Detection of Cytomegalovirus in Liver Tissue by Polymerase Chain Reaction in Infants With Neonatal Cholestasis.

AIM: Cytomegalovirus (CMV) is associated with neonatal cholestasis (NC). Diagnosis of CMV infection is most often based on either positive blood CMV IgM or CMV blood polymerase chain reaction (PCR). Isolation of CMV in liver tissues in patients with NC has rarely been reported. This study was undertaken to see if CMV is present in liver tissues of patients with NC and evaluate the correlation between positive CMV PCR in liver tissue with the serology and blood PCR. METHODS: This study was conducted in 31 infants with NC from June 2015 to December 2016. All patients underwent blood CMV IgM, blood CMV PCR and liver CMV PCR. Prevalence of CMV in NC based on positive liver CMV PCR was calculated. Sensitivity and specificity of the serologic markers and blood CMV PCR to identify CMV infection in the liver was determined. RESULTS: CMV IgM was positive in 13 (42%) patients, CMV IgG was positive in 26 (84%) patients and blood CMV PCR was positive in 23 (74%) patients. Liver CMV PCR was positive in 16 (52%) patients. Fifteen (48%) patients had biliary atresia (BA), 10 (32%) patients had neonatal hepatitis, 5 (16%) had paucity of bile ducts and 1 (3%) had ascending cholangitis. Of the 16 patients with positive liver CMV PCR, 8 (50%) had BA, 4 (25%) had neonatal hepatitis, 3 (19%) had paucity of bile ducts and 1 (6%) had ascending cholangitis. Sensitivity of blood CMV IgM in relation to liver CMV PCR was 69% and specificity was 61%. Sensitivity of blood CMV PCR was 61% and specificity was 71% when compared with liver CMV PCR. CONCLUSION: CMV is present in the liver tissues of more than half the patients with NC. Serology or blood CMV PCR is apparently not an accurate marker of CMV in the liver tissue. Also, CMV infection in children seems to be associated equally with BA or non-BA neonatal hepatitis.

Management of Congenital Diaphragmatic Hernia in Newborn - Paradigm Shift and Ethical Issues.

Management of congenital diaphragmatic hernia (CDH) begins soon after it is detected, whether antenatally or postnatally. Assessment of the severity of the condition, associated congenital anomalies, maternal health and related issues, weight of the fetus/baby, mode of delivery, timing of delivery, immediate appropriate management of the baby with CDH at birth, appropriate utilization of available treatment modalities as well as infrastructure of the treating institute have an impact on the outcome of the neonate. Survival without significant long-term/permanent morbidity is considered as good outcome. With advances in antenatal diagnosis, several legal and ethical considerations have cropped up. While on one hand there are proponents of early antenatal diagnosis and medical termination of pregnancy (MTP), on the other hand there are several socio-cultural groups who look upon human life as precious and argue against MTP. There is an ongoing ethical battle between maternal vs. fetal rights; there is no way to put a lid on the controversy whether the mother be allowed to choose in favor of MTP after being aware of the anomalous fetus or, we must attempt to save every fetus irrespective of the antenatal diagnosis of life-threatening anomalies. Notwithstanding, appropriate assessment of the condition, thorough counseling and sound evidence-based decisions could avert ethical dilemma in most cases. This review article provides information about the various choices available in the diagnostic and treatment armamentarium, though it should be kept in mind that the entire spectrum of management strategies may not be universally available.

Hemophagocytic Lymphohistiocytosis (HLH) in Children Presenting as Liver Disease.

Hemophagocytic lymphohistiocytosis (HLH) is a rare acute hyperinflammatory condition presenting with high fever, pancytopenia, splenomegaly with the pathologic finding of hemophagocytic lymphohistiocytosis in bone marrow and other tissues. Predominant hepatic manifestations at presentation are rare. We report a series of three cases which showcase the spectrum of liver disease as presentation in hemophagocytic lymphohistiocytosis.

Liver Abscesses and Hyper IgM Syndrome.

Hyper IgM (HIGM) syndrome is an immunodeficiency that can lead to liver disease in more than 80% of affected males by an age of 20 years. Hepatitis, sclerosing cholangitis, and hepatocellular malignancies are common among them. We encountered two cases in children of less than 12 years who presented with typical manifestations of liver abscess and were later detected to have a concomitant underlying HIGM syndrome.

Pyloric Atresia Type II.

Successful management of a neonate with type II pyloric atresia is reported and the relevant literature has been briefly reviewed.

Biliary atresia and cytomegalovirus and response to valganciclovir.

Biliary atresia has been commonly reported with cytomegalovirus (CMV) infection. CMV positive patients may present with a later onset however long term outcome is similar to non-CMV patients. There are very few case reports of role of antivirals in CMV and biliary atresia. We treated a 2 month old girl with biliary atresia who underwent portoduodenostomy at 2 and half months of age but continued to have jaundice (bilirubin = 23.6 mg/dl) even after 1 month of Kasai's surgery and subsequently was treated with valganciclovir for 6 weeks following which her jaundice resolved.

Utility of Tc99m-Mebrofenin hepato-biliary scintigraphy (HIDA scan) for the diagnosis of biliary atresia.

AIM: To determine the utility of Tc99m-Mebrofenin hepato-biliary scintigraphy (HIDA scan) for diagnosis of biliary atresia in patients with neonatal cholestasis. METHODS: Our study involves the retrospective analysis of 46 patients with neonatal cholestasis who underwent HIDA scans at the Pediatric Hepatobiliary Clinic, BJ Wadia Hospital for Children from May 2005 to July 2007. Biliary atresia (BA) was diagnosed on the basis of intra-operative cholangiogram. Non-BA patients were included in the neonatal hepatitis (NH) group. All patients received phenobarbitone and ursodeoxycholic acid for 5 days, prior to the HIDA scan. The HIDA scan was evaluated on the basis of uptake of the radioactive tracer by the liver at 5 minutes after intravenous injection; retention of radioactive tracer within the liver at 24 hours after injection and visualization of excretion of tracer into the intestine upto 24 hours after administration. The results of the HIDA scans were analyzed and correlated with the final diagnosis, gender and age of the patients. Chi-square test was employed for statistical analysis. RESULTS: The age of presentation of our patients ranged from 5 days to 6 months. The male: female ratio was 37:9. Of the total 46 patients, 28 had BA and 18 had NH. All 28 (100%) patients diagnosed with BA showed persistent radiotracer in the liver at 24 hours whereas 17 (94.4%) of the 18 NH patients showed hepatic radiotracer retention (p = 0.207), the difference being statistically insignificant. Twenty two (78.6%) patients of BA showed no excretion of the radiotracer at 24 hours whereas only 7 (38.9%) of the NH group did not excrete the radiotracer (p = 0.007), which was statistically significant. Neither the sex nor the age of the child contributed to any difference on the hepatic retention (p = 0.618 and 0.235, respectively) or on the intestinal excretion (p = 0.307 and 0.9, respectively) of the radiotracer. CONCLUSION: HIDA scan is a useful tool for screening of biliary atresia in patients with neonatal cholestasis. Non excretion of the radioactive radiotracer into the intestines even after 24 hours of radiotracer administration can suggest biliary atresia in majority of patients.

Extracranial malignant germ cell tumors.

The outstanding clinical trials undertaken for the management of pediatric extracranial malignant germ cell tumors (GCTs) in the developed world in the last three decades has led to excellent longterm outcomes. The scenario in developing country like India is different; results are poor owing to multiple factors such as delayed presentation, misdiagnosis, and early abandonment of the therapy. The authors address here several aspects of this heterogeneous group of malignant tumors in children and adolescents such as the different staging systems, the risk stratification, the guidelines to treatment modalities, the outcome and prognostication.

Wilms' tumor- roadmaps of management.

The management of Wilms' tumor emerging of the outstanding clinical trials undertaken in the developed world in the last four decades has led to excellent longterm outcomes. The scenario in developing country like India is different; late presentation with massive tumors and advanced stage, lacunae in staging, nonavailability of pediatric medical oncologists and poor follow-ups are common. A comprehensive summary of available therapeutic modalities is provided here along with clear roadmaps of management of Wilms' tumor as per Société Internationale d'Oncologie Pédiatrique (SIOP) and National Wilms' Tumor Study Group/Children's Oncology Group (NWTSG/COG) protocols in simple tabulated form.

Neuroblastoma: a review of management and outcome.

Solid tumors in children are a major cause of death in the developed countries and now even in the developing countries. Of these tumors, neuroblastoma, the most common tumor in children, despite extensive and on-going research and clinical trials still remains an enigma. About 50 % of children with neuroblastoma overall succumb to the disease. This tumor generates lot of curiosity in developing newer therapies for management, but creates equal amount of frustration albeit a risk-stratification system, patients with the same clinical-pathologic parameters and being treated with the same protocols may have markedly different clinical courses and outcomes. Most of the neuroblastomas are sporadic but some are familial. This article aims at understanding the different protocols existing for the risk stratification and management of neuroblastomas. Further, it also aims to study the outcomes of the several different stages of the tumor all across the country as well as in India.

Proceedings of tumor board meeting.

The Tumour Board Meeting was held on August 16, 2011, in the Seminar Hall at B.J. Wadia Hospital for children. The panelists of the meeting were Dr. S. Ranganathan, Pediatric Pathologist from Children's Hospital of Pittsburgh; Dr. Archana Swami, Consultant Pediatric Oncologist at Wadia Children's Hospital; Dr. Sajid Qureshi Onco-surgeon (Pediatric) at Tata Memorial Hospital and Dr. Sushmita Bhatnagar.