Hemolytic uremic syndrome associated with rhabdomyolysis - Need to look beyond kidneys in hemolytic uremic syndrome.

Hemolytic uremic syndrome, a part of thrombotic microangiopathy, is an important cause of acute kidney injury in children. Hemolytic uremic syndrome primarily targets kidney but extrarenal organ involvement is observed in 20-40% of patients. Extra-renal organ involvement in hemolytic uremic syndrome has been associated with greater disease severity and higher mortality. We describe a 31/2-year-old boy of hemolytic uremic syndrome with rhabdomyolysis, which is a rare extrarenal manifestation of hemolytic uremic syndrome. Unlike central nervous or gastrointestinal system involvement in hemolytic uremic syndrome which manifests clinically, muscle involvement may not and, if present, may worsen the existing acute kidney injury and may worsen disease prognosis. Considering the high morbidity and mortality in acute phase of hemolytic uremic syndrome, prompt evaluation to know the extent of extrarenal organ involvement at the earliest is important for management and prognosis of these patients.

Leukodystrophy presenting as acute-onset polyradiculoneuropathy.

BACKGROUND: Sulfatides, the most abundant glycosphingolipids, are a major component of myelin. They are degraded by the combined action of sphingolipid activator protein and arylsulfatase A. Deficiency of either of these entities causes metachromatic leukodystrophy (MLD). On the basis of age of onset, this entity is divided into late infantile, juvenile, and adult subtypes. Late infantile form, the commonest subtype, can exhibit peripheral neuropathy as the initial manifestation. The other two forms usually manifest peripheral neuropathy later in the disease course. PATIENT: A 1.5-year-old girl with preexisting isolated motor delay presented with acute-onset ascending flaccid quadriparesis, ptosis, and respiratory failure. Ptosis and respiratory failure responded completely to intravenous immunoglobulin, whereas quadriparesis showed minimal improvement. Nerve biopsy revealed metachromatic granules with demyelination, and serum arylsulfatase A levels were undetectable. CONCLUSION: The severity and nature of the disease coupled with the response to immunotherapy makes this case unusual. This child may represent either an atypical presentation of MLD with coincidental response to immunotherapy or an episode of immune mediated neuropathy in an individual with already diseased nerves due to MLD.

Pediatric Sepsis Guidelines: Summary for resource-limited countries.

JUSTIFICATION: Pediatric sepsis is a commonly encountered global issue. Existing guidelines for sepsis seem to be applicable to the developed countries, and only few articles are published regarding application of these guidelines in the developing countries, especially in resource-limited countries such as India and Africa. PROCESS: An expert representative panel drawn from all over India, under aegis of Intensive Care Chapter of Indian Academy of Pediatrics (IAP) met to discuss and draw guidelines for clinical practice and feasibility of delivery of care in the early hours in pediatric patient with sepsis, keeping in view unique patient population and limited availability of equipment and resources. Discussion included issues such as sepsis definitions, rapid cardiopulmonary assessment, feasibility of early aggressive fluid therapy, inotropic support, corticosteriod therapy, early endotracheal intubation and use of positive end expiratory pressure/mechanical ventilation, initial empirical antibiotic therapy, glycemic control, and role of immunoglobulin, blood, and blood products. OBJECTIVE: To achieve a reasonable evidence-based consensus on the basis of published literature and expert opinion to formulating clinical practice guidelines applicable to resource-limited countries such as India. RECOMMENDATIONS: Pediatric sepsis guidelines are presented in text and flow chart format keeping resource limitations in mind for countries such as India and Africa. Levels of evidence are indicated wherever applicable. It is anticipated that once the guidelines are used and outcomes data evaluated, further modifications will be necessary. It is planned to periodically review and revise these guidelines every 3-5 years as new body of evidence accumulates.

Management of hepatic encephalopathy in children.

Hepatic encephalopathy is a common metabolic condition in children, having a significantly different aetiopathogenesis from that in adults. The present paper reviews the medical interventions of proven efficacy and also discusses recent advances from various fields as applicable to management of children with this disorder, focusing on measures other than liver transplant. The most important component of managing a child with hepatic encephalopathy is basic intensive care with regulation of fluid status, glucose and electrolyte homeostasis. Specific management includes measures to reduce serum ammonia concentrations, and the prevention and prompt treatment of complications. Methods to reduce ammonia target various steps in its metabolism. This includes reducing its production in and absorption from the intestine and promoting its metabolism in the liver. Significant secondary complications occurring in fulminant hepatic failure which require urgent recognition and management include coagulopathy, cerebral oedema and renal dysfunction. Children with hepatic encephalopathy also have several other paediatric care issues such as fever, requirement for sedation, etc, where the choice of drug is not straightforward and is often different from other settings. This is reviewed here along with an attempt to provide rational choices based on available evidence. Certain controversial and experimental approaches to treatment of fulminant hepatic failure are also discussed, but clearly delineated from the established management protocol. Finally, the role of artificial liver support devices is discussed, with the realisation that they can provide an effective bridge during the time when a patient is waiting for a suitable donor for liver transplant.

Recent advances in sepsis and septic shock.

Sepsis remains a common problem in all age groups. Recently surviving sepsis campaign has taken up a worldwide initiative by publishing international guidelines 2008 with a hope to disseminate information regarding management of sepsis for all age groups. This article presents a review of recent advances as they apply to pediatric age group supported by the available evidence with reference to standard definitions of pediatric sepsis and septic shock and management in the emergency room and pediatric intensive care unit.