CPAP by helmet for treatment of acute respiratory failure after pediatric liver transplantation.

ARF after pediatric liver transplantation accounts for high rate of morbidity and mortality associated with this procedure. The role of CPAP in postoperative period is still unknown. The aim of the study was to describe current practice and risk factors associated with the application of helmet CPAP. In this retrospective observational cohort study, 119 recipients were divided into two groups based on indication to CPAP after extubation. Perioperative variables were studied, and determinants of CPAP application were analyzed in a multivariate logistic model. Sixty patients (60/114) developed ARF and were included in the CPAP group. No differences were found between the two groups for primary disease, graft type, and blood product transfused. At multivariate analysis, weight <11 kg (OR = 2.9; 95% CI = 1.1-7.3; P = .026), PaO2 /FiO2 <380 before extubation (OR = 5.4; 95% CI = 2.1-13.6; P < .001), need of vasopressors (OR = 2.6; 95% CI = 1.1-6.4; P = .038), and positive fluid balance >148 mL/kg (OR = 4.0; 95% CI = 1.6-10.1; P = .004) were the main determinants of CPAP application. In the CPAP group, five patients (8.4%) needed reintubation. Pediatric liver recipients with lower weight, higher need of inotropes/vasopressors, higher positive fluid balance after surgery, and lower PaO2 /FiO2 before extubation were at higher odds of developing ARF needing CPAP application.

Changes in mitochondrial stability during the progression of the Barrett's esophagus disease sequence.

BACKGROUND: Barrett's esophagus follows the classic step-wise progression of metaplasia-dysplasia-adenocarcinoma. While Barrett's esophagus is a leading known risk factor for esophageal adenocarcinoma, the pathogenesis of this disease sequence is poorly understood. Mitochondria are highly susceptible to mutations due to high levels of reactive oxygen species (ROS) coupled with low levels of DNA repair. The timing and levels of mitochondria instability and dysfunction across the Barrett's disease progression is under studied. METHODS: Using an in-vitro model representing the Barrett's esophagus disease sequence of normal squamous epithelium (HET1A), metaplasia (QH), dysplasia (Go), and esophageal adenocarcinoma (OE33), random mitochondrial mutations, deletions and surrogate markers of mitochondrial function were assessed. In-vivo and ex-vivo tissues were also assessed for instability profiles. RESULTS: Barrett's metaplastic cells demonstrated increased levels of ROS (p < 0.005) and increased levels of random mitochondrial mutations (p < 0.05) compared with all other stages of the Barrett's disease sequence in-vitro. Using patient in-vivo samples, Barrett's metaplasia tissue demonstrated significantly increased levels of random mitochondrial deletions (p = 0.043) compared with esophageal adenocarcinoma tissue, along with increased expression of cytoglobin (CYGB) (p < 0.05), a gene linked to oxidative stress, compared with all other points across the disease sequence. Using ex-vivo Barrett's metaplastic and matched normal patient tissue explants, higher levels of cytochrome c (p = 0.003), SMAC/Diablo (p = 0.008) and four inflammatory cytokines (all p values <0.05) were secreted from Barrett's metaplastic tissue compared with matched normal squamous epithelium. CONCLUSIONS: We have demonstrated that increased mitochondrial instability and markers of cellular and mitochondrial stress are early events in the Barrett's disease sequence.

A Barrett's esophagus registry of over 1000 patients from a specialist center highlights greater risk of progression than population-based registries and high risk of low grade dysplasia.

Barrett's esophagus (BE) arising from chronic gastro-oesophageal reflux (GERD) is the main pathologic precursor of esophageal adenocarcinoma (EAC). The risk of progression to high-grade dysplasia (HGD) and EAC is unclear, and recent population studies from Denmark and Northern Ireland suggest that this has been overestimated in the past. No data exist from the Republic of Ireland. A detailed clinical, endoscopic, and pathologic database was established in one center as a proposed pilot for a national registry, and initial and follow-up data were abstracted by a data manager. One thousand ninety-three patients were registered, 60 patients with HGD were excluded, leaving 1033, with a median age of 59 and 2 : 1 male to female ratio, and 3599 person-years of follow-up. The overall incidence of HGD/EAC was 1.33% per year overall, 0.85% if the first year is excluded. Within the first year after index endoscopy, 18 cases of HGD or EAC were identified, and 30 following the first year. Low-grade dysplasia (LGD) on index endoscopy was associated with an incidence of progression of 6.5% per year, and 3.1% when tertiary referrals were excluded. These data provide important demographic and clinical information on the population of Irish patients with BE, with incidence rates of progression higher than recently published population-based registry series, perhaps relating to sampling and pathological assessment. Low-grade dysplasia on initial biopsy is a significant proxy marker of risk of progression.

Disabling disease codes predict worse outcomes for acute medical admissions.

BACKGROUND: Concurrent with an extension in longevity, a prodrome of ill-health ('disability' identifiable by certain International Classification of Disease (ICD) 9/ICD10 codes) predates the acute emergency presentation. To date, no study has assessed the effect of such 'disability' on outcomes of emergency medical admissions. AIM: To devise a new method of scoring the burden of 'disability' and assess its relevance to outcomes of acute hospital admissions. METHODS: All emergency admissions (67 971 episodes in n = 37 828 patients) to St James' Hospital, Dublin, Ireland over an 11-year period (2002-2012) were studied, and 30-day in-hospital mortality and length of stay were assessed as objective end-points. Patients were classified according to a validated 'disability' classification method and scored from 0 to 4+ (5 classes), dependent on number of ICD9/ICD10 'hits' in hospital episode codes. RESULTS: A disabling score of zero was present in 10.6% of patients. Scores of 1, 2, 3 and 4+ (classified by the number of organ systems involved) occurred with frequencies of 23.3%, 28.7%, 21.9% and 15.5% respectively. The 'disability' score was strongly driven by age. The 30-day mortality rates were 0.9% (no score), 2.6%, 4.1%, 6.3% and 10.9%. Surviving patients remained in hospital for medians of 1.8 (no score), 3.9, 6.1, 8.1 and 9.7 days respectively. High 'disability' and illness severity predicted a particularly bad outcome. CONCLUSION: Disability burden, irrespective of organ system at emergency medical admission, independently predicts worse outcomes and a longer in-hospital stay.

Stress and ano-colorectal surgery in newborn/infant: role of anesthesia.

PURPOSE: The best anesthesia for newborns/infants necessitating colorectal surgery remains questionable. Endovenous and locoregional anesthesiological approaches were compared to determine the influence on stress response. METHODS: Patients with anorectal malformations or Hirschsprung's disease were randomized to inhalatory/epidural anesthesia (IPA) or inhalatory/endovenous anesthesia (IEA). Heart rate, blood pressure, oxygen saturation, serum concentrations of dehydroepiandrosterone, cortisol, and glucose were recorded 24 h before operation (T0), after tracheal intubation (T1), 120 min after skin incision (T2), 60 min (T3) and 24 h after operation (T4). RESULTS: Seventeen patients were enrolled in the study, 8 receiving IPA, and 9 IEA. Heart rate, blood pressure, oxygen saturation remained stable and normal, without statistical differences between the two groups, during the study period. Similar cortisol and glucose levels showed no statistical differences between groups. Dehydroepiandrosterone values were significantly higher in IEA during anesthesia (T1-T3) compared with IPA (T1: 494.0 vs. 266.5, p < 0.05; T2: 444.0 vs. 201.0, p < 0.05; T3: 385.0 vs. 305.0, p < 0.05). CONCLUSION: This study suggests that epidural and endovenous anesthesia are both effective in intra- and postoperative period. This preliminary report suggests that IPA is more efficient compared to IEA in controlling stress reaction related to surgery. Further larger studies are needed to confirm these findings.

Management of fluid balance in continuous renal replacement therapy: technical evaluation in the pediatric setting.

Fluid overload control and fluid balance management represent very important factors in critically ill children requiring renal replacement therapy. A relatively high fluid volume administration in children and neonates is often necessary to deliver adequate amounts of blood derivatives, vasopressors, antibiotics, and parenteral nutrition. Fluid balance errors during pediatric continuous renal replacement therapy (CRRT) might significantly impact therapy delivery and have been described as potentially lethal. The aim of this study was to evaluate the accuracy of delivered vs. prescribed net ultrafiltration (UF) during CRRT applied to 2 neonates and 2 small children, either as dialytic treatment alone or during extracorporeal membrane oxygenation (ECMO). In accordance with an Acute Dialysis Quality Initiative workgroup statement, net UF was defined as the ""overall amount of fluid extracted from the patient in a given time"". Mean prescribed net UF was 18.5 ml/h (SD=6.7) during neonatal treatments and 70.3 ml/h (SD=22.5) during CRRT in small children. Daily net UF ranged from 200 mL to about 600 mL in the 2 neonates and from 1,200 to 1800 mL in the 2 children. The percentage error of delivered net UF ranged from -1.6% to 5.8% of the prescribed level. The mean error of the ECMO/CRRT patients was 3.024 ml/h vs. 0.45 m/h for the CRRT patients (p<0.001). The same difference was not evident when the 2 neonates were compared with the 2 small children (without considering the presence of ECMO). CRRT and net UF delivery appeared to be accurate, safe, and effective in this small cohort of high-risk pediatric patients.

Continuous hemofiltration dose calculation in a newborn patient with congenital heart disease and preoperative renal failure.

OBJECTIVE: To report a case of a newborn patient with renal failure due to polycystic kidneys requiring renal replacement therapy, and total anomalous pulmonary venous return requiring major cardiosurgical intervention. SETTING: Pediatric cardiosurgery operatory room and pediatric cardiologic intensive care. PATIENT: A 6-day-old newborn child weighing 3.1 kg. RESULTS: Renal function (creatinine value and urine output) was monitored during the course of the operation and intraoperative renal replacement therapy was not initiated. Serum creatinine concentration decreased from 4.4 to 3 mg/dL at cardiopulmonary bypass (CPB) start and to 1.5 at the end of surgery: the creatinine decrease was provided by the dilutional effect of CPB priming and the infusion of fresh blood from transfusions together with an adequate filtration rate (800 m/L in about 120 minutes). After the operation, extracorporeal membrane oxygenation (ECMO) for ventricular dysfunction and continuous hemofiltration for anuria refractory to medical therapy were prescribed. The hemofiltration machine was set in parallel with the ECMO machine at a blood flow rate of 60 ml/min and a predilution replacement solution infusion of 600 ml/h (4.5 ml/min of creatinine clearance once adjusted on extracorporeal circuits; 3000 mL/m2 hemofiltration): after a single hemofiltration session lasting 96 hours, serum creatinine reached optimal steady state levels around 0.5 mg/dL on postoperative day 2 and 3. CONCLUSION: Administration of intraoperative continuous hemofiltration is not mandatory in the case of a 3-kg newborn patient with established renal failure needing major cardiosurgery: hemodilution secondary to CPB, transfusion of hemoderivates, and optimal UF rate appear to be effective methods for achieving solute removal. If postoperative continuous hemofiltration is started, however, a "dialytic dose" of 4.5 ml/min allows an adequate creatinine clearance, quick achievement of a steady state of serum creatinine concentration and an eventual acceptable rate of inflammatory mediator removal.

From intravenous to enteral ketogenic diet in PICU: A potential treatment strategy for refractory status epilepticus.

BACKGROUND: Ketogenic diet (KD) has been used to treat refractory status epilepticus (RSE). KD is a high-fat, restricted-carbohydrate regimen that may be administered with different fat to protein and carbohydrate ratios (3:1 and 4:1 fat to protein and carbohydrate ratios). Other ketogenic regimens have a lower fat and higher protein and carbohydrate ratio to improve taste and thus compliance to treatment. We describe a case of RSE treated with intravenous KD in the Pediatric Intensive Care Unit (PICU). CASE REPORT: An 8-year-old boy was referred to the PICU because of continuous tonic-clonic and myoclonic generalized seizures despite several antiepileptic treatments. After admission he was intubated and treated with intravenous thiopental followed by ketamine. Seizures continued with frequent myoclonic jerks localized on the face and upper arms. EEG showed seizure activity with spikes on rhythmic continuous waves. Thus we decided to begin KD. The concomitant ileus contraindicated KD by the enteral route and we therefore began IV KD. The ketogenic regimen consisted of conventional intravenous fat emulsion, plus dextrose and amino-acid hyperalimentation in a 2:1 then 3:1 fat to protein and carbohydrate ratio. Exclusive IV ketogenic treatment, well tolerated, was maintained for 3 days; peristalsis then reappeared so KD was continued by the enteral route at 3:1 ratio. Finally, after 8 days and no seizure improvement, KD was deemed unsuccessful and was discontinued. CONCLUSIONS: Our experience indicates that IV KD may be considered as a temporary "bridge" towards enteral KD in patients with partial or total intestinal failure who need to start KD. It allows a prompt initiation of KD, when indicated for the treatment of severe diseases such as RSE.

Prevalence of pain in the departments of surgery and oncohematology of a paediatric hospital that has joined the project "Towards pain free hospital".

BACKGROUND: Among hospitalized adults and children pain is undertreated. This study wants to assess the effectiveness of pain therapy in two departments of a large children's hospital. MATERIALS AND METHODS: During a single day work three committees, administering a questionnaire to patients or parents, have evaluated the adherence to international recommendations (JCI and WHO) in the management of analgesic therapy. Patient demographics, prevalence and intensity (moderate and/or severe) of pain (during hospitalization, 24 hours before and at the time of the interview), analgesia (type, route, duration and frequency of administration) and Pain Management Index (=analgesic score-pain score) were recorded. RESULTS: 75 patients participated in the study (age: 2 months up to 24 years, mean 7.8 ± 6). During hospitalization 43 children (57%) had no pain while 32 (43%) have experienced pain. 22 children (29 %) had pain 24 hours before and 12 (16%) at the time of the interview. The average value of the PMI was -0.8±1.3 with a minimum of -3 and a maximum of +2: 60% (19) of the children had a PMI less than 0 (undertreated pain) while 40% (13) had a value=or>0. Out of 32 patients who needed an analgesic therapy 14 (44%) received an around-the-clock dosing, 8 (25%) an intermittent therapy and 10 (31%) no treatment.17 (77 %) were the single drug therapy and 5 (23%) the multimodal ones. CONCLUSIONS: The prevalence of pain in the two departments is high. The main cause is that knowledge is not still well translated into clinical practice.

Balloon occlusion of the carotid artery for the angiographic visualization of Blalock-Taussig shunts and pulmonary arteries.

We describe the use of a balloon catheter to occlude the right or left carotid artery as a way of directing contrast material to the pulmonary arteries. This procedure was carried out in the postoperative study of 14 children who had undergone a Blalock-Taussig shunt. The method was reliable, and the angiograms provided excellent visualization of the pulmonary arteries using only small volumes of contrast material.

Bronchial compression by dilated pulmonary artery. Surgical treatment.

Bronchial compression by a dilated left pulmonary artery complicated the postoperative course in two infants: one after a Mustard operation for transposition of the great arteries, restrictive ventricular septal defect, and moderate pulmonary stenosis, the second after correction of tetralogy of Fallot with absent pulmonary valve. In both cases the bronchial compression was successfully treated by division of the left pulmonary artery and its prolongation by interposition of a conduit.

Extracardiac Fontan operation for complex cardiac anomalies: seven years' experience.

METHODS: Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22). RESULTS: Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years. CONCLUSION: These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

Discrete subaortic stenosis. Surgery in children based on two-dimensional and Doppler echocardiography.

Twenty pediatric patients underwent surgical resection of a "discrete" subaortic membrane. The diagnosis and the surgical indication were based on two-dimensional and Doppler echocardiography without cardiac catheterization and angiography. In all patients the echocardiographic diagnosis was confirmed at surgery in terms of presence, dimension and location of the membrane and in ten patients in terms of pressure gradients. Two-dimensional and Doppler echocardiography has proved to be a very reliable tool for the diagnosis and surgical indication in pediatric patients with a DSAS. Our criteria for the selection of surgical patients are the following: (1) isolated form of discrete subaortic stenosis with a short base of attachment to the ventricular septum; (2) pressure gradients higher than 25 mm Hg; (3) presence of significant aortic insufficiency. All of this information can be consistently obtained with two-dimensional and Doppler echocardiography.

Bidirectional cavopulmonary anastomosis in patients under two years of age.

Between December 1986 and December 1990, a bidirectional cavopulmonary anastomosis was performed in 27 patients younger than 2 years of age, including 12 with heterotaxia syndrome. Age and weight of patients averaged 14.2 +/- 6.6 months and 8.1 +/- 2.2 kg, respectively. Eleven had pulmonary atresia and 16 had pulmonary stenosis. The main pulmonary artery was ligated in seven patients in the latter group (subsequently reopened in one) and left open in nine (subsequently ligated in two). There were four hospital deaths (15%). All patients were discharged with anticoagulant/antithrombotic therapy to be continued for 6 months. There were two late deaths before further operations (8.7%). Two patients, one with acquired massive pulmonary arteriovenous fistulas and one with progressive common atrioventricular valve regurgitation, subsequently underwent definitive repair (biventricular in one), and both died. Heterotaxia syndrome (p = 0.087) and preoperative mean pulmonary artery pressure higher than 15 mm Hg (p = 0.09) were the only risk factors for overall mortality.

Bidirectional cavopulmonary shunts: clinical applications as staged or definitive palliation.

A standard Glenn anastomosis between the superior vena cava and the right pulmonary artery has been the accepted mode of treatment for patients with complex cyanotic congenital heart disease. We report our experience in 18 patients with such disease who underwent a bidirectional cavopulmonary shunt because of increasing cyanosis and growth cessation. All patients were considered less than "ideal" candidates for a Fontan procedure. We divided the patients into two groups: group 1 had azygos continuation and group 2 did not. Fourteen patients required hypothermic cardiopulmonary bypass. Bidirectional pulmonary blood flow was achieved in all patients. Only 1 death occurred (group 2). The improvement in oxygen saturation and overall clinical condition of these patients, together with the low mortality and morbidity, is encouraging. However, long-term follow-up is mandatory for a comprehensive evaluation of this surgical approach as definitive palliation or as a first stage for a Fontan operation.

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve.

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).