A retrospective cohort study of major adverse cardiac events in children affected by Kawasaki disease with coronary artery aneurysms in Thailand.

Kawasaki disease (KD) is a common form of vasculitis in children that can be complicated by coronary artery aneurysms (CAAs). Data of long-term outcomes and major adverse cardiac events (MACE) in children with CAAs following KD in developing country are limited. Our aims were to determine the rates of MACE and identify risk factors associated with MACE in children with KD and CAAs in Thailand. We performed a retrospective analysis of data from 170 children diagnosed with KD and CAAs in two tertiary hospitals between 1994 and 2019. During a median (range) follow-up of 5.4 years (22 days to 23 years), 19 patients (11.2%) experienced MACE, that included 12 coronary artery bypass grafting, 2 percutaneous coronary intervention and 5 children with evidence of myocardial ischemia and coronary occlusion. Coronary interventions were performed at a median time of 4 years (0.01 to 9.5 years) after KD diagnosis. Forty-nine patients (28.8%) had giant CAAs. No MACE was reported in children with small CAAs. Independent risks of MACE were from the absence of intravenous immunoglobulin treatment (HR 7.22; 95% CI 2.21 to 23.59; p = 0.001), the presence of giant aneurysms (HR 13.59; 95% CI 2.43 to 76.09; p = 0.003), and CAAs that involved bilateral branches of coronary arteries (HR 6.19; 95% CI 1.24 to 30.92; p = 0.026). Among children with giant CAAs, the intervention-free rate was 93.8%, 78.7% and 52.2%, at 1, 5 and 10 years, respectively. Of note, 81% of the small CAAs regressed to a normal size, and for medium CAAs, 50% regressed to normal size. Overall, ~10% of children with CAAs following KD experienced MACE in this cohort. Timely IVIG treatment in children with KD following symptom onset will reduce the risk of MACE. Cautious surveillance to identify cardiac complications should be recommended for children once medium or giant CAAs develop. Trial registration: TCTR20190125004.

INCREASED LEVELS OF MEDIAN URINARY IODINE EXCRETION OF PRIMARY SCHOOL CHILDREN IN THE SUBURBAN AREA, KHON KAEN, THAILAND.

Iodine deficiency disorder (IDD) is associated with a low IQ in children and is an important public health problem in northeastern Thailand. Despite campaigns to reduce IDD in northeastern Thailand, studies showed people in this region continue to have the lowest median urinary iodine (UI) excretion and Intelligence Quotient scores. We conducted a cross sectional study of median urinary iodine excretion among primary school children in suburban Khon Kaen Province, in northeastern Thailand, during December 2012 to evaluate the current status of IDD in this population. We studied 377 school children. Urine samples were collected and measured for UI using a simple microplate method. The median UI level was 229.0 µg/l (range 15.0-1,124.1). Forty school children (10.6%) had UI levels less than 100 µg/l and 10 children (2.7%) had UI levels less than 50 µg/l. One hundred nine children (28.9%) had UI levels greater than 300 µg/l. Our study shows that there are still children in the study population and study area with inadequate UI levels. Programs to prevent IDD need to include this population in this area.

Effectiveness and Safety of Radioactive Iodine Therapy in Childhood Graves' Disease in Khon Kaen, Thailand.

Background: Graves' disease (GD) is the most common cause of hyperthyroidism in children and adolescents. Treatments consist of medication, radioactive iodine (RAI) therapy and surgery. Currently, radioactive iodine therapy is the first line treatment in many medical centers. Objective: To evaluate the effectiveness and safety of RAI therapy in childhood GD. Material and Method: A retrospective study was performed in 46 GD patients, aged at onset <15 years, who had undergone RAI therapy at the age >10 years. Goiter grading, evidence of hypothyroidism, severity of ophthalmopathy, RAI dosage and side effects of RAI therapy were evaluated. Results: The cure rate was 95.6%. All participating patients had goiter reduction (p = 0.005). Hypothyroidism was induced in 33 (71.7%) and 11 (23.9%) patients after the first and second RAI therapy. The total RAI dosage was significantly higher in the patients with failure response (p = 0.001). The average time to induce hypothyroidism after the first RAI therapy was 127.5 (IQR: 94.5-223.0) days. All of the patients had improvement of ophthalmopathy and none had thyroid carcinoma during the follow-up period of 42.5 (IQR: 17-52) months. Conclusion: Radioactive iodine therapy is effective and safe in the treatment of children and adolescents with Graves' disease.

Assessment of Chromosome 22q11.2 Deletion in Patients with Isolated Cleft Palate: A Systematic Review of Prospective Studies.

Background: The prevalence of 22q11.2 deletion in patients presenting with isolated cleft palate has not been systematically assessed. Objective: To assess the evidence in the literature for the prevalence of 22q11.2 deletion in patients who were presenting with isolated cleft palate. Material and Method: A systematic literature search was conducted through PubMed between 1992 and June 2016 using search terms of 22q11.2 deletion OR 22q11 deletion AND cleft palate. Results: Of the six prospective studies reported, 328 patients with isolated cleft palate had been screened with FISH (Fluorescence In Situ Hybridization) test for 22q11.2 deletion. Among the 328 patients, there was one (0.3%) patient with positive FISH test for 22q11.2 deletion. This patient was clinically assessed and did not have an associated malformation or clinically recognized syndrome. Conclusion: The prevalence of 22q11.2 deletion among patients with isolated cleft palate is rather low. Of more than 400 genetic disorders involving occurrences of isolated cleft palate, FISH testing for 22q11.2 deletion in a patient with isolated cleft palate is recommended on clinical suspicion of additional clinical presentations of 22q11.2 deletion syndrome such as conotruncal congenital heart diseases, dysmorphic facies, velopharyngeal insufficiencies, immune deficiencies, hypoparathyroidisms, and neuropsychiatric disorders.

Birth Prevalence of Chromosome 22q11.2 Deletion Syndrome: A Systematic Review of Population-Based Studies.

Background: A birth prevalence of chromosome 22q11.2 deletion syndrome among population-based reports has been documented to vary, however, a systematic assessment is lacking. Objective: To assess the evidence in the literature for the birth prevalence of chromosome 22q11.2 deletion syndrome. Material and Method: A systematic literature search was conducted through PubMed between 1992 and June 2016 using search terms of 22q11.2 deletion OR 22q11 deletion and prevalence. Results: Of the six studies reported, there were 156 patients with 22q11.2 deletion syndrome found in total study populations of 1,111,336 live births. According to countries, the birth prevalence of this deletion syndrome (95% confidence interval) from United States, Belgium, Sweden, United Kingdom, France, and Singapore were 1.68 (1.22-2.26), 1.56 (1.33-1.72), 1.36 (0.91-2.08), 1.30 (0.45-2.15), 1.03 (0.53-2.23), and 1.02 per 10,000 live births, respectively. Estimates of minimum prevalence rates on the basis of the presence of this syndrome in cohorts of patients with cardiovascular malformations were from one in 4,000 to one in 7,092 live births. Conclusion: This systematic review indicates that the 22q11.2 deletion syndrome is rather common. The findings can help physicians, health care planners and other health professionals to plan and manage better care of these patients.

Prevalence of Congenital Heart Diseases in Patients with Orofacial Clefts: A Systematic Review.

BACKGROUND: The reports on prevalence rates of congenital heart diseases (CHDs) in patients with orofacial clefts (OFCs) have varied widely. OBJECTIVE: To systematically review the prevalence rates of CHDs in patients with OFCs. MATERIAL AND METHOD: A computer search was conducted through the PubMed from 1950 to June 2015 using key words or search terms of congenital heart diseases, orofacial clefts, cleft lip/palate and prevalence. RESULTS: The search resulted in nine studies with 598 CHDs cases identified in 5,707 patients with OFCs. The prevalence of CHDs in patients with OFCs ranged from 3.9% to 23.9%. The five prospective studies had prevalence rates of 12.0% (95% confidence interval [CI]: 10.9 to 13.2) whilst the four retrospective studies had prevalence rates of 8.6% (95% CI: 7.5 to 9.8). Concerning the prospective studies, the newborn study had a higher prevalence than those of other childhood studies [23.9% vs. 11.5% (95% CI: 10.4 to 12.7)]. The newborn study with the use of echocardiography had a higher prevalence than those without using echocardiography (23.9% vs. 12.8%). Atrial septal defect was the most frequent CHD found. CONCLUSION: CHD is commonly found in a patient with OFC. Echocardiography should be used to assess CHD in patients with OFCs.

Global Birth Prevalence of Orofacial Clefts: A Systematic Review.

BACKGROUND: A birth prevalence of orofacial clefts (OFCs) worldwide has been documented to vary. However a systematic assessment is lacking. OBJECTIVE: To assess the evidence in the literature for the birth prevalence of OFCs. MATERIAL AND METHOD: A systematic literature search was conducted using electronic databases through PubMed between 1950 and June 2015 using key words and search terms of cleft lip palate OR orofacial cleft AND prevalence. RESULTS: There were 45,193 patients with OFCs found in a study population of 30,665,615 live births. According to continents, the OFC birth prevalence (95% confidence interval)from Asia, North America, Europe, Oceania, South America, and Africa were 1.57 (1.54-1.60), 1.56 (1.53-1.59), 1.55 (1.52-1.58), 1.33 (1.30-1.36), 0.99 (0.96-1.02), and 0.57 (0.54-0.60) per 1,000 live births, respectively. The American Indians had the highest prevalence rates of 2.62 per 1,000 live births, followed by the Japanese, the Chinese, and the Whites of 1.73, 1.56, and 1.55 per 1,000 live births, respectively. The Blacks had the lowest rate of 0.58 per 1,000 live births. CONCLUSION: Observed differences may also be of ethnic origin, genetic, environmental factors, and methods of ascertainment. Further investigations are needed to manage this global health problem.

Treatment of 4-5 year old patients with cleft lip and cleft palate in Tawanchai Center: prevalence and type of associated malformations.

BACKGROUND: Patients with cleft lip/palate may have other associated malformations but the reported prevalence and type of associated malformations varied between different studies. OBJECTIVE: To report the prevalence and the type of associated malformations in Northeastern Thai patients with cleft lip/palate. MATERIAL AND METHOD: A retrospective study of 123 cleft lip/palate patients aged 4-5 years was carried out at the Tawanchai Cleft Center, Khon Kaen University during the periodfrom October to December 2011. Data were collected by reviewing the patients medical records. RESULTS: Seventeen (14%) of the 123patients had associated malformations. Four (21%) of the 19patients with cleft palate, eleven (15%) of the 74 patients with clefts lip and palate, and two (7%) of the 30 patients with cleft lip had associated malformations. The organ systems affected by associated malformations were cardiovascular system (41%), craniofacial anomaly (23%), skeletal system (12%), urogenital system (12%) and central nervous systemn (12%). Atrial septal defect and tetralogy ofFallot were most common associated cardiovascular malformation found. CONCLUSION: The high prevalence of associated malformationsfound in patients with cleft lip/palate emphasizes the needfor a thorough screening of associated malformations and congenital heart disease ofall cleft lip/palatepatients.

Enamel defect and gingival enlargement in pediatric patients with kidney disease at Srinagarind Hospital, Khon Kaen University, Thailand.

BACKGROUND: Although many complications from kidney disease therapy can be prevented or effectively treated, oral health problems are nevertheless a consequence. OBJECTIVE: The objective of this study was to explore the prevalence of enamel defect and gingival enlargement in pediatric patients with kidney disease at Srinagarind Hospital, Khon Kaen University, Thailand. MATERIAL AND METHOD: This cross-sectional study was conducted between January and August 2013, at SrinagarindHospital, Khon Kaen University. Ninety-seven pediatric patients with kidney disease were allowed by their parents to participate in this study. Data were collected from medical records, questionnaires and oral examination records. The enamel defect was recorded using the Developmental Defects of Enamel Index. Gingival enlargement was recorded using the GingivalEnlargement Index. An oral examination was conducted using a mouth mirror, explorer and periodontal probe. RESULTS: The average age of the pediatric patients with kidney disease was 11.53+3.7years (range, 4-17). The majority of subjects were able to (a) come for an appointment (97.9%), (b) take medication according to the medical directions (93.8%) and (c) avoid inappropriatefoodsfor those suffering kidney disease (84.5%). The prevalence of enamel defect was 27.8%. The most common enamel defects were demarcated opacities (13.4%) or diffuse opacities (9.3%). The prevalence ofgingival enlargement was 16.5%. CONCLUSION: This study revealed that the prevalence ofenamel defect was 27.8% and the prevalence ofgingival enlargement was 16.5%.

Prevalence and type of associated syndromes in patients with cleft lip and cleft palate who received the treatment in Tawanchai Center until 4-5 years of age.

BACKGROUND: Understanding the genetic etiologies of cleft lip and palate (CLP) is important for improved prevention, treatment, and prognosis for patients affected by CLP. OBJECTIVE: To report the prevalence and the type of associated syndromes in Northeastern Thai patients with CLP. MATERIAL AND METHOD: A retrospective study of123 cleft lip/palate children aged 4-5 years was carried out at the Tawanchai Cleft Centel; Khon Kaen University during the period from October to December 2011. Data were collected by reviewing the patient 's medical records. RESULTS: Seventeen (14%) of the 123 children had multiple malformations and five (4%) of these children had associated syndromes. Syndromes were identified in 5 (29%) of the 17 children who had associated malformations. The syndromes were Apert, Cleft lip/palate-ectodermal dysplasia, Kabuki, Oculo-Auriculo-Vertebral Spectrum, and Velocardiofacial syndrome. CONCLUSION: Recognition of the associated syndrome in a patient with CLP is essential to assess the problem of the patient, provide necessaty treatment and the appropriate methodology of prevention.

The rising incidence of type 1 diabetes in the northeastern part of Thailand.

OBJECTIVE: To determine the incidence of T1DM in children under 15 years in 19 provinces in northeast Thailand. MATERIAL AND METHOD: Data of new cases of T1DM during 10 years between 1996 and 2005 were collected retrospectively by a mail survey from 275 hospitals in northeast Thailand. RESULTS: Three hundred forty cases, 134 (39.4%) boys and 206 (60.6%) girls were identified. The incidence rate of T1DM was 0.6/100,000/year (95% confidence interval 0.57; 0.71), a two-fold increased from the previous study between 1991 and 1995. More than half of the cases were diagnosed between the ages of 10 and 14 and the incidence rate in girls was 1.5 fold that of boys. CONCLUSION: Though the increased in incidence rate, the study indicated that the incidence of T1DM in northeast Thailand is still one of the very low incidence rates in the world.

Prevalence of type II diabetes and metabolic syndrome among overweight school children in Khon Kaen, Thailand.

BACKGROUND: Childhood obesity has been accompanied by an increase in the prevalence of type 2 diabetes mellitus (T2DM) and metabolic syndrome (MetS) among children and adolescents worldwide. OBJECTIVE: To determine the prevalence of T2DM and MetS among overweight children and adolescents in Khon Kaen province, Northeast Thailand. MATERIAL AND METHOD: A cross-sectional, prospective pilot study was performed in school children between 10 and 15 years of age. The weight and height measurements and body mass index (BMI) calculations for 2156 school children were analyzed. The BMI for the age and sex value at > or = 85th percentile was considered overweight and the overweight children were evaluated for family history of diabetes, signs of insulin resistance, plasma fasting glucose and lipid level. RESULTS: Five hundred and ninety four (27.6%) overweight children were identified, of whom 186 (31.3%) participated in the present study. T2DM was documented in 4 (2.2%) while MetS was documented in 6 (3.2%) children. At least one type of dyslipidemia was found in 87 (46.8%) children. CONCLUSION: T2DM and MetS are common among overweight school children in Khon Kaen, Thailand Preventive interventions to reduce overweight and consequently prevent T2DM in Thai children should be provided at school and the community level.

Acute suppurative thyroiditis with thyrotoxicosis.

BACKGROUND: Acute suppurative thyroiditis (AST) is a rare condition in children, and most children with AST usually have normal thyroid function. OBJECTIVE: To report a case of thyrotoxicosis complicating AST in a child. CASE REPORT: A 6-year-old boy with AST presented with the unusual clinical features of severe thyrotoxicosis. Two palpable masses were found to be of firm to hard consistency with tenderness without any acute inflammatory signs on the overlying skin of the thyroid gland. The diagnosis of AST was confirmed by ultrasonography and fine needle aspiration. Thyroid function tests were normal within a week after antibiotic treatment and surgical drainage. CONCLUSION: Transient thyrotoxicosis complicating AST is very rare in children. Awareness of this unusual complication is important to avoid inappropriate treatment of hyperthyroid disease.

Evolution of valve damage in Sydenham's chorea during recurrence of rheumatic fever.

BACKGROUND: The evolution of valve damage during a recurrence, in a patient who did not have apparent carditis in the initial attack of Sydenham's chorea, has been a subject of debate. METHODS: Thirty-six patients with an initial episode of acute rheumatic fever and Sydenham's chorea (3 patients with subclinical mitral regurgitation) were followed up prospectively for 5 years. Clinical examinations and transthoracic color Doppler echocardiography were done for each patient at initial attacks of Sydenham's chorea and at recurrences of rheumatic fever. RESULTS: Six recurrences of rheumatic fever occurred in 6 of the 36 patients. One recurrence developed in the regular secondary prophylaxis group (27 patients) with a recurrence rate of 0.007 per patient-year. Three of the 18 patients who initially had no heart murmur and no echocardiographic finding of valvular regurgitation had three recurrences of pure chorea with no echocardiographic evidence of significant valvular regurgitation. Interestingly, one of the 3 patients with previous pure chorea and echocardiographic finding of significant mitral regurgitation had a recurrence of pure chorea and new echocardiographic evidence of mitral, aortic and tricuspid regurgitations. Furthermore, two recurrences occurring in 2 of the 15 patients with previous carditis developed echocardiographic evidence of new carditis. CONCLUSIONS: Rheumatic recurrence can develop new valve damage evident by echocardiography for the patient with pure Sydenham's chorea who had previous subclinical valvulitis. Longer duration of secondary prophylaxis is recommended to prevent a recurrence of rheumatic fever in the patient with pure Sydenham's chorea who initially had subclinical valvulitis.

Epidemiologic study of Kawasaki disease and cases resistant to IVIG therapy in Thailand.

The incidence of Kawasaki disease (KD) in Thailand has never been studied before. We reviewed the data from the National Registry of Thai Children who had KD between 1998-2002 to evaluate the incidence of KD and cases resistant to treatment with intravenous immunoglobulin (IVIG). Resistance to IVIG was defined as remaining febrile at least 48 hours after initial IVIG therapy. There were 710 KD patients in the registry. The incidence of KD was from 2.14 to 3.43 cases per 100,000 children aged 0-5 years. During the acute phase 15.6% of 435 patients were considered as resistant cases. Resistant cases of KD in Thai children are quite common (15.6%) even after IVIG treatment. We found that patients who had high white blood cell counts (> 16,500 cells/mm3) had a higher likelihood of being resistant.

Kawasaki disease in central area of Northeast Thailand.

Kawasaki disease (KD) is a leading cause of acquired heart disease of childhood. The authors retrospectively reviewed cases of KD in major referral centers of central Northeast Thailand from July 1991 to June 2003. Seventy-three episodes occurring in 72 patients were diagnosed with KD by the American Heart Association criteria with a mean age of presentation of 27 +/- 19 months. The annual incidence was 2.2 per 100,000 children < 5 years of age. Coronary artery abnormalities (CAA) were found in 15 (20.5%) children. Nine patients (18%) who were diagnosed before 10 days were not treated with intravenous immunoglobulin (IVIG). Two (13%) of the 15 patients still had coronary lesions at the end of the follow-up period of 35.5 +/- 13.4 months. Index of suspicious should be maintained in children who had clinical signs of KD for early diagnosis and prompt treatment with IVIG.

Aortoesophageal fistula: a life-threatening cause of upper gastrointestinal hemorrhage in double aortic arch, a case report.

The authors present the case of a 2-month-old infant with double aortic arch that developed massive bright red upper gastrointestinal hemorrhage from aortoesophageal fistula (AEF) after prolonged endotracheal and nasogastric intubation. Emergency thoracotomy with AEF and double aortic arch repaired were done successfully under cardiopulmonary bypass. Due to tracheomalacia and left phrenic nerve injury, tracheal extubation could not be done until 1 month after correction of the vascular ring. The endotracheal and nasogastric tube led to fistula formation by compression of the esophageal wall against an abnormal double aortic arch. When a double aortic arch is suspected, prolonged nasogastric intubation should be avoided.

The relationship of carditis to the initial attack of Sydenham's chorea.

BACKGROUND: The natural history of valvular regurgitation detected by echocardiography in Sydenham's chorea has been lacking. METHODS: Clinical assessment and transthoracic color Doppler echocardiography were independently performed for each patient with an initial attack of Sydenham's chorea and also for each normal control (Khon Kaen University, Thailand; 1991-2001) at the time of their presentations. Serial clinical examinations and echocardiography were done for each patient at 1- and at 5-year follow-up. RESULTS: Of 44 patients, 17 (39%) had carditis (valvulitis) evident by auscultation at the time of the initial attack. Three (11%) of the 27 patients with no clinical evidence of carditis had echocardiographic evidence of acute mitral regurgitation (subclinical valvulitis). All 17 patients with carditis had echocardiographic evidence of acute mitral regurgitation. None of the 88 control children had evidence of pathologically significant valvular regurgitation. Of the 15 patients with carditis regularly followed, the persistence of a mitral regurgitation murmur and of pathologically significant valvular regurgitation by echocardiography were 33% (5/15) and 60% (9/15), respectively, at 1-year follow-up, but the persistence of both was 25% (1/4) at 5-year follow-up. CONCLUSIONS: Color Doppler echocardiography is a useful tool in the early diagnosis of rheumatic carditis (valvulitis) and at 1-year follow-up of rheumatic valvular heart disease in the patients with initial Sydenham's chorea but the incremental benefit beyond 5 years after the initial attack might be minimal.