Successful hybrid management of hypertensive patent ductus arteriosus with unilateral absence of the right pulmonary artery - A case report.

Unilateral absence of pulmonary artery (UAPA) can be either isolated or associated with other congenital cardiac defects. It is a rare congenital lesion with a diverse clinical presentation. We present a case of absent right pulmonary artery and large patent ductus arteriosus (PDA) with severe unilateral pulmonary hypertension. We describe our strategy of staged ductus arteriosus closure in this patient and successful outcome on long term follow up.

Hyperviscosity syndrome revisited.

Secondary erythrocytosis occurs in cyanotic heart disease as a physiological response to chronic hypoxia, and this leads to hyperviscosity and various complications of the same. Microvascular stasis due to hyperviscosity results in symptoms including headache, fatigue, paraesthesia, and loss of vision. An important and dreadful feature of hyperviscosity is overt thrombosis in organ systems, resulting in cerebrovascular accident and myocardial infarction. Limited body iron store in a state of secondary erythrocytosis brings forth iron-deficient microcytic red cells; these being more rigid and less deformable than normocytic cells, further aggravate vascular occlusion. The management of hyperviscosity syndrome starts with intravenous hydration and correction of latent iron deficiency. However, therapeutic phlebotomy may be employed as a rescue measure if symptoms persist despite correction of dehydration and anemia. We present a series of four patients with uncorrected cyanotic congenital heart disease who presented with a spectrum of features of hyperviscosity and discuss pathophysiology, clinical features, and management of hyperviscosity in detail.

Are we ignoring coexisting rhabdomyolysis as an important aggravating factor for acute kidney injury among childhood diabetic ketoacidosis?

OBJECTIVES: Although Acute Kidney Injury (AKI) has been described among childhood diabetes ketocidosis (cDKA) there is scarcity of literature on the role of concomitant rhabdomyolysis. METHOD: A retrospective chart review was undertaken (2014-2018) to identify cDKA who developed AKI and had evidence of rhabdomyolysis defined by serum creatine phosphokinase (CPK) > 5 times upper limit of normal. RESULT: 46 cDKA were identified. Ten (22%) developed AKI with 6/10 reaching peak AKI Stage 3 and 8/10 had co-current rhabdomyolysis. In comparison to non rhabdomyolysis group, cDKA with rhabdomyolysis were at presentation significantly more likely to be hypotensive and have higher corrected sodium and calculated osmolality. Subsequently they were more likely to develop lower trough potassium levels during treatment. Five patients, all with rhabdomyolysis, needed dialysis: median duration 9 days (range 4-35). Three children in our cohort died, all from infection complications during treatment, one in AKI only group who did not receive dialysis and two in AKI with rhabdomyolysis on dialysis. CONCLUSION: Rhabdomyolysis was common among our cohort of cDKA with AKI and was associated with high morbidity and mortality. Rapid flux in electrolytes and osmolality may be important precipitating factors. We recommend larger prospective studies exploring the importance of rhabdomyolysis among cDKA with AKI.

Life-threatening Episodes of Malignant Hyperthermia Following Halothane Anesthesia in Three Children: A Case Series and Review of Literature.

Malignant hyperthermia (MH) is an inherited, pharmacogenetic disorder of the skeletal muscle, characterized by dangerous hypermetabolic state after anesthesia with succinylcholine and/ or volatile halogenated anesthetic agents, clinically manifested as hyperpyrexia and related complications like tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, rigid muscles, rhabdomyolysis and disseminated intravascular coagulation (DIC). Here we present a series of three cases of MH, admitted in our hospital in a span of 8 months for three different operative procedures to be done under general anesthesia (cleft lip repair, Duhamel's operation for Hirschsprung's disease and surgical repair of development dysplasia of hip), who developed probable hyperthermia owing to Halothane being used as an anesthetic agent. HOW TO CITE THIS ARTICLE: Laha S, Giri PP, Saha A, Gupta PP, De A. Life-threatening Episodes of Malignant Hyperthermia Following Halothane Anesthesia in Three Children: A Case Series and Review of Literature. Indian Journal of Critical Care Medicine, January 2019;23(1):47-50.