A rare cause of ischemic heart failure in a neonate.

Herein, we present a case of ischemic heart failure that occurred immediately after birth in a neonate due to coronary artery fistula (CAF) from the left main coronary artery to the left atrial appendage associated with high pulmonary artery pressure. Ischemic heart failure in a neonate with a structurally normal heart is rare. Furthermore, CAF resulting in ischemic heart failure is very rare in neonates. We believe that the small CAF caused symptoms during the first few days of life due to moderate pulmonary hypertension which resulted in a low cardiac output. The coronary perfusion improved after the normalization of the pulmonary blood pressure and improvement of the cardiac output. Echocardiography is helpful when a CAF is suspected and can be confirmed using a cardiac computed tomography scan. Small CAFs are unlikely to cause symptoms in infants, provided there are no other factors affecting the cardiac output status.

Refractory Kawasaki Disease-a Challenge for the Pediatrician.

Kawasaki disease (KD) is an acute, self-limiting febrile illness of childhood associated with vasculitis, mainly of the medium-sized arteries. The clinical significance and impact of this condition arise from its predilection for the coronary arteries. The criteria for classic Kawasaki disease are clearly defined, but many children present with atypical forms, and clinicians need to consider this possibility. Although most diagnosed cases respond to intravenous immunoglobulin (IVIG) and aspirin, some have proven resistant to the standard treatment. This article aims to provide a brief overview of Kawasaki disease, focusing on the resistant/refractory cases, and the treatment options available for such cases.

Stridor in an 11-year-old child.

Stridor in older children can be due to diverse aetiology that includes infections, anaphylaxis and rarely systemic conditions leading to hypocalcaemia. We report the case of a previously asymptomatic 11-year-old girl who presented to the casualty with stridor due to hypocalcaemia. The aetiological investigations for hypocalcaemia uncovered previously undetected chronic renal failure, possibly due to a rare autosomal recessive condition called nephronophthisis. We report this case to highlight the importance of widening the diagnostic focus for children presenting with stridor, especially when they fall outside the usual age group for infections like croup, or when the history is atypical.

N-terminal arm of Mcm1 is required for transcription of a subset of genes involved in maintenance of the cell wall.

The yeast Mcm1 protein is a member of the MADS box family of transcription factors that interacts with several cofactors to differentially regulate genes involved in cell-type determination, mating, cell cycle control and arginine metabolism. Residues 18 to 96 of the protein, which form the core DNA-binding domain of Mcm1, are sufficient to carry out many Mcm1-dependent functions. However, deletion of residues 2 to 17, which form the nonessential N-terminal (NT) arm, confers a salt-sensitive phenotype, suggesting that the NT arm is required for the activation of salt response genes. We used a strategy that combined information from the mutational analysis of the Mcm1-binding site with microarray expression data under salt stress conditions to identify a new subset of Mcm1-regulated genes. Northern blot analysis showed that the transcript levels of several genes encoding associated with the cell wall, especially YGP1, decrease significantly upon deletion of the Mcm1 NT arm. Deletion of the Mcm1 NT arm results in a calcofluor white-sensitive phenotype, which is often associated with defects in transcription of cell wall genes. In addition, the deletion makes cells sensitive to CaCl2 and alkaline pH. We found that the defect caused by removal of the NT arm is not due to changes in Mcm1 protein level, stability, DNA-binding affinity, or DNA bending. This suggests that residues 2 to 17 of Mcm1 may be involved in recruiting a cofactor to the promoters of these genes to activate transcription.