Bone demineralization in a cohort of Egyptian pediatric liver transplant recipients: Single center pilot study.

Liver transplantation (LT) is the definitive treatment of end-stage liver disease. The long-term survival following LT spurred more interest in improving the quality of life of patients. This was a cohort study that included 23 pediatric liver transplant recipients who underwent LT due to hereditary or metabolic liver diseases. Bone health assessment was performed at their last follow up clinically (anthropometric measures), biochemically and radiologically (Dual Energy X-ray Absorptiometry [DEXA] scans). Poor bone health was defined as z-score <-1. Mean age at LT was 5.77 years (standard deviation [SD] 3.64) and 43% were males. Biliary atresia was the most common cause of end stage liver disease (35%). Mean age at follow up was 14 years (SD 5.48) and mean follow up was 8 years (SD 4.12 years). Eleven patients (48%) had poor bone health (osteopenia 22% and osteoporosis 26%). On univariate analysis, being on steroids at last follow up (odds ratio [OR] 13.2, 95% confidence interval [CI] 1.23-140.67, P = .03), weight at last follow up (OR 0.45, 95% CI 0.20-0.99, P = .04), platelets at last follow up (OR 0.98, 95% CI 0.96-s0.99, P = .02), hemoglobin at last follow up (OR 0.33, 95% CI 0.12-0.89, P = .03) were significantly associated with poor bone health. None of the variables were significant on multivariate analysis. At most recent follow up, 48% of patients demonstrated poor bone health by DEXA scans. More studies are required to evaluate predictors of poor bone health after LT in children.

Combined liver-kidney transplantation for primary hyperoxaluria type I in children: Single Center Experience.

Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to surgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwent simultaneous CLKT, only two survived and are well, one with insignificant complications, and other suffered from abdominal Burkitt lymphoma managed by excision and resection anastomosis, four cycles of rituximab, cyclophosphamide, vincristine, and prednisone. The other two died, one due to uncontrollable bleeding within 36 hours of procedure, while the other died awaiting renal transplant after loss of renal graft to recurrent renal oxalosis 6 months post-transplant. PH1 with ESKD is a rare disease; simultaneous CLKT offers good quality of life for afflicted children. Graft shortage and renal graft loss to oxalosis challenge the outcome.

Pulmonary hypertension and cardiac hypertrophy in children recipients of orthotopic living related liver transplantation.

Surgical stress, liberation of cytokines associated with re-perfusion injury, and long standing use of immune suppressive medications in children recipients of orthotopic living related liver transplantation (OLRLT) pose cardiovascular risk. Reported cardiovascular adverse effects vary from left ventricular wall thickening, hypertrophic cardiomyopathy to resting ECG abnormalities, asymptomatic ST depression following increased heart rate and ventricular arrhythmias. Twenty-five consecutive children recipients of OLRLT were assessed by conventional 2-D, M-mode echocardiography and Doppler. The mean age ± SD at transplantation and at enrollment in study was 6.3 ± 4.5 and 13.5 ± 5.6 years respectively. All children were on immunosuppressive medications, with tacrolimus being constant among all. Long-term post-transplant echocardiography revealed statistically significant interventricular septal hypertrophy among all (mean thickness 0.89 ± 0.16 cm), (P = 0.0001) in comparison to reference range for age, 24 had pulmonary hypertension (mean mPAP 36.43 ± 5.60 mm Hg, P = 0.0001), and early diastolic dysfunction with a mean Tei index of 0.40 ± 0.10. However cardiac function was generally preserved. Children recipients of OLRLT have cardiac structural and functional abnormalities that can be asymptomatic. Pulmonary hypertension, increased cardiac mass, de novo aortic stenosis and diastolic heart failure were among abnormalities encountered in the studied population. Echocardiography is indispensible in follow-up of children recipients of OLRLT.

Therapeutic superiority of combined propranolol with short steroids course over propranolol monotherapy in infantile hemangioma.

UNLABELLED: Infantile hemangiomas are the most common benign childhood tumor that may have functional and/or cosmetic complications. We aimed to compare the clinical efficacy of propranolol alone and propranolol primed with systemic corticosteroids on the outcome of infantile hemangioma. A prospective randomized study included 40 infants aged less than 9 months with cutaneous hemangiomas. Patients were randomly assigned into two groups: group A were given oral prednisolone for the initial 2 weeks combined with oral propranolol, while group B were given oral propranolol alone for 6 months. The median age of the studied patients was 4.5 months (ranged 4 weeks-8 months). Sequential determination of the dimensions of the hemangiomas based on direct measurement and photographic analysis were performed. A significant reduction in the size of the lesions was found in group A in the 2-, 4-, and 8-week evaluation compared to group B (p < 0.001) with no statistical difference in the ultimate 6 month response (p = 0.134). Multiple logistic regression showed that early treatment before 6 months of age (OR 9.82, p = 0.007) and combined treatment with propranolol and prednisolone (OR 10.71, p = 0.006) were the predictors of best response. CONCLUSION: Combining propranolol with corticosteroids gives a faster response and should be considered in treating life- or function-threatening hemangiomas.

Topical mitomycin C application is effective in management of localized caustic esophageal stricture: a double-blinded, randomized, placebo-controlled trial.

BACKGROUND: Frequent sessions of endoscopic dilatation are usually required in the management of benign esophageal strictures, especially caustic induced ones. Topical mitomycin C (MMC) has been recently used in the management of resistant strictures. This study evaluated the efficacy of MMC application in prevention of stricture recurrence after endoscopic dilatation. PATIENTS AND METHODS: This double-blind, randomized, placebo-controlled trial included forty patients with caustic esophageal strictures dating from January 2008 to October 2010. Patients were randomized into 2 groups to undergo endoscopic dilatation with application of either MMC versus placebo on stricture site. Regular follow up and re-evaluation were done after 6 months of management. The number of dilatation sessions needed for resolution of dysphagia in each group was our primary outcome. RESULTS: During the specified follow up period, 80 % of strictures in the MMC group got completely resolved compared to only 35% in the placebo group. The mean number of dilatation sessions needed in the MMC group was n = 3.85 ± 2.08 compared to n = 6.9 ± 2.12 in the placebo group which was statistically significant (p < 0.001). CONCLUSION: Mitomycin C application significantly reduced the number of dilatation sessions needed to alleviate dysphagia in patients with caustic esophageal strictures.

Liver transplantation in an African setting.

Liver disease in children in the developing world is a frequent occurrence, which is generally inadequately managed because of lack of resources. However, increasingly, there has been a demand for liver transplantation, where primary medical or surgical therapies have failed. The expertise and infrastructure required for a successful outcome are no different from those in more developed countries; if anything, the challenges are greater. Lack of deceased donors because of cultural and religious factors has driven the use of living donors. Short-term survival has generally been good, but long-term outcomes have rarely been reported. In this article, we review the experience of 2 centers at opposite ends of the continent and share our experience of slightly different settings and show that success can be achieved even in resource-reduced environments.

Colonic replacement in cases of esophageal atresia.

Replacement of the esophagus in children is still a challenging problem; one of the major indications is failed esophageal atresia repair or long-gap ones. The colon is one of the best alternatives for replacement; long-term follow up has shown satisfactory results. In cases of complicated repair receiving frequent dilation and multiple operations, colon could be an alternative choice for these children to achieve normal swallowing.

Congenital esophageal stenosis associated with esophageal atresia: new concepts.

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen's fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of the LEP during primary esophageal repair for histologic studies is highly recommended.

Evolution of living donor liver transplantation in Egypt.

OBJECTIVE: To date, cadaveric organ donation is illegal in Egypt. Therefore, Egypt recently introduced living donor liver transplantation (LDLT), aiming to save those who are suffering from end stage liver disease. Herein, we study the evolution of LDLT in Egypt. METHODS: In Egypt, between August 2001 and February 2004, we approached all centers performing LDLT through personal communication and sent a questionnaire to each center asking for limited information regarding their LDLT experience. RESULTS: We identified and approached 7 LDLT centers, which collectively performed a total of 130 LDLT procedures, however, 3 major centers performed most of the cases (91%). Overseas surgical teams, mainly from Japan, France, Korea, and Germany, either performed or supervised almost all procedures. Out of those 7 LDLT centers, 5 centers agreed to provide complete data on their patients including a total of 73 LDLT procedures. Out of those 73 recipients, 50 (68.5%) survived after a median follow-up period of 305 days (range 15-826 days). They reported single donor mortality. Hepatitis C virus cirrhosis, whether alone or mixed with schistosomiasis, was the main indication for LDLT. CONCLUSION: Egypt recently introduced LDLT with reasonable outcomes; yet, it carries considerable risks to healthy donors, it lacks cadaveric back up, and is not feasible for all patients. We hope that the initial success in LDLT will not deter the efforts to legalize cadaveric organ donation in Egypt.

Caustic esophageal strictures in children: 30 years' experience.

Many children in developing countries continue to sustain caustic esophageal injures. The first line of treatment is dilatation, unless contraindicated, where 60% to 80% success rate is expected. In cases of failure, esophageal replacement is the only hope for achieving normal swallowing. Over the last 30 years, more than 850 cases of esophageal replacement were done in the Pediatric Surgery Department at Ain-Shams University. Three types of replacement were performed, gastric pull-up (75 cases), retrosternal colon replacement (550 cases), and, in the last 12 years, transhiatal esophagectomy with posterior mediastinal colon replacement (225 cases). Complications in the last 475 cases include 10% cervical leakage, 5% proximal strictures, 2% postoperative intestinal obstruction, 1% mortality, and 0.6% late graft stenosis. Colonic replacement of the esophagus is the ideal treatment in cases of caustic esophageal strictures after failure of dilatation. The posterior mediastinal route is shorter, and in long-term follow-up results show improved evacuation and less reflux than with the retrosternal route.