However, linalool decreased to negligible values after 20 months of maturation. β-Damascenone decreased but remained high enough to be useful for differentiating each variety. Sensory analysis indicated a decrease of tropical fruit and tree fruit characters with conservation time for Alvarinho wine, and the opposite for Loureiro; moreover, citrus fruit character decreased in both varieties.