Hirschsprung's disease:Historical notes and pathological diagnosis on the occasion of the 100^(th) anniversary of Dr.Harald Hirschsprung's death

Hirschsprung's disease(HSCR) or congenital megacolon is one of the differential diagnoses of chronic constipation mostly in infancy and may indeed represent a challenge for pediatricians, pediatric surgeons, and pediatric pathologists. The diagnosis relies clearly on the identification of the absence of ganglion cells at the plexuses(submucosus and myentericus) of the bowel wall. HSCR is usually located at the terminal(distal) rectum with potential pre-terminal or proximal extension to the less distal large bowel(sigmoid colon). Astonishingly, there is some evidence that Hindu surgeons of prehistoric India may have been exposed and had considerable knowledge about HSCR, but this disease is notoriously and eponymously named to Dr. Harald Hirschsprung(1830-1916), who brilliantly presented two infants with fatal constipation at the Berlin conference of the German Society of Pediatrics more than one century ago. Historical milestones and diagnosis of HSCR(originally called "Die Hirschsprungsche Krankheit") are reviewed. More than 100 years following his meticulous and broad description, HSCR is still a puzzling disease for both diagnosis and treatment. HSCR remains a critical area of clinical pediatrics and pediatric surgery and an intense area of investigation for both molecular and developmental biologists.

Transition of children with inflammatory bowel disease: Big task,little evidence

Children with chronic long-term disorders need to move to the adult practice at some point in their life. Establishing a smooth and efficient transition process is a complicated task. Transition of medical care to adult practice is def ined as the purposeful planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adultoriented health care systems. This step is of the utmost importance for several reasons. There is an obvious deficiency of research in this area especially when it comes to pediatric inflammatory bowel disease (IBD). There is a considerable difference in individual practice among different centers. Also, age of transition varies among different countries and sometimes, even within the same country, transition age may vary among different provinces and districts! Interestingly, local politics and many factors other than children’s welfare often play a role in deciding the age that older children move to adult practice at. This review discusses transition of children with IBD in view of the available evidence.

Acute kidney injury following spinal instrumentation surgery in children

AIM To determine acute kidney in jury （AKI） incidence and potential risk factors of AKI in children undergoing spinal instrumentation surgery. METHODS AKI incidence in children undergoing spinal instru-mentation surgery at British Columbia Children’s Hospital between January 2006 and December 2008 was determined by the Acute Kidney Injury Networ classification using serum creatinine and urine output criteria. During this specific time period, all patients following spinal surgery were monitored in the pediatric intensive care unit and had an indwelling Foley catheter permitting hourly urine output recording. Cases of AKI were identifed from our database. From the remaining cohort, we selected group-matched controls that did not satisfy criteria for AKI. The controls were matched for sex, age and underlying diagnosis （idiopathic vs non-idiopathic scoliosis）. RESULTS Thirty five of 208 patients met criteria for AKI with an incidence of 17% （95%CI： 12%-23%）. Of all children who developed AKI, 17 （49%） developed mild AKI （AKI Stage 1）, 17 （49%） developed moderate AKI （Stage 2） and 1 patient （3%） met criteria for severe AKI （Stage 3）. An inverse relationship was observed with AKI incidence and the amount of fluids received intra-operatively. An inverse relationship was observed with AKI incidence and the amount of fuids received intra-operatively classifed by fluid tertiles： 70% incidence in those that received the least amount of fluids vs 29% that received the most fluids （〉 7.9, P = 0.02）. Patients who developed AKI were more frequently exposed to nephrotoxins （non steroidal anti inflammatory drugs or aminoglycosides） than control patients during their peri-operative course （60% vs 22%, P 〈 0.001）. CONCLUSION We observed a high incidence of AKI following spinal instrumentation surgery in children that is potentially related to the frequent use of nephrotoxins and the amount of fuid administered peri-operatively.

加拿大儿科重症监护医师对脑死亡概念基础的理解

Objective:To determine whether pediatric intensivists in Canada are aware of the controversies regarding the concept of brain death (BD). Design:Prospective survey. Setting:From February to April 2004,a survey was mailed to each intensivist in the 15 pediatric intensive care units across the 8 provinces of Canada. Participants:Sixty-four practicing pediatric intensivists. Main Outcome Measures:Response rate,conceptual reasons to explain why BD is equivalent to death,and clinical findings that exclude a diagnosis of BD. Results:Of the 64 surveys,54 (84%) were returned.When asked to choose a conceptual reason to explain why BD is equivalent to death,26 (48%) chose a higher brain concept,17 (31%) chose a prognosisconcept,and only 19 (35%) chose a loss of integration of the organism concept. More than half the respondents answered that BD is not compatible with electroencephalographic activity,brain stem evoked potential activity,or some cerebral blood flow. More than a third of respondents answered that a brainstem with minimal microscopic damage was not compatible with BD. Of the 36 respondents who answered they were comfortable diagnosing BD because “the conceptual basis of brain death makes it equivalent to death of the patient,”in their own words,only 8 (22%) used a loss of integration of organism concept,9 (25%) used a prognosis concept,7 (19%) used a higher brain concept,and 13 (36%) did not articulate a concept.Conclusions:There is significant confusion about the concept of BD among pediatric intensivists in Canada. The medical community should reconsider whether BD is equivalent to death.

体外膜肺氧合支持的心肺复苏比传统心肺复苏有更高的出院存活率和更低的神经系统并发症发生率

背景 美国国内数据和国际儿科数据库都显示体外膜肺氧合（ECM0）支持的心肺复苏（E-CPR）的使用率在逐渐升高。考虑到E-CPR的使用需要消耗更多的医疗资源，患者的花费也更高，我们需要更审慎地使用这项技术以使患者获得最佳的预后。