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| 2. |
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| 3. |
- Alafuzoff, I, et al.
(författare)
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A comparison of multiplex and simplex families with Alzheimer's disease/senile dementia of Alzheimer type within a well defined population.
- 1994
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Ingår i: Journal of neural transmission. Parkinson's disease and dementia section. - 0936-3076. ; 7:1, s. 61-72
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Tidskriftsartikel (refereegranskat)abstract
- A study was made on 150 clinically demented patients presenting at autopsy at Umeå University Hospital in Sweden. In 90 of the cases dementia was considered to be primary in nature and of these forty six per cent (41 cases), fulfilled both the clinical and histopathological criteria for the diagnosis of Alzheimer's disease/Senile dementia of Alzheimer type (AD/SDAT). The families of these 41 AD/SDAT cases were then studied, and a family history obtained through interviews with multiple family informants and from civil and medical records. Additional diseased family members suffering from progressive dementia (multiplex families) were observed in 12 probands out of 41 (29%). Multiplex families exhibited similar clinical and histopathological characteristics as simplex families containing a single affected individual. The secondary cases in the multiplex families exhibited similar demographic and clinical characteristics as the probands. 39% of the multiplex and 14% of the simplex cases had an early age of onset of the disease, that was under 65 years. The overall prevalence of progressive dementia disorders in the 41 families was 5.9%. The prevalence of a progressive dementia disorder was 11% in the multiplex families (14% for the early onset cases) and 3.5% in the simplex families (2% for the early onset cases). The prevalence of progressive dementia disorder for family members who had passed the mean age of the onset of the disease for their family, was 45% for multiplex and 18% for simplex families. Furthermore the incidence rate for dementia was significantly higher (p < 0.005) in multiplex families (5.5 per 1,000 person years) when compared to simplex families (2.5 per 1,000 person years). No differences could be seen in parental age at birth of the diseased when comparing the two sets of families. However in multiplex families the duration of the disease was significantly (p < 0.025) shorter, in subjects with parental age at birth over 35 years compared to those with a parental age under 35 years. The multiplex families contained significantly (p < 0.025) larger sibships; and showed a significantly lower age of onset for the disease (p < 0.001), and a significantly longer duration of disease (p < 0.05) compared to the simplex families. A significant intra familial correlation of age at disease onset was observed in both sets of the families.
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| 4. |
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| 5. |
- Astermark, Jan, et al.
(författare)
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Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized
- 1999
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Ingår i: British Journal of Haematology. - : Wiley. - 0007-1048. ; 105:4, s. 1109-1113
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Tidskriftsartikel (refereegranskat)abstract
- The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10. 75 of the patients started before the age of 3, 31 at the age of 3-5 and 15 at the age of 6-9. Each subgroup was evaluated separately. In addition, a regimen of one infusion weekly was compared with that of two (haemophilia B) or three (haemophilia A) infusions weekly in each patient. A significant decrease in the overall number of joint bleeds per year was found after shortening the infusion interval (P<0.005), but the individual bleeding pattern varied. In survival analysis of the first pathologic joint score event, those who started prophylaxis before the age of 3 had a better outcome overall than those starting at later ages (P=0.001). However, in subgroup analysis, no significant difference was seen in the annual number of joint bleeds and the development of arthropathy between those starting with, or shifting to, the more intensive regimen before the age of 3 and those that were put on this regimen at the age of 3-5. Age at start of prophylaxis was found to be an independent predictor for the development of arthropathy (P=0.0002), whereas dose and infusion interval at start were not. Our data emphasize the importance of starting replacement therapy during the first years of life. However, it seems that when beginning the regimen it can be individualized and adjusted according to the bleeding pattern. In this way, the need for a venous access system may be assessed on an individual basis.
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| 6. |
- Berntorp, Erik, et al.
(författare)
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Centraliserad vård grundläggande i vårdprogram för blödarsjuka
- 1999
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Ingår i: Läkartidningen. - 0023-7205. ; 96:15, s. 1849-1852
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Tidskriftsartikel (refereegranskat)abstract
- Haemophilia is a rare and potentially life-threatening disease. In Sweden, with a population of approximately 8.5 million, about 350 people suffer from the more severe forms of haemophilia or von Willebrand disease. Meticulous management is important if the patients are to be spared chronic disability and serious treatment complications. The disease is lifelong and affects psychosocial aspects of life among patients and their families. With the help of a grant from the Swedish Board of Halth and Welfare, a care programme has been designed to guarantee Swedish haemophiliacs comparable and optimal care. The programme has been drawn up by representatives of the three haemophilia centres in Sweden (at University Hospital, Malmo, Sahlgrenska University Hospital, Gothenburg, and Karolinska Hospital, Stockholm) in co-operation with the World Federation of National Haemophilia Organisations. To ensure optimal individual application of the programme, individualised management strategies and patient information leaflets have been prepared.
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| 7. |
- Damm, S., et al.
(författare)
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Wall motion abnormalities in male elite orienteers are aggravated by exercise
- 1999
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Ingår i: Clinical Physiology. - : Wiley. - 0144-5979 .- 1365-2281. ; 19:2, s. 121-126
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Tidskriftsartikel (refereegranskat)abstract
- During the period 1979-92, 16 (15 men and one woman) sudden unexpected cardiac deaths occurred among young Swedish orienteers. This finding indicated a sharp increase in the death rate of orienteers, and necropsy demonstrated that myocarditis was a common histopathological finding. Therefore, an extensive non-invasive cardiac investigation was performed. A total of 59 male élite orienteers (mean age 23 years) and 36 cross-country skiers and middle-distance runners (mean age 22 years), serving as controls, were examined by both echocardiography at rest and radionuclide ventriculography at rest and during exercise. Wall motion abnormalities were found in eight orienteers using echocardiography. The purpose of this study was to examine whether the group of orienteers with wall motion abnormalities found using echocardiography had a smaller increase in ejection fraction from rest to exercise using radionuclide ventriculography than the rest of the orienteers and the controls, indicating an aggravation of the wall motion abnormalities during exercise. There were no significant differences in the ejection fraction at rest between the groups. In the orienteers with wall motion abnormalities (group 1), 62% (five out of eight) had less than a 0.05 unit increase in left ventricular ejection fraction compared with 27% (14 out of 51) of the remaining orienteers (group 2) and 19% (7 out of 36) of the controls (group 3). A comparison of athletes in group 1 with those in groups 2 and 3 combined revealed a statistically significant difference (P < 0.05). The divergent response in left ventricular ejection fraction during exercise suggests an aggravation of the wall motion abnormalities with exercise. Both the echocardiographic and the radionuclide ventriculographic findings indicate that the orienteers in group 1 had concealed left ventricular damage.
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| 8. |
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| 9. |
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| 10. |
- Eckstein, Rolf Lutz, et al.
(författare)
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Recycling of nitrogen among segments of Hylocomium splendens as compared with Polytrichum commune : Implications for clonal integration in an ectohydric bryophyte
- 1999
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Ingår i: OIKOS. - : Nordic Society Oikos. - 0030-1299. ; 86:1, s. 87-96
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Tidskriftsartikel (refereegranskat)abstract
- Physiological integration in clonal plants, which can be assumed to be dependent on vascular connections among ramets, is associated with several potential benefits, especially in nutrient-poor environments. However, some experimental evidence indicates that ectohydric bryophytes, i.e. species lacking specialised tissues for internal water conduction, also have physiologically integrated ramets. We tested this hypothesis by analysing nitrogen dynamics and tracing movements of a 15 N label among interconnected ramets of the ectohydric Hylocomium splendens over one season. The observed patterns were compared with translocation patterns in Polytrichum commune, an endohydric species that is known to show a high degree of clonal integration. Our aims were (1) to evaluate the degree of physiological integration among segments in H. splendens and (2) to study whether the pattern of 15 N movement obtained matched those depicted by changes in total nitrogen pool size. Current-year segments (G0) of both species were identified as strong sinks for nitrogen owing to their considerable increase in the 15 N pool during the season. In P. commune all other segments types showed a net loss of 15 N from June to September, which was probably due to autumn resorption of nitrogen to subterranean structures. In H. splendens one-year-old segments (G1) increased their 15 N pool, while older green segments (G2+) lost 50% of their initially absorbed 15 N. All the label lost from these source segments could be recovered in G0 and G1 segments. We suppose that most of the recycled nitrogen is provided by degeneration of three-year-old segments, which turn brown in parallel with the reallocation of nitrogen during the season. The high degree of physiological integration in H. splendens is discussed with respect to its life history and ecosystem nitrogen cycling.
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| 11. |
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| 12. |
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| 13. |
- Engström, C, et al.
(författare)
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Anticipation in unipolar affective disorder.
- 1995
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Ingår i: Journal of Affective Disorders. - 0165-0327 .- 1573-2517. ; 35:1-2, s. 31-40
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Tidskriftsartikel (refereegranskat)abstract
- Anticipation describes an inheritance pattern within a pedigree with an increase in disease severity and/or decrease in age at onset in successive generations. The phenomenon of anticipation has recently been shown to be correlated with the expansion of trinucleotide repeat sequences in a neuromuscular disease, various neurodegenerative disorders and mental retardation. We have studied parent-offspring differences in age at onset and disease severity in 31 pairs with unilineal inheritance of unipolar affective disorder (UPAD). Life-table analyses showed a significant decrease in survival to 1st episode of major depression in the offspring generation compared with the parental generation (P = 0.0007). There was also a significant difference in age at onset (P < 0.001) between parents and offsprings. The offspring generation experienced onset 15.6 years earlier and illness 1.5 x more severe than did the parent generation. Furthermore, there was a significant correlation (P < 0.05) in age at onset between parent and offspring generations. When we excluded pairs where the affected parent has an age of onset greater than the age of the child at the time of ascertainment (i.e., 23 pairs left), there was still a significant (P = 0.02) decrease in age at onset (8.4 years) and 1.5 x more severe disease in the offspring generation. No evidence for specific maternal or paternal inheritance was found. We found evidence of anticipation in 75-80% of this sample of unilineal family pairs of UPAD. Anticipation is, thus, an inheritance pattern in a large group of UPAD which suggests that the expansion of trinucleotide repeat sequences is a possible mode of inheritance in this group of UPAD. The findings of anticipation in this study of families with UPAD and previous findings in families with BPAD suggest that the variable expression of unstable expansions of trinucleotide repeats may turn out to be the basis of the continuum of liability in affective disorders.
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| 14. |
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| 15. |
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| 16. |
- Holm, M., et al.
(författare)
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A New Method for Analysis of Atrial Activation during Chronic Atrial Fibrillation in Man
- 1996
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Ingår i: IEEE Transactions on Biomedical Engineering. - : Institute of Electrical and Electronics Engineers (IEEE). - 1558-2531 .- 0018-9294. ; 43:2, s. 198-210
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Tidskriftsartikel (refereegranskat)abstract
- To further clarify the mechanisms maintaining chronic atrial fibrillation (CAF), a method identifying preferable activation patterns of the atria during fibrillation, by time averaging of multiple discrete excitation vectors, was developed. Repeated recordings, each of 56 atrial bipolar electrograms simultaneously acquired during 8 s, were made at multiple sites in the right atrial free wall and the left atrial appendage in 16 patients with CAF using a 2.17/spl times/3.54 cm electrode array. The local activation times (LAT's) in each recording were estimated as the median activation time at the respective measurement point. By calculating the time difference between the LAT's at adjacent measurement points in two spatial dimensions, a direction vector was created for each activation wave passing each set of measurement points, a total of 42 sets. By time averaging of the individual direction vectors (typically n=55) at each set of measurement points, preferable activation patterns were determined. Three types of activation patterns were found: 1) inconsistent activation (n=5), 2) consistent activation with preferential propagation directions (n=7) and 3) consistent activation with impulses originating from a localizable site within the recording area (n=4). All activation patterns were reproducible and the two latter patterns were proven significant using statistical tests. It is concluded that this new method is useful in further clarification of the mechanisms involved in the maintenance of atrial fibrillation.
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| 17. |
- Holm, Magnus, et al.
(författare)
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Determination of Preferable Wavelet Propagation Directions in Atrial Fibrillation
- 1993
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Konferensbidrag (refereegranskat)abstract
- M. Holm, P. Blomström, J. Brandt, R. Johansson, C. Lührs, and S. B. Olsson. Determination of preferable wavelet propagation directions in atrial fibrillation. In Erik K. Fernström Symp. Atrial Fibrillation, Lund, Sweden, June 1993.
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| 18. |
- Holm, M, et al.
(författare)
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Effect of cardiac exposure by median sternotomy on atrial fibrillation cycle length
- 1999
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Ingår i: Europace. - : Oxford University Press (OUP). - 1532-2092 .- 1099-5129. ; 1:4, s. 248-257
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Epicardial mapping is a powerful tool that has enabled us to gain insight into the electrical phenomena perpetuating atrial fibrillation and has guided the design of surgical and catheter-based therapeutic strategies. However, epicardial data are acquired during abnormal physiological conditions; the patients are anaesthetized, their chests opened, dislocating the heart and exposing it to air of room temperature, and the autonomic tone is modulated due to the surgery. The effect of intra-operative conditions on atrial electrophysiological properties have not been investigated before. Thus in the present study we assessed the atrial cycle length, shown to be an index of atrial refractoriness, and the ventricular rate before and during open-heart surgery in 10 patients with chronic atrial fibrillation and an underlying heart disease. METHODS AND RESULTS: Using a newly introduced and validated ECG method known as frequency analysis of fibrillatory ECG (FAF-ECG), the atrial cycle length and the ventricular rate were determined just before surgery. After anaesthesia and median sternotomy, epicardial mapping of the entire right atrial free wall was performed. The mean ventricular rate as well as the dominant atrial fibrillation cycle length consistently increased, the former from 71 to 92 beats x min(-1) (mean of all patients, P<0.05) and the latter from 156 to 172 ms (P<0.05). CONCLUSIONS: Atrial fibrillation cycle length, an index of atrial refractoriness, is increased as an effect of anaesthesia and heart exposure during open-heart surgery in patients with chronic atrial fibrillation, implying that atrial activation might be altered, which must be considered when interpreting data from epicardial conduction analysis.
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| 19. |
- Jacobsson, Leif S., et al.
(författare)
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Antiatherosclerotic Effects of the Angiotensin-Converting Enzyme Inhibitors Captopril and Fosinopril in Hypercholesterolemic Minipigs
- 1994
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Ingår i: Journal of Cardiovascular Pharmacology. - 0160-2446 .- 1533-4023. ; 24:4, s. 670-677
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Tidskriftsartikel (refereegranskat)abstract
- We evaluated the two angiotensin-converting enzyme (ACE) inhibitors captopril and fosinopril with regard to possible antiatherosclerotic effects in minipigs. Experimental hypercholesterolemia and atherosclerosis was produced in 33 minipigs of the Gottingen strain by an egg yolk/cholesterol-enriched diet for 1 year. One group (n = 11) was fed the atherogenic diet alone and served as a control. A second group (n = 11) received captopril (80 mg/kg/day) added to the atherogenic diet, and a third group (n = 11) was treated in the same manner but with fosinopril (8 mg/kglday). The drug treatments produced significant reduction in serum ACE activity associated with a reactive increase in plasma renin activity (PRA), but had only minor effects on plasma lipids and lipoproteins. At the end of the treatment period, all animals were killed and examined for degree of atherosclerosis. The percentage of atherosclerotic area in the abdominal aorta was significantly lower in both drug-treated groups as compared with controls. Furthermore, accumulation of cholesterol in the thoracic and abdominal aorta was inhibited by drug treatment. Finally, the percentage of intimal thickening in abdominal aorta was significantly reduced in the drug-treated groups. In conclusion, the ACE inhibitors captopril and fosinopril inhibited development of atherosclerosis in hypercholesterolemic minipigs.
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| 20. |
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| 21. |
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| 22. |
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| 23. |
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| 24. |
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| 25. |
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| 26. |
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| 27. |
- Ljung, Rolf, et al.
(författare)
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Cholelithiasis during the first year of life : Case reports and literature review
- 1992
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Ingår i: Acta Paediatrica, International Journal of Paediatrics. - : Wiley. - 0803-5253. ; 81:1, s. 69-72
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Tidskriftsartikel (refereegranskat)abstract
- Five cases are reported of children with gallstones diagnosed by ultrasound during their first 7 months of life. Of the four with symptomatic gallstones, one subsequently developed vitamin K deficiency syndrome with profuse bleedings. The children, who belonged to a defined population, were all diagnosed within an 18-month span, suggesting the frequency of early gallstone formation to be higher than formerly supposed. One child had haemolytic anaemia, but none of the conventional risk factors for stone formation was present in the other four cases.
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| 28. |
- LJUNG, ROLF, et al.
(författare)
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The impact of prenatal diagnosis on the incidence of haemophilia in Sweden
- 1995
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Ingår i: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 1:3, s. 190-193
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Tidskriftsartikel (refereegranskat)abstract
- Summary A demographic survey was made of all children (n= 137) born with severe or moderate haemophilia in Sweden during the period 1970‐92. Bn addition, all prenatal diagnoses (n= 86) performing during the period were evaluated. The annual incidence of severe and moderate haemophilia, having remained constant for decades, increased from 0.78/10,000 males in the 1970s to 1.34 in the 1980s, levelling off at 1.31 in the 1990s. Although prenatal diagnosis did not affect the incidence of haemophilia in the 1970s and 1980s, it did so in the 1990s, because the incidence would have been 40% higher (1.83) had not prental diagnosis been available and 16 affected fetuses been aborted. The average proportion of sporadic cases, 62%, remained almost unchanged during the study period, suggesting mutation rates to be constant. There were fewere children in families with known haemophilia than in sporadic families, but no evidence was found to suggest that the frequency of female offspring (i.e. potential carriers) born in haemophilia families had increased since the option of prenatal diagnosis was introduced.
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| 29. |
- Magnusson, Kerstin, 1957, et al.
(författare)
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Contamination and correlation with toxicity of sediment samples from the Skiagerrak and Kattegat
- 1996
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Ingår i: Journal of Sea Research. - 1385-1101. ; 35:1-3, s. 223-234
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Tidskriftsartikel (refereegranskat)abstract
- The pollution state in the Skagerrak and Kattegat was investigated by determination of pollutant concentrations and toxicity of sediment samples from 11 stations in the area. A comparison was made with the sediment from a reference site near the Faroe Islands. Polycyclic aromatic hydrocarbons (PAH) and organochlorines were determined in whole sediment and heavy metals and ammonia were analysed in filtered pore water. Toxicity was bioassayed in whole sediment with Nitocra spinipes and Daphnia magna, in pore water with Mytilus edulis larvae and in solvent extracts from sediment with tests measuring etoxyresorufin-O-deethylase (EROD) activity in Oncorhyncus mykiss and rate of denitrification. Sites close to Goteborg and in an area from the Oslo fjord to the Norwegian Trench were most polluted. Sediment from the Faroe Islands was least polluted and also least toxic. Multivariate statistical analysis indicates that the different tests were sensitive to different kinds of pollutants. Effects on mussel larvae correlated strongest with the occurrence of ammonia, manganese, cadmium and PAHs, Nitocra with alpha-hexachlorocyclohexane (HCH) and p,p'-DDD, Daphnia with arsenic and gamma-HCH, fish EROD activity with benzo[ghi]perylene and unknown compounds associated with organic carbon, and denitrification with chlordanes, dieldrin and a few PAHs. The results indicate that sampling sites close to Goteborg are so polluted that harmful effects on the ecosystem probably occur.
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| 30. |
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| 31. |
- Monstein, H J, et al.
(författare)
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Cholecystokinin-A and cholecystokinin-B/gastrin receptor mRNA expression in the gastrointestinal tract and pancreas of the rat and man. A polymerase chain reaction study
- 1996
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Ingår i: Scandinavian Journal of Gastroenterology. - : Informa UK Limited. - 1502-7708 .- 0036-5521. ; 31:4, s. 383-390
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Gastrin and cholecystokinin (CCK) are thought to exert trophic effects on the gastrointestinal tract and pancreas. Two types of receptors have been cloned, CCK-A and CCK-B/ gastrin. We have examined the occurrence of CCK-A and CCK-B receptor mRNA in the brain, digestive tract, pancreas, and kidney of the rat and man by Northern blot and reverse transcribed polymerase chain reaction (RT-PCR). METHODS: Total RNA was isolated from rat tissues and reverse transcribed into cDNA. cDNA from brain, kidney, and pancreas of the rat and man and from human whole stomach were commercially available. Northern blot and a PCR technique based on Taq polymerase-antibody interaction and using CCK-A and CCK-B receptor-specific primers, followed by Southern blot analysis, were the methods used. RESULTS: By means of Northern blots, CCK-A receptor mRNA was detected in rat fundus mucosa and pancreas but not in the remaining GI tract or brain. By means of RT-PCR, CCK-A receptor mRNA was demonstrated in the brain and the mucosa of the fundus, antrum, duodenum, and colon, kidney, pancreas and pancreatic islets. CCK-B receptor mRNA was detected by Northern blot analysis in the brain and the fundus mucosa but not in the rest of the digestive tract and not in the pancreas, pancreatic islets, or kidney. By RT-PCR, expression of CCK-B receptor mRNA could also be detected in antrum mucosa. In man, CCK-A receptor mRNA was detected in the brain, stomach, pancreas, and kidney, whereas CCK-B receptor mRNA was found in the brain, stomach, and pancreas but not in the kidney. Cloning and DNA-sequence analysis of the PCR-amplified rat and human CCK-A and CCK-B receptor DNA fragments, which cover the protein-encoding regions of the intracellular loop C3, showed complete sequence homology as compared with published rat and human sequences. CONCLUSIONS: It appears unlikely that CCK will have effects in the ileum, at least not effects mediated by CCK-A receptors. It also appears unlikely that physiologic concentrations of gastrin in the circulation will promote growth (or exert other effects) in the pancreas, duodenum, ileum, and colon, since CCK-B receptor mRNA is not expressed or is poorly expressed in these tissues.
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| 32. |
- Nylander, P O, et al.
(författare)
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Migraine : temperament and character.
- 1996
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Ingår i: Journal of Psychiatric Research. - 0022-3956 .- 1879-1379. ; 30:5, s. 359-68
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Tidskriftsartikel (refereegranskat)abstract
- The personality profile of 26 adult migraine patients from a large Swedish family with migraine and 87 controls were studied by means of Cloninger's seven-factor model of Temperament and Character (TCI; Temperament and Character Inventory). For the diagnosis of migraine, a questionnaire, slightly modified to fit the criteria according to the AD HOC committee on the classification of headaches of the International Headache Society, was used. The TCI assesses four dimensions of temperament, including novelty-seeking (NS), harm avoidance (HA), reward dependence (RD) and persistence (P), and three dimensions of character, including self-directedness (SD), co-operativeness (C) and self-transcendence (ST). Psychiatric morbidity did not differ between this family and the general population. One migraine patient had double depression (dysthymia and recurrent depression) and one had a personality disorder. No significant difference could be found in the higher order dimensions of temperament (NS, HA, RD and P) and character (SD, C and ST) between migraine patients and controls. However, on the subscale level, NS showed a slightly higher average in NS1 (exploratory excitability) and a significantly higher (p = 0.0448) average in NS2 (impulsivity) in migraine patients compared to controls. Somatic anxiety has been shown to be positively correlated with NS, and especially impulsivity. Our results showed a tendency of this personality profile, and may suggest an association between migraine and somatic anxiety.
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| 33. |
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| 34. |
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| 35. |
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| 36. |
- Salehi, S Albert, et al.
(författare)
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Gastrectomy induces impaired insulin and glucagon secretion: evidence for a gastro-insular axis in mice
- 1999
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Ingår i: Journal of Physiology. - 1469-7793. ; 514:2, s. 579-591
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Tidskriftsartikel (refereegranskat)abstract
- 1. Mice were subjected to gastrectomy (GX) or food deprivation (24 h). The release of insulin and glucagon in response to different secretagogues was monitored in vivo and in isolated islets 3-4 weeks after surgery. 2. GX animals responded to glucose with an impaired glucose tolerance and a poor increase in plasma insulin. Islets from GX or food-deprived mice displayed impaired insulin release to high glucose and enhanced glucagon release at low glucose. 3. After GX the insulinogenic index, Delta insulin (microU ml-1)/Delta glucose (mg ml-1), was suppressed by 65% after oral glucose and by 59% after i.v. glucose. The integrated insulin response after oral glucose was reduced by 90% in GX mice. After i.v. glucose the reduction was 67%. 4. Carbachol-induced insulin release in vivo was reduced after food deprivation and exaggerated after GX. Carbachol-stimulated glucagon secretion was suppressed after GX and after food deprivation. A similar pattern was found in vitro. 5. Cyclic AMP activation (by the phosphodiesterase inhibitor isobutylmethylxanthine or the adenylate cyclase stimulator forskolin) induced a greater insulin response in GX or food-deprived mice than in sham-operated, fed mice. A similar pattern was found in vitro. The glucagon response was enhanced in vitro but not in vivo. 6. Crude extracts of rat oxyntic mucosa enhanced basal as well as glucose-induced insulin release from isolated islets, whereas glucagon release was markedly inhibited. The effects were dose dependent, the inhibition of glucagon release being achieved at lower concentrations than the potentiation of glucose-induced insulin release. The active principle was inactivated by incubation with trypsin or leucine aminopeptidase. 7. The data suggest that a circulating agent, probably a peptide, from gastric oxyntic mucosa stimulates glucose-induced insulin secretion. It also suppresses glucagon secretion. The GX-evoked impairment of the insulin (and glucagon) response to glucose is partly compensated for by an enhanced insulin response to cholinergic and/or cyclic AMP activation.
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| 37. |
- Warrier, Indira, et al.
(författare)
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Factor IX inhibitors and anaphylaxis in hemophilia B
- 1997
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Ingår i: Journal of Pediatric Hematology/Oncology. - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114 .- 0192-8562. ; 19:1, s. 23-27
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: We present clinical and laboratory data on 18 children from 12 hemophilia treatment centers in the United States, Canada, and Europe with the purpose of disseminating information regarding a recently recognized, potentially life-threatening complication of treatment in very young children with hemophilia B. Patients and Methods: Twelve hemophilia centers from the United States, Canada, and Europe provided clinical information and laboratory data concerning 18 children who had severe allergic reactions to infused factor (F) IX in close association with the development of an inhibitor to FIX. Laboratory testing for establishment of the diagnosis of hemophilia B and inhibitor to FIX was done locally at the centers treating these patients. FIX gene analysis was performed at one of six molecular genetics institutes. Results: All 18 children had severe hemophilia B, and in each an inhibitor antibody to FIX developed. The median age at the time of anaphylaxis (or anaphylactoid reaction) was 16 months, and the median number of exposure days to FIX was 11. The FIX inhibitor was detected almost simultaneously with the first occurrence of anaphylaxis in 12 of 18 patients. Maximum inhibitor liters were 4.5-600 Bethesda units (BU), with a median titer of 48 BU. FIX gene analysis, performed in 17 of 18 patients, demonstrated complete deletion of the FIX gene in 10 and major derangements in seven. Immune tolerance induction (ITI) regimens have been attempted in 12 patients, with generally poor responses. Two of the 12 experienced nephrotic syndrome while on ITI. Recombinant FVIIa has been successfully used to treat bleeding episodes in 11 of these children. Conclusion: Physicians treating young children with hemophilia B should be aware of the potentially life- threatening complication of anaphylaxis. Children with complete gene deletions or major derangements of the FIX gene appear to be at greater risk. Those identified by genotype as being at greater risk may need to receive their first 10-20 treatments in a medical facility equipped for handling such emergencies. Recombinant FVIIa, although not licensed for use in the United States, appears to be the most suitable treatment option for bleeding episodes in such patients.
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