Efficacy and safety of rectal ibuprofen for patent ductus arteriosus closure in very low birth weight preterm infants.

BACKGROUND: To compare rectal ibuprofen with oral ibuprofen for the closure of hemodynamically significant patent ductus arteriosus (hsPDA) in very low birth weight (VLBW) preterm infants. STUDY DESIGN AND SUBJECTS: In a prospective, randomized study, 72 VLBW infants who had hsPDA received either rectal or oral ibuprofen. The plasma concentration of ibuprofen and renal functions were determined in both groups by the high-performance liquid chromatography (HPLC) method and cystatin-C (cys-C), respectively. RESULTS: The hsPDA closure rate of the group that received rectal ibuprofen was similar to oral ibuprofen (86.1% versus 83.3%) after the first course of the treatment (p = 0.745). A statistically significant difference was identified between the mean plasma cys-C levels before and after treatment in both the rectal and oral ibuprofen groups (p = 0.004 and p< 0.001, respectively). The mean plasma ibuprofen concentration was similar in both groups after the first dose (rectal 44.06 ± 12.4; oral, 48.28 ± 22.8) and the third dose (rectal, 45.34 ± 24.3; oral, 48.94 ± 24.8) (p > 0.05 for all values). CONCLUSIONS: Rectal ibuprofen is as effective as oral ibuprofen for hsPDA closure in VLBW infants. The rise in the cys-C level with rectal and oral treatment shows that patients with borderline renal function should be evaluated and followed closely.

Powder Topical Rifampin for Reducing Infections After Neural Tube Defect Surgery in Infants.

OBJECTIVE: The correct timing and technique of neural tube defect (NTD) repairs significantly decrease the morbidity and mortality of NTD cases. However, infections related to the surgery are still common. We investigated the effects of topical rifampin combined with routine prophylaxis in newborns with open NTDs. METHODS: This retrospective study included 86 patients who had undergone NTD surgery. The experimental group comprised 30 patients who were started on topical rifampin before surgery, and the control group comprised 56 patients who were not administered topical rifampin. Surgical site infections (SSIs) and meningitis/ventriculoperitoneal (VP) shunt infections that developed within 6 months after the surgical intervention were evaluated. RESULTS: In the postoperative period, meningitis/VP shunt infections and SSIs were observed in 6.7% and 3.3%, respectively, of the experimental group treated with topical rifampin. Meningitis/VP shunt infections and SSIs were observed in 37.5% and 21.4%, respectively, of the control group. External ventricular drainage and not using topical rifampin were identified as important relative risk (RR) factors for meningitis/VP shunt infections (RR 19.28, 95% confidence interval [CI] [3.53, 105.33], P = 0.001; RR 18.10, 95% CI [2.38, 137.68], P = 0.005). A flap transposition, cerebrospinal fluid leaks, and not using topical rifampin were identified as RR factors for SSIs (RR 22.21, 95% CI [4.81, 102.47], P < 0.001; RR 13.04, 95% CI [1.22, 139.33], P = 0.034; RR 7.09, 95% CI [1.12, 53.99], P = 0.042). We did not observe any local or systemic side effects resulting from the use of rifampin. CONCLUSIONS: The use of topical rifampin is an easy and effective method for reducing SSIs and meningitis/VP shunt infections related to NTD surgery.

An Extraordinary Cause of the Sucking Difficulty: Ecthyma Gangrenosum.

Ecthyma gangrenosum is a cutaneous lesion often associated with pseudomonas aeruginosa bacteremia, even though it may develop without bacteremia and may originate from other bacterial and fungal organisms. Pseudomonas aeruginosa bacteremia or sepsis, which mainly affects immunocompromised patients, frequently occurs in hospitals. This lesion typically occurs on the extremities and gluteal and perineal regions. In this report we present a case of ecthyma gangrenosum in a premature newborn occurring secondary to pseudomonas sepsis causing sucking dysfunction due to tissue loss in the lip, soft palate, and tongue.

The Use of Acute Peritoneal Dialysis in Critically Ill Newborns.

BACKGROUND To evaluate the efficacy, complications, and mortality rate of acute peritoneal dialysis (APD) in critically ill newborns. MATERIAL AND METHODS The study included 31 newborns treated in our center between May 2012 and December 2014. RESULTS The mean birth weight, duration of peritoneal dialysis, and gestational age of the patients were determined as 2155.2 ± 032.2 g (580-3900 g), 4 days (1-20 days), and 34 weeks (24-40 weeks), respectively. The main reasons for APD were sepsis (35.5%), postoperative cardiac surgery (16%), hypoxic ischemic encephalopathy (13%), salting of the newborn (9.7%), congenital metabolic disorders (6.1%), congenital renal diseases (6.5%), nonimmune hydrops fetalis (6.5%), and acute kidney injury (AKI) due to severe dehydration (3.2%). APD-related complications were observed in 48.4% of the patients. The complications encountered were catheter leakages in nine patients, catheter obstruction in three patients, peritonitis in two patients, and intestinal perforation in one patient. The general mortality rate was 54.8%, however, the mortality rate in premature newborns was 81.3%. CONCLUSIONS APD can be an effective, simple, safe, and important therapy for renal replacement in many neonatal diseases and it can be an appropriate treatment, where necessary, for newborns. Although it may cause some complications, they are not common. However, it should be used carefully, especially in premature newborns who are vulnerable and have a high mortality risk. The recommendation of APD therapy in such cases needs to be verified by further studies in larger patient populations.

Is platelet mass a more significant indicator than platelet count of closure of patent ductus arteriosus?

BACKGROUND: The purpose of this study was to evaluate whether or not platelet mass contributes to closure of patent ductus arteriosus (PDA) in premature newborns. STUDY DESIGN AND SUBJECTS: This retrospective study included 115 preterm newborns with hemodynamically significant PDA (hPDA) and 120 newborns without PDA. The newborns' platelet count, mean platelet volume (MPV) and platelet distribution width (PDW) were noted from their files and the platelet mass (platelet count plus MPV/10(3)) was calculated. Patients with congenital abnormality, persistent pulmonary hypertension or sepsis were not included in the study. RESULTS: Platelet count and PDW were found to not be risk factors for closure of hPDA (p > 0.05), but both high platelet mass (OR 1.25; 95% CI 1.12-1.41) and MPV (OR 1.87; 95% CI 2.52-3.85) were determined to be independent risk factors for hPDA. CONCLUSIONS: Platelet mass may be a more significant indicator than platelet count of closure of hPDA in preterm newborns.

The levels of vitamin B12, folate and homocysteine in mothers and their babies with neural tube defects.

The aim of the present study was to determine the serum levels of vitamin B12, folate, and homocysteine (Hcy) in mothers and their babies, and to assess the association between these levels and neural tube defect (NTD). The study group included 92 baby-mother pairs, where the babies had NTD, and the control group included 102 pairs, where the babies had no NTD, from May 2012 to May 2015. Plasma vitamin B12, folate, and Hcy levels of the babies and mothers were measured, and compared with each other. NTD was diagnosed in 2.6% of our babies. The vitamin B12 levels in the mothers and the babies in the study group were determined as 166.2 ± 63.7 pg/mL and 240.3 ± 120.3 pg/mL, and in the control group as 1 9 0 ± 80.2 pg/mL and 299.5 ± 151.4 pg/mL, respectively. There was a significant difference between the two groups in terms of both the mothers' and the babies' vitamin B12 levels (p = 0.024 and p = 0.003, respectively). The plasma folate levels of the mothers in the study group (5.2 ± 3 ng/mL) were significantly lower than control group (6.4 ± 4.3 ng/mL, p = 0.032).The plasma Hcy level of the mothers in the study group (9.3 ± 3.8 µmol/L) was significantly higher than the control group (7 ± 3.8 µmol/L, p < 0.001). High plasma Hcy levels and low plasma folate and vitamin B12 levels are risk factors for NTD. Our results show that the risk for NTD can be decreased by fortification of mothers-to-be, particularly in rural areas with folate and vitamin B12 deficiency, which would lower the plasma Hcy level.

A Single-Center Experience of CNS Anomalies or Neural Tube Defects in Patients With Jarcho-Levin Syndrome.

Jarcho-Levin syndrome (JLS) is a genetic disorder characterized by distinct malformations of the ribs and vertebrae, and/or other associated abnormalities such as neural tube defect, Arnold-Chiari malformation, renal and urinary abnormalities, hydrocephalus, congenital cardiac abnormalities, and extremity malformations. The study included 12 cases at 37-42 weeks of gestation and diagnosed to have had Jarcho-Levin syndrome, Arnold-Chiari malformation, and meningmyelocele. All cases of Jarcho-Levin syndrome had Arnold-Chiari type 2 malformation; there was corpus callosum dysgenesis in 6, lumbosacral meningmyelocele in 6, lumbal meningmyelocele in 3, thoracal meningmyelocele in 3, and holoprosencephaly in 1 of the cases. With this article, the authors underline the neurologic abnormalities accompanying Jarcho-Levin syndrome and that each of these abnormalities is a component of Jarcho-Levin syndrome.

A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome.

Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.

Factors affecting infection development after meningomyelocele repair in newborns and the efficacy of antibiotic prophylaxis.

AIM: The purpose of this study was to evaluate the clinical and surgical variables that may be associated with wound infection and meningitis/ventriculoperitoneal (VP) shunt infection in newborns diagnosed with meningomyelocele (MMC), as well as the efficacy of antibiotic prophylaxis in reducing these complications. MATERIAL AND METHODS: The data of 91 neonates diagnosed with MMC, who underwent surgical intervention between May 2012 and December 2014, were retrospectively evaluated. Multivariant logistic regression analysis was used to determine the possible clinical and neurosurgical variables associated with meningitis/VP shunt infection and surgical wound infection. Spearman's test was performed for the correlation analysis. RESULTS: Following MMC closure, of the 91 neonates, 18 (16.4%) developed meningitis/shunt infection and 12 (11%) developed surgical wound infection. The operation time was not a significant independent risk factor for the development of meningitis (RR 0.618 [0.199-1.922], p = 0.406). Open neural placodes that were not covered by any pseudomembrane (myeloschisis), external ventricular drainage (EVD) use, and flap transposition were determined as significant relative risk factors for the development of meningitis (RR 8.655 [2.329-32.157], p = 0.001; RR 9.404 [1.183-74.743], p = 0.034; RR 8.125 [2.496-26.448], p = 0.001; and RR 3.150 [1.963-10.308], p = 0.048, respectively). Deep surgical wound infection was not correlated with the operation time or wound surface area. However, there was an intermediate but very significant positive correlation between meningitis and cerebrospinal fluid (CSF) leakage, length of hospitalization, and flap transposition (r = 0.377, 0.420, 0.357, and 0.503, respectively; for all values, p < 0.001). CONCLUSIONS: There was no association between MMC closure and development of infection. Since it carries a high risk for the development of meningitis, the EVD system should be avoided unless necessary. Routine prophylactic antibiotic use did not reduce the infection risk in MMC repair surgery. Thus, antibiotics should not be used if there are no risk factors predisposing to infection.

Impact of patent ductus arteriosus and subsequent therapy with ibuprofen on the release of S-100B and oxidative stress index in preterm infants.

BACKGROUND: Hemodynamically significant patent ductus arteriosus (hsPDA) leads to injury in tissues/organs by reducing perfusion of organs and causing oxidative stress. The purpose of this study was to evaluate the oxidant/antioxidant status in preterm infants with hsPDA by measuring the total antioxidant capacity and total oxidant status and to assess neuronal damage due to oxidant stress related to hsPDA. MATERIAL AND METHODS: This prospective study included 37 low-birth-weight infants with echocardiographically diagnosed hsPDA treated with oral ibuprofen and a control group of 40 infants without PDA. Blood samples were taken from all infants, and than the total antioxidant capacity (TAC), total oxidant status (TOS), and S-100B protein levels were assessed and oxidative stress index was calculated before and after therapy. RESULTS: The mean pre-therapy TOS level and oxidative stress index (OSI) value of the patients with hsPDA were significantly higher, but TAC level was lower than in the control group. There were no statistically significant differences in the mean post-therapy values of TOS, TAC, OSI, and S-100B protein between the two groups. CONCLUSIONS: hsPDA may cause cellular injury by increasing oxidative stress and damaging tissue perfusion; however the brain can compensate for oxidative stress and impaired tissue perfusion through well-developed autoregulation systems to decrease tissue injury.

A confusing coincidence: neonatal hypoglycemic seizures and hyperekplexia.

Hyperekplexia is a rare, nonepileptic, genetic, or sporadic neurologic disorder characterized by startle responses to acoustic, optic, or tactile stimuli. Genetic defects in glycine receptors as well as encephalitis, tumors, inflammation, and disgenesis are among the etiologic causes of the disease. The main problem in hyperekplexia is the incomplete development of inhibitory mechanisms or exaggerated stimulation of excitatory mediators. Hyperekplexia is often confused with epileptic seizures. Here we present a case with hypoglycemic convulsions coexisting with hyperekplexia, causing diagnostic difficulty.

Idiopathic neonatal colonic perforation.

Though the perforation of the colon in neonates is rare, it is associated with more than 50% mortality in high-risk patients. We report a case of idiopathic neonatal perforation of the sigmoid colon in an 8-day-old, healthy, male neonate without any demonstrable cause.

The importance of administration of early surfactant and nasal continuous positive airway pressure in newborns with respiratory distress syndrome.

AIM: Mechanical ventilation is an invasive method and causes to important problems in the respiratory tract and lung parenchyma. The objective of our study was to investigate if administration of early surfactant and nasal continuous positive airway pressure (nCPAP) was superior to delayed surfactant administration and mechanical ventilation. MATERIAL AND METHODS: The study was conducted in the Van 100th Year University, Medical Faculty Hospital, Neonatal Intensive Care Unit. One hundred and nine infants with respiratory distrss syndrome (RDS) with a gestational age of 32 weeks and/or below were included in the study. Surfactant was given to 61 infants in the delivery room or intensive care unit and subsequently nCPAP was administered. Surfactant was administered in 48 infants in the control group and mechanical ventilation was inititated subsequently. Informed consent was obtained from the relatives of all patients and ethics committee approval was also obtained (Approval number: 03.02.2011/15). RESULTS: There was no statistically significant difference between the two groups in terms of gestational age, birth weight, gender, height and head circumference measurements (p>0.05). The mean hospitalization time in the patients in the study group was 24.4±17.8 days, whereas the mean time of nCPAP was 28.4 (4-120) hours. In the study group, intracranial hemorrhage was found with a rate of 27.85%, bronchopulmonary dysplasia was found with a rate of 4.91%, pneumothorax was found with a rate of 3.27%, necrotizing enterocolitis was found with a rate of 3.27%, patent ductus arteriosus was found with a rate of 16.39, sepsis was found with a rate of 22.95% and retinopathy of prematurity was found with a rate of 1.63%. No statistically significant difference was found between the study and control groups in terms of the rates of complications. During the follow-up period, 17 (27.86%) patients were lost. The length of stay on mechanical ventilation in the study group was found to be statistically significantly shorter compared to the control group (p<0.05). CONCLUSIONS: In our study, it was observed that administration of early surfactant and nCPAP in treatment of preterm newborns with a diagnosis of RDS markedly decreased the lenght of stay on mechanical ventilation, but had no significant impact on morbidity and mortality.

A very rare entity of diabetes insipidus associated with Edwards syndrome.

Edwards syndrome is the second most commonly seen trisomy. It was first described by John Hamilton Edwards in 1960. Although most cases result in termination or foetal loss, live births have been documented in 5%. Edwards syndrome is characterized by multisystem anomalies, of which holoprosencephaly (HPE) is observed in 4-8% of cases. The clinical findings correspond to the degree of HPE malformation. Convulsions and endocrinopathies are among the severe clinical findings. The most common endocrinopathies are central diabetes insipidus (DI), hypothyroidism, hypocortisolism and growth hormone deficiency. The coexistence of holoproencephaly and DI in Edwards syndrome was discussed under the light of literature.

Clinical and neurological findings of severe vitamin B12 deficiency in infancy and importance of early diagnosis and treatment.

AIM: Nutritional vitamin B12 deficiency among children in developing countries may lead to a severe clinical status. In this article, the clinical manifestations of vitamin B12 deficiency and the consequences of delay in its diagnosis have been evaluated. METHODS: A total of 41 patients who were hospitalised and treated with the diagnosis of severe vitamin B12 deficiency in the paediatric haematology department of the hospital were enrolled in the trial. The diagnosis of severe vitamin B12 deficiency was based on haematological values, a serum vitamin B12 level of <100 pg/mL and a normal level of folic acid. Mental, neuro-motor and social developments of the enrolled patients were assessed by the same physician using the Ankara Developmental Screening Inventory. RESULTS: The mean age of 25 male and 16 female patients was determined as 12 (6-18 months) months. Almost all of the children had been fed with breast milk and a poor nutritional state was found in all of the mothers. Non-specific findings such as growth retardation (78%), hyperpigmentation of the skin (78%), diarrhoea (63.4%), convulsion (14.6%), weakness, reluctance to eat, vomiting, irritability and tremor were found in all the patients, in addition to hypotonia, motor retardation and pallor. Treatment with vitamin B12 provided recovery in all the patients. The mean age of the patients with full recovery was 11.7 months, while the mean age of patients with partial recovery was 12.9 months. CONCLUSION: As a delay in the diagnosis causes irreversible neurological damage, early diagnosis and treatment is highly important.

Spectrophotometric analysis in umbilical cords of infants with meconium aspiration syndrome.

We compared spectrophotometric analysis of the umbilical cords of infants with meconium aspiration syndrome (MAS) or with meconium-stained amniotic fluid (MSAF) and healthy infants. In a prospective study, 15 infants with MAS and 37 infants with MSAF were enrolled. Twenty healthy infants formed a control group. The absorption peak of umbilical cords with meconium was significantly higher in the infants with MAS or MSAF than in controls. Spectrophotometric analysis of the umbilical cords with meconium may be useful to identify developed neonates with MAS or MSAF.

Are consanguineous marriage and swaddling the risk factors of developmental dysplasia of the hip?

The purpose of this study was to investigate prospectively the effects of swaddling and consanguineous marriage on developmental dysplasia of the hip and associated risk factors. We screened by ultrasound 265 infants using the Graf method. The Pediatrics Department referred all newborn infants with suspected instability or a recognized risk factor to the orthopedic clinic. Risk factors of developmental dysplasia of the hip were searched and noted in these patients. Swaddling and consanguineous marriage were also determined and noted. We observed 164 of 265 infants (61.9 %) who had been swaddled and that 64 of 265 infants' parents were in a consanguineous marriage (24.2 %). In the statistical analysis that was conducted for swaddling and consanguineous marriage, highly significant differences were found. Our study showed that the rate of developmental dysplasia of the hip is very high, 11.7 %, in our region, eastern Turkey. Also, we commonly see improper swaddling and consanguineous marriage in our region, which affects many infants.

Acute appendicitis in two children with Henoch-Schönlein purpura.

In Henoch-Schönlein purpura (HSP), involvement of the ileum and ascending colon with vasculitis can mimic appendicitis and cause unnecessary appendicectomy. A 13-year-old boy presented with signs of HSP and abdominal pain. He was treated with prednisolone (2 mg/kg/day) for 10 days, but there was no improvement. At laparotomy he had acute suppurative appendicitis. A 12-year-old girl presented with HSP associated with abdominal pain and bloody diarrhoea and at laparotomy was also found to have suppurative appendicitis. Both patients had vasculitic areas in the ileum. In HSP, although suppurative appendicitis is rare, it should always be considered and appropriate investigations, including ultrasonography, undertaken.

Molecular analysis and clinical findings of Griscelli syndrome patients.

Griscelli syndrome (GS) is a rare autosomal recessive disorder associated with skin or hair hypopigmentation, hepatosplenomegaly, pancytopenia, and immunologic and central nervous system abnormalities. GS type II is caused by RAB27A mutations. We present RAB27A mutation analysis of 6 cases diagnosed as GS type II. Missense mutations (L26P and L130P) in 2 cases, deletion of 5 bases (514delCAAGC) in 2 cases, and 1 base deletion (148delA) in 2 cases were detected. This report has importance in phenotype-genotype correlation of different types of mutations including missense mutations and deletions within the RAB27A gene in GSII syndrome.