Double outlet right ventricle and aortopulmonary window in a neonate with Bohring-Opitz (Oberklaid-Danks) syndrome: First case report

Bohring-Opitz syndrome (BOS) is a rare, sporadic genetic disorder, characterized by feeding difficulties, developmental delay, flexion abnormalities, dysmorphic facial features and typical body posture (BOS posture). This syndrome is diagnosed on the basis of distinctive clinical features with or without confirmation by genetic studies. Cardiac abnormalities are seen in almost half of the patients, but are nonspecific. We present a case of a 3-week-old male baby with BOS who was referred to our hospital with congestive heart failure, seizures and failure to thrive. He was diagnosed to have double outlet right ventricle and aortopulmonary window (DORV and APW). To our knowledge, this is the first case of Bohring-Opitz Syndrome ever reported with such clinical presentation

Congenital heart disease in harlequin ichthyosis: Case series

Harlequin ichthyosis (HI) is the most severe form of congenital ichthyosis and inherited in an autosomal recessive manner. The disease is marked by severe thickened and scaly skin on the entire body. It is a lethal disease, but patients can rarely survive for several months or years with treatment. We present here seven cases of HI, where cardiac evaluation was done by echocardiography. To our knowledge, this is the first study to report associated cardiac abnormalities in such patients

Askin tumor: A rare neoplasm of thoracopulmonary region

Askin tumor is a rare neoplasm of thoracopulmonary region. But it mimics other common pediatric disorders, such as empyema, lymphoma, and tuberculosis, posing a great diagnostic and therapeutic challenge to the treating clinicians. So it is of utmost importance to make an early diagnosis and proper referral/treatment in such cases. We highlighted diagnostic challenge, treatment, and favorable outcome of a case that presented to us

Waste heat recovery from diesel engine using custom designed heat exchanger and thermal storage system with nanoenhanced phase change material

In this research study an attempt has been made to recover the heat energy of the exhaust gas from a Diesel engine, using a triangular finned shell and tube heat exchanger with segmental baffle at 20°, and efficiently store as sensible and latent heat energy using thermal storage tank having phase change material with CuO nanoparticles. The nanoparticles and the phase change material form the nanoparticle-enhanced phase change material and mainly the thermal conductivity of the phase change material can be enhanced through the dispersion of the nanoparticles. The temperature variations of the heat transfer fluid in the heat recovery heat exchanger with various load conditions of the Diesel engine are studied. The performance of the heat exchanger is evaluated using heat extraction rate and effectiveness. Evaluation of the performance of the thermal storage system can be analyzed by using the total heat energy stored and charging rate during the charging period for the selected nanoparticle-enhanced phase change material