Rare diagnosis of Melkersson-Rosenthal syndrome in a paediatric patient.

Melkersson-Rosenthal syndrome (MRS) is a rare neurocutaneous syndrome characterised by the triad of recurrent orofacial swelling, facial nerve palsy and fissured tongue. This diagnosis is particularly rare in children. We aim to increase awareness of the syndromic association of these clinical features since most patients present with a monosymptomatic form, reiterating the importance of detailed history and thorough physical examination, for the timely identification of these patients. Not only the recurring of symptoms, but also the association of MRS with other medical conditions, make 'earlier' diagnosis of the Syndrome beneficial. The average delay in diagnosis is 4-9 years. Although most cases resolve without treatment, when treated,steroids are most commonly used. Variable options have been tried for refractory and frequently recurrent cases. We present a case of MRS in a 12-year-old girl, diagnosed 3 years after onset of symptoms. We reviewed updated literature for MRS and associated clinical conditions as well as published treatment options.

Necrotizing Fasciitis of the Abdominal Wall Caused by Serratia Marcescens.

In this article, we present the first case of necrotizing fasciitis affecting the abdominal wall caused by Serratia marcescens and share results of a focused review of S. marcescens induced necrotizing fasciitis. Our patient underwent aorto-femoral bypass grafting for advanced peripheral vascular disease and presented 3 weeks postoperatively with pain, erythema and discharge from the incision site in the left lower abdominal wall and underwent multiple debridement of the affected area. Pathology of debrided tissue indicated extensive necrosis involving the adipose tissue, fascia and skeletal muscle. Wound cultures were positive for Serratia marcescens. She was successfully treated with antibiotics and multiple surgical debridements. Since necrotizing fasciitis is a medical and surgical emergency, it is critical to examine infectivity trends, clinical characteristics in its causative spectrum. Using PubMed we found 17 published cases of necrotizing fasciitis caused by Serratia marcescens, and then analyzed patterns among those cases. Serratia marcescens is prominent in the community and hospital settings, and information on infection presentations, risk factors, characteristics, treatment, course, and complications as provided through this study can help identify cases earlier and mitigate poor outcomes. Patients with positive blood cultures and those patients where surgical intervention was not provided or delayed had a higher mortality. Surgical intervention is a definite way to establish the diagnosis of necrotizing infection and differentiate it from other entities.

Intracranial abscess due to Mycobacterium avium complex in an immunocompetent host: a case report.

BACKGROUND: Mycobacterium avium complex (MAC) is a ubiquitous pathogen, widely distributed in the environment including water, soil and animals. It is an uncommonly encountered clinical pathogen; primarily causing pulmonary infections in patients with underlying lung disease or disseminated disease in immunocompromised hosts. Sporadically, extra-pulmonary infections have been documented including involvement of the liver, spleen, skin, soft tissue and lymph nodes. Central nervous system (CNS) infections due to MAC are exceedingly rare and carry a poor prognosis. Additionally, such infections are largely reported in patients infected with HIV. Herein we report the first case of intracranial abscess due to MAC in an immunocompetent man with a normal CD4 count and negative HIV status. CASE PRESENTATION: A previously healthy 40-year-old male presented to us with progressively worsening CNS symptoms. The patient's presentation was uncharacteristic of MAC infection in immunocompetent hosts, as he developed subacute, progressive symptoms that included severe frontal headaches, left eyelid swelling, blurry vision, and diplopia, without any pulmonary or systemic manifestations. Neuroimaging revealed multiple ring-enhancing lesions, which required neurosurgical intervention. MAC was the only pathogen that grew from intraoperative tissue cultures. The patient was subsequently treated with a 12-month regimen consisting of Clarithromycin, Ethambutol, and Rifampin, with successful clinical resolution. CONCLUSION: Our findings indicate that it is important to consider rare infections such as MAC in immunocompetent patients, regardless of atypical symptoms. Despite the severity of this infection, with timely diagnosis effective treatment is available.

Evaluating the safety and efficacy of Glucommander, a computer-based insulin infusion method, in management of diabetic ketoacidosis in children, and comparing its clinical performance with manually titrated insulin infusion.

The Glucommander is a computer-based system for directing intravenous insulin infusion. Using a physician-selected glucose target range and a weight-based multiplier, it recommends an insulin infusion rate and interval to next glucose measurement. We evaluated the safety and efficacy of this system by conducting a retrospective chart review of 65 new-onset or existing type 1 diabetic children, admitted with diabetic ketoacidosis (DKA), managed with the Glucommander. We compared outcomes with 22 patients managed using manually titrated infusion. Time to glycemic control and correction of acidosis, number of insulin units used per kilogram per hour, and length of pediatric intensive care unit (PICU) and total hospital stay were analyzed using measures of central tendency. Children managed with Glucommander achieved equally rapid glycemic control and correction of acidosis, used less intravenous insulin, and spent less time in both PICU and hospital overall, compared to those managed with manual insulin infusion.

Ketamine reduces the cell death following inflammatory pain in newborn rat brain.

Premature infants experience untreated repetitive pain that may alter their brain development. Effects of ketamine and repetitive pain on cellular death and subsequent behavior were studied in neonatal rats. Rat pups were randomized to undisturbed controls (C), 4% formalin injection (F), ketamine alone (K, 5 mg/kg) or formalin plus ketamine (KF) and were assessed for neuroactivation with Fos protein, cellular death with FluoroJade-B, cognition with the radial arm maze, and pain thresholds with the hot-plate. Greater Fos expression and cell death occurred in F vs. C groups in defined brain areas at 1 and 4 h in F compared with other groups. Cell death was accentuated 3.3-fold in cortical areas and 1.6-fold in subcortical areas in the F compared with the C group following repetitive pain and sacrifice 18-20 h later. These effects were ameliorated by ketamine. Compared with the F group, all other groups demonstrated greater exploratory and rearing behaviors and decreased time for bait consumption at 1-h and 3-h intervals. Significantly greater thermal pain latencies occurred in the KF and F groups. Repetitive neonatal pain accentuates neuronal excitation and cell death in developmentally regulated cortical and subcortical areas, which decreases the acquisition of visual-spatial clues, short-term and long-term memory, and increases pain latencies. Ketamine analgesia mitigates most of these effects.

Case of the month. Symptomatic chronic calcified subdural hematoma in a 5-year-old child.

Head trauma is a frequent cause of morbidity and mortality in the pediatric population. The pathophysiology and clinical outcomes of head trauma differ between children and adults. Traumatic mass lesions such as subdural and epidural hematomas occur less frequently in children and, when present, are associated with lower mortality. Subdural hematoma is the collection of blood on the cortical surface beneath the dura with bleeding from bridging veins or cortical arteries. These hematomas are usually associated with trauma. Chronic subdural hematomas are much more common in infants and frequently exist as a single entity; it is rare for chronic subdural fluid accumulations to occur after one year of age. Specific traumatic events are usually unrecognized or unreported. In rare instances, subdural hematoma may indicate an underlying bleeding disorder, hematological malignancy or benign expansion of subarachnoid space. We report the dramatic presentation of a strikingly large calcified chronic subdural hematoma in a 5-year-old with increased intracranial pressure and subfalcine herniation.

Developmental Neurobiology of Pain in Neonatal Rats.

The authors examine the biological basis for the behavioral evidence of pain perception in neonatal rats, and discuss the potential link to a greater understanding of chronic pain