Plasma Levels of Nitrate in Congenital Heart Disease: Comparison with Healthy Children

Nitric oxide (NO) is an endothelium- derived relaxing factor, and plasma nitrate is the stable end product of NO production. The aim of this study was to investigate the change in levels of plasma nitrate according to age and to elucidate the effect of pulmonary hypertension (PH) associated with congenital heart disease on NO production. We measured plasma levels of nitrate in 48 healthy children aged 5 days to 12 years to establish the normal range. Forty-six preoperative patients aged 4 months to 12 years with congenital heart disease were studied by cardiac catheterization. Plasma nitrate in healthy children decreased with age, from 1 month to 1 year, and then remained almost constant until the age of 12 years. Plasma nitrate was significantly increased in 22 preoperative patients with PH (mean pulmonary arterial pressure >?25 mmHg) compared with age-matched normal controls: (mean 56.9 vs 33.5 μmol/L, p<0.05) and was significantly correlated with pulmonary to systemic pressure ratio (r= 0.83, p < 0.0001). There was no significant difference between plasma nitrate levels in 24 preoperative patients without PH and those in the age-matched normal control (mean 25.6 vs 24.9 μmol/L). In 10 patients with preoperative PH who were examined before and after surgery, plasma nitrate levels remained high in the cases with residual PH but decreased to the normal range in the cases without residual PH. Plasma nitrate level is useful for evaluating PH both before and after operation in patients more than 4 months of age, and it is important to note differences in normal plasma nitrate levels according to age.     

Plasma nitrate concentrations in children with infectious and noninfectious diarrhea

BACKGROUND: In patients with intact renal function and low dietary nitrate intake, plasma nitrate concentrations reflect endogenous nitric oxide production and are shown to be increased during inflammatory processes. The aim of this study was to compare plasma nitrate concentrations and hence endogenous nitric oxide production in children with infectious and noninfectious diarrhea and to determine whether plasma nitrate concentrations could serve as a discriminant test between acute and chronic diarrhea in children. METHODS: Three groups of patients were identified: 14 patients with acute gastroenteritis, 13 patients with chronic noninfectious diarrhea, and 14 patients with no evidence of gastrointestinal pathology and no underlying infectious process, who served as control subjects. Plasma nitrate concentrations were determined spectrophotometrically using the Greiss reaction before reduction to nitrite with a copper-coated cadmium column. RESULTS: Mean plasma nitrate concentrations were 405.3 micromol/L +/- 281.6 micromol/L (standard deviation) in patients with infectious diarrhea, 134.7 micromol/L +/- 77.0 micromol/L in patients with chronic diarrhea, and 54.1 micromol/L +/- 20.1 micromol/L in control subjects (F = 42.6, P < 0.0001; analysis of variance). Plasma nitrate concentrations were significantly higher in the infectious diarrhea group compared with the noninfectious diarrhea and control groups (Student-Newman-Keuls test, P < 0.5). CONCLUSIONS: Although an optimal cutoff concentration cannot be defined, plasma nitrate concentrations in excess of 300 micromol/L are suggestive of an infectious process whereas values less than 100 micromol/L are indicative of noninfectious diarrhea.     

Homocysteine levels during fasting and after methionine loading in adolescents with diabetic retinopathy and nephropathy

OBJECTIVE: To assess plasma homocysteine levels in adolescents and young adults with type 1 (insulin-dependent) diabetes with and without microvascular complications. STUDY DESIGN: Homocysteine levels were measured during fasting and after methionine loading in plasma of 61 patients with onset of diabetes before the age of 12 years and duration of disease longer than 7 years. They had an albumin excretion rate (AER) between 20 and 200 microg/min in 2 of 3 overnight urine collections in a period of 6 months and/or retinopathy. Patients with persistent microalbuminuria were divided into 2 groups: subjects with AER of 20 to 70 microg/min and patients with AER of 70 to 200 microg/min. Adolescents (n = 54) without signs of diabetic retinopathy or nephropathy and matched control subjects (n = 63) were also studied. RESULTS: Homocysteine concentrations before and after methionine load were higher in adolescents with diabetic complications than in healthy subjects (fasting values: 12. 4 +/- 7.9 micromol/L vs 7.8 +/- 4.2 micromol/L; P <.01; after methionine load: 28.1 +/- 13.2 micromol/L vs 16.6 +/- 7.3 micromol/L; P <.005). Values of 11.9 micromol/L or higher were considered to constitute fasting hyperhomocysteinemia. The increase of homocysteine concentrations was particularly evident in young diabetic patients with AER >70 microg/min (fasting values: 14.7 +/- 5.6 micromol/L; after methionine load: 34.2 +/- 12.6 micromol/L) and in patients with proliferative retinopathy (fasting values: 15.1 +/- 5.0 micromol/L; after methionine load: 36.8 +/- 12.5 micromol/L). CONCLUSIONS: Increased plasma homocysteine concentrations may contribute to increased morbidity and death from cardiovascular disease in adolescents and young adults with diabetic retinopathy and nephropathy.     

鼠尾草酸增加1,25(OH)2D3诱导HL-60细胞分化及其机制研究

目的：已证实1, 25(OH)2D3可以诱导HL-60细胞向成熟单核细胞分化,但其不足之处是高钙血症和形成耐药株。该实验应用鼠尾草酸（CA）联合1, 25(OH)2D3研究对HL-60细胞分化的影响及引起细胞内活性氧（ROS）水平和细胞内钙离子浓度的改变。方法：应用鼠尾草酸,1,25（OH）2D3单独和联合应用处理HL-60细胞,共分为5组：空白对照组;低浓度1,25（OH）2D3（1 nmol/L）组、鼠尾草酸组;联合处理组（10 μmol/L鼠尾草酸和1 nmol/L 1,25（OH）2D3）;高浓度1,25（OH）2D3（100 nmol/L）组。每24 h监测1次,通过四唑氮蓝(MTT)法观察细胞生长,共72 h;收集处理后72 h的HL-60细胞,通过光学显微镜观察细胞形态,用流式细胞仪（FCM）检测不同处理组细胞周期及单核细胞分化标记CD14的表达,ROS和细胞内钙离子浓度。结果：72 h后,联合处理组HL-60细胞与空白对照组相比,细胞增殖明显受抑制（Ab= 0.560±0.020; P<0.01）,细胞形态具有成熟单核细胞特征,CD14的表达率升高（57.62%;P<0.01）,G0/G1 期细胞显著增多, ROS水平下降［（52.67±10.76）%,P＜0.01］;联合处理组细胞内钙离子水平同空白对照组比较差异无显著性（115.64±17.74 nmol/L,P>0.05）,但与高浓度1,25（OH）2D3组相比细胞内钙离子水平明显下降(P<0.01)。结论：鼠尾草酸可增强1,25（OH）2D3对HL-60细胞的诱导分化、增强抑制HL-60细胞增殖作用,细胞阻滞于G0/G1期,细胞内活性氧水平降低,这可能与细胞的诱导分化机制有关,且这一联合作用不增高细胞内钙离子水平。     

Plasma zinc and growth status in preadolescent children with cystic fibrosis

OBJECTIVE: To investigate plasma zinc status in relation to dietary and supplemental zinc intake, growth and pulmonary status in preadolescent children with cystic fibrosis (CF) and pancreatic insufficiency (PI). METHODS: Fasting plasma zinc was assessed in children (age, 8-11 years) with CF and PI. Food (7-day weighed records) and supplemental zinc intake, serum alkaline phosphatase and albumin, pulmonary function (spirometry), coefficient of fat absorption (%COA, 72-hour fecal fat) and growth status [height adjusted for genetic potential (AHAZ), weight (WAZ) and BMI Z scores (BMIZ)] were assessed. RESULTS: For the 62 children (32 males), mean plasma zinc (+/-SD) was 16.8 +/- 3.1 micromol/L (110 +/- 20 ug/dL). Sixty-five percent of the subjects had levels above the study reference range of 9.2 to 15.3 micromol/L (60-100 ug/dL); no subjects had low zinc levels. Median (range) total daily zinc intake was 279% (83-988%) recommended dietary allowance, growth status was suboptimal (mean +/- SD: AHAZ, -0.8 +/- 1.0; WAZ, -0.5 +/- 1.2; BMIZ, -0.2 +/- 1.1), and forced expiratory volume at 1 second (FEV1) was 92 +/- 13% predicted. Plasma zinc was not correlated with growth, pulmonary or alkaline phosphatase status. Plasma zinc was correlated with serum albumin (r = 0.25, P < 0.05) and was inversely correlated with coefficient of fat absorption (as %; r = -0.30, P = 0.02). CONCLUSIONS: Under current patterns of care in CF Centers, total zinc intake and plasma zinc status were adequate. These findings suggest that zinc was not a limiting micronutrient for preadolescent children with CF and PI and mild-to-moderate lung disease, and not likely contributing to their suboptimal growth status.     

Adrenomedullin and total nitrite levels in children with familial Mediterranean fever

AIM: Familial Mediterranean fever (FMF) is the most frequent periodic syndrome characterised by recurrent attacks of polyserositis. However, recent studies revealed that there might be an ongoing subclinical inflammation between the attacks. As nitric oxide (NO) and adrenomedullin (AM) are both synthesised in the endothelium, and mediates many functions within immune system, we considered them to be an interesting target of investigation in FMF. METHODS: Fifteen children with FMF receiving regular colchicine, ranging in age from 3 to 16 years, were investigated in comparison with 15 healthy age- and sex-matched controls. The mean age of the patients was 9.7 +/- 3.9 years. Total nitrite, a stable product of NO, was quantitated by means of the Griess reaction, while AM was measured by HPLC. RESULTS: Plasma-urinary AM and total nitrite levels were significantly higher in children with FMF. Plasma AM levels (pmol/mL) in patients and controls were 40.95 +/- 5.99 vs. 34.86 +/- 5.24, P < 0.05, and urinary AM excretion (pmol/mg creatinine) was 51.16 +/- 28.15 vs. 37.5 +/- 24.26, P < 0.05 respectively. Plasma total nitrite levels (micromol/L) in patients and controls were 44.80 +/- 10.36 vs. 32.13 +/- 9.28, P < 0.05, and urinary nitrite excretion (micromol/mg creatinine) was 2.24 +/- 1.71 vs. 1.09 +/- 0.96, P < 0.05 respectively. CONCLUSION: This study considered that AM and NO may have a role in the immuno-inflammatory process of FMF, although, whether these act to preserve, or protect against, further inflammatory injury is not clear. Our results further supports the hypothesis that these patients have subclinical inflammation between attacks.     

Pancreatic endocrine function in cystic fibrosis

To characterize pancreatic endocrine secretion and to examine interrelationships among alterations in alpha, beta, and pancreatic polypeptide cell function in patients with cystic fibrosis (CF), we studied 19 patients with exocrine insufficiency (EXO), including 9 receiving insulin therapy (EXO-IT); 10 patients with no exocrine insufficiency (NEXO); and 10 normal control subjects. First-phase C-peptide response to intravenously administered glucose was significantly impaired in CF patients with exocrine insufficiency (EXO-IT = 0.02 +/- 0.01; EXO = 0.11 +/- 0.02; NEXO = 0.25 +/- 0.05; control subjects = 0.30 +/- 0.04 nmol/L). Lowering fasting glucose levels with exogenous insulin administration in EXO-IT did not improve beta cell responsivity to glucose. The C-peptide response to arginine was less impaired (EXO-IT = 0.12 +/- 0.02; EXO = 0.15 +/- 0.02; NEXO = 0.23 +/- 0.06; control subjects = 0.28 +/- 0.04 nmol/L). Alpha cell function, measured as peak glucagon secretion in response to hypoglycemia, was diminished in EXO but not NEXO (EXO-IT = 21 +/- 10; EXO = 62 +/- 19; NEXO = 123 +/- 29; control subjects = 109 +/- 12 ng/L). Despite diminished glucagon response, EXO patients recovered normally from hypoglycemia. Peak pancreatic polypeptide response to hypoglycemia distinguished CF patients with exocrine insufficiency from those without exocrine insufficiency (EXO-IT = 3 +/- 2; EXO = 3 +/- 1; NEXO = 226 +/- 68; control subjects = 273 +/- 100 pmol/L). Thus CF patients with exocrine disease have less alpha, beta, and pancreatic polypeptide cell function than CF patients without exocrine disease. These data suggest either that exocrine disease causes endocrine dysfunction in CF or that a common pathogenic process simultaneously and independently impairs exocrine and endocrine function.     

碳氧血红蛋白测定对新生儿黄疸诊断的价值

目的：探讨碳氧血红蛋白测定在新生儿黄疸诊断中的临床价值。方法：189例新生儿黄疸患儿(新生儿溶血病75例,感染52例,颅内出血32例,晚发母乳黄疸30例)及142例对照组患儿同步测定动脉化毛细血管血碳氧血红蛋白(COHb)和血清总胆红素(STB);溶血组予大剂量静脉免疫球蛋白治疗后测定COHb及STB,应用SAS6.12统计软件进行处理。结果：溶血组COHb及STB分别为(3.64±0.83)%,330.84±77.15μmol/L,显著高于对照组的(2.38±0.35)%和130.18±32.86μmol/L(P<0.01);颅内出血组COHb及STB分别为(2.48±0.53)%,184.15±29.35μmol/L,高于对照组的(2.24±0.32)%及112.11±17.45μmol/L(P<0.05);感染及母乳黄疸组STB分别为286.71±45.66μmol/L,299.15±44.14μmol/L,显著高于对照组146.23±31.26μmol/L及57.33±7.83μmol/L(P<0.01),而COHb为(2.36±0.50)%及(1.84±0.49)%与对照组(2.20±0.39)%及(1.67±0.43)%比较,差异无显著性(P>0.05)。溶血性高间胆组STB低于非溶血性高间胆组(P<0.01),而COHb显著高于后者(P<0.01)。溶血组大剂量静脉免疫球蛋白治疗前后COHb分别为(3.64±0.83)%及(2.68±0.51)%,STB分别为330.84±77.15μmol/L及230.18±42.96μmol/L,治疗前后比较差异有显著性(P<0.01)。结论：COHb测定可作为胆红素产量的指标,有助于新生儿黄疸病因诊断及指导治疗。     

Plasma homocysteine levels and folate status in children with sickle cell anemia.

PURPOSE: A sensitive inverse relationship between plasma homocysteine concentration and folate status has been demonstrated. Although children with sickle cell anemia (SCA) are at potential risk for folate deficiency, plasma homocysteine levels have not been reported in such patients. Therefore, a study was designed to assess plasma homocysteine levels as a marker of folate status. DESIGN: Plasma homocysteine concentrations were measured in 120 children with SCA (102 in steady state and 18 during an acute complication) who had never received supplemental folic acid. Folate status was directly assessed in 34 of these patients. RESULTS: Plasma homocysteine levels in the patients with SCA and control subjects were similar. The mean value +/- 1 SD was 5.8+/-2.5 micromol/L (range, 1.6 to 14.1 micromol/L) in the patients with SCA and 6.1+/-2.7 micromol/L (range, 1.7 to 15.3 micromol/L) in 73 pediatric control subjects. In a subpopulation of the study group (34 children), simultaneous serum folate, red cell folate, and total homocysteine concentrations were also measured. Their serum folate and red cell folate concentrations were normal: 12.4+/-10.0 nmol/L (range, 1 to 42 nmol/L) and 604+/-374.7 nmol/L (range, 205 to 1741 nmol/L), respectively. There was no correlation of plasma homocysteine concentration with various clinical or laboratory measures or with red cell folate concentration. CONCLUSION: Folate stores in children with SCA not receiving folic acid supplements are adequate despite an underlying hemolytic anemia.     

Burkholderia cepacia in cystic fibrosis: novel Australian cluster strain without accelerated respiratory deterioration

OBJECTIVE: To determine whether 4 years of colonization with a novel Australian cluster strain (The 'Hunter' strain) of Burkholderia cepacia (B. cepacia) in cystic fibrosis (CF) patients was associated with more rapid decline in nutritional status and pulmonary function than in non-colonized contemporaries from the same CF clinic. METHODOLOGY: A retrospective review of respiratory function and nutritional data from a single multidisciplinary paediatric CF clinic over 4 years (1993-97). RESULTS: Paired spirometry data for 1993 and 1997 were available in 47 patients without (n = 31) and with B. cepacia (n = 16) colonization (mean (+/- SD) ages in 1993: 12.1 years +/- 4.0 vs 12.6 years +/- 6.5; P = 0.83). Their percentage predicted forced expiratory volume in 1 s (FEV1) (94.2% +/- 16.7 vs 85.9% +/- 21.2; P = 0.19) were not significantly different. The averaged annual fall in FEV1 over 4 years was also not significantly different (3.8% +/- 3.8 vs 3.6% +/- 3.7; P = 0.82). Weight percentile (Wt%), height percentile (Ht%) and percentage age weight for height (%WFH) were not significantly different between groups in 1993. By 1997, Wt% (36.7% +/- 25.1 vs 22.3% +/- 19.6; P = 0.04) and Ht% (42.5% +/- 29.6 vs 17.6% +/- 19.4; P = 0.002) but not %WFH (102% +/- 10.0 vs 106% +/- 11.2; P > 0.10) were lower in subjects with B. cepacia. CONCLUSIONS: In adolescent CF patients, colonization with the Hunter strain of B. cepacia was associated with a deterioration in some nutritional parameters but not with an accelerated decline in FEV1 over 4 years. As varying pathogenicity of B. cepacia strains may account for differing rates of pulmonary decline, further assessment of the consequences of colonization with certain strains of B. cepacia in CF is needed.     

Relationship between vasoactive intestinal peptide and intrapulmonary vascular dilatation in children with various liver diseases

AIM: To evaluate the potential of vasoactive intestinal peptide (VIP) as a pathogenic factor of intrapulmonary vascular dilatation (IVD) in hepatopulmonary syndrome (HPS). BACKGROUND: HPS comprises a triad comprising liver dysfunction, IVD and hypoxaemia. Although the pathogenesis of the process has not been elucidated, many vasodilating substances, such as VIP, have been implicated in the development of pulmonary vascular abnormalities. IVD can be detected by contrast-enhanced echocardiography (CEE) before the development of abnormal gas exchange. METHODS: Forty-two children (20M, 22F; mean age 4.39 +/- 4.17 y) with various liver diseases who attended the paediatric liver clinic of King Chulalongkorn Memorial Hospital between March 2000 and February 2001 were recruited to the study. Each patient was tested for transcutaneous O2 saturation, CEE (applying the agitated normal saline technique), liver function test and serum VIP level. RESULTS: Fourteen of the 42 patients (33%) were CEE positive. Only one of the 14 patients had associated hypoxia and clinical cyanosis. The serum VIP levels of children with liver disease were significantly higher than those of the controls (60.21 +/- 35.04 pg/ml vs 43.71 +/- 34.61 pg/ml, p = 0.03). CEE-positive children tended to have higher serum VIP levels than CEE-negative children (72.65 +/- 40.31 vs 53.99 +/- 31 pg/ml, p = 0.3). The serum VIP levels of biliary atresia (BA) patients with favourable outcomes (serum bilirubin < or = 34 micromol/L) were not significantly different from those with unfavourable outcomes (serum bilirubin > 34 micromol/L) (42.95 +/- 14.53 pg/ml vs 66.07 +/- 32.17 pg/ml, p = 0.5). CONCLUSIONS: CEE is a non-invasive test for early detection of IVD in children with liver disease. VIP is not solely responsible for the pathogenesis of IVD in HPS. Further studies are required to determine which substances cause the development of IVD.     

Nitrites in induced sputum as a simple and cheap non-invasive marker of airway inflammation for asthmatic schoolchildren.

To determine if there are differences in the nitric oxide metabolites (nitrites) in sputum of patients with persistent asthma and healthy schoolchildren, we performed a case-control study in a tertiary care hospital in Arequipa, Perú. Nitrites in induced sputum samples were measured using the Griess assay in 30 persistent asthmatics (mean age of 10.1 yr) and 30 controls (mean age of 11.9 yr). The mean +/- s.d. of nitrites among asthmatics was significantly higher than the controls (16.30 +/- 8.6 vs. 10.25 +/- 4.68 nmol/ml, respectively, p = 0.001). Moreover, the nitrite level in the sputum in children with severe persistent asthma was higher than in the level found in the moderate and mild asthmatics (32.83 +/- 9.48 vs. 18.10 +/- 1.96 vs. 11.84 +/- 4.73 nmol/ml, respectively, p < 0.01 for linear trend). This study showed for the first time in children that asthmatics have significantly higher levels of nitrites in induced sputum than healthy controls and that the level of nitrite correlates with the severity of the asthma. Nitrite levels in sputum, a simple and cheap, non-invasive method, may be a good alternative to measure the severity of inflammation in asthmatic children.     

Differences in resting energy expenditure between male and female children with cystic fibrosis

OBJECTIVES: To evaluate which factors might contribute to raised resting energy expenditure (REE) in patients with cystic fibrosis (CF). STUDY DESIGN: REE and anthropometry were measured in 134 (males = 68) children with CF and 100 (males = 51) controls (range, 3-18.7 years) in an outpatient setting. Bacterial colonization, liver disease, inhaled steroid use, pancreatic and pulmonary function, sex, and genotype were determined and regression analysis was used to determine the predictors of REE in the group with CF. RESULTS: REE for children with CF was increased on average by 7.2% compared with controls. This increase was greater for females than for males. REE in males was positively associated with fat-free mass (FFM), pancreatic insufficiency (PI), and liver disease, and negatively associated with pulmonary function, whereas in females, REE was positively associated with FFM and PI. REE (adjusted for FFM) was higher in children with a severe mutation (5495 +/- 47 kJ) compared with a mild mutation (5,176 +/- 124 kJ, P <.02). CONCLUSIONS: PI, severe mutations, and female sex are the main contributing factors to elevated REE in patients with CF with near normal pulmonary function.     

肾病综合征患儿血清铁与转铁蛋白的变化

目的：肾病综合征(NS)患儿尿中丢失白蛋白的同时也伴有转铁蛋白的丢失,测定血清铁及转铁蛋白等铁代谢相关指标以及尿转铁蛋白,了解其变化及其相互关系。方法：NS患儿37例,测定其治疗前和恢复期铁代谢相关指标(血清铁、铁蛋白、转铁蛋白、转铁蛋白饱和度、总铁结合力以及外周血红细胞参数)及尿转铁蛋白,并与正常对照组比较。结果：①在NS治疗前血清铁为18.8±3.8μmol/L,分别与恢复期的21.0±3.5μmol/L,及对照组的22.2±3.8μmol/L比较,差异有显著性(P<0.01);转铁蛋白为1.9±0.3g/L,分别与恢复期的2.9±0.6g/L和对照组的3.1±0.5g/L比较,差异有显著性(P<0.01);总铁结合力为56.4±9.2μmol/L,分别与恢复期的51.9±7.7μmol/L和对照组的50.7±6.8μmol/L比较,差异亦有显著性(均P<0.01);转铁蛋白饱和度为(55.7±9.2)%,与NS恢复期及对照组的(47.4±13.3)%,(46.4±8.2)%比较,差异有显著性(P<0.01)。②血清白蛋白与转铁蛋白呈正相关(r=0.609,P<0.01)。③血清转铁蛋白浓度与尿转铁蛋白呈负相关(r=-0.550,P<0.01)。结论：NS患儿血清铁及转铁蛋白明显降低,可能与转铁蛋白从尿中丢失有关。     

Water-miscible tocopherol is not superior to fat-soluble preparation for vitamin E absorption in cystic fibrosis

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.     

Effect of feeding type on the efficacy of phototherapy

OBJECTIVE: The objective of this study was to assess the efficacy of phototherapy for nonhemolytic hyperbilirubinemia and rebound bilirubin levels in breast-fed newborns as compared with mixed-fed (breast milk and formula) newborns. STUDY DESIGN/SETTING: Prospective study of effects of feeding type on response to phototherapy in newborns. METHODS: The subjects were 53 full-term healthy newborns with nonhemolytic hyperbilirubinemia [defined as total serum bilirubin 12 mg/dL (205.2 micromol/L) in the first 48 hours of life or 15 mg/dl (256.5 micromol/L), on subsequent days]. Groups were formed according to type of feeding. Group 1 consisted of 28 breast-fed newborns and group 2 consisted of 25 mixed-fed newborns. Phototherapy was terminated when total serum bilirubin concentration fell to 14 mg/dL (< 239.4 micromol/L). Rebound bilirubin measurements were obtained 24 hours after phototherapy ended. RESULTS: The groups were comparable with respect to age at the start of phototherapy. The amount of weight loss (relative to birth weight) recorded at the start of phototherapy was significantly greater in group 1 than in group 2 (8.1+/- 3.9% vs. 5.4+/- 2.6% p = 0.004). The duration of phototherapy was significantly longer in group 1 than in group 2 (38.6+/- 12.6 h vs. 26.8+/- 9.4 h; P < 0.001). The 24-hour rate of decrease in bilirubin concentration in group 2 was significantly higher than that in group 1 [5.4+/- 2.2 mg/dL/d (92.3+/-37.6 micromol/L/d) vs. 4+/- 1.3 mg/dL/d (68.4+/- 22.2 micromol/L/d); p = 0.01]. The overall rate of decrease in bilirubin concentration in group 1 was significantly lower than that in group 2 [0.16+/- 0.05 mg/dL/h (2.73+/- 0.85 micromol/L/h) vs. 0.22+/- 0.09 mg/dL/h (3.76+/- 1.53 micromol/L/h); p = 0.01]. There was no significant difference between the two groups with respect to rebound bilirubin concentration (P = 0.184). Conclusion: Phototherapy effectively reduced bilirubin levels in breastfed newborns with hyperbilirubinemia, but these patients show significantly slower response to this treatment than mixed-fed newborns.     

头部贴敷式亚低温对HIBD新生大鼠脑组织线粒体ATP酶活性的影响

目的：研究头部贴敷式亚低温治疗对缺氧缺血脑损伤（hypoxic-ischemic brain damage, HIBD）新生大鼠的脑组织线粒体ATP酶活性的影响,以进一步探讨亚低温对缺氧缺血脑组织的保护机制。方法：将84只Wistar新生鼠随机分为4组：假手术常温对照组、假手术亚低温对照组、HIBD模型常温恢复组、HIBD模型亚低温治疗组。各组动物在HI后不同时间点（2,6,12 h）断头取脑,提取脑组织线粒体并测定其中Na+K+ATP酶和Ca2+ATP酶活性。结果①HIBD常温恢复组及亚低温治疗组在2,6,12 h时Ca2+ATP酶活性均呈下降趋势,分别为3.17±0.81,2.26±0.53,1.31±0.78 μmol/mgPr．h及5.25±0.61,4.59±0.81,4.61±0.62 μmol/mgPr．h,但亚低温治疗各组该酶活性均明显高于相应的常温组（P<0.01）。②HIBD常温及亚低温2 h组Na+K+ATP酶活性较之假手术组无明显改变,6 h、12 h组该酶活性明显低于假手术组,分别为3.76±0.78,3.12±0.53 μmol/mgPr．h及5.25±0.66、 4.74±0.80 μmol/mg Pr．h,但亚低温治疗组明显高于相应常温组（P<0.01）。结论 贴敷式局部亚低温可增加HIBD后脑线粒体ATP酶活性,保护脑组织。［中国当代儿科杂志,2007,9（4）：305-307］     

Effects of montelukast on symptoms and eNO in children with mild to moderate asthma

BACKGROUND: Asthma is a chronic inflammatory airway disease. Exhaled nitric oxide (eNO) is a marker reflecting airway inflammation. This study was conducted to investigate whether montelukast, a leukotriene receptor antagonist, could be used for the management of asthma and how fast the montelukast sodium decreased airway inflammation as demonstrated by eNO levels. METHODS: Twenty children aged 6-14 years (mean age: 9.2 +/- 2.4 years; mean weight 30 +/- 4.6 kg) with mild to moderate asthma were recruited for the study. They received montelukast plus an inhaled short-acting beta2 agonist as open and uncontrolled therapy. Asthma score (AS) and peak expiratory flow rate (PEFR) and eNO concentrations were measured at pretreatment (0 week) and post-treatment (1 and 2 weeks) as well as 2 weeks after withdrawal of therapy. RESULTS: In one week, the eNO levels (33.3 +/- 15.5 p.p.b. vs 14.8 +/- 8.6 p.p.b.; P < 0.05), and AS (4.2 +/- 1.3 vs 1.8 +/- 1.3; P < 0.05) decreased rapidly, and PEFR (206.9 +/- 69.7 L/min vs 236.2 +/- 69.8 L/min; P < 0.05) increased. Concurrent beta2 agonist use decreased from a mean +/- SD of 2.2 +/- 0.4-1.3 +/- 0.3 puffs per weeks (P < 0.05). After the withdrawal of treatment for 2 weeks, the eNO levels (29.2 +/- 16.1 p.p.b) rebounded again, although the improvements in AS (1.1 +/- 1.3) and PEFR (245.0 +/- 91.3 L/min) persisted. CONCLUSION: Oral montelukast sodium treatment of these children with mild to moderate asthma effectively improved asthmatic symptoms and suppressed airway inflammation in 1 week, suggesting that this leukotriene antagonist combined with short-acting beta2 agonists may provide effective treatment option in mild to moderate childhood asthma. Larger, controlled, and double-blinded studies are needed to confirm these preliminary open uncontrolled observations.     

Insufficient adrenarche in patients with combined pituitary hormone deficiency caused by a PROP-1 gene defect

Adrenarche was evaluated in five patients, aged 17.4 +/- 3 years, with combined pituitary hormone deficiency (CPHD), caused by a PROP-1 gene defect. Adrenocorticotrophic hormone (ACTH), cortisol and dehydroepiandrosterone sulfate (DHEAS) were determined prior to and following the administration of corticotropin-releasing hormone (CRH) in four of the five patients, while only basal values of ACTH, cortisol and DHEAS were determined in the fifth. In the four patients in whom a CRH test was carried out, the mean basal values of cortisol, ACTH and DHEAS were 289 +/- 140 nmol/l, 4.5 +/- 1.7 pmol/l and 0.26 +/- 0.36 micromol/l, respectively. The corresponding post-CRH peak values were 584 +/- 204 nmol/l, 12.7 +/- 3.9 pmol/l and 0.43 +/- 0.41 micromol/l. In the fifth patient, basal ACTH, cortisol and DHEAS values were 4 pmol/l, 411 nmol/l, and 2.33 micromol/l, respectively. Thus the basal and post CRH values of DHEAS (a marker of adrenarche) were low for age, while basal and post-CRH cortisol and ACTH values were within normal limits. For the interpretation of these findings two hypotheses can be proposed: 1) The PROP-1 gene is only expressed in the pituitary, and the role of PROP-1 is related to the maturation of the cells which synthesize the presumed adrenal androgen stimulating hormone (AASH). 2) The PROP-1 gene is also expressed in the adrenal cortex and, when defective, the zona reticularis does not function appropriately. Regardless of the interpretation     

Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis

OBJECTIVE: To determine the relation of growth and nutritional status to pulmonary function in young children with cystic fibrosis (CF). STUDY DESIGN: The relation of weight-for-age (WFA), height-for-age (HFA), percent ideal body weight (%IBW), and signs of lung disease at age 3 years with pulmonary function at age 6 years was assessed in 931 patients with CF. Associations of changes in WFA from age 3 to 6 on pulmonary function were also assessed. RESULTS: WFA, HFA, and %IBW were poorly associated with lung disease at age 3 years, but all were strongly associated with pulmonary function at age 6 years. Those with WFA below the 5th percentile at age 3 had lower pulmonary function at age 6 compared with those above the 75th percentile (FEV(1): 86 +/- 20 [SD] versus 102 +/- 18 % predicted, respectively). Pulmonary function was highest in those whose WFA remained >10th percentile from age 3 to 6 (FEV(1): 100 +/- 19 % predicted) and lowest in those who remained <10th percentile (84 +/- 21 % predicted). Patients with signs and symptoms of lung disease at age 3 years had lower pulmonary function at age 6 years. CONCLUSIONS: Aggressive intervention early in life aimed at growth and nutrition and/or lung disease may affect pulmonary function.