ABO-incompatible Pediatric Liver Transplantation With Antibody and B-cell Depletion-free Immunosuppressive Protocol in High Consanguinity Communities.

The success of orthotopic liver transplantation as a life-saving treatment has led to new indications and a greater competition for organ grafts. Pediatric patients with acute liver-related crises can benefit from orthotopic liver transplantation, but organ availability in the limited time can be a major obstacle. Crossing ABO blood group barriers could increase the organs available to such patients. Methods: From November 2010 to June 2015, 176 children aged 0.2-to18 y were transplanted in the King Faisal Specialist Hospital and Research Center. Out of those, 19 children were transplanted across blood group barriers (ABO incompatible). The underlying diseases were biliary atresia (n = 6); progressive familial intrahepatic cholestasis type 2 (n = 4); Crigler-Najjar syndrome (n = 3); hepatoblastoma (n = 2); and urea cycle disorder, Caroli disease, cryptogenic cirrhosis, and neonatal sclerosing cholangitis (n = 1 each). Immunosuppression consisted of basiliximab, mycophenolate, tacrolimus, and steroids. Pretransplant prophylactic plasmapheresis, high-dose immunoglobulins, and rituximab were not administered. Results: The grafts were from living donors (n = 17) and deceased donors (n = 2). Living donor morbidity was nil. The recipient median age was 21 mo (5-70 mo). After a median follow-up of 44 mo, 2 recipients (10%) died because of sepsis, 1 because of uncontrolled acute myeloid leukemia. The overall rejection rate was 7%, and no grafts were lost because of antibody-mediated rejection (AMR). HLA matching was 3.8 of 6 (A, B, DR), and there were 2 patients presented with acute cellular rejection, 1 patient with AMR, and 1 patient with biliary strictures. Conclusions: ABO incompatible liver transplantation is a feasible and life-saving option even with antibody and B-cell depletion-free protocol without increasing the risks for AMR. We speculate that this excellent result is most likely because of presence of relatively low titer ABO isoagglutinins and the high HLA match compatibility caused by habit of longstanding interfamilial marriages as typical of Saudi Arabia.

Early Hepatocellular Carcinoma Associated With Fibrocystic Liver Disease in a 10-Year-Old Child: A Case Report.

BACKGROUND: Fibrocystic liver-kidney disease is caused by a group of rare and genetically diverse disorders that are associated with kidney cysts or dysplasia and ductal plate malformation in the liver. There have been several reports of liver neoplasias arising in hepatobiliary fibrocystic diseases. However, most were cholangiocarcinoma; cases involving hepatocellular carcinoma (HCC) are rare, and all the reported cases are related with adults. CASE REPORT: A 10-year-old girl with a history of repeated gastrointestinal bleeding underwent banding and sclerotherapy multiple times and had a history of a Portosystemic shunt without any significant benefit. She was referred to us as a case of fibrocystic liver disease with decompensated liver disease for liver transplantation. The patient underwent living donor liver transplantation, and the explanted liver histopathology report is documented. The explant liver weighed 838 g and measured 21 × 13 × 8.5 cm with the attached gallbladder measuring 7 × 3 × 0.2 cm (in wall thickness). The external surface was covered with multiple white nodules ranging in size from 0.4 to 1 cm. Serial slicing revealed an ill-defined, yellow, soft lesion (4 × 2.5 × 2.5 cm) localized in the subcapsular area of the left lobe (segment 4). The rest of the cut surface was green and nodular (cirrhotic). Microscopy from largest nodule was consistent with early hepatocellular carcinoma.The rest of the liver was cirrhotic, and the morphology was consistent with fibrocystic disease of liver. CONCLUSION: We report a rare case of HCC associated with fibrocystic liver disease. When diagnosing fibrocystic liver disease without known risk factors, the presence of HCC must be considered, and vice versa. To our knowledge, this is the first reported case of HCC associated with fibrocystic liver disease in a 10-year-old child.

Frequency of retinopathy of prematurity in premature neonates with a birth weight below 1500 grams and a gestational age less than 32 weeks: a study from a tertiary care hospital in a lower-middle income country .

INTRODUCTION: Retinopathy of prematurity (ROP) is a treatable cause of blindness in neonates. In Pakistan, ROP is often not recognized early because screening and treatment programs are not yet in place in most neonatal units, even in tertiary care hospitals. It is hoped that this report will help inform medical professionals of the magnitude of the problem and help to design appropriate management strategies. OBJECTIVES: The aim was to determine the frequency of ROP in premature and very low birth weight (BW) neonates (BW<1500 g and gestational age (GA) <32 weeks). STUDY DESIGN: Cross-sectional study. STUDY SETTING: Neonatal intensive care unit (NICU) of a tertiary care hospital in Karachi, Pakistan. STUDY DURATION: From June 2009 to May 2010. SUBJECTS AND METHODS: Neonates with a Birth weight (BW) <1500 g and Gestational Age (GA) <32 weeks who were admitted to the NICU and received an eye examination, or were referred for a ROP eye examination as an outpatient, were included in the study. GA was estimated from intrauterine ultrasound findings. Neonates with major congenital malformations, syndromes or congenital cataracts or tumors of the eyes, and those that died before the eye examination or did not attend the out patients department for an eye examination, were excluded. The neonatal eye examination was performed by a trained ophthalmologist at 4 or 6 weeks of age. RESULTS: Out of 86 neonates, ROP was identified in nine neonates (10.5%) at the first eye examination. ROP was significantly associated with BW (P = 0.037), GA (P = 0.033), and chronological age (P<0.001). CONCLUSIONS: we identified ROP in 10.5% of neonates at first eye examination. Significant associations between ROP and a GA<32 weeks and a BW<1500 g were also observed.we also stress that serial follow-up of neonates at risk for ROP is important when making a final diagnosis.