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1.
Introduction Impaired vitamin K status in cystic fibrosis (CF) has been considered as a newly emerged pathogenetic factor for reduced bone mineral density (BMD).Objectives Our aim was to evaluate the effectiveness of vitamin K supplementation in managing bone formation abnormalities in children and adolescents with CF.Materials and methods The statuses of vitamins K and D in relation to biochemical markers of bone metabolism and BMD were examined in 20 CF children receiving vitamin D supplements but not vitamin K supplements. Laboratory tests were carried out at the beginning of the study period and after 1 year of vitamin K supplementation (10 mg single oral dose/week) and the results were compared; the results were also compared with those of 25 healthy controls.Results and discussion Ten of the CF patients had BMD z-score ≤2.5 (n=5) or between −1 and −2.5 (n=5). Biochemical tests on patients before vitamin K supplementation revealed that the levels of osteoblastic activity markers, namely, bone alkaline phosphatase (BAP), serum osteocalcin (Gla-OC), serum carboxy-terminal propeptide of type I procollagen (PICP) and serum amino-terminal propeptide of type I procollagen (PINP), were significantly reduced compared with those of the controls. These patients had also lower 25-hydroxy-vitamin D (25(OH)D) and vitamin K serum levels, higher undercaboxylated osteocalcin (Glu-OC) and parathormone (PTH) levels and a higher calcium to creatinine ratio (Ca/Cr) than the controls. Vitamin K intake was associated with an increase in Gla-OC, PINP, PICP levels and a decrease in Glu-OC levels. PTH levels were lower after vitamin K supplementation without any difference in BMD z-scores.Conclusion Our data indicate that vitamin K supplementation may have a beneficial role in bone health in CF children.  相似文献   

2.
Aims:   Screening enables early nutritional deficiencies to be detected in those with cystic fibrosis (CF). Although vitamin deficiency is considered unlikely in older subjects with normal vitamin E levels, few studies have determined vitamin D status at diagnosis and its relationship to other fat-soluble vitamins.
Methods:   We reviewed vitamin levels in infants diagnosed with CF by newborn screening over a 5-year period in Melbourne, Australia. Vitamin D levels were determined using the IDS gamma-B 25-OH Vitamin D radio-immunoassay (Immunodiagnostic Systems Limited, Boldon, UK). Vitamins A and E were evaluated by high-performance liquid chromatography. We assessed the association between vitamin D level and sex, month of birth, pancreatic status, and vitamin A and E levels.
Results:   Fifty-eight infants were diagnosed at a median age of 1 month (range: 0–3 months). Initial vitamin D levels were assessed between 0.2 and 3.5 months in 30 (vitamin D) and 45 (vitamins A and E) infants. The number of infants deficient with vitamins D, E and A were 11 (37%), 7 (16%) and 27 (60%), respectively. Vitamin D levels were unrelated to sex, vitamin A or E levels, month of birth or pancreatic status, whereas vitamin A and E levels were significantly lower in those who were pancreatic insufficient. With supplementation, vitamin D increased over time.
Conclusions:   Vitamin D deficiency is common in infants newly diagnosed with CF by newborn screening and is unrelated to pancreatic status or predicted low vitamin E levels. Vitamin D deficiency is less common over time following treatment.  相似文献   

3.
OBJECTIVE: To prospectively evaluate the biochemical status of vitamins A, D, and E in children with cystic fibrosis (CF). SUBJECTS: A total of 127 infants identified by the Colorado CF newborn screening program. DESIGN: Vitamin status (serum retinol, 25-hydroxy vitamin D, ratio of alpha-tocopherol/total lipids) and serum albumin were assessed at diagnosis (4 to 8 weeks), ages 6 months, 12 months, and yearly thereafter, to age 10 years. RESULTS: Deficiency of 1 or more vitamins was present in 44 (45.8%) of 96 patients at age 4 to 8 weeks as follows: vitamin A 29.0%, vitamin D 22.5%, and vitamin E 22.8%. Of these patients with initial deficiency, the percent that was deficient at 1 or more subsequent time points, despite supplementation, was vitamin A 11.1%, vitamin D 12.5%, and vitamin E 57.1%. Of the initial patients with vitamin sufficiency, the percent who became deficient at any time during the 10-year period was as follows: vitamin A 4.5%, vitamin D 14.4%, and vitamin E 11.8%. The percent of patients deficient for 1 or more vitamins ranged from 4% to 45% for any given year. CONCLUSIONS: Despite supplementation with standard multivitamins and pancreatic enzymes, the sporadic occurrence of fat-soluble vitamin deficiency and persistent deficiency is relatively common. Frequent and serial monitoring of the serum concentrations of these vitamins is therefore essential in children with CF.  相似文献   

4.
5.
We report two infants with cystic fibrosis (CF), presenting with meconium ileus and meconium plug, who had no clinical or biochemical evidence of pancreatic insufficiency during infancy. They underwent pancreatic secretory function testing at 11 and 9 months of age, respectively. Both patients had sufficient lipase and colipase secretion to maintain normal digestion of fat, confirming that meconium disease in CF does not necessarily imply pancreatic insufficiency and the need for enzyme supplementation in infancy. Nonetheless, we documented markedly reduced enzyme secretion in both patients, implying a potential role for the pancreas in the pathogenesis of meconium disease, even when clinical pancreatic insufficiency is absent. In addition, our patient with meconium ileus had a severely limited fluid secretory capacity (10.3% of mean normal values). In contrast, the patient with the milder presentation of meconium plug had a far greater ability to secrete fluid (75% of mean normal), but had poorer pancreatic proteolytic activity. We suggest that impaired fluid secretion may be a very significant factor in the pathogenesis of meconium ileus, and we speculate that an inability to maintain sufficient intraluminal fluid relative to the degree of pancreatic proteolytic deficiency may more adequately explain the risk of occurrence and the severity of intestinal obstruction in CF than either factor alone.  相似文献   

6.
OBJECTIVE: The aim of this study was to define the role of possible risk factors for the development of cystic fibrosis (CF)-related liver disease and to analyze the association between liver disease and the different genotypes present in the Israeli CF patient population. PATIENTS AND METHODS: All patients followed at the seven CF centers in Israel were included in this study. Liver disease was determined by persistently elevated serum liver enzymes and/or bilirubin, and/or significant ultrasonographic changes suggestive of chronic liver disease. The following clinical parameters were evaluated: ethnic origin, age at assessment of liver function, sex, history of meconium ileus, pancreatic function, history of distal intestinal obstruction syndrome, pulmonary function, and cystic fibrosis transmembrane conductance regulator mutation analysis. RESULTS: Of the 288 patients screened, 80 (28%) had liver disease. Of the 256 patients with pancreatic insufficiency, 80 (31%) had liver disease compared with none of the 32 patients with pancreatic sufficiency. Genotype-phenotype correlation was performed on 207 patients carrying identified mutations that were previously classified according to phenotype severity. Liver disease was found in 56 (32%) of 173 patients carrying mutations associated with a severe phenotype and in 6 (38%) of 16 patients carrying at least one mutation associated with a variable genotype (G85E and/or 5T allele). None of the 18 patients carrying the 3849+10kb C->T mutation had liver disease. Prevalence of liver disease increased with age. No correlation was found between liver disease and severity of lung disease, nutritional status, history of meconium ileus, or distal intestinal obstruction syndrome. CONCLUSION: CF patients who have pancreatic insufficiency and carry mutations associated with a severe or a variable genotype are at increased risk to develop liver disease.  相似文献   

7.
目的调查肥胖症儿童脂溶性维生素A、D、E水平,并分析其影响因素。方法选取2019年1月至2021年4月就诊于西安市儿童医院营养科的273例肥胖症儿童(肥胖症组)为研究对象,同期健康体检的226例正常体重儿童为对照组。对两组儿童进行体格及体成分的测量,并检测血清维生素A、D、E浓度。结果与对照组比较,肥胖症组血清维生素A[(1.32±0.21)μmol/L vs(1.16±0.21)μmol/L]、维生素E[(9.3±1.4)mg/L vs(8.3±1.2)mg/L]水平较高(P<0.001),25羟维生素D[25-hydroxyvitamin D,25(OH)D]水平[(49±22)nmol/L vs(62±24)nmol/L]较低(P<0.001)。在肥胖症组中,边缘型维生素A缺乏率为5.5%(15/273),维生素D缺乏/不足率为56.8%(155/273),维生素E不足率为4.0%(11/273)。控制体重指数和腰身比后,肥胖症儿童维生素A水平与年龄呈正相关(P<0.001),维生素E和25(OH)D水平与年龄呈负相关(P<0.001)。在控制年龄因素后,未发现肥胖症儿童血清维生素A、维生素E、25(OH)D水平与其肥胖程度、体脂百分比、肥胖时长的相关性,但维生素A和维生素E水平与其腰身比呈正相关(P<0.001)。结论肥胖症儿童的血清维生素A和维生素E水平较高,尤其是腹型肥胖者,而血清维生素D营养状况较差,且随着年龄的增长,状况愈差。因此,应关注肥胖症儿童维生素D营养状况并积极补充。  相似文献   

8.
We evaluated neurologic function in 18 patients, ages 5 to 26 years, with cystic fibrosis. Eight were deficient in vitamin E. Sural nerve conduction latency was increased and nerve action potential amplitude decreased in the vitamin E-deficient group in comparison with the vitamin E-sufficient group. Two vitamin E-deficient patients had absent deep tendon reflexes; findings of clinical neurologic examinations were otherwise normal. We recommend early supplementation with vitamin E for patients with cystic fibrosis who have pancreatic insufficiency, to prevent neurologic dysfunction.  相似文献   

9.
Objective: Thalassemia along with hematopoietic stem cell transplantation (HSCT) can lead to major oxidative stress. Vitamins A and E are antioxidants which protect membrane from lipid peroxidation. We sought to determine for the first time, whether vitamins A and E supplementation is efficacious in maintaining or increasing plasma level of these vitamins in thalassemic children undergoing HSCT. Methods: A cross-sectional study was performed on 50 children with β-thalassemia major hospitalized for HSCT. Patients took a daily multivitamin. Plasma vitamins A and E levels were measured at four different times: on admission, HSCT day (day 0), day 7 and day 14 after HSCT. Findings : Plasma vitamin A and E were abnormal on admission in most patients (62.0% and 60.0% respectively). Ratio of patient with normal to abnormal plasma level of the vitamins improved from baseline to a peak on day 7 then deteriorated afterward until day 14. There was an increasingly positive correlation between daily oral intake and plasma vitamin A at different times, but plasma vitamin E showed inverse correlation at first which tended towards no correlation subsequently. In multivariate analysis, supplementation significantly changed plasma level of vitamin A at different measurement time (P=0.001) within study subjects. But, plasma level of vitamin E showed no significant difference (P=0.2). Conclusion: Our findings suggest that oral supplementation could have beneficial effects due to increasing plasma vitamin A level and preventing plasma vitamin E depletion.Key Words: Thalassemia, Hematopoietic Stem Cell Transplantation, Vitamin A, Vitamin E, Dietary Supplements  相似文献   

10.
Vitamin E deficiency is a major problem in cystic fibrosis. Modern pancreatic enzymes may improve the absorption of fat-soluble vitamins to make water dispersion less important. In order to compare the absorption of fat-soluble (F) and water-miscible (W) tocopheryl acetate, equivalent amounts (10 mg/kg body weight) were given to five patients with cystic fibrosis (CF) and five age- and sex-matched healthy controls. Serum was analysed for vitamin E using HPLC before and 1, 2, 3, 4, 6, 8, 10, 24 and 28 h after the oral intake. The median peak concentrations in the sera of controls were 41.9 micromol/L after F and 39.8 micromol/L after W preparations, corresponding values in CF patients being 21.3 and 26.8 micromol/L, and corresponding means +/- SD were 41.6+/-6.7 and 38.4+/-3.3, and 27.1+/-14.5 and 28.8+/-8.3, respectively. Although the area under the curve was lower for W than F preparations in both CF patients and controls, these differences were not significant. CONCLUSION: The results in our study are consistent with those of an earlier study using the same preparation and suggest that it is not necessary to use water-miscible preparation of alpha-tocopheryl acetate in CF patients receiving modern pancreatic enzyme supplementation.  相似文献   

11.
Patients with cystic fibrosis (CF) have significantly decreased plasma concentrations of antioxidant vitamins which is considered to result both from fat malabsorption and from chronic pulmonary infection. The aim of this study was to investigate levels of vitamin A and beta-carotene in plasma of CF subjects with pancreatic exocrine insufficiency. In thirty CF patients mean plasma concentrations of vitamin A (1.17 micromol/l) was lower by 30% and that of beta-carotene (1.3 micromol/l) by 60% than in healthy children. Vitamin A level was positively correlated with beta-carotene (r=0.67; p<0.001). Ratios of beta-carotene to total cholesterol and cholesterol of LDL (low density lipoprotein) were 40% lower than in the control group. We conclude that in our CF patients the concentration of beta-carotene does not seem to be sufficient to inhibit lipid peroxidation especially of LDL fraction.  相似文献   

12.
We set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 +/- 610 gm) than patients with no meconium ileus (3169 +/- 534 gm; p less than 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p less than 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 +/- 4.67 vs 7.18 +/- 5.19 years), but there was no difference in age of acquisition of P. cepacia. In families in which the first child had meconium ileus, 29% of subsequent siblings with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ileus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.  相似文献   

13.
Abstract

Vitamin D deficiency and insufficiency are associated with serious sequelae in childhood cancer survivors. However, data on vitamin D deficiency in children with newly diagnosed cancer are scarce and the role of sociodemographic factors and vitamin D supplementation is largely unknown. We assessed vitamin D status and its socio-demographic and clinical correlates in 163 children with newly diagnosed cancer, using 25-hydroxy vitamin D (25(OH)D) concentrations and assessed longitudinal changes following vitamin D supplementation. Sixty-five percent of the patients with newly diagnosed cancer had low 25(OH)D concentrations. Fifty-two patients (32%) were vitamin D deficient (≤20?ng/mL 25(OH)D concentration), and 53(33%) were insufficient (21-29?ng/mL 25(OH)D concentration). Age over 10 (P?=?0.019), Hispanic ethnicity (P?=?0.002), and female sex (P?=?0.008) were significantly associated with lower 25(OH)D concentration at diagnosis. Vitamin D supplementation resulted in significant increase in 25(OH)D concentrations (P?<?0.001). However, following supplementation in the longitudinal analysis, this increase was less pronounced in Hispanic patients vs. non-Hispanic (P?=?0.007), and in children with solid tumors vs. hematological malignancies (P?=?0.003). Vitamin D deficiency and insufficiency are common in children with newly diagnosed cancer. Hispanic patients, females and older children were at higher risk for vitamin D deficiency and insufficiency. Although supplementation appeared to increase 25(OH)D concentrations over time, this increase was not as pronounced in certain subsets of patients. Prospective trials of the effects of vitamin D supplementation on bone health in children with newly diagnosed cancer are warranted, particularly in Hispanics and patients with solid tumors.  相似文献   

14.
The water-soluble (B1, B2, B6, C, folic acid) and fat-soluble vitamin (A, carotene, E, and D) status of 36 patients with cystic fibrosis was assessed and compared with a control group of 21 age-matched normal children. Twenty-seven of the patients were receiving vitamin supplements (except folic acid and vitamin E) at the time of investigation. Vitamin B1, B2, and B6 status was adequate in all patients, and there was little evidence of folic acid deficiency. Vitamin C stores might not have been adequate in some of these patients, despite daily supplements with 50 mg of the vitamin. Steatorrhoea, often severe, was present in most of them. Serum carotene and vitamin E concentrations were low in over 90% of patients and were related to the severity of steatorrhoea. Vitamin A was low in over 40% of the patients despite daily vitamin supplements of 4000 IU and correlated with the serum retinol-binding protein level. Serum 25-OH cholecalciferol was low in some patients whether or not they were receiving a daily supplement of 400 IU vitamin D. In a short-term supplementation trial with water-miscible preparations of vitamin A and E in 14 patients, the serum levels of both vitamins responded well to 2 weeks of treatment with 50 mg vitamin E and 4000 IU vitamin A. Except for serum vitamin A, which was lowest in patients with the poorest clinical grading, the other vitamins were not influenced by the clinical grade of the patients.  相似文献   

15.
A patient is reported in whom neonatal small left colon syndrome (NSLCS) was present with caecal perforation in conjunction with cystic fibrosis, presenting as meconium ileus. The pregnancy, in a 36-year old primigravida, was characterised by alcohol and nicotine abuse but was otherwise normal. There was no maternal diabetes. Management by temporary bowel diversion, in conjunction with pancreatic enzyme replacement, was effective. Correspondence to: G. P. hadley  相似文献   

16.
BACKGROUND: The assessment of severe pancreatic insufficiency in cystic fibrosis (CF) is not a diagnostic problem. However, identification of mild cases remains a challenge. The aim of this study was to assess the ability of serum lipase after secretin stimulation to identify mild pancreatic insufficiency in patients with CF. MATERIAL AND METHODS: Thirty patients with CF and pancreatic insufficiency (CF-PI) and 30 patients with CF and pancreatic sufficiency (CF-PS) were studied. Thirty healthy subjects with no known gastrointestinal disease served as controls. In all subjects, fecal fat excretion, fecal elastase-1 (E1) concentration and basal and secretin-stimulated serum lipase concentration were measured. RESULTS: All patients with CF-PI and 3 with CF-PS had abnormally low fecal E1 concentrations. The remaining 27 CF-PS patients and all controls had normal values. Basal and post-stimulation lipase levels were extremely low in patients with CF-PI. Mean basal and poststimulation serum lipase concentrations were significantly higher in CF-PS who had normal fecal E1 concentrations but were still below those of controls (P < 0.001). Among the 27 CF-PS patients with normal fecal elastase, high basal and poststimulation lipase values were found in 6 and 17 patients respectively. CONCLUSION: In patients with CF-PS who have normal fecal elastase-1 concentration, the measurement of basal or secretin-stimulated lipase levels might be helpful in identifying the progression of the destructive process in the pancreas.  相似文献   

17.
Specimens of meconium and random stools were collected sequentially from 25 healthy newborn babies over the first 8-14 days of life. The stool chymotrypsin concentrations increased from birth to a maximum at 4 days of age and then fell again over the next four days. The lowest individual stool concentrations either side of the four day peak were both, coincidentally, 120 micrograms/g stool. In a second group of 22 newborn babies suspected of meconium ileus and later confirmed to have cystic fibrosis, faecal chymotrypsin concentrations were all appreciably reduced. In eight babies, also with suspected meconium ileus but with negative sweat tests, chymotrypsin concentrations were within the healthy newborn range. Measuring faecal chymotrypsin concentrations is a reliable procedure for identifying pancreatic exocrine insufficiency in the newborn.  相似文献   

18.
OBJECTIVES: Vitamin A and E deficiency is common in cystic fibrosis (CF). These vitamins have immunomodulating properties and we determined whether decreased serum vitamin A and E levels in young infants are associated with early CF lung disease and lower airway inflammation. METHODS: A post-hoc analysis was undertaken on previous data collected prospectively in 39 newly diagnosed infants identified by a newborn CF screening programme. Assessment of CF genotype, nutrition, pancreatic status, serum retinol and alpha-tocopherol levels was performed at diagnosis. Pulmonary status was determined clinically, by Brasfield chest radiographic scores and analysis of bacterial counts and inflammatory indices in bronchial lavage (BL) fluid. These assessments were repeated 12 months later. RESULTS: At diagnosis, 20 out of 39 (51%) CF infants had low serum retinol (mean (SD) 0.7 (0.3) micromol/L) and 9/38 (24%) had low alpha-tocopherol (mean (SD) 13.4 (8.4) micromol/L) levels. Dietary energy intake was related to serum retinol concentrations at diagnosis (r(2) = 0.27; P = 0.001). At 1 year, serum retinol and alpha-tocopherol levels had normalized following vitamin A and E supplementation. Respiratory symptoms, radiographic scores and BL inflammatory indices systematically deteriorated during infancy, reaching significance for free neutrophil elastase activity (9 out of 29 vs 21 out of 33; P = 0.01) and IL-8 levels (79 vs 416; P = 0.046) in BL fluid. No association was seen between serum vitamin levels at diagnosis and airway inflammatory indices at either diagnosis or 12 months later. CONCLUSION: We found in this CF birth cohort no evidence to implicate vitamin A or E deficiency in the development of lung disease or airway inflammation during infancy.  相似文献   

19.
目的 探讨晚期早产儿25-羟维生素D[25-hydroxyvitamin D,25(OH)D]水平及维生素D3补充对婴幼儿神经行为发育的影响。 方法 前瞻性选取2017年6月—2020年6月收治的晚期早产儿161例为研究对象,根据脐血25(OH)D水平分为充足组(52例)、不足组(53例)、缺乏组(56例),每组按随机数字法分为A亚组(维生素D3 800 IU/d)、B亚组(个体化补充维生素D3)。分析比较各组生后3个月25(OH)D水平、纠正胎龄10个月及纠正胎龄18个月25(OH)D水平及Gesell发育量表评分的差异。 结果 生后24 h内及3个月时,不足组、缺乏组25(OH)D水平低于充足组(P<0.05),不足组25(OH)D水平高于缺乏组(P<0.05);缺乏组生后3个月时B亚组25(OH)D水平高于A亚组(P<0.05)。不足组和缺乏组纠正胎龄10个月、纠正胎龄18个月时Gesell发育量表5个能区得分均低于充足组(P<0.05);缺乏组纠正胎龄10个月时言语能、纠正胎龄18个月时粗大动作能得分低于不足组(P<0.05)。缺乏组B亚组纠正胎龄10个月时适应能、纠正胎龄18个月时适应能和应物能得分高于A亚组(P<0.05)。 结论 晚期早产儿脐血25(OH)D水平存在明显差异,个体化补充维生素D方案对于纠正维生素D缺乏更为有效。出生时及婴儿早期维生素D水平对神经行为发育有一定影响。  相似文献   

20.
目的 观察血流感染患儿与健康儿童血清25(OH)D 水平的差别。方法 采用病例对照研究方法,选取2010 年1 月至2013 年12 月间住院治疗且双份血培养阳性的60 例血流感染患儿为血流感染组,另选取60 例同年龄段同时期行健康体检的儿童为健康对照组。化学发光法检测两组儿童血清25(OH)D 水平,比较两组儿童不同水平25(OH)D 的构成比。结果 血流感染组患儿血清25(OH)D 水平明显低于健康对照组(P<0.01);血流感染组维生素D 正常(8%)、不足(22%)的构成比均显著低于健康对照组(分别为35%、43%,P<0.05),而缺乏(42%)、严重缺乏(28%)的构成比均显著高于健康对照组(分别为13%、8%,P<0.01)。结论 维生素D 不足在儿童中普遍存在,血流感染患儿血清25(OH)D 水平显著低于健康儿童。  相似文献   

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