Kasai portoenterostomy: differences related to race.

PURPOSE: The aim of this study was to evaluate the Kasai portoenterostomy in African-American and white children with respect to differences in presentation and outcome. METHODS: A retrospective review of all children with biliary atresia who underwent a portoenterostomy at our institution over the last 15 years (n = 63) was performed. Sex, age at the time of Kasai, preoperative laboratory tests, success rates (defined as postoperative total serum bilirubin < or = 2.0 mg/dL), and survival rate were recorded. Differences between African-American (AA; n = 30) and white (W; n = 33) children were analyzed. Long-term follow-up was available on 59 of 63 patients. RESULTS: Sixty-three percent of all patients (40 of 65) were girls, and 48% were AA (30 of 63). A higher percentage of AA children (73%) were girls than were white children (55%), although this difference did not achieve statistical significance. African-Americans underwent portoenterostomy at a later age, had higher alkaline phosphatase levels, and higher AST. These differences were statistically significant. Preoperative ALT, total bilirubin level, and GGTP levels all were greater in African-Americans, although these differences did not achieve statistical significance. There was a trend toward decreased success and survival rate, although these results also were not statistically significant. CONCLUSIONS: African-Americans underwent primary therapeutic intervention for biliary atresia at an older age than white children with a trend toward less favorable results. These differences related to race may be attributed to greater difficulty in diagnosing jaundice or poorer access to health care in this patient population. Increased effort at identifying biliary atresia in AA children may lead to earlier diagnosis and treatment and improved outcomes.

The Kasai portoenterostomy: when is it too late?

BACKGROUND/PURPOSE: Kasai portoenterostomy is recommended as the primary initial therapy for extrahepatic biliary atresia if the procedure can be performed within 10 to 12 weeks of life. The optimal management for infants with delayed presentation of biliary atresia remains controversial. The purpose of this study was to determine the success rate and outcome for patients who underwent a "late" Kasai portoenterostomy. METHODS: The authors conducted a retrospective review of the medical records of all patients with biliary atresia who underwent a Kasai portoenterostomy at their institution from 1986 to 1999 (n = 31). The authors analyzed success rates compared with age at the time of the Kasai procedure and the association with patient demographics. Surgical success was defined as achievement of a total serum bilirubin < or = 2 mg/dL. Long-term follow-up assessments included the need for liver transplantation and patient survival rate. RESULTS: The demographics of this study cohort showed a predominance of African-Americans, 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of success compared with subject age at the time of the initial portoenterostomy showed that 52% (13 of 25) had successful Kasai procedure at 0 to 75 days, compared with 83% success rate (5 of 6) at age 76 days or older (P = .359). Liver transplantation was performed in 16 of 31 patients (45%). Overall survival rate for the entire cohort is 23 of 31 (74%), whereas 12 of 31 (39%) are currently alive without a liver transplant. CONCLUSION: These data suggest that there is no contraindication to performing a Kasai portoenterostomy for biliary atresia in children over 75 days of age.

Inflammatory bowel disease in African-American children living in Georgia.

We describe the clinical characteristics of inflammatory bowel disease (IBD) African-American compared with non-African-American children. We identified 172 children with IBD; forty-nine (29%) were African-American. Median symptom duration before IBD diagnosis in African-American children (6 months) was shorter than that of non-African-American children (10 months). The most frequent presenting symptom was hematochezia (ulcerative colitis) and abdominal pain (Crohn's disease) in both racial groups. The estimated incidence of Crohn's disease in African-Americans ranged from 7 per 100,000 to 12 per 100,000, whereas the observed incidence in those with ulcerative colitis was between 5 and 7 per 100,000 during the 10 years of the study. Our pilot study suggests that IBD may be more common in African-American children than previously reported. Prospective population-based studies would be useful to determine whether inheritable factors linked with ethnicity are associated with IBD.

Safety and efficacy of percutaneous endoscopic gastrostomy in children.

OBJECTIVES: To establish the safety and efficacy of percutaneous endoscopic gastrostomy (PEG) placement for nutritional support in children. METHODS: The charts of 70 children who underwent the procedure between 1989 and 1992 were reviewed. Three of the 70 had repeat PEG placement. Patients ranged in age from 3 months to 24 yr, and included 28 females and 42 males. In all patients, the weights at the time of insertion of the PEG, and in 64, weight after 6 months of nutritional support, were compared by Z-score, and the incidence of major and minor complications was determined. RESULTS: Forty-five of 70 patients (70%) had improved nutritional status after initiation of PEG feedings, and in three (4%), the weight was maintained despite limited oral intake. Patients with congenital heart disease (86%) and cystic fibrosis (80%) derived the greatest benefit from the enteral feedings. Major complications were noted in 13 (19%) patients and minor complications in 16 (22%). Nine of 13 (70%) major complications and four of 116 (25%) minor complications occurred in the 12 (17%) children with multi-system organ failure. The rate of major complication was significantly greater in children with multi-system organ failure than in all patients (p < 0.001) and their relative risk of complication was increased by a factor of 40. CONCLUSIONS: Our data indicate that PEG is a safe and effective modality for nutritional support in children without multi-system organ failure. Those with multi-system organ failure have an increased rate of complications and a poor response to nutritional support, suggesting that the risk of PEG may outweigh its benefit for this population.

Pediatric liver transplantation in Georgia: a paradigm for the health care crisis in the United States?

The United States health care system, felt by many to be the most technologically advanced program in the world, has many critics. Two indisputable facts that drive such criticism are 1) inequitable access and 2) rising costs out of proportion to other countries. Although Georgia is a poor state and ranks nationally near the bottom in most measures of child and adolescent care, we decided to start a pediatric liver transplant program at Egleston Children's Hospital at Emory, Atlanta. Over the past 2 1/2 years, 18 transplants have been performed in 14 patients; 10 children are presently surviving. Looking carefully at the expenses of the first 10 patients, the average cost of orthotopic liver transplantation for the eight survivors was $206,375. The hospital costs for providing care to these 10 children were over $2 million. In a state that ranks 49th out of 50 states in infant mortality and with nearly one-third of its pre-school children not immunized against preventable diseases, is this a fair and equitable distribution of our resources?