Cryptocapsinepoxide-type Carotenoids from Red Mamey, Pouteria sapota

Three new carotenoids, cryptocapsin-5,6-epoxide, 3ʹ-deoxycapsanthin-5,6-epoxide, and cryptocapsin-5,8-epoxides, have been isolated from the ripe fruits of red mamey (Pouteria sapota). Cryptocapsin-5,6-epoxide was prepared by partial synthesis via epoxidation of cryptocapsin and the (5R,6S)- and (5S,6R)-stereoisomers were identified by HPLC-ECD analysis. Spectroscopic data of the natural (anti) and semisynthetic (syn) derivatives obtained by acid-catalyzed rearrangement of cryptocapsin-5,8-epoxide stereoisomers were compared for structural elucidation. Chiral HPLC separation of natural and semisynthetic samples of cryptocapsin-5,8-epoxides was performed and HPLC-ECD analysis allowed configurational assignment of the separated stereoisomers

Factor XII mutations, estrogen-dependent inherited angioedema, and related conditions

The clinical, biochemical and genetic features of the conditions known as estrogen-dependent inherited angioedema, estrogen-associated angioedema, hereditary angioedema with normal C-1 inhibitor, type III angioedema, or factor XII angioedema are reviewed. Discussion emphasizes pathogenesis, diagnosis, and management

Urticaria and infections

Urticaria is a group of diseases that share a distinct skin reaction pattern. Triggering of urticaria by infections has been discussed for many years but the exact role and pathogenesis of mast cell activation by infectious processes is unclear. In spontaneous acute urticaria there is no doubt for a causal relationship to infections and all chronic urticaria must have started as acute. Whereas in physical or distinct urticaria subtypes the evidence for infections is sparse, remission of annoying spontaneous chronic urticaria has been reported after successful treatment of persistent infections. Current summarizing available studies that evaluated the course of the chronic urticaria after proven Helicobacter eradication demonstrate a statistically significant benefit compared to untreated patients or Helicobacter-negative controls without urticaria (p < 0.001). Since infections can be easily treated some diagnostic procedures should be included in the routine work-up, especially the search for Helicobacter pylori. This review will update the reader regarding the role of infections in different urticaria subtypes

Guidelines for diagnosis and management of the cobalamin-related remethylation disorders cblC, cblD, cblE, cblF, cblG, cblJ and MTHFR deficiency

BACKGROUND: Remethylation defects are rare inherited disorders in which impaired remethylation of homocysteine to methionine leads to accumulation of homocysteine and perturbation of numerous methylation reactions. OBJECTIVE: To summarise clinical and biochemical characteristics of these severe disorders and to provide guidelines on diagnosis and management. DATA SOURCES: Review, evaluation and discussion of the medical literature (Medline, Cochrane databases) by a panel of experts on these rare diseases following the GRADE approach. KEY RECOMMENDATIONS: We strongly recommend measuring plasma total homocysteine in any patient presenting with the combination of neurological and/or visual and/or haematological symptoms, subacute spinal cord degeneration, atypical haemolytic uraemic syndrome or unexplained vascular thrombosis. We strongly recommend to initiate treatment with parenteral hydroxocobalamin without delay in any suspected remethylation disorder; it significantly improves survival and incidence of severe complications. We strongly recommend betaine treatment in individuals with MTHFR deficiency; it improves the outcome and prevents disease when given early

Identification of sleep disruption and sleep disordered breathing from the systolic blood pressure profile.

BACKGROUND--Respiratory sleep studies are frequently performed to identify sleep disruption resulting from upper airway obstruction. Traditional polysomnographic studies may not detect brief recurrent sleep disruption and thus fail to recognise a significant problem when apnoea, hypopnoea, or arterial desaturation are not present. Arousal from sleep causes a transient blood pressure rise, and each inspiration causes a transient blood pressure fall. This study assesses whether these blood pressure changes are a useful indirect marker of disturbed sleep, obstructed sleep apnoea, and snoring related sleep disturbance. METHODS--Computer algorithms were developed to identify blood pressure falls caused by inspiration and rises related to arousal from 286 sleeping blood pressure samples of a consistent respiratory state drawn from 51 polysomnographic studies. From these samples, normal ranges for the number of arousal related systolic rises and the average size of the inspiratory falls were established. These were then applied prospectively to all night unedited blood pressure recordings from a further 20 subjects. RESULTS--The size of the inspiratory falls in blood pressure progressively increased from normal sleep, through snoring, to frank obstructive sleep apnoea. The 95th centile of normal was 12.5 mm Hg. The number of arousal related blood pressure rises also increased during obstructive sleep apnoea and periods of snoring with associated arousals, compared with normal undisturbed sleep, and all these periods of disturbed sleep included more than 30 such rises per hour. When these blood pressure features were examined in the 20 subjects studied prospectively, the six with a sleep related breathing disorder could all have been identified from their systolic blood pressure profile alone. CONCLUSIONS--The systolic blood pressure profile may be helpful in identifying patients with obstructive sleep apnoea, snoring with arousals, or other sleep disruption syndromes

Non-invasive beat to beat arterial blood pressure during non-REM sleep in obstructive sleep apnoea and snoring.

BACKGROUND: Obstructive sleep apnoea, and possibly snoring, are associated with a poorly understood increase in cardiovascular mortality which may be explained by their effects on systemic blood pressure during sleep. This study compares changes in mean blood pressure during obstructive sleep apnoea and snoring without apnoeas with those in matched control subjects during non-REM sleep. METHODS: Eighteen men with obstructive sleep apnoea, 16 men who snored without apnoeas, and 34 control subjects matched for age, sex, obesity, smoking, and alcohol intake were studied. During polysomnography non-invasive mean blood pressure (Finapres) was recorded from each cardiac cycle during non-REM sleep and averaged over a 10 minute period. This was compared with the blood pressure during 10 minutes before sleep onset. The changes in the patients' sleeping blood pressure were compared with those in their individually matched control subjects. RESULTS: Compared with the control subjects the change in mean (SD) arterial blood pressure between being awake and asleep was higher during obstructive sleep apnoea (+6.5 (9) mm Hg v-2 (6.5), difference 8.5 (11)), and the rise from wakefulness to sleep in the obstructive sleep apnoea group was itself significant. The average mean arterial pressure was not raised in those who snored without apnoeas compared with either the control subjects or during wakefulness. CONCLUSIONS: Average mean arterial pressure is higher during obstructive sleep apnoea than it is during wakefulness, while normal subjects show a fall in blood pressure at sleep onset. This sleep related rise in blood pressure may contribute to the excess cardiovascular morbidity and mortality experienced by patients with this condition