Utility of the Infantile Hemangioma Referral Score (IHReS) as a decision-making tool for referral to treatment.

Background: The 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. Objectives: The 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. Methods: The 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. Results: Substantial 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). Conclusion: IHReS 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.

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children: 20 years study in a tertiary care hospital.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe lifethreatening skin conditions. The most common cause of these manifestations is medications. Beside discontinued of the culprit drug, systemic corticosteroids were used as a primary treatment option among pediatric population. This study aimed to explore causative drugs (drug group/ latent period), treaments, complications, and treatment outcome (morbidity, mortality, length of hospital stay) of SJS and TEN in children. METHODS: A retrospective chart was reviewed during the period of 1992 to 2012 at Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. SJS and TEN were clinically diagnosed and confirmed by pediatric dermatologists. Other possible causes other than druginduced SJS and TEN were excluded. RESULTS: A total of 30 patients was recorded, including 24 (80%) SJS patients and 6 (20%) TEN patients. The mean age was 6.9 years (SD 4.4). Male to female ratio was 1.5:1. Antiepileptic drug group was the most common causative drug (n=18, 60%), followed by antibiotic drug group (n=8, 26.6%), and others (n=4, 13.3%) which included nonsteroidal antiinflammtory drugs (NSAIDs) and chemotherapy drugs. Systemic corticosteroids were used in 29 patients (96.6%). Intravenous immunoglobulin was used in one TEN patient (3.3%). There was a medium correlation between time to treatment (systemic corticosteroids) and the length of hospital stay (Spearman correlation coefficient=0.63, P=0.005). Two TEN patients (6.6%) died. CONCLUSIONS: 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.

Propranolol was effective in treating cutaneous infantile haemangiomas in Thai children.

AIM: The aim of this study was to explore the efficacy and safety of propranolol in treating infantile haemangiomas, the most common benign vascular tumours in children. METHODS: We carried out a retrospective chart review of infantile haemangioma patients admitted to the Faculty of Medicine, Khon Kaen University, Thailand, from January 2009 to January 2015. RESULTS: There were 53 infantile haemangioma cases treated with oral propranolol. Treatment responses occurred as early as two weeks after propranolol administration in 91.5% of the follow-up patients, with all 53 cases achieving the desired treatment responses two months after propranolol was initiated. No significant differences in treatment responses were found between propranolol as a mono-therapy or as a combination therapy with prednisolone at the two-week (p value 0.13) and one-month follow-ups (p value 0.98). Complications were documented in three cases (5.6%) when the propranolol dose was increased, and these were asymptomatic hypoglycaemia in two cases and one case of hypotension. CONCLUSION: 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.

Vascular Anomalies: The Epidemiological Profile at Srinagarind Hospital.

BACKGROUND: Vascular anomalies or vascular birthmarks can be divided in to 2 major groups: (i) vascular tumors and (ii) vascular malformations. Currently, there are many treatment modalities for these diseases and the treatment plans are varied among sub-specialty physicians. OBJECTIVE: To explore the epidemiology of vascular anomalies at Srinagrind Hospital during 2009-2011. MATERIAL AND METHOD: Retrospective chart was reviewed from the out patient clinic's database at Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. RESULTS: There were total of 126 vascular anomalies cases. 89 cases were diagnosed with vascular tumors and 37 cases were vascular malformations. Among 89 cases of vascular tumors, infantile hemangiomas are the most common type (95.5%). The treatment methods for vascular tumors were medical treatments, which were used in majority of the cases (60%), followed by surgical excision, laser treatment, intralesional corticosteroids injection, and the combination of medical, laser and surgical treatment. There were total of 37 cases of vascular malformations. Most of the cases were venous and lymphatic malformations. Treatment methods for these patients were surgical excision, bleomycin injection, and radiation. CONCLUSION: Vascular anomalies have various presentations. Treatment is challenging and multidisciplinary teams are involved in taking care the patients with this entity of disease. Setting up vascular anomalies clinic is essential and suggested for the patients with vascular anomalies' problems.

Carbamazepine-Induced Incomplete Stevens-Johnson Syndrome: Report of a Case in Children without Mycoplasma pneumoniae Infection.

Incomplete Stevens-Johnson syndrome (SJS) is a rare reactive skin condition. Most cases are occurred in children and all are associated with Mycoplasma pneumoniae (M. pneumoniae) infection. We reported an unusual case of a 6-year-old boy who developed the presentation of isolated mucosal erosion with a lack of skin findings, which indicated incomplete SJS after two weeks of carbamazepine (CBZ) administration. Findings of positive HLA-B*1502 allele supported a possible causative influence of carbamazepine inducing SJS. Interestingly, this patient was tested negatively for M. pneumoniae. This is a significant finding since there is no previous report of incomplete SJS without M. pneumoniae infection. Discontinuation of CBZ and administration of systemic corticosteroids were accomplished to treat SJS, which resulted in complete recovery. Our interesting findings highlighted the manifestation of incomplete SJS, which can present with other causes rather than M. pneumoniae infection. Early manifestation of mucosal change without typical skin lesions should not be neglected in the diagnosis of incomplete SJS.

Retrospective study on the combination of desferrioxamine and deferasirox for treatment of iron-overloaded thalassemic patients: first evidence of more than 2 years.

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.

Infections and illnesses in children with Bb E beta-thalassemia: a prospective controlled study.

The authors performed a prospective, controlled, 3-year, follow-up study on infections and illnesses in Hb E beta-thalassemic pediatric patients. Fifty severe and 24 non-severe patients and 24 controls were included. Siblings with an age difference of no more than 4 years served as controls. All patients and controls were asked to write postcards every two weeks to report on their illnesses and treatments. The respective median follow-up was 32.5, 35.5 and 34 months in 1501, 707 and 785 patient-months at 11.50 +/- 4.74, 10.50 +/- 4.18 and 10.75 +/- 4.56 years of age (+/- SD) for the severe, non-severe Hb E beta-thalassemic patients, and controls. The rate per 1000 patient-months of infections was not significantly different between groups despite having 26 (52%) splenectomised patients in the severe group. The infection rate among severe, non-severe, Hb E beta-thalassemic, patients and controls was not significantly different. Regular blood transfusions and iron chelation might decrease infections among Hb E beta-thalassemic, pediatric patients.

Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome in monozygotic twins: two cases report and reviews.

Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome (or Hay-Wells syndrome) is a rare congenital malformation. Our first cases were a pair of female monozygotic twins with AEC syndrome at Srinagarind Hospital. In this study, we describe monozygotic female twins concordant for ankyloblephaon, ectodermal dysplasia and helical rim deformities, but discordant for cleft, syndactyly of toes, heart and urinary tract abnormalities. Twin A had syndactyly of the right third and fourth toes with incomplete bilateral cleft lip and complete bilateral cleft palate. Twin B had left ventricular enlargement, caliectasia of both kidneys with complete left unilateral cleft lip and cleft palate. The twins were treated by multidisciplinary teams with satisfactory results.

Thai girl with ring chromosome 18 (46XX, r18).

Chromosomal anomalies occur in 0.4% of live births. Ring chromosomes have been found for all human chromosomes and when it is replacing a normal chromosome, it results as a partial monosomy. The phenotype often overlaps that seen in comparable deletion syndromes of the same chromosomes. In the present report the authors describe the clinical manifestations of a girl with ring chromosome 18 (46XX,r18) including dysmorphic features, failure to thrive, global delay of development, hypothyroidism, atopic dermatitis, bilateral chronic otitis media, aortic regurgitation with patent foramen ovale and immunoglobulin A deficiency.

Development of severe anemia during fever episodes in patients with hemoglobin E trait and hemoglobin H disease combinations.

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.