Pasireotide treatment for severe congenital hyper-insulinism due to a homozygous ABCC8 mutation

ABCC8 and KCJN11 mutations cause the most severe diazoxide-resistant forms of congenital hyperinsulinism (CHI). Somatostatin analogues are considered as secondline treatment in diazoxide-unresponsive cases. Current treatment protocols include the first-generation somatostatin analogue octreotide, although pasireotide, a second-generation somatostatin analogue, might be more effective in reducing insulin secretion. Herein we report the first off-label use of pasireotide in a boy with a severe therapy-resistant form of CHI due to a homozygous ABCC8 mutation. After partial pancreatectomy, hyperinsulinism persisted; in an attempt to prevent further surgery, off-label treatment with pasireotide was initiated. Short-acting pasireotide treatment caused high blood glucose level shortly after injection. Long-acting pasireotide treatment resulted in more stable glycemic control. No side effects (e.g., central adrenal insufficiency) were noticed during a 2-month treatment period. Because of recurrent hypoglycemia despite a rather high carbohydrate intake, the boy underwent near-total pancreatectomy at the age of 11 months. In conclusion, pasireotide treatment slightly improved glycemic control without side effects in a boy with severe CHI. However, the effect of pasireotide was not sufficient to prevent near-total pancreatectomy in this case of severe CHI

A rare case of an isolated triquetrum body fracture in a 14-year-old boy

Isolated carpal fractures are uncommon injuries usually caused by a fall on the outstretched hand. The patient might present with non-specific clinical signs and X-ray diagnosis might be difficult due to bone overlay. An isolated triquetrum body fracture is an absolute rarity. The treatment is easy and the outcome excellent but if missed, degenerative changes with chronic pain and impaired movement might be the consequence.Key words: Carpal bone fracture, triquetrum bone fracture, wrist fractur

Self-bougienage of oesophageal stricture by an 8-year-old child

Oesophageal corrosive injuries have a high potential to result in stricture formation with the requirement for repeat oesophageal dilation. Especially in children, oesophageal bougienage is performed under general anaesthesia or strong sedation. In developing countries without comprehensive medical care, this service might not be available. We report the case of daily oesophageal self-bougienage performed by an 8-year-old Afghan child as highly effective treatment of recurrent oesophageal stricture formation after caustic substance ingestion