BackgroundThe general paediatricians and primary care physicians sometimes face immense difficulty in referral judgements regarding which infantile hemangiomas (IHs) require referrals and when is the appropriate time to refer IHs for treatment. This resulted in the treatment being delayed beyond IHs’ critical timeframe. The Infantile Hemangioma Referral Score (IHReS) has been recently developed, with the aim to solve this problem.ObjectivesThe objective of the present study is to evaluate the reliability of IHReS and to assess the possibility of using this instrument in our country where a similar problem of delaying treatment of IHs is currently existing.MethodsThe present study was a prospective, cross-sectional study. Thirteen selected clinical cases were used to assess the reliability of IHReS among physicians who may have had the chance to deal with patients with IHs. The target physicians across the country were asked to participate in the study via an online platform (Google Forms) to decide whether to refer patients with IHs for treatment or observe. There were 3 steps of assessment: step 1, usual practice evaluation; step 2, using IHReS; step 3, retesting by using IHReS.ResultsSubstantial agreement was observed after using IHReS (step 2) for interrater reliability, with Fleiss’ Kappa values of 0.80 and 0.78 among IH experts and non-expert physicians, respectively. Regarding repeatability, in the test–retest assessments, Cohen’s Kappa coefficient values revealed almost perfect agreement in intrarater repeatability for both experts and non-expert physicians (1.00).ConclusionIHReS is a simple, easy-to-assess tool for non-expert physicians. The benefit in the increase of interrater agreement was found in both IH experts and non-expert physicians. It has had the reliability to be used in making referral decisions regarding patients with IH for treatment among Thai physicians. Using IHReS can improve clinical outcomes by identifying which patient needs early intervention to minimise the possible complications.

Carbamazepine was the most common causative drug of SJS and TEN in our study. The severity of skin detachment is not correlated to severity of ocular findings. However, the persistent of ocular complications up to one year is suggested for promptly appropriate ocular treatment in all SJS and TEN patients. Our data suggested that early administration of systemic corticosteroid may reduce the length of hospital stay and should be considered for the treatment of pediatric druginduced SJS and TEN.

Some iron-overloaded patients have problems being treated with iron chelators. We therefore retrospectively studied 7 iron-overloaded thalassemic patients. Within the same week, patients received 20 to 30 mg/kg/d of oral deferasirox for 4 consecutive days, then a subcutaneous infusion of 20 to 40 mg/kg/d of desferrioxamine for 8 to 12 hours on the next 3 consecutive days. The median treatment duration was 25 months (range, 8 to 32). All of the patients showed a decrease in serum ferritin without any side effects. The protocol, combining deferasirox and desferrioxamine in sequence, was effective and safe: more cases should be studied.

Globin chain imbalance and tissue hypoxia are important determinants of the clinical severity of thalassemias. Phenotypic expression may be further modified by interactions between alpha- and beta-thalassemia defects. We retrospectively and prospectively studied the clinical and hematologic features in children and adults with hemoglobin (Hb) E trait/Hb H disease (SEA/Paksé) (seven cases) and Hb E trait/Hb H disease (SEA/Constant Spring) (29 cases) and found that they had similar presentations. The severity of these two intermediate thalassemic manifestations ranged from very mild to severe. Severe anemia developed in accordance with very high fever, whereupon the range of Hb and hematocrit (Hct) levels declined to 5.2-5.8 g/dL and 13%-19%, respectively. In one case, during a hemoconcentrated state as occurs in dengue hemorrhagic fever, the Hb and Hct were 10 g/dL and 31%; the latter rose to 35% after fluid therapy. In some patients, the range of Hb and Hct levels was constantly low (4.3-5.8 g/dL and 15%-19%, respectively). (If dengue hemorrhagic fever is misdiagnosed, a fatal outcome may occur for thalassemic patients.) After a hemodiluted condition as in acute post-streptococcal glomerulonephritis, the respective Hb and Hct were 5.4 g/dL and 19%. These observations suggest that the instability of Hb E, especially during fever, may play an important role in the clinical manifestations of Hb E trait/Hb H disease with Hb Paksé and with Hb Constant Spring.

Propranolol was effective in treating infantile haemangiomas, and combining it with prednisolone achieved no significant differences in treatment outcome. Cases should be monitored for hypoglycaemia and hypotension. More data on using propranolol for infantile haemangiomas are needed, including long-term follow-up studies.