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The article focuses on the dysmorphic features of congenital nephritic sysndrome (CNS) and its consideration as the cause of death in early infancy. Topics discussed include an overview of CNS, the diagnosis of CNS, its causes, its symptoms, its transferrin mutation, its treatment options, and its manifestations.

The article presents a case study of a boy diagnosed with congenital nephritic syndrome (CNS) with dysmorphic features as well as its consideration as the cause of death in early infancy. Topics discussed include an overview of CNS, the diagnosis of the boy's CNS, its causes, its symptoms, its treatment, its manifestations, and the medical history of the patient.

Abstract: Objective: Because of their poor clinical status, infants may require surgery for congenital heart disease regardless of weight or prematurity. This retrospective review describes a single-centre experience with open-heart surgery in low-weight infants. Methods: From November 1997 to December 2006, 411 open-heart surgery procedures were performed in neonates. This included 46 consecutive infants weighing less than 2500g, who underwent cardiopulmonary bypass for correction of congenital heart defects (n =34) or Norwood stage I palliation of hypoplastic left heart syndrome (HLHS) (n =12). In the low-weight group were 23 males and 23 females with a median age of 10 days and a median weight of 2.26kg (range: 1.28–2.49kg). Results: Early mortality was 8.2% in patients weighing more than 2.5kg and 13% in the low-weight group. Within the low-weight group, weight at surgery, history of prematurity and prevalence of additional extracardiac malformations did not influence early mortality. At a median follow-up time of 32 months overall mortality was 21%. Thirty-four patients had a neurological follow-up examination 30 months postoperatively. Of the 34 survivors, 11 showed neurological deficits. Conclusions: In our patient population, early mortality was higher for infants weighing less than 2.5kg. However, within the low-weight group, lower weight at surgery or history of prematurity was not associated with a higher mortality or bad neurological outcome. [Copyright &y& Elsevier]