Mediastinal cavernous haemangioma in a patient with Klippel-Trenaunay syndrome

The Klippel-Trenaunay syndrome (KTS) is a rare syndrome characterised by the triad of varicose veins, bony and soft tissue hypertrophy, and cutaneous haemangioma. A 30 year old man with KTS with a right mediastinal mass which progressively enlarged over 5 years is described. Computed tomography, magnetic resonance imaging, and bronchial angiography revealed a vascular lesion in the azygous area. After complete excision of the mass, histological examination revealed cavernous haemangioma. To our knowledge, this is the first report of intrathoracic haemangioma in KTS.     

Early neonatal discharge guidelines: Have we dropped the ball?

OBJECTIVES:

To determine patterns of follow-up and prenatal education by family physicians and to assess whether practice patterns comply with the 1996 Canadian Paediatric Society/Society of Obstetricians and Gynecologists of Canada (CPS/SOGC) guidelines for early neonatal discharge.

DESIGN:

Mail survey.

SETTING:

A community of 300,000 people who were served exclusively for obstetrical care by a tertiary care hospital that performs 5000 deliveries per year and provides an early discharge program (EDP).

PARTICIPANTS:

Family physicians who provide prenatal and/or newborn care.

MAIN OUTCOME MEASURES:

The timing of neonatal follow-up and parental teaching by family physicians.

RESULTS:

Thirty-two per cent of the respondents scheduled their first postnatal visits two or more weeks after early discharge. There was no significant difference (P=0.7) in scheduling of follow-up for babies who were part of an EDP compared with those who were not. Fewer than 20% of physician respondents provided antenatal education in preparation for early discharge.

CONCLUSIONS:

The 1996 CPS/SOGC guidelines for physician follow-up after early neonatal discharge and for anticipatory parental education are not being followed consistently; however, these guidelines were disseminated without reinforcement. Until further study supports a change in practice guidelines, appropriate implementation strategies must be employed to ensure compliance.     

Neuroprotective effects of coenzyme Q10 at rostral ventrolateral medulla against fatality during experimental endotoxemia in the rat

Coenzyme Q10 (CoQ10, ubiquinone) is a highly mobile electron carrier in the mitochondrial respiratory chain that also acts as an antioxidant. We evaluated the neuroprotective efficacy of CoQ10 against fatality in an experimental model of endotoxemia that mimics systemic inflammatory response syndrome using a novel water-soluble formulation of this quinone derivative. Experiments were conducted in adult male Sprague-Dawley rats that were maintained under propofol anesthesia. Intravenous administration of Escherichia coli lipopolysaccharide (LPS; 30 mg/kg) induced progressive hypotension, with death ensuing within 4 h. The sequence of cardiovascular events during this LPS-induced endotoxemia can be divided into a reduction (Phase I), followed by an augmentation (Phase II; "pro-life" phase) and a secondary decrease (Phase III; "pro-death" phase) in the power density of the vasomotor components (0-0.8 Hz) of systemic arterial pressure signals. Pretreatment by microinjection bilaterally of CoQ10 (1 or 2 microg) into the rostral ventrolateral medulla (RVLM), the medullary origin of sympathetic vasomotor tone, significantly diminished mortality, prolonged survival time, and reduced the slope or magnitude of the LPS-induced hypotension. CoQ10 pretreatment also significantly prolonged the duration of and augmented the total power density of the vasomotor components of systemic arterial pressure signals in Phase II endotoxemia. The increase in superoxide anion production induced by LPS at the RVLM during Phases II and III endotoxemia was also significantly blunted. We conclude that CoQ10 provides neuroprotection against fatality during experimental endotoxemia by reducing superoxide anion production at the RVLM, whose neuronal activity is intimately related to the "life-and-death" process.     

Increased chromosome-type chromosome aberration frequencies as biomarkers of cancer risk in a blackfoot endemic area

To examine whether biomarkers such as sister chromatid exchanges (SCEs) and chromosome aberrations (CAs) can predict cancer development, a nested case-control study was performed in a blackfoot endemic area with a known high cancer risk. A cohort of 686 residents was recruited from three villages in the blackfoot endemic area. Personal characteristics were collected, and venous blood was drawn for lymphocyte culture and stored in a refrigerator. The vital status and cancer development were followed using the National Death Registry, Cancer Registry, and Blackfoot Disease Registry. The follow-up period was from August 1991 to July 1995. During this 4-year period, 31 residents developed various types of cancer. Blood culture samples from nine of these subjects were unsuitable for experiments due to improper storage. Finally, a total of 22 cancer cases had cytogenetic samples that could be analyzed. Twenty-two control subjects were selected from those who did not develop cancer in the study period, and these subjects were matched to cases by sex, age, smoking habits, and residential area. The results showed that there was no significant difference in the frequencies of SCE and chromatid-type CAs between the case and control groups. However, the frequencies of chromosome-type CAs, e.g., chromosome-type gaps, chromosome-type breaks, chromosome-type breaks plus exchanges, total chromosome-type aberrations, and total frequencies of CAs in the case group, were significantly higher than those in the control group (P < 0.05). The odds ratio of cancer risk in subjects with more than zero chromosome-type breaks was 5.0 (95% confidence interval = 1.09-22.82) compared to those with zero chromosomal breaks. The odds ratios for more than zero chromosome-type breaks plus exchanges and a frequency of total chromosome-type aberrations of >1.007% were 11.0 and 12.0, respectively (P < 0.05). Subjects with a total CA frequency of >4.023% had a 9-fold increase for cancer risk. These results indicate that chromosome-type CAs are good biomarkers for the prediction of cancer development, whereas SCEs and chromatid-type CAs cannot predict cancer risk.     

Hypoaldosteronism in three sibs due to 18-dehydrogenase deficiency

Three sibs all presented in the early neonatal period with a salt-losing syndrome. The salt-losing form of congenital adrenal hyperplasia was diagnosed and appropriate treatment with glucocorticosteroids, mineralocorticosteroids, and additional dietary salt started. Although early life was maintained with difficulty, with age all 3 children required decreasing amounts of replacement steroids to maintain normal plasma electrolyte balance. They were reinvestigated at the ages of 15 years and 8 years (twins), when cortisol synthesis and metabolism proved normal, but aldosterone synthesis was blocked by deficiency of 18-dehydrogenase. Rational treatment of these cases of a salt-losing syndrome in which aldosterone synthesis alone is blocked due to lack of the enzyme 18-dehydrogenase requires the administration of a mineralocorticosteroid drug only. Since deoxycorticosterone (acetate or pivalate) requires intramuscular administration, as life-long therapy oral fludrocortisone is preferable. Although fludrocortisone has glucocorticoid activity, the "hydrocortisone equivalent" effect of the small dosage used was unlikely to inhibit either pituitary corticotrophin or growth hormone production.     

Inhibitory action of insulin on suprachiasmatic nucleus neurons in rat hypothalamic slice preparations

In order to elucidate a role of insulin on neurons in the suprachiasmatic nucleus (SCN) involved in the generation of circadian feeding behavior, we examined the effect of insulin on SCN neuronal activity at in vitro condition. Bath application of insulin (1-100 microU) mainly inhibited the SCN neuronal activity, and this inhibitory effect was still observed in a Ca2+-free Krebs solution. The present result strongly suggests that insulin directly inhibits SCN neurons and may explain an increase in food intake by insulin infusion into the SCN during the light period.