ESBL Gram-negative sepsis is a substantial problem in neonatal infections. Maternal exposure to cephalosporins and maternal PPROM are important risk factors for ESBL Gram-negative EOS.
Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis.
Thrombocytopenia absent radius (TAR) syndrome is a very rare and infrequently seen congenital disorder with an approximate frequency of 0.42/100,000 live births. It is associated with bilateral absence of radii, hypo-megakaryocytic thrombocytopenia, and presence of both thumbs. The other systems which are affected by TAR syndrome include skeletal, hematologic, and cardiac systems. Intracranial hemorrhages due to thrombocytopenia and cardiac disorders are a common association usually seen with this syndrome and are usual cause of death. We describe a 3-month-old infant who was diagnosed with TAR syndrome on the basis of clinical features (thrombocytopenia and bilateral absent radius bone and confirmed by genetic analysis). The patient was diagnosed to have Tetralogy of Fallot, for which the infant was managed with definitive repair and thrombocytopenia was managed with platelet transfusion. Infants with TAR syndrome should be assessed for other associated malformations of various systems and followed up regularly and parents should be counseled for associated expected complications in these patients. We report an infant with TAR syndrome with Tetralogy of Fallot, which has not been reported in medical literature until now and this is the first case of its type.
Aim
Heart failure is a global problem that is increasing in prevalence. We undertook the initiative to compile the Vellore Heart Failure Registry (VHFR) to assess the clinical profile, mortality, risk factors and economic burden of heart failure by conducting a prospective, observational, hospital-based cohort study in Vellore, Tamil Nadu.
Methods and results
This study was a prospective observational cohort study conducted at the Christian Medical College and Hospital, Vellore, between January 2014 and December 2016. A total of 572 patients who satisfied the Boston criteria for “definite heart failure” were included and the primary outcome was all-cause mortality. The median duration of hospital stay was eight days and the in-hospital, one, three and six month mortalities were 13.25%, 27.3%, 32.53% and 38.15%, respectively. The median duration of survival was 921 days. Readmission for heart failure constituted 42%, and the most common cause of decompensation was an infection(31.5%). The presence of cyanosis at admission, history of previous stroke or transient ischemic attack, and American College of Cardiology (ACC)/American Heart Association (AHA) stage D at the time of discharge were independently associated with mortality at six months. The median total direct cost of admission was INR 84,881.00 ($ 1232.34)
Conclusion
The VHFR cohort had younger, more diabetic, and fewer hypertensive subjects than most cohorts. Admission for heart failure is a catastrophic health expenditure. Attempts should be made to ensure a reduction in readmission rates by targeting goal-directed therapy. As the most common cause of acute decompensation is pneumonia, vaccinating all patients before discharge may also help in this regard.
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