PurposeTo determine the efficacy and safety of oral propranolol as a first-line treatment for superficially located infantile hemangioma (IH) and propose an assessment tool to measure treatment response.MethodsPatients with superficial IH under 1 year of age were prospectively recruited between May 2012 and December 2013 at the Department of Pediatrics of Chungbuk National University Hospital. Propranolol was administered to 12 infants (median age, 3.8 months) while monitoring cardiovascular and adverse metabolic effects. If a patient showed no adverse events, the dosage was gradually increased up to 3 mg/kg/day and maintained for 1 year. We used our own scoring system to assess treatment response using parameters like change in color, and longest diameter, and thickness of the IH.ResultsEleven out of 12 patients completed the protocol with consistent improvement of hemangiomas during therapy. Patients on propranolol showed a more than 50% involution in the first 3 months, with additional steady involution until 1 year. Patients with the highest scores at 1 month maintained their score and showed better responses until treatment termination. The patient with the lowest score at 1 month did not show any further regression and stopped propranolol treatment 4 months after initiation. In two children with recurrences after successful therapeutic regression, propranolol was effective after being reintroduced. Propranolol treatment was not interrupted in any patient due to adverse events.ConclusionOral propranolol at 3 mg/kg/day showed a consistent, rapid, and therapeutic effect on superficial IHs without significant adverse events.

Abstract.The use of a GnRH agonist (GnRHa) in central precocious puberty (CPP) is known to slow puberty progression, subsequently prevent early menarche, and attenuate the height loss caused by advanced skeletal maturation. But enhancing the final height has been so controversial that an additional approach has been used. We investigated the menarcheal age and near final height (NFH) in girls with CPP treated with GnRHa (N = 61) or GnRHa combined GH (N = 24). GnRHa was started at 8.1 ± 0.7 yr and administered for 2.1 ± 1.0 years. GH was used for 2.1 ± 1.1 yr in subjects with a short predicted adult height (PAH). Menarche occurred at 11.6 ± 0.8 yr of age, which was 15.7 ± 6.4 mo after GnRHa discontinuation. PAH increased significantly from 152.0 ± 7.2 cm to 158.8 ± 5.6 cm during treatment, and the NFH (159.7 ± 4.8 cm) was taller than the midparental height (157.8 ± 3.4 cm). The combined treatment group showed a greater height increment during treatment. Younger age, taller height at the start of treatment, taller parental height and longer duration of treatment were the factors influencing NFH. In conclusion, GnRHa treatment in girls with CPP could improve NFH and delay menarche close to the general population. If GnRHa combined with GH is used in girls with CPP and a short midparental height, it would improve the NFH to a value similar to that in the general population.

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune regulation. HLH consists of two forms: familial and acquired, the latter which occurs in association with infection, malignancy, rheumatic disease and acquired immune deficiency. Herein, we report a case of acquired HLH in a child who had received allogeneic hematopoietic stem cell transplantation for familial HLH with UNC13D mutation. Based on microbiology, only rotavirus was identified as a possible organism triggering HLH. The patient's fulminant clinical course included acute respiratory failure, a sepsis-like pattern, disseminated intravascular coagulopathy, and rhabdomyolysis, leading to multiorgan failure and death from septic shock.