Neurofibromatosis Type I Presenting with Incomplete Ileal Volvulus in a Pediatric Patient.

BACKGROUND Neurofibromatosis 1 is a neurocutaneous disorder with multisystemic manifestations. When patients are lacking overt cutaneous manifestations, diagnosis may be delayed and may complicate diagnosis and management of atypical presentations of this disease. It is thus important to strive to obtain relevant and/or complete history to arrive at the appropriate diagnosis. Furthermore, maintaining an index of suspicion in cases of vague abdominal pain may guide the clinician in establishing the correct diagnosis of mesenteric plexiform neurofibroma in the setting of known/presumed neurofibromatosis 1 patients presenting with acute and/or chronic vague abdominal symptoms. CASE REPORT This is a case of a teenage boy who presented with acute, vague abdominal pain over a period of 2 weeks. Laboratory tests and physical exam findings in primary and secondary care settings were unremarkable, and thus the patient was discharged home only to continue with abdominal pain, thus seeking additional medical care. After admission to our facility and exhaustive history taking, physical examination, and imaging, a prospective diagnosis of neurofibromatosis with mesenteric neurofibroma was made. Upon surgical exploration, a mesenteric mass with corresponding volvulized, ischemic small bowel was removed. Histopathology confirmed a plexiform neurofibroma. The patient recovered adequately and was discharged home without complications. CONCLUSIONS This case highlights the importance of exhaustive history taking to obtain an accurate diagnosis as well as the importance of a high index of clinical suspicion for mesenteric neurofibromatosis in patients with presumed or known neurofibromatosis and presenting with vague abdominal symptoms.

Prognostic Value of Variations in Serum Biomarkers and Prognostic Scores Values Between Admission and Second Day in Intensive Care Unit Septic Patients.

Objective To determinate the prognostic value of procalcitonin (PCT) and C-reactive protein (CRP) changes during the first two days of admission to the ICU with sepsis and/or septic shock, and to compare it with changes in Acute Physiology And Chronic Health Evaluation II (APACHE-II) and Sepsis-related Organ Failure Assessment (SOFA) prognostic scores. Methods A single-center prospective observational study was performed. Fifty consecutive patients admitted to the ICU, diagnosed of severe sepsis/septic shock were included. We considered risk factors for infection: diabetes mellitus, chronic obstructive pulmonary disease (COPD), previous antibiotic treatment, central intravascular catheter, bladder catheter, active neoplasia. Results Median aged 67(52-75) years with median APACHE-II 19(14-25) points and SOFA scores 7(5-11) points on admission, and 28-day mortality of 42%. When we studied the relationship between mortality and the changes between the day of admission and the second day of the variables studied, we found that APACHE-II (p = 0.001) and SOFA (p = 0.002) between admission and second day raised significantly in no survivors, with no significant changes in CRP and PCT. Multivariate analysis showed that mortality was significantly associated to changes in SOFA score (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.18-3.86) and to the presence of one or more risk factors for infection (OR, 6.01; 95% CI, 1.01-35.78) but not with PCT changes. Mortality was also related to the variations between the day of admission and the fifth day on APACHE-II (p = 0.002), SOFA (p < 0.001) and PCT (p = 0.012). Conclusions Changes in SOFA and APACHE-II scores between admission and second day in ICU septic patients are more sensitive mortality predictors than the observed changes in CRP and PCT values. Changes in PCT levels between the day of ICU admission and the fifth day are significantly related to mortality and may be useful as an additional marker in patient outcome.

Validation of the ICH score in patients with spontaneous intracerebral haemorrhage admitted to the intensive care unit in Southern Spain.

OBJECTIVE: Validation of the intracerebral haemorrhage (ICH) score in patients with a diagnosis of spontaneous ICH admitted to the intensive care unit (ICU). METHODS: A multicentre cohort study was conducted in all consecutive patients with ICH admitted to the ICUs of three hospitals with a neurosurgery department between 2009 and 2012 in Andalusia, Spain. Data collected included ICH, Glasgow Coma Scale (GCS) and Acute Physiology and Chronic Health Evaluation II (APACHE-II) scores. Demographic data, location and volume of haematoma and 30-day mortality rate were also collated. RESULTS: A total of 336 patients were included. 105 of whom underwent surgery. Median (IQR) age: 62 (50-70) years. APACHE-II: 21(15-26) points, GCS: 7 (4-11) points, ICH score: 2 (2-3) points. 11.1% presented with bilateral mydriasis on admission (mortality rate=100%). Intraventricular haemorrhage was observed in 58.9% of patients. In-hospital mortality was 54.17% while the APACHE-II predicted mortality was 57.22% with a standardised mortality ratio (SMR) of 0.95 (95% CI 0.81 to 1.09) and a Hosmer-Lemenshow test value (H) of 3.62 (no significant statistical difference, n.s.). 30-day mortality was 52.38% compared with the ICH score predicted mortality of 48.79%, SMR: 1.07 (95% CI 0.91 to 1.23), n.s. Mortality was higher than predicted at the lowest scores and lower than predicted in the more severe patients, (H=55.89, p<0.001), Gruppo Italiano per la Valutazione degli Interventi in Terapia Intensiva calibration belt (p<0.001). The area under a receiver operating characteristic (ROC) curve was 0.74 (95% CI 0.69 to 0.79). CONCLUSIONS: ICH score shows an acceptable discrimination as a tool to predict mortality rates in patients with spontaneous ICH admitted to the ICU, but its calibration is suboptimal.

Prolonged Mechanical Ventilation as a Predictor of Mortality After Cardiac Surgery.

BACKGROUND: Mortality among the small percentage of cardiac surgery patients receiving prolonged mechanical ventilation is high, but this issue appears to be inadequately addressed in guidelines. METHODS: This study is a retrospective analysis of prospective, multi-center, and observational study in Spain including all adults undergoing cardiac surgery in 3 Andalusian hospitals between June 2008 and December 2012. RESULTS: The study included 3,588 adults with mean ± SD age of 63.5 ± 12.8 y and with median (interquartile range) EuroSCORE of 5 (3-7) points. Prolonged mechanical ventilation (> 24 h) was required by 415 subjects (11.6%), with ICU mortality of 44.3% (184 subjects), and was not required by 3,173 subjects (88.4%), with ICU mortality of 3.1% (99 subjects, P < .001). Prolonged mechanical ventilation was associated with more complications and was required by 4.5% of subjects with a EuroSCORE <5, 11.2% with a score of 5-7, 27.2% with a score of 8-10, and 32.2% with a score > 10. In the multivariable analysis, ICU mortality was associated with illness severity, duration of bypass surgery, surgery type, and prolonged mechanical ventilation (odds ratio 15.19, 95% CI 11.56-22.09). The main cause of death was multiple organ failure and sepsis in subjects who required prolonged mechanical ventilation (50.3%) and cardiogenic shock in those who did not (59.2%). CONCLUSION: Prolonged postoperative mechanical ventilation was required by 10-20% of cardiac surgery subjects, who constitute a specific group that represents most of the postoperative mortality, which is associated with multiple organ failure and sepsis.

Soluble membrane receptors, interleukin 6, procalcitonin and C reactive protein as prognostic markers in patients with severe sepsis and septic shock.

BACKGROUND: The objective of this study was to explore the diagnostic and prognostic value of soluble triggering receptor expressed on myeloid cell 1 (sTREM-1), soluble cluster of differentiation 14 (sCD14), soluble cluster of differentiation 163 (sCD163), interleukin-6 (IL-6), procalcitonin (PCT), and C-reactive protein (CRP) serum levels for patients with severe sepsis and septic shock in an intensive care unit (ICU). METHODS: Fifty patients admitted at the ICU with the diagnosis of severe sepsis or septic shock were studied. SOFA and APACHE II scores as well as serum biomarkers were measured at days 0, 2 and 5. The influence of these variables on 28-day mortality was analyzed. Twenty healthy individuals served as controls. RESULTS: Baseline serum concentrations of sTREM-1, sCD163, IL-6 and PCT correlated with SOFA score. Only sTREM-1 levels correlated with APACHE II score. The 28-day mortality rate for all patients was 42%. The absence of risk factors for infection, presence of septic shock, baseline values of sCD14 and decrease of PCT and IL-6 from baseline to day 5 were variables associated to mortality in the univariate analysis. The unique independent factor associated to mortality in the multivariate analysis was a decrease of PCT higher than 50% from days 0 to 5. CONCLUSIONS: Serum levels of sTREM-1 are correlated with the severity of sepsis. A 50% decrease of PCT was the unique variable associated with survival in the multivariate analysis.

Survival analysis of surgically evacuated supratentorial spontaneous intracerebral hemorrhage with intraventricular extension.

OBJECTIVE: To conduct a survival study and evaluation of surgical treatment in a cohort of patients with diagnosis of supratentorial spontaneous intracerebral hemorrhage (ICH). MATERIALS AND METHODS: The study included all consecutive patients with supratentorial ICH admitted to the Intensive Care Units of three Spanish hospitals with Neurosurgery Department between 2009 and 2012. DATA COLLECTED: age, APACHE-II, Glasgow Coma Score (GCS), and pupillary anomalies on admission, intracerebral hemorrhage (ICH) score, location/volume of hematoma, intraventricular hemorrhage (IVH), surgical evacuation alone or with additional external ventricular drain, and 30-days survival and at hospital discharge RESULTS: A total of 263 patients were included. Mean age: 59.74±14.14 years. GCS: 8±4 points, APACHE II: 20.7±7.68 points. ICH Score: 2.32+1.04 points. Pupillary anomalies were observed in 30%. The 30-day mortality: 51.3% (45.3% predicted by ICH-score), and 53.2% at hospital discharge. A significant difference (p=0.004) was observed in hospital mortality rates between surgically treated patients (39.7%, n=78) versus those conservatively managed (58.9%, n=185); specifically in those with IVH surgically treated (34.2%, n=38) versus non-operated IVH (67.2%, n=125), p<0.001. No significant difference was found between mortality rates in patients without IVH. Multiple logistic regression analysis showed an OR for surgery of 1.04 (95% CI; 0.33-3.22) in patients without IVH versus 0.19 (95% CI; 0.07-0.53) in patients with IVH (decreased mortality with surgical treatment). The propensity score analysis for IVH patients showed improved survival of operated group (OR 0.23, 95% CI; 0.07-0.75), p=0.01. CONCLUSIONS: Hospital mortality was lower in patients who underwent surgery compared to patients conservatively managed, specifically for the subgroup of patients with intraventricular hemorrhage.

Mortality and complications in elderly patients undergoing cardiac surgery.

PURPOSE: The purpose of the study was to analyze postoperative complications, mortality, and related factors of elderly patients undergoing cardiac surgery. METHODS: An observational, retrospective, and multicenter study of cardiac surgery patients, obtained from the ARIAM registry, was performed between 2008 and 2011. Clinical-surgical data, postoperative complications, and mortality were analyzed in a group of patients older than 75 years and in a younger group. RESULTS: A total of 4548 patients were analyzed, with 882 (19.4%) patients at least 75 years old. Elderly patients had worse functional status (New York heart Association class) and comorbidities. The complication rate was higher in the elderly group (40.4% and 33.5%, respectively; P = .0001). Mortality in the elderly was 1.1%, 12%, and 15.1% (during surgery, intensive care unit [ICU], and 30-day mortality, respectively). Thirty-day mortality in elderly patients was higher when adjusted for EuroSCORE (European System for Cardiac Operative Risk Evaluation) and cardiopulmonary bypass time. The interaction between multiorgan dysfunction syndrome (MODS) and age more than 75 years was assessed by logistic regression, obtaining an odds ratio of 9.27 (5.88-14.60) for younger patients and 29.44 (12.22-70.94) for elderly patients who died during the ICU stay. CONCLUSIONS: Age more than 75 years is an independent risk factor for ICU mortality when adjusted for EuroSCORE and cardiopulmonary bypass time. Elderly patients also have a higher rate of complications during ICU stay. Elderly patients develop MODS more frequently and present a higher mortality rate than younger patients with MODS.

[Consequences of obesity in outcomes after cardiac surgery. Analysis of ARIAM registry]. / Consecuencias de la obesidad en los resultados de la cirugía cardiaca. Análisis del registro ARIAM.

BACKGROUND AND OBJETIVE: Obesity is a disease that affects a large part of the population and has been associated with worse outcomes after cardiac surgery. The aim of our study is to evaluate the consequences of obesity related to postoperative complications, hospital length of stay and mortality. METHODS: Observational, prospective, multicenter study of patients included in ARIAM registry of adult cardiac surgery between March 2008 to March 2011. We analyzed clinical variables, the surgical procedure, postoperative complications and mortality, comparing the group of patients with body mass index (BMI) greater or less than 30 kg/m(2). RESULTS: The study included 4,172 patients with a mean age of 64.03 (SD 12.08) years, BMI 28.53 (4.7) and EuroSCORE 5.58 (2.91). In 1,490 patients (35.7%) BMI was greater than 30. There were no differences in the development of overall postoperative complications (33% in obese and non-obese 35.8%, P=.07), although there were less appreciated reoperation rate or stroke (P<.05), as well as further development postoperative renal failure (P=.009). After adjusting for severity and length of cardio by pass time, obese patients had lower mortality without being statistically significant. OR 0.94 (0.79-1.04). There were no differences in ICU length of stay, but obese patients had greater Ward length of stay 9.04 (10.43) vs. 8.18 (9.2) days, P=.01. CONCLUSIONS: Obese patients undergoing cardiac surgery have a mortality, rate of complications and length of stay similar to non-obese. Obese patients required less reoperations but developed more frequently postoperative renal failure.