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.

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.

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.