Systemic Lupus Erythematosus (SLE) is a multi-systemic autoimmune disorder. Fifty percent will have gastro- intestinal symptoms and 1 to 4% have jaundice.

Two Filipino adolescents with uncommon presentation of SLE are reported: a 14-year old female with intermittent epigastric pain and a 17-year-old male with jaundice. Epigastric pain and jaundice are uncommon presentations in patients with SLE. A high index of suspicion is needed and SLE must be considered in the presence of other clinical and immunologic features.

Key Words: lupus, jaundice, epigastric pain

Background: Acute cholangitis (AC) in children is a rare but life-threatening infection. Symptoms vary from mild to severe disease. There are no local published data on pediatric AC. Objective: To determine the clinical, biochemical, ultrasonographic, microbiologic features, and treatment outcome of pediatric patients with definite AC. Methodology: Cross-sectional study using medical records of pediatric patients diagnosed with definite AC based on the Modified Tokyo Guidelines of 2018 admitted from January 2016 to June 2021. Results: Twenty-seven patients aged 0 to 18 years old (10.06 + 7.34), predominantly male (51.85%) were included. Choledocholithiasis (22%) and post-Kasai biliary atresia (22%) were the common underlying biliary conditions. Fever (88.89%) was the most frequent presenting symptom. Majority were classified as moderate AC (40.74%). Leukocytosis (mean 16×109/L), elevated inflammatory markers (93.33% with CRP >12mg/L and 100% with serum procalcitonin >0.25ng/mL), hyperbilirubinemia (total bilirubin 192.54±126.87umol/L) and elevated alanine transferases (mean 59 IU/L) were noted. Twenty-one out of 27 cases (87%) had a negative blood culture. Only 4 patients underwent bile culture, of which two (50%) grew Klebsiella pneumoniae resistant to empiric antibiotics. Dilated biliary ducts were observed on abdominal ultrasound in 92.59% of patients. Ampicillin-sulbactam (29.63%) was the most commonly utilized antibiotic. Discharge rate was high (88.89%). Conclusions AC affects all pediatric age groups but clinical presentations vary. Drug resistant organisms are a significant concern but despite this, favorable outcomes have been documented.
Cholangitis ; Child ; Choledocholithiasis

We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.
Butterflies ; Bile Ducts, Intrahepatic ; Jaundice ; Musculoskeletal Abnormalities ; Spine

A 12-year-old female had a three-year history of fever, non-bilious vomiting and abdominal pain. Upper gastrointestinal series showed a filling defect at the duodenum. Esophagogastroduodenoscopy exhibited circumferential mass extending from the duodenal bulb to the 2nd part of the duodenum which on histology disclosed chronic granulomatous inflammation. Chest X-ray suggested miliary tuberculosis; endotracheal tube aspirate was PCR positive for Mycobacterium tuberculosis. Patient was diagnosed as disseminated tuberculosis of the duodenum and lungs. Quadruple anti-tuberculosis medication was started but patient succumbed to nosocomial sepsis.
Tuberculosis, Miliary ; Gastric Outlet Obstruction ; Granuloma ; Inflammation

Objective. To determine factors predictive of obstructive neonatal cholestasis among Filipino infants and to describe their outcome.

Methods. Jaundiced infants within the first eight weeks of life with liver biopsy were included. Excluded were cholestasis secondary to metabolic or infective causes. Retrospective chart review (2009-2012) and prospective recruitment of patients (2013) were done. A final diagnosis of non-obstructive or obstructive neonatal cholestasis was made on clinical, biochemical, ultrasonographic, and histologic findings, using histology and/or operative cholangiogram as the gold standard. The outcome was assessed on the 6th and 12th months from diagnosis. The crude odds ratio for obstructive jaundice was computed. Multiple logistic regression on significant variables (p-value <0.05) was done.

Results. Two hundred sixty-three (263) patients were included: 161 with non-obstructive and 102 with obstructive cause. Mean age at first consult was higher in those with obstruction. On logistic regression, females (OR:2.3), absence of a family history of idiopathic neonatal hepatitis (OR:4), and persistently pale/acholic stools (OR:13) were predictive of obstruction. 85% of patients with a non-obstructive cause are alive and well, while 80% of patients with obstruction have died.

Conclusion. Among jaundiced infants females, the absence of a family history of idiopathic neonatal hepatitis and persistently pale yellow/acholic stools were predictive of obstruction. The outcome was poor in patients with obstructive jaundice.

Objective. Hepatic abscess is a rare childhood infection with incidence of 1 to 140 per 105 admissions. It has signs and symptoms that mimic other liver diseases. This study determined the clinical, biochemical, microbiologic and imaging features and outcome of children diagnosed with hepatic abscess at a tertiary hospital in Manila.

Method. We conducted a review of medical records of admitted patients aged ?18 years diagnosed with hepatic abscess in a tertiary referral center from 2007-2018. A diagnosis was confirmed if with (1) fever; (2) imaging study of solitary or multiple hepatic focus; AND (3) at least one more sign or symptom. We computed for mean (SD) for continuous variables or n (%) for categorical variables.

Result. We included 40 patients out of 559, 583 pediatric admissions during the study period (0.007%), with a mean age of 8 years (SD 6.5), and 57% of whom were males. Thirty-seven (92%) were probable pyogenic in etiology, while three were tuberculous abscess. The most common signs and symptoms were fever (100%), abdominal mass (31%), abdominal distension (34%) and weight loss (31%). Majority had anemia (63%) and leukocytosis (89%). The most common imaging finding was a solitary mass (58%) with right lobe (80%) involvement. Nine abscess aspirates yielded Staphylococcus aureus (3), Mycobacterium tuberculosis (3), Klebsiella pneumoniae (1) and no growth (2). All patients were medically treated but eight also required percutaneous/surgical drainage. Majority (36) were discharged improved; four were improving but left before treatment completion.

Conclusion. Majority had pyogenic hepatic abscess, presenting with non-specific clinical and laboratory features. Most abscess were solitary and involved the right lobe. Limited abscess aspirates yielded Staphylococcus aureus and Mycobacterium tuberculosis. Prognosis is favorable with treatment.

There is conflicting evidence on the efficacy of intravenous immunoglobin G in the treatment of COVID-19 patients with severe disease. Intravenous immunoglobulin G (IVIg) is a mixture of polyclonal immunoglobulin G (IgG3, IgG4) antibodies as well as variable amounts of proteins; IgA, IgE and IgM antibodies isolated and pooled from healthy donors. IgG is involved in viral neutralization, modulates anti-inflammatory cytokines and cytokine antagonists. Immediate adverse effects of IVIg include flu-like syndrome, dermatologic side effects, arrhythmia, hypotension, and transfusion-related acute lung injury (TRALI). Delayed adverse effects can involve any organ which could be severe or even lethal There was a retrospective study (Yun Xie 2020) and several case reports that described recovery of COVID positive patients with severe disease. However, a retrospective study showed that immunoglobulin G with steroids and antivirals did not improve COVID patients with acute respiratory distress syndrome (Liu Y 2020). Similarly, another study showed no significant difference in the 28- and 60-day mortality between the IVIg and non-IVIg groups but subgroup analyses reported that in those with critical COVID illness, 28 day mortality is decreased with IVIg (Shao Z). There are eight registered clinical trials on the use of intravenous immunoglobulin G in COVID-19 patients.
Covid-19

Objective: We investigated the effect of personal protective equipment (PPE) on prevention of COVID-19 on health care workers (HCW) assigned in the wards, intensive care (ICU) and emergency room (ER). Methods: We searched MEDLINE, Cochrane CENTRAL as of 30 April 2021, as well as trial registers, preprint sites and COVID-19 living evidence sites. We included studies that compared use of PPE versus no use in the prevention of COVID-19. We screened studies, extracted data, assessed risk of bias and certainty of evidence using GRADE approach. Results: Five observational studies (three cohort and two case control) were found. There was moderate certainty of evidence that the use of Level 2 PPE (OR 0.03 [95% CI 0, 0.19]; 1 study, n = 5542) was protective for HCW. Level 2 PPE used N95 or higher standard respirators, goggles/protective mask, medical protective clothing and disposable hats, gloves and shoe covers. We also confirmed with moderate certainty evidence the protective use of N95 respirators (OR 0.035 [95% CI 0.002, 0.603]; 1 study, n = 493). There was very low certainty of evidence that demonstrated the protective effect of face shield (OR 0.338 [95% CI 0.272, 0.420]; 2 studies, n = 6717, I2 = 45% P < 0.00001). Very low certainty of evidence showed no significance difference with use of face/surgical mask (OR 1.40 [95% CI 0.30, 6.42]; 1 study, n = 186), gowns (OR 0.768 [95% 0.314, 1.876]; 1 study, n = 179) and disposable gloves (OR 0.62 [95% CI 0.13, 2.90]; 1 study, n = 179) when attending to patients with COVID-19. Conclusion There was lower odds of COVID-19 infection in HCW assigned to the wards, ICU and ER with possible direct contact with COVID-19 patients who wear Level 2 PPE including N95 respirators and face shields.
Personal Protective Equipment ; SARS-CoV-2 ; Pandemics

Background: Human Pegivirus (HPgV), previously called Hepatitis G virus or GB virus C, is an RNA virus. It can be transmitted vertically (mother to infant), parenterally and sexually. HPgV share common routes of transmission to other viruses such as Hepatitis B virus, Hepatitis C virus and Human Immunodeficiency virus (HIV) thus co-infection is usually observed. Risk groups of HPgV include injection drug users, HIV-positive individuals, multi-transfused patients, hemodialysis patients, hemophiliacs, chronic liver disease patients and organ transplant recipients. The clinical significance of HPgV is not yet established and warrants further studies. Research on HPgV in the Philippines is scarce and has not been updated for over 10 years. There is no published data on HPgV prevalence in Filipino pediatric population specifically among risk groups like multi-transfused children with decompensated liver disease secondary to biliary cirrhosis and liver transplant pediatric patients. The lack of local data warrants conduct of this study. Objective: To determine the presence of HPgV RNA, HPgV E2 antibody (anti-E2) and HBsAg among Filipino children with decompensated liver disease secondary to biliary cirrhosis (DBC) and liver transplant pediatric patients (LTP). Methods: Included were 15 children with DBC and 15 LTP recruited from the Section of Pediatric Gastroenterology, Hepatology and Nutrition of the UP PGH. All patients’ sera were tested for HPgV RNA by Real Time RT-PCR, HPgV anti-E2 by Enzyme-linked Immunosorbent Assay (ELISA) and hepatitis B surface antigen (HBsAg) by immunochromatographic test. Twenty age and sex matched children with no history of liver disease and blood transfusion served as controls. Results: All patient and control samples were negative for HPgV RNA. HPgV anti-E2 was detected in 6 of 15 LTP, 5 of 15 DBC and 1 of 20 controls. HBsAg was detected in 2 of 15 LTP, 5 of 15 DBC and 0 of 20 controls. Four patients (two LTP, two DBC) were positive for both HPgV anti-E2 and HBsAg. Conclusion This study showed that a proportion of liver transplant patients and those with decompensated biliary cirrhosis are positive for HPgV anti-E2, which indicates that these individuals previously had HPgV infection but is now resolved. Possible source of infection is infected blood from the blood transfusions, infected transplant organ or infected mother. Since routine HPgV screening is not yet recommended for the general population, blood donors and organ donors, the confirmation of exact source of infection may be difficult. Co-infection with HBsAg was also observed in both risk groups which suggests that at some point in time, these children were infected by both HPgV and HBV and also the possibility of simultaneous infection by the two viruses. This study provides preliminary data on the proportion of HPgV infection in Filipino children belonging to two of the HPgV risk groups. Studies with a larger and more significant sample size to determine HPgV prevalence as well as studies regarding the pathogenicity of HPgV are warranted. As this may provide basis for routine HPgV screening among risk groups and blood donations in the future.
GB virus C

We present a 15-year-old male with a two-year history of gynecomastia and a four-month history of gradually enlarging abdomen and right flank pain. Examination revealed severe stunting with breast mass Tanner Stage 3, penile stage 2, and hepatosplenomegaly. Laboratory investigations showed normal blood counts, liver function tests, alpha-fetoprotein, and beta-human chorionic gonadotropin. The imaging findings demonstrated multiple confluent masses in the liver, histologically diagnosed as fibrolamellar hepatocellular carcinoma.
gynecomastia ; fibrolamellar hepatocellular carcinoma ;