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Infants developing necrotizing enterocolitis (NEC) have a different metabolomic profile compared to controls. The potential of specific metabolomics, i.e. amino acids and amino alcohols (AAA), as early diagnostic biomarkers for NEC is largely unexplored. In this multicenter prospective case-control study, longitudinally collected fecal samples from preterm infants (born <30 weeks of gestation) from 1-3 days before diagnosis of severe NEC (Bell's stage IIIA/IIIB), were analyzed by targeted high-performance liquid chromatography (HPLC). Control samples were collected from gestational and postnatal age-matched infants. Thirty-one NEC cases (15 NEC IIIA;16 NEC IIIB) with 1:1 matched controls were included. Preclinical samples of infants with NEC were characterized by five increased essential amino acids-isoleucine, leucine, methionine, phenylalanine and valine. Lysine and ethanolamine ratios were lower prior to NEC, compared to control samples. A multivariate model was rendered based on isoleucine, lysine, ethanolamine, tryptophan and ornithine, modestly discriminating cases from controls (AUC 0.67; p < 0.001). Targeted HPLC pointed to several specific AAA alterations in samples collected 1-3 days before NEC onset, compared to controls. Whether this reflects metabolic alterations and has a role in early biomarker development for NEC, has yet to be elucidated.

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OBJECTIVE: The objective of this study is to determine whether irradiance levels of phototherapy (PT) devices in Dutch neonatal intensive care units (NICUs) increased between 2008 and 2013. STUDY DESIGN: Irradiance of all types of PT devices, used in combination with incubators, was measured with a Dale 40 Radiometer (Fluke Biomedical, Everett, WA, USA) in all 10 Dutch NICUs. RESULTS: Irradiance increased in seven NICUs. Median (range) irradiance increased from 9.7 (4.3-32.6) to 16.4 (6.8-41) µW cm-2 nm-1 for 24 overhead devices (P=0.004) and from 6.8 (0.8-15.6) to 22.3 (1.1-36.3) µW cm-2 nm-1 for 12 underneath devices (P=0.014). Five light-emitting diode (LED)-based devices were used in 2013 and one in 2008. The mean distance between overhead PT device and infant decreased by ~9 cm (P<0.001). Significantly more devices delivered minimal (10 µW cm-2 nm-1) recommended irradiance levels (80 vs ~45%; P=0.002). CONCLUSION: Irradiance of PT devices still varies, but has markedly improved since 2008 due to shorter distances between PT device and infant, and introduction of better performing LED-based devices.

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BACKGROUND: Cytokines might be helpful to diagnose late onset sepsis (LOS) in newborn infants. Many studies on cytokines did not discriminate culture-proven from clinically-suspected sepsis; however, such differentiation is clinically useful. OBJECTIVES: To evaluate the feasibility to differentiate among culture-proven LOS, clinical LOS and controls using a battery of cytokines. STUDY DESIGN: This prospective study was conducted at the NICU of Harapan-Kita Women and Children's Hospital, Jakarta-Indonesia. Three groups of infants with postnatal age >72 hours of age were enrolled in the study: culture-proven sepsis group (PS) (n = 18), clinical sepsis group (CS) (n = 25) and control group (n = 34). A battery of 25 cytokines was measured in each infant five times: at enrollment, after 4 hrs, 12 hrs, 24 hrs, and 48 hrs using Invitrogen-immunoassays-Luminex™100. RESULTS: There were no significant differences in gestational age or mode of delivery among the three groups. IL-1ß, IL-2r, IL-6, IL-8, IL-10 and MIP-1a were significantly higher at all measurement points in group PS compared to controls. IL-13 was lower at all measurement moments in group CS compared to controls, IL-12 was lower and IP-10 higher between 0 and 24 hrs. IL-1Ra, IL-6, IL-8, IL-13, IL-15, TNFα, MIP-1a and MIP-1b were higher at all the measurement moments in group PS compared to group CS. The ROC curves show that IL-6, IL-8, IL-15, MIP-1a, MIP-1b and TNFα have a sensitivity and specificity between 80 and 85% during the first 24-48 hours after the onset of infection. IL-6, IL-15, MIP-1a, MIP-1b and TNFα showed the best likelihood ratios. CONCLUSIONS: IL-6, IL8, IL 15, MIP-1a, MIP-1b and TNFα are potentially good markers for detecting a proven LOS. In case these cytokines are not elevated in sick infants, other causes than an infection have to be identified.

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OBJECTIVE: To determine the differences in albumin levels between donors and recipients with twin anemia-polycythemia sequence (TAPS). METHODS: We included all consecutive monochorionic twins with TAPS with double survivors. Each twin pair was matched for gestational age at birth with 2 control monochorionic twin pairs unaffected by TAPS or twin-twin transfusion syndrome. We measured levels of albumin, total protein, and hemoglobin on the first day of life in donors and recipients (TAPS group) and the control group. RESULTS: A total of 25 TAPS twin pairs and 50 control twin pairs were included in the study. The median gestational age at birth was 32 weeks in both groups. In the TAPS group, median levels (IQR) of albumin in donor twins were significantly lower than in recipient twins, i.e. 28.0 g/l (24.0-32.0) versus 32.0 g/l (30.0-34.5) (p = 0.008). Median levels (IQR) of total protein in donor twins were also lower than in recipients, i.e. 44.0 g/l (36.5-49.0) versus 49.0 g/l (46.5-51.0), respectively (p = 0.004). The median (IQR) intertwin albumin difference was significantly higher in the TAPS group than in the control group, i.e. 4.0 g/l (2.5-10.5) versus 2.0 g/l (1.0-4.0) (p = 0.003). The rate of hypoalbuminemia (<20 g/l) and hypoproteinemia (<40 g/l) in donor twins with TAPS was 20% (5/25) and 32% (8/25). CONCLUSIONS: In addition to lower hemoglobin levels, donor twins with TAPS also have significantly lower albumin and total protein levels compared to recipient twins.

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OBJECTIVE: To estimate the differences in albumin levels between donors and recipients with twin-twin transfusion syndrome (TTTS). METHODS: We performed a matched case-control study including twin pairs with TTTS treated conservatively (conservative group) or with fetoscopic laser surgery (laser group) and analyzed the albumin levels at birth in donor and recipient twins. RESULTS: We included 18 twin pairs in the conservative group and 36 control twin pairs (laser group), matched for gestational age at birth. Median albumin levels in donor twins in the conservative group were significantly lower than in recipient twins, 25.0 versus 33.0 g/l, respectively (p = 0.001). In the laser group, albumin levels in donors and recipients were similar, 32.0 versus 32.0 g/l, respectively (p = 0.633). Hypoalbuminemia (albumin level <20 g/l) occurred in 22% (4/18) of donor twins in the conservative group. CONCLUSIONS: Hypoalbuminemia occurs frequently in donor twins with TTTS treated conservatively. In TTTS treated with laser, donor twins have similar and normal albumin levels compared to recipients, confirming a successfully performed fetoscopic laser procedure.

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Severe unconjugated hyperbilirubinemia, seen mainly in neonates, may cause kernicterus and death. Conventional treatment for severe unconjugated hyperbilirubinemia consists of phototherapy and exchange transfusion. Phototherapy, however, has several known disadvantages while exchange transfusion is associated with a significant morbidity, and even mortality. These harmful effects indicate the need to develop alternative pharmacological treatment strategies for unconjugated hyperbilirubinemia. Generally, these strategies aim to decrease the plasma concentration of unconjugated bilirubin (UCB) by inhibiting production, stimulating hepatic clearance, or interrupting the enterohepatic circulation of the pigment. To be considered for routine clinical use, an alternative treatment strategy should be less invasive and at least as effective and safe as phototherapy. Several pharmacological therapies such as metalloporhyrins, clofibrate, bile salts, laxatives and bilirubin oxidase may meet these criteria in the future, but none of them has yet been evaluated sufficiently to allow routine application. This review aims to discuss the state of the art and future perspectives in pharmacological treatment of neonatal jaundice.

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Unconjugated hyperbilirubinaemia occurs in almost all premature infants and is potentially neurotoxic. Treatment is based on total serum bilirubin (TSB), but treatment thresholds are not evidence based. Free bilirubin (Bf)-that is, not bound to albumin, seems a better parameter for bilirubin neurotoxicity, but measurements of Bf are not available in clinical practice. The bilirubin/albumin (B/A) ratio is considered a surrogate parameter for Bf and an interesting additional parameter in the management of hyperbilirubinaemia. This paper reviewed the evidence supporting the use of B/A ratios for predicting bilirubin-induced neurological dysfunction (BIND) including neurodevelopmental delay in jaundiced premature infants (gestational age less than 32 weeks). A literature search was performed and six publications reviewed regarding B/A ratios in the management and outcome of jaundiced premature infants. No prospective clinical trials had been undertaken to show whether bilirubin-induced neurotoxicity is reduced or whether unnecessary treatment is avoided by using the B/A ratio in addition to TSB. Recently, a randomised controlled trial evaluating the effect of the additional use of the B/A ratio on neurodevelopmental outcome in jaundiced premature infants has been initiated. Based on the prevailing evidence many authorities suggest that the additional use of the B/A ratio may be valuable when evaluating jaundiced premature infants.

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We describe a girl with a mosaic isodicentric chromosome 18q with discrete features of trisomy 18. She presented with prenatal growth retardation, prominent occiput, small face, high nasal bridge, large nose, thin lips, a perimembranous ventricular septal defect, and subsequent slow psychomotor development and slow growth. Amosaic isopseudodicentric chromosome 18q was detected in cultured lymphocytes: mos 46,XX,psu idic(18)(q23)[74]/ 46,XX[26]. Monosomy of the distal end of 18q23 could not be confirmed by fluorescent in situ hybridization (FISH) with RP 1l-565D23, one of the most telomere located probes of 18q23. Isopseudodicentric chromosome 18q is very rare. Most cases are mosaics. The phenotype varies. More or less distinct features of trisomy 18 and monosomy 18q can be found depending on the degree of mosaicism and the breakpoint in 18q.

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UNLABELLED: Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of skin followed by unilateral craniofacial atrophy of subcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH in one of two identical male twins reported here makes this possibility unlikely. PFH usually occurs in the first two decades of life, and the clinical presentation resembles linear scleroderma. PFH may be complicate by autoimmune, neurological, ocular and dental disorders. Management of PFH comprises a long term follow-up of somatic disorders, and prevention of psychological problems. Treatment of PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. CONCLUSION: The occurrence of PFH in one of monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow-up is essential to disclose the occurrence of complications.

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A stable isotope dilution method is described that allows measurement of cholic acid (CA) kinetics, that is, pool size, fractional turnover rate (FTR), and synthesis rate in mice, rats, and humans. Decay of administered [2,2,4,4-2H4]CA enrichment was measured in time in 50-microl plasma samples by gas-liquid chromatography/electron capture negative chemical ionization-mass spectrometry, applying the pentafluorobenzyl-trimethylsilyl derivative. The kinetic data expressed species-dependent differences. The CA pool sizes were 16.8 +/- 2.1, 10.6 +/- 1.2, and 2.4 +/- 0.7 micromol/100 g body weight for mice, rats, and humans, respectively. The FTR values were 0.44 +/- 0.03, 0.88 +/- 0.10, and 0.46 +/- 0.14 per day for mice, rats, and humans. The corresponding synthesis rates were 7.3 +/- 1.6, 9.3 +/- 0.1, and 1.0 +/- 0.2 micromol/100 g body weight per day. The human data agreed well with literature data obtained by conventional isotope dilution techniques. For rats and mice these are the first reported isotope dilution data. The method was validated by confirmation of isotopic equilibrium between biliary CA and plasma CA in the rat. Its applicability was demonstrated by the observation of increased CA FTR and CA synthesis rate in rats fed cholestyramine, which is known to increase fecal bile acid excretion. The presented stable isotope dilution method enables the determination of CA kinetic parameters in small plasma samples. The method can be applied in unanesthetized rodents with an intact enterohepatic circulation and may also be valuable when studying the development of human neonatal bile acid kinetics.

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We describe a patient with vertebral osteomyelitis and paravertebral soft-tissue collections associated with cat-scratch disease (CSD). Diagnosis was established on the basis of histologic examination and serological and polymerase chain reaction (PCR) tests. Treatment consisted of administration of antibiotics, and although skeletal lesions were persistently evident on radiography the patient showed complete clinical recovery. In addition, 15 cases of documented osteomyelitis associated with CSD are reviewed.

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OBJECTIVE: To differentiate between neonates with high and low risk of infections caused by group B beta-haemolytic streptococci (GBS), by using the urinary group B streptococcal antigen test. DESIGN: Retrospective. SETTING: Medical Centre Leeuwarden and Public Health Laboratory, Friesland, the Netherlands. METHODS: In a period of two years clinical, haematological and microbiological (including urinary group B streptococcal antigen detection) data were collected in newborns and their mothers who met one or more of the following criteria: a previous affected child, prolonged (> or = 12 hrs) rupture of membranes, fever in labour, unexpected preterm delivery, unexplained perinatal asphyxia. On the basis of surveillance cultures a colonization rate was made. GBS infection was 'suspected' in an unwell infant with a 'high' colonization rate; infection with GBS was 'proved' by a positive blood culture with GBS. RESULTS: 6 of 342 neonates had an infection with GBS. Risk of invasive infection increased with higher colonization rates. Sensitivity of the antigen test to detect colonization was low, sensitivity to detect neonatal infection was high (51 versus 100%). The negative predictive value of urinary antigen testing was 100%. Prolonged rupture of membranes (1.5% risk of infection) and maternal fever (5%) were the most important risk factors. CONCLUSION: In healthy neonates with risk factors but with a negative antigen detection test the risk of GBS infection is extremely low. In children with a risk factor a positive test result can indicate heavy colonization or infection. These children should be carefully observed and examined.

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Splenomegaly is a common problem. In the absence of systemic illness or malignancy splenic cysts must be considered, especially the epithelial variety. For large cysts total splenectomy has long been recommended. Recognition of the risk of an overwhelming postsplenectomy infection (OPSI), especially in children, has led to spleen conserving surgery. We describe the use of an absorbable Vicryl net after partial splenectomy with total cystectomy in the management of splenic epithelial cysts.

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Accidental ingestion of cigarettes (and butts) is mainly seen in young children. Nicotine in tobacco products is easily absorbed by the oral mucosa and intestines; absorption depends on nicotine content and pH of tobacco. Symptoms are caused by the nicotine component and usually develop rapidly (< 4 hours). The most common symptom is vomiting. Although cigarettes are potentially toxic, their ingestion by children is generally benign. Decontamination of the mouth with water may be useful. Induction of emesis is not advised. Gastric lavage is not needed in asymptomatic patients (with an unreliable history) or after vomiting. Children who ingested cigarettes should receive medical observation for four hours after ingestion. Children with significant symptoms should be admitted and eventually treated by supportive care. In symptomatic children or children with a reliable history of ingestion of large quantities who have not vomited gastric lavage with administration of activated charcoal has to be performed. When after vomiting other symptoms persist activated charcoal can be given via a nasogastric tube.

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Progressive partial lipodystrophy (PPL) was diagnosed in two girls aged 6 and 8 years. PPL is characterized by loss of subcutaneous fat, starting in the face and progressing to trunk and arms. Diagnosis is based upon the cachectic appearance and the normal growth parameters. It is a rare disease of unknown aetiology, usually beginning in childhood and more frequent in females. An association with diabetes mellitus, hypertriglyceridaemia and glomerulonephritis has been described. Follow-up should be focused on these and on psychological effects. No causal therapy is available. The facial appearance can be restored by injection of liquid silicones. Life expectancy does not appear to be affected.

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