Risk Factors for Clinical Seizures in Neonates with Hypoxic-ischemic Encephalopathy Treated with Therapeutic Hypothermia.

BACKGROUND: This study aimed to assess the risk factors for clinical seizures in newborns treated with whole body cooling (WBC) for hypoxic ischemic encephalopathy (HIE). METHODS: Infants with gestational age≥36 weeks and birth weight≥2.000 g who were treated with WBC due to HIE were retrospectively enrolled in this study. Patients were assigned to two groups: infants without clinical seizures (Group 1) and infants with clinical seizures (Group 2). The two groups were compared to determine the risk factors for the occurrence of clinical seizures. RESULTS: A total of 25 patients (Group 1=10 and Group 2=15) were included in the study. Prothrombin time (PT) was determined as independent risk factor for clinical seizures (p=0.046) and the odds ratio for the effect of PT was found as 1.475 (%95 CI:1.006-2.299). PT (area under the curve [AUC]=0.764; p=0.041), and increased cardiac troponin-I (cTnI) (AUC=0.935; p=0.002) were found to be significant risk factors for predicting the occurrence of clinical seizures. The optimal PT cut-off value was 22.7 sec, with a sensitivity and specificity of 45.4% and 90%, respectively; as well as positive and negative predictive value of 83.3% and 60.0%, respectively. The chest compression in the delivery room, severely abnormal amplitude integrated electroencephalography and high encephalopathy score were also found risk factors for occurrence of clinical seizures. CONCLUSION: Chest compression in the delivery room, high encephalopathy score, prolonged PT, and increased cTnI are significant factors for clinical seizures in newborns treated with WBC for HIE.

A Newborn with Infantile-Onset Pompe Disease Improving after Administration of Enzyme Replacement Therapy: Case Report.

Pompe disease (PD) is an autosomal recessive lysosomal storage disorder caused by a deficiency of acid α-1,4-glucosidase enzyme (GAA). PD has two forms, namely the infantile-onset and the late-onset form. In untreated cases, infantile-onset form usually leads to cardio-respiratory failure and death in the first year of life. Herein, we report a newborn with infantile-onset PD characterized by muscular hypotonia, respiratory distress, hypertrophic cardiomyopathy, hepatomegaly, elevated serum enzyme levels of aspartate aminotransferase of 117 IU/L (three times the normal value), alanine aminotransferase of 66 IU/L (1.8 times the normal value), lactate dehydrogenase of 558 IU/L (1.2 times the normal value), and creatine kinase >5,000 IU/L (16 times the normal value). Dried blood spot testing was performed and revealed decreased GAA enzymatic activity (0.07 nmol/mL/h, normal 0.93-7.33 nmol/mL/h). GAA gene analysis performed for confirming the diagnosis showed homozygous mutation c.896T >C (p.Leu299Pro). Initiation of enzyme replacement therapy (ERT) (ERT; 20 mg/kg, once every week) at 28 days of age resulted in weaning off from respiratory support within 1 week after treatment, normalization of cardiac abnormalities, and normal neuromotor development in the 16th month of age. Early diagnosis and early treatment with ERT, especially in the neonatal period, is of great importance to improve cardiac function and motor development in infantile-onset PD.

Carbapenem-Resistant Klebsiella pneumoniae Outbreak in a Neonatal Intensive Care Unit: Risk Factors for Mortality.

AIM: The aim of our study was to determine the factors associated with mortality in neonates with carbapenem-resistant Klebsiella pneumoniae (CRKP). MATERIAL AND METHODS: This retrospective, single-center study was conducted in the Neonatal Intensive Care Unit of Harran University Faculty of Medicine between January 2017 and July 2018 who had CRKP growth in their blood, urine or cerebrospinal fluid cultures. The discharged group was designated as the control group (Group 1), whereas the group that faced mortality was classified as the case group (Group 2). The demographic data, clinical findings and laboratory and microbiological results of the two groups were compared to identify risk factors. RESULTS: A total of 58 patients (36 in Group 1 and 22 in Group 2) exhibited CRKP growth during the study period. Low birth weight (p = 0.039), previous antifungal (p = 0.002) or amikacin use (p = 0.040), congenital anomalies (p = 0.002), total parenteral nutrition (TPN) administration (p = 0.002), surgery (p = 0.035), thrombocytopenia (p = 0.007), low platelet mass index (p = 0.011), elevated C-reactive protein (p = 0.004), high carbapenem minimum inhibitory concentration (MIC) (p = 0.029) and high amikacin MIC (p = 0.019) were associated with mortality. In a multivariate regression analysis, previous antifungal use (p = 0.028), congenital anomalies (p = 0.032) and TPN use (p = 0.013) were independent factors in predicting mortality. CONCLUSION: Previous antifungal use, congenital anomalies and TPN use were found to be independent risk factors for mortality in neonates with CRKP infection.

Effects of Single Loading Dose of Intravenous Caffeine on Cerebral Oxygenation in Preterm Infants.

OBJECTIVE: The aim of this study was to evaluate the effects of caffeine on cerebral oxygenation in preterm infants. STUDY DESIGN: This was a prospective study of infants with a gestational age (GA) of < 34 weeks who were treated intravenously with a loading dose of 20 mg/kg caffeine citrate within the first 48 hours of life. Regional cerebral oxygen saturation (rSO2C) and cerebral fractional tissue oxygen extraction (cFTOE) were measured using near-infrared spectroscopy before administering caffeine (baseline), immediately after administering caffeine, and 1, 2, 3, 4, 6, and 12 hours after dose completion; postdose values were compared with the baseline values. RESULTS: A total of 48 infants with a mean GA of 29.0 ± 1.9 weeks, birth weight of 1,286 ± 301 g, and postnatal age of 32.4 ± 11.3 hours were included in the study. rSO2C significantly decreased from 81.3 to 76.7% soon after administering caffeine, to 77.1% at 1 hour, and to 77.8% at 2 hours with recovery at 3 hours postdose. rSO2C was 80.2% at 12 hours postdose. cFTOE increased correspondingly. Although rSO2C values were lower and cFTOE values were higher compared with the baseline values at 3, 4, 6, and 12 hours after caffeine administration, this was not statistically significant. CONCLUSION: A loading dose of caffeine temporarily reduces cerebral oxygenation and increases cerebral tissue oxygen extraction in preterm infants. Most probably these changes reflect a physiological phenomenon without any clinical importance to the cerebral hemodynamics, as the reduction in cerebral oxygenation and increase in cerebral tissue oxygen extraction remain well within acceptable range.

Effects of Caffeine on Splanchnic Oxygenation in Preterm Infants.

OBJECTIVE: The aim of this study is to assess the effects of administering 20 mg/kg loading dose of caffeine citrate intravenously on splanchnic oxygenation in preterm infants. STUDY DESIGN: The infants with a gestational age (GA) of <34 weeks who were administered with a 20 mg/kg intravenous loading dose of caffeine citrate within 48 hours after birth were investigated prospectively. Regional splanchnic oxygen saturation (rsSO2) and splanchnic fractional tissue oxygen extraction rate (sFTOE) were measured using near-infrared spectroscopy before caffeine infusion, immediately after caffeine infusion and 1, 2, 3, 4, and 6 hours (h) after dose completion; postdose values were compared with predose values. RESULTS: A total of 41 infants with a mean GA of 29.2 ± 1.6 weeks and birth weight of 1,315 ± 257 g as well as postnatal age of 32.2 ± 10.8 hours were included in the study. rsSO2 significantly reduced from 63.1 to 57.5% immediately after caffeine infusion, 55.1% after 1 hour, and 55.2% after 2 hours with partial recovery at 3-hour postdose. sFTOE increased correspondingly. CONCLUSION: Caffeine reduces splanchnic oxygenation and increases splanchnic oxygen extraction for at least 2 hours with partial recovery to predose levels at 3-hour postdose.

Factors associated with early complications in inpatients who were treated in our clinic between 1992 and 2011 with a diagnosis of acute bacterial meningitis.

AIM: To evaluate factors associated with the development of early complications in acute bacterial meningitis. MATERIAL AND METHODS: In our study, 389 patients diagnosed with acute bacterial meningitis between January 1992 and January 2011 at Cerrahpasa Medical Faculty were retrospectively analyzed to determine the risk factors for the development of early complications. RESULTS: The causative agent was N. meningitidis in 17% of cases, S. pneumoniae in 13.6%, and H. influenzae type b in 6.4%. In 55.5% of cases, the causative agent could not be identified. The mortality rate was found as 1% and the early complication rate was 27.8%. The complications observed included septic shock and disseminated intravascular coagulation (33.3%), hydrocephalus (23.1%), subdural effusion (19.4%), and epilepsy (12%). Risk factors for early complications included being aged below two years (p<0.010), restlessness (p<0.010), rash (p<0.010), leukocytosis in complete blood count (p<0.010), and a cerebrospinal fluid glucose level of <45 mg/dL (p<0.010). Three of the four patients who died were male. The incidence of hydrocephalus was higher in patients who used ampicillin-cefotaxime and who did not receive steroid therapy before treatment (p<0.050). CONCLUSION: When acute bacterial meningitis is treated properly and adequately, recovery without sequela is possible. Knowing the risk factors for early complications will guide in the monitoring of patients and decrease morbidity and mortality rates.

Pseudohypoaldosteronism Type 1 Newborn Patient with a Novel Mutation in SCNN1B.

Pseudohypoaldosteronism is a rare disease characterized by resistance to aldosterone-targeted organs, hyponatremia, hyperkalemia, metabolic acidosis, and severe salt loss in hyperaldosteronism. Homozygous mutations in SCNN1A , SCNN1B, and SCNN1G genes were found to be responsible for the etiology. About 80 cases with molecular basis have been reported to date. In this case, a newborn patient admitted to our neonatal intensive care unit due to feeding problems was examined. The parents of the patient had a consanguineous marriage, and they had lost their three sons due to hyperkalemia. Since she had hyponatremia and hyperkalemia, congenital adrenal hyperplasia was primarily considered. Although the initial evaluation was made in this direction, the patient was diagnosed as pseudohypoaldosteronism type 1 with the findings obtained during the process such as dehydration, cortisol levels, adrenocorticotropic hormone levels, and negative CYP21A2 analysis result. This clinical diagnosis was confirmed by the novel homozygous frame-shift variant c.1245_1246insC (p.N416Qfs*35) in SCNN1B shown by gene analysis. In this report, we seek to emphasize that aldosterone deficiency should be one of the first diagnoses to be considered in neonatal patients with hyponatremia, hyperkalemia, metabolic acidosis, and dehydration.

Randomized trial of mask or prongs for nasal intermittent mandatory ventilation in term infants with transient tachypnea of the newborn.

BACKGROUND: The aim of this study was to compare nasal masks (NM) with binasal prongs (NP) for applying nasal intermittent mandatory ventilation (NIMV) by assessing the duration of respiratory distress, rate of intubation, and nasal trauma in term infants with transient tachypnea of the newborn (TTN). METHODS: Infants with a gestational age ≥37 weeks and birthweight ≥2,000 g who had NIMV administered for TTN were enrolled. We randomly allocated 80 neonates to the NM (n = 40) or NP (n = 40) group. Duration of respiratory distress was the primary outcome of this study. RESULTS: There were no statistically significant differences between the groups for the duration of tachypnea and NIMV (P = 0.94 and P = 0.13, respectively). No significant differences were observed between the two groups in terms of duration of oxygen supplementation and length of hospitalization (P = 0.72 and P = 0.70, respectively). The incidence of any grade of trauma and moderate trauma (grade II) was significantly higher in the NP group than in the NM group (P = 0.004 and P = 0.04, respectively). The rate of NIMV failure and other complications, including pneumothorax, pneumonia and feeding intolerance, was not significantly different in the groups. CONCLUSIONS: In term infants with TTN, delivering NIMV using NP in comparison to using NM appears to be similar with regard to the duration of respiratory distress and preventing intubation. However, the use of NP involves a greater risk of trauma than that of NM.

Platelet mass index and prediction of severity of transient tachypnea of the newborn.

BACKGROUND: Platelet mass index (PMI) is associated with platelet functionality. The aim of this study was to evaluate the role of PMI in predicting the severity of transient tachypnea of the newborn (TTN). METHODS: Infants with gestational age ≥37 weeks and birthweight ≥2,000 g who were given nasal intermittent mandatory ventilation for TTN ≤6 h after birth were retrospectively enrolled in this study. PMI was calculated using the following formula: PMI = platelet count × mean platelet volume/103 (fL/nL). The study infants (n = 101) were divided into two groups according to the duration of tachypnea: ≤48 h (n = 45) and >48 h (n = 56). RESULTS: The PMI and platelet count were significantly lower in the group with tachypnea duration >48 h than in the tachypnea duration ≤48 h group (P < 0.001 and P = 0.04, respectively). A negative significant correlation was noted between PMI and the duration of tachypnea (r = -0.43, P < 0.001). A PMI cut-off of 1,562 fL/nL can predict prolonged tachypnea (>48 h) with a sensitivity of 62.5%, specificity of 68.9%, positive predictive value of 71.4%, and negative predictive value of 59.6% (area under the curve, 0.682 ± 0.053; P = 0.002). CONCLUSIONS: Lower PMI and lower platelet count are associated with longer duration of tachypnea in patients with TTN.

Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate.

Development of hypertrophic pyloric stenosis (HPS) after a few weeks of repair of an oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) is a rare condition in early infancy. Although vomiting or feeding intolerance in operated cases of OA+TOF are attributed to oesophageal stricture, gastro-oesophageal reflux and oesophageal dysmotility, it may also be caused by HPS. Herein, we report a newborn infant who had OA and TOF operation on day 2 of life and diagnosed to have HPS at 15th day of age. Even though it is a rare anomaly, HPS should be kept on mind in the presence of persistent vomiting following repair of OA.

Spontaneous resolution of a 'ping-pong' fracture at birth.

'Ping-pong' fractures are depressed skull fractures in newborn infants that occur as inward buckling of the calvarial bones, forming a cup shape. These fractures are often associated with maintenance of bone continuity. These fractures may occur spontaneously during the intrauterine period or secondary to birth trauma. Currently, there is no standard protocol for the management of depressed skull fractures. Neurosurgical or non-surgical approaches may be administered depends on the severity of the fracture. Most untreated ping-pong fractures resolve spontaneously within 6 months. Therefore, it is recommended to reserve surgical elevation or non-surgical techniques for infants not demonstrating spontaneous resolution during this period. In addition, neurosurgical interventions are usually considered for cases with intracranial pathology or neurological deficits or for infants who do not respond to conservative treatment. Herein, we report a case of a newborn infant with a spontaneous intrauterine ping-pong fracture, which spontaneously resolved, without surgical or non-surgical intervention.

Efficacy and Safety of Intravenous Colistin in Very Low Birth Weight Preterm Infants.

BACKGROUND: Given the severity and high mortality of multidrug-resistant Gram-negative bacilli (MDR-GNB) infections, the use of colistin will increase in patients with MDR-GNB infection. OBJECTIVE: This study aims to assess the efficacy and safety of intravenous colistin in very low birth weight (VLBW; birth weight < 1500 g) preterm infants. METHODS: We retrospectively analyzed the medical records of patients who received colistin between June 2016 and December 2017. The patients were assigned to two groups: the VLBW group and the non-VLBW group. Both groups were evaluated for response to treatment and adverse effects. RESULTS: In total, 66 infants who received colistin therapy were included; of these, 28 infants were VLBW. All of our patients received standard colistin treatment of 5 mg/kg per day in three doses and the median duration of colistin treatment was 14 days. No significant differences were observed between the groups with respect to the efficacy of colistin (defined as showing microbiological clearance in control cultures and the absence of mortality during treatment) (89.3 vs 86.8%, p > 0.99). Serum magnesium and potassium levels were significantly lower in the VLBW group than in the non-VLBW group during colistin therapy (magnesium, 1.30 vs 1.70 mg/dL, p < 0.001; potassium, 3.6 vs 4.6 mEq/L, p < 0.001). Acute kidney injury was observed in four infants in the VLBW group and one in the non-VLBW group, without significant differences (p = 0.15). CONCLUSIONS: Colistin administration appears to be efficacious in VLBW infants; however, renal function tests and serum electrolytes should be monitored more closely in these infants during treatment.

Cardiac rhythm abnormalities during intravenous immunoglobulin G(IVIG) infusion in two newborn infants: coincidence or association?

We report the occurrence of supraventricular tachycardia during intravenous immunoglobulin (IVIG) infusion. Supraventricular tachycardia was observed in two newborn patients during IVIG infusion. Both of the babies responded to adenosine treatment. Cardiorespiratory monitoring during IVIG infusion can be recommended because of the possibility of this potentially lifethreatening adverse effect.

Ambulatory blood pressure and subclinical cardiovascular disease in patients with juvenile-onset systemic lupus erythematosus.

BACKGROUND: The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors, particularly hypertension in juvenile-onset systemic lupus erythematosus (SLE). METHODS: A total of 24 patients with normal renal function were examined for subclinical CVD by using non-invasive methods, including the measurement of carotid intima-media thickness (IMT), carotid distensibility, aortic pulse wave velocity (PWV) and left ventricular mass (LVM). Blood pressure (BP) pattern and the presence of hypertension were assessed by 24-h ambulatory blood pressure monitoring (ABPM). RESULTS: The patients had higher aortic PWV than the controls (p = 0.011). Increased carotid IMT was present in 48 % of the patients and reduced carotid distensibility in 17 %. Left ventricular hypertrophy (LVH) was present in 22 % of the patients. Eight patients were hypertensive; hypertensive patients had a significantly lower distensibility coefficient (DC)-SDS, higher aortic PWV and higher LVM index than the normotensive patients (p = 0.008, p = 0.023 and p = 0.001, respectively). Higher carotid IMT-standard deviation score (SDS) significantly correlated with higher nighttime diastolic BP-SDS (R (2) = 0.204, p = 0.046); lower DC-SDS correlated with higher nighttime systolic BP-SDS (R (2) = 0.290, p = 0.014). Increased aortic PWV was significantly associated with higher daytime systolic BP-SDS and elevated erythrocyte sedimentation rate (R (2) = 0.607, p = 0.003 and p = 0.010, respectively). PWV was the only independent predictor of LVM index (R (2) = 0.396, p = 0.004). CONCLUSION: These results provide additional evidence for the presence of subclinical CVD and its relation to hypertension in juvenile-onset SLE. They also indicate a significant relation between LVH and increased arterial stiffness. It is also important to note that our findings reveal significant relationships between ambulatory BP and cardiovascular changes and underline the importance of ABPM to predict CVD.

Macrocephaly-capillary malformation syndrome in a newborn with tetralogy of fallot and sagittal sinus thrombosis.

Macrocephaly-capillary malformation syndrome is characterized by cutaneous vascular malformations with associated anomalies as macrocephaly, macrosomia, hemihypertrophy, hypotonia, developmental delay, lax joints, loose skin, polysyndactyly, and neuroimaging abnormalities. We present a newborn with a prenatal diagnosis of macrosomia and tetralogy of Fallot. He also had macrocephaly; a high forehead; capillary hemangioma on the forehead, upper lip, and philtrum; generalized loose skin; postaxial polydactyly of both hands and feet, with neuroimaging findings of polymicrogyria and thrombosis in sagittal sinus and sinus rectus. His condition was diagnosed as macrocephaly-capillary malformation syndrome in the neonatal period and he died suddenly during sleep at 6 months of age. The clinical course in this syndrome is not as benign as was previously thought. Careful follow-up of these patients with particular emphasis on neuroradiologic and cardiologic evaluation might help decrease the risk of sudden death and to improve long-term outcome.