Development of a biomarker of efficacy in second-line treatment for lymphangioma of the tongue: a pilot study.

Lymphangioma of the tongue is a rare lymphatic malformation, and various authors have reported the successful use of sirolimus for its treatment. However, the safety of sirolimus in children needs further evaluation so that those who do not respond are not necessarily exposed to its potential adverse effects. We hypothesised that assessment of lymphangiogenesis can be used to predict whether the patient will respond to sirolimus, so we organised a prospective study after ethics committee approval had been given. After clinical and histological diagnoses of lymphangioma of the tongue had been confirmed, 16 patients were given sirolimus 0.8mg/day in three divided doses. Clinical response was assessed and compared with lymphatic microvessel density (LMVD), which was calculated immunohistochemically using the monoclonal antibody D2-40 as the lymphatic endothelial marker. Nine patients responded well, five partially, and two failed to respond. Mean (SD) LVD among the good responders was 21.00 (3.74), whereas among non-responders it was 8.00 (4.24). There was a significant difference in mean LVD between good responders, partial responders, and non-responders (p=0.04). Sirolimus is effective in treating children with lymphangioma of the tongue, and lymphangiogenesis is a useful therapeutic predictive marker.

Antiphospholipid antibody-related Bell's palsy in young women.

The present report describes three young women aged 25, 20 and 15 years who presented with Bell's palsy. Two of the patients had a past history of the disease, which responded to steroid treatment. All three patients were positive for antiphospholipid antibody (APLA). In addition, one of the patients tested positive for antinuclear antibodies; however, there was no clinical evidence of systemic lupus erythematosus. All three patients responded well to steroid therapy. We conclude that Bell's palsy may be one of the manifestations of APLA syndrome, and thus, APLA testing should be done in such cases.

Chronic bilious vomiting in children in developing countries due to high bowel obstruction: not always malrotation or tuberculosis.

BACKGROUND: Bilious vomiting, in conjunction with abdominal pain is considered to be a surgical problem, unless proved otherwise. In children, besides tuberculosis (TB), we have found jejunal stricture (JS) due to non-specific jejunoileitis (NSJI) to be an important cause of chronic high small bowel obstruction and bilious vomiting. MATERIALS AND METHODS: In this retrospective study, the records of all children with complaint of intermittent bilious vomiting and failure to thrive were evaluated. Investigations included oral contrast study, ultrasound abdomen, chest X-ray and Mantoux test. Final confirmation was made at laparotomy. Treatment included jejuno-jejunal resection and anastomosis. Histopathology of the specimen was done to look for caseation, granuloma formation and other details. RESULTS: Out of total 100 patients with the complaint of bilious vomiting, 25 were having JS. Radiologic confirmation was possible in 19 (76%) patients of JS. No patient had evidence of TB as per our protocol. Histopathology revealed non-specific ischemic changes in all specimens. CONCLUSION: Jejunal stricture due to NSJI is a common entity in our setup leading to bilious vomiting. Contrast study can provide high index of suspicion in most of the patients. The diagnosis must be confirmed after proper histopathological examination. The results of the surgery are excellent.