A Healthy Toddler With Fever and Lethargy.

A 21-month-old previously healthy girl presented to the emergency department initially with fever, rhinorrhea, and poor oral intake. She was subsequently discharged from the hospital on amoxicillin for treatment of acute otitis media but presented hours later on the same day with continued poor oral intake, decreased urine output, and lethargy. The patient was afebrile on examination without a focal source of infection or evidence of meningismus, but she was lethargic and minimally responsive to pain and had reduced strength in the upper and lower extremities. Initial laboratory analysis revealed leukocytosis with a neutrophil predominance and bandemia, hyponatremia, mild hyperkalemia, hyperglycemia, elevated transaminases, a mild metabolic acidosis, glucosuria, ketonuria, and hematuria. Follow-up tests, based on the history and results of the initial tests, were sent and led to a surprising diagnosis.

Metabolically based liver damage pathophysiology in patients with urea cycle disorders - A new hypothesis.

The underlying pathophysiology of liver dysfunction in urea cycle disorders (UCDs) is still largely elusive. There is some evidence that the accumulation of urea cycle (UC) intermediates are toxic for hepatocyte mitochondria. It is possible that liver injury is directly caused by the toxicity of ammonia. The rarity of UCDs, the lack of checking of iron level in these patients, superficial knowledge of UC and an underestimation of the metabolic role of fumaric acid, are the main reasons that are responsible for the incomprehension of the mechanism of liver injury in patients suffering from UCDs. Owing to our routine clinical practice to screen for iron overload in severely ill neonates, with the focus on the newborns suffering from acute liver failure, we report a case of citrullinemia with neonatal liver failure and high blood parameters of iron overload. We hypothesize that the key is in the decreased-deficient fumaric acid production in the course of UC in UCDs that causes several sequentially intertwined metabolic disturbances with final result of liver iron overload. The presented hypothesis could be easily tested by examining the patients suffering from UCDs, for liver iron overload. This could be easily performed in countries with a high population and comprehensive national register for inborn errors of metabolism. CONCLUSION: Providing the hypothesis is correct, neonatal liver damage in patients having UCD can be prevented by the supplementation of pregnant women with fumaric or succinic acid, prepared in the form of iron supplementation pills. After birth, liver damage in patients having UCDs can be prevented by supplementation of these patients with zinc fumarate or zinc succinylate, as well.

Life-threatening parapharyngeal and retropharyngeal abscess in an infant.

Severe extensive deep neck abscess in an infant is uncommon. We share the case of a previously well 4-month old infant who was referred for a 4-day history of fever, lethargy and left lateral neck swelling. Contrast-enhanced CT scan revealed a large 5.3×8 cm collection involving the left parapharyngeal and retropharyngeal space, causing significant airway narrowing. 40 mL of frank pus was drained via intraoral incision and drainage with the aid of endoscope, and undesirable complications from an external approach were averted. The infant was extubated 48 hours postsurgery and was discharged home well after completion of 1 week of intravenous antibiotics. The child was discharged well from our follow-up at 1 month review. We discuss the pathophysiology of deep neck space abscesses, its incidence in the paediatric population and the various management options.

A young boy with recurrent headache, lethargy, and hyponatremia.

BACKGROUND: To investigate and differentiate the causes of hyponatremia in an 8-y old boy. METHODS: An 8-y boy presented with headache, vomiting, and diplopia. Magnetic resonance imaging of the brain confirmed a mass in the pineal region. Pathology report demonstrated a mixed germ cell tumor with a yolk sac component. A multi-agent chemotherapy and radiation regimen was initiated. He developed hyponatremia, with sodium concentrations varying from 116 to 133 mEq/l. RESULTS: Serum levels of sodium, chloride, phosphorous, uric acid, and osmolality were low. Serum α-fetoprotein, ß-HCG, and lactate dehydrogenase were highly elevated. Urine sodium and osmolality were increased. CONCLUSIONS: These presentations suggest that the patient has cerebral salt-wasting syndrome caused by intracranial germ cell tumor. Recognition and differentiation of cerebral salt-wasting syndrome from other disorders are essential.

A Man in His 40s With Headache, Lethargy, and Altered Mental Status.

A man in his 40s presented with 1 month of worsening confusion, fatigue, and headache. Results from laboratory analyses were notable for a complete white blood cell count of 17 000/µL (31% blast cells), a platelet count of 76 000/µL, and a hemoglobin level of 16.6 g/dL. Imaging studies revealed a large mixed-attenuation subdural collection in the right frontal region with prominent mass effect. The patient underwent an emergency neurosurgical procedure. The differential diagnosis, pathologic findings, and diagnosis are discussed.

The lethargic diabetic: cerebral edema in pediatric patients in diabetic ketoacidosis.

Diabetic ketoacidosis (DKA) is the leading cause of hospitalizations for pediatric patients with diabetes mellitus. The most severe complication of DKA is cerebral edema that may lead to brain herniation. We present a case report that highlights the subclinical presentation of DKA-related cerebral edema in a pediatric patient and review the acute care management of suspected cerebral edema during transport.

Prevalence, clinical predictors, and outcome of hypocalcaemia in severely-malnourished under-five children admitted to an urban hospital in Bangladesh: a case-control study.

Hypocalcaemia is common in severely-malnourished children and is often associated with fatal outcome. There is very limited information on the clinical predicting factors of hypocalcaemia in hospitalized severely-malnourished under-five children. Our objective was to evaluate the prevalence, clinical predicting factors, and outcome of hypocalcaemia in such children. In this case-control study, all severely-malnourished under-five children (n=333) admitted to the Longer Stay Ward (LSW), High Dependency Unit (HDU), and Intensive Care Unit (ICU) of the Dhaka Hospital of icddr,b between April 2011 and April 2012, who also had their total serum calcium estimated, were enrolled. Those who presented with hypocalcaemia (serum calcium <2.12 mmol/L) constituted the cases (n=87), and those admitted without hypocalcaemia (n=246) constituted the control group in our analysis. The prevalence of hypocalcaemia among severely-malnourished under-five children was 26% (87/333). The fatality rate among cases was significantly higher than that in the controls (17% vs 5%; p < 0.001). Using logistic regression analysis, after adjusting for potential confounders, such as vomiting, abdominal distension, and diastolic hypotension, we identified acute watery diarrhoea (AWD) (OR 2.19, 95% CI 1.08-4.43, p = 0.030), convulsion on admission (OR 21.86, 95% CI 2.57-185.86, p = 0.005), and lethargy (OR 2.70, 95% CI 1.633-5.46, p = 0.006) as independent predictors of hypocalcaemia in severely-malnourished children. It is concluded, severely-malnourished children presenting with hypocalcaemia have an increased risk of death than those without hypocalcaemia. AWD, convulsion, and lethargy assessed on admission to hospital are the clinical predictors of hypocalcaemia in such children. Presence of these features in hospitalized children with severe acute malnutrition (SAM) should alert clinicians about the possibility of hypocalcaemia and may help undertake potential preventive measures, such as calcium supplementation, in addition to other aspects of management of such children, especially in the resource-poor settings.