Related Experiment Videos
[Growth in children with the exudative enteropathy syndrome due to a congenital heart defect--cor triatriatum
S Kolousková1, J Lebl, M Snajderová
12. dĕtská klinika 2. LF UK a FN, Praha-Motol.
Insights
Severe growth retardation in a child was linked to exudative enteropathy caused by a rare heart defect. Surgical correction of cor triatriatum dextrum led to rapid growth recovery.
Area of Science:
- Pediatric Endocrinology
- Pediatric Cardiology
- Gastroenterology
Background:
- Growth retardation is a significant indicator of underlying chronic pediatric diseases.
- Persistent growth failure necessitates comprehensive diagnostic evaluation.
Observation:
- A six-year-old boy exhibited severe growth retardation, dropping to the 3rd-10th percentile.
- Clinical signs included abdominal distension, chronic diarrhea, and refractory hypocalcemia with tetany.
- Laboratory findings revealed severe hypoproteinemia and markedly elevated fecal alpha-1-antitrypsin, indicative of exudative enteropathy.
Findings:
- Exudative enteropathy was diagnosed, stemming from venous congestion caused by cor triatriatum dextrum, a rare congenital heart anomaly.
- Surgical resection of the right atrial septum successfully addressed the underlying cardiac pathology.
Implications:
- This case highlights cor triatriatum dextrum as a rare but treatable cause of pediatric exudative enteropathy and growth failure.
- Timely surgical intervention can reverse severe malnutrition and promote catch-up growth in affected children.
- Integrated pediatric subspecialty care is crucial for diagnosing and managing complex cases involving cardiac and gastrointestinal manifestations.
Abstract:
Retarded growth in a child can be the sign of serious chronic disease. The authors present an account of a six-year-old boy where growth retardation persisted at least from the age of three. During this period his height dropped from the zone between the 25th and 50th percentile into the zone between the 3rd and 10th percentile. From the clinical point of view a large abdomen, loose stools and hypocalcaemia with tetany were striking, as they were moreover refractory to vitamin D2, calcitriol and calcium administration by the oral route. The authors revealed severe hypoproteinaemia, a 150 times increased value of alpha-1-antitrypsin in faeces, and exudative enteropathy syndrome was diagnosed. The cause was venous congestion due to a rare heart disease--cor triatriatum dextrum. The septum in the right atrium was resected. Immediately after surgery the consistency and frequency of stool decreased. Calcaemia and plasma protein levels reached normal levels within two months. A growth spurt of 11 cm/year followed. Fifteen months after operation the patient's height reached almost the 50th percentile.