Liver transplantation for hepatic cirrhosis in cystic fibrosis.

Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved.     

Successful renal transplantation accelerates development in young uremic children

To examine the impact of renal transplantation on subsequent development of children with chronic renal failure, 37 children undergoing primary renal transplantation at or before 30 months of age whose allograft functioned for at least 1 year were prospectively studied. Psychometric tests were performed an average of 4 months before transplantation; reevaluation was done an average of 14 months after surgery. Comparison of individual pretransplantation and posttransplantation mental development scores in 33 patients, assessed by either Bayley Mental Development Index or Stanford-Binet Intelligence Quotient, revealed an average increase of 12.6 (P less than .001). After transplantation, there was a significant improvement in mental performance in 12 of 18 patients (P less than .02) from the range of mild delay (Mental Development Index or Stanford-Binet IQ score = 50 to 69) to the range of normal mental development (greater than or equal to 70). The Bayley Psychomotor Development Index scores were frequently lower than Mental Development Index scores and also increased an average of 14.4 (P less than .01) after transplantation in all 12 patients with paired data. Significant individual improvement in occipital-frontal circumference standard deviation score (P less than .001) was noted in 24 children after transplantation. It is concluded that successful renal transplantation in young children with chronic renal failure is often associated with significant improvements in cognitive and psychomotor function, as well as improved cephalic growth.     

Changes in life-style after liver transplantation

Sixty-five pediatric patients who received liver transplants between May 1981 and May 1984 were observed for as many as 5 years and examined for changes in life-style. Children were less frequently hospitalized, spent less time hospitalized, required fewer medications, and generally had excellent liver and renal function after hepatic transplantation as compared with their pretransplantation status. Most children were in age-appropriate and standard school classes or were only 1 year behind. Cognitive abilities remained unchanged. Children improved in gross motor function and patients' behavior significantly improved according to parents' perceptions. Enuresis was more prevalent, however, than in the population of children who had not received liver transplants. Parental divorce rates were no greater than those reported for other families with chronically ill children. Overall, objective changes in life-style as well as parents' perceptions of behavior of children appear to be improved after liver transplantation.     

Changes in psychosocial adjustment after renal transplantation.

The psychological aspects of transplantation were studied in 29 children and adolescents with functioning renal transplants. Most parents rated their child''s physical health as considerably improved after transplantation. They also noted improvements in the child''s behaviour and in the quality of family life. When compared with a sample of children being treated by hospital haemodialysis, children who had received transplants had more favourable parental views of behavioural and emotional adjustment, better parental psychiatric adjustment, and indications of superior child rated mood, self concept, and social functioning. Children who had received transplants did, however, show an increase in minor behavioural symptoms compared with healthy controls, and most parents required continuing psychological and social support as well as attention to physical aspects of the child''s condition. Kidney transplantation is associated with improved psychosocial behaviour in children, but there is a continuing need for support for parents in dealing with minor psychological problems after the transplant.     

Early experience of heart-lung transplantation.

We report our experience of heart-lung transplantation for the treatment of children with terminal respiratory disease. Between May 1987 and October 1988 we performed heart-lung transplantation in five children under the age of 16 (age range 11-15). All the patients were severely disabled by dyspnoea and hypoxia. Two had primary pulmonary hypertension, two cystic fibrosis, and one had Eisenmenger''s syndrome. All five children are alive and well five to 17 months after operation and have returned to activities normal for their age. Three of the five patients had episodes of infection after operation. These were staphylococcal pneumonia, herpes simplex pneumonitis and, in one of the patients with cystic fibrosis, persistent purulent sputum. The mean number of episodes of rejection per child was 2.7 per half year. Heart-lung transplantation is a practical treatment for children in these disease groups with terminal respiratory failure.     

Bronchiectasis in children after renal or liver transplantation: a report of five cases

More effective immunosuppressive treatment in children following organ transplantation has significantly improved the survival of the grafts. Therefore, quality of life, long-term prognosis and adverse drug reactions have become more important. One of the main complications of immunosuppressive drugs is infections of the respiratory tract, but irreversible damage to the airways has not been described after renal or liver transplantation. Five children following transplantation of kidney or liver were referred to the Paediatric Pulmonology department because of chronic respiratory complaints. Pulmonary function tests and HRCT scan were performed as routine patient care. Four children with a renal transplant and one with a liver transplant showed chronic bronchitis and moderate to severe airways obstruction. HRCT showed bronchiectasis in all of them. We speculate that the immunosuppressive treatment (in)directly contributes to irreversible airway damage. We recommend including follow-up of lung function in the post-transplantation protocol and considering bronchiectasis in case of respiratory symptoms, to try preventing further damage to the lung.     

Linear growth after pediatric liver transplantation.

To determine growth patterns in a large cohort of unselected children undergoing liver transplantation, the outcomes of 294 orthotopic liver transplantations performed in 221 children at The University of Chicago between October 1984 and October 1992 were retrospectively reviewed; 66% were alive at the time of this analysis. The mean age at transplantation was 4.1 +/- 5.0 years; 44% of the children were male and 16% of the transplants were from living-related donors. The mean height z score at the time of transplantation was -1.6 +/- 1.8, and 39% of children had height z scores of < -2.0 at transplantation. When children with growth retardation at the time of transplantation (height z scores of < -2. 0) were compared with children with more normal growth, there were no significant differences in gender or re-transplantation rates, although children with growth retardation at transplantation were significantly younger than those with more appropriate growth (2.8 +/- 4.1 years vs 4.7 +/- 5.1 years, P <.05). The height z score of all children with biliary atresia at the time of transplantation was -1.9 +/- 1.7 compared with -1.2 +/- 2.0 in those children with underlying diseases other than biliary atresia. Catch-up growth was seen in 37% to 47% of children at any given time point after transplantation. Children with evidence of catch-up growth (growth velocity z score >0) 2 years after transplantation were more likely to be first-time transplant recipients, had more growth retardation at the time of transplantation, and were receiving lower doses of prednisone at 2 years after transplantation. Younger children were most likely to demonstrate catch-up growth after transplantation. In summary, a large proportion of children have growth retardation at the time of liver transplantation. This growth retardation is inversely correlated with age. Before transplantation, children with biliary atresia grow less well than children with other forms of liver disease. Up to one half of children demonstrate catch-up growth after liver transplantation. Growth after transplantation is proportional to the degree of growth retardation at transplantation and inversely correlated to age at transplantation. Children with poor growth after transplantation are more likely to be receiving higher doses of corticosteroid.     

Respiratory status and allergy nine to 10 years after acute bronchiolitis

In order to evaluate further the relationship between acute bronchiolitis in infancy and subsequent respiratory problems, children prospectively followed up from the time of their admission to hospital were reviewed along with a group of matched controls recruited at the previous five and a half year assessment. Sixty one index children and 47 controls took part. The groups were well matched for age, height, parental smoking, and social class. Although the prevalence of respiratory symptoms had fallen when related to the previous review, there remained an excess of coughing (48 and 17% in index and control children respectively; odds ratio 4.02) and wheezing (34 and 13% in index and control children respectively; odds ratio 3.59). Bronchodilator therapy was used by 33% of index children compared with 3% of controls. Lung function tests revealed no significant differences in the measurements of lung growth-for example, forced vital capacity, functional residual capacity, and total lung capacity-but the index children had significant reductions in measurements of airways obstruction-for example, forced expiratory volume in one second, maximum expiratory flow at 75, 50 and 25% of vital capacity, and airways resistance. Family history and personal skin tests showed no excess of atopy in the index group. This study supports the claim that the excess respiratory symptoms after acute bronchiolitis are not due to familial or personal susceptibility to atopy.     

自体外周血干细胞移植治疗难治性学龄前儿童风湿病

目的探讨自体外周血干细胞移植（auto-PBHSCT）治疗难治性学龄前儿童风湿病的可行性、安全性和有效性。方法幼年皮肌炎（JDM）、幼年系统性红斑狼疮（JSLE）、幼年类风湿关节炎（JRA）各1例,均为男性患儿,年龄分别为3、6,6岁,病程分别为14、3．6、22个月,常规治疗中病情进展,库欣征明显,患病后患儿身高均无增长。JDM患儿肌力Ⅱ级,吞咽肌和呼吸肌受损,明显Gottron’s征和向阳性皮疹;JSLE患儿表现为蝶形红斑、蛋白尿、贫血,脑MRI显示脱髓鞘病变,系统性红斑狼疮疾病活动性指数（SLEDAI）≥12分;JRA患儿双膝、踝、腕、肘严重多关节炎。以环磷酰胺（CTX）＋粒细胞集落刺激因子（G-CSF）进行外周造血干细胞动员,经CliniMACS细胞分选仪分选CD34‘细胞。预处理方案：JSLE和JRA为卡氮芥（BCNU）＋足叶乙甙（VP16）＋阿糖胞苷（Ara-C）＋马法兰（MEL）及抗胸腺球蛋白（ATG）;JDM为CTX＋Mel及ATG。回输CD34^＋细胞数分别为9．45×10^6／kg、5．46×10^6／kg和9．60×10^6／kg。观察移植治疗前后风湿病状态和免疫学指标的变化。结果3例患儿移植后分别于＋9d、＋13d、＋11d粒细胞≥0．5×10^9／L,＋14d、＋18d和＋13d血小板≥20×10^9／L。CIM处于低水平,CD4／CD8倒置。JDM移植后1个月皮疹消失,肌力Ⅴ级,移植后2个月血清肌酶、肌电图恢复正常。JSLE移植后3个月皮疹消退,无蛋白尿,脑MRI病变吸收,移植后8个月自身抗体转阴性,SLEDAI为2～3分。JRA移植后3周关节炎好转,移植后3个月无关节肿胀及活动受限。均停用激素和免疫抑制药物,库欣征消退,18个月身高增长10～15cm,均已上小学或学前班,随访25～27个月无复发。结论auto-PBHSCT治疗难治性学龄前儿童风湿病近期疗效显著,安全性及远期疗效有待进一步观察。     

Continuous positive airway pressure with modified helmet for treatment of hypoxemic acute respiratory failure in infants and a preschool population: a feasibility study.

OBJECTIVE: To analyze the feasibility of using continuous positive airway pressure (CPAP) delivered via a modified helmet to treat children with hypoxemic acute respiratory failure. DESIGN: A single-center, prospective, clinical study. SETTING: Pediatric intensive care unit in a university hospital. PATIENTS: Fifteen consecutive children (from 1 month to 5 yrs of age) with hypoxemic acute respiratory failure (defined as Pao2/Fio2 <300). INTERVENTIONS: CPAP was delivered via a modified helmet (CaStar, Starmed, Italy) of reduced size, fastened by a device we call a "baby-body." The feasibility of CPAP with the helmet was the primary end point. The improvement of gas exchange was the secondary one. MEASUREMENTS AND MAIN RESULTS: Ten of 15 children had multiple organ failure. Nine of 15 children were 相似文献   

Chronic lung disease of prematurity and respiratory outcome at eight years of age

AIM: The study aimed to determine the respiratory outcome of children who had chronic lung disease of prematurity (CLD) compared with a preterm control group of children at school age. METHODS: Fifty-two preterm infants with CLD born between 26 and 33 weeks gestation were assessed regarding respiratory illness with 47 having lung function testing. Information regarding respiratory illness was obtained from 52 children in the birthweight-matched control group of whom 45 had lung function testing. The results were compared between the CLD and control groups. RESULTS: There was no difference in respiratory symptomatology between CLD groups and control preterm infants. On lung function testing, a significantly lower mean forced expiratory flow at 25-75% of vital capacity was identified compared with the preterm controls (P=0.024). This significant difference did not persist after bronchodilator therapy. There was no evidence of increased air trapping or bronchial hyper-reactivity in the CLD children compared with the controls. CONCLUSION: Lung function in CLD children is largely normal in comparison with preterm controls, apart from some evidence of reversible small airway obstruction. Respiratory symptomatology is not increased in chronic disease children in comparison with control preterm children.     

Esophageal atresia

Respiratory complications such as recurrent aspiration, infections, and gastroesophageal reflux are common in children who have been treated for esophageal atresia (EA). Some of these children later present abnormal respiratory function. In this study, we compared two groups of children with repaired EA, each consisting of 9 patients (age 8–21 years). Group I comprised children who had only had minor respiratory complications before the age of 4 years, whereas group II included children who had had severe respiratory complications such as aspiration and pneumonia that continued after that age. The follow-up included esophagography, spirometry, working capacity, and esophageal pH-monitoring. There was no difference between the two groups concerning results in pH-monitoring. Three patients in group II had been treated with antireflux surgery at a younger age. Esophageal function was disturbed, with dysmotility in both groups, but dysfunction was more severe in patients in group II. Symptoms of asthma or bronchitis occurred in 7 patients equally distributed between the groups. There was a significant difference in spirometry results: patients in group II showed more obstructive as well as restrictive changes. There was no difference in maximal working capacity between the two groups. Capillary blood gases were normal in both groups, indicating that the spirometric changes were without significant importance.     

Cytoreductive procedures in the early management in cases of leukemia and hyperleukocytosis in children

Cytoreduction for hyperleukocytosis before the initiation of primary therapy may reduce morbidity and mortality from blast cell lysis in children with acute lymphoblastic leukemia (ALL) and from leukostasis in children with acute nonlymphoblastic leukemia (ANLL) or chronic myelogenous leukemia (CML). The clinical features of 35 children (23 with ALL, 5 with ANLL, and 7 with CML) who underwent cytoreduction before the institution of definitive therapy were studied. Twelve children had exchange transfusions and 23 underwent leukaphereses. The cytoreductive procedures were equally effective in removing peripheral leukocytes (median decrease, 60%) and produced no complications. Ten children required additional cytoreduction because of further leukocyte increase before chemotherapy became effective. Three children with ALL who had renal insufficiency and metabolic derangement prior to leukapheresis subsequently required additional therapeutic measures. Three children with respiratory symptoms attributable to leukostasis improved after cytoreduction, and there were no episodes of intracerebral hemorrhage. These observations demonstrate the safety and efficiency of exchange transfusion and leukapheresis, and provide support for the role of cytoreduction in the early management of cases of hyperleukostasis and leukemia in children.     

Respiratory status and allergy nine to 10 years after acute bronchiolitis

Accepted 30 September 1996
In order to evaluate further the relationship between acute bronchiolitis in infancy and subsequent respiratory problems, children prospectively followed up from the time of their admission to hospital were reviewed along with a group of matched controls recruited at the previous five and a half year assessment. Sixty one index children and 47 controls took part. The groups were well matched for age, height, parental smoking, and social class. Although the prevalence of respiratory symptoms had fallen when related to the previous review, there remained an excess of coughing (48 and 17% in index and control children respectively; odds ratio 4.02) and wheezing (34 and 13% in index and control children respectively; odds ratio 3.59). Bronchodilator therapy was used by 33% of index children compared with 3% of controls. Lung function tests revealed no significant differences in the measurements of lung growth—for example, forced vital capacity, functional residual capacity, and total lung capacity—but the index children had significant reductions in measurements of airways obstruction—for example, forced expiratory volume in one second, maximum expiratory flow at 75, 50 and 25% of vital capacity, and airways resistance. Family history and personal skin tests showed no excess of atopy in the index group. This study supports the claim that the excess respiratory symptoms after acute bronchiolitis are not due to familial or personal susceptibility to atopy.

     

Improved control of hypertension in children after renal transplantation: results of a two-yr interventional trial

Hypertension is a frequent complication in children after renal transplantation and the control of post-transplant hypertension is unsatisfactorily low. The aim of this prospective interventional study was to improve the control of hypertension in children after renal transplantation. Thirty-six children fulfilled the inclusion criteria (> or =6 months after transplantation and no acute rejection in the last three months). BP was measured using ABPM. Hypertension was defined as mean ambulatory BP > or =95th-centile for healthy children and/or using antihypertensive drugs. The study intervention consisted of using intensified antihypertensive drug therapy - in children with uncontrolled hypertension (i.e., mean ambulatory BP was > or =95th centile in treated children), antihypertensive therapy was intensified by adding new antihypertensive drugs to reach goal BP <95th centile. ABPM was repeated after 12 and 24 months. Daytime BP did not change significantly after 12 or 24 months. Night-time BP decreased from 1.57 +/- 1.33 to 0.88 +/- 0.84 SDS for systolic and from 1.10 +/- 1.51 to 0.35 +/- 1.18 SDS for diastolic BP after 24 months (p < 0.05). The number of antihypertensive drugs increased from 2.1 +/- 0.9 to 2.7 +/- 0.8 drugs per patient (p < 0.05), this was especially seen with the use of ACE-inhibitors (increase from 19% to 40% of children, p < 0.05). In conclusion, this interventional trial demonstrated that, in children after renal transplantation, the control of hypertension, especially at night-time, can be improved by increasing the number of antihypertensive drugs, especially ACE-inhibitors.     

Does lung growth occur when mature lobes are transplanted into children?

Lung volume increases after living donor lobar lung transplantation (LD) in children. The mechanism responsible for this increase may be alveolarization (lung growth) or alveolar dilation. The diffusing capacity of the lung for carbon monoxide adjusted for lung volume (DLco/VA) should decrease if alveolar dilation occurs, but not if lung growth occurs. Pulmonary function tests were measured 1-12 months after transplant in 20 children receiving LD transplants and in 11 children receiving cadaveric whole lung transplantation (CL). One month after transplant there were no differences between LD and CL recipients in age, gender, or height. Compared to the first month after transplant, height increased at 6-12 months after LD (p < 0.05), and only at 12 months after CL (p = 0.02). Total lung capacity (TLC) showed an 11-22% increase at 3-12 months after LD, and an 11-14% increase at 6-12 months after CL. DLco/VA showed an 11-17% decrease at 3-12 months after LD. However, in recipients of CL, DLco/VA showed a transient decrease of 10% at 3-6 months post-transplant, but was not significantly lower at 9-12 months. LD recipients had lower DLco/VA values than CL recipients at 6-12 months after transplant (p < 0.05). We conclude that following LD, lung volume increases, but DLco/VA decreases. We speculate that alveolar dilation, rather than alveolarization, is the primary mechanism of increased lung volume in children following LD.     

Favorable outcome of primary liver transplantation in children with cirrhosis and hepatocellular carcinoma

The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation. We considered children with cirrhosis and HCC who had a primary OLT. We retrospectively recorded demographic, medical and surgical features, and MC as predictors of outcome. Among 456 children transplanted in the last 15 yr, 10 (2%), median age at diagnosis 1.8 yr (range 0.5-7.2), had HCC in biliary atresia (3), BSEP deficiency (3), tyrosinemia type 1 (2), complications of choledocal cyst and glycogen storage disease type IV (1 each). At HCC discovery, median AFP was 2322 ng/mL (3-35,000), high or rising in 9/10 patients. Six patients were outside the MC. Median time on the waiting list was 38 days (1-152). Two patients died from early complications of OLT. In the other eight patients, there was no tumor recurrence after a median follow-up of four yr. Children with cirrhosis may develop HCC at a very young age. The outcome appears excellent even outside MC. Primary liver transplantation is advisable for children with cirrhosis, HCC, and no extrahepatic disease.     

Methicillin resistant Staphylococcus aureus (MRSA) infection in cystic fibrosis.

BACKGROUND: Methicillin resistant Staphylococcus aureus (MRSA) infection is increasingly found in patients with cystic fibrosis (CF). AIMS: To determine whether MRSA infection has a deleterious effect on the clinical status of children with CF. METHODS: Children with MRSA in respiratory cultures during a seven year period were identified and compared with controls matched for age, sex, and respiratory function. Respiratory function tests, anthropometric data, Shwachman-Kulczycki score, Northern chest x ray score, intravenous and nebulised antibiotic therapy, and steroid therapy were compared one year before and one year after MRSA infection. RESULTS: From a clinic population of 300, 10 children had positive sputum or cough swab cultures for MRSA. Prevalence rose from 0 in 1992-1994 to 7 in 1998. Eighteen controls were identified. Children with MRSA showed significant worsening of height standard deviation scores and required twice as many courses of intravenous antibiotics as controls after one year. They had significantly worse chest x ray scores at the time of the first MRSA isolate and one year later, but showed no increase in the rate of decline in chest x ray appearance. There was a trend towards lower FEV(1) and FEF(25-75) in children with MRSA. There were no significant differences between the two groups with respect to change in weight, body mass index, or Shwachman score. There was no significant difference in prior use of steroids or nebulised antibiotics. CONCLUSION: MRSA infection in children with CF does not significantly affect respiratory function, but may have an adverse effect on growth. Children with MRSA require significantly more courses of intravenous antibiotics and have a worse chest x ray appearance than controls.     

Continuous veno-venous hemofiltration may improve survival from acute respiratory distress syndrome after bone marrow transplantation or chemotherapy

PURPOSE: Acute respiratory distress syndrome (ARDS) may result from immunologic activity triggered by irradiation and/or chemotherapy. Hemofiltration removes plasma water and soluble components below 25 kilodaltons. The authors hypothesized that early hemofiltration might attenuate the inflammatory component of ARDS, resulting in increased survival in immunocompromised children and young adults. METHODS: Ten children (6 bone marrow transplantation, 3 chemotherapy, 1 lymphoma/hemophagocytosis) with ARDS (Pao2/Fio2 94 +/- 37 torr) received early continuous veno-venous hemodiafiltration as adjunctive therapy for respiratory failure, regardless of renal function. Six children had normal urine output and initial serum creatinine (range 0.1-1.2 mg/dL); four had renal insufficiency (initial creatinine 1.7-2.4 mg/dL). Hemofiltration was instituted coincident with intubation. Respiratory failure was precipitated by Enterobacter sepsis in two patients and by Aspergillus in one. RESULTS: Hemodiafiltration was performed for 13 +/- 9 days. A high rate of clearance was achieved (52 +/- 17 mL/min/1.73 m2). Duration of mechanical ventilation was 14 +/- 9 days. Nine of the 10 children were successfully extubated; 8 survived. CONCLUSIONS: Early hemofiltration may improve survival from ARDS following bone marrow transplantation or chemotherapy. Possible mechanisms include strict fluid balance, immunomodulation through filtration of inflammatory constituents, and immunomodulation through intensive extracellular water exchange that delivers biochemicals to organs of metabolism as well as the hemofilter.     

The effect of long-term calcineurin inhibitor therapy on renal function in children after liver transplantation

Calcineurin inhibitor drugs (CNI), cyclosporin and tacrolimus are potent immunosuppressants, which have improved survival after liver transplantation. We evaluated long-term renal function in children receiving calcineurin inhibitors after liver transplantation. A retrospective analysis of all children undergoing orthotopic liver transplantation (OLT) from 1989 to 1999 who survived 1-yr post-transplantation was performed. All received prednisolone and either cyclosporin and azathioprine or tacrolimus. Steroids were withdrawn at 3 months and cyclosporin/tacrolimus monotherapy was initiated 12 months post-OLT. Calculated glomerular filtration rate (cGFR) was calculated using the modified Counahan-Barratt formula and measured pretransplant, 3, 6 and 12 months post-transplant and annually thereafter. Data were analysed in a serial manner to evaluate the trend of cGFR over time selectively using the Wilcoxon signed rank test and paired t-tests as appropriate. A total of 113 patients (65 males:48 females) were followed up for more than 1 yr (maximum 5 yr). Median (range) age at transplantation was 26 months (3-177). There was a significant fall of 35% in cGFR at 3 months compared with the pretransplant value (p = 0.001). By 12 months following the reduction in immunosuppression dosage, renal function stabilized with a slight improvement in cGFR which reached 76% of the pretransplant value at 5 yr (p < 0.001). Children who were <1 yr of age at the time of OLT had better recovery of renal function than older children (p = 0.02). No association was seen with sex, the type of immunosuppression or the underlying diagnosis. Renal dysfunction is a known complication of CNI therapy. Despite an initial reduction in cGFR, which was associated with maximal immunosuppression, long-term low dose CNI therapy was not associated with continued deterioration of renal function, particularly in children who were transplanted as infants.