Thromboembolic complications in Thai pediatric patients.

Sixty patients with thromboembolic complications from 1987 to 1997 at the Department of Pediatrics, Ramathibodi Hospital were retrospectively studied. Twenty patients were infants and 40 patients were children and adolescents with a mean age of 18 days and 8 years, respectively. The sites of thromboembolic complications were in the central nervous system, 27.5 per cent; skin as purpura fulminans or necrotic lesions, 24.5 per cent; gangrene of the toe, finger or colon, 19 per cent; deep vein thrombosis, 16 per cent; and other sites such as heart and lungs, 13 per cent. Most of them had triggering conditions (80%) and underlying diseases (76.7%) causing thromboembolism. The low levels of either antithrombin III, protein C or protein S were found in 42 per cent (15/36). The management included administration of standard or low molecular weight heparin if not contraindicated, replacement of fresh frozen plasma 10 ml/kg twice a day and treatment of underlying and triggering conditions. The fatality rate was 15 per cent (9/60). Subsequent episodes of thromboembolism occurred in 6 patients including: pulmonary emboli in one patient with protein C deficiency who refused warfarin administration, deep vein thrombosis in 2 patients with unidentified etiology, and necrotic skin lesions in 3 patients with vasculitis who did not respond to treatment. In conclusion, a comprehensive investigation and specific treatment for patients with thromboembolic complications are emphasized in order to prevent recurring episodes.

Residual patent ductus arteriosus shunting after single Gianturco coil occluding.

Transcatheter occlusion with Gianturco coils has become the treatment of choice for small patent ductus arteriosus (PDA). Coil occlusion was attempted in 20 patients with ductus diameter less than 4 mm who did not require other cardiac surgery. Sixteen of 20 patients had successful implantation. The mean age was 4.2 years. Their mean weight was 14.1 +/- 5.9 kg. The mean ductus diameter was 2.21 +/- 0.91 mm (range 1-3.7 mm). Nine patients had complete occlusion but 7 had residual shunting immediately after the procedure. However, 4 patients had spontaneous resolution of residual shunts at 6 months after the procedure. The other 3 who had diameter of ductus greater than 3 mm still had significant residual shunt at 6 months and 1 year after the procedure. The second coil was successfully implanted in one of these 3 patients and the closure of PDA was accomplished. We concluded that the second coil should be implanted if the ductus diameter is greater than 3 mm and significant residual shunt is still demonstrated angiographically after the first coil implantation.

Indomethacin prophylaxis for patent ductus arteriosus (PDA) in infants with a birth weight of less than 1250 grams.

BACKGROUND: Very low birth weight (VLBW, less than 1500 g) and extremely low birth weight infants (ELBW, less than 1000 g) are the premature infants that are most likely to develop symptomatic PDA. Intravenous indomethacin has proven effective in prevention of PDA in many prospective trials. This strategy will be a useful adjunctive therapy for premature infants in Thailand. OBJECTIVE: To answer the following questions: 1. Will multiple doses of intravenous indomethacin, given to VLBW infants within the first day of life, effectively prevent the occurrence of symptomatic PDA? Are there any side effects or complications? 2. Will this strategy be more beneficial in ELBW? METHODS AND SUBJECTS: The study included thirty VLBW infants born at Ramathibodi Hospital, with birth weights ranging from 630 to 1230 g. They were randomized into 2 groups of 15 infants each. One group received 3 doses of intravenous indomethacin at the dosage of 0.2 mg/kg initially and then 0.1 mg/kg every 12 hours for 2 more doses; the second group received a placebo. The study was performed by a double blind control. RESULTS: Sixteen infants developed symptomatic PDA, 4 in the indomethacin group and 12 in the placebo group. The decrease in incidence of PDA is statistically significant. But when the data was analyzed separately for the VLBW and ELBW groups. The effects were only significantly different in ELBW but not yet significant in the VLBW group. There was a statistically significant difference in the incidence of severe intraventricular hemorrhage (IVH) (grade 3 or higher) in the ELBW infants. CONCLUSION: Intravenous indomethacin therapy given to VLBW infants with a birth weight of less than 1250 g decreased incidence of symptomatic PDA with no significant permanent side effects. The effect was markedly noticeable in ELBW infants. Incidence of severe IVH was also markedly decreased in the ELBW infants who received indomethacin.

Williams syndrome and the elastin gene in Thai patients.

Williams syndrome (WS) has long been known as a complex disorder of dysmorphic facial features, described as elfin face, mental retardation or learning disability, loquacious personality, and supravalvular aortic stenosis. The etiology is now known to be due to deletion of the elastin gene (ELN) on long arm of chromosome 7. Thai patients were previously reported by clinical diagnosis. This study reports the first two cases of WS with ELN deletion diagnosed by fluorescent in situ hybridization (FISH) technique. Clinically, hyperacusis is a common finding in WS associated with otitis media. Neither of the patients had hyperacusis, but one of them had bilateral sensorineural hearing loss, which to our knowledge, has never been reported.

Disseminated intravascular coagulation findings in 100 patients.

A retrospective study of 100 patients with disseminated intravascular coagulation from 1993 to 1997 is reported. Forty-five patients were neonates with a mean age of 12.6 days and 55 patients were infants, children and adolescents with a mean age of 6 years and 3 months. Most of them (91.5%) had complicated underlying conditions which included congenital anomalies, prematurity, malignancy, hematological and various diseases. Additionally, every patient had triggering conditions commonly identified as gram-negative septicemia. Bleeding and thromboembolic manifestations were found in 59.4 per cent and 19.8 per cent, respectively. The laboratory findings revealed red blood cell fragmentation, 89.6 per cent and thrombocytopenia, 85.8 per cent. Natural anticoagulants were studied in a few cases and revealed low levels of antithrombin III and protein C. The prompt effective management included treatment of underlying diseases, identification and relief of triggering conditions, correction of thrombocytopenia and coagulopathy, and fully supportive care. The overall case-fatality rate was 41.6 per cent which was not correlated with age, underlying diseases, triggering conditions, manifestation of bleeding, thromboembolism or shock, and exchange transfusion. However, a significant lower case-fatality rate was found in patients with positive culture (25%) as compared to those with sepsis and negative culture (51.7%) (p = 0.044). In addition, the febrile neutropenic patients, who showed good response to the administrated granulocyte-colony stimulating factor (G-CSF), survived from the DIC.

Factors affecting mortality in Ebstein's anomaly of the tricuspid valve.

Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect. Twenty-one patients (11 boys and 10 girls) with Ebstein's anomaly were reviewed regarding clinical characteristics and factors related to the outcome of this lesion. Their ages at presentation ranged from 1 day to 13 years (median = 11 months). Eleven patients presented in infancy period, 6 of them were neonates. Common clinical findings were systolic murmur (85.7%) and cyanosis (57.1%). Laboratory findings included cardiomegaly on chest X-rays (95.2%), right bundle branch block pattern (76.2%) and right atrial enlargement (61.9%) on electrocardiography. Diagnosis and grading of severity were established by echocardiography. Among 21 patients, 4 were lost during follow-up. Seventeen patients were followed for 3-72 months. Six patients (28.6%) required surgery, 5 of whom died following surgery. Two patients died during the medical follow-up. Factors affecting cardiac death were the younger age at presentation, onset of cyanosis in infancy period, associated PS or PA, the lower insertion of the septal leaflet of the tricuspid valve and the higher ratio of the combined area of right atrium and atrialized right ventricle to that of functional right ventricle and left heart chambers.

Chromosome 22q11 deletion syndrome: the first three cases reported in Thailand.

The DiGeorge, velocardiofacial, and conotruncal anomaly face syndromes were originally described as separate disorders due to different concerns regarding phenotypes. However, all these disorders have some common clinical manifestations, including congenital heart defect, facial anomaly, and developmental delay. It is now clear that most cases of these syndromes have a common cause resulting from microdeletion of chromosome 22q11. This study reports the first three cases of Thai children presented with developmental delays. All are females who were known cases of congenital heart diseases. Their minor facial anomalies were subtle and not previously recognized as of any syndromes. The chromosome study by fluorescent in situ hybridization technique yielded microdeletion of chromosome 22q11. Without known prevalence in Asian populations, except in Japanese children, further study for chromosome 22q11 deletion syndrome in Asian children with conotruncal heart defects, who also have minor facial anomalies or developmental delays, should be undertaken.

Balloon pulmonary valvuloplasty in children, one year follow-up experience at Ramathibodi Hospital.

Balloon valvuloplasty has become the treatment of choice in pulmonary valvular stenosis. The objective of this report is to review the experience of this procedure in children at the Department of Pediatrics, Ramathibodi Hospital in the past 4 years (1991-1994) with at least one year follow-up. During the study period, 19 children aged 2.1-14.3 years (mean 5.11 years) with the diagnosis of pulmonary valvular stenosis had successful pulmonary balloon valvuloplasty at this institution. The immediate peak systolic pressure gradient across the pulmonary valve by cardiac catheterization decreased from 92.05 +/- 46.92 to 34.26 +/- 25.30 mmHg, by Doppler from 78.58 +/- 26.55 to 34.83 +/- 15.60 mmHg. Peak pressure gradient across the pulmonary valve by Doppler of the last examinations, one to 4 years after the procedure (mean 1.9 years), was 23.05 +/- 9.40 mmHg in 17 patients. The mean ratio of balloon size and pulmonary valve ring was 1.06. One patient still has a residual gradient of 54 mmHg and two were lost to follow-up. No serious complication was encountered during the procedures. CONCLUSION: Balloon pulmonary valvuloplasty is effective in the management of children with valvular pulmonary stenosis. The intermediate term result is excellent.

Spontaneous closure of small ventricular septal defect first six months of life.

This study was designed to determine the incidence, types of the defects and the rate of spontaneous closure of the small ventricular septal defect (VSD) in infants who were born at Ramathibodi Hospital during the one year period April 1, 1991 to March 31, 1992. The diagnosis was confirmed by 2D-echocardiogram and Doppler and color flow imaging. RESULT: During the study period, the 15 infants diagnosed with small VSD, an incidence of 1.95 per 1,000 livebirths. The male to female ratio was 9 to 6. There were 11 muscular and 4 membranous VSD. Small VSD had a maximal diameter of 3 mm. of less. Spontaneous closure occurred in 9 to 15 (60%) during 6 months follow-up. The small muscular VSD spontaneously closed in 9 of 11 (82%) patients. CONCLUSION: This data supports the previous studies that the rate of spontaneous closure of small VSD is high and the muscular defect has a higher incidence of spontaneous closure.

Malignant pericardial tumor in childhood: a case report diagnosed by cross-sectional echocardiogram and magnetic resonance imaging.

Since primary malignant pericardial tumors are seldomly found in children, we reported a case with massive pericardial effusion. Pericardial tumor was diagnosed by cross-sectional echocardiogram. The extent of the tumor was well demonstrated by magnetic resonance imaging. The tumor was removed successfully and proved to be low grade fibrosarcoma. Modern technics can be very helpful in the diagnosis of pericardial tumor.