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1.
Aim: To investigate the association between gastroduodenal mucosal damage and symptoms of the digestive tract in children with juvenile chronic arthritis (JCA) Methods: This was a prospective, open, non-randomized study. Gastroscopy was performed on 45 children with active JCA in 1996-2000. Gastrointestinal symptoms before and during the treatment were noted, as was the length of antirheumatic medication, for which the data were retrospectively assessed. Plasma haemoglobin (Hb) and mean corpuscular volume (MCV) levels and erythrocyte sedimentation rate (ESR) were analysed. Mucosal biopsies were obtained for histology and Helicobacter pylori culture. All patients were taking non-steroidal anti-inflammatory drugs (NSAIDs) and 11 (24.4%) were on peroral steroids; 16 (35.6%) were receiving hydorxychloroquine, 9 salazopyrine, 5 myocrisine and 14 methotrexate. Results: Seven children (15.6%) were found to have active inflammation in their gastric and/or duodenal mucosa, two having ulcers and two being infected with H. pylori. Abnormal endoscopic findings were more common in symptomatic children (n = 24) than in children without symptoms (n = 21) (75% vs 38%, p = 0.017). There was no clear association between the Hb or MCV level and the degree of gastroduodenal inflammation (p = 0.98 and 0.7, respectively). Significantly more children (66.6% vs 33.3%) experienced abdominal pain after beginning medical therapy than before therapy (p = 0.02).

Conclusion: Endoscopic evaluation of patients with JCA and receiving NSAIDs should be considered at least in symptomatic cases.  相似文献   

2.
Aim: To compare three different schedules in severe meconium aspiration syndrome (MAS) treatment: standard, bronchoalveolar lavage (BAL) with diluted surfactant, and diluted surfactant BAL plus a single early dexamethasone dose. Methods: Twenty-four full-term newborns with severe MAS (needing mechanical ventilation and with oxygenation index ≥15) were divided into three groups: group I (historical control group; n = 6) treated with standard therapy; group II (n = 7) treated in the first hours of life with one BAL using diluted surfactant (beractant 5 mg/mL) in a volume of 15 mL/kg in four aliquots; and group III (n = 11) treated with one diluted surfactant BAL and a previous single dose of intravenous dexamethasone (0.5 mg/kg) Results: At 12 h, groups II and III showed a significant improvement in oxygenation index (OI) compared with group I (14.7% and 27.0% vs -19.6% respectively; p = 0.012). Group III also showed a significantly lower OI than group I at 24 h (63.6% vs -27.9%) and at 48 h (87.1% vs 49.6%). Group III, in comparison to group I, showed a lower FiO 2 requirement at 12 h (0.66 vs 1), at 24 h (0.4 vs 0.87) and at 48 h (0.35 vs 0.67), and a decrease in the number of days of inhaled nitric oxide administration, mechanical ventilation, oxygen therapy and hospitalisation period. All patients from groups II and III survived and none developed pneumothorax or respiratory infections.

Conclusion: Diluted surfactant BAL in the first hours of life combined with an intravenous single dose of dexamethasone may be an effective treatment for severe MAS.  相似文献   

3.
Aim: To review the long-term efficacy and safety of splenectomy in children with chronic idiopathic thrombocytopenic purpura (cITP). Patients and methods: Data from 33 splenectomized children were retrospectively analysed (median follow up period: 18.8 y from the removal of the spleen). The median age of children at splenectomy was 12 y and the median ITP duration 3.3 y. Indications for splenectomy were: persistent severe thrombocytopenia with extensive purpura, epistaxis and/or gum bleeds, menorrhagia (n = 5) and severe or recurrent haemorrhage from various sites (n = 11). Results: Eighty-five per cent of the patients showed an excellent (n = 26) or partial response to splenectomy. Five children (15%), all females, failed to respond. Of the responders, 25% experienced a transient recurrence of thrombocytopenia within 6 mo to 4 y from splenectomy. The mortality rate due to severe sepsis was 3%. However, the majority of the splenectomized patients have not so far suffered any severe or mild bacterial infection, despite incomplete vaccination and/or antibiotic prophylaxis.

Conclusion: Splenectomy remains the only effective therapeutic modality for children with cITP, although it is associated with transient recurrence and rarely with post-splenectomy sepsis, which could be fatal. Nonetheless, splenectomy should be the last treatment option for the cITP patient, after all available therapeutic modalities have been exhausted and the child still remains profoundly thrombocytopenic and symptomatic.  相似文献   

4.
Background: Infants with severe respiratory failure are frequently transferred to high volume strategy, high frequency oscillation (HFO). Mean airway pressure (MAP) is then elevated, the aim being to open up atelectatic lungs and hence improve gas exchange. Aim: To test the hypothesis that lung volume prior to transfer would predict the response to high volume strategy HFO and identify which factors related to poor outcome (death). Methods: Lung volume was assessed by measurement of functional residual capacity (FRC) and the response to HFO determined by the change in the alveolar arterial gradient (AaDO 2 ) on transfer from conventional mechanical ventilation (CMV) to the optimal MAP on high volume strategy HFO. Patients: Forty-two infants with a median gestational age of 28 (range 23 to 40) wk were studied. Results: FRC prior to transfer correlated significantly with the change in MAP necessary to optimize oxygenation (p = 0.012), but not the change in AaDO 2 in response to HFO. There were no significant differences in the lung volumes of survivors and non-survivors, but those who died were more immature (p = 0.0009) and had a smaller response to HFO (p = 0.035).

Conclusion: Lung volume prior to transfer to high volume strategy HFO might be helpful to guide oscillatory settings, but is a poor predictor of the response to high volume strategy HFO.  相似文献   

5.
Background: Cigarette smoking and cocaine use in pregnancy are common in the US and both are risk factors for sudden infant death syndrome (SIDS). Although the cause of SIDS is not known, one postulated mechanism involves abnormalities of arousal and arousal regulation. Cigarette smoking and cocaine use may cause deficits of arousal. Many believe arousal deficits occur prenatally. Aims: The aim of this study was to assess the effects of cigarette smoke and cocaine exposure during pregnancy on measures of fetal arousal and arousal competency: 1) the fetal response to vibroacoustic stimulation (VAS) and 2) habituation to VAS. Hypothesis: Maternal cigarette smoking and cocaine use in pregnancy are associated with altered arousal and arousal regulation in the fetus. Methods: Three groups of mother-fetal dyads were enrolled: 1) cigarette smokers (n = 54), 2) cocaine users (n = 30), and 3) controls (n = 60). One hundred eight fetuses were tested at 29-31 wk gestation, 119 at 32-35 wk, and 118 at 36+ wk. The fetal response to VAS was assessed using real-time ultrasound and a paradigm of arousal responsiveness. Responders were tested with repeated VAS to assess habituation. Also, the quality of fetal reactivity to repeated stimuli was assessed as a measure of arousal and arousal regulation competence (Behavioral Reactivity Scale). Results: The control group had a larger proportion of fetuses who were too active to initiate testing ("too active to test") (p = 0.013); the proportion of fetuses too active to test decreased with increasing gestational age. The majority of the fetuses who could be tested responded to the initial VAS, and there were no group differences. The proportion of fetuses that habituated and the rate of habituation did not differ between the groups. Behavioral reactivity did not differ between groups.

Conclusions: The original hypotheses were not confirmed. However, the chosen assessment paradigms may have lacked sensitivity. The proportion of fetuses that were "too active to test" decreased with gestational age. The control group had a larger proportion of fetuses that were "too active to test" compared with the exposure groups. We speculate that these findings indicate that prenatal exposure to these neuroteratogens may have produced an acceleration of the behavioral response to vibroacoustic stimulation.  相似文献   

6.
Aim: To compare intrapleural streptokinase and placebo in paediatric empyema. Methods: Children with empyema greater than stage 5 received intrapleural streptokinase (n = 19) or normal saline (n = 21) along with intercostal drainage. Clinical and serial sonographic outcomes were compared. Results: Although there was no difference in clinical and sonographic outcome, none of the children with stage 7 empyema (multi-loculated empyema) who received streptokinase developed pleural thickening 30 d later.

Conclusion: There is no short-term clinical benefit of intrapleural streptokinase in paediatric empyema; this therapy may be reserved for those with stage 7 empyema to prevent pleural thickening in the long term.  相似文献   

7.
Aim: To establish reference values for disaccharidase activities in Belgian children and to compare enzyme activities with those of non-Belgian Caucasian children. Methods: Data from Belgian children who had undergone endoscopic jejunal biopsies (1994-2000) for suspected malabsorption were reviewed. The patients were divided into three groups based on histology: (A) normal (n = 201), (B) moderate changes (n = 58) and (C) (sub)total atrophy (n = 14). The 95% reference limits for disaccharidase activities (U/g protein) were calculated for group A after exclusion of patients with a positive hydrogen breath test, a history of lactose intolerance or coeliac disease (final population: n = 151, 0.1-12 y). Values were compared with those of 34 non-Belgian Caucasian children with normal histology (28 of Mediterranean origin). Results: The reference limits (90% confidence interval) were 86 (65-111)-423 (366-494) for maltase, 9 (6-12)-91 (78-122) for lactase and 24 (18-30)-155 (120-184) for sucrase. No gender-related differences in enzyme activities were found. Lactase levels showed a slight decrease with increasing age. Disaccharidase activities of children with histologically confirmed mucosal injury were significantly lower than those of children with normal histology: median values for groups A, B and C were 208, 181 and 96, respectively, for maltase, 40, 28 and 7, respectively, for lactase and 69, 54 and 25, respectively, for sucrase. Median disaccharidase activities in biopsies with normal histology were lower in non-Belgian children, the difference being only statistically significant for lactase, 33 versus 40.

Conclusion: The reference values for Belgian children are well in line with other reported values from Caucasian children. Although enzyme activities are lower in children with histologically confirmed mucosal damage, they do not allow differentiation between histology groups. Lower lactase values were found in non-Belgian children.  相似文献   

8.
Aim: To evaluate whether very preterm babies can be extubated successfully to nasal continuous positive airway pressure (nCPAP) within one hour of birth after receiving one dose of surfactant in the treatment of respiratory distress syndrome (RDS). Methods: Forty-two infants of 25 to 28[Formula: See Text] wk of gestation were intubated at birth and given one dose of surfactant. They were then randomized within one hour of birth to either continue with conventional ventilation or to be extubated to nCPAP. Results: Eight out of 21 (38%) babies randomized to nCPAP did not require subsequent reventilation. (Ventilation rates of 62% vs 100%, p = 0.0034). The smallest baby successfully extubated weighed 745 g. There were also significantly fewer infants intubated in the nCPAP group at 72 h of age (47% vs 81%, p = 0.025). There was no significant difference between the two groups in the number of babies that died, developed chronic lung disease or severe intraventricular haemorrhage.

Conclusion: A significant number of very preterm babies with RDS can be extubated to nCPAP after receiving one dose of surfactant. nCPAP is a potentially useful modality of respiratory support even in very premature infants.  相似文献   

9.
Aim: To compare the haemodynamic status during high-frequency oscillatory ventilation and conventional mechanical ventilation in very preterm infants with respiratory distress syndrome. Methods: Thirty-two neonates of less than 30 wk gestation randomly assigned to high-frequency oscillatory ventilation (n = 15) or conventional mechanical ventilation (n = 17) had three echocardiographies and one cerebral Doppler-echography under the same ventilation during the first 48 h of life. Results: Mean airway pressure was 2 cm H[Formula: See Text]O higher in infants ventilated with high-frequency oscillatory ventilation at the different echocardiographies. Comparable right ventricular indexes were observed in the two groups. Reduction of the ductus arteriosus diameter and ductal closure were significant only in neonates ventilated conventionally. Left ventricular performance and left ventricular contractility did not differ between the groups. The high-frequency group had lower end diastolic velocity and a higher resistance index in the anterior cerebral artery.

Conclusion: Compared with conventional mechanical ventilation, high-frequency oscillatory ventilation was achieved without altering cardiac function. However, the inability of the left ventricle to improve its performance in the presence of a significant ductal shunt suggests a narrow range of optimal pressures under this ventilatory mode.  相似文献   

10.
Early observations emphasized the possible development of hyponatraemia in hypothyroid children and adults, but recently this has been questioned. Aim: To investigate whether hyponatraemia develops in hypothyroid status by examining sodium handling in screening-detected neonates and infants with congenital hypothyroidism (CH). Methods: Serum thyroid-stimulating hormone (TSH), free thyroxine (FT4), sodium (Na), creatinine (Cr), urinary Na, Cr, fractional sodium excretion rate (FENa) and other chemicals were measured before and after L-thyroxine (LT4) replacement therapy in 32 screening-detected CH neonates (11M, 21F) and 16 age-matched control neonates. Results: No cases of hyponatraemia were found in the 32 CH neonates. Their serum Na concentrations (139.1 ± 1.5 mmol/L, ranging from 136 to 142 mmol/L, median 139 mmol/L) were not statistically different from those of 16 control neonates (139.3 ± 1.3 mmol/L, ranging from 137 to 142 mmol/L, median 139 mmol/L,). No correlation was found between serum levels of TSH and FT4 and serum Na or FENa. No significant changes were found in serum Na concentrations in hypothyroid neonates two months after LT4 replacement therapy. The serum Na concentration (139.1 ± 0.3 mmol/L, n = 25) before treatment did not change statistically (138.9 ± 0.2 mmol/L, n = 25) two months after LT4 replacement therapy.

Conclusion: As seen in various earlier reports, hyponatraemia can occur in hypothyroid patients, but no causal relationship exists between them. When hyponatraemia is detected in hypothyroid children, it does not seem to be directly related to lack of thyroid hormones and therefore other possible causes should be sought.  相似文献   

11.
Aim: To examine whether regulatory problems in infancy predict later hyperkinetic symptoms in childhood and pre-adolescence. Methods: In a prospective longitudinal study of 319 children at risk of later developmental problems and psychopathology, hyperkinetic behaviour problems were assessed at the ages of 2, 4.5, 8 and 11 y by means of a standardized parent interview. Infant regulatory problems at the age of 3 mo were determined from multiple sources of information. An observational procedure was used to assess the quality of mother-infant interaction. Results: At the age of 3 mo, 17% of the infants (n = 55; 27 boys, 28 girls) suffered from multiple regulatory problems. Compared to a control group (n = 264), these children presented more hyperkinetic symptoms throughout childhood. Negativity in the mother-infant interaction and early family adversity each contributed to later hyperkinetic symptoms. When controlling for family adversity, the association between infant multiple regulatory problems and later hyperkinetic problems was rendered insignificant.

Conclusions: These findings suggest that multiple regulatory problems may not be a key variable for later hyperkinetic problems. The impact of early family adversity factors clearly outweighed that of infant psychopathology on later behaviour disorder.  相似文献   

12.
Aim: To examine infant morbidity risks associated with refraining from breastfeeding where it is used in an attempt to prevent mother-to-child transmission (MTCT) of human immunodeficiency virus (HIV). Methods: The population consisted of infants born to HIV-infected women in South Africa who were participating in a vitamin A intervention trial to prevent MTCT of HIV. Women chose to breastfeed or formula feed their infants according to UNAIDS guidelines. Actual feeding practices and morbidity were recorded at clinic follow-up visits at 1 wk, 6 wk, 3 mo and every 3 mo thereafter until 15 mo of age or cessation of breastfeeding. The infant's HIV status was assessed according to a predetermined algorithm. Results: HIV-infected infants who were never breastfed had a poorer outcome than those who were breastfed; 9 (60%) of those who were never breastfed had 3 or more morbidity episodes compared with 15 (32%) of breastfed children [odds ratio (OR) 4.05, 95% confidence interval (95% CI) 0.91-20.63, p = 0.05]. During the first 2 mo of life, never-breastfed infants (regardless of HIV status) were nearly twice as likely to have had an illness episode than breastfed infants (OR 1.91, 95% CI 1.17-3.13, p = 0.006).

Conclusion: The significant extra morbidity experienced in the first few months by all never-breastfed infants and at all times by HIV-infected infants who are not breastfed needs to be considered in all decisions by mothers, health workers and policy makers so as not to offset any gains achieved by decreasing HIV transmission through avoiding breastfeeding.  相似文献   

13.
Aims: To describe the base deficit (BD) values and the rate of postnatal recovery of the BD of infants with hypoxic ischemic encephalopathy due to intrapartum asphyxia; and to explore the relationships between the rate of recovery of BD, severe adverse outcome and different time patterns (acute total vs prolonged partial) of asphyxia. Methods: Clinical and laboratory data were collected from the neonatal period (n = 244) and outcome data to a minimal age of 1 y (n = 218). Rates of recovery of BD were described in four 60 min blocks of time. The values of rate of recovery were compared between the outcome groups, ignoring correlation structure within subjects and with adjustment by the generalized estimating equations method. Results: The BD normalized within 4 h of birth in all but 9 of 244 infants. The rates of recovery of BD in infants with good and severe adverse outcome respectively were 0.11 [95% confidence interval (95% CI) 0.07, 0.14] and 0.09 (95% CI 0.07, 0.12) mmol l -1 min -1 over the first 4 h after birth. The rates of recovery were similar with or without buffer therapy, and after acute near-total and prolonged partial asphyxia.

Conclusion: The BD in the great majority of infants with severe intrapartum asphyxia normalizes within 4 h of birth. The BD recovery rate of infants with adverse outcomes was similar to those with relatively good outcome. The different time patterns of asphyxial episode were not associated with differential recovery profiles.  相似文献   

14.
Aim: To investigate whether inherited factors, other than those already known, influence the bilirubin concentration in neonates of northern European descent, by comparison of monozygotic and dizygotic twins with respect to differences in the plasma bilirubin concentrations between the twins. Methods: 77 healthy pairs of twins of the same gender with a gestational age ≥250 d and of northern European descent were included. Fourth postnatal day blood sampling was done. A multiple linear regression analysis was carried out with the difference in serum bilirubin concentration between the twins as the independent factor, and zygosity, gender, gestational age, postnatal age, maternal smoking, ABO blood-type incompatibility, and the differences between the twins in blood haemoglobin concentration, formula feeding and weight loss as dependent factors. Results: 27 pairs of twins were monozygotic and 50 pairs dizygotic. The analysis showed that the difference in serum bilirubin concentration between the twins was dependent on whether the twins were monozygotic or dizygotic, i.e. the estimated difference in serum bilirubin concentration between the monozygotic twins was 17.8 μmol l-1 [SE 6.6 μmol l-1, p = 0.02, 95% confidence interval (95% CI) 3.4, 32.3 μmol l-1] less than the difference between the dizygotic twins, adjusted for the above-mentioned potential confounders. The difference in serum bilirubin concentration between the twins was positively correlated to the difference in weight loss (%) between the twins (adjusted estimate 5.2 μmol l-1, SE 2.1 μmol l-1, p = 0.01, 95% CI 1.2, 9.3 μmol l-1).

Conclusion: In a population of northern European descent, other genetic factors than gender and ABO blood type were significant for the plasma bilirubin concentration in healthy infants.  相似文献   

15.
Aim: Antiepileptic drugs (AEDs) are known teratogens. Some specificity between different AEDs has been noted in the literature. The aim was to compare the teratogenic effect of valproic acid (VPA) and carbamazepine (CBZ) in monotherapy. Methods: Infants exposed to AEDs (n = 1398) in early pregnancy were identified from the Swedish Medical Birth Registry. The number of infants with congenital malformations and exposed to AED was compared with the expected number estimated from all infants born (n = 582 656). Results: 90% (1256) of the AED exposed children were exposed to AEDs in monotherapy, 56% were exposed to CBZ and 21% to VPA. The odds ratio (OR) for having a malformation in the AED exposed group was 1.86 [95% confidence interval (95% CI) 1.42-2.44]. Exposure to VPA in monotherapy compared with CBZ in monotherapy gave OR = 2.51 (95% CI 1.43-4.68) for a neonatal diagnosis of malformations. However, there is no information available on the number of therapeutic abortions, or the different types of epilepsy or drug dosage in the two treatment groups.

Conclusion: There was a small increase in the risk of having a major malformation after exposure to AEDs in monotherapy. Exposure to VPA seems to carry a higher risk than exposure to CBZ.  相似文献   

16.
Aim: To evaluate the trends in the incidence, clinical course and outcome of respiratory distress syndrome (RDS) in the newborn in the Oulu University Hospital region in northern Finland. Methods: In the population of 58 990 infants, the incidence rates of RDS specific to gestational age and birthweight in two consecutive periods, 1990-95 and 1996-99, were calculated. Clinical course and other neonatal morbidities were reported. All surviving infants were followed up until 1 y of corrected age. Results: The overall incidence of RDS did not change significantly (8.7/1000 livebirths in 1990-95 vs 7.6 in 1996-99; p = 0.15), but the gestational age-adjusted incidence decreased between the two consecutive periods (p = 0.005). The frequency of infants with gestational age below 28 wk tended to increase towards the late 1990s, while their RDS incidence remained unchanged. RDS-related neonatal mortality decreased in parallel with neonatal mortality, accounting for 15% of all neonatal deaths. The duration of oxygen therapy shortened (8.0 vs 5.5 d) and the incidence of pneumothorax decreased (9.7 vs 4.1%), whereas the rate of chronic lung disease at 36 wk of postconceptional age (16.4 vs 16.7%) and at 1 y of corrected age (9.2 vs 8.2%) remained unchanged, as did also associated neurosensory morbidity (8.8 vs 9.5%).

Conclusion: During the 1990s, the incidence of RDS shifted towards more immature infants and the gestational-age specific incidence decreased. The course of the disease shortened and acute complications decreased. The frequency of chronic pulmonary sequelae (and associated neurosensory morbidity) at the age of 1 y did not change significantly.  相似文献   

17.
Aim: To identify differences in baseline characteristics and outcome between long-stay and other patients admitted to a paediatric intensive care unit with the same diagnosis. Methods: Over a period of 6 y, data of paediatric intensive care unit patients with a length of stay of 30 or more days (long-stay patients) and aged 1 to 18 y were retrospectively collected. Long-stay patients were matched with the next patient who was admitted to the paediatric intensive care unit with the same diagnosis. Evaluated characteristics on admission included: age, sex, presence of chronic morbidity, functional status, Pediatric Risk of Mortality score, presence of multiple organ system failure and complications during admission. Outcome (survival and functional status) was assessed 2.5 to 8.5 y after admission. Results: Of 19 long-stay patients identified, 15 could be matched with a control patient admitted with the same diagnosis. No significant difference in baseline characteristics was found between long-stay patients and the matched controls. The mean number of complications per long-stay patient was 2.9, compared to 1.2 per control patient (p = 0.02). Infection accounted for half of the complications. Mortality rate in long-stay patients was not higher than in the matched controls (36.8 vs 26.7%, p = 0.54). Paediatric intensive care unit stay did not change functional status in either long-stay patients or controls.

Conclusion: Long-stay patients in the paediatric intensive care unit had more complications, but baseline characteristics, mortality and functional outcome were not different from a control group admitted with the same diagnosis.  相似文献   

18.
Aim: To assess trends in neuro-muscular and cardio-respiratory fitness and morphological fitness in Swedish adolescents between 1987 and 2001. Methods: Comparison of data from two samples, one from 1987 (n = 479) and one from 2001 (n = 1470). Subjects underwent functional tests of muscular strength in the lower limbs, trunk and upper body, sub-maximal ergometrics, and measurement of body mass and height. Results: Cardio-respiratory fitness showed only small changes between 1987 and 2001 among boys, with no changes in girls. Neuro-muscular fitness, as measured by three functional tests, was lower in 2001 compared to 1987. The most pronounced changes in these functional tests were found in the arm-hang test, while changes in lower body and trunk strength tests were less. Our results indicate that the difference in the prevalence of overweight in adolescents between 1987 and 2001 is mainly due to a large change in the most unfit group. The fittest group showed only marginal changes.

Conclusion: We suggest that the differences in neuro-muscular fitness are at least partly due to differences in the amount and nature of physical activity, and that this might lead to consequences for health later in life.  相似文献   

19.
Aim: To investigate the final adult heights and pubertal growth patterns in Japanese patients with congenital hypothyroidism (CH) detected by neonatal screening. Methods: A retrospective chart review was conducted of female patients >15 y of age (n = 18) and male patients >18 y of age (n = 9), who were detected by neonatal screening and kept on continuous thyroid hormone replacement therapy. Final height standard deviation scores (FHSDS) and target height standard deviation scores (THSDS) were determined. Parameters characterizing the pubertal growth process (such as age at onset of pubertal growth spurt and age at peak pubertal growth) were obtained from each patient's growth rate chart. Menarchial age was determined in each female patient by reviewing the medical record. The impact on FHSDS of the etiology of CH, the severity of CH, the time of initiation of therapy and the adequacy of treatment during the first year of life was assessed. Results: All patients had received initial thyroid hormone treatment no later than 50 d of age, and had reached their final height. The mean FHSDS for female and male patients were +0.17 ± 0.99 and -0.03 ± 0.99, respectively. The mean FHSDS-THSDS for female and male patients was +0.09 ± 0.77 and -0.19 ± 0.53, respectively. No difference was seen in pubertal growth parameters for either gender compared with that of the reference population, except for a greater peak height velocity and pubertal height gain in male patients. The mean menarchial age was identical to that of the reference population. No significant relationship was found between the FHSDS and any of the factors investigated.

Conclusion: The adult height of patients with CH detected by neonatal screening was equivalent to that of the reference population and their target height. As long as early intervention and satisfactory management are ensured, severe CH does not appear to reduce final adult height.  相似文献   

20.
Aim: To determine whether a moderately reduced fat diet affects longitudinal growth in children with hypercholesterolaemia with a mean duration of 7.42 ± 1.93 y. Methods: Subjects with hypercholesterolaemia, total cholesterol above 5.18 mmol/L and LDL-cholesterol above 3.49 mmol/L (n = 144; 69 males and 75 females) from 2 to 13 y of age were studied. Patients were seen in our outpatient department for regular health check-ups. Weight and height were measured every year. At the same time, cholesterol, triglycerides, LDL-C, HDL-C, A-apoprotein and B-apoprotein levels were analysed. Furthermore, degrees of compliance at 1 mo, 6 mo and each year after starting the dietary treatment were determined. Results: No significant change in height or weight was found after fat restriction. In contrast, significant reductions in total cholesterol, LDL cholesterol and B-apoprotein levels of 19%, 24% and 14%, respectively, were detected. Finally, no significant differences were seen in HDL-cholesterol, A-apoprotein or triglycerides.

Conclusions: These data support the hypothesis that growth is not influenced by moderate fat restriction in healthy children who otherwise have normal nutrition.  相似文献   

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