Low serum albumin levels prior to pediatric allogeneic HCT are associated with increased need for critical care interventions and increased 6-month mortality.

Poor nutritional status in HCT patients is a negative prognostic factor. There are no pediatric studies evaluating albumin levels prior to HCT and need for critical care interventions. We hypothesized that pediatric patients with low albumin levels, routinely measured 30 days (±10 days) prior to allogeneic HCT, have a higher risk of critical care interventions in the post-transplant period. We performed a 5-year retrospective study of pediatric patients who underwent allogeneic HCT for any indication. Patients were categorized based on albumin level. Hypoalbuminemia was defined as <3.1 g/dL. A total of 73 patients were included, with a median age of 7.4 years (IQR 3.3, 13.2). Patients with hypoalbuminemia had higher needs for critical care interventions including non-invasive ventilation (44% vs 8%, P=.01), mechanical ventilation (67% vs 17%, P<.01), and vasoactive therapy (56% vs 16%, P=.01). Patients with hypoalbuminemia also had a higher 6-month mortality (56% vs 17%, P=.02). Our data demonstrate that children undergoing allogeneic HCT with hypoalbuminemia in the pretransplant period are more likely to require critical care interventions and have higher 6-month mortality. These findings identify an at-risk population in which nutritional improvements may be instituted prior to HCT in hopes of improving outcomes.

Clinical significance of serum cortisol levels following surgery for congenital heart disease.

OBJECTIVE: Use of hydrocortisone to treat refractory haemodynamic instability in patients recovering from surgery for congenital heart disease is common practice at many centres. We aimed to determine whether there is a relationship between total serum cortisol concentrations and haemodynamic response to this therapy. Material and methods We retrospectively reviewed patients <21 years who underwent cardiac surgery from 2011 to 2013, received hydrocortisone within 72 hours postoperatively, and had total serum cortisol measurements contemporaneous with its administration. Favourable responders were defined as patients in whom, at 24 hours after hydrocortisone initiation, either (1) systolic blood pressure was increased or unchanged and vasoactive-inotrope score was decreased or (2) systolic blood pressure increased by ⩾10% of baseline and vasoactive-inotrope score was unchanged. Variables were compared using t-tests or Mann-Whitney U tests as appropriate. RESULTS: In total, 24 patients were reviewed, with a median age of 1.4 months and range of 0.1-232 months. Among them, 14 (58%) patients responded favourably to hydrocortisone. At 24 hours, the median change in vasoactive-inotrope score was -18% in favourable responders and +31% in those who did not respond favourably, p=0.001. The mean pre-hydrocortisone total serum cortisol in favourable responders was 17.4±10.9 µg/dl compared with 46.1±44.7 µg/dl in those who did not respond favourably, p=0.03. CONCLUSION: Total serum cortisol obtained before initiation of hydrocortisone was significantly lower in patients who responded favourably to this therapy. Total serum cortisol may therefore be helpful in identifying children recovering from cardiac surgery who may or may not haemodynamically improve with hydrocortisone.

Devastating Ischemic Stroke Following Selective Arterial Embolization of a Large Chest Wall Aneurysmal Bone Cyst.

Aneurysmal bone cysts (ABC) are benign bone lesions found in children and young adults. Rarely, these lesions can arise from ribs, and there is disagreement on the best treatment because of proximity to vital structures. Frequently, surgeons remove ABC with en bloc resection. Selective arterial embolization has been used as an adjunct to surgery, or rarely as the primary treatment. We report a case of embolic stroke complicating embolization of a rib ABC, likely from the presence of collateral circulation between the mass and vertebral artery. Caution should be taken when performing embolization of lesions in this location because of potential complications.

Brain Death Secondary to Rocky Mountain Spotted Fever Encephalitis.

A two-year-old female presented with acutely altered mental status following eight days of fever and rash. She had been camping at an Indiana campground 11 days prior to the onset of illness and was evaluated twice for her fever and rash prior to admission. Laboratory evaluation on admission revealed thrombocytopenia, hyponatremia, and elevated transaminases. The patient developed diffuse cerebral edema, and despite intensive care, the edema led to brain death from Rocky Mountain spotted fever (RMSF). We present this case to highlight the importance of considering RMSF and other tick-borne illnesses in a child with prolonged fever and rash in a nonendemic area and also the difficulty of diagnosis in early stages of disease. A detailed travel history, evaluation of key laboratory findings (white blood count, platelet count, and transaminases), and close follow-up if rash and fevers persist may help to improve detection of RMSF. If a tick-borne illness such as RMSF is suspected, empiric doxycycline therapy should be started immediately, as lab confirmation may take several days and mortality increases greatly after five days of symptoms.

Food Protein-Induced Enterocolitis Syndrome Causing Hypovolemic Shock and Methemoglobinemia.

A 5-week-old previously healthy male presented with vomiting and diarrhea leading to hypovolemic shock and profound metabolic acidosis. He was subsequently found to have severe methemoglobinemia. The acidosis and shock improved with fluid resuscitation and methemoglobinemia was successfully treated with methylene blue. An extensive workup, including evaluations for infectious and metabolic etiologies, was unremarkable. However, a detailed dietary history revealed a recent change in diet, supporting a diagnosis of food protein-induced enterocolitis syndrome (FPIES). We present this case to highlight the importance of considering FPIES in an infant with vomiting and diarrhea, in the setting of a recent dietary change, leading to profound dehydration, metabolic acidosis, and methemoglobinemia. Diagnosis of FPIES, although difficult to make and one of exclusion, can be potentially life-saving.

Thiamine Deficiency Leading to Refractory Lactic Acidosis in a Pediatric Patient.

Thiamine plays a critical role in energy metabolism. Critically ill children and adults may develop thiamine deficiency with ultimately increased mortality due to potentially irreversible consequences of severe type B lactic acidosis. We report a case of an unvaccinated term neonate with malignant pertussis requiring extracorporeal membrane oxygenation and continuous renal replacement therapy, who developed profound lactic acidosis of unknown etiology. After countless evaluations for likely causes, the patient was ultimately determined to have thiamine deficiency and her acidosis resolved rapidly with vitamin supplementation.