Decreased Renal Clearance of Sodium in Cystic Fibrosis

ABSTRACT. In 10 patients with cystic fibrosis (CF) and 10 controls of similar age quantitative segmental handling of sodium was estimated by lithium clearance. In the CF group, there was tendency for an increased glomerular filtration rate (GFR) and increased absolute proximal sodium reabsorption. The fractional distal sodium reabsorption was significantly ( p =0.015) increased and sodium clearance was significantly ( p ≤0.01) decreased in CF.     

Trace Element Profiles in Individual Blood Cells from Patients with Down's Syndrome

ABSTRACT. The concentrations of trace elements in three different types of blood cells–erythrocytes, thrombocytes and neutrophils–were determined by nuclear microprobe analysis in 11 children with Down's syndrome (DS) and compared with those in 13 controls. The median concentration of copper in all three types of blood cells and that of calcium in erythrocytes were significantly higher in DS children than in controls. In contrast, the median levels of zinc in erythrocytes, of manganese and iron in erythrocytes and neutrophils, and of magnesium in erythrocytes and thrombocytes were significantly lower in DS than in control children. In 10 of the 11 DS children, detectable erythrocyte levels of titanium were found, which is remarkable, since this was not observed in any of the cells from the control children.     

Glucose Tolerance and Insulin Receptor Binding to Monocytes and Erythrocytes in Patients with Cystic Fibrosis

ABSTRACT. Cystic fibrosis (CF) is the most frequent life threatening hereditary disease in the Western World with an incidence of approximately 1:2000. Due to increasing survival rates the high frequency of abnormal glucose tolerance has become an important problem. We compared insulin concentrations during oral glucose tolerance test and insulin receptor binding to both monocytes and erythrocytes from 9 patients with CF, with results from 10 healthy controls of similar body weight. The insulin: glucose ratio was increased in the fasting state ( p < 0.05) in patients with CF compared to controls, indicating an increased insulin resistance in CF-patients. The total insulin secretion during oral glucose tolerance test as judged by the area beneath the insulin curve was similar in the two groups, but insulin secretion was significantly delayed in patients with CF. Insulin receptor binding to monocytes and the number of receptors were significantly increased ( p < 0.01 and 0.02, respectively) in patients with CF whereas the dissociation constant was similar in patients with CF and controls. No difference was observed in insulin receptor binding to erythrocytes between the two groups. No correlations were found between insulin receptor binding to monocytes or erythrocytes and glucose tolerance or insulin concentrations.     

Sweat Sodium Related to Amount of Sweat after Sweat Test in Children with and without Cystic Fibrosis

ABSTRACT. The sweat concentration of sodium was found to be inversely correlated with the amount of sweat obtained after a sweat test according to the method of Gibson & Cook in children without and with cystic fibrosis. Reference intervals for sweat sodium overlapped for the two groups but two-dimensional reference distributions for the amount of sweat (range 20-440 mg) correlated with its sodium content were completely separated. The establishment of similar distributions in centres carrying out sweat tests could serve to assess the performance of this investigation at local level.     

Sodium, Calcium, and Magnesium Levels in Nails of Children with Cystic Fibrosis of the Pancreas

Nail sodium, calcium, and magnesium levels were measured in 15 children with cystic fibrosis (CF) of the pancreas and compared with values found in 78 controls. There was a significant increase in the mean value of all three parameters in the CF group. There was an overlap in the values between the affected and control groups for all three measurements. Summing the sodium and calcium values or the sodium, calcium, and magnesium values gave a clear-cut separation between the groups, this being better in the latter case. It is felt that this approach might also prove fruitful in the detection of the heterozygote.     

Comparison of Classic Sweat Test and Crystallization Test in Diagnosis of Cystic Fibrosis

Objective

Sweat chloride measurement is considered a standard diagnostic tool for cystic fibrosis (CF). This study was performed to compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with sweat crystallization detected by direct observation of a drop of perspiration under light microscopy in patients with and without CF.

Methods

The tests using both techniques were performed simultaneously in patients with and without CF. Cutoff values of ≥60 mmol/L of chloride concentration for the classic sweat test was considered for diagnosis of CF. In crystallization method, observation of typical dendritic forms of salt crystals under light microscopy was interpreted positive.

Findings

Sixty patients suspected to CF (31 males and 29 females) with age range of 9 months to 2 years underwent the sweat test using both techniques. Median sweat chloride values was 26.13+10.85 in group with negative and 72.76+12.78 mmol/L in group with positive sweat test, respectively. All the patients who had positive sweat test in classic method showed typical dendritic forms of salt crystal in sweat crystallization test, which provided the test with 100% sensitivity (95%CI: 93.1–100). Only one of the 31 subjects with negative results for classic sweat test had positive result for crystallization sweat test, which provided the test with 96.7% specificity (95%CI: 92.9–100). Time spent to perform the crystallization test was significantly shorter than the classic method whereas its cost was also lower than the second method.

Conclusion

There was a good correspondence between two studied methods of sweat test. These results suggested the sweat crystallization test as an alternative test for detecting CF disease with high sensitivity and specificity.     

Long-term Effects of Physical Exercise on Working Capacity and Pulmonary Function in Cystic Fibrosis

ABSTRACT. Seven patients with cystic fibrosis aged 6 to 20 were enrolled for 30 months in a daily exercise program. After 12 months conventional chest physiotherapy was withdrawn. Patients with low initial Shwachman scores improved as regards maximal working capacity. Spirometric data and volume of trapped gas indicated opening of closed airways. We suggest that physical exercise in general should be the basis of pulmonary therapy in cystic fibrosis. Other forms of physiotherapy are advisable when hard physical exercise is not feasible.     

Sodium and Potassium in Red Blood Cells of Premature Infants during the First Few Days: Risk of Hyperkalaemia

ABSTRACT. Erythrocyte sodium and potassium were studied in 64 newborn infants including 21 very low birthweight infants (birthweight < 1500 g) during the first three days after birth. Erythrocyte sodium showed a positive correlation with gestational age ( r =0.63, p <0.01) and birthweight ( r =0.66, p <0.01). Erythrocyte potassium was negatively correlated with birthweight ( r = -0.33, p <0.05). The Na/K ratio in red blood cells showed a positive correlation with gestational age ( r =0.60, p <0.01) and birthweight ( r =0.65, p <0.01). In VLBW infants plasma potassium rose significantly ( p <0.01) from 0–6 h to 12–30 h and decreased from 12–30 h to 30–60 h ( p <0.05). Erythrocyte potassium decreased slightly from 0–6 h to 12–30 h, but not significantly. A new finding that "more immature infants have higher potassium and lower sodium concentration in RBC" may suggest a potential risk of hyperkalaemia in tiny infants.     

Scanning Electron Microscopic Study of the Airways in Normal Children and in Patients with Cystic Fibrosis and Other Lung Diseases

Scanning electron microscopy (SEM) and transmission electron microscopy (TEM) were used to examine pulmonary tissue from 9 patients with cystic fibrosis (CF), 12 patients with diseases other than CF, and from two surgically resected specimens with no known airways disease. A region of the human airways, the transition zone, was observed between the end of the terminal bronchiole and the type II alveolar cell lining of the respiratory bronchioles. This region was lined predominantly by nonciliated bronchiolar (NCB) cells. Patients with CF exhibited indistinct transition zones, epidermoid metaplasia, large dilated bronchial gland, copious surface mucus, alveolar destruction, and unusual microvilli; no single lesion specific for cystic fibrosis was identified.     

Microdissection Study of the Myentric Plexus in Acardia, Ataxia-telangiectasia, Cystic Fibrosis, Extrahepatic Biliary Atresia, Pediatric Aids and Werdnig-Hoffmann Disease

Microdissection-point count morphometric study of the myenteric (Auerbach) plexus or esophagus, small intestine, and colon was done for infants and children with acardia (2), ataxia-telangiectasia (5), cystic fibrosis of the pancreas (CFP) (25), extrahepatic biliary atresia (EBA) (17), pediatric AIDS (10), and Werdnig-Hoffmann disease (WHD) (8). Values for fractional area of neural tissue in the plane of the plexus were compared to those of control patients in same age range as those in each disease category by t-test. Statistically abnormal values included low values for small intestine and colon in Werdnig-Hoffmann disease, high values for small intestine and colon in biliary atresia, and high value for colon but a low value for small intestine in cystic fibrosis. Values for all three loci were within the normal range for ataxia telangiectasia and pediatric AIDS. The mechanisms of the low value for small and large intestines in WHD, which causes chronic constipation as a result of skeletal muscle weakness, and of the high values for colon in CFP and EBA, both causing malabsorption with bulky stools, are unclear. The value for small intestine in acardia was normal for term but lower than expected for fetal bowel of the same size, possibly because of reduced neural crest inflow to the fetal bowel.     

铅中毒幼鼠血铅、锌、铜、铁、钙水平变化

目的探讨慢性铅中毒幼鼠血铅、锌、铜、铁、钙水平变化。方法选取健康21 d断乳昆明小鼠48只,随机分为4组,每组12只。蒸馏水为对照组,余3组分别为10、20、40 mg/kg醋酸铅染毒组,按10 mL/kg灌胃染毒,1次/d,连续染毒46 d后摘眼球采血,用BS微量元素分析仪对血铅、锌、铜、铁水平进行测定,血浆钙用Dimension-RXL全自动生化分析仪测定。结果随着给铅水平的增加,小鼠血铅和铜均升高,而锌下降,铅中毒组与对照组相比,血铅、锌、铜均有显著性差异(P<0.01,0.05),而全血铁和血浆钙水平均无显著性差异(P均>0.05)。结论铅中毒可引起血锌下降和血铜升高,锌可能具有拮抗铅的毒性作用。实用儿科临床杂志,2006,21(14):936-937     

Early Changes of Some Serum Proteins and Metals in Diabetic Children

ABSTRACT. Serum concentrations of prealbumin, albumin, orosomucoid, magnesium, zinc and calcium were studied in 30 children with newly diagnosed IDDM aged 3–15 years, during the first two years of the disease, and in 44 healthy control children. On admission serum prealbumin was significantly lower in IDDM children (149±48 mg/1) (M±SD) than in healthy controls (194±39 mg/1) ( p <.001). During the two years follow-up prealbumin increased significantly, but did not reach the level of healthy controls. Serum albumin was slightly increased at diagnosis ( p <0.01), but later decreased significantly ( p <0.01). Orosomucoid concentrations did not differ between diabetics and controls. Serum magnesium was initially within the reference interval but later decreased, and after 2 years of IDDM it was highly significantly reduced ( p <0.001). Serum zinc was significantly reduced in IDDM children at diagnosis ( p <0.001), but was within the reference interval after one and 2 years. Serum calcium was increased in the IDDM group at diagnosis ( p <0.01), but later normalized. It is concluded that in early IDDM there is a decrease in serum prealbumin and albumin, and serum magnesium decreases progressively, while serum zinc is only transiently reduced.     

The effects of a lipid‐based nutrient supplement and antiretroviral therapy in a randomized controlled trial on iron,copper, and zinc in milk from HIV‐infected Malawian mothers and associations with maternal and infant biomarkers

We evaluated effects of antiretroviral (ARV) therapy and lipid‐based nutrient supplements (LNSs) on iron, copper, and zinc in milk of exclusively breastfeeding HIV‐infected Malawian mothers and their correlations with maternal and infant biomarkers. Human milk and blood at 2, 6, and 24 weeks post‐partum and blood during pregnancy (≤30 weeks gestation) were collected from 535 mothers/infant‐pairs in the Breastfeeding, Antiretrovirals, and Nutrition study. The participants received ARV, LNS, ARV and LNS, or no intervention from 0 to 28 weeks post‐partum. ARVs negatively affected copper and zinc milk concentrations, but only at 2 weeks, whereas LNS had no effect. Among all treatment groups, approximately 80–90% of copper and zinc and <50% of iron concentrations met the current adequate intake for infants at 2 weeks and only 1–19% at 24 weeks. Pregnancy haemoglobin was negatively correlated with milk iron at 2 and 6 weeks (r = ?.18, p < .02 for both). The associations of the milk minerals with each other were the strongest correlations observed (r = .11–.47, p < .05 for all); none were found with infant biomarkers. At 2 weeks, moderately anaemic women produced milk higher in iron when ferritin was higher or TfR lower. At 6 weeks, higher maternal α‐1‐acid glycoprotein and C‐reactive protein were associated with higher milk minerals in mildly anaemic women. Infant TfR was lower when milk mineral concentrations were higher at 6 weeks and when mothers were moderately anaemic during pregnancy. ARV affects copper and zinc milk concentrations in early lactation, and maternal haemoglobin during pregnancy and lactation could influence the association between milk minerals and maternal and infant iron status and biomarkers of inflammation.