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.

Paradoxical response to anti-tuberculosis treatment, defined as clinical or radiologic worsening of pre-existing lesions or the development of new lesions while ongoing treatment, poses diagnostic dilemma. Intestinal perforation as a paradoxical response is rare. We report a 10-year-old female who presented with recurrent abdominal pain, anorexia, and bloody diarrhea, and was diagnosed with disseminated tuberculosis. She had marked improvement after one month of anti-tuberculosis treatment but developed a recurrence of initial symptoms on the third month of therapy and was treated for cecal perforation. Histopathology of cecum revealed chronic granulomatous inflammation. The patient improved after the surgery and the resumption of anti-tuberculosis medications. Recognition of paradoxical reactions and differentiating it from drug resistance of other pathology is important as these necessitates different management strategies.
Human ; Female ; Child: 6-12 yrs old ; intestinal perforation

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