Nilay Hakan

Acute peritoneal dialysis in the newborn period: a 7-year single-center experience at tertiary neonatal intensive care unit in Turkey.

OBJECTIVE To evaluate the underlying causes and outcomes of neonates who underwent acute peritoneal dialysis (APD). STUDY DESIGN This report describes a 7-year experience with APD in 77 neonates. RESULTS Underlying causes requiring APD were acute tubular necrosis (ATN; n = 53), inborn error of metabolism (n = 18), bilateral renal vein thrombosis (n = 3), obstructive uropathy (n = 2; posterior urethral valve and neurogenic bladder), and bilateral renal artery thrombosis (n = 1). Fifteen of the 53 patients developed post-cardiac surgery ATN. The mean dialysis duration was 6.2 ± 10.7 days (range 1 to 90 days). Complications of procedure were hyperglycemia (n = 35), leaking of dialysate (n = 13), peritonitis (n = 10), catheter obstruction (n = 3), bleeding when inserting the catheter (n = 3), exit site infection (n = 2), and bowel perforation (n = 1). There were 57 deaths (74%) in this high-risk group due to underling causes. Of the 20 survivors, 16 patients showed a full renal recovery, but mild chronic renal failure developed in 1 patient and proteinuria with/without hypertension in 3 patients. CONCLUSION Peritoneal dialysis is an effective means of renal replacement therapy in the neonatal period in the management of metabolic disturbances as well as renal failure. Although major complications of procedure are not so common, these patients have high mortality rates due to the serious nature of the primary causes.

Exchange transfusion for neonatal hyperbilirubinemia: an 8-year single center experience at a tertiary neonatal intensive care unit in Turkey

Abstract Objective: The aim of present study was to evaluate the indications and the complications associated with neonatal exchange transfusion (ET) performed for hyperbilirubinemia. Methods: This study included overall 306 neonates who underwent ET between 2005 and 2012. The demographic characteristics of patients, causes of jaundice and adverse events occurred during or within 1 week after ET were recorded from their medical files. Those newborns that underwent ET were classified as either “otherwise healthy” or “sick” group. Results: Of the 306 patients who underwent ET, 244 were otherwise healthy and had no medical problems other than jaundice. The remaining 62 patients were classified as sick that had medical problems other than jaundice ranging from mild to severe. The mean gestational age was 37.6 ± 2.5 weeks and the mean peak total bilirubin levels was 25.8 ± 6.6 mg/dl. The mean age at presentation was 5.4 ± 3.8 d for all infants. The most common cause of hyperbilirubinemia was ABO isoimmunization (27.8%). None of newborns died secondary to ET. Three infants had had necrotizing enterocolitis, and also three infants had had acute renal failure. The most common encountered complications of ET procedure were hyperglycemia (56.5%), hypocalcaemia (22.5%) and thrombocytopenia (16%). Conclusions: Our data showed that ABO isoimmunization was the most common cause of hyperbilirubinemia. Even mortality was not seen, very rare but major gastrointestinal and renal complications were associated with ET. The majority of adverse events associated with ET were laboratory abnormalities mainly hyperglycemia, hypocalcaemia and thrombocytopenia which were asymptomatic and treatable.

Novel CRLF1 gene mutation in a newborn infant diagnosed with Crisponi syndrome

Crisponi syndrome is an infrequently described disorder with autosomal recessive trait. It is characterized by extensive muscular contractions in the face after even minimal stimuli or crying, hypertonia, opisthotonus, camptodactyly, and typical facial features. Muscle contractions attenuate during rest or when the infant calms down. As a recently described new disease, Crisponi syndrome may be confused with epileptic manifestations. Most of the patients die in the first months of life due to hyperthermia and feeding problems. Recently, it has been demonstrated that mutations of the CRLF1 gene ‘cytokine receptor‐like factor 1’ are associated with Crisponi syndrome. Here, we present a newborn diagnosed with Crisponi syndrome and report a novel homozygous CFRL1 gene mutation.

Ketamine is a neurotoxic agent that could adversely affect the brains of preterm babies

level of hypoxic-ischaemic encephalopathy, percentage of infants with moderate or severe HIE, as well as on fluid and electrolyte intakes and urinary output. Also, daily serial measurements of creatinine levels were significant lower in post-TH when compared to pre-TH infants. Post-TH infants had significantly lower levels of sodium at < 48h of life, and the fractional excretion of sodium (FeNa) is effective in distinguish between pre-renal from renal failure in infants with >48 h of age (3). Therefore, it is unlikely that the higher incidence of mild hyponatraemia after cooling implementation was related to a more severe degree of renal dysfunction in the post-TH group.

Lidocaine should only be used in neonatal seizures that do not respond to first‐generation AEDs

Sir, We read with great interest the article by Lundqvist et al. (1), which evaluated the efficacy and safety of lidocaine for treating neonatal seizures in infants (gestational age ≥37 week, age ≤28 days) following benzodiazepines, but not preceding phenobarbital treatment. The authors reported that the treatment stopped seizures in 16 of the 30 infants studied. Suspected adverse effects were only seen in one patient, who developed a transient bradycardia. Indeed, several studies indicate that lidocaine is an effective agent for intractable neonatal seizures that do not respond to firstgeneration antiepileptic drugs (AEDs). However, data on the use of lidocaine are limited and it is used off-label in neonates with refractory seizures (2). Phenobarbital, a firstgeneration AED, is still widely used for the treatment of neonatal seizures. It is still preferable because of its efficacy and tolerability. In addition, it is a cost-effective pharmacologic treatment for lowand middle-income countries. There are some disadvantages of lidocaine in clinical practice. For example, the distribution volume and unbound fraction of lidocaine is greater in neonates than in adults who have a low concentration of plasma proteins. In addition, lower glomerular filtration rates reduce renal clearance of lidocaine and its active metabolites, thereby increasing the risk of toxicity in neonates when used for prolonged periods. There is no consensus regarding the dose of lidocaine infusion. Its higher plasma concentrations may cause seizure activity (‘proconvulsant’ effect), and one study highlighted the risk of developing cardiac arrhythmias (bradycardia, tachycardia, prolonged QRS complex and/or irregular heart) (3). For this reason, continuous cardiac and serum monitoring of neonates is indicated. Furthermore, neurotoxic effects of lidocaine in newborns are also reported. Therefore, we think that lidocaine should only be used as an alternative treatment in infants who are not responding to first-generation AEDs. There is an urgent need for prospective, randomised, controlled trials to assess the pharmacokinetic, efficacy and safety of lidocaine in neonates.

Hemolytic disease of the newborn caused by irregular blood subgroup (Kell, C, c, E, and e) incompatibilities: report of 106 cases at a tertiary-care centre.

OBJECTIVE To determine the clinical spectrum of hemolytic disease due to irregular blood subgroup incompatibility in hospitalized neonates. STUDY DESIGN The medical records of the all hospitalized newborn patients diagnosed with indirect hyperbilirubinemia due to subgroup incompatibility in Kell, C, c, E, and e systems were included in the study. Data from 106 newborns with hemolytic jaundice due to irregular blood subgroups were retrospectively evaluated, and clinical and laboratory findings were compared between patients . The treatment modalities given to the patients of each subgroup types and the laboratory findings and treatment modalities of the cases according to Coombs tests results were also analyzed. Fetal affection of the hemolysis and also fetal losses due to irregular red-cell alloimmunization were not detected in prenatal course, as there was no follow-up of these pregnancies. RESULTS The mean postnatal hospitalizing age was 6.1 ± 5.2 days after birth. The mean total bilirubin level and the mean hemoglobin value on hospitalization were 343.7 ± 63.3 µmol/L (=20.1 ± 3.7 mg/dL) and 14.9 ± 3.4 g/dL, respectively. Of 106 patients identified with irregular subgroup incompatibility, 40 infants (37.7%) were associated with C, 22 (20.8%) with c, 30 (28.3%) with E, 9 (8.5%) with e, and 5 (4.7%) with Kell subgroup system. Positive Coombs tests (either direct and/or indirect) occurred in 28.3% of the study cases. Hydrops fetalis was determined in 5 of 106 neonates (4.7%). Twenty-two of 106 (20.8%) patients required total exchange transfusion. Positive Coombs test in cases required total exchange transfusion was 63.6%. CONCLUSION Our data expose the magnitude and spectrum of the potential developing severe hemolytic disease and immune hydrops due to irregular subgroup incompatibility. Minor group antibody screening is recommended both in the mother and the high-risk infants with hyperbilirubinemia and hemolytic disease of the newborn.

Alendronate for the treatment of hypercalcaemia due to neonatal subcutaneous fat necrosis

Dear Editor, We read with great interest the article by Mitra et al. [3] who described subcutaneous fat necrosis (SCFN) in a preterm infant after severe perinatal hypoxic injury complicated by hypercalcaemia. Mitra et al. [3] state that significant SCFN can develop in both preterm and term infants and preterm infants also develop significant complications including hypercalcaemia. Most patients can be treated successfully by hyperhydration, furosemide, corticosteroids, and also by citrate, calcitonin, and bisphosphonates [4, 5]. Among the bisphosphonates, both etidronate [4] and pamidronate [2] have been used in the treatment of hypercalcaemia due to SCFN of the newborn. However, the use of alendronate for this purpose has not been reported yet, as far as we know. Alendronate as a bisphosphonate is a potent inhibitor of osteoclast-mediated bone resorption. Previously, it has been used safely in patients with hypercalcaemia associated with vitamin D intoxication [1]. Recently, we successfully treated with alendronate sodium a patient with hypercalcaemia associated with SCFN after a severe perinatal asphyxic event. In our patient, the indurated erythematous plaques and nodules appeared on the back, upper arm and gluteal areas by postnatal day 7 (Fig. 1). When the lesions had almost disappeared on postnatal day 30, serum calcium level still reached a peak of 11.5 mg/dL with a urine calcium/urine creatinine ratio of 0.74 mg/mg. Laboratory testing showed serum parathyroid hormone level of 7.5 pg/mL (normal range, 15–65 pg/mL) and serum 25-hydroxyvitamin D level of 38.5 ng/mL (normal range, 10–40 ng/mL). Because of the clear clinical findings, a histopathological examination was not required. Renal ultrasonography showed bilaterally punctiform renal medullary hyperechogenicities. Despite adequate hydration, the patient’s serum calcium level remained high. Because the degree of hypercalcaemia was mild and there was nephrocalcinosis, furosemide treatment was not chosen in our patient. Moreover, corticosteroids were not considered because of their potential side effects. Hence, the bisphosphonate treatment was intended and then, because availability of oral preparation, the patient was given oral alendronate at a dosage of 5 mg/day. Oral alendronate treatment was discontinued on postnatal day 50 when the serum calcium level and urinary calcium/ creatinine ratio had returned to normal. During the alendronate therapy, no side effects were observed. In conclusion, patients with SCFN should be closely monitored for developing metabolic problems like hypercalcaemia. Moreover, alendronate can be used safely and effectively in the treatment of hypercalcaemia associated with SCFN. N. Hakan :M. Aydin (*) :A. Zenciroglu :N. Demirel : N. Okumus :M. S. Ipek Department of Neonatology, Dr. Sami Ulus Maternity and Children’s Hospital, Babur Street, no: 44 (06080) Altindag, Ankara, Turkey e-mail: dr1mustafa@hotmail.com

Is phenobarbital a neuroprotective agent in newborn infants with perinatal asphyxia

Their study included 67 term newborns with perinatal asphyxia who were randomized into three groups: supportive treatment; high-dose phenobarbital; or erythropoietin. In their study, longterm neurologic follow up results were better in the phenobarbital group than the erythropoietin group with respect to motor and cognitive functions at 3 and 6 months, and expressive language skills. At 18 months of age, however, the differences between these two groups were insignificant. Therefore, they suggested that high-dose phenobarbital or erythropoietin along with supportive treatment has a positive influence on the outcome of newborns with perinatal asphyxia. 1 There are some controversies, however, about the safety of phenobarbitalevenattheactualdosageusedforneonatalseizures, which is an initial loading dose of 15‐20 mg/kg followed by a maintenance dose of 3‐4 mg/kg per day. 2 It has significant side

Neonatal hypoglycemia associated with the antenatal corticosteroids may be secondary to fetal adrenal suppression

Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded.

Distribution of congenital anomalies in a neonatal intensive care unit in Turkey.

Abstract Background: Congenital anomalies are one of the important reasons of mortality and morbidity in newborns. The aim of this study is to determine the incidence, distribution and the mortality of the congenital anomalies in a single neonatal intensive care unit (NICU) from Turkey. Method: A retrospective analysis was performed between 2005 and 2012 in NICU using a computerized database. Variables including the type of anomaly, antenatal and postnatal history, gestational age, birth weight, consanguinity and other demographic, clinical and related laboratory variables were extracted from the computerized database using ICD-10 codes. Congenital anomalies were classified according to involved organ systems and also classified as single and multiple anomalies. Results: A total of 1024 newborns with congenital anomaly (CA) (13.7%) were identified among the 7450 hospitalized newborns in NICU. The most affected system was the cardiovascular system (68.8%). Most of the anomalies (67.1%) were single anomalies. Of all, 59.4% had single major, 7.7% had single minor, 9% had single major plus single minor, 18.4% had multiple major and 2% had multiple minor anomalies. On the other hand, 96.3, 1.9, 0.1 and 1.7% of the newborns had malformation, deformation, disruption and dysplasia, respectively. Chromosomal analysis was only performed 24.8% of the newborns with CA and among them, 65.3% of these were in normal limits. The most frequently detected chromosomal abnormality was trisomy 21. Overall, mortality rate was 15.5% among the newborns with CA. Conclusion: In conclusion, the most common and mortal CA was cardio-vascular malformations in our hospital. The overall prevalence of cardio-vascular malformations among the newborn was higher than previously reported studies in Turkey. Further, studies with larger sample size are needed to determine CA in Turkey.