Canan Aygun

Findings of the International Nosocomial Infection Control Consortium (INICC), Part II: Impact of a multidimensional strategy to reduce ventilator-associated pneumonia in neonatal intensive care units in 10 developing countries.

Design. Before-after prospective surveillance study to assess the efficacy of the International Nosocomial Infection Control Consortium (INICC) multidimensional infection control program to reduce the rate of occurrence of ventilator-associated pneumonia (VAP). Setting. Neonatal intensive care units (NICUs) of INICC member hospitals from 15 cities in the following 10 developing countries: Argentina, Colombia, El Salvador, India, Mexico, Morocco, Peru, Philippines, Tunisia, and Turkey. Patients. NICU inpatients. Methods. VAP rates were determined during a first period of active surveillance without the implementation of the multidimensional approach (phase 1) to be then compared with VAP rates after implementation of the INICC multidimensional infection control program (phase 2), which included the following practices: a bundle of infection control interventions, education, outcome surveillance, process surveillance, feedback on VAP rates, and performance feedback on infection control practices. This study was conducted by infection control professionals who applied National Health Safety Network (NHSN) definitions for healthcare-associated infections and INICC surveillance methodology. Results. During phase 1, we recorded 3,153 mechanical ventilation (MV)-days, and during phase 2, after the implementation of the bundle of interventions, we recorded 15,981 MV-days. The VAP rate was 17.8 cases per 1,000 MV-days during phase 1 and 12.0 cases per 1,000 MV-days during phase 2 (relative risk, 0.67 [95% confidence interval, 0.50-0.91]; [Formula: see text]), indicating a 33% reduction in VAP rate. Conclusions. Our results demonstrate that an implementation of the INICC multidimensional infection control program was associated with a significant reduction in VAP rate in NICUs in developing countries.

Associations among genotype, clinical phenotype, and intracellular localization of trafficking proteins in ARC syndrome

Arthrogryposis–renal dysfunction–cholestasis (ARC) syndrome is a rare autosomal recessive multisystem disorder caused by mutations in vacuolar protein sorting 33 homologue B (VPS33B) and VPS33B interacting protein, apical–basolateral polarity regulator (VIPAR). Cardinal features of ARC include congenital joint contractures, renal tubular dysfunction, cholestasis, severe failure to thrive, ichthyosis, and a defect in platelet alpha‐granule biogenesis. Most patients with ARC do not survive past the first year of life. We report two patients presenting with a mild ARC phenotype, now 5.5 and 3.5 years old. Both patients were compound heterozygotes with the novel VPS33B donor splice‐site mutation c.1225+5G>C in common. Immunoblotting and complementary DNA analysis suggest expression of a shorter VPS33B transcript, and cell‐based assays show that c.1225+5G>C VPS33B mutant retains some ability to interact with VIPAR (and thus partial wild‐type function). This study provides the first evidence of genotype–phenotype correlation in ARC and suggests that VPS33B c.1225+5G>C mutation predicts a mild ARC phenotype. We have established an interactive online database for ARC (https://grenada.lumc.nl/LOVD2/ARC) comprising all known variants in VPS33B and VIPAR. Also included in the database are 15 novel pathogenic variants in VPS33B and five in VIPAR. Hum Mutat 33:1656–1664, 2012.

Severe Hypothyroidism Caused by Hepatic Hemangioendothelioma in an Infant of a Diabetic Mother

Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothyroidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L-thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected.

An unusual cause of acute renal failure in a newborn: hydrometrocolpos

Here we report on an unusual cause of acute renal failure in a newborn: hydrometrocolpos due to imperforate hymen. Hymenotomy resolved all of the laboratory and clinical abnormalities of the patient and the baby was sent home healthy.

International Nosocomial Infection Control Consortium (INICC) national report on device-associated infection rates in 19 cities of Turkey, data summary for 2003–2012

BackgroundDevice-associated healthcare-acquired infections (DA-HAI) pose a threat to patient safety, particularly in the intensive care unit (ICU). We report the results of the International Infection Control Consortium (INICC) study conducted in Turkey from August 2003 through October 2012.MethodsA DA-HAI surveillance study in 63 adult, paediatric ICUs and neonatal ICUs (NICUs) from 29 hospitals, in 19 cities using the methods and definitions of the U.S. NHSN and INICC methods.ResultsWe collected prospective data from 94,498 ICU patients for 647,316 bed days. Pooled DA-HAI rates for adult and paediatric ICUs were 11.1 central line-associated bloodstream infections (CLABSIs) per 1000 central line (CL)-days, 21.4 ventilator-associated pneumonias (VAPs) per 1000 mechanical ventilator (MV)-days and 7.5 catheter-associated urinary tract infections (CAUTIs) per 1000 urinary catheter-days. Pooled DA-HAI rates for NICUs were 30 CLABSIs per 1000 CL-days, and 15.8 VAPs per 1000 MV-days. Extra length of stay (LOS) in adult and paediatric ICUs was 19.4 for CLABSI, 8.7 for VAP and 10.1 for CAUTI. Extra LOS in NICUs was 13.1 for patients with CLABSI and 16.2 for patients with VAP. Extra crude mortality was 12% for CLABSI, 19.4% for VAP and 10.5% for CAUTI in ICUs, and 15.4% for CLABSI and 10.5% for VAP in NICUs. Pooled device use (DU) ratios for adult and paediatric ICUs were 0.54 for MV, 0.65 for CL and 0.88 for UC, and 0.12 for MV, and 0.09 for CL in NICUs. The CLABSI rate was 8.5 per 1,000 CL days in the Medical Surgical ICUs included in this study, which is higher than the INICC report rate of 4.9, and more than eight times higher than the NHSN rate of 0.9. Similarly, the VAP and CAUTI rates were higher compared with U.S. NHSN (22.3 vs. 1.1 for VAP; 7.9 vs. 1.2 for CAUTI) and with the INICC report (22.3 vs. 16.5 in VAP; 7.9 vs. 5.3 in CAUTI).ConclusionsDA-HAI rates and DU ratios in our ICUs were higher than those reported in the INICC global report and in the US NHSN report.

Is Vitamin D Deficiency a Risk Factor for Respiratory Distress Syndrome

BACKGROUND Previous studies have shown the relationship between in utero lung development and vitamin D [25(OH)D], but there have been no studies to investigate whether vitamin D deficiency is a risk factor for respiratory distress syndrome (RDS) in preterm babies. OBJECTIVES In this study, we investigated if 25(OH)D deficiency is a risk factor for RDS. METHODS One hundred fifty-two preterm newborns, born at 29 - 35 weeks gestational age, were included in the study following informed consent from the parents. Peripheral blood samples were collected within the first 24 hours of life and 25(OH)D levels were measured by liquid chromatography-tandem mass spectrometry. Demographic characteristics of the babies and the diagnosis of RDS were recorded. RESULTS In 64 % of preterm infants, 25(OH)D levels were compatible with severe deficiency (≤ 10 ng/mL), 33 % with moderate deficiency (10 - 20 ng/mL), and 3 % with mild deficiency (20 - 30 ng/mL). In none of the babies was a normal 25(OH)D level observed. Serum 25(OH)D levels were not correlated with gestational age. Respiratory distress syndrome was more common in preterm babies with severe (28 %) compared to mild-moderate 25(OH)D deficiency (14 %) (p < 0.05). CONCLUSIONS None of the preterm infants in this study had normal vitamin D level, which underlined the burden of vitamin D deficiency in pregnant women and their offspring. RDS was more common in severely vitamin D-deficient preterms. Determination of vitamin D status of the mothers and appropriate supplementation might be a valuable strategy to reduce RDS, in addition to antenatal steroids. Besides, since vitamin D is a regulatory factor in many organs during fetal development, long-term effects of in utero vitamin D deficiency warrant further studies.

Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism

Background: The utility of amplitude-integrated electroencephalography (aEEG) monitoring has been established for patients with neonatal hypoxic-ischemic encephalopathy. Objective: To evaluate the role of aEEG in the diagnostic process and treatment of patients with encephalopathy due to inborn errors of metabolism. Methods: Cases collected through an international registry were divided into 5 groups of metabolic disorders. Common aEEG features were sought for each group. Results: In total, 21/30 (70%) cases had abnormal aEEG background patterns, 18/30 (60%) showed seizure activity. Patients with disorders of energy metabolism, hyperammonemia, and organic/amino acidopathies often showed marked aEEG depression with seizure activity. In contrast, aEEGs of patients with peroxisomal disorders did not show major background abnormalities but seizures were present in 5/6 subjects. We report two features of interest: firstly, two tracings displayed an unusual upward shift of the lower aEEG amplitude margin. Secondly, aEEGs of infants with non-ketotic hyperglycinemia showed a pattern we refer to as ‘high-frequency burst-suppression pattern’. Conclusions: aEEG in patients with inborn errors of metabolism frequently reveals abnormalities and assists clinicians in the clinical assessment, management and monitoring of these patients.

Kidney Injury Molecule-1 as a Promising Biomarker for Acute Kidney Injury in Premature Babies

OBJECTIVE To evaluate the role of urinary kidney injury molecule-1 (uKIM-1) in early determination of renal injury in premature infants with respiratory distress syndrome (RDS). STUDY DESIGN Forty-eight premature babies hospitalized in the neonatal intensive care unit were included in the study and divided into three groups: group I, healthy premature infants; group II, preterm infants with RDS without acute kidney injury (AKI); group III, preterm infants with RDS and AKI. uKIM-1 and creatinine along with serum creatinine levels were measured with enzyme-linked immunosorbent assay on days 1, 3, and 7 of life. RESULTS On day 1, uKIM-1 levels in babies with RDS and AKI were higher than the other two groups. In this group, a significant increase in uKIM-1 levels were detected on day 3 (p = 0.015). The sensitivity and specificity of uKIM-1 were calculated as 73.3% and 76.9%, respectively, along with the increase of 0.5 ng per milligram of creatinine of uKIM-1 in day 3, when compared with values on day 1. Elevated uKIM-1 on day 7 was found to increase the risk of death by 7.3 times. CONCLUSION Serial uKIM-1 measurements can be used as a noninvasive indicator of kidney injury and uKIM-1 can be an ideal biomarker in premature infants.

Simplified gyral pattern with cerebellar hypoplasia in Sedaghatian type spondylometaphyseal dysplasia: A clinical report and review of the literature†

We report on a patient with Sedaghatian type spondylometaphyseal dysplasia (SSMD) who presented with metaphyseal dysplasia, congenital atrioventricular block, simplified gyral pattern, hypogenesis of corpus callosum, and severe cerebellar hypoplasia. We want to emphasize that in this rare congenital lethal skeletal dysplasia with unknown etiology, central nervous system malformations might be a major component of the disorder and should be evaluated in detail to possibly uncover the underlying pathophysiology.

Does early enteral feeding of very low birth weight infants increase the risk of necrotizing enterocolitis

Background/Objective:In this retrospective study, we intended to test whether early enteral feeding (EEF) of very low birth weight (VLBW) preterm babies increases the risk of necrotizing enterocolitis (NEC) or not.Subjects and Methods:Overall, 297 VLBW preterm babies admitted to the neonatal intensive care unit (NICU) between April 2003 and April 2006 were included. The study consisted of two periods: the first period was between April 2003 and October 2004, when babies were not fed enterally until they were extubated (167 preterm VLBWs). The second period was between November 2004 and April 2006, when babies were fed even when they were intubated, starting preferably on the first day of life (130 preterm VLBWs). Criteria for withholding enteral feeding in both periods were hypotension necessitating vasopressor agent use, abdominal distention, abdominal tenderness and suspected or proven NEC. Possible risk factors for NEC were also recorded.Results:The overall incidence of NEC in VLBW preterm babies was 6.7% and did not differ between the two study periods: 7.2% in the late and 6.2% in the EEF regimens. On logistic regression analysis, the most important risk factors associated with NEC were sepsis (P<0.001) and blood culture positivity (P<0.001). The average daily weight gain was significantly higher in the early fed babies (P=0.011).Conclusions:The EEF of VLBW preterm babies does not increase the risk of NEC. Increased daily weight gain is an important reason to feed these babies earlier.