Alkylglycerols reduce serum complement and plasma vascular endothelial growth factor in obese individuals.

Alkylglycerols (AKGs), isolated or present in shark liver oil have anti-inflammatory properties. Complement 3 (C3) and 4 (C4) participate in lipid metabolism and in obesity, contributing to the metabolic syndrome and to the low-grade inflammation associated with obesity. In a randomized, controlled, crossover study, 26 non-diabetes obese individuals were assigned two preparations with low (LAC, 10 mg AKGs) and high (HAC, 20 mg AKGs) AKG content. Intervention periods were of 3 weeks preceded by 2-week washout periods in which shark liver oil was avoided. Cholesterol, C3, C4, and vascular endothelial growth factor (VEGF) decreased in a linear trend (P < 0.01) from baseline (control) to LAC and HAC. Values after HAC were significantly lower (P < 0.05) versus both baseline and after LAC. No adverse effects were observed or reported. Data from this pilot study open a promising field for the study of the beneficial effects of AKGs on cardiovascular risk factors in obese individuals.

Antiproliferative effect of alkylglycerols as vehicles of butyric acid on colon cancer cells.

The anticarcinogenic activity of synthetic 1-O-octadecyl-2,3-dibutyroilglycerol (D-SCAKG) in tumor-cell line of colonocytes (SW620) was performed. The effect of the previously digested D-SCAKG under in vitro intestinal conditions was compared to the bioactivity of non-digested D-SCAKG. Antiproliferative activity of each individual product from digestion (1-O-octadecyl-2-butyroilglycerol; 1-O-octadecyl glycerol; butyric acid) was also performed. The impact of solubilization of lipid products within micellar structures was also tested. The 1-O-octadecyl glycerol was the most active compound, followed by 1-O-octadecyl-2-butyroilglycerol, D-SCAKG and butyric acid. The 1-O-octadecyl glycerol and butyric acid were the only molecules that showed antiproliferative effect in absence of micelles. Digested D-SCAKG was 4-fold more effective than non-digested D-SCAKG. A synergism between 1-O-octadecyl-2-butyroilglycerol and 1-O-octadecyl glycerol was evidenced. As summary, the synthetic D-SCAKG seems to be an interesting antitumoral lipid against colonocytes, especially after previous intestinal digestion, and mainly due to the synergism of the major products, namely 1-O-octadecyl-2-butyroilglycerol and 1-O-octadecyl glycerol. At the same time, 1-O-octadecyl-2-butyroilglycerol would constitute a stable esterified form of butyric acid for its vehiculization.

Clostridial sepsis: is death avoidable?

Massive intravascular hemolysis is a rare yet often fatal complication of clostridial sepsis. The only chance for survival is an early diagnosis and prompt initiation of treatment. We report a rapidly fatal case who developed electrocardiographic changes of acute myocardial injury. Autopsy showed gas-filled bubbles and cysts in the myocardium partially filled with sporulating bacilli with the morphology of clostridia. Gas filled bubbles were also present in the lungs, liver, kidneys and spleen. The gastric mucosa showed hemorrhagic and necrotizing changes, the probable site of entry of the infection

Clostridial sepsis: is death avoidable?

Massive intravascular hemolysis is a rare yet often fatal complication of clostridial sepsis. The only chance for survival is an early diagnosis and prompt initiation of treatment. We report a rapidly fatal case who developed electrocardiographic changes of acute myocardial injury. Autopsy showed gas-filled bubbles and cysts in the myocardium partially filled with sporulating bacilli with the morphology of clostridia. Gas filled bubbles were also present in the lungs, liver, kidneys and spleen. The gastric mucosa showed hemorrhagic and necrotizing changes, the probable site of entry of the infection.

Multiple ependymomas in a patient with Turcot's syndrome.

A 21-year-old woman was diagnosed with Turcot's syndrome (TS) at age 16 years. She had two ependymomas, one was located in the left middle cerebellar peduncle and the other in the low sacral spinal canal. Her mother and brother both had colectomies for colonic polyposis. Her maternal uncle and grandfather also had this disease and both died from cancer of the colon in their fourth decade of life. The patient was found to have hyperpigmented spots in the retina, skull osteomas and normal neurological examinations. The bone scan and CSF were normal and she had a germline mutation in the segment 3 of the adenomatous polyposis coli (APC) gene. Following partial resection of the two ependymomas, she was treated with radiation and chemotherapy. One year after surgery, paraspinal desmoid tumors were found and removed. She is presently 42 months postsurgical resection of the neural tumors and has remained central nervous system tumor-free. The occurrence of multiple ependymoma in TS has not been reported, and the control of this patient's ependymomas is consistent with other reports of long-term survival with TS and glial tumors.

Primary leptomeningeal histiocytic lymphoma in a young child.

A 20-month-old boy had an 8-week history of vomiting, lethargy, generalized muscle weakness, and seizures. There was no history or clinical signs of an underlying systemic disease or an immunodeficiency. Cerebrospinal fluid (CSF) had 99 nucleated cells/cu mm, malignant cells, high protein and normal glucose. CT and MRI scans showed diffuse meningeal enhancement around the brain and spinal cord, but no parenchymal involvement. Biopsy of the leptomeninges showed malignant cells with marked nuclear pleomorphism and prominent clear to eosinophilic cytoplasm. The immunohistochemical studies were positive for histiocyte-macrophage markers and were negative with T and B cells, Ki-1, neural and glial cell antibodies. Multiple tests revealed no other site of disease. The patient died 3 months after onset of treatment despite intensive i.v. and intrathecal chemotherapy. We have not found any other reported case of primary histiocytic leptomeningeal lymphoma in a young child.

Surveillance scanning of children with medulloblastoma.

BACKGROUND: The standard follow-up care for children with medulloblastoma includes regular clinical evaluations and surveillance scanning of the central nervous system with computed tomography or magnetic resonance imaging. The evaluations and scanning assess the response of the tumor to treatment, detect any recurrence of disease, and monitor any complications of treatment. We compared the effectiveness of a periodic history taking and physical examination with that of surveillance scanning in detecting recurrent tumors. METHODS: We reviewed the medical records, including 794 scanning reports or scans, of 86 children with posterior fossa medulloblastoma who were followed regularly between 1980 and 1991. Recurrent tumors were classified as symptomatic if neuroimaging studies had been prompted by clinical symptoms or signs and as radiographic if the tumor had been detected by imaging in an asymptomatic patient. RESULTS: Twenty-three of the 86 children (27 percent) had a recurrence of tumor. Four recurrences (17 percent) were detected on scanning only, and 19 (83 percent) were associated with symptoms arising a median of four months after the previous scan. The median and range of survival after a recurrence of the tumor were 5 months and < 1 to 38 months, respectively, for a symptomatic recurrence and 20 months and 10 to 32 months, respectively, for a radiographic recurrence (P = 0.03). No patient survived after a recurrence. The longer survival of patients with recurrent tumors detected by scanning most likely reflects the small number of patients and lead-time and length biases associated with screening. CONCLUSIONS: Among children with medulloblastoma, surveillance scanning is of little clinical value. Scanning detected a minority of recurrences, and no patient who had a recurrence survived.

Generalized cortical dysplasia. Clinical and pathologic aspects.

Three children with profound mental retardation and intractable seizures died at ages 10 months, 3 years, and 7 years, respectively. Complete examination of their brains showed generalized cortical dysplasia, without any major malformation of the external gyral pattern. The neuropathologic features of cortical dysplasia include abnormally thickened cortex with indistinct demarcation of the gray-white matter junction. In many areas, the cortex contained increased numbers of large neurons with disordered cortical lamination. Heterotopic neurons were scattered throughout the white matter with decreased myelination of the underlying white matter. To our knowledge, these cases represent the first fully detailed neuropathologic study of diffuse cortical dysplasia--a newly recognized entity of abnormal neuronal migration.