Backround. Acute kidney injury (AKI) is a common serious complication of severe acute pancreatitis (SAP) and an important marker of morbidity and mortality in critically ill septic patients. AKI due to severe acute pancreatitis can be the result of hypoxemia, release of pancreatic amylase from the injured pancreas with impairment ...

Scrub typhus is a major infectious threat in the Asia-Pacific region. We report an unusual case of scrub typhus in Singapore that presented with sepsis and acute respiratory distress syndrome, but lacking the pathognomonic eschar. The patient recovered after appropriate diagnosis and doxycycline treatment. Rickettsial diseases should be included in ...

OBJECTIVES:: Acute respiratory distress syndrome develops commonly in critically ill patients in response to an injurious stimulus. The prevalence and risk factors for development of acute respiratory distress syndrome after spontaneous intracerebral hemorrhage have not been reported. We sought to determine the prevalence of acute respiratory distress syndrome after intracerebral ...

BACKGROUND & AIMS: Gilbert's syndrome is a common familial hyperbilirubinemia that may reduce the risk of various age-related diseases due to the antioxidant properties of bilirubin. We conducted a large cohort study using The Health Improvement Network (THIN) primary care database and compared all-cause mortality rates in those with and ...

The hemophagocytic lymphohistiocytosis (HLH) syndrome is a hyperimmune disorder characterized by lymphohistiocytic infiltrations, elevated cytokine levels in the blood, macrophage activation, and hemophagocytosis, frequently presenting with a febrile septic picture. This unusual disease is more common in infancy and childhood than adulthood. It is classified as primary or familial when ...

Puumala virus (PUUV) is considered a classic Old World etiologic agent of nephropathia epidemica (NE), or hemorrhagic fever with renal syndrome (HFRS). HFRS is considered to be distinct from hantavirus (cardio-)pulmonary syndrome (HPS or HCPS), described in the New World. Here, we report a severe case, which fulfilled most, if ...

The failure of the PROWESS-SHOCK study of recombinant human activated protein C (drotrecogin alfa) has generated much scepticism about the future of immunomodulatory interventions in sepsis. This review presents a summary of the few remaining promising strategies for immunointervention. These comprise intravenous clarithromycin, haemoperfusion through a polymyxin B-embedded fibre device ...

Acute lung injury (ALI), and its more severe form, acute respiratory distress syndrome (ARDS), are syndromes of acute hypoxemic respiratory failure resulting from a variety of direct and indirect injuries to the gas exchange parenchyma of the lungs. Current treatment of ALI/ARDS is primarily supportive, with lung protective ventilation and ...

We report a patient with Ehlers-Danlos syndrome and mitral valve endocarditis. The case was complicated due to the initial septic status of the patient and the fragility of the mitral valve both of which required further early operative intervention. The patient also had pre-existing pulmonary septic emboli, the significance of ...

This is a report of a 42 year old male amateur body builder using anabolic androgenic steroids, who developed acute respiratory distress syndrome, acute kidney injury and refractory supraventricular tachycardia. He required extracorporeal membrane oxygenation, continuous veno-venous hemodialysis, and catheter ablation. It was thought that long-term anabolic androgenic steroid abuse ...

The aim of our study was to document the estimated sensitivity and specificity of continuous intracompartmental pressure monitoring for the diagnosis of acute compartment syndrome. From our prospective trauma database, we identified all patients who had sustained a tibial diaphyseal fracture over a ten-year period. A retrospective analysis of 1184 ...

BACKGROUND: In spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors. METHODS: From November 1994 until February 2012, all patients that had a functional ...

Tracheal agenesis is a rare and essentially lethal anomaly with divergent prenatal imaging findings depending on the presence or absence of a tracheoesophageal fistula. All prenatally diagnosed cases of tracheal agenesis reported to date have not had a tracheoesophageal fistula and presented with thoracoabdominal findings similar to congenital high airway ...

Acute respiratory distress syndrome (ARDS) poses a major challenge in intensive care settings. The main underlying causes of ARDS are trauma, pancreatitis, and pulmonary manifestation of systemic inflammatory response syndrome/sepsis.Lemierre syndrome represents a nearly forgotten entity arising from oropharyngeal infections with Fusobacterial species, and it is of renewed and increasing ...

Gorham syndrome is a rare disease that presents as progressive osteolysis and may affect any part of the skeleton. The pathologic process involves the replacement of normal bone by aggressively expanding but non-neoplastic vascular tissue resulting in massive osteolysis of the adjacent bone. If the spine and ribs are affected, ...

Guillain-Barre syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are clinically distinct demyelinating disorders that share an autoimmune pathogenesis and prior history of viral infection or vaccination. Concurrent GBS and ADEM are uncommon with few reported cases. Our patient is a 10-year-old girl who presented with acute quadriparesis, areflexia, and urinary ...

Intermediate syndrome (IMS) was described a few decades ago, however, there is still a controversy regard ing its exact etiology, risk factors, diagnostic parameters and required therapy. Considering that acute poisonings are treated in different types of medical institutions this serious complication of organophosphate insecticide (OPI) poison ing is frequently ...

A 22-year-old obese asthmatic woman with Influenza A (H1N1)-associated acute respiratory distress syndrome died from cerebral artery gas emboli with massive cerebral infarction while being treated with High-Frequency Oscillatory Ventilation in the absence of a right to left intracardiac shunt. We review and briefly discuss other causes of systemic gas ...

Cloverleaf skull deformity (Kleeblattschädel-Syndromen, trilobular skulls) results from synostosis of multiple cranial sutures. The number of sutures involved, the pathogenesis of the synostosis, and the associated anomalies and syndromes are variable. All forms of cloverleaf skull are associated with a high morbidity and mortality. Management of surviving infants requires multiple ...

OBJECTIVE: Total cavopulmonary connection (TCPC) is rarely performed for a functional single ventricle in children with Down syndrome; therefore, the postsurgical outcomes are not well known. We evaluated mortality and related factors after TCPC in children with Down syndrome. METHODS: Between January 2004 and March 2010 we identified 8 patients ...

Key points In Hurler Syndrome patients early referral for HCT, with the best available HLA-matched donor offers the best "event free survival".In Hurler syndrome patients HCT with a well matched unrelated cord blood unit is particularly attractive as the unit is readily available.

Snake bite is an environmental and occupational hazard in many tropical and subtropical countries. It demands a high level of knowledge and skill in managing the envenomation syndrome. Herein, we describe a rare case of acute acalculous cholecystitis (AAC) that developed in a 36-year-old man after an Indian cobra (Naja ...

Surfactant dysfunction in meconium aspiration syndrome (MAS) is caused by meconium components, by plasma proteins leaking through the injured alveolocapillary membrane and by substances originated in meconium-induced inflammation. Surfactant inactivation in MAS may be diminished by several ways. Firstly, aspirated meconium should be removed from the lungs to decrease concentrations ...

Guillain-Barré syndrome is an acute inflammatory demyelinating polyradiculopathy characterized by progressive motor weakness, areflexia, and ascending paralysis. Guillain-Barré syndrome is extremely rare in pregnant patients, and there are no established guidelines for delivery or safest anesthetic methods. We report a Cesarean delivery in the case of a 32-year old woman ...

The authors report a case of a 35-year-old man with no known underlying disease who presented with fever, cellulitis with hemorrhagic blebs on the left leg, monoarthricular left knee arthritis, multiple organ failure and septic shock. His clinical syndrome was compatible with toxic shock syndrome and his blood grew alpha ...

Sepsis, defined as infection-induced systemic inflammatory response syndrome, invariably leads to hemostatic abnormalities ranging from insignificant coagulopathy to disseminated intravascular coagulation (DIC). The inflammation-induced activation of coagulation, the downregulation of physiologic anticoagulant pathways, and impairment of fibrinolysis play a pivotal role in the pathogenesis of microvascular fibrin thrombosis and multiple ...

Lemierre's syndrome is only very rarely caused by Porphyromonas asaccharolytica. Here, we report the case of a 35-year-old man who developed a left peritonsillar abscess, thrombophlebitis of the left internal jugular vein, and septic embolization of both lungs. Anaerobic P. asaccharolytica was isolated in the blood cultures, and we subsequently ...

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but increasingly described phenomenon of immune activation and organ dysfunction in association with a wide variety of medications. This reaction shows a broad spectrum of clinical presentation and severity, ranging from mild to lethal. Treatment strategies of immune ...

Acute respiratory distress syndrome (ARDS) is a pulmonary syndrome with growing prevalence and high mortality and morbidity that increase with age. There is no current therapy able to restore pulmonary function in ARDS patients. Preclinical models of ARDS have demonstrated that intratracheal or systemic administration of mesenchymal stem cells (MSCs) ...

BACKGROUND: The occurrence of toxic shock syndrome from an intrauterine device (IUD) is very rare. OBJECTIVES: To raise awareness of the risk of toxic shock syndrome caused by an IUD, to educate others about when to suspect this complication, and to provide treatment recommendations. CASE REPORT: A 49-year-old woman presented to the ...

Introduction Lemierre's syndrome is a potentially fatal condition characterised by spread of an oropharyngeal infection, resulting in thrombosis of the internal jugular vein. This leads to septicaemia and possible metastatic abscesses. Case presentation We discuss the case of a previously healthy 17-year-old male who developed Lemierre's syndrome following dental sepsis. ...

Kidney disease due to Aspergillus fumigatus is a rare finding in hyper-IgE syndrome. We report a patient with autosomal dominant hyper-IgE syndrome, recurrent pneumonia, and acute necrosuppurative pyelonephritis caused by Aspergillus fumigatus with a fatal outcome. We emphasize the severity and the difficulties in the management of renal complications that ...

OBJECTIVE: To describe an extreme presentation of the chylomicronemia syndrome resulting in multiorgan system dysfunction. PATIENT: A 40-year-old African American male with no past medical history presented with multiorgan system dysfunction manifested by acute respiratory failure and acute kidney injury. He was noted to have very-high triglyceride levels (>5000 mg/dL) ...

As many as 20% of patients undergoing cardiac surgery will have acute respiratory distress syndrome during the perioperative period, with a mortality as high as 80%. If patients at risk can be identified, preventative measures can be taken and may improve outcomes. Care for patients with acute respiratory distress syndrome ...

The incremental changes made in the definition of ALI/ARDS over the last two decades represent a series of landmark events in the history of the condition. The first description of ARDS (Adult Respiratory Distress Syndrome) established the recognition of this syndrome(1) ; the 1994 AECC (America-European Consensus Conference) definition set ...

Here we report a lethal case of Netherton syndrome presenting with neurologic complications, hypernatremic dehydration, failure to thrive, and episodes of sepsis. Molecular analysis of the serine protease inhibitor Kazal-type 5 gene identified a homozygous mutation (c.1111C>T, p.Arg371X). This case highlights the importance of early diagnosis to start appropriate care ...

OBJECTIVE: We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. METHODS: We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy ...

This study focuses on the effect of chemotherapy on endocrinopathies and the metabolic syndrome in adult survivors of childhood acute myeloid leukemia (AML). Endocrine function and metabolic syndrome were evaluated in 12 AML survivors, treated with chemotherapy, and in 9 survivors of myeloid leukemias treated with stem cell transplantation (SCT), ...

Patients presenting with acute coronary syndromes (ACS) remain amongst the highest-risk of all acute medical admissions. Despite significant reductions in morbidity and mortality via refinements in treatment methods in recent years, such individuals remain at a high risk of recurrent ischaemic events and death. Whilst 2012 has brought a wealth ...

A rare case of neonatal Bartter syndrome presenting with severe hyperkalemia is reported in a preterm child born to consanguineous parents. This child also had ileal atresia, and meconium plugs were found at laparotomy. The diagnosis of cystic fibrosis was subsequently made on genetic testing. Despite full intensive care management ...

Acute lung injury and acute respiratory distress syndrome are syndromes of severe respiratory failure. Children with acute lung injury or acute respiratory distress syndrome have high mortality and the survivors have significant morbidity. Partial liquid ventilation is proposed as a less injurious form of respiratory support for these children. Uncontrolled ...

Neonatal sepsis is a leading cause of neonatal mortality. Congenital heart disease accounts for additional risk of sepsis in neonates. Here we report a case of Down's syndrome with late onset neonatal sepsis associated with multiple superficial skin abscesses simulating staphylococcal infection. The baby was empirically treated with vancomycin. Subsequently, ...

Haddad syndrome is a rare genetically conditioned disease. We present a female newborn with congenital central hypoventilation syndrome associated with Hirschprung`s disease. The infant is mechanically ventilated and parentally fed in a home setting. The diagnosis has been confirmed by the presence of 20/26 PHOX2B genetic mutation.

We describe a 2.5 year-old child with toxic shock syndrome due to group A beta-hemolytic streptococcus (GABHS) who presented with purpura fulminans and limb ischemia treated with early microsurgical arteriolysis. The clinical picture of toxic shock syndrome (TSS) presenting with purpura fulminans and limb ischemia is an exceptionally uncommon finding ...

We studied the causes of death of patients in Sweden with diagnoses of hemorrhagic fever with renal syndrome (HFRS) during 1997-2009. Cardiovascular disorders were a common cause of death during acute-phase HFRS and were the cause of death for >50% of those who died during the first year after HFRS.

A young drug abuser was admitted to our intensive care unit for organophosphorus poisoning. He required mechanical ventilation and was started empirically on linezolid for suspected nosocomial infection. The patient developed high-grade fever with altered sensorium and clonus. Serotonin syndrome was suspected and the patient was started on cyproheptadine. He ...

Although, historically, shock associated with traumatic injury has been evaluated through knowledge of the 4 recognized shock patterns-cardiogenic, obstructive, distributive, and hypovolemic-many trauma practitioners view traumatic shock as a unique fifth shock pattern. Although secondary to a systemic inflammatory response syndrome triggered by endogenous danger signals, traumatic shock represents a ...

ABTRACT: Antley-Bixler syndrome is an autosomal recessive disorder characterized by multiple bone and cartilaginous abnormalities. The main features of this syndrome include brachycephaly, midface hypoplasia, dysplasia of ears and nose, radiohumeral synostosis, choanal stenosis, or atresia. Distinctive features are based on craniofacial deformity and humeroradial synostosis. In this report, we ...

Lactate is the endogenous end product of the anaerobic glycolysis, whose production is favoured in situations of hypoperfusion or mitochondrial dysfunction. Leigh syndrome is a rare, progressive encephalomyopathy that represents a spectrum of mitochondrial genetic diseases phenotypically distinct, but with neuroradiological and pathological uniform presentation. We present the case of ...

Syndromic surveillance is designed for early detection of disease outbreaks. An important data source for syndromic surveillance is free-text chief complaints (CCs), which are generally recorded in the local language. For automated syndromic surveillance, CCs must be classified into predefined syndromic categories. The n-gram classifier is created by using text ...