Background: Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs). Methods: A 15-year retrospective review was conducted in KK Women's and Children's Hospital in Singapore, from January 2003 to October 2017. Results: Ten patients were identified, the majority male (60.0%). The median age at presentation of symptoms of BCG infections was 3.8 (0.8 -7.4) months. All the patients had likely underlying PIDSfour with Severe Combined Immunodeficiency (SCID), three with Mendelian Susceptibility to Mycobacterial Diseases (MSMD), one with Anhidrotic Ectodermal Dysplasia with Primary Immunodeficiency (EDA-ID), one with combined immunodeficiency (CID), and one with STAT-1 gain-of-function mutation. Definitive BCGosis was confirmed in all patients by the identification of Mycobacterium bovis subsp BCG from microbiological cultures. The susceptibility profiles of Mycobacterium bovis subsp BCG are as follows: Rifampicin (88.9%), Isoniazid (44.47%), Ethambutol (100.0%), Streptomycin (100.0%), Kanamycin (100.0%), Ethionamide (25.0%), and Ofloxacin (100.0%). Four patients (40.0%) received a three-drug regimen. Five patients (50.0%) underwent hematopoietic stem cell transplant (HSCT), of which three (60%) have recovered. Overall mortality was 50.0%. Conclusion: Disseminated BCG disease (BCGosis) should prompt immunology evaluation to determine the diagnosis of the immune defect. A three-drug regimen is adequate for treatment if the patient undergoes early HSCT.

Gastric pneumatosisis a very rare site of pneumatosis intestinalis (PI), and we report this finding in a preterm female infant with cyanotic congenital heart disease. The infant was stable initially on nasal intermittent mandatory ventilation; however, torrential pulmonary flow through a large patent ductus arteriosus prompted closure using oral ibuprofen. After an episode of haematochezia, she developed PI, affecting mainly the gastric wall and small intestine with portal venous gas. Her bowel movements were regular, with no abdominal distension or significant gastric aspirates. She was haemodynamically stable with negative infective markers. Management consisted of endotracheal intubation and ventilation, gastric decompression and broad-spectrum antibiotics. Both the gastric and intestinal pneumatosis resolved within 24 hours and she made an uneventful recovery. If PI is not due to necrotising enterocolitis, enteral nutrition can be initiated early and prolonged course of broad-spectrum antibiotics could have been avoided.