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世界核心医学期刊文摘：儿科学分册

世界核心医学期刊文摘：儿科学分册

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世界核心医学期刊文摘：儿科学分册 >2006年第A10期

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  • 摘要：The effects of illness and treatment of diabetes mellitus extend beyond medica l outcomes. We therefore evaluated health-related quality of life (HRQOL) in ch ildren (aged 8-12 years) and adolescents (aged 13-16 years) with type 1 diabet es to compare their results with healthy peers and to identify HRQOL determinant s. A total of 68 children and adolescents from a tertiary care clinic which spec ialises in the management of diabetes, completed the generic KINDL-R questionna ire. This instrument for children and adolescents has six dimensions and an addi tional module assessing condition-related HRQOL. Overall, the HRQOL was not dif ferent between patients with type 1 diabetes and healthy controls. In some areas , children and adolescents with diabetes reported a better HRQOL compared to hea lthy peers: adolescents reported better psychological wellbeing ( P < 0.05) and children higher levels of well-being in the school domain ( P < 0.05). In gener al, children reported a better HRQOL ( P < 0.05) than adolescents with type 1 di abetes confirming age-related differences in HRQOL in the general popu lation. Lower HbA1c ( < 8%) and intensified insulin therapy ( > 3 injections/ day) were associated with a better HRQOL in different domains ( P < 0.05). The s ubscale “chronic illness”showed a better HRQOL ( P < 0.001) in children and ad olescents with diabetes compared to age-matched controls with other chronic con ditions. Conclusion:Children and adolescents from a paediatric department specia lising in diabetes management report good health-related quality of life. Young er age, good metabolic control and intensified insulin therapy are associated wi th a better health-related quality of life. Dimensions of health-related quali ty of life appear to play different roles at different ages, emphasising the imp ortance of the multidimensional health-related quality of life concept and the value of age-appropriate self-reports.
  • 摘要：Intrahepatic portosystemic anastomoses are macroscopic communications between the venous portal system and the systemic circulation and located partly in the liver. We report 4 new cases of type II shunts, which illustrate the circumstances of the diagnosis of these exceptional anomalies. For 2 children, the diagnosis was done antenataly by US and spontaneous involution in a few months was observed. In the third case the malformation was evidenced fortuitously at 3 weeks of life, and this 6-year-old child remains completely asymptomatic so far. Then, in the fourth case, a cerebral venous thrombosis was fortuitously and antenatally evidenced in an otherwise uneventful pregnancy and portosystemic shunt was demonstrated postnataly in the extensive work up of the neonate.
  • 摘要：Background: The transanal one-stage endorectal pull-thro-ugh operation for Hirschsprung’s disease is relatively new and makes assessment of the functional outcome and colo-nic motility difficult. The aim of this study was to evaluate the stooling patterns and colonic motility after a one-stage transanal pull-through operation for Hirschsprung’s disease in children. Methods: Twenty-two children who underwent a one-stage transanal pull-through operation for Hirschspru-ng’s disease were followed up for at least 6 months. The children (17 boys and 5 girls) were from 12 months to 13 years of age (mean age, 4 years). All patients had an aga-nglionic segment confined to the rectosigmoid area (confir-med by preoperative barium enema and postoperative histology). Clinical outcome was assessed by interviews and questionnaires, and children were divided into symptomatic and nonsymptomatic groups. Contrast barium enema and defecography and determination of total and segmental colonic transit time (using radio-opaque markers) were performed on all 22 children. Results: The stooling patterns were considered satisfactory in 17 children. Of all the children, the mean stool times were 1 to 2 per day and only 2 were 8 to 10 per day; postoperative soiling was found in 4, constipation was observed in 2, and Hirschsprung-asso-ciated enterocolitis in 1. There was no incontinence, cuff infection, anastomotic leak, or mortality noted. Barium enema showed that the dilated and spastic colonic segment disappeared in all 22 children. The dilated sigmoid loops decreased in 17 (2 symptomatic, 15 nonsymptomatic) and disappeared in 5 (4 symptomatic, 1 nonsymptomatic). There was a significant difference between the decreasing and disappearing loop group in regard to stooling disorders (P < .05). Postoperative defecography showed that the anorectal angle of all children was open, fixed, and significantly larger than that of the preoperative and control groups (123.3° ± 15.1° vs 84.7° ± 8.3° vs 79.0° ± 11.6° , P < .01) and larger in the symptomatic group when compared with the nonsymptomatic group (135.6° ± 15.9° vs 111.0° ± 14.3° , P < .05). Postoperatively, the total gastrointestinal transit time, left colonic transit time, and rectosigmoid colonic transit time of all the children were shorter than preoperatively (26.8 ± 8.2 vs > 188 hours, P < .01; 6.3 ± 4.1 vs > 60 hours, P < .01; 11.8± 4.4 vs > 120 hours, P < .01) and similar to controls. The total gastrointestinal transit time and rectosigmoid colonic transit time of the symptomatic group were significantly shorter than the nonsymptomatic group (25.2± 5.6 vs 28.1± 10.1 hours, P < .05; 12.2± 6.7 vs 9.8± 4.0 hours, P < .05). Conclusions: The stooling pattern and colonic motility are satisfactory in most children after the one-stage transanal pull-through operation for Hirschsprung’s disease. Normalization of colon appearance and total and segmental colonic transit time are signs of recovery of colonic motility. Stooling disorders were noted in a few cases and may be related to decrease or disappearance of the sigmoid loop, dysfunction of the “ neorecto-sigmoid” , an open and fixed anorectal angle, and ischemia of the pull-through segment.
  • 摘要：Background: Most congenital cystic lung lesions (CCLLs) do not require in utero or perinatal intervention. The management of asymptomatic lesions is controversial: the theoretical risk of infection and malignancy is offset by whether thoracotomy in asymptomatic children is justified. We examined our recent experience and the role of minimally invasive surgery. Methods: We analyzed the pre- , peri- , and postnatal findings of all consecutive CCLLs diagnosed between 1997 and 2005. We reviewed records for pre- , and postnatal imaging, management, and outcome. Results: Thirty- five CCLL were diagnosed prenatally. Since 2000, all asymptomatic lesions were removed endoscopically at 6 to 18 months (thoracoscopy for 6 extralobar sequestrations, 3 intralobar sequestrations/- congenital cystic adenomatoid malformations, 5 bronchogenic cysts, and retroperitoneal laparoscopy for 2 intraabdominal sequestrations). Congenital cystic adenomatoid malformation elements were present in more than 70% . Two abdominal lesions have regressed, and 2 patients are awaiting intervention. One symptomatic infant underwent thoracotomy for congenital lobar emphysema. Conclusions: It has been argued that the risks associated with congenital lung lesions (infection and malignancy) justify intervention in the asymptomatic patient. In our experience, all these lesions could be safely removed using endosurgical techniques. Counseling of (future) parents should be updated to include minimally invasive surgery in the management algorithm.
  • 摘要：Introduction: Optimal treatment of recurrent papillary thyroid carcinoma (PTC) in children remains controversial. We reviewed our experience with recurrent PT C to better identify children diagnosed with it. Aims: The objective of this stu dy was to determine the risk factors, optimal treatment, complications, and prog nosis of recurrent PTC in children. Methods: This is a retrospective review of a ll thyroid resections for children aged 18 years or younger who have PTC at a si ngle institution from 1987 to 1999. Results: Thirty-six children, 7 boys (19%) and 29 girls (81%), underwent initial cervical exploration for PTC. Lymph node involvement was noted in 25 patients (69%); however, there was no distal disea se. An equal number of children underwent subtotal thyroidectomy (n = 18) and to tal (n = 18) thyroidectomy as their initial operation. Papillary thyroid carcino ma recurrences developed in 17 patients (47%) a median of 7 months (range, 1-4 3 months) after their initial operation. Recurrence was more common for patients with lymph node involvement (P < .01) and multiple nodules (P < .05) at present ation. Recurrence developed in 5 patients after subtotal thyroidectomy and in 12 patients after total thyroidectomy. Sixteen children with recurrent PTC had a s econd operation and 6 required a third operation. Total operative complications included 2 patients with permanent hypocalcemia and 1 patient with permanent rec urrent laryngeal nerve injury, all of whom had a total thyroidectomy. No patient died; however, 3 continue to harbor disease. Mean follow-up for patients with PTC was 65 months (range, 15 to 144 months). Conclusions: Thyroid resection comb ined with selective use of radioactive iodine ablation is a safe and effective t reatment for recurrent PTC in children. The best predictors of this recurrent di sease are lymph node involvement and multiple thyroid nodules at presentation.
  • 摘要：Background: The merits of primary nephrectomy (PN) vs preoperative chemotherapy (PC) for patients with Wilms’ tumor (WT) are much debated. Early data from the International Society of Pediatric Oncology suggested decreased intraoperative spillage but increased risk of local recurrence after PC. Patients with WT at our institution were managed with PC until 1996; subsequently, they underwent PN. This study compares these approaches as they affect tumor spillage, local recurrence, and survival. Methods: Patients with WT diagnosed at the Hospital for Sick Children from 1985 to 2003 were reviewed. Results: One hundred sixty patients were identified (114 PC and 46 PN). Tumor spill occurred in 6 (5.3% ) of 114 PC and 2 (4.3% ) of 46 PN patients. Tumor inhomogeneity, tumor size, and inferior vena cava compression/clot at diagnosis did not affect incidence of spill. Of 6 PC patients with surgical spill, 1 (17% ) had significant tumor shrinkage, compared with 87 (81% ) of 108 without spill (P < 0.001). Preoperative chemotherapy and PN had equal rates of surgical complications. Preoperative chemotherapy resulted in 12 (10.5% ) of 114 local recurrences vs 5 (10.8% ) of 46 with PN. Event- free survival and overall survival were 80% and 92% for PC at 129 months vs 85% and 96% for PN at 61 months. Conclusions: Preoperative chemotherapy and PN are equally effective in the treatment of WT with no difference in tumor spillage. Failure of the tumor to shrink in size with PC was significantly associated with an increase in tumor spillage and would suggest that a more cautious surgical approach be undertaken in these cases.
  • 摘要：Background/Purpose: Bilateral Wilms’tumors present a therapeutic challenge, p articularly with delay in presentation, when there is poor response to chemother apy and when associated with nephroblastomatosis. The primary aim of management is eradication of neoplasm, while at the same time preserving of renal function. Methods: Nineteen bilateral Wilms’tumors were seen in our service between 1981 and 2003. This represented 10%of the 190 patients with Wilms’tumors seen duri ng this period. There were 12 female and 7 male patients ranging in age from 7 m onths to 8 years. Fourteen had synchronous presentation, one of whom had liver m etastasis at diagnosis. Of the 5 patients with metachronous tumors, 3 had their initial nephrectomies done elsewhere. Nephroblastomatosis was identified in 18 ( 95%) of the patients. Treatment was, in most cases, according to National Wilms Tumor Study Group protocols, with initial bilateral biopsy, neoadjuvant chemoth erapy, and tumorectomy. Where indicated, nephrectomy (partial or complete) invol ved using ice dam topical cooling and vascular control, and in one case, bench s urgery and extensive renal reconstruction with orthotopic autotransplantation. R evision tumorectomy was used on 3 occasions for recurrence in areas of nephrobla stomatosis. There were 6 extrarenal relapses. Results: Ten patients are alive an d free of disease 1 to 15 years after treatment, all with well preserved renal f unction (lowest recorded glomerular filtration rate was 85 mL/min per 1.73 m2). Nine have died (2 of unrelated disease), including 6 of the 7 with spread outsid e the kidney. All 3 with unfavorable histology are alive. Of the 5 with metachro nous presentations, 4 are alive, as are 7 of 10 who presented in the last decade . Conclusions: Appropriate chemotherapy and conservative nephron-sparing and in novative surgery can achieve good results with preservation of adequate renal fu nction in nearly all cases. Nephroblastomatosis was an almost universal finding and requires close monitoring because Wilms’tumors developed in residual suspec t areas. Revision surgery was effective. Unfavorable histology did not have a re duced survival in our series. Metastatic spread outside the kidney had a poor pr ognosis.
  • 摘要：Enuresis nocturna is regularly treated by desmopressin,a vasopressin analog. Its side effects,notably neurological,are fortunately rare. We comment on 5 enuretic children on desmopressin who suffered from hyponatremic encephalopathy (natremia 115-127,median 117 mmol/l). Results. -Side effects appeared at therapeutic doses (10-40 mg/d intranasal). An excessive fluid intake at night was often noted,leading to a dilutional hyponatremia. This may be due to a lack of correct information to the parents. These children presented after a period of warning symptoms,such as headache,vomiting and altered consciousness. Parents could have sought earlier medical attention if they had been informed about these symptoms. Conclusion. -In the absence of fluid restriction,severe hyponatremia can occur in enuretic children on desmopressin. It is therefore mandatory for the prescribing doctor to adequately inform patients and parents to limit fluids at night when desmopressin is used,and seek medical help quickly if any sign of intracranial hypertension appears.
  • 摘要：Background: Children with esophageal foreign bodies are frequently seen by pediatric surgeons. Choking and dysphagia are common presentations; however, esophageal perforation has been reported. Historically, rigid esophagoscopy with extraction of the foreign body has been the recommended treatment. Alternatively, Foley balloon extraction is a safe and effective approach. Methods: Over a 16- year period, 555 children presented with an esophageal foreign body. Retrospective analysis of the medical record was undertaken. Statistics were by univariate analysis. Results: Two hundred ninety- eight boys and 257 girls presented with a mean age of 3.24 years. Dysphagia (37% ) and drooling (31% ) were the most common symptoms. Foreign bodies were lodged in the superior esophagus in 73% , and 88% of the objects were coins. Balloon extraction with fluoroscopy was performed in 468 children. Eighty percent of the objects were successfully removed with a mean fluoroscopy time of 2.2 min, and 8% were advanced into the stomach. The overall success rate was 88% , with failures necessitating rigid esophagoscopy under general anesthesia. Children younger than 1 year were the most likely to fail (25% failure rate). Airway aspiration never occurred. Significant savings in patient charges were observed with this approach. Conclusions: Balloon extraction of pediatric esophageal foreign bodies is a safe and cost- effective procedure. This technique is applicable for infants, children, and adolescents. Experienced practitioners should be able to achieve greater than 80% success rate.
  • 摘要：Background: The safety and efficacy of Seprafilm (Genzyme Corporation, Cambrid ge, Mass) in adult surgery patients have been established. The aim of this study was to evaluate the safety and efficacy of Seprafilm in pediatric surgical pati ents. Methods: One hundred twenty-two pediatric abdominal surgery patients were enrolled. Sixty-seven patients received Seprafilm application. Of these patien ts, 18 again received Seprafilm at abdominal closure during a second surgery, an d of the 18, 4 received Seprafilm at closure after a third surgery. Of the 55 co ntrol patients who did not receive Seprafilm, 14 had a second surgery, and of th ese 14 patients, 4 had a third surgery. Adverse events, operation time, and bloo d loss were compared with assessed Seprafilm safety. Seprafilm efficacy evaluati ons included incidence and severity of adhesions in those patients who required relaparotomy. Results: The incidence (Seprafilm, 40.9%; control, 82.4%) and se verity (Seprafilm: 59.1%, grade 0; control: 17.6%, grade 0) of adhesions under the abdominal incision site were significantly reduced in the Seprafilm group ( P = .007 and P = .0009, respectively). In addition, mean relaparotomy operation time was significantly shorter for Seprafilmpatients (P = .004). At relaparotomy , blood loss/body weight ratio for Seprafilm patients compared with control pati ents showed a trend toward but did not reach significance (P = .09). Conclusions : Decreased incidence and severity of postsurgical adhesions with Seprafilmin pe diatric patientsmay lead to reduction of the risks associated with subsequent op eration.
  • 摘要：Background/Purpose: Recently, the transanal 1-stage pullthrough operation has beenwidely used inHirschsprung disease (HD), and it is obviously superior to tr aditional approach in early term for its noninversion. However, the procedure is relatively so new that it makes assessment of the functional outcome and stooli ng patterns difficult. The aim of this study was to evaluate the clinical outcom es of the transanal 1-stage endorectal pull-through operation in the managemen t of rectosigmoid HD. Methods: Fifty-eight children (39 boys and 19 girls) aged 12 months to 13 years (mean, 2 years) who underwent transanal 1-stage endorect al pull-through operation for HD were followed up from 6 to 24 months. Clinical outcome was assessed by interviews and questionnaires. All patients had an agan glionic segment confined to the rectosigmoid area which was confirmed by the pre operative barium enema and postop erative pathological examination. Results: Forty-six patients had satisfactor y results without complications. In all the children, the mean stool times were 1 to 2 per day; only 4 had mean stool times of 8 to 10 per day. Postoperative so iling was present in 9, constipation in 5, and HD-associated enterocolitis in 3 . There were no incontinence, cuff infection, anastomotic leak, and mortality in any of the patients. In the 12 symptomatic patients, there were 4 children with length of aganglionic segment less than 30 cm, and 8 had 30 cm or more. In the 46 asymptomatic patients, 42 had length of aganglionic segment less than 30 cm, and 4 had 30 cm or more. There was a significant difference between the group wi th less than 30 cm and the group with 30 cm or more of aganglionic segment. For statistical analysis, the Fisher exact test showed P < .05. Conclusions: The tra nsanal 1-stage endorectal pull-through is a feasible and safe procedure in chi ldren with rectosigmoid HD. The clinical outcome is satisfactory. A gradual reco very could be noted in the stooling patterns along with the time after surgery. The younger the patient operated on and the shorter the aganglionic segment, the lower do the stooling disorders occur and the faster does the stooling function recover.
  • 摘要：Purpose: In fetuses with gastroschisis, the importance of ultrasonographic bowel dilation remains controversial. The outcome of patients with gastroschisis with and without prenatal bowel dilation is reported. Methods: From 2000 to 2004, 27 neonates with gastroschisis were followed at a single center. Thirteen patients had prenatal ultrasonographic bowel dilation (diameter, ≥ 6 mm; range, 6- 35 mm). Outcomes of those with and without dilation were compared using 2 sample t tests and logistic regression. Results: Time to initiation of enteral nutrition varied significantly between groups (20.4 ± 11.7 days vs 12.5 ± 4.3 days, P < 0.05). A trend toward a reduced rate of primary closure was seen in those with dilation (23% vs 50% , P = 0.06). No significant difference was found when considering mortality, gestational age, time in the intensive care unit (ICU), time on parenteral nutrition, or length of stay. Prenatal bowel dilation, a longer ICU stay, and later gestational age independently predicted readmission for bowel obstruction (P < 0.001). Conclusion: Infants with gastroschisis and prenatal bowel dilation were significantly slower to initiate enteral feeding and tended to have a reduced incidence of primary closure. This did not translate into increased mortality, time on parenteral nutrition, time in the ICU, or length of stay. However, dilation was associated with readmission for bowel obstruction.
  • 摘要：Background/Purpose: The minimally invasive Nuss procedure is emerging as the p referred technique for repair of pectus excavatum. Original methods of pectus ba r placement have been modified to improve safety and efficacy and avoid cardioth oracic complications. The currently reported modifications to facilitate retrost ernal pectus bar placement include routine use of right thoracoscopy or a subxip hoid incision. The purpose of this article is to describe additional modificatio ns of the Nuss procedure to improve safety and efficacy. Methods: A retrospectiv e analysis was performed on 51 patients who have had a thoracoscopic-assisted N uss procedure at The Children’s Hospital, Denver, Colo, between 1999 and 2002. Technical modifications included patient positioning, routine use of left thorac oscopy, and an Endo-kittner. Results: Fifty-one patients have successfully und ergone the Nuss procedure using the new modifications. Surgical time ranged from 45 to 120 minutes. There have been no intraoperative or postoperative bleeding complications. There have been 2 large pneumothoraces requiring needle thoracent eses in the operating room before extubati’on. No chest tubes were required pos toperatively. Subjectively, all patients have been satisfied with their surgical correction. Average length of hospital stay was 4 to 6 days. Conclusions: By us ing left chest thoracoscopy and Endo-kittner dissectors, the risk of cardiothor acic injury can be eliminated. Moreover, other methods to ensure safe substernal dissection are unnecessary.
  • 摘要：When caring for children who become suddenly and catastrophically ill, clinicians must simultaneously attend to a complex and rapidly evolving medical situation, as well as to the equally challenging demands of establishing compassionate relationships with family members and communicating well with colleagues. An 18-month-old toddler was brought to the hospital with severe head injury after being struck by a car. Over a period of hours, her condition evolved from prognostic uncertainty to the diagnosis of brain death and considerations of organ donation.Against this medical backdrop, the clinicians successfully established a trusting relationship with family members by careful attention to their emotional, informational, and care needs as they absorbed the devastating prognosis,took in the results of the brain death examination, and considered the option of organ donation. This case illustrates the importance of interdisciplinary communication,the vital role of social workers and other psychosocial providers with expertise in working with families, and the critical significance of mutual care and support for the clinicians who accompany families through these tragiclife events.
  • 摘要：Traumatic thoracic aortic transections are uncommon in the pediatric population. These injuries are currently treated by open operative repair via thoracotomies. We present 2 adolescent patients with traumatic thoracic aortic transections who were repaired by endovascular techniques. Both adolescents, aged 16 and 17 years, were in high- speed motor vehicle collisions and presented with multisystem trauma. Patient 1 had a transection of the descending aorta and was repaired with two 23- mm (diameter), 3- cm- long endograft cuffs at 48 hours after injury because of hermultiple organ injuries. She was hospitalized for 40 days. Patient 2 had a thoracic aortic transection just distal to the aortic arch. He was repaired within 12 hours of injury with a 24- mm and two 22- mm AneuRx endograft cuffs. He was hospitalized for 8 days. Both patients have recovered without complications at 13 and 21 months, respectively. Endovascular stenting, especially in critically ill patients, allows for definitive treatment of the vascular injury without the need for bypass and reduces the recovery time that is associated with thoracotomies. Short- term recovery and follow- up are encouraging for endovascular stenting in the adolescent population; however, further long- term follow- up is required.
  • 摘要：Background: The optimal treatment of pectus carinatum (PC) deformities is unclear. We propose a nonoperative approach using a lightweight, patient- controlled dynamic chest- bracing device. Material and Methods: With ethical approval, 24 patients with PC were treated at the Alberta Children’ s Hospital between January 1998 and April 2005. There were 6 (25% ) females and 18 (75% ) males, with a mean age of 12.9 years at the onset of treatment. Treatment involved fitting of a lightweight, patient- controlled chest brace, worn for 23 hours per day (correction phase [CP]) until the convex deformity was corrected. Following correction of the deformity, bracing was reduced to 8 hours per day (maintenance phase) until axial skeletal maturation ceased. Monitoring was done by measurement of the external pectus carinatum protrusion as well as subjective patient and surgeon appraisal of appearance and exercise tolerance. Results: Nineteen (79.2% ) patients have completed initial treatment (mean CP time, 4.3 ± 2.1 months). There were 3 patients (12.5% ) who were noncompliant, and 2 (8.3% ) are still in the initial CP phase of therapy. Fourteen (58.3% ) patients are presently in maintenance phase, nocturnally braced, and 2 (8.3% ) have completed therapy. In patients completing initial treatment, the protrusion pectus carinatum protrusion (pre 22 ± 6 vs post 6.0 ± 6.2) and subjective appearance (change + 1.8± 0.4) showed a significant improvement (P < 0.001 for both) with no change in exercise tolerance. Conclusion: Compressive bracing results in a significant subjective and objective improvement in PC appearance in skeletally immature patients. However, patient compliance and diligent follow up appear to be paramount for the success of this method of treatment. Further studies are required to show the durability of this method of treatment.
  • 摘要：Background/Purpose: Conjoined twins are rare and complex anomalies of the newborn. They require a highly experienced team and a center equipped to deal with such challenging anatomy. Method: A review of 31 sets of conjoined twins was managed by one team over a 15- year period. The spectrum of the anomalies, management strategies, and outcomes will be analyzed with future recommendations. Results: Ten sets of complete conjoined twins and two sets of parasitic conjoined twins were successfully separated. Tissue expanders and prosthetic meshes were not required. A separation of one set of craniopagus parasiticus conjoined twins was attempted, but abandoned owing to major vascular and brain sharing. The remaining 18 sets were not separable owing to major cardiac anomalies and/or sharing, and all died within several weeks of birth. Conclusion: Careful planning, a multidisciplinary approach, rehearsal, and experience are important factors in dealing with conjoined twins. Tissue expanders and prosthetic meshes are not required in most cases. Selective abortion or fetal intervention may play a role in the future.
  • 摘要：Purpose: This study, by its mere size and uniformity (1 pediatric surgeon), aims to corroborate or refute the teachings and myths of the pediatric inguinal hernia. Methods: From July 1969 to January 2004, 6361 infants and children with inguinal hernias were seen, operated on, and followed by the senior author. A retrospective survey of their charts was carried out to evaluate the demographics and clinical aspects of these patients. The hospital’ s research ethics board approved of this study. Results: The ages ranged from premature to 18 years (mean age, 3.3 years) with a male- to female ratio of 5:1. There were 59% right, 29% left, and 12% bilateral hernias (almost all indirect). Hydroceles were found in 19% . Incarceration occurred in 12% . A modified Ferguson repair was used. An opposite- side hernia developed in 5% , 95% within the first 5 years, and was not sex or age specific. There were 1.2% recurrences, 96% within 5 years. Thirteen percent had ventriculo- peritoneal shunts, 1.2% wound infections, and 0.3% testicular atrophy. There were no postoperative deaths. One percent had a documented hernia disappearance. Conclusions: Three of our results have not corresponded with previous teachings and myths: (1) a hernia of a premature baby should be fixed sooner than later; (2) routine contralateral groin exploration is not indicated in any situation; and (3) teenage recurrence rate is 4 times greater than the overall series.
  • 摘要：Background/Purpose:Because severe dysplasia and carcinoma in children with familial adenomatous polyposis (FAP) younger than 18 years is rare,earlier surgical intervention is not common. The purpose of this study is to report the prevalence of dysplasia and carcinoma among children with FAP in our institution. Methods:From 1998 through 2004,children 18 years or younger with FAP that underwent total proctocolectomy at a large children’s hospital were retrospectively reviewed. Results:Eleven children underwent surgery for FAP. The mean age at surgery was 13 ± 3.2 years. Approximately half of the patients who underwent preoperative endoscopy had evidence of dysplasia. Nine (82% ) patients had dysplasia on preoperative biopsy and/or operative specimen,and 3 (27% ) of these patients had severe dysplasia or carcinoma in situ. Four of the 5 patients without symptoms had evidence of dysplasia. No patients had invasive carcinoma. Conclusion:We observed a higher incidence of severe colonic dysplasia in young children with FAP compared with the current literature. A significant number of patients with dysplasia at surgery had no symptoms and no evidence of dysplasia on preoperative endoscopic biopsies. These data suggest that earlier intervention in children with FAP may be beneficial and should be investigated further.
  • 摘要：Purpose:Extracorporeal membrane oxygenation (ECMO) is a means of respiratory and hemodynamic support for patients failing conventional therapies. Children requiring hematopoietic stem cell transplantation who develop complications during therapy may require ECMO. Such patients pose medical and ethical challenges for clinicians considering initiation of ECMO. The authors review the outcomes of these patients and propose recommendations. Methods:The Extracorporeal Life Support Organization Registry was queried for all patients younger than 18 years with an International Classification of Diseases,Ninth Revision,or Current Procedural Terminology code related to bone or stem cell transplant. Results:Nineteen children in the registry met inclusion criteria. The median age was 9.6 years (7 months to 17.5 years). Initiation of ECMO was for pulmonary support (n=17),cardiac support (n=1),or cardiopulmonary resuscitation (n=1). The median duration of ECMO support was 5.1 days (range,30 hours to 42 days). Pulmonary infections included 3 parainfluenza,2 Pneumocystis carinii,1 influenza A,and 1 respiratory syncytial virus. Overall,15 (79% ) died during their ECMO run,whereas only 4 (21% ) survived to come off ECMO. Furthermore,of those who survived their ECMO run,only one patient survived to discharge from the hospital. Risk factors for death on ECMO include development of renal complications and development of multiorgan dysfunction. Conclusion:Patients who require ECMO for cardiopulmonary support after hematopoietic stem cell transplantation have a poor prognosis. Clinicians must be cautious in presenting this option to parents and present them with appropriate expectations in this high-risk population.
  • 摘要：Porin A (PorA), which determines the serosubtype of Neisseria meningitidis, is the main antigen of a candidate vaccine against serogroup B meningococci,which has been shown to induce high-avidity antibodies in children. We characterized the immune response of children after convalescing from meningococcal infection with a serosubtype P1.7-2,4 strain. Acute-and convalescent-phase sera of 21 c hildren with meningococcal septic shock caused by strains with PorA subtype P 1. 7-2,4 were collected. The serum bactericidal antibody titers, IgG isotype distr ibution, and antibody avidity were measured. We determined whether the differenc es in avidity of anti-outermembrane vesicle antibodies were PorA specific. Seru m bactericidal activity against H44/76 P 1.7-2,4 was < 4 in all convalescent sera. The IgG i sotype distribution of the convalescent sera was dominated by IgG1, followed by IgG3 , whereas no IgG2 or IgG4 was found. The geometric mean avidity index (GMAI ) of convalescent sera measured against a strain with the identical subtype as t he infective isolate was significantly higher than that against a strain with a heterologous PorA subtype or a PorA negative mutant strain (57 versus 35 and 23 %, respectively; p = 0.005 and p < 0.001). Geometric mean avidity titers were h ighest for P1.7-2,4, corresponding with the highest GMAI. The GMAI after invasi ve meningococcal disease was lower than after vaccination of healthy toddlers wi th a monovalent P1.7-2,4 outer membrane vesicle vaccine.
  • 摘要：Objective:To evaluate risk factors for recurrent thrombosis in pediatric patients. Study design:This prospective observational cohort study enrolled 120 patients with acute venous thromboembolism from January 2003 to April 2005. Datacollection included medical and family history,radiologic and laboratory studies,therapy,and follow-up. Results:The overall prevalence of recurrent thrombosis in our cohort was 19/120(15.8%). Patients with recurrence were older,with a median age of 14.8 years (range 2 weeks-23.6 years),compared with 10.1years (range newborn 23.4 years) in patients without recurrence(P = 0.03). Six of the 19 patients with recurrent thrombosis had cystic fibrosis (CF),compared with 0/101without recurrence (P< 0.001). Five of these 6 patients were colonized with Burkholderia cepacia in their sputum. Central venous catheters were associated with most,but not all,of the thromboses in patients with CF. Conclusions:In this study,patients with CF had a high risk of recurrent venous thrombosis,as well as a high prevalence of colonization with B cepacia. The cause of this risk has not been defined. This observation may have important implications for thromboprophylaxis,particularly in the setting of central venous catheters.
  • 摘要：Increased resting energy expenditure (REE) is a possible explanation for the negative energy balance seen in children with Alagille syndrome (AGS).We evaluated 16 subjects with AGS and 37 healthy controls and did not find a significant difference in REE (101%±12%,105%±13%,respectively).
  • 摘要：Despite that advances in neonatal medicine have significantly reduced the earl y mortality of premature infants, a considerable number of them are still prone to develop chronic lung disease (CLD) later. To find a method of early preventio n, we investigated the efficacy of using certain early proinflammatory responses to predict the development of CLD. In the present study, 34 premature infants w ho required endotracheal intubation within 4 h of birth were recruited for analy sis of IL-8, IL-10, and TNF-αlevels in their bronchoalveolar lavage (BAL) fl uid and blood. It was found that level of IL-8 but not TNF-αor IL-10 in init ial BAL fluid was significantly correlated to neutrophils in the BAL and inverse ly correlated to the gestational age of prematurity. Elevation of IL-8 level in BAL on the first day of life was correlated to the development of CLD. Further studies showed that neonatal cord blood released significantly higher IL-8 but lower TNF-αlevels after stimulation by endotoxin. The augmented IL-8 mRNA exp ression in cord blood was inhibited by actinomycin D but enhanced by cycloheximi de, suggesting that IL-8 production is controlled by de novo transcriptional in duction as well as posttranscriptional up-regulation of IL-8 by neonatal leuko cytes, relating to the development of CLD. Thus, an appropriate modulation of in itial IL-8 production in premature infants might be beneficial for the preventi on of the development of CLD.
  • 摘要：Background and Purpose:Prenatal corticosteroids have been used in fetuses with congenital diaphragmatic hernia (CDH)-. We tested the utility of steroids by 2 methods. Methods:Mothers carrying fetuses with CDH were randomized to 3 weekly doses of betamethasone or placebo starting at 34 weeks. Patients were followed until death or discharge. In a separate cohort study,the CDH Registry was used to compare infants who received prenatal steroids to those who had not. Results:Thirty-four patients were enrolled at 7 centers,with 32 completing the trial. There were 15 placebo and 17 steroid patients. There was no difference in survival,length of stay,duration of ventilation,or oxygen use at 30 days. For the cohort study,we looked at infants older than 34 weeks who were born after October 2000 when data on prenatal steroids were collected. There were 1093 patients; 390 were evaluable,with 56 receiving steroids. There was no difference in survival,length of stay,ventilator days,or oxygen use at 30 days. Conclusion:Neither the trial nor the CDH Registry suggest that late prenatal corticosteroids benefit fetuses with CDH. More than 1700 mothers and fetuses would need to be enrolled in a trial to show a 10% improvement in survival. It is unlikely that late steroids offer benefit to most fetuses with CDH.
  • 摘要：Objectives: To determine the risk of bronchopulmonary dysplasia(BPD) in subgroups of infants with and without patent ductus arteriosus (PDA) who were randomized to indomethacin prophylaxis or placebo, and to examine whether adverse drug effects on edema formation and oxygenation may explain why indomethacin prophylaxis does not reduce BPD. Study design:We studied 999 extremely low birth weight infants who participated in the Trial of Indomethacin Prophylaxis in Preterms(TIPP) and who survived to a postmenstrual age of 36 weeks.Results: The incidence of BPD in the 2 subgroups of infants with PDA was 52%(55/105) after indomethacin prophylaxis and 56%(137/246) after placebo. In contrast, rates of BPD in the 2 subgroups without a PDA were 43%(170/391) after indomethacin prophylaxis and 30%(78/257) after placebo (P[interaction] = 0.015). Logistic regression analysis with adjustment for prognostic base line factors showed that adverse and independent effects of indomethacin prophylaxis on the need for supplemental oxygen and on weight loss by the end of the first week of life may increase the risk of BPD in infants without PDA. Conclusions: Harmful side effects on oxygenation and edema formation may explain why indomethacin prophylaxis does not prevent BPD even though it reduces PDA.
  • 摘要：Opsoclonus-myoclonus syndrome (OMS) is an autoimmune disorder with serious neurodevelopmental morbidity and limited treatment options. We treated a toddler with moderately severe OMS with rituximab, a monoclonal anti-B cell antibody. The patient’s clinical response was documented on videotape and scored with the OMS Evaluation Scale. Cerebrospinal fluid lymphocyte subsets were evaluated by flow-cytometric immunophenotyping, with a comprehensive panel of monoclonal antibodies. Eradication of cerebrospinal fluid B cells, which previously were expanded, was associated with dramatic clinical improvement. There also were secondary changes in other lymphocyte subsets that might be relevant to the clinical response and lack of serious infections. In addition to clarifying the immune response to B-cell depletion, these data reveal a promising new therapy for OMS that warrants a phase I clinical trial.
  • 摘要：Insulin treatment of prepubertal children with insulin-dependent diabetes imp roves body protein balance by decreasing the rate of protein degradation without stimulating protein synthesis. However, insulin also causes hypoaminoacidemia, so the inability of insulin to stimulate protein synthesis may have been limited by substrate availability. We investigated the ability of insulin to stimulate protein synthesis in growing pubertal children who were given sufficient amino a cids to counter insulin-induced hypoaminoacidemia. Protein metabolism in six pu bertal children with type 1 diabetes was assessed from leucine kinetics during a primed, 6-h infusion of L-[1-13C] leucine. The children were studied in the postabsorptive state during a basal (insulin withdrawn) period and during the in fusion of 0.83 mU ·kg -1 ·min-1 human regular insulin. Amino acids and gluco se were given with insulin to prevent hypoaminoacidemia and hypoglycemia. Net le ucine balance was significantly higher with insulin than in the basal state, the result of decreased protein degradation but also decreased protein synthesis. T he data suggest that insulin alone does not increase protein synthesis in pubert al children with type 1 diabetes.
  • 摘要：Context: Streptococcus pneumoniae is a serious infection in young infants. A heptavalent pneumococcal conjugate vaccine (PCV7) was licensed in 2000 and recommended for all children aged 2 to 23 months. Objective: To determine the rates of invasive pneumococcal disease (IPD) in young infants before and after PCV7 was incorporated into the childhood immunization schedule in June 2000. Design, Setting, and Participants: A prospective, population- based study of infants aged 0 to 90 days who resided in areas in 8 US states with active laboratory surveillance for invasive S pneumoniae infections from July 1, 1997, to June 30, 2004. Main Outcome Measures: Rates of laboratory- confirmed IPD before ( July 1, 1997- June 30, 2000) and after ( July 1, 2001- June 30, 2004)- PCV7 introduction, excluding a transition year ( July 1, 2000- June 30, 2001). Results: There were 146 cases of IPD, 89 before and 57 after PCV7 introduction. Isolated bacteremia occurred in 94 cases (64% ), pneumonia in 27 (18% ), meningitis in 22 (15% )- , and septic arthritis and/or osteomyelitis in 3 (2% ). Mean rates of IPD for infants aged 0 to 90 days decreased 40% from 11.8 (95% confidence interval [CI], 9.6- 14.5) to 7.2 (95% CI, 5.6- 9.4; P=0.004) per 100 000 live births following PCV7 introduction. Among black infants, mean rates of IPD decreased significantly from 17.1 (95% CI, 11.9- 24.6) to 5.3 (95% CI, 2.8- 10.1; P=.001) per 100 000 live births, with a nonsignificant decrease from 9.6 (95% CI, 7.3- 12.7) to 6.8 (95% CI, 4.9- 9.4) per 100 000 live births for white infants. Rates of PCV7- serotype isolates decreased significantly from 7.3 (95% CI, 5.3- 10.1) to 2.4 (95% CI, 1.6- 3.8; P < .001) per 100 000 live births, while rates of non- PCV7 serotypes remained stable (P=.55)- . Conclusions: Since PCV7 introduction, rates of IPD in young infants have decreased significantly, providing evidence that vaccinating children aged 2 to 23 months has led to changes in pneumococcal carriage in infants too young to receive PCV7. With a significant decrease in rates of IPD among black infants, the previous racial difference has been eliminated.
  • 摘要：Background:Limitation of resident work hours has created the need to explore alternatives to surgeon presence during initial assessment and resuscitation for selected life-threat-ening injuries in children. We recently eliminated the requirement for surgeon presence during Level II alerts. The purpose of this study was to evaluate the impact of this change on patient care. Methods:A retrospective analysis of trauma alert activity was performed using data from our trauma registry. In March 2003,responsibility for level II alerts was transferred from the pediatric surgeons (PSs) to the Emergency Department (ED)-physicians. We compared the activity in the 18-month period before this change (period 1; n=627) to that afterward (period 2; n=587). Outcome measures included injury severity score,emergency department length of stay,missed injuries,abdominal computed tomography use,and mortality. Data were analyzed using log-rank statistic,χ 2,or t test,where appropriate,with significance level at P < 0.05. Results:During the entire study period,1499 patientsmet the trauma alert activation criteria of which 1214 (81% ) were level II alerts. The mean injury severity score for period 1 (8.5 ± 7.3 SD) was similar to period 2 (9.0 ± 7.1 SD). When ED physicians replaced PS for Level II alerts,ED length of stay increased from 135 minutes to 165 minutes (P < 0.001). In addition,the use of abdominal computed tomography was significantly decreased (53.6% vs 42.6% ; P < 0.001). However,there were no missed injuries and no significant differences in the rate of mortality. Conclusions:When ED physicians replaced PS for Level II alerts,trauma room length of stay was increased,but use of abdominal imaging was decreased with no differences in rate of missed injury or mortality. Emergency Department physicians can safely replace PS during Level II alerts. These findings may be useful to institutions experiencing surgical workforce limitations for trauma alerts.

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