Hemoconcentration and predictors in Shiga toxin-producing E. coli-hemolytic uremic syndrome (STEC-HUS).

BACKGROUND: Hemoconcentration has been identified as a risk factor for a complicated course in Shiga toxin-producing E. coli-hemolytic uremic syndrome (STEC-HUS). This single-center study assesses hemoconcentration and predictors at presentation in STEC-HUS treated from 2009-2017. METHODS: Data of 107 pediatric patients with STEC-HUS were analyzed retrospectively. Patients with mild HUS (mHUS, definition: max. serum creatinine < 1.5 mg/dL and no major neurological symptoms) were compared to patients with severe HUS (sHUS, definition: max. serum creatinine ≥ 1.5 mg/dL ± major neurological symptoms). Additionally, predictors of complicated HUS (dialysis ± major neurological symptoms) were analyzed. RESULTS: Sixteen of one hundred seven (15%) patients had mHUS. Admission of patients with sHUS occurred median 2 days earlier after the onset of symptoms than in patients with mHUS. On admission, patients with subsequent sHUS had significantly higher median hemoglobin (9.5 g/dL (3.6-15.7) vs. 8.5 g/dL (4.2-11.5), p = 0.016) than patients with mHUS. The product of hemoglobin (g/dL) and LDH (U/L) (cutoff value 13,302, sensitivity 78.0%, specificity of 87.5%) was a predictor of severe vs. mild HUS. Creatinine (AUC 0.86, 95% CI 0.79-0.93) and the previously published score hemoglobin (g/dL) + 2 × creatinine (mg/dL) showed a good prediction for development of complicated HUS (AUC 0.87, 95% CI 0.80-0.93). CONCLUSIONS: At presentation, patients with subsequent severe STEC-HUS had a higher degree of hemoconcentration. This underlines that fluid loss or reduced fluid intake/administration may be a risk factor for severe HUS. The good predictive value of the score hemoglobin (g/dL) + 2 × creatinine (mg/dL) for complicated HUS could be validated in our cohort. A higher resolution version of the Graphical abstract is available as Supplementary Information.

Effect of hemodialysis on impedance cardiography (electrical velocimetry) parameters in children.

BACKGROUND: Pediatric hemodialysis (HD) patients have a high incidence of cardiovascular morbidity and mortality. The study aim was to investigate whether impedance cardiography (electrical velocimetry, EV) is suitable as a hemodynamic trend monitoring tool in pediatric patients during HD. METHODS: Measurements by EV were obtained before, during, and after HD in a prospective single-center pediatric observational study. In total, 54 dialysis cycles in four different pediatric patients with end-stage kidney disease on chronic HD were included. EV parameters analyzed were heart rate (HR), stroke volume (SV), stroke volume index (SI), cardiac output (CO), cardiac index (CI), thoracic fluid content (TFC), index of contractility (ICON), stroke volume variation (SVV), variation of ICON (VIC), R-R interval (TRR), pre-ejection period (PEP), left ventricular ejection time (LVET), and systolic time ration (STR). Systemic vascular resistance index (SVRI) was calculated. RESULTS: EV did measure significant changes in cardiovascular parameters associated with HD. The following parameters increased after HD: HR (9%), SVV (19%), VIC (33%), PEP (8%), and STR (18%). A decrease after HD was measured in SV (18%), SI (18%), CO (10%), CI (10%), TFC (10%), ICON (7%), TRR (7%), LVET (8%), and LVET (8%). SVRI was not affected by HD. The changes were correlated to ultrafiltration. HD cycles without fluid withdrawal also altered cardiovascular parameters. CONCLUSIONS: Pediatric HD with and without fluid withdrawal changes hemodynamic EV monitoring parameters. Possibly EV may be useful to optimize HD management in pediatric patients.

Successful Stem Cell Apheresis Using Spectra Optia in a 6 kg Child With Atypical Teratoid/Rhabdoid Tumor.

Peripheral blood stem cell apheresis has become a routine procedure for the collection of peripheral blood stem cells to enable high-dose chemotherapy followed by autologous stem cell transplantation in high-risk pediatric malignancies. However, the procedure remains challenging in very low-weight infants due to high extracorporeal blood volume and citrate toxicity. Our case report demonstrates in detail a successful and complication-free large-volume leukapheresis in a very small infant weighing 6 kg using a Spectra Optia apheresis system after placing a femoral double-lumen Shaldon catheter. Anticoagulation was achieved by citrate dextrose solution without the use of heparin. The total amount of blood being processed during the procedure equaled almost 4 times the total blood volume of the patient. The final apheresis product contained 14.0×10 CD34 cells/kg body weight. The infant was diagnosed with an atypical teratoid/rhabdoid tumor of the thalamus and third ventricle at the age of 3 months and had a history of epileptic seizures.

A prospective observational trial evaluating factors predictive of accurate endotracheal tube positioning in neonates and small infants.

BACKGROUND: There is a high incidence of endotracheal tube malposition in neonates and small infants. Yet, verification of accurate endotracheal tube location via radiographic imaging involves radiation exposure. AIMS: This study aimed to identify demographic and clinical parameters associated with accurate endotracheal tube positioning. METHODS: We conducted a prospective single-center study with term and preterm neonates and small infants between January 2018 and November 2019. We investigated correlations between ten variables and accurate endotracheal tube position. RESULTS: One hundred and sixty eight nasal intubations in 139 patients (weight 390-5000 g) were analyzed. An accurate tube position was confirmed by radiographic imaging in 71.4% of the intubations. The endotracheal tube was high in 8.3% and low in 20.2% of the cases. Male gender was the only variable that significantly correlated with an accurate endotracheal tube position (OR 2.5; 95% CI: 1.3, 5.0; P = .010). CONCLUSION: So far, no parameter has proven to be able to predict accurate endotracheal tube position in neonates reliably. These findings emphasize the indispensability of postintubation imaging in neonates and small infants.

The first case of domino-split-liver transplantation in maple syrup urine disease.

The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet. The organ was allocated to a 14-year-old girl (55 kg) with primary sclerosing cholangitis. Due to excellent organ quality and suitable anatomy, a backward split for a girl of 3 months (5 kg) with decompensated liver cirrhosis due to biliary atresia was performed. The postoperative course was without relevant complications, and the three recipients were discharged on postoperative days 28, 29, and 45, respectively, with good organ function. BCAAs in plasma were normal in the two domino graft recipients, and the MSUD patient showed mildly elevated but stable BCAA concentrations despite an unrestricted diet. Split-domino LTX enabled successful transplantation of three patients of the waiting list with only one deceased donor graft.

Functional assessment of the kidneys in a 10 month-old child with renal artery stenosis by intravoxel incoherent motion.

Renovascular stenosis is an important cause for arterial hypertension in childhood. We report a 10-month-old girl with arterial hypertension caused by right-sided renal artery stenosis detected by Doppler ultrasound. Magnetic resonance imaging (MRI) was performed before renal artery angioplasty to depict vascular anatomy in detail and to retrieve additional functional information of the kidneys by analysis of intravoxel incoherent motion (IVIM). The value of quantitative diffusion weighted imaging of the kidneys prior to percutaneous transluminal renal angioplasty (PTRA) is discussed.

Effect of patent ductus arteriosus and patent foramen ovale on left ventricular stroke volume measurement by electrical velocimetry in comparison to transthoracic echocardiography in neonates.

This prospective single-center observational study compared impedance cardiography [electrical velocimetry (EV)] with transthoracic echocardiography (TTE, based on trans-aortic flow) and analyzed the influence of physiological shunts, such as patent ductus arteriosus (PDA) or patent foramen ovale (PFO), on measurement accuracy. Two hundred and ninety-one triplicate simultaneous paired left ventricular stroke volume (LVSV) measurements by EV (LVSVEV) and TTE (LVSVTTE) in 99 spontaneously breathing neonates (mean weight 3270 g; range 1227-4600 g) were included. For the whole cohort, the mean absolute LVSVEV was 5.5 mL, mean LVSVTTE was 4.9 mL, resulting in an absolute Bland-Altman bias of -0.7 mL (limits of agreement LOA -3.0 to 1.7 mL), relative bias -12.8 %; mean percentage error MPE 44.9 %; true precision TPEV 33.4 % (n = 99 aggregated data points). In neonates without shunts (n = 32): mean LVSVEV 5.0 mL, mean LVSVTTE 4.6 mL, Bland-Altman bias -0.4 mL (LOA -2.8 to 2.0 mL), relative bias -8.2 %; MPE 50.7 %; TPEV 40.9 %. In neonates with shunts (PDA and/or PFO; n = 67): mean LVSVEV 5.8 mL, mean LVSVTTE 5.0 mL, bias -0.8 mL (LOA -3.1 to 1.5 mL), relative bias -14.8 %, MPE 41.9 %, TPEV 29.3 %. Accuracy was affected by PDA and/or PFO, with a significant increase in the relative difference in LVSVEV versus LVSVTTE: Subjects without shunts -2.9 % (n = 91), PFO alone -9.6 % (n = 125), PDA alone -14.0 % (n = 12), and PDA and PFO -18.5 % (n = 63). Physiological shunts (PDA and/or PFO) in neonates affect measurement accuracy and cause overestimation of LVSVEV compared with LVSVTTE.

Near-normal values of extravascular lung water in children.

OBJECTIVES: To define near-normal values of extravascular lung water indexed to body weight in children. DESIGN: Prospective multicenter observational study. SETTING: Medical/surgical PICUs of 5 multinational hospitals. PATIENTS: Fifty-eight children with a median age of 4 years (range 1 month to 17 year) with heterogeneous PICU admission diagnoses were included. Extravascular lung water measurements from these children were collected after resolution of their illness. Obtained values were indexed to actual body weight and height and subsequently related to age. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Extravascular lung water indexed to body weight correlated with age (r2 = 0.7) and could be categorized in three-age groups consisting of significantly different median extravascular lung water indexed to body weight values (5th-95th percentile): less than 1 year, 9-29 mL/kg; 1-5 years, 7-25 mL/kg; and 5-17 years, 5-13 mL/kg. Extravascular lung water indexed to height did not correlate to age and resulted in an age-independent near-normal value of less than 315 mL/m. CONCLUSIONS: Younger children have higher values of extravascular lung water indexed to actual body weight. Age categorized near-normal values of extravascular lung water indexed to body weight are presented for possible clinical use. Furthermore, we suggest to index extravascular lung water to height, which seems to be age independent.

Impedance cardiography (electrical velocimetry) and transthoracic echocardiography for non-invasive cardiac output monitoring in pediatric intensive care patients: a prospective single-center observational study.

INTRODUCTION: Electrical velocimetry (EV) is a type of impedance cardiography, and is a non-invasive and continuously applicable method of cardiac output monitoring. Transthoracic echocardiography (TTE) is non-invasive but discontinuous. METHODS: We compared EV with TTE in pediatric intensive care patients in a prospective single-center observational study. Simultaneous, coupled, left ventricular stroke volume measurements were performed by EV using an Aesculon® monitor and TTE (either via trans-aortic valve flow velocity time integral [EVVTI], or via M-mode [EVMM]). H0: bias was less than 10% and the mean percentage error (MPE) was less than 30% in Bland-Altman analysis between EV and TTE. If appropriate, data were logarithmically transformed prior to Bland-Altman analysis. RESULTS: A total of 72 patients (age: 2 days to 17 years; weight: 0.8 to 86 kg) were analyzed. Patients were divided into subgroups: organ transplantation (OTX, n = 28), sepsis or organ failure (SEPSIS, n = 16), neurological patients (NEURO, n = 9), and preterm infants (PREM, n = 26); Bias/MPE for EVVTI was 7.81%/26.16%. In the EVVTI subgroup analysis for OTX, NEURO, and SEPSIS, bias and MPE were within the limits of H0, whereas the PREM subgroup had a bias/MPE of 39.00%/46.27%. Bias/MPE for EVMM was 8.07%/37.26% where the OTX and NEURO subgroups were within the range of H0, but the PREM and SEPSIS subgroups were outside the range. Mechanical ventilation, non-invasive continuous positive airway pressure ventilation, body weight, and secondary abdominal closure were factors that significantly affected comparison of the methods. CONCLUSIONS: This study shows that EV is comparable with aortic flow-based TTE for pediatric patients.

Effect of cerebral circulatory arrest on cerebral near-infrared spectroscopy in pediatric patients.

BACKGROUND/AIMS: The aim was to investigate whether cerebral transcutaneous near-infrared spectroscopy (NIRS) or two-site NIRS is a suitable monitoring tool to detect or confirm a cerebral circulatory arrest in pediatric intensive care unit (PICU) patients. METHODS: Prospective single-center pediatric observational study. Simultaneous NIRS measurements over forehead (cNIRS, crS02) and kidney (rNIRS, rrSO2), at the same time, the cardiac output were determined by transthoracic echocardiography. Area under the curve (AUC) in the receiver-operating curve (ROC) was analyzed for NIRS regarding cerebral circulatory arrest. RESULTS: There were two groups of patients (weight 2.1-73 kg): Group A: patients with intact cerebral perfusion (n = 36). Group B: patients with cerebral circulatory arrest (n = 8) proven by Doppler ultrasound scan or perfusion scintigraphy. There was no difference in cardiac output between the groups. PICU mortality for Group A was 3/36 (8.3%), for Group B 8/8, (100%). Mean cNIRS values were significantly higher with 68.92 (SEM = 2.54, SD = 15.25) in Group A compared with 34.63 (SEM = 5.36, SD = 15.15) in Group B (P < 0.001). ROC analysis for cNIRS detecting cerebral circulatory arrest was significant (AUC 0.948, 95% confidence interval 0.876-1.000, SE = 0.037, P < 0.001). Discrimination was optimal at 46 for cNIRS, at 36.5 for the difference rNIRS-cNIRS and at 0.5646 for the quotient cNIRS/rNIRS. The probability of a cerebral circulatory arrest was 77.8% (cNIRS) and 87.5% (combinations of cNIRS and rNIRS) at these cutoffs. CONCLUSIONS: cNIRS did detect cerebral circulatory arrest with high sensitivity. Specificity was, however, not high enough to confirm a cerebral circulatory arrest.

Thyroid function, thyroid antibodies and early postnatal development in neonates of mothers with thyroid disorders.

BACKGROUND: Thyroid dysfunction during pregnancy is relatively common and can cause obstetric complications and significantly influence fetal development. AIMS: We aimed to evaluate postnatal clinical and laboratory characteristics in the first days of life in infants born to mothers with a thyroid disorder. STUDY DESIGN AND SUBJECTS: We conducted a retrospective single-center study with neonates born between January 2010 and May 2020. Early laboratory parameters and clinical findings in neonates of mothers with different maternal thyroid disorders were analysed. RESULTS: We included 314 newborns of mothers with Hashimoto's thyroiditis, 171 with non-Hashimoto's hypothyroidism, 42 with Graves' disease, 12 with non-Graves' hyperthyroidism, and 190 neonates born to mothers without thyroid dysfunction. No demographic, clinical, and laboratory differences were observed between neonates from mothers with a thyroid disorder and healthy mothers. FT3 and fT4 correlated positively with gestational age (p < 0.001; p < 0.001) and negatively with maximum postnatal weight loss (p = 0.043; p < 0.001). High fT3 values were associated with lower maximum bilirubin levels (p = 0.020). CONCLUSION: Despite an increased morbidity risk due to the transplacental exposure to maternal antibodies, most neonates born to mothers with thyroid disorders show normal postnatal development and thyroid function tests during the first days of life.

Mucha-Habermann disease: a pediatric case report and proposal of a risk score.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare inflammatory dermatological disease. A case of a 13-year-old boy with FUMHD possibly triggered by mycoplasma infection is presented. Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14/119 (12%, CI 6-17%), and lethality in children was lower (1/54, 2%, CI 0-6%) compared to adults (13/65, 20%, CI 11-31%). Risk factors for a fatal outcome (likelihood ratio; P) were sepsis (24.97, P < 0.001), adult vs. pediatric patient age (11.19; P = 0.001), systemic involvement (19.97, P < 0.001), and mucosal involvement (4.58; P = 0.032). The proposed FUMHD mortality risk score = Age/10 + 4 + 4 (if systemic involvement) + 1 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune-suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.

Iatrogenic Blood Loss in Very Low Birth Weight Infants and Transfusion of Packed Red Blood Cells in a Tertiary Care Neonatal Intensive Care Unit.

An adequate blood volume is important for neonatal adaptation. The study objective was to quantify the cumulative iatrogenic blood loss in very low birth weight (VLBW) infants by blood sampling and the necessity of packed red cell transfusions from birth to discharge from the hospital. In total, 132 consecutive VLBW infants were treated in 2019 and 2020 with a median birth weight of 1180 g (range 370-1495 g) and a median length of stay of 54 days (range 0-154 days) were included. During the initial four weeks of life, the median absolute amount of blood sampling was 16.5 mL (IQR 12.3-21.1 mL), sampling volume was different with 14.0 mL (IQR 12.1-16.2 mL) for non-transfused infants and 21.6 mL (IQR 17.5-29.4 mL) for transfused infants. During the entire length of stay, 31.8% of the patients had at least one transfusion. In a generalized logistic regression model, the cumulative amount of blood sampling (p < 0.01) and lower hematocrit at birth (p = 0.02) were independent predictors for the necessity of blood transfusion. Therefore, optimized patient blood management in VLBW neonates should include sparse blood sampling to avoid iatrogenic blood loss.

Diagnostic accuracy of interleukin-6 for early-onset sepsis in preterm neonates.

Background: Early-onset sepsis (EOS) is a leading cause of morbidity and mortality among neonates. Yet, accurate diagnosis remains a major challenge in clinical routine.Objective: The aim of this study was to evaluate the diagnostic accuracy of Interleukin-6 (IL-6) in combination with other objective perinatal data for early-onset sepsis (EOS) in preterm neonates.Methods: We conducted a retrospective nested case-control study with preterm neonates with a birth weight < 2000 g born in our NICU between January 2007 and June 2016. Differences of IL-6 levels and other perinatal clinical and laboratory data between neonates with and without EOS were statistically analyzed.Results: Sixty-seven preterm infants with and 115 neonates without EOS were included in this study. Specificity and sensitivity for IL-6 were 72.8% and 75.0%, respectively, with an area under the curve of 0.804 at a cut-off point of 40 ng/l. Depending on the statistical method applied, combining IL-6 with a second perinatal factor led either to an increase of specificity (82.4-100%) or sensitivity (75.0-92.2%).Conclusion: The combination of IL-6 with other perinatal factors can significantly increase specificity and sensitivity in the diagnosis of EOS. However, overall diagnostic accuracy cannot be notably improved as there is a tradeoff between sensitivity and specificity. Although these findings do not necessarily apply in clinical routine, they can be of substantial value in the assistance of individual decision making.

Improved Less Invasive Surfactant Administration Success in Preterm Infants after Procedure Standardization.

Less invasive surfactant administration (LISA) has been introduced at our tertiary Level IV perinatal center since 2016 with an unsatisfactory success rate, which we attributed to an inconsistent, non-standardized approach and ambiguous patient inclusion criteria. This study aimed to improve the LISA success rate to at least 75% within 12 months by implementing a highly standardized LISA approach combined with team training. The Plan Do Study Act method of quality improvement was used for this initiative. Baseline assessment included a review of patient medical records 12 months before the intervention regarding patient characteristics, method success rate, respiratory, and adverse outcomes. A multi-professional team developed a standardized LISA approach and a training program including an educational film, checklists, pocket cards, and team briefings. Twenty-one preterm infants received LISA before and 24 after the intervention. The mean LISA success rate improved from 62% before the intervention to 92% (p = 0.029) after the intervention. Implementing a highly standardized LISA approach and multi-professional team training significantly improved the methods' success rate.