Wilson disease is an autosomal recessive disorder of copper metabolism that is rarely reported among Filipinos. Four children with Wilson disease presenting with various hepatic manifestations, namely, an asymptomatic elevation of transaminase levels, prolonged jaundice and acute liver failure are presented. The diagnosis was based on a combination of clinical and biochemical findings. Early recognition and management is important as effective treatment could reverse the damage caused by copper toxicity.

Human ; Female ; Child ; Child Preschool ; Hepatolenticular Degeneration ; Copper ; Liver Failure, Acute ; Jaundice ; Transaminases

BACKGROUND: The Pediatric Quality of Life (PedsQL4.0TM) scale has been shown to be reliable and valid in assessing health related quality of life (HRQOL). However, its Tagalog version has not been field-tested.

OBJECTIVES: To determine the reliability and validity of the PedsQL4.0TM Tagalog version and to compare the HRQOL of Filipino liver transplant (LT) recipients, children with chronic liver disease (CLD) and healthy controls.

METHODOLOGY: This is a cross-sectional study that included Filipinos 2-18 years who had undergone LT and those with CLD. PedsQL4.0TM Tagalog version was administered as a proxy-report for caregivers and child-report for children >5 years.

RESULTS: 237 PedsQL4.0 questionnaires were completed. Reliability was demonstrated for psychosocial (Cronbach =0.86-0.88), physical ( =0.86-0.88) and total ( =0.89-0.92) health summary scores. Construct validity showed a medium to large effect size (0.39-1.34) between patients and controls. No difference was noted on the total health summary scores and the individual domains between LT recipients and controls while the scores of patients with CLD were significantly lower compared to LT recipients and healthy subjects.

CONCLUSIONS: The PedsQL4.0TM Tagalog version is a valid and reliable HRQOL tool. The HRQOL of LT recipients is similar to healthy children while CLD patients had poorer HRQOL.

Human ; Male ; Female ; Adolescent ; Child ; Child Preschool ; Caregivers ; Quality Of Life ; Liver Transplantation ; Healthy Volunteers ; Reproducibility Of Results ; Liver Diseases

We report seven adolescents presenting with hematochezia. Five had a family history of colonic polyps or cancer. Colonoscopy showed either multiple pedunculated and/or sessile polyps with partial or total colonic involvement. Polyphistopathology was of the juvenile retention, hamartomatous or tubular adenomatous type. Total proctocolectomy was done in four patients, two underwent repeated polypectomy, and one is awaiting further treatment. An increased awareness of colonic polyposis is important due to the risk of malignant transformation.

Human ; Male ; Female ; Adolescent ; Child ; Adolescent ; Colonic Polyps ; Proctocolectomy, Restorative ; Colonoscopy ; Colon ; Gastrointestinal Hemorrhage ; Adenoma

Solid cystic pseudopapillary tumor (SCPT) of the pancreas is rare and is difficult to diagnose pre-operatively. We describe four children with abdominal pain and abdominal mass who were diagnosed with SCPT. Three underwent resection of the mass, one an open liver biopsy. Histopathology showed solid epithelioid cells, cystic areas, and pseudopapillary structures. SCPT is a low-grade malignant tumor with good prognosis and should be suspected in any child with an abdominal mass and abdominal pain.

Key Words: Solid cystic pseudopapillary tumor, pancreas

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

OBJECTIVE: To determine the prevalence of and factors associated with severe mucosal injury on esophagogastroduodenoscopy (EGD) after caustic ingestion. METHODS: Consecutive patients ?19 years old with history of caustic ingestion were included. Factors considered were age, sex, caustic agent (acid or alkali) and presence of signs/symptoms (oral lesions, drooling, vomiting, abdominal pain, dysphagia).  RESULTS: 320 patients were investigated: 155 (48%) accidental and 165 (52%) intentional case. In accidental intake, majority (84%) were ?6 years old [mean(SD) age: 3.7 (4.3) yrs, 59% males]. 10% had severe mucosal injury. The odds of severe injury increased in the presence of ?2 signs/symptoms: OR=7.0 for 2 and OR=62.2 for >2. In intentional cases, the mean (SD) age was 16 (1.6) years, 74% females. Severe mucosal injury was seen in 5% and associated with acidic agent (OR=54.8). CONCLUSIONS: Severe mucosal injury on EGD occured in 10% and 5% among accidental and intentional cases, respectively. In accidental cases, probability of severe injury increased in the presence of ?2 signs/symptoms. In intentional cases, acid intake was the only factor associated with severe injury. In the local setting, presence of these factors among patients with caustic ingestion will help identify high-risk patients who need EGD-guided management.
Human ; Male ; Female ; Adolescent ; Child ; Child Preschool ; Caustics ; Alkalies ; Deglutition Disorders ; Sialorrhea ; Endoscopy, Digestive System ; Abdominal Pain ; Vomiting ; Probability

BACKGROUND: Pancreatitis is uncommon in childhood and there is presently no study among Filipino children.

OBJECTIVE: To determine the clinical features and outcome of pancreatitis among Filipino children.

METHOD: Review of medical records of all patients diagnosed to have pancreatitis based on standard criteria from 2005 to 2009.

RESULTS: A total of 23 children (mean age: 12 years; 13 male, 10 female) were included, 21 with acute and two with chronic pancreatitis. Twenty one (91%) presented with abdominal pain and two with jaundice. Nine had idiopathic pancreatitis. In 14 patients, the etiology was identified: bile duct obstruction (7), trauma (2), drugs (2), infection (2) and hypertriglyceridemia (1). Only four of 20 patients with ultrasound examination showed an enlarged pancreas. Complications were pseudocyst formation (6), pancreatic abscess (4), diabetes mellitus (2) and hypocalcemia (1). Of the 23 patients, eight required surgery: pancreatic debridement (4), choledochal cyst excision (2), cholecystectomy (1) and Whipple's procedure (1). All pseudocyst resolved spontaneously. One patient with pancreatic tumor declined surgery and another with pancreatitis due to choledochal cyst died of sepsis.

CONCLUSIONS: In our study, severe abdominal pain was the most frequent presenting symptom of childhood pancreatitis. Sixty percent had an identifiable cause for pancreatitis. A favorable outcome was observed.

Human ; Male ; Female ; Adolescent ; Child ; Child Preschool ; Pancreatitis, Chronic ; Choledochal Cyst ; Abscess ; Hypocalcemia ; Pancreatic Cyst ; Pancreatic Pseudocyst ; Pancreas ; Abdominal Pain ; Jaundice ; Cholestasis ; Hypertriglyceridemia ; Cholecystectomy ; Diabetes Mellitus

BACKGROUND: Histological assessment is important in evaluating liver disease. We determined the clinical diagnose and predominant histological patterns of children with liver disease and association of histological pattern with outcome.

METHODS: Consecutive patients

RESULT: 470 cases (1month-18years; 65% males; 85% <1year) were included. Most common clinical diagnose were neonatal hepatitis (49%) and biliary atresia (29%). 229 neonatal hepatitis with giant cell pattern had better outcome (96% alive after 1year vs. 136 biliary with obstructive/fibrotic pattern of whom 74% died, p=<0.05). In the other disorders namely alagille's syndrome, bile duct abnormalities, liver tumor and both chronic hepatitis and portal vein thrombosis, the predominant histologies were bile duct paucity ,fibrotic, neoplastic and near normal patterns, respectively. In these cases association with outcome could not be done due to limited patients.

CONCLUSION: Most common clinical diagnoses were neonatal hepatitis and biliary atresia. Predominant histological patterns were giant cell and obstructive type. Histological patterns of giant cell hepatitis seen in neonatal hepatitis resulted in better outcome.

Human ; Hepatitis ; Biliary Atresia ; Hepatitis ; Liver Diseases

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