[Heart failure in young children; a serious cause of feeding problems]. / Hartfalen bij jonge kinderen.

Feeding problems in children are usually harmless and common, but can rarely exist as a result of pathology. Heart failure is one of them and has to be recognized early because of its many consequences. We present a 15-week-old female infant who was seen at the outpatient clinic. She had already been evaluated several times by a youth doctor and general practitioner because of feeding problems and transpiration, for which several nutritional interventions had already been carried out. With no effect of nutritional interventions she was referred to a pediatrician. Physical examination showed clear signs of heart failure and echocardiogram showed a severe dilation of the left ventricle, with poor contractility of the lateral wall and papillary muscle, with end stage heart failure due to an Anomalous Left Coronary Artery from the Pulmonary Artery (ALPACA). In this article we discuss the clinical presentation of heart failure: stagnant growth, exercise intolerance, tachypnea and sometimes hepatomegaly, edema, murmurs or cyanosis. In addition, when a patient does not respond to initial therapy, we recommend to reconsider differential diagnosis and/or refer to a pediatrician.

Cardiopulmonary Adaptation During First Day of Life in Human Neonates.

OBJECTIVE: To characterize the natural history of cardiopulmonary physiology in the first 24 hours after birth. STUDY DESIGN: A prospective observational study of healthy newborns was conducted at a large tertiary perinatal center. Echocardiography was performed at <0.5, 2-3, 7-10, and 22-24 hours of age. Specifically, assessment of pulmonary vascular resistance (PVR) (pulmonary artery acceleration time [PAAT], right ventricular ejection time, right ventricular ejection time:PAAT [PVR index], and PAAT indexed to heart rate [PAATi]), ventricular outputs (right and left), and ventricular function (tricuspid annular planar excursion, right ventricular [RV] fractional area change [FAC], RV/left ventricular [LV] global peak longitudinal strain, and LV ejection fraction) were performed. One-way repeated-measures ANOVA analysis was performed for time-dependent variables. RESULTS: In total, 15 neonates (9 males), born at 40 ± 0.8 weeks and 3.5 ± 0.5 kg, respectively, were studied. We observed increased PAATi (P < .05) by 2-3 hours, followed by a subsequent decline in all indices of PVR (PVR index, PAATi, midsystolic notching, and right-to-left ductal flow [P < .0001]). Although right and left ventricular stroke volume increased over the study interval (P < .001), LV output remained stable. All indices of RV function (tricuspid annular planar excursion, RV fractional area change 4-chamber, and RV global peak longitudinal strain-3 chamber [P < .001]) increased during the study interval. CONCLUSION: The immediate transition after birth is characterized by lower PVR, reversal of the transductal shunt, and increased biventricular stroke volume. The differential adaptive response of the RV and LV is novel and may relate to loading conditions and patent ductus arteriosus closure.

Coronary artery Doppler patterns are associated with clinical outcomes post-arterial switch operation for transposition of the great arteries.

Aims: Transposition of the great arteries (TGA) is generally repaired using the arterial switch operation (ASO) involving coronary transfer. The objective of this prospective study was to determine whether specific coronary Doppler patterns intra-operatively predicted adverse early myocardial events. Methods and results: Patients < 3 months old with TGA undergoing the ASO were eligible. All patients (when feasible) underwent an intra-operative transoesophageal echo (TEE) plus an epicardial echo and had pre-op, early post-op, pre-discharge and follow-up functional echocardiograms. The primary endpoint was a composite myocardial ischaemic event (any of: post-operative ST changes, ventricular tachycardia, need for extracorporeal membrane oxygenation (ECMO). Associations of coronary Doppler flow patterns with outcomes were modelled with logistic regression models. From May 2009 to December 2012, 40 patients (29 male, birth weight 3.29 ± 0.58 kg) were recruited. n = 32 had TEE + epicardial, four TEE only, four epicardial only. Seven (18%) patients had an adverse myocardial event (five ST changes, two ventricular tachycardia (one also ECMO). There was one death. n = 3 had a coronary artery (CA) revision post-operatively, and three had re-operation for non-CA causes. By TEE, flow reversal in the left coronary artery was associated with the composite endpoint [Odds Ratio (OR) 31.5, P = 0.004], and for chest open > 3 days (OR 6.67, P = 0.0537). Coronary Doppler flow patterns were similar by TEE and epicardial echo. The tissue Doppler parameters showed an early post-op decrease (P < 0.001 for all measures), with full recovery at follow-up in 31/37 cases. Conclusion: Intra-operative evaluation of coronary artery flow patterns should be considered for patients undergoing the ASO.

Left Ventricular Function in Healthy Term Neonates During the Transitional Period.

OBJECTIVES: To evaluate whether incorporating conventional, tissue Doppler imaging and speckle tracking echocardiography are reliable and can characterize changes in left ventricular (LV) function properly in healthy neonates in the early transitional newborn period. STUDY DESIGN: A prospective observational study was conducted in 50 healthy term neonates with a mean ± SD gestational age and birth weight of 39.3 ± 1.2 weeks and 3.5 ± 0.44 kg, respectively. All infants underwent serial echocardiograms at 15 ± 2 (day 1) and 35 ± 2 hours (day 2) of age. The LV dimensions and various functional indices including tissue Doppler imaging velocities and speckle tracking echocardiography-derived peak longitudinal strain, and systolic and diastolic strain rate were acquired and compared between time points. RESULTS: All measurements were feasible from each scan except speckle tracking echocardiography in 10% and 20% of images on days 1 and 2 of age, respectively. LV dimensions, but not functional measures, demonstrated a small to moderate positive correlation with birth weight. On day 2, a small reduction was observed in LV basal diameter, mitral valve inflow velocity time integral, and systolic velocity of the lateral wall and septum. Other indices remained unchanged. Tissue Doppler imaging-derived functional and flow-derived hemodynamic measures demonstrated the least measurement bias, and strain measurements demonstrated better reliability than strain rate, fractional shortening, and ejection fraction. CONCLUSION: The relative reliability of various echocardiographic indices to quantify LV function in neonates establish a normative dataset and provide evidence for their validity during the first 2 days of life.

[A neonate with an interlabial cyst]. / Een neonaat met een interlabiale cyste.

During a routine physical examination of a term, healthy neonate of Somalian origin we observed an anteriorly located interlabial yellow cyst with visible vascularisation on the outer surface. It caused lateralisation of the urinary meatus without notable obstruction. A Skene's duct cyst, or paraurethral cyst, was clinically diagnosed with spontaneous regression. This is a self-limiting phenomenon of unknown origin that rarely requires surgical drainage in case of urinary obstruction.

Autologous re-transfusion drain compared with no drain in total knee arthroplasty: a randomised controlled trial.

BACKGROUND: Post-operative anaemia following total knee arthroplasty is reported to impede functional mobility in the early period following surgery, whereas allogeneic blood transfusions, used to correct low post-operative haemoglobin levels, have concomitant disadvantages. The use of a post-operative autologous blood re-transfusion drainage system as well as no drainage system following total knee arthroplasty have been shown to reduce peri-operative blood loss and allogeneic blood transfusions, compared to the regularly used closed-suction drains. No randomised studies have been performed, to the best of our knowledge, that indicate the superiority of either method. MATERIALS AND METHODS: An open, randomised controlled study was conducted in 115 patients undergoing total knee arthroplasty who were randomly allocated to an autotransfusion drain or no drainage system. The primary end-point was haemoglobin level on the first post-operative day. RESULTS: In the autotransfusion group 515 mL (0-1,500 mL) of drained blood was re-transfused within the first 6 hours after surgery. Haemoglobin levels on the first (11.6 vs 11.0 g/dL), second (11.0 vs 10.3 g/dL) and third (10.5 vs 9.8 g/dL) days after surgery were significantly higher in the autotransfusion group. Total peri-operative net blood loss (1,576 mL vs 1,837 mL; -P=0.03) and allogeneic transfusion rates (10.2% vs 19.6%; P=0.15) were lower in the autotransfusion group. There were no differences in pain scores, range of motion or adverse events during hospital stay and the first 3 months after surgery. DISCUSSION: Compared with no drainage, the use of a post-operative autologous blood re-transfusion drainage system following total knee arthroplasty results in higher post-operative haemoglobin levels and less total blood loss.

Diagnostic accuracy of a serotype-specific antigen test in community-acquired pneumonia.

Our aim was to evaluate the diagnostic accuracy and clinical utility of a serotype-specific urinary antigen detection multiplex assay for identification of 13 pneumococcal serotypes (1, 3, 4, 5, 6A, 6B, 7F, 9V, 14, 18C, 19A, 19F and 23F) in urine of patients with community-acquired pneumonia. Adult patients with clinical suspicion of community-acquired pneumonia were included. In addition to standard diagnostic procedures, a urine sample was collected to perform the urinary antigen detection test. Demographic, clinical, radiological and microbiological data were collected. Among 1095 community-acquired pneumonia patients Streptococcus pneumoniae was identified as causative pathogen in 257 (23%), when using conventional diagnostic methods and in 357 (33%) when urinary antigen detection was added. Of the 49 bacteraemic episodes caused by one of the 13 serotypes covered by the urinary antigen detection, 48 were detected by the urinary antigen detection, indicating a sensitivity of 98%. Of the 77 community-acquired pneumonia episodes with a "non-urinary antigen detection" causative pathogen, none had a positive urinary antigen detection result, indicating a specificity of 100%. Addition of the urinary antigen detection test to conventional diagnostic methods increased the prevalence of S. pneumoniae community-acquired pneumonia by 39%. Using bacteraemic episodes as reference sensitivity and specificity of the urinary antigen detection was 98% and 100%, respectively.

Two patients with intellectual disability, overlapping facial features, and overlapping deletions in 6p25.1p24.3.

The clinical and molecular characterizations of two patients with a 1.4 Mb overlapping deletion in the 6p25.1p24.3 region are reported. In addition to the mild intellectual disability, they shared feeding problems in infancy and several dysmorphic facial features including a prominent forehead, almond-shaped eyes, a short philtrum, and low-set ears with square helices. The overlapping deleted region harbors six genes (RREB1, NRN1, CAGE1, LY86, SSR1, and F13A1), of which NRN1 and RREB1 are considered as candidate genes for the intellectual disability and the overlapping dysmorphism, respectively.

Postoperative autologous blood transfusion drain or no drain in primary total hip arthroplasty? A randomised controlled trial.

PURPOSE: Postoperative maintenance of high haemoglobin (Hb) levels and avoidance of homologous blood transfusions is important in total hip arthroplasty (THA). The introduction of a postoperative drainage autologous blood transfusion (ABT) system or no drainage following THA has resulted in reduction of homologous blood transfusion requirements compared with closed-suction drains. The purpose of this study was to examine which regimen is superior following THA. METHODS: A randomised controlled blinded prospective single-centre study was conducted in which 100 THA patients were randomly allocated to ABT or no drainage. The primary endpoint was the Hb level on the first postoperative day. RESULTS: The postoperative collected drained blood loss was 274 (±154) ml in the ABT group, of which 129 (±119) ml was retransfused (0-400). There was no statistical difference in Hb levels on the first postoperative day (ABT vs no drainage: Hb 11.0 vs 10.9 g/dl), on consecutive days (day 3: Hb 10.7 vs 10.2, p = 0.08) or in total blood loss (1,506 vs 1,633 ml), homologous transfusions, pain scores, Harris Hip Score, SF-36 scores, length of hospital stay or adverse events. CONCLUSIONS: The use of a postoperative autologous blood retransfusion drain did not result in significantly higher postoperative Hb levels or in less total blood loss or fewer homologous blood transfusions compared with no drain.

Minimally invasive versus classic procedures in total hip arthroplasty: a double-blind randomized controlled trial.

BACKGROUND: For total hip arthroplasty (THA), minimally invasive surgery (MIS) uses a smaller incision and less muscle dissection than the classic approach (CLASS), and may lead to faster rehabilitation. QUESTIONS/PURPOSES: Does minimally invasive hip arthroplasty result in superior clinical outcomes? PATIENTS AND METHODS: In this double-blind randomized controlled trial, 120 consecutive primary noncemented THAs in 120 patients were assigned to one of two groups (MIS or CLASS). The randomization sequence was stratified for two groups of surgeons, ie, those using a posterolateral approach (PL-CLASS or PL-MIS) and those using an anterolateral approach (AL-CLASS or AL-MIS). Length of the incisions was 18 cm for the CLASS procedures. MIS incisions were extended at the skin level to 18 cm at the end of the procedure. The primary end point was the Harris hip score (HHS) at 6 weeks postoperatively. Patient-centered questionnaires were obtained preoperatively and after 6 weeks and 1 year. RESULTS: For the patients in the MIS group (average 7.8 cm incision length), statistically significant increased mean HHSs were seen compared with the CLASS group at 6 weeks and 1 year. This difference was small and mainly caused by the favorable results of the PL-MIS. In the MIS group, surgical time was longer. A learning curve was observed based on operation time and complication rate. Although not statistically significant, the perioperative complication rate was rather high in the (anterolateral) MIS group. CONCLUSIONS: The minimal invasive approach in THA did not show a clinically relevant superior outcome in the first postoperative year. LEVEL OF EVIDENCE: Level I, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Factors associated with reduced early survival in the Oxford phase III medial unicompartment knee replacement.

The aim of this study was to determine the prognostic value of preoperative patellofemoral osteoarthritis, BMI and age for implant survival of unicompartmental knee arthroplasty (UKA) performed in patients meeting strict admission criteria. The data and radiographs of 437 unilateral Oxford phase III procedures (Biomet, Bridgend, UK) were analysed. All procedures were carried out or supervised by 13 specialised knee surgeons in three different hospitals. The study group comprised 437 patients with a median follow of 2.6 years (0.1-7.9). The cumulative standard case survival rate at 5 years, when there were still 101 patients at risk, was 84.7% (CI-95%: 80.1-89.3%). Young age (<60 years) was associated with a 2.2-fold increased adjusted risk of revision (CI: 1.08-4.43; p=0.03). The preoperative presence of radiological features of patellofemoral osteoarthritis was associated with a 0.3-fold reduced adjusted risk of revision (CI: 0.11-0.89; p=0.03). BMI>30 kg/m(2), gender, the surgeon performing the operation (either as an individual or categorised by annual surgical UKA caseload, i.e., more or less than 10 UKAs) and the hospital in which surgery took place did not predict implant survival of UKA. We conclude that young patients (<60 years) experience an increased early risk of revision for UKA when compared to older patients (>60 years). Obesity (BMI>30 kg/m(2)) and preoperative patellofemoral osteoarthritis are not associated with a decreased implant survival and therefore should not be considered risk factors in this context.

Comparison of the Diskus inhaler and the Handihaler regarding preference and ease of use.

Many chronic obstructive pulmonary disease (COPD) patients use their inhaler ineffectively and there is a trend towards increased inhaler resistance. We wanted to answer two questions: Is there a difference in preference and ease of use between Diskus (DK) and Handihaler (HH)? How acceptable are inhalation resistances? Sixty COPD patients, naive to DK and HH, but experienced in the use of other inhalers, had to read the instruction leaflet and demonstrate their inhalation technique. If errors were made, instruction was given and inhalation technique was checked again. Patients had to state a preference for DK or HH. Subsequently they inhaled through a range of resistances and scored the acceptability. There was no difference in the number of instructions needed for both inhalers. One third inhaled perfectly after reading the instruction leaflet, which increased to 85% after one instruction. More patients preferred the DK (43) than the HH (16). With decreasing resistance acceptability increases, but it reaches a plateau. Patients have a clear preference for the DK. There is no difference in the number of instructions needed to obtain a perfect inhalation technique, but for some patients one instruction is not enough. The trend to increase the resistance of inhalers has reached a critical point with regard to acceptability.

Reduced cortical activity during maximal bilateral contractions of the index finger.

The bilateral deficit refers to the phenomenon in which homologous muscles produce per muscle less force when contracting simultaneously than when contracting individually. The mechanism underlying the bilateral deficit is still unknown, but the most likely cause is a decline in the activation of motor units during bilateral contractions. In the present study, we used functional magnetic resonance imaging (fMRI) to measure the degree of brain activity during unilateral and bilateral maximal contractions in combination with force and EMG measurements. Subjects performed, in a semi-randomized order, maximal isometric contractions (MVC) with the right index finger, the left index finger and with both fingers simultaneously. During the task, brain activation was measured with a 3 T MR scanner, in combination with force and EMG recordings. The most important activated areas in the brain during the contractions were the sensorimotor cortex (precentral and postcentral gyrus), cerebellum, premotor cortex and supplementary motor area. During bilateral contractions, a significant decline in force and EMG values was found and detailed analysis of the brain activation data showed that this decline was accompanied with a significant decline in the activation of the precentral gyrus. This result suggests that the bilateral decline is the resultant of a decline in input to the primary motor area and shows that the main source of the bilateral deficit lies upstream of the primary motor cortex.

Ruling out clinically suspected pulmonary embolism by assessment of clinical probability and D-dimer levels: a management study.

D-dimer test combined with clinical probability assessment has been proposed as the first step in the diagnostic work-up of patients with suspected pulmonary embolism (PE). In a prospective management study we investigated the safety and efficiency of excluding PE by a normal D-dimer combined with a low or moderate clinical probability. Of the 202 study patients this combination ruled out PE in 64 (32%) patients. The 3-month thromboembolic risk in these patients was 0% (95% CI, 0.0-5.6%). The prevalence of PE in the entire cohort was 29% (59 patients), whereas in the low, moderate and high clinical probability groups this was 25%, 26% and 50%, respectively. We conclude that ruling out suspected PE by a normal D-dimer combined with a low or moderate clinical probability appears to be a safe and efficient strategy. The accuracy of the clinical probability assessment is modest.