Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study.

Introduction Budd-Chiari syndrome (BCS) is a rare cause of ascites in children, and its clinical manifestation depends upon the extent and rapidity of the occlusion of hepatic veins. This study aimed to identify the clinical manifestations, causes, treatment options, and outcomes of BCS in children. Materials and methods A retrospective descriptive study of BCS in children under 15 years of age was conducted. This study was approved by the Pakistan Kidney and Liver Institute and Research Centre on June 23, 2023, with approval number 0128. The patients' medical records from December 2020 to July 2023 were obtained from Sisoft Healthcare Information System. In this study, we employ a set of predetermined questions to retrieve relevant data retrospectively and then organise it in Excel spreadsheets. SPSS version 26 (Armonk, NY: IBM Corp.) was used to analyse the data. Categorical variables are shown as frequencies (%), while continuous variables are reported as mean±SD. Results Of 37 (n) patients diagnosed with BCS, 19 (51.35%) were male and 18 (48.65%) were female. The mean age of presentation was 9.8±4.1 years. Ascites are the predominant clinical manifestation (100%), followed by hepatomegaly (37.8%). A total of 45.9% of patients had deranged liver function tests. Chronic BCS is the predominant mode of presentation. Protein C deficiency was present in nine patients (24.3%), two patients (5.4%) had protein S deficiency and two patients (5.4%) had antithrombin III deficiency. Hepatic veins exhibited the highest incidence of obstruction (73.0%). Liver biopsies were done in 15 (40.54%) patients to determine the staging of fibrosis. Eight patients (21.62%) had undergone radiological interventions, two patients had liver transplants and the rest were treated with medications, including anticoagulants. Conclusion BCS can present in acute, subacute or chronic forms. Ascites and hepatomegaly should raise the suspicion of BCS in children. Common radiological findings are non-visualisation of the hepatic veins. BCS has a wide range of aetiologies and treatment options. Protein C deficiency is the most predominant procoagulant disorder. Radiological interventions during the acute and subacute forms of BCS usually have excellent results. Liver transplant remains the definite treatment.

The Intensity and Pattern of Syndecan-1 (CD138) Expression in Normal and Diseased Livers.

Introduction Heparan sulfate proteoglycans (HSPGs) belong to the syndecan family, and syndecan-1 (CD138) is a heparan sulfate proteoglycan. Syndecan-1 has a potential role in cell-matrix and cell-cell communications as they are present in cell epithelium. Its expression is different in an extensive range of benign, inflammatory, and neoplastic diseases. In routine histopathology, it is used as a marker for plasma cells. However, it is expressed in a large variety of normal and neoplastic epithelia including squamous epithelium and gastric glandular epithelium expressed in other tissues, i.e., the liver. In the liver, variable expression is seen in cirrhosis, hepatitis, and carcinoma. The objective of this study was to investigate the expression of this marker in normal, inflammatory, and neoplastic lesions of the liver. This in turn may help clinicians to select patients who may benefit from anti-CD138 therapy. It is currently used in the diagnosis and management of plasma cell proliferations. Material and methods This is a retrospective study in which we retrieved 53 formalin-fixed paraffin-embedded (FFPE) liver specimen blocks and selected one block from each case by reviewing the hematoxylin and eosin (H&E) slides of each case. Syndecan-1 (CD138), pancytokeratin, and CD68 expression were analyzed immunohistochemically (IHC) to evaluate the percentage and intensity of CD138 expression in various hepatic entities and identify those entities where syndecan-1 can be consistently used to make a definitive diagnosis. Results The expression of pancytokeratin and CD68 was analyzed in hepatocytes and Kupffer cells, respectively. For syndecan-1 (CD138), 15.4% of cases showed basolateral membranous positivity, 44.6% of cases showed complete membranous positivity, and 40% of cases showed no positivity in hepatocytes. Cytokeratin (CK) was positive as expected in hepatocytes, and CD68 was expressed in Kupffer cells. Conclusion CD138 does not appear to be a reliable surrogate marker for liver disease. However, it may be included with other ancillary markers as a predictor of the stage of chronic liver disease and metastatic potential. The response to anti-CD138 therapy needs to be further studied.

Managing Hepatocellular Carcinoma in Children.

Hepatocellular carcinoma (HCC) is a rare pediatric tumor. It differs from its adult counterpart in many ways like etiology, biological behavior, and association with cirrhosis. Treating HCC requires a multidisciplinary team involving pediatric gastroenterology, oncology, hepatobiliary surgery, and interventional radiology. This case series aims to describe presenting features and management plan of three children with HCC treated at a tertiary care liver transplant center in Pakistan.

Spectrum of chronic gastrointestinal diseases with final outcome in children at a tertiary care centre: A single-centre study.

Chronic gastrointestinal (GI) diseases in children present with a wide range of symptoms. Limited resources in Paediatric Gastroenterology in developing countries like Pakistan cause considerable difficulties in managing children with chronic GI diseases in a timely fashion. This retrospective descriptive study aimed to determine the spectrum and outcomes of chronic GI diseases in children. The study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute & Research Centre (PKLI &RC) in Lahore. The duration of the study was from August 2019 to August 2020. A total of 40 children below the age of 15 years with chronic GI diseases were included. The diagnosis was principally established with the assistance of esophagogastroduodenoscopy and ileo-colonoscopy. Coeliac disease was the most common chronic GI disease. Our systematic approach, in addition to an extensive workup, assisted in the diagnosis and management of the illness, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where facilities and expertise are available to decrease morbidity and mortality.

Outcome of Treatment in Children With Chronic Viral Hepatitis C: A Single Centre Study.

Objective To review the efficacy of the combination of pegylated interferon-α 2b and ribavirin, and sofosbuvir and ribavirin in achieving sustained viral response (SVR) in chronic hepatitis C genotypes 1 and 3 in children. Methods A retrospective descriptive study was performed for children under 15 years of age treated for chronic hepatitis C at Pakistan Kidney and Liver Institute and Research Centre, Lahore, between 2018 and 2019. Demographic data and clinical information were collected. In addition, treatment outcome was assessed by SVR, defined as the absence of detectable viral RNA in blood after 24 weeks of initiation of treatment. Results A total of 30 children aged 15 years and below were included in this study. Sixteen of 30 children were males, and 14 were females. Of these 30 patients, four had hepatitis C virus (HCV) genotype 1, and 26 children had HCV genotype 3. The children with genotype 1 (a+b) were given the combination of ribavirin and pegylated interferon alfa-2b (Peg-IFN-α-2b). The remaining with HCV genotype 3 were given the combination of ribavirin and sofosbuvir for 24 weeks. Overall, 27 out of 30 (90%) children attained SVR at six months (100% children with genotype 1 and 88.4% children with genotype 3). Conclusion The combined therapy of ribavirin and sofosbuvir or Peg-IFN-α-2b and ribavirin is highly effective in treating chronic HCV infection in children.

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study.

BACKGROUND AND OBJECTIVES: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. METHODS: This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined. RESULTS: Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing. CONCLUSION: Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality.

Is It Tuberculosis Or Crohn's Disease?

Tuberculosis is very prevalent in countries like Pakistan. It can be a diagnostic challenge when it has extra pulmonary organ involvement. Variation in clinical signs and symptoms, lack of access to a specialist and limited financial resources often result in delayed diagnosis. This leads to an increase in overall morbidity and mortality. We are presenting a case of disseminated tuberculosis with predominant intestinal symptoms in an eleven years old boy. He remained undiagnosed for several years despite frequent visits to multiple general practitioners. After appropriate investigations and initiation of treatment for tuberculosis, he showed an excellent recovery.

Diagnosing Primary Sclerosing Cholangitis in Children.

Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease in children caused by chronic inflammatory process affecting either intrahepatic or extrahepatic bile ducts. Although it is infrequent, incidence is increasing worldwide, may be due to more awareness, understanding and expertise in managing children with chronic liver disease (CLD). In the developing world like Pakistan where resources and expertise are limited, very few tertiary centers are equipped to manage CLD in children. This case report is about a teenage child who presented to us with decompensated CLD in the form of jaundice and ascites. In our center, after a much needed workup he was commenced on appropriate treatment for PSC. After six months of treatment, he has managed to clear jaundice. The liver synthetic functions have improved with normal coagulation profile. His MELD (model for end-stage liver disease) score, which has come down from 19 to 9, delays liver transplant (LT) for years, which remains the best available treatment. MELD is a scoring system to assess the severity of CLD and remains an important tool to determine the outcome and ranking for receipt of an LT. Subsequently, he developed colitis and colonoscopy confirmed lymphocytic colitis (LC), which is a rare association of PSC.

Frequency and Associated Risk Factors of Hepatitis B Virus and Hepatitis C Virus Infections in Children at a Hepatitis Prevention and Treatment Clinic in Lahore, Pakistan.

Objective This study evaluated the frequency of hepatitis B virus (HBV) and hepatitis C virus (HCV) infection and the associated horizontal risk factors in children being screened for viral hepatitis in Lahore, Pakistan. Methods Children aged 15 years or younger who were brought to a specialized outpatient viral hepatitis clinic affiliated with a tertiary hospital in Lahore, Pakistan, for viral hepatitis screening from March 2017 to March 2018 were enrolled. Children were screened for HBV and HCV infection by enzyme-linked immunosorbent assay; if results were positive, HBV and HCV concentrations were quantitatively assayed by polymerase chain reaction. Children positive for HBV or HCV infection were matched with 100 controls of the same age and sex. All subjects completed a questionnaire on viral infection and its associated risk factors. Results During the study period, 3500 children living in the Punjab Province of Pakistan were screened for HBV and HCV infection. Of these children, 28 (0.8%) were positive for HBV and 66 (1.88%) were positive for HCV. A comparison of the 94 (2.68%) children positive for HBV or HCV with 100 controls identified several risk factors associated with infection. Unexpectedly, ten (35.7%) of the 28 HBV-positive children were born of HBV-negative mothers and had been fully vaccinated for HBV during infancy. Conclusion The frequency of HCV infection was higher than that of HBV infection among Pakistani children aged ≤15 years. Several horizontal risk factors were found to cause viral hepatitis. Several children born of HBV-negative mothers and vaccinated for HBV during infancy later developed HBV infection.

Could It Be Schistosomiasis?

Schistosomiasis may cause diverse symptoms and it is usually not considered as a cause of disease especially in patients with normal immune system. We are reporting an eleven-year-old child who was initially diagnosed as a case of lymphoma but later on proved to be a case of acute schistosomiasis.