Kawasaki Disease Complicated with Cerebral Vasculitis and Severe Encephalitis.

We report a case of a 7-year-old boy with Kawasaki disease (KD) complicated with cerebral vasculitis and encephalitis. The patient was admitted with signs of encephalopathy, seizures, and coma. The diagnosis of KD was made on the 2nd day of hospitalization based on the clinical features (fever >5 days, maculopapular rash, nonpurulent conjunctivitis, fissured lips, and cervical adenopathy). Brain magnetic resonance imaging findings suggested cerebral vasculitis. Treatment with intravenous immunoglobulin was followed by mild improvement. After a single dose of immunoglobulin, pulse methylprednisolone therapy was started resulting in gradual improvement of consciousness and eventual complete motor and cognitive function recovery with regression of brain magnetic resonance lesions. KD can present with marked neurological symptomatology. Therefore, it should be considered in the differential diagnosis of encephalitis and encephalopathy etiologies in children.

Serum glutathione S-transferase Pi as predictor of the outcome and acute kidney injury in premature newborns.

BACKGROUND: The incidence of acute kidney injury (AKI) among the neonates treated at the Neonatal Intensive Care Unit is high with high mortality rates. Glutathione S-transferase (GST) class Pi plays an important role in the protection of cells from cytotoxic and oncogenic agents. The aim of the study was to examine whether the levels of serum glutathione S-transferase Pi (GST Pi) determined after birth have any predictive value for the outcome and development of AKI in premature neonates. METHODS: The prospective study included 36 premature neonates. The data about morbidity was gathered for all the neonates included in the study. The blood samples were taken in the first 6 h of life and GST Pi levels were measured. RESULTS: The mean values and standard deviations of GST Pi among the neonates who died and who survived were 1.904 ± 0.4535 vs 1.434 ± 0.444 ng/ml (p = 0.0128). Logistic regression revealed a statistically significant, positive correlation between GST Pi levels and death (p = 0.0180, OR7.5954; CI 1.4148-40.7748).The mean value of GST Pi levels in the neonates with AKI was higher than in neonates without AKI (p = 0.011). CONCLUSIONS: The conclusion of our study is that high levels of serum GST Pi in the first 6 h after birth are associated with an increased mortality and development of AKI in prematurely born neonates.

Risk factors associated with failure of extubation in very-low-birth-weight newborns.

AIM: To identify risk factors associated with the failure of extubation of mechanically ventilated very-low-birth-weight newborns. STUDY DESIGN: Prospective observational study. Assessment of the occurrence of extubation failure in relation to demographic and ventilation parameters, the SpO2/FiO2 ratio, the spontaneous breathing test (SBT) and values of the Silverman-Andersen score (SAS). Extubation failure was defined as the need for reintubation for any reason within 72 h after extubation. RESULTS: Extubation failed in 14/50 (28%) patients. Tidal volume applied at the moment of extubation (p = 0.030), the values of the SpO2/FiO2 ratio (p = 0.006), SBT (p = 0.034) and SAS measured for 60 min after extubation and later (p = 0.010, p = 0.000001, p∼0.000, respectively) showed a significant association with reintubation. CONCLUSIONS: Measured TV, SpO2/FiO2 ratio, SBT at the moment of extubation and values of SAS starting 1 h after extubation might be valuable parameters in identifying those VLBW newborns in the risk to fail extubation.

Etiology and Outcome of Severe Apparent Life-Threatening Events in Infants.

INTRODUCTION: An apparent life-threatening event (ALTE) is defined as "an episode that is frightening to the observer and is characterized by some combination of apnea, color change, marked change of muscle tone, choking, or gagging." OBJECTIVE: The aims of this study were to determine etiology and outcome of severe ALTE (requiring resuscitation measures) and to review diagnostic approaches in infants hospitalized after such an episode of ALTE. METHODS: Retrospective analysis included patients hospitalized at the Intensive Care Unit, Institute of Child and Youth Healthcare of Vojvodina, after an episode of severe ALTE over a 4-year period. RESULTS: The study included 23 infants, 18 male (78.3%), and 5 female (21.7%). The average age at presentation was 78 days (1 day to 11 months). In 8 infants (34.7%), ALTE resulted in death. The most frequent conditions after diagnostic evaluation were lower respiratory tract infections (39.1%), intracranial and extracranial hemorrhages (13.0%), and central nervous system infections (8.6%). The cause remained unknown in 8.7% of cases. Initial investigations included complete blood cell count, C-reactive protein or procalcitonin, blood gasses, lactate, electrolytes, glucose, blood culture, urinalysis, and chest x-ray. CONCLUSIONS: Apparent life-threatening event represents a diverse disorder. Lower respiratory tract infections and neurological disorders were the most common established etiology. Prematurity and congenital heart diseases stood out as important risk factors. Diagnostic evaluation varied according to suspected cause and trigger factors.

Peritoneal Dialysis in Very Low Birth Weight Neonates.

BACKGROUND: The aim of this retrospective study is to evaluate clinical characteristics and outcomes of very low birth weight (VLBW) neonates with acute kidney injury (AKI) treated with peritoneal dialysis (PD). METHODS: This retrospective study included 10 VLBW neonates treated with PD. Intravenous (IV) cannula and umbilical venous catheter were used for the peritoneal access. RESULTS: Mean age at the moment of starting PD was 14.9 ± 9.3 days. Mean body weight (BW) was 825 ± 215 g. The average gestational age was 26.3 ± 1.1 weeks. The average duration of dialysis was 20.5 ± 14.7 h. The average ultrafiltration was 7.7 ± 4.2 mL/kg/h. At the moment of starting PD, the average BW was 302 ± 317g (22 ± 13%), higher than at birth (in patients who had PD started in first 2 weeks of their lives) or higher than the BW before AKI was diagnosed (patients who had PD started when they were older than 2 weeks). The main cause of AKI was sepsis (n = 8/10). Dialysate leak was registered in 2 patients, 1 patient had peritonitis and the other had a blocked PD catheter. Six patients died during PD (severe sepsis), 1 died due to hypoxic encephalopathy and coma, and 2 patients survived. One patient (with hypoxic encephalopathy and coma) died 10 days after PD was stopped due to sepsis. The overall mortality was 80%. CONCLUSION: Acute PD is still an appropriate treatment choice for VLBW neonates with AKI. In VLBW neonates, PD can be performed with an improvised PD system and catheters.

Acute kidney injury in premature newborns-definition, etiology, and outcome.

BACKGROUND: Neonatal acute kidney injury (AKI) is common and is associated with poor outcomes. New criteria for the diagnosis of AKI were introduced based on the increase in serum creatinine (SCr) levels and/or reduction of urine output (UOP). Yet, there is no generally accepted opinion so far, which criteria (whether SCr, UOP, or their combination) are the most appropriate to diagnose neonatal AKI. METHODS: The retrospective study included 195 prematurely born neonates who fulfilled all inclusion criteria (with at least two SCr measurements). In all the neonates included in the study, AKI was diagnosed using three different definitions: (1) SCr criteria (an increase in SCr values of ≥0.3 mg/dl), (2) UOP criteria (UOP < 1.5 ml/kg/h), and (3) SCr + UOP criteria. RESULTS: Out of all of the patients the study included, 85 (44%) were diagnosed with AKI. The neonates who had AKI had a significantly lower gestational age, birth weight, and Apgar score, longer duration of mechanical ventilation, and a higher mortality rate. SCr + UOP criteria showed higher sensitivity for prediction of death compared to SCr or UOP alone (p = 0.0008, 95% CI 0.040-0.154, and p = 0.0038, 95% CI 0.024-0.125, respectively). If only SCr or only UOP criterion are used, they fail to identify AKI in 61 and 67%, respectively. AKI was an independent risk factor for death (OR 7.4875; CI 3.1887-17.5816). CONCLUSIONS: Similar to other studies, our data showed that neonates with AKI have worse outcome. Neonatal AKI defined based on SCr + UOP criteria is a better predictor of death than neonatal AKI defined based only on the SCr or UOP criteria. Also, by using SCr + UOP criteria for diagnosing neonatal AKI, more patients with AKI are recruited than when only one of those criteria is used.

Interleukin-17A Levels Increase in Serum of Children With Juvenile Idiopathic Arthritis.

OBJECTIVES: This study aims to determine the serum levels of interleukin-17A (IL-17A) in children with juvenile idiopathic arthritis (JIA) and analyze the correlation between IL-17A values and disease activity, certain clinical features, and laboratory markers of inflammation. PATIENTS AND METHODS: The study included 30 children (7 boys, 23 girls; mean age 8.8±5.3 years; range 1 to 18 years), who had been diagnosed with JIA (18 children were diagnosed during the study period and 12 children were diagnosed before the start of the study) and had active disease during the study period. Control group included 30 healthy, age- and sex- matched children (9 boys, 21 girls; mean age 8.3±4.8 years; range 1 to 18 years). The enzyme-linked immunosorbent assay was used to assess the serum IL-17A levels of children with JIA in the active phase of the disease and control group. Clinical and laboratory features of the disease were evaluated for the children with JIA. RESULTS: Serum levels of IL-17A in children with JIA were significantly higher in comparison to control group. In children with JIA who were prospectively monitored, statistically significantly decreased IL-17A level was recorded in the inactive phase of the disease. The incidence of arthritis of coxofemoral joints was significantly more common, and the mean levels of erythrocyte sedimentation rate and C-reactive protein were significantly higher in the group of children with JIA with detectable levels of IL-17A. Children with JIA and detectable levels of IL-17A had significantly higher values of Juvenile Arthritis Disease Activity Score-27 in comparison to children with JIA and non-detectable IL-17A. CONCLUSION: Assessment of serum IL-17A levels in early phases of JIA gives an opportunity for early detection of children that have higher risk for worse functional outcome.

Severe neonatal subgaleal hemorrhage as the first presentation of hemophilia A.

INTRODUCTION: Subgaleal hemorrhage is a rare but potentially fatal birth trauma. It is caused by rupture of the emissary veins (connections between the dural sinuses and scalp veins), followed by the accumulation of blood between the epicranial aponeurosis and the periosteum. Usually, it is associated with instrumental delivery (vacuum extraction, forceps delivery), but it may also occur spontaneously, suggesting the possibility of congenital bleeding disorder. CASE OUTLINE: A full term male neonate was born at 40 weeks gestation by spontaneous vaginal delivery, with birth weight of 3,700 g. The Apgar scores were 9 and 10 at 1 and 5 minutes, respectively. At the age of 23 hours, the baby became pale and lethargic. Large fluctuant swelling on his head was noted. He developed severe anemia and hypovolemia as a result of massive subgaleal hemorrhage. After successful treatment, the baby fully recovered. Follow-up and further evaluation revealed hemophilia A as a result of a de novo mutation. CONCLUSION: This case illustrates that subgaleal hemorrhage may be the first presentation of hemophilia A. Infants without obvious risk factors for developing subgaleal hemorrhage should be evaluated for congenital bleeding disorder. Successful outcome in affected infants requires early diagnosis, careful monitoring and prompt treatment.

Urinary kidney injury molecule-1 rapid test predicts acute kidney injury in extremely low-birth-weight neonates.

BACKGROUND: The new urinary and serum biomarkers are discovered and are being investigated. With them we can diagnose acute kidney injury (AKI) faster and more precisely and they also have a significant role in the outcome prediction. METHODS: The study included 22 extremely low-birth-weight neonates who were hospitalized in the neonatal intensive care units. They were divided into two groups based on serum creatinine (SCr) level-with and without AKI. Detection and quantification of urinary kidney injury molecule-1 (uKIM-1) was done on the third day of life, using commercially available KIM-1 rapid test. Subsequently, measurements were repeated only in subjects who were diagnosed with AKI, at different values of SCr. RESULTS: Logistic regression analysis showed that AKI is an independent risk factor for mortality. In a group of neonates with AKI, 50% of neonates administered the KIM-1 rapid test showed positive findings. KIM-1 rapid test was positive in patients with a wide range of SCr levels (range of 78.73-385 µmol/l), but all subjects had oliguria and died in the next 24 h. CONCLUSION: KIM-1 is a significant predictor of death. On the other hand, our study failed to prove that KIM-1 rapid test has any significance for early prediction of AKI.

Evaluation of Surfactant Replacement Therapy Effects--A New Potential Role of Lung Ultrasound.

INTRODUCTION: Previous studies suggested that effects of the surfactant administration in preterm intants with respiratory distress syndrome cannot be followed by lung ultrasound (L-US). OBJECTIVE: The aim of the paper is to evaluate the surfactant replacement therapy effects using a new, proposed grading system for L-US findings. METHODS: We report the series of 12 preterm infants with clinical and radiographic signs of respiratory distress syndrome, in whom L-US examinations were performed prior to, and within the first 24 hours after surfactant administration. To evaluate the surfactant replacement therapy effects, we proposed a new grading system (1 to 6) for L-US findings at each examined lung area, based on the presence of normal finding, the amount of B-lines and subpleural consolidations. RESULTS: All preterm infants had an improvement of L-US findings from one to four grades observed within the first 24 hours after surfactant administration, which has not been previously reported. The improvement of L-US findings was most commonly observed in anterior lung areas. CONCLUSION: L-US might enable an early detection of the surfactant replacement therapy effects. Further prospective studies are necessary to define the role of L-US in this field.

Acute kidney injury in preterm infants admitted to a neonatal intensive care unit.

BACKGROUND: The factors that contribute to the development of acute kidney injury (AKI) and treatment outcome among prematurely born neonates are not clearly understood. METHODS: This retrospective study included 150 prematurely born neonates. AKI was defined as an increase of serum creatinine levels ≥0.3 mg/dl compared to basal values. RESULTS: The majority of neonates with AKI (94.8 %) had a body weight <1,500 g. Logistic regression analysis showed that the Apgar score in the 5th minute <5, serum lactate levels >5 on the first day of life, core body temperature <36 ºC on the first day of life, occurrence of sepsis, intracranial hemorrhage, necrotizing enterocolitis, patent ductus arteriosus, as well as a treatment with vancomycin or dopamine were independent risk factors for the development of AKI. After the groups of neonates with and without AKI were adjusted, the calculated risk ratio for a negative outcome of treatment (death) was 2.215 (CI 1.27-3.86) for neonates with AKI. Neonates with AKI had higher serum sodium levels in the third and fourth days of life. CONCLUSIONS: AKI is associated with high mortality in preterm neonates. It is very important to identify, as quickly as possible, all infants who are at high risk of developing AKI.

Early biomarkers of renal injury and protective effect of erythropoietin on kidneys of asphyxiated newborn rats.

BACKGROUND: The aims of this study were to determine which of the two biomarkers of renal injury, kidney injury molecule-1 or cystatin C, is more sensitive and to evaluate whether erythropoietin protects kidneys injured by perinatal asphyxia. METHODS: Animals were split into three groups designated as follows: AE, pups that survived perinatal asphyxia and subsequently received 2.5 µg (0.1 ml) of darbepoetin-α (i.p.); A, the pups that survived perinatal asphyxia and received 0.1 ml of 0.9% NaCl; and C, control group. The pups were killed at different ages of life (6 h, 24 h, 48 h, 7 d, and 14 d of age; 10 rats in each subgroup). Immunohistopathological evaluation of kidneys was performed. RESULTS: At 48 h and on days 7 and 14, absolute injury scores were significantly lower in group AE as measured by both biomarkers. Cystatin C expression was the most intensive 6 h after the hypoxic event (average value of absolute injury score was 2.82) and declined over time. Expression of kidney injury molecule-1 was less intensive, with the average value of absolute injury score being 2.02 at 6 h and 2.105 at 24 h; the peak value (2.155) was recorded 48 h after the hypoxic event. CONCLUSION: Erythropoietin has a protective effect on hypoxic kidneys. Cystatin C is more sensitive as an early biomarker of acute kidney injury in comparison with kidney injury molecule-1.

Idiopathic arterial calcification of infancy-- peritoneal dialysis for treatment of hypertension.

Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disease usually diagnosed postmortem. The clinical presentation is not typical, but usually implies refractory hypertension and cardiorespiratory failure. We present a case of a newborn with IIAC who had fetal hydrops and refractory hypertension which normalized soon after initialization of peritoneal dialysis. With this case report, we wanted to highlight that peritoneal dialysis may be beneficial an effective therapeutic option for patients with IIAC and severe refractory hypertension. Until now, peritoneal dialysis was never performed in the treatment of patients with IIAC.

Neuroprotective effects of hypothermia and erythropoietin after perinatal asphyxia in newborn rats.

OBJECTIVE: Evaluation of neuroprotective effects of hypothermia, erythropoietin and their simultaneous use after perinatal asphyxia in newborn rats. METHOD: Hysterectomy was performed to Wistar female rats on the last day of gestation. Perinatal asphyxia was induced by submersion of uterus containing pups in saline for 15 min. After resuscitation, pups were randomized into 4 groups, 15 animals in each: G1 - asphyxia; G2 - asphyxia + hypothermia (rectal temperature 33 °C for 1 h); G3 - asphyxia + erythropoietin (Darbepoetin-α 2.5 µg, intraperitoneally) and G4 - asphyxia + erythropoietin + hypothermia. Pups were sacrificed on 7th day of life and histopathological analysis of hippocampus was performed. RESULTS: Measure of damage to dorsal, ventral and entire hippocampus was significantly lower in groups G2, G3 and G4 than in group G1 (p ~ 0.00; respectively). Measure of damage to hippocampus in group G4 was significantly lower than in group G2 (p = 0.029). CONCLUSIONS: This study demonstrates that simultaneous use of hypothermia and erythropoietin has more expressed neuroprotective effects than sole use of hypothermia after perinatal asphyxia in newborn rats.

Mild form of 3-methylglutaconic aciduria type IV and mutation in the TMEM70 genes.

The case study presents a 3-year-old boy diagnosed with a mild form of 3-methylglutaconic aciduria. During infancy and early childhood, he had lactic acidosis, dilated cardiomyopathy and failure to thrive with growth retardation. A genetic analysis revealed a mutated TMEM70 gene.

Infantile peripheral neuropathy, deafness, and proximal tubulopathy associated with a novel mutation of the RRM2B gene: case study.

Mitochondrial DNA depletion syndromes are a group of autosomal recessive hereditary disorders characterized by reduction of the amount of mitochondrial DNA in the affected tissue (muscle, liver, brain, or kidneys). We report a case of an infant with myopathy, deafness, peripheral neuropathy, nephrocalcinosis, proximal renal tubulopathy, moderate lactic acidosis, and a novel mutation of the RRM2B gene.

[Tests of screening criteria for retinopathy of prematurity].

INTRODUCTION: Retinopathy of prematurity is a disease of the eye, i.e. the retinal blood vessels, which occurs exclusively in premature infants. The level of blindness in one country depends on the level of development of neonatal care and the opportunities to implement screening. The aim of this study was to examine the possibilities of changing screening criteria, provided that not a single child was left out from the survey. MATERIAL AND METHODS: A two-year prospective study, which was carried out in the period from January 1st 2007 to December 31st 2008, included 191 premature infants who were treated at the Institute for Child and Youth Healthcare of Vojvodina. RESULTS: Different inclusion criteria regarding body mass and gestational age were applied for screening retinopathy of prematurity and we assessed the coverage of the sample if certain screening criteria were applied. According to the results of the research, when the applied screening criterion was 37/2000, there was not a single case of a blind, prematurely born baby. DISCUSSION: Great migrations of population as well as big differences in characteristics of premature infants together with underlying multi-factor diseases besides retinopathy of prematurity send a warning signal to be very cautious. CONCLUSION: Although this study has given ground to shift the limits of screening, we will adhere to broad screening criteria.

The influence of EPO and hypothermia on the kidneys of rats after perinatal asphyxia.

The aim of this study was to determine the effects of erythropoietin (EPO), moderate hypothermia, and a combination thereof on the kidneys of newborn rats damaged during perinatal asphyxia. An animal model of perinatal asphyxia (Wistar rats) was used in which after birth, newborn rats were divided into four groups of 15 animals each: G1, rats exposed only to asphyxia; G2, rats exposed to asphyxia and hypothermia (rectal temperature 32°C) and which received EPO (darbepoetin alpha) intraperitoneally; G3, rats exposed to asphyxia and hypothermia; G4, rats exposed to asphyxia and which received EPO. The rats were sacrificed on the 7th day of life and histopathological evaluation of kidneys was performed. Damage to the proximal tubules was significantly higher in group G1 rats than in groups G2, G3, and G4 rats (p < 0.01). Damage to the distal tubules was found only in group G1 rats. Histological changes in the proximal tubules were more prominent than in the distal tubules (p < 0.01). The immature glomeruli zone was less expressed in group G4 rats than in groups G1, G2, and G3 rats (p < 0.01). Based on these results, we conclude that EPO and hypothermia, as well as the combination thereof, have a protective effect on rats' kidneys damaged during perinatal asphyxia.