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.

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.

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.

Hemostatic derangement in dengue haemorrhagic fever.

Forty children, aged 1/2-14 years, with serologically proven dengue haemorrhagic fever were daily studied for hemostatic tests. There were 4, 20 and 16 cases of grade I, II III respectively. Hemostatic derangements in DHF is a multifactorial mechanism. Vasculopathy, thrombocytopenia, platelet dysfunction were found in most cases. Mild to moderate degree of prothrombin complex deficiency was observed in 15% and 50% of grade II and grade III respectively while laboratory evidence of consumptive coagulopathy was noted in 30% of shock cases and 10% of non-shock cases. Hypofibrinogenemia and increased PTT are commonly seen in grade III reflect the presence of stimulation of intrinsic coagulation pathway probably from immunologic reaction. Frank DIC is very rarely observed. FDP is slightly increased but not as high as in classical DIC. Further study on the role of platelet-endothelial interaction should be elucidated including the efficient management to stop bleeding in severe shock cases.

The value of an indirect hemagglutination test for streptococcal antibodies in children with acute rheumatic fever.

This study was made to evaluate the value of the IHA test for streptococcal antibodies in children with acute rheumatic fever in comparison with the conventional ASO titer. The upper limit titers of ASO and IHA in control group were 170 and 640 respectively. All sera from 37 patients with rheumatic fever had IHA titers over 640. In contrast, the level of ASO titer above upper normal limits was found in only 62% of these cases. This report present data which favours the use of IHA test. Its sensitivity is very similar to that of the commercial Anti Streptozyme test. In addition, the test is simple and could be performed economically. More studies are needed to evaluate the test further. If the results proved as favourable as this report a negative test would be of a considerable value in excluding the diagnosis of acute, rheumatic fever especially in questionable cases.

Aetiologic agents of septic sore throat in Thai children.

A bacteriological study of children with respiratory infections in Bangkok during January to November 1976 revealed that 37% of the patients had symptoms and sign of bacterial pharyngotonsillitis. Twenty-six per cent of these children harboured Streptococcus pyogenes in their throats. The numbers of streptococci other than group A and Staphylococcus aureus were increased in the children with respiratory infections. However, Staph. aureus was found as the sole organism in children with exudate more often than in the children with only URI. The possible role of Staph. aureus in bacterial pharyngitis should not be ignored. Penicillin remains a drug of choice for the treatment of streptococcal pharyngitis. If penicillin is contraindicated, erythromycin should be preferred over lincomycin as a second choice of drug in order to avoid treatment failure if lincomycin resistant streptococci are present.