A renal, pancreatic and hepatic dysplasia sequene (RPHD sequence) was found in a male premature baby who died a few minutes after birth. Autopsy documented multicystic dysplastic kidneys, a dysplastic pancreas with dilated ducts, cysts, fibrosis and inflammatory infiltrates, prominent portal tracts containing dilated bile ducts and hypoplastic lungs. Other organs were normal. This triad constitutes a dysplastic sequence and was first reported by Ivemark et al. [6] as familial dysplasia of kidneys, liver and pancreas. Since then, this combination of abnormalities has been named polycystic dysplasia [4] and renal-hepatic-pancreatic dysplasia [1], but mostly Ivemark syndrome [8], at the risk of being confused with asplenia-cardiac anomaly syndrome, which was reviewed by Ivemark et al. [5] and also bears Ivemark's name.Abbreviation RHPD
renal pancreatic and hepatic dysplasia sequence 相似文献
Objective: To estimate the correlates of mood and anxiety disorders among rural, urban and metropolitan residents in the United States. Methods: We analyzed the National Co-morbidity Survey (NCS), which yields the distribution and correlates of psychiatric disorders in a probability sample of U.S. population using DSM-III-R for diagnosis. Logistic regressions of mood and anxiety disorders were stratified by geographical area. Results: We found gender differences in mood disorders among urban (O.R. = 1.8, P < 0.0001) and metropolitan (O.R. = 1.6, P < 0.0001) but not among rural residents. Rural (O.R. = 0.2, P < 0.05) and urban (O.R. = 0.5, P < 0.05) African Americans were less likely to report mood disorders compared to rural and urban Whites. Similarly, we found gender differences in anxiety disorders among urban (O.R. = 2.0, P < 0.0001) and metropolitan (O.R. = 1.7, P < 0.0001), but not among rural residents. Conclusion: Rural men reported more mood and anxiety disorders than urban men, thus erasing expected rural gender differences in these disorders. Rural male mood and anxiety disorders may be a function of diminishing resources (steady, high paying jobs) or increasing financial strain particularly among Whites, who comprise a majority of rural residents. 相似文献
We investigated a risk of developing radiation myelitis during four prospective studies using hyperfractionated radiation therapy (HFX RT) with and without concurrent chemotherapy (CHT) during which a portion of thoracic spinal cord received a dose > or = 50.4 Gy given via 1.2 Gy b.i.d. fractionation. Of 536 patients with Stage III non-small cell lung cancer (NSCLC) which were treated on three prospective randomised Phase III studies and one Phase II study, 336 patients received irradiation dose > or = 50.4 Gy to a portion of their spinal cord and survived >1 year after the beginning of therapy. None of these 336 patients developed thoracic radiation myelitis. Therefore, the influence of potentially contributing factors on the occurrence of radiation myelitis, such as cord length, interfraction interval, or administration of concurrent CHT was not possible to investigate. These results give new insight about the influence of total dose/dose per fraction/interfraction interval with or without concurrent CHT on the thoracic spinal cord toxicity. 相似文献
In both obese and nonobese women, polycystic ovary syndrome (PCOS) is essentially a disorder of hyperinsulinemic insulin resistance, and it may be heralded by precocious pubarche (PP; appearance of pubic hair in girls aged <8 y). The risk of progression from PP to PCOS is related to low birth weight, but there are no early biochemical markers of this risk. As increased plasminogen activator-inhibitor type 1 (PAI-1) activity (act) is an early marker of cardiovascular risk in PCOS, we have sought abnormalities in young girls with PP. In 33 young PP girls (age range 6-11 y), PAI-1-act was increased (mean + SEM: 15.6 +/- 1.5 IU/mL) compared with age-, sex-, and pubertal stage-matched controls (n = 13, 10.7 +/- 1.9, p < 0.05). PAI-1-act levels were inversely related to birth weight SD score (r = -0.33, p < 0.05), and PAI-1-act levels were therefore higher in PP girls with low birth weights (n = 14, 19.5 +/- 2.5 IU/mL) than normal birth weights (n = 19, 12.8 +/- 1.5, p < 0.01). During longitudinal observation in 10 PP girls (mean time interval 2.7 y), PAI-1-act levels in early puberty were positively related to postmenarcheal insulin levels (mean serum insulin SDS postoral glucose, r = 0.65, p < 0.05), and showed a similar relationship to postmenarcheal testosterone levels (r = 0.61, p = 0.06). Together with low birth weight, increased plasma PAI-1-act levels in early pubertal PP girls may indicate those girls with greater risk of developing hyperinsulinemic-hyperandrogenism features of PCOS. 相似文献
Objectives: French Guiana has the highest birth rate in South America. This French territory also has the highest premature birth rate and perinatal mortality rate of all French territories. The objective was to determine the premature birth rate and to identify the prevalence of risk factors of premature birth in French Guiana.
Methods: A retrospective study of all births in French Guiana was conducted between January 2013 and December 2014 using the computerized registry compiling all live births over 22 weeks of gestation on the territory.
Results: During this period 12 983 live births were reported on the territory. 13.5% of newborns were born before 37 (1755/12 983). The study of the registry revealed that common sociodemographic risk factors of prematurity were present. In addition, past obstetrical history was also important: a scarred uterus increased the risk of prematurity adjusted odds ratio =1.4, 95%CI (1.2–1.6). Similarly, obstetrical surveillance, the absence of preparation for birth or of prenatal interview increased the risk of prematurity by 2.4 and 2.3, the excess fraction in the population was 69% and 72.2%, respectively.
Conclusions: Known classical risk factors are important. In the present study excess fractions were calculated in order to prioritize interventions to reduce the prematurity rate. 相似文献
BACKGROUND AND OBJECTIVE: The aim of this study was to evaluate prostate anaesthetic block for haemodynamic tolerance and quality of analgesia during and after transurethral surgery. METHODS: Ninety adult males, ASA III/IV, were randomly assigned to receive a prostate anaesthetic block (n = 45) or spinal anaesthesia (n = 45). The main outcome measurement during anaesthesia, surgery and recovery was haemodynamic tolerance (number of hypotensive episodes, heart rate, systolic and mean blood pressures). Other outcome variables were pain scores during anaesthesia, surgery and every 4 h until 24 h after the end of surgery and amount of oral morphine required during the 24-h postoperative period. RESULTS: Sixty-six patients were ASA III and 24 ASA IV. Ongoing cardiovascular therapies were comparable between groups. All surgical procedures were performed under good conditions. For patients receiving prostate anaesthetic block, the blood pressure and heart rate did not change significantly during the study period. For patients receiving spinal anaesthesia, the hypotension rate was 55.6% (n = 25) compared to 0 in the prostate anaesthetic block group (P < 0.001); 25 and 4 patients, respectively, required vascular filling and additional ephedrine administration. In both groups, all pain scores were <40 (100 mm visual analogue scale) during the study period. Oral morphine was given during the postoperative period to two prostate anaesthetic block patients and four who had received spinal anaesthesia (not significant). CONCLUSION: Transurethral surgery under prostate anaesthetic block is safe and assures adequate analgesia during and after surgery. 相似文献
We investigated the intestinal transport of D-glucose (D-Glc) and 3 essential amino acids in a model of intestinal inflammation, and the effects of dietary supplementation with animal plasma proteins on this function. Wistar Lewis rats were fed a diet containing an isonitrogenous amount of milk protein (control group) or a diet supplemented with either spray-dried animal plasma (SDAP) or immunoglobulin concentrate (IC) from porcine plasma, from d 21 of life (weaning) until d 35. On d 30 and 33, rats were challenged intraperitoneally with Staphylococcus aureus enterotoxin B (SEB; groups SEB, SEB-SDAP, and SEB-IC) and on d 35, brush border membrane vesicles (BBMVs) were prepared and used for transport and binding studies. Administration of SEB reduced D-Glc transport across sodium glucose transporter 1 [SGLT1; 20% reduction in maximal transport rate (Vmax); P < 0.05], without affecting the Michaelis constant (Km). The results from specific phlorizin binding, Western blot, and immunohistochemistry supported the view that the effects of SEB are due to reduced expression of D-Glc transporters in the apical membrane. SEB increased the passive diffusion constant (Kd) for D-Glc 3-fold (P < 0.05). SEB did not affect mediated or passive amino acid fluxes of L-leucine, L-methionine, or L-lysine. Dietary SDAP increased the D-Glc Vmax in the SEB group without affecting the passive component. Changes in d-Glc Vmax due to SEB and to the dietary treatments were correlated with changes in the number of SGLT1 transporters present in the BBMVs (r = 0.9468; P < 0.05). Dietary IC had no observed effect. We estimate that, in rats challenged with SEB, SDAP supplementation can increase glucose absorption by 8-9% during the interdigestive periods. 相似文献