高频超声在新生儿锁骨骨折中的诊断价值探讨

目的研究高频超声在新生儿锁骨骨折筛查、诊断以及愈合过程中动态观察与疗效评估中的应用价值。方法利用高频超声技术对存在锁骨骨折高危因素或临床检查怀疑锁骨骨折的新生儿进行筛查,若超声显示骨皮质连续性中断、低回声区或无回声区等异常声像图,则诊断为锁骨骨折,并拍摄X线片,以X线检查作为诊断金标准,进行对比分析。间隔10d、20d后,分别进行第2次、第3次高频超声检查,对比骨痂形成与正常锁骨声像图。结果完成全程研究的106例锁骨骨折新生儿中,第1次高频超声检查阳性103例,阴性3例,敏感度97．2％,特异度100％,X线与超声检查方法阳性率比较,无统计学意义（P=0．083）。在骨折愈合过程中,骨痂声像图显示骨痂边缘骨皮质为条索状弱强回声、骨痂中间髓腔部分为无回声区或低回声区中有散在弱强回声斑点,声像强度与同期骨痂边缘骨皮质强度相似,随着愈合进展骨痂边缘骨皮质逐渐增厚,中间回声斑点逐渐增多增大,回声强度逐渐增强。结论高频超声技术诊断新生儿锁骨骨折,具有可靠、无X线损害的优点,值得在新生儿锁骨骨折筛查与愈合过程中的动态观察、疗效评估中推广应用。     

Indomethacin treatment of infantile cortical periostosis in twins

Twin girls presented with infantile cortical periostosis (Caffey's disease) at 2 and 3 weeks of age, respectively. This disorder initially involved their upper and lower limbs and resulted in fever, irritability and tenderness. X-rays showed extensive periosteal new bone formation. Multiple relapses occurred in the first year of life and during some of these relapses mandibular and clavicular involvement was noted. Prednisolone, 1 mg/kg per day, was used to treat relapses until 9 months of age. Indomethacin therapy at this age at a dose of 3 mg/kg per day allowed the cessation of prednisolone therapy and disease flares were thereafter infrequent and responsive to indomethacin.     

钛制弹性稳定性髓内针治疗儿童锁骨干骨折近期疗效观察

目的 应用闭合穿钉微创钛制弹性髓内针治疗儿童锁骨骨折,探讨此治疗方法的手术技巧、注意事项和并发症,评价治疗效果,以提高锁骨骨折治疗的安全性和稳定性.方法 钛制弹性髓内针闭合复位穿钉微创治疗锁骨骨折26例,随访时间平均10个月(6~15个月).结论 患儿均获得满意解剖复位,闭合复位25例,切开复位1例.无严重并发症发生,Constant和Murley肩关节评分平均99.4分,DASH(disability of the arm,shoulder,and hand)评分平均0.46分,优良率92.3%,患儿对功能和外观恢复满意,满意率100%.结论 钛制弹性髓内针治疗儿童锁骨骨折具有创伤小、固定可靠、功能恢复快、并发症少等优点,可作为钢板内固定和非手术治疗替代方法.

Abstract：

Objective To investigate the application of titanium elastic intramedullary nail in treating children with clavicular fractures without incision.Methods Twenty-six children with clavicular fractures were operated on by titanium elastic intramedullary nail,all of them were followed up 6 to 15 months(average of 10 months).Results Twenty-five patients were treated by closed reduction,an accessory incision had to be made above the fracture in one patient.All patients were satisfied with anatomical reduction,good functional recovery and excellent cosmetic appearance,and no severe complications happened,the excellent and good rate was 92.3%.Constant and Murley Shoulder Score was 99.4,DASH score was 0.46.Conclusions Using titanium elastic intramedullary nail to treat children with clavicular fractures has the advantages of easy operation,less invasive,reliable fixation,less complications.The patients can benefit from rapid posttraumatic pain relief,early mobilization,function improvement of shoulder and good cosmetic appearance.It is an alternative option to non-operative treatment and plate osteosynthesis in clavicular fractures in children.     

弹性髓内针固定治疗青少年锁骨中段骨折

目的探讨闭合复位、经皮钛制弹性髓内针固定治疗青少年锁骨中段骨折,并观察其临床疗效。方法作者自2008年5月至2010年3月收治青少年锁骨中段移位骨折11例,均为男性,年龄9—15岁,平均年龄11岁,按Robison分型均为ⅡB1型,术中采用闭合复位、锁骨内侧经皮钛制弹性髓内针固定。术后早期恢复自主活动。结果患儿术后平均住院3d,随访8～22个月（平均10．5个月）,均一期获得骨性愈合,愈合时间4—8周（平均5．6周）。术后4周均基本恢复正常活动及运动,肩关节功能Constant评分94～97分（平均96分）,术后3—6个月（平均4个月）去除内固定。结论闭合复位、钛制弹性髓内针固定治疗青少年锁骨中段骨折,可获得良好疗效,具有微创、可恢复锁骨长度、固定稳定、恢复患肢活动早、对日常生活影响小等优点。     

Inherited multicentric osteolysis: case report of three siblings treated with bisphosphonate

Inherited Multicentric Osteolysis (IMO) is an uncommon familial condition of idiopathic pathophysiology causing bone osteolysis and dysplasia. These patients present with common rheumatologic complaints of pain, dysfunction and disability, and are often initially misdiagnosed as a chronic rheumatic disease of childhood such as juvenile idiopathic arthritis. We report a case of three siblings diagnosed with IMO. Diagnosis was made during childhood, with each sibling having different manifestations and course of disease. One had a previous history of bilateral hip dysplasia. Two had osteolysis of the foot, distal tibia and femur (lower limb bones), whilst one had osteolysis of the rib and unusual clavicular fractures. Unusually, all siblings appear to experience decreased pain sensation compared to norms. All siblings were treated with bisphosphonates and experienced a rapid improvement in pain symptoms, decreased analgesic requirements. Two had bone mineral density testing performed and both had increases post-bisphosphonate. In all three, there was subjective evidence of stabilisation of bone disease. Testing for matrix metalloproteinase-2 (MMP2) gene was negative.     

A single-institution experience with treatment of severe acute chest syndrome: lack of rebound pain with dexamethasone plus transfusion therapy

The use of corticosteroid therapy for the treatment of acute chest syndrome (ACS) in patients with sickle cell disease has been infrequently used owing to concerns for rebound pain. Here, we report a cohort of patients<21 years of age with sickle cell disease treated between January 2001 and June 2006 for severe ACS with both corticosteroids and transfusion therapy. We reviewed 53 episodes of severe ACS with an average hospital duration of 4.9 days. Only 1 patient out of 6 who were transferred to the intensive care unit required intubation. None of the ACS episodes resulted in death and none of the 4 readmissions after discharge were due to pain. There was no acute toxicity related to either corticosteroid or transfusion therapy.     

Palpable spongy mass over the clavicle, an underutilized sign of clavicular fracture in the newborn

This prospective study emphasizes the importance of an early physical finding of neonatal clavicular fracture, termed "the palpable spongy mass sign." Of the 1,661 term neonates examined at our tertiary center over a 20-month period, 24 had clinical signs of a clavicular fracture. In 22 of the 24 neonates, the fractures were documented by positive radiographs or callus formation. None of the fractures was recognized because of an asymmetric Moro reflex, visible swelling, or bruising. The palpable spongy mass was present in 18 of the 22 fractures (82%), crepitus in 10 (45%), angulation deformity in two (9%), and localized tenderness in one (5%). Any combination of crepitus, deformity, and localized tenderness was detected in 11 of the 22 (50%) fractures. When the palpable spongy mass sign was added to these three signs, all but one fracture was clinically detected (95%), emphasizing the importance of using all physical findings. We conclude that "the clavicular spongy mass sign" is highly sensitive and predictive of neonatal clavicular fractures.     

The use of Lactobacillus GG in irritable bowel syndrome in children: a double-blind randomized control trial

OBJECTIVE: To determine whether oral administration of the probiotic Lactobacillus GG under randomized, double-blinded, placebo-controlled conditions would improve symptoms of irritable bowel syndrome (IBS) in children. STUDY DESIGN: Fifty children fulfilling the Rome II criteria for IBS were given Lactobacillus GG or placebo for 6 weeks. Response to therapy was recorded and collected on a weekly basis using the Gastrointestinal Symptom Rating Scale (GSRS). RESULTS: Lactobacillus GG was not superior to placebo in relieving abdominal pain (40.0% response rate in the placebo group vs 44.0% in the Lactobacillus GG group; P=.774). There was no difference in the other gastrointestinal symptoms, except for a lower incidence of perceived abdominal distention (P=.02 favoring Lactobacillus GG). CONCLUSIONS: Lactobacillus GG was not superior to placebo in the treatment of abdominal pain in children with IBS but may help relieve such symptoms as perceived abdominal distention.     

Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis

OBJECTIVE: To assess the relationship between pancreatic enzyme therapy (PET) and the clinical outcomes of growth, abdominal pain, constipation, gassiness, and number of stools in cystic fibrosis (CF). STUDY DESIGN: Patients (n = 1215) >4 weeks of age from 33 Cystic Fibrosis Foundation accredited sites who had a sweat chloride >60 mmol/L or two CF-causing mutations were enrolled using a proportionate sampling strategy in a nonblinded study. Patients submitted a stool sample and completed a questionnaire. The study coordinator also completed a questionnaire for each patient. Enzyme dosing and growth, abdominal pain, gassiness, constipation, and number of stools were compared. RESULTS: Of the 1215 enrolled patients, 1131 (93.1%) were prescribed PET. Only 14.9% had pancreatic function assessed before enrolling in this study. Stool elastase-1 analysis identified 1074 (89%) patients as pancreatic insufficient (PI). There was no association between PET and the outcomes: growth, abdominal pain, gassiness, constipation, and number of stools. CONCLUSION: PET dose is not correlated with growth or gastrointestinal symptoms. More sensitive outcome measures of the effectiveness of PET in patients with CF are needed to guide treatment of PI.     

The natural history of medium-chain acyl CoA dehydrogenase deficiency in the Netherlands: clinical presentation and outcome

OBJECTIVES: To describe the clinical presentation and long-term follow-up of a large cohort of patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. STUDY DESIGN: A nationwide, retrospective analysis of clinical presentation and follow-up in 155 Dutch patients with MCAD deficiency. RESULTS: Most patients presented between 3 months and 5.1 years of age; 13% had symptoms as neonates not exclusively related to breast-feeding. An acute presentation before the diagnosis was made resulted in a mortality of 22% (25/114), whereas 21% (19/89) developed disabilities after the diagnosis. On follow-up, a total of 44 patients reported fatigue (35%; 28/80), muscle pain (31%; 25/80), and/or reduced exercise tolerance (39%; 31/80). Cardiac evaluation in 11 adult patients revealed no abnormalities in cardiac function explaining these complaints. Children with MCAD deficiency readily become overweight. CONCLUSIONS: Mortality and morbidity were high in undiagnosed children with MCAD deficiency; establishment of the diagnosis significantly improves outcome. Strikingly, after the diagnosis and initiation of treatment, overweight and chronic complaints (fatigue, muscle pain, and reduced exercise tolerance) were prominent.     

Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial

OBJECTIVE: To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Sch?nlein purpura (HSP) in a placebo-controlled trial. STUDY DESIGN: A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis. RESULTS: Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024). CONCLUSIONS: The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.     

Erb’s palsy – Who is to blame and what will happen?

Erb’s palsy is initially frightening. The infant’s arm hangs limply from the shoulder with flexion of the wrist and fingers due to weakness of muscles innervated by cervical roots C5 and C6. Risk factors are macrosomia (large baby) and shoulder dystocia. However, Erb’s palsy may occur following cesarian section. The experience of the delivering physician may not influence the risk of Erb’s palsy (0.9 to 2.6 per 1000 live births). Differential diagnosis includes clavicular fracture, osteomyelitis and septic arthritis. Fortunately, the rate of complete recovery is 80% to 96%, especially if improvement begins in the first two weeks. Recommended treatment includes early immobilization followed by passive and active range of motion exercises (although there is no proof that any intervention is effective). For the few infants with no recovery by three to five months, surgical exploration of the brachial plexus may improve the outcome. Three infants with Erb’s palsy who illustrate variations in the evolution of this disorder are presented.     

Failure to eradicate HIV despite fully successful HAART initiated in the first days of life

Highly active antiretroviral therapy (HAART) started shortly after birth resulted in reversion of human immunodeficiency virus (HIV) plasma viremia, proviral DNA in PBMC, viral culture, and serum HIV antibodies to negative. Discontinuation of HAART 2 years after apparent HIV eradication, however, was followed by virus replication, CD4 decline, and destruction of HIV-specific lymphocytes, epitomizing the impossibility of HIV eradication.     

Abdominal migraine in children with neurofibromatosis type 1: a case series and review of gastrointestinal involvement in NF1

BACKGROUND: Symptomatic involvement of the gastrointestinal tract in children with neurofibromatosis type 1 (NF1) is rare. Most reported complications in adults are caused by the presence of neurofibromas in the stomach, small bowel, or mesentery. In contrast, abdominal pain in children with NF1 may be the result of nonanatomic causes, such as migraine. There are no previous reports of an association between abdominal migraine and NF1. METHODS: Children with abdominal migraine were identified from a group of children with NF1, all of whom had been followed up for a minimum of 3 years. Medical records of cases were reviewed independently by two authors. MEDLINE was searched via PubMed for all reports of children with NF1 and any associated gastrointestinal involvement. RESULTS: Six children with NF1 and intermittent, episodic, severe abdominal pain are reported. Investigations for obstructive or inflammatory causes of abdominal pain were negative. All patients had previously been diagnosed with migraine headaches by a neurologist. In five of the six patients, propranolol (10-15 mg three times daily) resulted in relief of their abdominal pain within days of starting therapy. Our review identified 24 children in the medical literature with gastrointestinal complications of NF1, mostly secondary to visceral neurofibromas. In almost all of these cases, clinical examination and simple radiologic investigations led to the definitive diagnosis. There were no reports of abdominal migraine complicating NF1. CONCLUSIONS: Abdominal pain secondary to migraine is an unrecognized cause of abdominal pain in children with NF1 and may be more common than anatomic causes of abdominal pain in children with NF1. In children with NF1 and severe recurrent abdominal pain in whom an evaluation for anatomic lesions is negative, a trial of migraine therapy may be indicated.     

Post-thrombotic syndrome is uncommon in childhood cancer survivors

Deep vein thrombosis occurs in up to 50% of children with tunneled central venous catheters (CVCs). CVC-related deep vein thrombosis involving the upper extremity is usually asymptomatic but can result in post-thrombotic syndrome (swelling, pain, skin changes, and functional impairment). In a cohort of childhood cancer survivors evaluated clinically a mean of 7.5 +/- 2.8 years after completion of therapy who previously had CVCs in place for a median 15.5 months, none of 50 patients (95% CI = 0% to 6%) had these features diagnostic of post-thrombotic syndrome. Five patients had arm circumference 3% to 5% greater ipsilateral to the prior CVC.     

Crohn's disease penetrating into the spinal canal

An 11-year-old boy who had suffered from Crohn's disease of the large bowel for 4 years developed high fever, severe back pain and flexion-contracture of the right hip joint with inability to walk. After several weeks radicular irritation also became apparent. CT-scan demonstrated an abscess of the right psoas muscle that had also infiltrated the spinal canal, leading to a spinal extradural abscess extending from L2-S4. Therapy comprised surgical drainage of the psoas abscess and conservative therapy (mainly steroids and hypercaloric diet) and resulted in complete cure of the psoas and the spinal abscess. Spinal infiltration is a rare and serious complication of Crohn's disease and must be considered in every case of significant back pain with or without obvious neurological signs.     

Intraarterial management of giant-cell tumors of the spine in children

Transcatheter arterial management of giant cell tumors of the spine was performed in two female patients aged 12 and 15 who had failed to respond to conventional therapy. Response was determined clinically by alleviation of pain and radiographically by regression and/or calcification of the tumor. Minor side effects included transient nausea, elevated body temperature, and pain.     

Crohn's Disease Penetrating into the Spinal Canal

ABSTRACT. An 11-year-old boy who had suffered from Crohn's disease of the large bowel for 4 years developed high fever, severe back pain and flexion-contracture of the right hip joint with inability to walk. After several weeks radicular irritation also became apparent. CT-scan demonstrated an abscess of the right psoas muscle that had also infiltrated the spinal canal, leading to a spinal extradural abscess extending from L₂-S₄. Therapy comprised surgical drainage of the psoas abscess and conservative therapy (mainly steroids and hypercaloric diet) and resulted in complete cure of the psoas and the spinal abscess. Spinal infiltration is a rare and serious complication of Crohn's disease and must be considered in every case of significant back pain with or without obvious neurological signs.     

Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis

Ten children with cystic fibrosis, aged 3.5 to 12 years, whose weights were lower than 90% of the expected weight for height, received high-calorie elemental enteral alimentation for four weeks. Clinical, anthropometric, and biochemical evaluations as well as blood gas analyses and chest radiograph scoring were performed in all. Pulmonary function tests were performed in the five older children, and progressive exercise tests in three. These evaluations were done before, immediately after, and two months after termination of therapy. Nutritional therapy resulted in an increase of caloric intake and in dramatic weight gain, which persisted only for a short time and was mainly related to adipose tissue accretion. No functional improvement accompanied the amelioration in nutritional status. This short-term nutritional therapy in malnourished children with cystic fibrosis was effective in increasing relative weight and energy stores, but there was no evidence of any long-term functional benefit.     

The importance of gut motility in the metabolic control of propionic acidemia

We hypothesized that gut motility likely plays a critical role in the metabolic stability in propionic acidemia (PA). Therefore, 4 known patients with PA (aged 47 months to 185 months) were prospectively studied over 7 days in the Clinical Research Center at Children's Hospital, Boston. Determinations of ammonia, bicarbonate, and amino acids in blood; organic acids and propionylglycine in urine; and a lactulose breath test were conducted under two study conditions: on regular therapy (for 4 days) and on regular therapy plus Senekot (Purdue Frederick Company, Norwalk, Conn), an intestinal motility agent (for 3 days). The total gastrointestinal transit time was calculated using 20 nonabsorbable, inert, radio-opaque markers. The addition of an intestinal motility agent resulted in a significant decrease in blood ammonia, urinary excretion of propionylglycine, and a rise in the ratio of free to total carnitine over baseline. We concluded that enhancement of gut motility can improve metabolic stability in patients with PA.