[The Brandenburg Network Healthy Children: Early Prevention Shows Effectiveness]. / Brandenburger Netzwerk Gesunde Kinder: Frühe Prävention zeigt Wirkung.

BACKGROUND AND AIM OF THE STUDY: The Brandenburg Network Healthy Children (NHC) is a regional health programme for families with toddlers (0-3) run by trained volunteers. Based on the analysis of School Entry Medical Examination (SEME), the study investigates the possible influence of NHC on children's health. METHOD: A retrospective epidemiological analysis of the sociodemografic and health-related differences among subgroups nc and n-nc based on SEME, school year 2016/2017 (network children/nc: 1,152, not network children/n-nc: 20,954), using descriptive statistics; a logistic regression analysis assessing, for instance, the power of NHC's influence on health adjusting also for social status and region. RESULTS: Parents with low/middle social status and one-parent families participated more frequently in the NHC (p<0.001). Nc compared to n-nc brought check-up documents (94.3 / 91.5%, p<0.001) and vaccination certificates (95.7 / 91.7%, p<0.001) more frequently to SEME. A higher tetanus-diphtheria-pertussis booster rate was observed after network participation. The adjusted model showed nc were less likely to have incomplete (U2-U6) check-ups (OR 0.347 [95%-KI: 0.192-0.627, p<0.001]), vaccination gaps (OR 0.621 [95%-KI: 0.508-0.758, p<0.001]) and more likely to be "optimally cared for" (OR 1.355 [95%-KI: 1.175-1.562, p<0.001]). CONCLUSION: Children's health showed benefit from network participation.

Short- and Long-Term Renal Outcome of Hemolytic-Uremic Syndrome in Childhood.

Introduction: Hemolytic-uremic syndrome (HUS) is a common cause for intrarenal acute kidney injury in childhood. More than 90% of HUS cases are associated with an infection by Shigatoxin-producing Escherichia coli (STEC) whereas the reminder comprises a heterogeneous group (here classified as Non-STEC-HUS). Renal impairment can persist in patients with HUS. This study presents data from four decades investigating the short- and long-term outcome of HUS in childhood. Materials and Methods: In a retrospective single-center-study clinical and laboratory data of the acute phase and of 1- to 10-year follow-up visits of children with HUS were analyzed. Results: 92 HUS-patients were identified from 1996 to 2014 (STEC-HUS-group: n = 76; Non-STEC-HUS-group: n = 16) and 220 HUS-patients between 1976 and 1995. STEC-HUS was increasingly caused by Non-O157 strains and mortality rate declined over the past decades (1.3 vs. 9.5%). Renal sequelae persisted more often in the group 1976-1995 (39.3%) than in the group 1996-2014 (28.3%), but more than 50% of all patients were lost to follow-up. Conclusion: Although renal outcome has improved over the investigated last decades, patients with HUS still face a high risk of permanent renal damage. These findings underline the importance of a consequent long-term follow-up in HUS-patients.

Clinical, Laboratory, and Placental Findings in Perinatal Listeriosis.

Clinical, laboratory, and placental manifestations of perinatal listeriosis are highly variable. Herein, we retrospectively analyzed all patients treated for neonatal listeriosis at the Charité University Medical Center in Berlin, Germany, 1999-2013. A total of 16 cases were identified. In 14 patients listeriosis was confirmed in neonatal specimens, while in two only the placenta tested positive. Elevated C-reactive protein and/or interleukin-6 levels were only inconsistently found, while a marked white blood cell left shift was present in all infants, if available. All but one infant manifested symptoms on the first day of life. Most patients required respiratory support, while none developed meningoencephalitis as evidenced by clinical or cerebrospinal fluid findings. Two patients died, all other patients survived without sequelae. In conclusion, perinatal listeriosis is still associated with significant morbidity and mortality. Clinical and laboratory findings are highly heterogeneous, but extreme leukocyte left shift seems to be a common feature.

Fetal intracardiac rhabdomyoma in beckwith-wiedemann syndrome.

Fetal cardiac tumors are a rare finding in prenatal ultrasonography. Most of them are rhabdomyoma, which are thought to be pathognomonic for tuberous sclerosis complex. We present an infant with prenatally diagnosed cardiac rhabdomyoma (CR), who was found to suffer from Beckwith-Wiedemann syndrome (BWS). This congenital overgrowth syndrome is characterized by macrosomia, macroglossia, omphalocele, hypoglycemia, and hemihypertrophy. BWS patients have an increased risk for formation of benign and malignant tumors, typically intra-abdominally located, but, to the best of our knowledge, fetal CRs have not been reported before. BWS must be added to the list of differential diagnoses and to the prenatal counseling of the parents in cases of prenatal detection of CR.

Laboratory markers of perinatal acidosis are poor predictors of neurodevelopmental impairment in very low birth weight infants.

BACKGROUND: In asphyxiated term and near-term infants, therapeutic hypothermia increases survival without neurologic morbidity, and extending this new treatment to preterm infants is being debated. AIMS: To investigate the association of low pH and base excess (BE) at birth or admission, as used as entry criteria in cooling trials, and evolving brain damage in preterm infants. STUDY DESIGN AND MEASUREMENTS: Rates of death and neurodevelopmental impairment at 12 and 20 months corrected age were assessed in a cohort of 1137 preterm infants with a gestational age <35 weeks and birth weight <1500 g in relation to severe perinatal acidosis (umbilical artery pH≤7.0, pH at admission ≤7.0, BE at admission ≤-16 mmol/l, lowest BE during first 12 h of life ≤-16 mmol/l). RESULTS: Umbilical artery pH was not linked to death or neurodevelopmental impairment. There was only weak predictive power of pH or BE at admission for death (positive predictive values [PPV] 0.36/0.30, receiver operator characteristics [ROC] areas 0.591/0.701), and lowest 12-h BE for death or neurodevelopmental impairment at 12 or 20 months (PPV 0.29/0.30/0.27, ROC 0.720/0.656/0.658). CONCLUSION: In very preterm infants, there is little association between laboratory markers of severe perinatal acidosis and neurodevelopmental outcome at 12 or 20 months.

Dysphonia at 12 months corrected age in very low-birth-weight-born children.

Preterm newborn infants may suffer laryngeal injuries after multiple intubations and long-term mechanical ventilation. Former studies have focused on acute laryngeal injuries diagnosed by endoscopy, performed within the neonatal period. This retrospective case-control study aims to investigate the prevalence and clinical risk factors for voice disorders in former very low-birth-weight (< 1,500 g) infants (VLBW) at 1-year follow-up examinations. We screened former VLBW infants for presence of dysphonia at the corrected age of 1 year and compared cases with unaffected infants matched by birth weight and gestational age. Of the 843 former VLBW infants, admitted from January 1998 to May 2006, 18 subjects had persistent dysphonia. All cases had a birth weight below 1,000 g. Surgical ligation of a ductus arteriosus had been performed in ten infants. Duration of ventilation and number of intubations were not different between cases and controls, but a documented difficult intubation was a predictor of subsequent dysphonia. The rate of dysphonia at 1 year of life was 6.6% among formerly ventilated infants with birth weights <1,000 g (extremely low-birth-weight infants). Persistent dysphonia has to be added to the list of specific long-term consequences of extremely immature birth and given attention at follow-up examinations.

Resolution of human immunodeficiency virus type 1 infection-related severe pulmonary hypertension in a very low-birth-weight infant.

Pulmonary arterial hypertension (PAH) affects approximately 0.5% of human immunodeficiency virus (HIV)-infected adults with poor prognosis. The effectiveness of highly active antiretroviral therapy for treatment of HIV-related PAH (HIV-PAH) remains controversial. Little is known about the incidence, clinical course, and therapy options for PAH in HIV-1-infected pediatric patients. Here, we report the case of a preterm infant with HIV-related life-threatening PAH, which resolved after initiation of highly active antiretroviral therapy.

Interleukin-1beta, interleukin-18, and interferon-gamma expression in the cerebrospinal fluid of premature infants with posthemorrhagic hydrocephalus--markers of white matter damage?

Posthemorrhagic hydrocephalus (PHHC) represents a major complication of preterm birth. The aim of this study was to determine whether cerebrospinal fluid (CSF) levels of the pro-inflammatory cytokines IL-1beta, IL-18, and interferon (IFN)-gamma are altered in the CSF of preterm infants with PHHC and may serve as a marker of white matter damage (WMD). Twenty-seven preterm infants with PHHC were included in the study; 13 of them had signs of cystic WMD (cWMD) on ultrasound examinations. CSF sample 1 was obtained at first ventriculostomy, sample 2 at shunt implantation. Results were compared with a control group of 20 age-matched patients without neurologic diseases. IL-1beta concentrations were elevated in CSF sample 1 of PHHC patients without WMD and in sample 1 of patients with cWMD. Concentrations of IL-18 were increased in both samples of patients without WMD and in sample 2 of patients with cWMD. CSF levels of IFN-gamma were elevated in sample 1 of PHHC patients with cWMD. The pro-inflammatory cytokine IL-1beta and IL-18 levels in the CSF are elevated in patients with PHHC. Higher IFN-gamma levels are detected in a subgroup of patients developing cWMD, indicating its involvement in the pathogenesis of cWMD in the context of PHHC.

Genetic deafness in a preterm infant with a critical postnatal course.

OBJECTIVE: We present a case of deafness in a preterm infant with several predisposing factors of an acquired hearing impairment that, however, turned out to have a genetic cause. We describe the severe postnatal course and review the relevant literature. DESIGN: Case report. SETTING: University-based tertiary neonatal intensive care unit. PATIENT: Preterm infant (gestational age, 26/37; wks). MEASUREMENTS AND MAIN RESULTS: A preterm infant exhibited hearing impairment after a complicated clinical course with pneumothoraces, a hemodynamically relevant patent ductus arteriosus, treatment with potentially ototoxic drugs, intraventricular hemorrhage, and periventricular leukomalacia. Despite the absence of a family history for deafness, genetic testing was performed. Surprisingly, genetic analysis revealed the presence of two compound heterozygous mutations in the patient's GJB2 gene as the cause for his early-onset nonsyndromic deafness. CONCLUSION: To elucidate the nature of a hearing disorder, it is worthwhile to consider a genetic cause, despite the fact that it may seem unlikely in a severely sick preterm infant with numerous risk factors for a postnatally acquired hearing impairment and without a positive family history.