Childhood bronchiolitis obliterans in Hong Kong-case series over a 20-year period.

BACKGROUND AND OBJECTIVE: Bronchiolitis obliterans (BO) is a rare but serious condition. The natural history and outcomes remain poorly understood. In this clinical review, we aimed to describe the clinical characteristics and outcomes of children diagnosed with BO in Hong Kong (HK). METHODS: This was a retrospective study of pediatric patients with BO under the care of six respiratory units in HK from January 1996 to December 2015. Information was retrieved from medical records. RESULTS: Fifty-six patients were included with a male predominance (67.9%). The median age at diagnosis was 1.98 years (interquartile range [IQR]: 0.84-4.99 years). Postinfectious BO (PIBO) was the commonest cause (64.3%) followed by posthematopoietic stem-cell transplant (21.4%). Adenovirus (63.2%) was the commonest causative pathogen among PIBO. The median follow-up duration was 9.7 years (IQR: 2.9-14.3 years). Twenty-five patients (44.6%) could achieve symptom-free recovery at the time of follow-up. Five (8.9%) and three (5.4%) were oxygen or ventilator dependent, respectively. There were two deaths, both had posttransplant BO. Patients who developed BO after transplant had significantly worse lung function than those with PIBO. There were no risk factors significantly associated with worse clinical outcomes (oxygen/ventilator dependence or death) by logistic regression. Among patients with PIBO, coinfection at presentation was significantly associated with persistent symptoms at follow-up (p = .028). CONCLUSIONS: The most common cause of childhood BO in HK is postinfectious and coinfection at presentation was associated with persistent symptoms at follow-up. Further studies are needed to better elucidate disease progression, treatment options and long term outcomes.

A 7-year-old boy dying of acute encephalopathy.

A 7 year old Chinese boy died of a rapidly progressive encephalopathy after influenza infection. MRI showed bilateral and symmetrical lesions including the thalamus and brainstem tegmentum. The pathology of necrosis and vasculopathy were in keeping with acute necrotizing encephalopathy (ANE). ANE was first described in Japan and carries a high mortality and morbidity. A vasculopathy with breakdown of the blood-brain-barrier was incriminated but the pathogenesis remained obscure and autopsy studies have been limited. A review of the literature showed only nine postmortem reports in the acute stage. Symmetrical brain necrosis always involved the thalamus followed by the tegmentum of the pons and other regions. Exudative vasculopathy was commonly observed and often accompanied by endothelial cell necrosis. In the present case there was inflammatory fibrinoid vasculitis which has not been previously described.

Spontaneous neonatal chylothorax treated with octreotide.

Chylothorax is the most common cause of pleural effusion causing respiratory distress in the neonate. Cases unresponsive to conservative management usually require surgery. We report a case of spontaneous neonatal chylothorax where surgery was avoided with the compassionate trail of octreotide. The chylothorax resolved promptly with no observed side effects. After reviewing the literature reporting the use of octreotide in the neonatal period, we found that neonates with isolated spontaneous chylothorax might be viewed as a group different from those with major cardiac malformations. They are at a lower risk for adverse effect of octreotide and lower threshold for commencement of octreotide may benefit them most.