Study draft: "UVC-You Will See" study: longer vs. shorter umbilical venous catheter (UVC) dwell time (6-10 vs. 1-5 days) in very premature infants with birth weight < 1250 g and/or gestational age < 30 weeks.

BACKGROUND: Umbilical venous catheters (UVCs) are often used in preterm infants. Their use is associated with complications (infections, clot formation, organ injury). Very preterm infants with acquired bloodstream infection are at a higher risk for death and important morbidities (e.g., adverse neurodevelopmental outcomes). It is standard clinical practice to remove UVCs in the first days of life. Replacement of intravenous access is often performed using percutaneously inserted central catheters (PICCs). It is unclear whether serial central line use affects the rates of catheter-related complications. METHODS: A multicenter randomized controlled trial (random group assignment) was performed in 562 very premature (gestational age < 30 weeks) and/or very low birth weight infants (< 1250 g) requiring an UVC for administration of parenteral nutrition and/or drugs. Group allocation was random. HYPOTHESIS: A UVC dwell time of 6-10 days (281 infants) is not associated with an increased rate of central venous catheter (UVC, PICC)-related complications compared to 1-5 days (281 infants), and a longer UVC dwell time will significantly reduce the number of painful, invasive procedures associated with the need for vascular access as well as radiation exposure, use of antibiotics, and medical costs. PRIMARY OUTCOME PARAMETER: The number of catheter-related bloodstream infections and/or catheter-related thromboses and/or catheter-associated organ injuries related to the use of UVC/PICC was the primary outcome. CONCLUSION: Extending the UVC dwell time may significantly reduce the number of painful invasive procedures, with the potential to positively impact not only long-term pain perception but also important social competencies (attention, learning, and behavior). Thus, the "UVC-You Will See" study has the potential to substantially change current neonatal intensive care practice.

Expression of TRAIL, IP-10, and CRP in children with suspected COVID-19 and real-life impact of a computational signature on clinical decision-making: a prospective cohort study.

PURPOSE: We evaluated the host-response marker score "BV" and its components TRAIL, IP-10, and CRP in SARS-CoV-2 positive children, and estimated the potential impact on clinical decision-making. METHODS: We prospectively analyzed levels of TRAIL, IP-10, CRP, and the BV score, in children with suspected COVID-19. Classification of infectious etiology was performed by an expert panel. We used a 5-point-questionnaire to evaluate the intention to treat with antibiotics before and after receiving test results. RESULTS: We screened 111 children, of whom 6 (5.4%) were positive for SARS-CoV-2. A total of 53 children were included for the exploratory analysis. Median age was 3.1 years (interquartile range [IQR] 1.3-4.3), and 54.7% (n = 29) were girls. A viral and a bacterial biomarker pattern was found in 27/53 (50.9%) and 15/53 (28.3%), respectively. BV scores differed between COVID-19, children with other viral infections, and children with bacterial infections (medians 29.5 vs. 9 vs. 66; p = 0.0006). Similarly, median TRAIL levels were different (65.5 vs. 110 vs. 78; p = 0.037). We found no differences in IP-10 levels (555 vs. 504 vs. 285; p = 0.22). We found a concordance between physicians' "unlikely intention to treat" children with a viral test result in most cases (n = 19/24, 79.2%). When physicians expressed a "likely intention to treat" (n = 15), BV test revealed 5 bacterial, viral, and equivocal scores each. Antibiotics were withheld in three cases (20%). Overall, 27/42 (64%) of pediatricians appraised the BV test positively, and considered it helpful in clinical practice. CONCLUSION: Host-response based categorization of infectious diseases might help to overcome diagnostic uncertainty, support clinical decision-making and reduce unnecessary antibiotic treatment.

Standardized Treatment and Diagnostic Approach to Reduce Disease burden in the early postoperative phase in children with congenital heart defects-STANDARD study: a pilot randomized controlled trial.

To explore the effect of a daily goal checklist on pediatric cardiac intensive care unit (PCICU) length of stay (LOS) after congenital heart surgery. This study is a prospective randomized single-center study. Group characteristics were as follows: STANDARD group: n = 30, 36.7% female, median age 0.9 years; control group: n = 33, 36.4% female, median age 1.1 years. Invasive ventilation time, STAT categories, mean vasoactive-inotropic score (VIS)24h, maximal (max.) VIS24h, mean VIS24-48h, max. VIS24-48h, VIS category, number of sedatives, analgesics, diuretics, number of deployed diagnostic modalities, morbidities, and mortality did not differ between both groups. Median PCICU LOS was 96.0 h (STANDARD group) versus 101.5 h (control group) (p = 0.63). In the overall cohort, univariate regression analysis identified age at surgery (b = -0.02), STAT category (b = 18.3), severity of CHD (b = 40.6), mean VIS24h (b = 3.5), max. VIS24h (b = 2.2), mean VIS24-48h (b = 6.5), and VIS category (b = 13.8) as significant parameters for prolonged PCICU LOS. In multivariate regression analysis, age at surgery (b = -0.2), severity of CHD (b = 44.0), and mean VIS24h (b = 6.7) were of significance. Within the STANDARD sub-group, univariate regression analysis determined STAT category (b = 32.3), severity of CHD (b = 70.0), mean VIS24h (b = 5.0), mean VIS24-48h (b = 5.9), number of defined goals (b = 2.6), number of achieved goals (b = 3.3), number of not achieved goals (b = 10.8), and number of unevaluated goals (b = 7.0) as significant parameters for prolonged PCICU LOS. Multivariate regression analysis identified the number of defined goals (b = 2.5) and the number of unevaluated goals (b = -3.0) to be significant parameters.   Conclusion: The structured realization and recording of daily goals is of advantage in patients following pediatric cardiac surgery by reducing PCICU LOS. What is known: • Communication errors are the most frequent reasons for adverse events in intensive care unit patients. • Improved communication can be achieved by discussion and documentation of the patients' goals during daily rounds. What is new: • In the overall cohort age at surgery, severity of congenital heart defect and mean vasoactive inotropic score within the first 24 hours had significant impact on pediatric cardiac intensive care unit (PCICU) length of stay (LOS). • In the intervention group, the number of defined goals and the number of unevaluated goals were significant parameters for prolonged PCICU LOS.

A rare coronary artery anomaly in a 14-year-old boy with sudden cardiac arrest.

Congenital coronary artery anomalies represent a rare cause for cardiac arrest in children and adults; however, most of these anomalies are asymptomatic and incidental findings. We report on a 14-year-old boy who was admitted to our hospital after cardiopulmonary resuscitation at home. Diagnostic workup including histopathology revealed parvovirus B19 in endomyocardial biopsy. Moreover, cardiac catheterisation as well as CT angiography identified an anomalous origin of the right coronary artery with an interarterial course. Since this anomalous coronary artery might have caused impaired myocardial perfusion causing cardiac arrest, surgical correction and implantation of a cardioverter defibrillator were performed. The further post-operative clinical course (7 months) has been uneventful.

Inferior vena cava thrombosis-rope ladder sign.

Congenital heart disease comprises one of the largest groups of congenital defects, affecting approximately 1% of births. Advances in pre- and postoperative critical care treatment as well as surgery and interventional procedures have improved survival rates, but treatment and long-term care of children with complex congenital heart disease remains challenging, and is associated with a number of complications.Here, we report on a 17-month-old infant with congenital univentricular heart disease who devloped post-operatively inferior vena cava (IVC) thrombosis. IVC thrombosis was confirmed by a bedside contrast media study (X-ray) demonstrating collateral paravertebral circulation along the paravertebral sinuses bilaterally into the azygos and hemiazygos vein ("rope ladder sign"), with no contrast media detected in the IVC. The infant was subsequently started on aspirin and clopidogrel.

[Pseudotumor cerebri in children and adolescents at the Saarland University Medical Center: a retrospective study]. / Pseudotumor cerebri bei Kindern und Jugendlichen am Universitätsklinikum des Saarlandes: Eine retrospektive Studie.

Pseudotumor cerebri (PTC) is defined as a rare disease with a pathological increase in intracranial pressure of unknown origin. The aim of this retrospective study was to establish a uniform diagnostic and therapeutic protocol for children and adolescents for the Saarland University Medical Center. Data from 28 patients with pseudotumor cerebri aged 0-17 years in the period 2008-2018 were retrospectively collected and statistically analyzed. The purpose of this study was to generate a better understanding of the clinical entity of pseudotumor cerebri in children and adolescents. Distinctive features, such as pubertal or adolescent age, female gender and obesity could be highlighted. The data collected in this study were used to develop an in-house standard for the diagnosis and treatment of children and adolescents with pseudotumor cerebri.

[Assessment of the importance of neuropediatric diagnostics in the initial clarification of autism]. / Beurteilung des Stellenwertes der neuropädiatrischen Diagnostik im Rahmen der initialen Autismusabklärung.

BACKGROUND: The diagnostics of autism spectrum disorder is complex due to missing biological markers and numerous comorbidities. The aim was to assess the role of neuropediatric diagnostics and to develop a standard operating procedure for a targeted assessment. METHOD: All patients presenting to the neuropediatric outpatient clinic at Saarland University Hospital between April 2014 and December 2017 with ICD code F84 pervasive developmental disorders were included. RESULTS: A total of 82 patients were included (male 78%, female 22%; mean age 5.9 ± 2.9 years, range 2-16 years). The most frequent examination was electroencephalography (EEG) (74/82; 90.2%) with pathological findings in 33.8% (25/74). Based on the history and/or EEG epilepsy was diagnosed in 19.5% (16/82). Magnetic resonance imaging (MRI) was performed in 49/82 (59.8%) patients, 22/49 (44.9%) showed at least 1 cerebral abnormality and definite pathologies could be detected in 63.6% (14/22). A metabolic diagnostic work-up was performed in 44/82 (53.7%) cases and in 5/44 (11.4%) it resulted in a diagnosis or suspicion of a metabolic disease. Genetic testing results were available in 29/82 (35.4%) children and 12/29 (41.4%) showed abnormal results. Delay in motor development was more frequently associated with comorbidities, EEG abnormalities, epilepsy and abnormalities in metabolic and genetic testing. CONCLUSION: Neuropediatric examination in cases of suspected autism should include a detailed history, a thorough neurological examination and an EEG. An MRI, comprehensive metabolic and genetic testing are only recommended if clinically indicated.

Something is up in the air: pneumothorax and pneumopericardium in a 29-week preterm infant.

This report highlights the early and unusual detection of a pneumopericardium by echocardiography prior to potential development of cardiocirculatory compromise. It is important to consider pneumopericardium into the differential diagnosis when difficulties arise in the visualization of the heart by conventional echocardiography. Pneumopericardium is associated with a high mortality rate and may be effectively treated by immediate insertion of a pericardial catheter.

Umbilical venous catheter- and peripherally inserted central catheter-associated complications in preterm infants with birth weight < 1250 g : Results from a survey in Austria and Germany.

BACKGROUND AND OBJECTIVE: Umbilical venous catheters (UVC) and peripherally inserted central catheters (PICC) are commonly used in preterm infants but have been associated with a number of serious complications. We performed a survey in Austria and Germany to assess the use of UVCs and PICCs in preterm infants with a birth weight < 1250 g and associated rates of catheter-related adverse events. METHODS: Electronic survey of participating centers of the NeoVitaA trial. Main outcome parameter was the reported rates of UVC- and PICC-associated complications (infection, thrombosis, emboli, organ injury, arrhythmia, dislocation, miscellaneous). RESULTS: In total, 20 neonatal intensive care units (NICU) providing maximal intensive care in Austria and Germany (level I) were contacted, with a senior neonatologist response rate of 12/20 (60%). The reported rates for UVC with a dwell time of 1-10 days were bacterial infection: 4.2 ± 3.4% (range 0-10%); thrombosis: 7.3 ± 7.1% (0-20%); emboli: 0.9 ± 2.0% (0-5%); organ injury: 1.1 ± 1.9% (0-5%); cardiac arrhythmia: 2.2 ± 2.5% (0-5%); and dislocation: 5.4 ± 8.7% (0-30%); and for PICCs with a dwell time of 1-14 days bacterial infection: 15.0 ± 3.4% (range 2.5-30%); thrombosis; 4.3 ± 3.5% (0-10%); emboli: 0.8 ± 1.6% (0-5%); organ injury: 1.5 ± 2.3% (0-5%); cardiac arrhythmia: 1.5 ± 2.3% (0-5%), and dislocation: 8.5 ± 4.6% (0-30%). CONCLUSION: The catheter-related complication rates reported in this survey differed between UVCs and PICCs and were higher than those reported in the literature. To generate more reliable data on this clinically important issue, we plan to perform a large prospective multicenter randomized controlled trial investigating the non-inferiority of a prolonged UVC dwell time (up to 10 days) against the early change (up to 5 days) to a PICC.

New Insights into the Education of Children with Congenital Heart Disease with and without Trisomy 21.

Background and Objectives: Patients with congenital heart disease (CHD), especially as a concomitant syndromal disease of trisomy 21 (T21), are at risk for impaired neurodevelopment. This can also affect these patients' education. However, there continues to be a research gap in the educational development of CHD patients and T21 CHD patients. Materials and Methods: In total, data from 2873 patients from the German National Register for Congenital Heart Defects were analyzed. The data are based on two online education surveys conducted among patients registered in the National Register for Congenital Heart Defects (2017, 2020). Results: Of 2873 patients included (mean age: 14.1 ± 4.7 years, 50.5% female), 109 (3.8%) were identified with T21 (mean age: 12.9 ± 4.4 years, 49.5% female). T21 CHD participants had a high demand for early specific interventions (overall cohort 49.1%; T21 cohort 100%). T21 CHD children more frequently attended special schools and, compared to non-trisomy 21 (nT21) CHD patients, the probability of attending a grammar school was reduced. In total, 87.1% of nT21 CHD patients but 11% of T21 CHD patients were enrolled in a regular elementary school, and 12.8% of T21 CHD patients could transfer to a secondary school in contrast to 35.5% of nT21 CHD patients. Most of the T21 CHD patients were diagnosed with psychiatric disorders, e.g., learning, emotional, or behavioral disorders (T21 CHD patients: 82.6%; nT21 CHD patients: 31.4%; p < 0.001). Conclusions: CHD patients are at risk for impaired academic development, and the presence of T21 is an aggravating factor. Routine follow-up examinations should be established to identify developmental deficits and to provide targeted interventions.

Is There a Role for Cerebral Ultrasonography in Near-Term/Term Neonates Following Assisted Vaginal Delivery? A Prospective, Single-Center Study.

AIM: To evaluate the role of cerebral ultrasonography studies (CUSS) in detecting intracerebral and cranial pathologies (hemorrhages, ischemia, skull fractures) in near-term and term neonates following assisted vaginal delivery. PATIENTS AND METHODS: Prospective single-center study (11/2017-11/2018) at the University Children's Hospital of Saarland, Homburg, Germany including newborns with a gestational age ≥ 36 weeks born by assisted vaginal delivery. In all newborns, a standardized CUSS was performed within the first three days of life prior to discharge. RESULTS: 200 neonates (43.0 % female, 57.0 % male; gestational age 39.6 ±â€Š1.3 weeks) were included in this study (birth weight 3345.6 ±â€Š450.6 g, body length 51.7 ±â€Š2.5 cm, head circumference 35.0 ±â€Š1.5 cm). 67 (33.5 %) neonates had minor external injuries of the scalp. 5 children showed clinical neurologic abnormalities: 4 (2.0 %) seizures and 1 facial palsy (0.5 %). In 34 (17.0 %) patients, minor incidental intracranial abnormalities unrelated to mode of delivery were detected on CUSS. No intracerebral, cranial pathologies or skull fractures were seen on routine CUSS. CONCLUSION: Routine CUSS in newborns after assisted vaginal delivery did not yield clinically relevant results in our cohort. Clinical observation and selective CUSS in symptomatic newborns might be more efficient than routine CUSS.

Soft tissue infection as a rare cause of neonatal sepsis.

Umbilical venous and peripherally inserted central venous catheters are often used in preterm infants, but complications include late-onset catheter-associated infections. Conversely, other sites of infection have to be taken into account in the case of clinical deterioration. In this Image in Science and Medicine paper, we report on a preterm infant with a rare cause of neonatal sepsis.

Distended abdomen due to a pseudocyst around a ventriculoperitoneal shunt.

Described herein is a case of distended abdomen in a 4-year-old boy with a ventriculoperitoneal (VP) shunt due to bilateral intraventricular hemorrhage following premature birth. Physical examination and laboratory tests revealed tenderness in the lower quadrants, with mild leukocytosis and normal C­reactive protein levels. X­ray demonstrated an intact VP shunt catheter but cranial displacement of the large intestine. Ultrasonography confirmed a large pseudocyst around the VP shunt, with extension from the symphysis to the sternum. The distal part of the VP shunt was surgically revised and 2.5 l of cerebrospinal fluid were evacuated. The boy made a full clinical recovery. Conventional X­rays, routinely used to confirm or exclude VP shunt continuity, may provide important clues regarding to the etiology of VP shunt dysfunction.

[Syncope in children and adolescents: are the current guidelines being followed?] / Synkopenabklärung bei Kindern und Jugendlichen ­ Handeln wir gemäß der aktuellen Leitlinie?

BACKGROUND: Syncope in childhood and adolescence is frequent and in most cases benign. A thorough history taking, complete physical examination, electrocardiography and further diagnostic work-up as indicated should rule out possible cardiac syncope. OBJECTIVE: To evaluate whether the diagnosis of syncope was performed according to the currently valid S2k guideline. MATERIAL AND METHODS: Retrospective study (January 2015-December 2017), University Children's Hospital of Saarland, Homburg, Germany. All patients aged 1-18 years presenting with the primary complaint of syncope were included. RESULTS: In this study 262 patients presented with a history of syncope (161 female (61.5%), 101 male (38.5%), median age 12.5 ± 3.9 years). Of these, 183 (69.8%) were reflex syncopes, 36 (13.7%) presyncopes, 35 (13.4%) undefined and 8 (3.1%) cardiac syncope. Out of 262 patients, 43 (16.4%) were diagnosed in accordance with the published guidelines and 13/43 (30.2%) correctly received further diagnostic work-up. In 219/262 patients (83.6%) basic diagnostic testing was not sufficient and 135/219 (61.6%) were submitted to further unnecessary diagnostic tests. CONCLUSION: Better adherence to the syncope guidelines bears the potential to avoid unnecessary and costly auxiliary medical tests while correctly diagnosing patients with syncope.

Birth weight, Apgar scores and gentamicin were associated with acute kidney injuries in VLBW neonates requiring treatment for patent ductus arteriosus.

AIM: We assessed the risk factors for transient acute kidney injury in very low birth weight (VLBW) infants treated for patent ductus arteriosus (PDA) using the serum creatinine-based criteria in Kidney Disease: Improving Global Outcomes. METHOD: This retrospective study of infants requiring ibuprofen and, or, surgery for haemodynamic relevant PDAs was performed at the University Children's Hospital of Saarland, Homburg, Germany, from January 2009 to December 2015. RESULTS: We studied 422 infants with a mean birth weight of 1059 ± 308.2 g. Acute kidney injuries developed in 150/295 infants (50.9%) with spontaneous PDA closure, in 46/82 (56.1%) who received intravenous ibuprofen treatment, in 18/24 (75.0%) who had surgery and in 15/21 infants (71.4%) who received both medical and surgical treatment. Acute kidney injuries were associated with birth weight and gestational age, Apgar scores at 10 minutes, the PDA size corrected for birth weight, a PDA with three affected circulatory territories, PDA surgery and gentamicin. Multiple logistic regression analysis showed particular associations between acute kidney injury and birth weight (p = 0.001), the 10-minute Apgar score (p = 0.02) and gentamicin (p = 0.043). CONCLUSION: Birth weight, the 10-minute Apgar score and gentamicin were particularly associated with acute kidney injuries in our cohort.

[Non-ambulatory patients with Duchenne muscular dystrophy : Recommendations for monitoring disease progression and course of treatment]. / Nichtgehfähige Patienten mit Duchenne-Muskeldystrophie : Empfehlungen zur Dokumentation des Krankheits- und Therapieverlaufs.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a severe X­linked recessive neuromuscular disorder. In children without corticosteroid therapy, progressive muscular weakness is associated with loss of ambulation on average by the age of 9.5 years. OBJECTIVE, MATERIAL AND METHODS: On the basis of current guidelines, a group of experts in this field defined a number of clinical parameters and examinations that should be performed on a regular basis to assess changes over time in non-ambulant patients. RESULTS AND CONCLUSION: To assess function of the upper extremities the Brooke upper extremity functional rating scale or the performance of upper limb test should be used. For assessment of pulmonary function measurement of forced vital capacity (FVC) is recommended. The extent of cardiac involvement can best be evaluated using cardiac magnetic resonance imaging (MRI), measurement of the ejection fraction (EF) and the left ventricular shortening fraction (LVSF) by echocardiography. The pediatric quality of life inventory should be used for assessment of quality of life. In addition, the body mass index (BMI), the number of infections and need for in-hospital treatment as well as early detection of orthopedic problems, most importantly the development of scoliosis should be monitored. After transition from pediatric to adult care DMD patients should be primarily cared for by adult neurologists and specialists in pulmonary and cardiac medicine.

[Parenteral nutrition in premature babies with a birth weight <1500g: a systematic single-center analysis and comparison with current guidelines]. / Parenterale Ernährung bei Frühgeborenen mit einem Geburtsgewicht <1500 g: eine systematische Zentrumsanalyse und Vergleich mit publizierten Leitlinien.

BACKGROUND: Parenteral and enteral nutrition are essential for both growth and development of preterm infants. Based on the results of many studies, the rate of nutritional growth and the amount of substrate delivered parenterally are under debate. OBJECTIVE: The main aim of this study was to assess parenteral nutrition in very and extremely immature preterm infants, i.e. very low birth weight (VLBW, birth weight <1500g) and extremely low birth weight (ELBW, birth weight <1000g) neonates, and to compare the amount of parenterally delivered substrate in our neonatal intensive care unit (NICU) to current German guidelines. METHODS: Retrospective audit at our tertiary NICU at the University Children's Hospital of Saarland, Homburg, Germany between 1 January 2009 and 31 December 2010. RESULTS: In total, 100 premature neonates were included. The mean gestational age was 29.6 weeks (range 24.4-34.1 weeks) and the mean birth weight was 1119 g ± 260 g (range 570 g-1490 g). Comparing the amount of fluids, glucose, amino acids, lipids and kcals with the current guidelines of the German Society for Nutritional Medicine in preterm infants, only glucose was adequately given; however, a substantial number of weight-dependent (more often in ELBW neonates) episodes of hyperglycemia requiring insulin treatment were also seen. During the first 3 weeks of life a substantial drop in body weight, length and head circumference occurred in our study cohort. In contrast, at 2 years corrected age, catch-up growth was seen in our cohort with anthropometric data now comparable to healthy term infants. Using the Bayley II test for developmental outcome assessment, at 2 years corrected age 78.6% (33/42) of infants demonstrated normal development. CONCLUSIONS: This retrospective data analysis demonstrated inadequate provision of parenteral nutrition in our NICU, which was often not in line with current German guidelines. This was associated with inadequate growth in our cohort, most notably during the first 3 weeks of life; however, implementation of current guidelines is impeded by metabolic disturbances in this cohort, most notably in ELBW neonates. Whether adherence to published guidelines will result in better early ex utero growth, and whether this normalized growth pattern will translate into better long-term outcome on a metabolic and neurological level, remains unclear.

Nucleated red blood cells and serum lactate values on days 2 and 5 are associated with mortality and morbidity in VLBW infants.

AIM: To correlate nucleated red blood cell counts and serum lactate concentrations on day 2 and 5 of life with morbidity and mortality in very low birth weight infants and to determine corresponding cutoff values. METHODS: Retrospective analysis in a cohort of very low birth weight infants. RESULTS: 250 very low birth weight infants were included in this study. Gestational age ranged from 23 to 35 weeks (mean 29.04) and birth weight was 320-1500 g (mean 1047.9). 55 (22%) patients developed intraventricular hemorrhage, 55 (22%) bronchopulmonary dysplasia, 12 (4.8%) periventricular leukomalacia, 93 (37.2%) retinopathy of prematurity, and 1 (0.4%) necrotizing enterocolitis. Mortality rate was 25/250 (10%). Nucleated red blood cells and serum lactate on day 2 of life were associated with mortality (p < 0.001). Serum lactate on day 5 of life demonstrated an association with retinopathy of prematurity (p = 0.017), bronchopulmonary dysplasia (p = 0.044), and intraventricular hemorrhage (p < 0.001). Cutoff values predicting mortality were >89.5 nucleated red blood cells/100 leucocytes (sensitivity 68.2%, specificity 89.0%) and serum lactate concentrations >8.5 mmol/l (sensitivity 69.6%, specificity 93.5%) on day 2 of life. CONCLUSION: We conclude that both nucleated red blood cell count and serum lactate concentration are valuable biomarkers in predicting important outcome parameters in very low birth weight infants.

Somatic Development in Children with Congenital Heart Defects.

OBJECTIVES: Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. STUDY DESIGN: Our study population consisted of all patients included in the PAN study (Prävalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. RESULTS: There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures-most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. CONCLUSION: Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

Diagnosis and Treatment of Tuberous Sclerosis Manifestations in Children: A Multicenter Study.

Tuberous sclerosis complex (TSC) is a genetic disease with a significant morbidity and mortality. We conducted a retrospective analysis of two cohorts (Vall d'Hebron University Hospital [HVH], Barcelona, Spain, 1982-2015, and at Saarland University Medical Center [UKS], Homburg, Germany, 1998-2015) to assess prevalence and treatment of TSC associated manifestations and to evaluate if the follow-up was in line with published recommendations. This was considered if more than 15% of patients did not receive adequate examination with regard to potential organ involvement. A definite diagnosis was made in 52 patients (96%), and a possible diagnosis was made in 2 patients (4%). Thirty-four (63%) patients were from HVH and 20 (37%) from UKS. Median age at first presentation was 6 months (interquartile range: 0-38 months), and median time of follow-up was 6 years (interquartile range: 2-13 years). Clinical symptoms that led to a diagnosis of TSC were cardiac rhabdomyoma (22/54), epilepsy (20/54), and cutaneous manifestations (4/54). Assessment of neuropsychiatric, renal, and ocular manifestations was inadequate in both hospitals, whereas cutaneous manifestation was inadequate at UKS only. Our data demonstrate insufficient examinations in a substantial number of TSC patients with regard to neuropsychiatric, renal, ocular, and cutaneous manifestations. The recently published guidelines may prove valuable in establishing a more comprehensive approach.