单气囊小肠镜在小肠出血患儿中的临床应用

目的：探讨单气囊电子小肠镜在小肠出血患儿中的诊断价值和安全性。方法：临床怀疑小肠出血患儿共70例,其中男 38 例,女 32 例,年龄 4～13 岁;均在全麻下行单气囊小肠镜检查,其中经口途径检查 26例,经肛门途径检查 32 例,同时经口和肛门检查12例。结果：70例患儿中检查出小肠病变58例,检查阳性率 83％,其中非特异性炎症 24例,过敏性紫癜 12 例,克隆病 8 例,美克尔憩室 8 例,Peutz-Jeghers综合征 6 例。结论：单气囊小肠镜检查是诊断小肠出血患儿一项较为安全、有效的检查手段。     

Roux‐en‐Y loop varices in children with portal hypertension after liver transplantation: An unusual cause of “obscure” gastrointestinal bleeding

Chu J, Kerkar N, Miloh TA, Rodriguez‐Laiz G, Lewis B, Stangl A, Newton KP, Iyer K, Arnon R. Roux‐en‐Y loop varices in children with portal hypertension after liver transplantation: An unusual cause of “obscure” gastro intestinal bleeding.
Pediatr Transplantation 2011: 15: E156–E161. © 2010 John Wiley & Sons A/S. Abstract: PHALT may result from graft dysfunction, portal vein thrombosis, arterio‐venous fistulas and can lead to GIB, commonly from bleeding esophageal varices. We present three children with GIB requiring multiple blood transfusions that were diagnosed with RY Loop bleeding. Routine EGD, colonoscopy, and CE failed to reveal the bleeding source. However, enteroscopy revealed large varices at the site of hepaticojejunostomy anastomosis in all. Our experience demonstrates that RY loop varices in children with PHALT are a rare and treatable cause of obscure GI bleeding.     

改良肝总管空肠Roux-Y型吻合术治疗小儿先天性胆总管囊肿

目的 探讨小儿先天性胆总管囊肿的治疗方法。方法 本组２８例,男９例,女１９例,年龄１０ｄ￣１４岁,均采用改良的肝总管空肠Ｒｏｕｘ－Ｙ型吻合术治疗先天性胆总管囊肿。结果 ２８例均痊愈出院。随访６个月￣８年,全组患儿未见出现腹痛、黄疸、发热。其中２０例患儿,经Ｂ超检查,未见胆管狭窄及扩张。Ｘ线钡餐检查,未见有钡剂反流入胆道。结论 改良肝总管空肠Ｒｏｕｘ－Ｙ型吻合术是治疗先天性胆总管囊肿的较好方法。     

Jejunal varices diagnosed by capsule endoscopy in patients with post‐liver transplant portal hypertension

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%‐10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7‐16 years) with a median Hgb of 2.8 g/dL (range 1.8‐6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid‐jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant.     

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??Enteroscopy has high diagnostic and therapeutic value in adult intestinal diseases and is a safe and effective method. However??enteroscopy is rarely used in children. Enteroscopy is used for the diagnosis and treatment of obscure gastrointestinal bleeding??intestinal polyps??Crohn’s disease??intestinal stenosis and biliary stricture in children. The paper summarizes the clinical application of enteroscopy in small bowel disorders in pediatric patients.     

Improving adherence to medications in pediatric liver transplant recipients

Abstract:  We describe results from a clinical program, which aimed at improving adherence to medications in children who had a liver transplant. We followed the medical outcomes of 23 children and adolescents who participated in a clinical adherence-improvement protocol during the years 2001–2002. The protocol included identification of non-adherent patients by examining tacrolimus blood levels and intervention by increasing the frequency of clinic visits for non-adherent patients. In the two-yr preintervention (1999–2000), there was no improvement in any of the outcomes. After the intervention, the number of patients with high alanine aminotransferase levels (100 and above) decreased significantly, from eight before the intervention to four afterwards. Other outcomes, including the number of rejection episodes (three before, none after) and the degree of adherence to tacrolimus, also improved, but the improvement did not reach statistical significance. Although non-adherent patients were called to clinic more often under the protocol, the intervention did not lead to increased outpatient costs. This adherence--improvement intervention appears to be promising in improving outcomes in pediatric liver transplant recipients. Larger, controlled studies are needed to establish the efficacy of this or other approaches.     

Simultaneous surgical and interventional radiological approach to treat complicated biliary strictures after pediatric liver transplantation

Post-transplantation biliary strictures occur in 5-15% of the pediatric liver transplant patients and are conventionally managed by interventional radiological techniques. Failure of this treatment leads to reoperation and sometimes to retransplantation. Herein, we describe a surgical approach and interventional radiologic approach to manage biliary strictures that failed the conventional radiologic treatment, in order to avoid retransplantation. Included in the study were eight children who underwent liver transplantation at our center or referred to our institution for evaluation of the biliary strictures that failed radiological treatment. Biliary strictures were confirmed by a narrowing of the biliary anastomosis on the percutaneous transhepatic cholangiogram. At surgery, a guide wire was introduced into the distal bile system through the use of an enterotomy in Roux limb. Over the guide wire, the stricture was ballooned and the diameter of the biliary tree was determined. A pigtail catheter was introduced on the biliary tree across the abdominal wall, the liver, the stricture and the anastomosis into the enterotomy. A final cholangiogram confirmed the positioning of the catheter. Mean follow-up was 39.8 +/- 20.8 months. All patients had their strictures successfully treated and survived the procedure. Three patients were readmitted to the hospital with fever. It was necessary to revise the hepaticojejunostomy in three patients because of cholangitis and/or recurrence of biliary stricture. Of the eight patients of this study, two required retransplantation and one died. We conclude that an aggressive combined surgical and radiologic approach can avoid retransplantation in patients with complicated post-transplant biliary strictures.     

Massive gastrointestinal bleeding from splenojejunal varices in a child: A complication of splenic-vein occlusion following near-total pancreatectomy for hyperinsulinemic hypoglycemia

Severe jejunal variceal bleeding occurred in a child with splenic-vein occlusion following a near-total pancreatectomy for hyperinsulinemic hypoglycemia. This rare sequela of splenic-vein occlusion required dismantling of the varices and splenectomy.     

Reversal of tacrolimus-related hypertrophic cardiomyopathy after conversion to rapamycin in a pediatric liver transplant recipient

Tacrolimus (Tac)-related hypertrophic cardiomyopathy (HCM) has been reported to be an unusual but serious complication affecting pediatric patients after solid organ transplantation. Herein, we present a case of young liver transplant recipient with Tac-induced HCM, treated by discontinuation of Tac followed by conversion to rapamycin (Rap). Our case report points out the potential but rather low risk of HCM during Tac immunosuppression in pediatric liver transplants and demonstrates that replacement of calcineurin inhibitors with mammalian target of Rap (mTOR) inhibitors may be an efficacious therapeutic tool to effect regression of established cardiac hypertrophy.     

Evaluating non-adherence to immunosuppressant medications in pediatric liver transplant recipients

Abstract:  Non-adherence with recommended immunosuppressant medications is common post-pediatric liver transplant and is the most important reason for organ rejection in long-term survivors. However, there is currently no validated, standard method to measure adherence, with a well-defined threshold, making it extremely difficult to evaluate interventions to improve adherence. Previous studies have suggested that the degree of fluctuation of medication blood levels over time can provide an idea about how regularly the medication is being taken. The present study, conducted at UCLA medical center, sought to identify a specific threshold value of the s.d. of individual tacrolimus blood levels in pediatric liver transplant recipients which would be associated with rejection episodes in these patients. A threshold of 3.0 has been identified in other studies, and was supported by the analysis of retrospective data from 96 subjects. However, further analysis found that a s.d. of 2.5 appeared to have a better fit with the data. These findings suggest the utility of monitoring the s.d. of routine tacrolimus blood levels in pediatric liver transplant recipients for detecting non-adherence to immunosuppressant medication prior to clinical rejection, allowing earlier interventions.     

Transition of pediatric liver transplant recipients to adult care: patient and parent perspectives

The need to prepare pediatric transplant recipients for the transfer to adult-centered transplant care has received increased attention. This study aimed to determine adolescent and young adult LTR and parent perceptions and attitudes about the transition process. LTR and their parents completed a survey assessing level of prior thought and interest in learning about transferring care, knowledge of the transition process, perceived importance of self-management skills, concerns about moving to the adult clinic, and responsibility for health management tasks. Responses were analyzed by age, gender, and time since transplantation. Participants included 46 LTR (mean age = 16.6 yr; range 12-21), and 31 parents. Recipients and parents reported moderate concern about transition, with leaving pediatric providers being a primary worry. LTR ≥16 yr reported greater health care responsibility and increased thought, interest, and knowledge about transition. There were significant differences between parent and LTR perceptions of health care responsibility, indicating that LTR perceive having more independence than what their parents report. Overall, results suggest that adolescent and young adult LTR and their parents perceive the importance of transitional care, but demonstrate poor knowledge of the process. There remains a need for improved transition planning for both adolescents and parents.     

The relationship between procollagen-III-peptide and the severity of esophageal varices in children with biliary atresia after Kasai operation

Biliary atresia (BA) is a common cause of infantile cholestasis. Disease progression leads to intra hepaticfibrosis, and thus to the development of PH and EV. Our objective has been to study the relationship between procollagen-III-peptide (PIIIP) and the severity of EV in children with BA after Kasai operation. Children below 15 years of age (n=29) with BA after a Kasai operation were evaluated for EV by endoscopy. Healthy (n=26) children of the same age and sex distribution who participated in the hepatitis B vaccination program served as the controls. Serum PIIIP was determined by radioimmunoassay. The BA patients were classified on the basis of severity of EV (Paquet's classification) into three groups: group 1 (n=15) had grade 0, group 2 (n=8) grade 1–2, and group 3 (n=6) grade 3–4 EV. In group 3, serum PIIIP (2.9 ± 1.3 IU/ml) was significantly higher than in group 2 (1.5 ± 0.4 IU/ml) (P < 0.05). Serum PIIIP levels were increased in group 2 compared with group 1 (1.2 ± 0.4 IU/ml) and in group 1 compared with the control group (1.2 ± 0.2 IU/ml), but this difference was not significant. PIIIP levels increased with severity of the EV in the BA patients. Hence, high PIIIP levels may serve as a non invastive indicator of EV developing in postoperative BA patients. Accepted: 8 January 2001     

Serum C-reactive protein in pediatric kidney and liver transplant patients

The clinical manifestations of different complications after organ transplantation (Tx) are often vague, and simple laboratory tests for early diagnosis would be valuable. In this work we retrospectively analyzed our data on the daily measurements of serum C-reactive protein (CRP) in 132 children after 63 liver and 83 kidney Txs. A total of 3,886 CRP measurements were performed and 353 episodes of elevated CRP were recorded. One-third of these were regarded as a response to surgery. The CRP level normalized within 5-10 days post-operatively in patients who had a favorable outcome, but in patients with a poor outcome CRP tended to remain elevated. Half of the CRP elevations were associated with complications such as acute rejection, infection or thrombotic events. An elevated serum CRP level was found in 68% of the rejection episodes, in 88% of the bacterial infections, and in 73% of the viral infections. The most significant elevations were associated with bacterial infection. In nine of 11 vascular complications, an elevation of CRP was also recorded. Serum CRP responded to rejection therapy in 86% of the episodes. The initial CRP level did not predict steroid-resistant rejection. CRP seemed to be a more sensitive marker than fever or white blood cell (WBC) count in all complications. We conclude that the daily measurement of serum CRP is a simple and fairly sensitive, but non-specific, method for detecting rejection and infectious complications after pediatric liver and kidney Tx.     

Diaphragmatic hernias after pediatric liver transplantation: Experience of a high‐volume transplant center

Diaphragmatic hernias (DHs) are rare complications after pediatric liver transplantation (PLT). It is now widely accepted that DHs after liver transplantation (LT) is a pediatric related condition. PLTs (under of age 18) performed between January 2013 and June 2019 at Malatya Inonu University Institute of Liver Transplantation were retrospectively scanned. Study group consisting DHs and a control group were compared. Among 280 PLTs, 8 of them were complicated with DHs (%2.9). Median age of the patients with DH was 3.0 (0.8‐9.5) years. Median graft recipient weight ratio was 2.5 (0.9‐4.4). Five patients were below 5th percentiles in terms of pediatric weight growth chart at the time of LT. Also, 6 patients were below 5th percentiles in terms of pediatric height growth chart. There was no statistical difference between study and control groups. There are many risk factors mentioned in literature that may be primarily responsible for DHs after PLT. These factors are left lobe and large‐for‐size grafts, malnutrition, trauma or diathermy of diaphragmatic nerve and vessels and immunosuppressants. In our study, we could not specify any reason that differs in DHs. In our aspect, narrow diaphragma and thorax are exposed to high intra‐abdominal pressure from abdomen. Large‐for‐size grafts, which are specific to children, also may contribute to this affect. Excessive diathermy and trauma to diaphragmatic collaterals may aggravate the risk of DH. More patients are needed to make an exact conclusion, in order to evaluate with comparable study on this aspect.     

Transition to adult care for pediatric liver transplant recipients: the Western Australian experience

Transition to adult care is a vulnerable period for pediatric transplant recipients and is associated with reduced medication compliance, graft loss, and increased mortality. Psychosocial outcomes in young adults differ between pediatric transplant recipients and their healthy peers. We conducted a single‐center, retrospective cohort study of all pediatric liver transplant recipients who were transitioned through our center. This study aimed to assess the outcomes of transitioned pediatric liver transplant recipients at an Australian center, including mortality, adherence, and psychosocial morbidity. The 1‐ and 5‐year survival rates following transition were 100% and 92%, respectively. There were no episodes of late rejection. In total, 66.7% of patients were compliant with immunosuppression and 61.1% of patients were compliant with clinic attendance. There was a significant relationship between medication compliance and clinic attendance, as well as presence of psychological issues with clinic non‐attendance. Psychosocial outcomes were in keeping with age‐matched data from the general population. All patients were employed or studying following transition. This is the first study of its kind in the Australian population, and outcomes were superior to published international data. Despite the demonstrated good outcomes, transition programs may improve healthcare engagement in this cohort.