Type 1 fiber size disproportion: morphometric data from 37 children with myopathic, neuropathic, or idiopathic hypotonia

We reviewed clinical data and quantitative muscle biopsy data from infants with hypotonia and developmental delay who had relatively small type 1 muscle fibers (fiber size disproportion; FSD). Study material consisted of 49 thigh muscle specimens from 37 patients, 1 week to 11.5 years old, who had FSD diagnosed between 1971 and 1985. Beyond 2 months of age, FSD was greater than 12% in at least one biopsy from all but 2 patients. In sequential specimens obtained from 11 patients, we observed that FSD may resolve, persist unchanged, or intensify with age. In 6 patients, hypotonia regressed, and development tended to normalize during 1-4 years of observation; FSD resolved in 4 cases, but numerical predominance of type 1 fibers persisted in 3 of them. In a small group designated congenital myopathy with FSD only, the disproportion in fiber diameter persisted unchanged or intensified with time and was more severe after the age of 6 months than in the other subgroups. We classified FSD as definitely or probably myopathic (15 cases), definitely or probably neuropathic (17 cases), and benign maturation delay (5 cases). Six of 17 neuropathic cases had a history of perinatal asphyxia. In most cases, type 1 fibers were absolutely small and type 2 fibers were hypertrophied, independent of classification. FSD is a manifestation of muscle maturation delay of variable etiology. The prevalence of nervous system disorders in our series suggests that neuronal dysfunction plays an important role in pathogenesis. We were unable to define morphometric criteria that consistently distinguished congenital myopathy with FSD from definable neuropathic FSD or from benign maturation delay.     

Effect of abdominal irradiation on growth in boys treated for a Wilms' tumor

To study the effect of abdominal irradiation on spinal growth in childhood we have measured final height, sitting height, and leg length in 30 male survivors of a Wilms' tumor. Twenty-one patients received whole abdominal irradiation by either megavoltage therapy (MV: n = 11) or orthovoltage therapy (OV: n = 10); the remainder received flank irradiation. To examine the effect of the adolescent growth spurt on the irradiated spine we have followed prospectively seven patients who received whole abdominal irradiation and nine patients who received flank irradiation through puberty. Compared to a normal population there is a modest reduction in median final standing height SDS (H.SDS: -1.15) accompanied by a marked reduction in median final sitting height SDS (S.HT SDS: -2.41) with no apparent effect on median subischial leg length SDS (SILL.SDS: 0.04). This reduction in spinal growth is reflected by a strongly positive disproportion score (DPS; [SILL SDS-S.HT SDS] + 2.81). The incidence of scoliosis after abdominal irradiation has been low (10%). During puberty there is a significant fall in median sitting height SDS after both whole abdominal (median fall: -0.9, P = 0.02) and flank irradiation (median fall: -1.85, P = 0.01), and this is reflected in a significant increase in disproportion (DPS: whole abdominal; median rise +1.4, P = 0.02: flank, median rise +1.34, P = 0.01). After MV irradiation there is a significant correlation between the degree of disproportion and the age at treatment (P less than 0.0005). The younger the patient is at treatment the more severe is the restriction on spinal growth and the shorter and more disproportionate they become as an adult. The estimated eventual loss in potential height from abdominal irradiation at the age of one is 10 cm and at five years is 7 cm.     

Uveitis in juvenile idiopathic arthritis

OBJECTIVE: To evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. PATIENTS AND METHODS: We retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months. RESULTS: Forty patients were male (55.5%) and 36 were Caucasoid (50%). The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years) and the mean age at the beginning of the study was 10.4 years (1 to 19 years). According to the type of disease at onset, 32 were pauciarticular (44.4%) (17 boys and 15 girls), 30 were polyarticular (41.6%) (17 boys and 13 girls) and 10 were systemic (14%) (6 boys and 4 girls). We observed chronic anterior uveitis in five patients (6.5%) (mean age = 11.4 years). Among them, four (80%) had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies) and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor). In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60%) with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67). Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The frequency of antinuclear antibodies was statistically higher in the patients with uveitis (P< 0.05). CONCLUSION: Although the incidence of uveitis in our study was lower than that reported in the literature, the frequency of uveitis was higher in females, in those with pauciarticular juvenile idiopathic arthritis and in patients with positive antinuclear antibodies.     

25例慢性肌酸磷酸肌酶升高患儿的临床分析

目的明确慢性血清肌酸磷酸肌酶(CK)升高的儿童患者的病因诊断。方法对2005-01—2007-03,北京协和医院遗传门诊入院的CK持续升高超过3个月患儿的临床资料进行回顾性分析。结果16例获得诊断,确诊率64%,诊断分别为:7例dystrophin相关肌营养不良症,1例携带者;2例Pompe病,1例糖原累积症;1例线粒体肌肉病,1例戊二酸尿症,2例炎症性肌肉病,1例脊肌萎缩症。其余9例未能明确诊断。结论血清肌酶持续增高在儿童病人多提示神经肌肉疾病或累及肌肉的代谢性疾病,病因复杂,除常规生化检查外,肌电图、肌活检、酶生化测定、基因检测等多种检查的综合应用有助于提高诊断率。     

Pediatric occipitocervical arthrodesis. A review of current options and early evaluation of rigid internal fixation techniques

OBJECT: Few reports exist on the options and effectiveness of craniocervical stabilization in the pediatric population compared with the adult literature. Traditional options in children include onlay grafting and semi-rigid occipitocervical wiring. Recently, reports on the use of rigid internal fixation devices such as occipitocervical plates and contoured loops have provided excellent results in adults, and their use has often obviated the need for external orthosis. The purpose of this article is to report our experience with both traditional and newer rigid internal fixation methods for occipitocervical fusion in children. METHODS: During the past 4.5 years, 14 children (ages 4 months to 16 years) have undergone occipitocervical fusion. Indications for fusion included trauma (n = 4), congenital instability/stenosis (n = 6), postinfectious instability (n = 1) and basilar invagination (n = 3). Techniques using onlay grafting (n = 3) as well as rigid internal fixation using plates (n = 1) and contoured craniocervical titanium loops (n = 10) were used. Postoperative orthosis included halo vests (n = 7), minerva jackets (n = 3), sterno-occipital mandibular immobilizer (n = 1), and a cervical collar (n = 3). Long-term follow-up (range 13-58 months) was available for 13 of the 14 children. CONCLUSIONS: While each occipitocervical fusion in pediatric patients requires a customized treatment plan, we believe children older than 12 months of age should be considered candidates for rigid internal fixation methods. The rigidity afforded by this method may eliminate the need for rigid external orthotic support in selected individuals. In our experience, anatomic constraints in children less than 1 year old usually require fusion with more traditional onlay techniques. Long-term follow-up studies are still required to assess the effects of rigid internal fixation in the skeletally immature spine.     

Sclerotherapy in aneurysmal bone cysts in children: a review of 17 cases

OBJECTIVE: To determine the efficacy of percutaneous sclerotherapy in the treatment of aneurysmal bone cysts. MATERIALS AND METHODS: Seventeen patients (7 girls, 10 boys) with aneurysmal bone cysts were treated by the percutaneous approach with Ethibloc ( n=14) and histoacryl glue ( n=3) in our institution between January 1994 and June 2000. The cysts were located in the extremities ( n=6), pelvis ( n=2), spine ( n=2), mandible ( n=5), rib ( n=1) and sphenoid bone ( n=1). Percutaneous sclerotherapy was performed with fluoroscopic and/or computed tomographic guidance under general anesthesia. Clinical and imaging follow-up lasted from 24 months to 9 years and 6 months (mean: 57.3 months). The results were quantified as: excellent (residual cyst less than 20% of the initial involvement), satisfactory (residual cyst 30-50%), unsatisfactory (residual cyst more than 50%). RESULTS: The age of the patients ranged from 4 years and 6 months to 15 years and 8 months (mean: 11 years and 2 months). In nine patients, the therapeutic procedure was repeated 2-5 times. Excellent regression was observed in 16 (94%), satisfactory results in 1 (6%). There was no failure (unsatisfactory result or no response to treatment) in this reported series. The complications were minor and included: local inflammatory reaction ( n=2), small blister ( n=1), and leakage ( n=1). Relief of symptoms was achieved in all patients. No recurrence was noted during follow-up. CONCLUSION: Percutaneous sclerotherapy of aneurysmal bone cysts with Ethibloc is safe and effective. It is an important alternative to surgery, especially when surgery is technically impossible or not recommended in high-risk patients.     

Sleep-related breathing disorder in Duchenne muscular dystrophy: disease spectrum in the paediatric population

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease with death usually occurring because of respiratory failure. Signs of early respiratory insufficiency are usually first detectable in sleep. OBJECTIVE: To study the presentation of sleep-related breathing disorder (SRBD) in patients with DMD. METHOD: A retrospective review of patients with DMD attending a tertiary paediatric sleep disorder clinic over a 5-year period. Symptoms, lung function and polysomnographic indices were reviewed. RESULTS: A total of 34 patients with DMD were referred for respiratory assessment (1-15 years). Twenty-two (64%) reported sleep-related symptomatology. Forced vital capacity (FVC) was between 12 and 107% predicted (n = 29). Thirty-two progressed to have polysomnography of which 15 were normal studies (median age: 10 years) and 10 (31%) were diagnostic of obstructive sleep apnoea (OSA) (median age: 8 years). A total of 11 patients (32%) showed hypoventilation (median age: 13 years) during the 5-year period and non-invasive ventilation (NIV) was offered to them. The median FVC of this group was 27% predicted. There was a significant improvement in the apnoea/hypopnoea index (AHI) (mean difference = 11.31, 95% CI = 5.91-16.70, P = 0.001) following the institution of NIV. CONCLUSIONS: The prevalence of SRBD in DMD is significant. There is a bimodal presentation of SRBD, with OSA found in the first decade and hypoventilation more commonly seen at the beginning of the second decade. Polysomnography is recommended in children with symptoms of OSA, or at the stage of becoming wheelchair-bound. In patients with the early stages of respiratory failure, assessment with polysomnography-identified sleep hypoventilation and assisted in initiating NIV.     

Effect of abdominal irradiation on growth in boys treated for a wilms′ tumor

To study the effect of abdominal irradiation on spinal growth in childhood we have measured final height, sitting height, and leg length in 30 male survivors of a Wilms′ tumor. Twenty-one patients received whole abdominal irradiation by either megavoltage therapy (MV: n=11) or orthovoltage therapy (OV: n=10); the remainder received flank irradiation. To examine the effect of the adolescent growth spurt on the irradiated spine we have followed prospectively seven patients who received whole abdominal irradiation and nine patients who received flank irradiation through puberty. Compared to a normal population there is a modest reduction in median final standing height SDS (H.SDS: ?1.15) accompanied by a marked reduction in median final sitting height SDS (S.HT SDS: ?2.41) with no apparent effect on median subischial leg length SDS (SILL.SDS: 0.04). This reduction in spinal growth is reflected by a strongly positive disproportion score (DPS; [SILL SDS — S.HT SDS] +2.81). The incidence of scoliosis after abdominal irradiation has been low (10%). During puberty there is a significant fall in median sitting height SDS after both whole abdominal (median fall: ?0.9, P=0.02) and flank irradiation (median fall: ?1.85, P=0.01), and this is reflected in a significant increase in disproportion (DPS: whole abdominal; median rise +1.4, P=0.02: flank, median rise +134, P=0.01). After MV irradiation there is a significant correlation between the degree of disproportion and the age at treatment (P <0.0005). The younger the patient is at treatment the more severe is the restriction on spinal growth and the shorter and more disproportionate they become as an adult. The estimated eventual loss in potential height from abdominal irradiation at the age of one is 10 cm and at five years is 7 cm.     

Respiratory syncytial virus infections in children admitted to the intensive care unit

During the winter of 1986-1987, 64 children with respiratory syncytial virus (RSV) infection were admitted to our hospital. The diagnosis was made by direct immunofluorescent antibody technique. Twenty-three children (36%) needed intensive care treatment. Nearly 11 (52%) had a preexisting disease state, identified as a risk factor i.e., prematurity (n = 8), bronchopulmonary dysplasia (n = 2), congenital heart disease (n = 1). Twelve patients (50%) were intubated and ventilated. Conditions for intubation and ventilation were repetitive apnea with or without bradycardia (n = 4), clinical deterioration (n = 3) or hypercarbia (n = 5). Seventy-five percent of the patients who needed intensive care management were under three months of age compared to 34% of the children who were admitted to the clinical ward. The mean age for ventilated patients was 7.9 weeks. The mean duration of ventilation was 5.5 days. Volume controlled ventilation was initially applied to all patients. Pulmonary complications (atelectasis, pneumonia, pneumothorax or adult respiratory distress syndrome) were present in 15 (65%) IC patients. Nine (39%) of them also had symptoms of inappropriate antidiuretic hormone secretion (IADHS). Only two patients had symptoms of IADHS and two others had convulsions. Three children (5%) died as a result of respiratory insufficiency. Two of these infants belonged to the risk group.     

Reduced bone mineral density at diagnosis and bone mineral recovery during treatment in children with Graves' disease

OBJECTIVES: In children with Graves' disease, the prevalence of osteopenia is unknown, and the possible restoration of bone mass by antithyroid treatment has not been evaluated. The aim of this study was to prospectively evaluate the bone mineral density (BMD) and bone metabolism at diagnosis and after 1 and 2 years of medical treatment. Twenty-six children (19 girls and 7 boys) aged 11 +/- 3.4 years (range 3.4 to 15.3 years) were studied. STUDY DESIGN: BMD of the lumbar spine and proximal femur were measured by dual-energy x-ray absorptiometry. Values were compared with those of healthy children of similar age, sex, and pubertal stage. RESULTS: At diagnosis the mean BMD (standard deviation score [SDS]) was significantly reduced in both sites (P <.001) with a preferential loss of cortical bone (femoral BMD = -1.7 +/- 1.0 SDS) rather than trabecular bone (lumbar spine BMD = -0.8 +/- 1.1 SDS) (P =.003). Severe osteopenia (below -2 SDS) was found in 11 (42%) of 26 patients. Osteocalcin was significantly higher than in the control group (P <.0001), but other bone metabolism markers were normal. During treatment (n = 19) a significant gain in femoral (F = 14.7; P =.001) and lumbar spine (F = 5; P =.02) BMD (SDS) was observed, and none of the patients showed osteopenia. The annual percent change in the BMD values at the femoral (+23% +/- 11% and +6% +/- 4%, respectively, during the first and second years) and lumbar spine (+19% +/- 9% and +6% +/- 5%, respectively, during the first and second years) sites was greater during the first year than during the second year of treatment (P <.02 for femoral, P <.04 for lumbar spine). No significant age difference in BMD SD score or in BMD percent change values was observed. Osteocalcin returned rapidly to normal values, and all other bone metabolism markers remained in the normal range. CONCLUSIONS: In conclusion, severe osteopenia was observed at diagnosis in children with Graves' disease but was rapidly corrected after 1 and 2 years of treatment. Initial reduced bone mass with high bone turnover caused by hyperthyroidism was corrected after 1 year of euthyroid conditions.     

Sigmoidoscopy in children with chronic lower gastrointestinal bleeding

Objective: This study was done to assess the role of rigid sigmoidoscopy in diagnosis and prognosis of children with chronic and minor lower gastrointestinal bleeding. Methods: Retrospective review of the clinical notes of children under 15 years of age with minor and chronic lower gastrointestinal bleeding who had rigid sigmoidoscopy and biopsy between October 1998 and April 2002 at Liaquat Medical College Hospital, Hyderabad, Pakistan. Demographic data, clinical presentation, morbidity, sigmoidoscopic and histopathological findings were analysed to determine the role of rigid sigmoidoscopy in the management of rectal bleeding in children. Results: A total of 229 sigmoidoscopic examinations were carried out in 207 children with a mean age of 6 years. Seventy-seven per cent (160) of children were symptomatic for a year or more. Causes of bleeding were juvenile colorectal polyps (155 cases; 75%), non-specific proctitis (38 cases; 18%), solitary rectal ulcer (seven cases; 3.5%), lymphoid nodular hyperplasia (six cases, 3%) and foreign body (betel nuts) impaction (one case; 0.5%). Polyps were mostly in the rectosigmoid region and solitary in 136 (88%) children. All polyps were removed by rigid sigmoidoscopy. Histological examination of 137 polyps revealed juvenile type in 136 (99%) cases and low-grade dysplastic changes in one patient. Non-specific proctitis (n = 38) was confirmed histologically in 92% (n = 35) of cases and it was self-limiting in 86% of cases. Other findings include solitary rectal ulcer in seven, lymphoid nodular hyperplasia in six and telangiectasic (pyogenic) granuloma in one patient. Conclusion: Rigid sigmoidoscopy was useful in diagnosis, treatment and prognostic evaluation of children with chronic and minor lower gastrointestinal bleeding. Final diagnosis was confirmed by histopathology.     

Long term outcome of treatment of end stage renal failure.

The most common causes of end stage renal failure in 46 children (mean age 11 years, range 4-14) treated between January 1972 and June 1977 were: reflux nephropathy (n = 12), cystinosis (n = 7), focal and segmental glomerulosclerosis (n = 6), and Schönlein-Henoch disease (n = 5). The quality of life, degree of renal function, and height attainment of the 31 survivors were assessed in June 1985, when their mean age was 22 years (range 14-27), using hospital records and a questionnaire designed to highlight social and psychological problems. Twenty six patients had a functioning transplanted kidney. Average growth during treatment for all survivors was normal, but most were disappointed with their ''final height''. Though five patients had some form of disabling bone disease, all 31 could walk and 27 could run. Sixteen (67%) were in full or part time employment and nine were living independently. A group of 32 patients with juvenile onset diabetes treated at this hospital for at least five years were also asked to complete the questionnaire and of these, 17 responded. On average, their data could usefully be compared with those of cases of end stage renal failure. More of the diabetics had jobs, but most sexually mature patients with renal disease were concerned about their physical appearance and had not achieved any stable long term sexual relationships. We suggest that a poor body image resulting in low self esteem may be responsible for the deficiency and believe that further study in this group is warranted.     

Congenital diaphragmatic defects that present late.

Twenty two patients (age range: 1 month to 11 years) were treated for congenital diaphragmatic defects (excluding hiatus hernia) in the six year period 1983-8. Presenting features were failure to thrive (n = 7), abdominal pain and vomiting (n = 4), chronic respiratory symptoms (n = 3), and inability to wean from ventilatory support (n = 3). The defect was an incidental finding in five patients. Operative repair was performed with no mortality or serious postoperative morbidity. Dramatic improvement occurred in 15 of the 17 symptomatic patients. Awareness of the differential diagnosis should avoid delay in diagnosis or inappropriate treatment. Surgical correction is strongly recommended in all cases.     

Clinical features of hospitalised children with 2009 H1N1 influenza virus infection

Clinical features and outcome of 2009 H1N1 influenza virus in the paediatric setting is ill-defined. The epidemiologic and clinical features of children with confirmed H1N1 influenza virus infection admitted to an Italian tertiary paediatric hospital from August through December 2009 were evaluated. A total of 63 children (mean age 4.3 years) were studied; of these, 29 (46%) had chronic underlying diseases. The most frequent symptoms and signs at admission were fever (97%), cough (60%) and respiratory disturbances (24%). Forty patients (63.5%) had H1N1-related complications: 32 (51%) pulmonary diseases, three (5%) neurological disorders, such as acute encephalitis or acute disseminated encephalomyelitis, and two (3%) haematological alterations. Three patients were admitted to the Intensive Care Unit. Most children (81%) were treated with oseltamivir: one developed rash during treatment; no other adverse events were noticed. All children survived without sequelae. In conclusions, 2009 H1N1 influenza virus infection in children is associated with a wide spectrum of clinical manifestations. Neurological disorders are not exceptional complications. Oseltamivir therapy seems safe also in infants.     

Home mechanical ventilation in children: Retrospective survey of a pediatric population

BACKGROUND: Home care support is beneficial for children needing mechanical ventilation, when clinically stable. METHODS: A retrospective analysis was carried out of the long-term home ventilation management of a pediatric population with chronic respiratory failure composed of 20 ventilator-dependent children categorized according to age, diagnosis and ventilation support. Age groups consisted of 10% under 1 year, 30% between 2 and 5 years, 30% between 6 and 12 years, and 30% older than 12 years. Diagnostic categories included myopathic disorder, n = 5; congenital central hypoventilation syndrome, n = 6; chest wall disorder, n = 5; cystic fibrosis, n = 1; pulmonary hypertension, n = 1; and diaphragmatic paralysis, n = 2. RESULTS: Sixty-five percent were ventilated using non-invasive mode (NIMV): eight with nasal mask, five with full-face mask, and two children in NIMV also used negative pressure mode; 35% were ventilated using tracheostomy, one of them also used a diaphragmatic pacer. Seventy percent needed nocturnal ventilatory support, (20% 12-18 h, 10% full-day). A total of 18 children were included in the home care and follow-up program. Two children died: one because of worsening of his chronic disease and one because of septic shock. CONCLUSION: Although home care ventilation is not yet widely diffused, it represents a valid alternative to long hospitalization for children with stable chronic respiratory failure.     

Reduced lung function both before bronchiolitis and at 11 years.

BACKGROUND AND AIMS: We have previously shown an association between reduced premorbid lung function (V'maxFRC) and bronchiolitis. We hypothesised that individuals with bronchiolitis will go on to have reduced lung function and increased respiratory symptoms in childhood. METHODS: V'maxFRC was measured at 1 month of age; individuals with bronchiolitis were prospectively identified. Annual symptom questionnaires were completed from 3 to 6 years. At 11 years of age, children underwent an assessment including questionnaire, lung function, airway response to histamine (AR), and skin prick testing. RESULTS: Eighteen individuals with bronchiolitis were ascertained from 253 cohort members. Children with bronchiolitis had increased viral induced wheeze at 3 (OR 5.8, 95% CI 1.4 to 25.2; n = 103) and 5 years (OR 5.3, 95% CI 1.1 to 25.5; n = 101). At 11 years of age, 194 children were assessed including 16 with past bronchiolitis. These 16 individuals had reduced mean z scores for % V'maxFRC compared with other children (-0.56 and 0.06 respectively) and mean z scores for % FEF(25-75) at 11 years (-0.53 and 0.06 respectively). At 11 years, FEV(1), FVC PEF, AR, atopy, wheeze, and diagnosed asthma were not different between groups. CONCLUSIONS: Reduced lung function is present before and after bronchiolitis; the level of reduction is comparable. The mechanism for wheeze and reduced lung function after bronchiolitis appears to be related to premorbid lung function and not bronchiolitis per se.     

Reduced lung function both before bronchiolitis and at 11 years

Background and Aims: We have previously shown an association between reduced premorbid lung function (V‘maxFRC) and bronchiolitis. We hypothesised that individuals with bronchiolitis will go on to have reduced lung function and increased respiratory symptoms in childhood. Methods: V‘maxFRC was measured at 1 month of age; individuals with bronchiolitis were prospectively identified. Annual symptom questionnaires were completed from 3 to 6 years. At 11 years of age, children underwent an assessment including questionnaire, lung function, airway response to histamine (AR), and skin prick testing. Results: Eighteen individuals with bronchiolitis were ascertained from 253 cohort members. Children with bronchiolitis had increased viral induced wheeze at 3 (OR 5.8, 95% CI 1.4 to 25.2; n = 103) and 5 years (OR 5.3, 95% CI 1.1 to 25.5; n = 101). At 11 years of age, 194 children were assessed including 16 with past bronchiolitis. These 16 individuals had reduced mean z scores for % V‘maxFRC compared with other children (-0.56 and 0.06 respectively) and mean z scores for % FEF_25–75 at 11 years (-0.53 and 0.06 respectively). At 11 years, FEV₁, FVC PEF, AR, atopy, wheeze, and diagnosed asthma were not different between groups. Conclusions: Reduced lung function is present before and after bronchiolitis; the level of reduction is comparable. The mechanism for wheeze and reduced lung function after bronchiolitis appears to be related to premorbid lung function and not bronchiolitis per se.     

儿童脊柱肿瘤

目的 探讨儿童脊柱肿瘤的临床特点、诊断、治疗方法及预后。方法回顾性分析我院1991年1月-2003年10月治疗的16岁以下脊柱肿瘤及瘤样病变44例，男27例，女17例；平均9．6岁。良性肿瘤（包括瘤样病变）38例，恶性肿瘤3例，转移瘤3例。疼痛39例，肿块9例，侧弯或斜颈8例，不同程度神经损害14例。18例初诊不确定或与最后诊断不符。发病至确诊时间平均8．5个月。手术治疗17例，化疗10例，放疗8例，手术与化疗1例，手术与放疗2例，放弃治疗3例，观察3例。结果围手术并发症4例。35例获平均5．5年随访。良性肿瘤及瘤样病变患儿手术或放疗或化疗后疗效较满意，所有患儿的局部疼痛消失，除1例外，所有患儿神经功能均有改善，3例病变复发再次手术。4例出现后弯畸形。6例恶性肿瘤和转移瘤患儿全部死亡。2例嗜酸性肉芽肿患儿症状消失，病变得到控制。结论对于儿童脊柱肿瘤，根据肿瘤的性质选择恰当的治疗方法，良性肿瘤与肿瘤样病变预后良好，恶性肿瘤及转移瘤治疗效果欠佳。     

儿童颈椎外伤的诊断及治疗

目的 探讨儿童颈椎外伤的临床特点、诊断、治疗方法及难点和要点.方法 回顾性分析我院1995年1月至2005年12月期间治疗的儿童颈椎外伤病例的临床资料,同时检索分析国内外关于儿童颈椎外伤的文献.结果 11例患儿纳入本研究,其中男8例,女3例;平均年龄(12.1±4.0)岁;受伤机制包括坠落伤(3例),运动伤(3例),车祸伤(2例),直接暴力伤(2例),因娱乐致伤(1例).受伤部位分布为C1-34例,C4-77例.4例合并神经系统症状,其中1例为无骨折脱位型脊髓损伤.4例接受手术治疗,7例行保守治疗.结论 儿童颈椎外伤相对少见,其在临床表现、影像学检查及治疗方法上均存在与年龄相关的特点.熟悉这些特点对儿童颈椎外伤患儿的诊断及治疗至关重要.     

累及肝门部小儿肝脏肿瘤的外科处理

目的 累及肝门的巨大和原发于肝门的小儿肝脏肿瘤往往被视为切除最为困难的肝脏肿瘤,本文探讨此类肿瘤手术术前判断的重要性和其可切除性及手术的安全性.方法 单独或同时累及第一、第二、第三肝门的巨大或原发于肝门的肝脏肿瘤27例,年龄3个月～15岁,平均5.2岁.男12例,女15例.23例手术切除,4例累及肝门的同时存在肝内多发性占位,家属放弃治疗.肿瘤主要压迫的为肝动、静脉根部、门静脉或/和肝后腔静脉;进腹允分暴露肿瘤后精细处理被肿瘤累及的肝门,然后在间歇件第一肝门阻断下切除肿瘤,切肝前根据需要预置腔静脉阻断带备用.结果 23例肝脏肿瘤均顺利切除,无手术死亡及严重并发症发牛;平均手术时间170 min,平均输血量120 ml;术后病理诊断:肝母细胞瘤10例、肝错构瘤5例、肝细胞癌4例、肝脏腺瘤2例、肝脏内皮细胞肉瘤1例、肝畸胎瘤1例.8例良性肝肿瘤术后随访11个月～9年均健康.15例恶性肿瘤中,6例于手术后5个月～2年半死于复发、转移或其他并发症,其余临床无瘤存活.结论 切除累及肝门的巨大或原发于肝门的小儿肝脏肿瘤虽具一定挑战性,但只要方法得当仍然是可行和安全的手术,提高恶性肿瘤的长期牛存率仍是今后努力的方向.