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

Jaundice in older children can occur when any obstruction is found within the bile ducts, either from bile stones, parasites, choledochal cysts and rarely, secondary to tumors. We present a previously well, 10-year-old Filipino boy with three-week history of progressive jaundice and tea-colored urine, and was initially assessed to have biliary ascariasis. Ultrasound showed a heterogeneous focus spanning the gallbladder neck and confirmed on endoscopic retrograde cholangiopancreatography as an exophytic mass at the ampulla of Vater. Tumor biopsy and immunohistochemical staining confirmed the diagnosis of Embryonal Rhabdomyosarcoma of the Bile Ducts. Chemotherapy was initiated to reduce the size of the tumor before any surgical intervention could be attempted. Despite chemotherapy, the tumor progressively grew. He acquired a respiratory infection which led to sepsis and his eventual demise. The disease should be considered in the differential diagnosis of a child with obstructive jaundice after exclusion of other more common causes.

: Schistosomiasis, a snail-borne disease caused by infection with a trematode parasite of the genus Schistosoma, is one of the most neglected tropical diseases in the world. One of its rare complications is hepatosplenic schistosomiasis which ultimately leads to fibrosis and presinusoidal portal hypertension. : We report a case of a 13-year-old Filipino male from Quezon City with previous one year residence in the endemic island of Leyte, presenting with melena. Diagnostic work-up revealed hepatosplenomegaly and periportal fibrosis with multiple hepatic nodules on ultrasound, positive Schistosoma japonicum eggs on Kato-Katz stool examination technique, and findings of esophageal varices on upper endoscopy. The patient was managed with praziquantel, propranolol, and endoscopic rubber band ligation of the esophageal varices, with note of resolution of bleeding, and improvement on sonographic liver findings. The degree of liver fibrosis from schistosomiasis is affected by poorly understood mechanisms which affect its severity, progression, and complications, regardless of biosocial factors including egg burden and duration of parasite exposure. This is the first case report on a Filipino adolescent to document significant interval improvement, within four weeks of treatment, of the characteristic fibrotic pattern in hepatosplenic schistosomiasis. Hepatosplenic schistosomiasis is still often missed out as the diagnosis in patients who consult with common symptoms, and high index of suspicion is recommended for those with history of residence in endemic areas. Likewise, treatment focusing on parasite eradication can aid in promptly addressing the resulting fibrosis and its complications.
fibrosis ; hypertension, portal

BACKGROUND

Peritoneal tuberculosis comprises 5% of all forms of tuberculosis in children. There are limited reports of peritoneal TB in children.

To determine the clinical, biochemical, radiologic, histologic, and microbiologic features and outcome of pediatric patients diagnosed with peritoneal tuberculosis.

Review of medical records from 2011-2020 of patients fulfilling diagnostic criteria of peritoneal TB. Patient was considered as bacteriologically-confirmed if with positive AFB smear, culture, or PCR on peritoneal or omental tissue; and clinically-diagnosed if with clinical findings AND presence of histologic and/or radiologic evidence of extra-pulmonary TB. Data was presented as mean (SD) or n (%), as appropriate.

Eighteen patients [Mean (SD) age: 14.27 (± 4.1) years old, 56% males] were included. All had disseminated TB with peritoneal involvement. One case was bacteriologically-confirmed (TB PCR positive omental tissue); 17 were clinically-diagnosed. Most common presentation was abdominal distention (83%) and abdominal pain (61%). Most common physical finding was abdominal distention (83%) and abdominal tenderness (44%). Seven patients (39%) had anemia, 11 (61%) had leukocytosis, and three (17%) had thrombocytosis. Thirteen (72%) had hypoalbuminemia. Ten (56%) were positive on AFB smear, TB culture, and PCR of various specimens. Fourteen of sixteen (88%) with abdominal CT scan had ascites and intrabdominal lymphadenopathy. Nine of 12 tissue samples from seven patients demonstrated chronic granulomatous inflammation. Seventeen were given quadruple anti-TB. Six also had surgery. Overall, 15 were discharge improved after mean of 4.2 weeks of hospital stay, while three died of sepsis. Eleven of the 15 were well one month after discharge.

Peritoneal TB presents with non-specific clinical and laboratory features. Radiologic and histologic findings increase the likelihood of diagnosis. The prognosis is favorable for patients who are diagnosed and treated with anti-TB drugs.

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.

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 ;