Thyroid functions in children with Down's syndrome.

OBJECTIVE: To evaluate thyroid function in children with Down's syndrome, and to ascertain the presence of a relationship between overt thyroid diseases and congenital anomalies. MATERIAL AND METHOD: One hundred and forty Down's syndrome patients, aged from 3 days to 13 years 9 months, were evaluated for karyotype, thyroid functions and the coexistence of congenital anomalies. RESULTS: Trisomy 21 was found in the majority of cases (95.7%). Fifty-six patients (40%) had abnormal thyroid functions: 53 (37.9%) hypothyroidism and 3 (2.1%) hyperthyroidism. Ten patients (7.1%) were diagnosed with overt thyroid disease: congenital hypothyroidism 3.6%, acquired hypothyroidism associated autoimmune thyroiditis 1.4% and hyperthyroidism 2.1%. None of the patients with congenital hypothyroidism had athyreosis or ectopic thyroid gland. Sub-clinical hypothyroidism accounted for 32.9% of all cases; 10.7% showed a spontaneous decrease to normal TSH levels and 13.6% had persistently elevated TSH levels with the median follow-up time of 6 and 12 months, respectively. Congenital heart disease, gastrointestinal anomalies and hematological disease were found in 73.6, 10 and 3.6 percent of patients, respectively. There was no statistical correlation between the coexistence of cardiovascular or gastrointestinal disease in Down's syndrome patients with overt thyroid diseases or sub-clinical hypothyroidism to those having normal thyroid functions. CONCLUSION: Sub-clinical hypothyroidism was the most common thyroid abnormality in children with Down's syndrome. A longitudinal and timely-scheduled evaluation of thyroid function is needed to establish the natural course of this abnormality and the proper management guideline.

Factors determining immediate and medium-term results after pulmonary balloon valvuloplasty.

OBJECTIVE: To determine factors influencing immediate and medium-term results of Pulmonary Balloon Valvuloplasty (PBV) for pulmonary valve stenosis. MATERIAL AND METHOD: Between 1995 and 2001, the authors carried out PBV in 25 consecutive patients. Two treatment outcomes including immediate and medium-term results were analyzed. The immediate results were classified into two groups based on the pulmonary systolic pressure gradient (PG) immediately after dilation: group 1 with a PG < or = 35 mmHg and group II with a PG > 35 mmHg. At medium-term follow-up of 6 to 60 months, echocardiographic evaluations were analyzed and divided into two groups: group A with a PG < or = 25 mmHg and group B with a PG > 25 mmHg. Demographic characteristics and hemodynamic parameters of immediate and medium-term results were compared and analyzed. RESULTS: Immediately after PBV the right ventricular systolic pressure (RVSP) decreased from 115.3 +/- 37.6 mmHg to 67.0 +/- 28.5 mmHg (p < 0.001). The pulmonary systolic pressure gradient decreased from 90.4 +/- 37.9 mmHg to 39.3 +/- 25.6 mmHg (p < 0.001). Nine of the 25 patients (group 11; 36%), with incomplete immediate relief of the obstruction, had more symptoms, a higher baseline PG, higher right atrial pressure, higher RVSP, and a higher systolic pressure ratio. Six of the 25 patients (group B; 24%), with suboptimal medium-term results, had a higher right ventricular voltage on the electrocardiogram, higher pre-dilation PG, and higher RVSP. CONCLUSION: Successful medium-term outcomes following pulmonary balloon valvuloplasty were achieved in 76% of the patients, with a greater success rate (91%) in patients with a lower right ventricular voltage electrocardiogram (R wave amplitude in V1) < or = 21 mm, a prevalvuloplasty systolic gradient < or = 90 mmHg, and right ventricular systolic pressure < or = 125 mmHg.

A mediastinal mass resembling lymphoma: an unusual manifestation of probable case of invasive zygomycosis in an immunocompetent child.

A 2-year-old girl presented with prolonged fever and progressive dyspnea for 3 weeks. A chest radiograph revealed a left lung infiltrate and associated pleural effusion. Echocardiography revealed a large posterior mediastinal mass extending to the left atrial wall and massive pericardial effusion. The presumptive diagnosis was lymphoma. At operation, a large brownish-yellow mass was noted at the posterior mediastinum, with matted hilar, and subcarinal lymph nodes. Pericardial and pleural effusions with left lung consolidation were also noted. Histopathological examination of biopsy specimens revealed a granulomatous inflammatory reaction with a diffuse eosinophilic infiltrate and broad septated fungal hyphae with right angle branching compatible with zygomycosis. Surgical removal of the mass could not be performed due to the adjacent great vessels and carina. She subsequently died from airway obstruction and respiratory failure ten days later.

Pediatric heart surgery waiting time in Thailand and its effect on mortality: A cooperative study from Chulalongkorn, Children and Chiang Mai University hospitals.

BACKGROUND: Thai children who need cardiac surgery are often put on a waiting list. The waiting time and mortality during waiting have not been previously systematically studied. MATERIAL AND METHOD: A cooperative study was conducted at King Chulalongkorn Memorial hospital (Chula), Children hospital (CH), and Chiang Mai University hospital (CMU). The status of children who were referred for cardiac surgery at these hospitals in the year 1999-2000 (Chula and CMU) and the year 2000 (CH) were analyzed by Kaplan-Meier survival curve. The patients who lost to follow up and could not be contacted were censored at the time of last clinic visit. Log-Rank test was used to compare the survival curve and waiting time between three hospitals. RESULTS: The averaged annual referrals for cardiac surgery at the three hospitals were 846 cases (205 for Chula, 462 for CH and 179 for CMU). Mean age was 4.3 +/- 4.2 years and 51% were male. Follow up data were complete in 96.3%. Surgical procedures were correction of simple shunt lesions (ASD, VSD, AV canal) in 33.9%, close heart surgery (PDA ligation, coarctation repair, BT shunt) in 29.9%, total repair TOF in 19.6%, complex surgery in neonate and infants (arterial switch, TAPVR repair, Norwood procedure, truncus and interrupted aortic arch repair) in 4.2% and others in 12.3%. Median waiting time was 195 days and was significantly different between the three hospitals (p < 0.01). Mortality while waiting were approximately 5% at 2 years at Chula and CH, which was significantly higher than CMU (0%, p = 0.02). Further analysis revealed difference in age (lower Chula than CH than CMU) and types of surgery (more complex at Chula and CH) which may be the causes for difference in the mortality observed. CONCLUSION: Waiting time for cardiac surgery for children in Thailand is long and should be viewed as a problem in public health policy. Optimal waiting time at each hospital may not have to be the same, depending on the type and severity of the disease seen at each particular center Attempt should be made to solve this problem at the national level.

Pseudoaneurysm following modified Blalock-Taussig shunt: a rare complication mimicking pulmonary disease.

The authors report a pseudoaneurysm in a 2-year-old boy presenting with fever, increasing cyanosis and right upper lung shadowing on a chest radiograph at six weeks following modified Blalock-Taussig shunt surgery. Echocardiography and a CT scan of the chest revealed a large pseudoaneurysm originating from the right subclavian artery at the proximal insertion of modified Blalock-Taussig shunt. The patient underwent aneurysmal resection, Blalock-Taussig shunt removal, right subclavian artery ligation and the creation of a central shunt between the ascending aorta and main pulmonary artery. Unfortunately, the patient died 3 hours after the operation.

Isolated primary chylopericardium: a case report.

Isolated primary chylopericardium is a rare entity with an obscure etiology. The authors report a 10-week-old male infant presenting with tachypnea and enlarged cardiac silhouette. Echocardiography revealed a large pericardial effusion. A specific diagnosis of chylopericardium was made by pericardiocentesis and analysis of the fluid. Despite the pericardial tube drainage and medium-chain triglyceride diet, pericardial effusion reaccumulated. Ligation of the thoracic duct with the establishment of a pleuropericardial window was performed through a left thoracotomy. Follow-up echocardiograms have shown no reaccumulation of the pericardial fluid.