Efficiency of topical rifampin on infection in open neural tube defects: a randomized controlled trial.

OBJECTIVES: Neural tube defects are the second most common congenital malformation in humans. Despite significant decreases in neural tube defects and related mortality and morbidity with recent developments, infections remain an important problem. Research on the role of topical therapy for managing neural tube defects and associated infections in the neonatal period has been limited. This randomized controlled trial aimed to investigate the efficiency of topical Rifampin on infection control in paraplegic newborns with open neural tube defects. METHODS: Thirty-seven patients who underwent an operation for neural tube defects were included. Topical Rifampin and cefotaxime were administered to 19 patients constituting the case group and local saline and cefotaxime were administered to a control group. Patients were examined for ventriculoperitoneal shunt infection/dysfunction, surgical site infection, urinary tract infection, and sepsis. RESULTS: None of the patients using topical rifampin had ventriculoperitoneal shunt infection/dysfunction, surgical site infection, urinary tract infection, or sepsis. In the control group, ventriculoperitoneal shunt infection/dysfunction was found in 4 (22.2%) cases, surgical site infection in 3 (27.7%), urinary tract infection in 3 (27.7%), and sepsis in 5 (27.7%), with statistically significant differences between the groups (p = 0.01, p = 0.032, p = 0.032, and p = 0.002, respectively). No local or systemic side effect was observed regarding rifampin use. CONCLUSION: Topical Rifampin is effective in minimizing complications like sepsis, surgical site infection, urinary tract infection, and ventriculoperitoneal shunt infection due to neural tube defect operations. Further research with larger numbers of cases is needed to implement this practice routinely.

Wiskott-Aldrich syndrome: Two case reports with a novel mutation.

BACKGROUND: The Wiskott-Aldrich syndrome (WAS) is X-linked recessive disorder associated with microplatelet thrombocytopenia, eczema, infections, and an increased risk of autoimmunity and lymphoid neoplasia. The originally described features of WAS include susceptibility to infections, microthrombocytopenia, and eczema. AIM: In this case report, we present our experience about two cases diagnosed with a new mutation. METHODS: We report phenotypical and laboratory description of two cases with WAS. RESULTS: We, for the first time, detected a new hemizygote mutation of WAS gene (NM_000377.2 p.M393lfs*102 (c.1178dupT)) in two patients. The first case was an 11-month-old boy presenting with complaints of recurrent soft tissue infection, ear infection, anemia, and thrombocytopenia with a low platelet volume. The second case was a 2-month-old boy presenting with thrombocytopenia and a low platelet volume. Both cases were the first-degree relatives: they were cousins and their mothers were sisters. CONCLUSION: Herein, we report two cases of WAS and a new gene mutation which would disrupt the WAS protein function within the Polyproline (PPP) domain. This report adds to the growing number of mutations which cause complex clinical manifestations associated with WAS.

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.

The Syndrome of Inappropriate Secretion of Anti-Diuretic Hormone (SIADH) and Brucellosis.

BACKGROUND Our study aimed to demonstrate the frequency of the syndrome of inappropriate ADH secretion (SIADH) and associated factors during the course of brucellosis in children and adolescents. MATERIAL AND METHODS The study included children and adolescents aged 0-18 years old diagnosed with brucellosis between 2012 and 2014. The data were collected from patient charts. The diagnosis of brucellosis was made based on titrations >1:160 in standard Wright tube agglutination tests and/or positive culture tests. SIADH diagnosis was made based on the following criteria: euvolemic hyponatremia, serum Na+ <135 mmol/L, presence of serum hypoosmolarity (serum osmolarity <275 mOsm/L), increased urinary sodium (>25 mmol/L with normal dietary salt intake), low uric acid (<2 mg/dL), absence of kidney, thyroid or adrenal disease, and any anti-diuretic use. RESULTS The study included 160 children and adolescents with mean age of 9.58±3.95 years (range: 2-18 years) including 70 girls (43.8%) and 90 boys (56.2%). When the patients were stratified based on SIADH, it was found that SIADH was present in 35 patients (21.9%). SIADH was associated with elevated glucose (p<0.001), ALT (p<0.05), AST (p<0.05), LDH (p<0.001), CRP (p<0.001), and MPV (p<0.001); and decreased potassium (p<0.05), chloride (p<0.001), albumin (p<0.001), total protein (p<0.05), and hemoglobin (p<0.05) levels. CONCLUSIONS Our study reports on the frequency, clinical characteristics, predisposing factors, and management of SIADH that can develop in children and adolescents diagnosed with brucellosis.

Investigation of the effect of the efficiency of noise at different intensities on the DNA of the newborns.

Hearing loss can occur in newborns exposed to high-level noise; noise exposure can cause more physiological stress and can lead to DNA damage. This study was designed to determine DNA damage in newborn rats exposed to sound at different concentrations. For this purpose, 28 newborn (3-6 days old) rats were divided into four groups of 7 rats in each group (Control and Groups of 40 decibel (dB), 70 dB, and 110 dB]. In the experimental groups, 40 dB, 70 dB, and 110 dB (7.5-15 kHz) of sound was applied to the experimental groups for 30 min a day for 7 days. DNA damage levels in the serums obtained from this study were determined by the enzyme-linked immunosorbent assay (ELISA) method. According to this, it was determined that DNA damage in the group exposed to 110 dB showed a statistically significant increase (P < 0.05) compared to the compared to the control, 40 dB, and 70 dB groups. Related to the subject, it was concluded that DNA damage may occur in newborns exposed to 110 dB or higher sound in neonatal units, wards, and home environments with newborn babies. Mothers should be warned about this situation and noise should be kept under 110 dB volume in the environments with the newborns.

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.

How frequently external ventricular drainage device should be changed in children with ventriculoperitonel shunt infection?

OBJECTIVE: The purpose of the presenting study was to determine how frequently external ventricular drainage (EVD) device should be changed in children with ventriculopertienal shunt (VPS) infection during prolonged intravenous antimicrobial therapy. METHODS: In this retrospective study, 25 children with VPS infection were evaluated between January 2012 and December 2013. In these children VPS was surgically removed and appropriate antimicrobial therapy was administered according to cerebrospinal culture results. Data noted about how frequently EVD device had been changed, the number of cells on direct observation of cerebrospinal fluid (CSF), glucose and protein levels of CSF, and CSF culture results were obtained from patients' records. RESULTS: Total 25 children were included in the study. The median age was three months (1 and 65 months). In 44% of children, Staphylococcus epidermidis was isolated. During treatment period, EVD catheter has changed one to six times. A total of 68 EVD catheters were changed in these patients. When the duration of ventriculostomy catheter and leukocyte count in CSF were evaluated on daily basis, leukocyte count was decreased 5 units per day in children whose catheter remained less than 10 days. However, in children whose catheter remained more than 10 days leukocyte count was decreased 2.21 units per day. CONCLUSIONS: In children with VPS infection, EVD device should be changed at every 10 days for the rapid resolution of the infection.

Assessment of cardiac functions in infants with cow's milk allergy.

BACKGROUND: Cow's milk allergy is the most common food allergy in children, with rates estimated at 1.9% to 4.9%. Clinical phenotypes of cow's milk allergy are varied and involve 1 or more target organs, with the main targets being the skin, respiratory system, and gastrointestinal tract. To date, no studies have investigated detailed cardiac function in children with cow's milk allergy. The current study aimed to investigate cardiac function in infants with cow's milk allergy. MATERIAL/METHODS: We studied 42 infants with cow's milk allergy and 30 age- and sex-matched healthy subjects. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. RESULTS: There were no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the 2 groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Ea/Aa ratios in the left ventricle posterior wall and right ventricle free wall were lower in patients with cow's milk allergy than in the control group. The E/Ea ratio in the left ventricle, isovolumic relaxation time, deceleration time, and right and left ventricular myocardial performance indices were higher in patients in the study group. CONCLUSIONS: Our study identified reduced early diastolic tissue Doppler velocities in infants with cow's milk allergy.

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.

Dermatological findings of vitamin B12 deficiency and resolving time of these symptoms.

AIM: The mucocutaneous changes observed during vitamin B12 deficiency in children have been published only as case studies and small case series. In this study, we aimed to demonstrate the frequency of mucocutaneous changes (particularly hyperpigmentation) seen during vitamin B12 deficiency and resolving time of these symptoms with vitamin B12 treatment. MATERIAL AND METHODS: This prospective study was conducted at the pediatrics outpatient clinic of Harran and Yuzuncu Yil University Faculty of Medicine, among 57 patients, aged between 6 and 24 months, who were diagnosed with vitamin B12 deficiency following various examinations and tests. A detailed examination was performed in respect to skin and mucosal findings. Patients with vitamin B12 deficiency were administered intramuscular cyanocobalamin. Prospective examination was continued, and resolving time of symptoms after treatment was recorded. RESULTS: The mean age of the patients enrolled in the study was found to be 12.75 ± 4.75. Hyperpigmentation was reported in 49 (85.96%) patients enrolled in the study; atrophic glossitis in 40 (70.17%), brittle and matt hair in 13 (22.80%), skin lesions (particularly diaper dermatitis) in eight (15.78%) and cheilosis in four (7.01%) patients. Three months after the treatment initiation, hyperpigmentation improved in 87.75%, atrophic glossitis in 97.5% and brittle and matt hair in 92.3% of the patients. Five patients (8.77%) with continuing pigmentation by the end of sixth months were considered as nonresponsive to the treatment. CONCLUSION: Deficiency of vitamin B12 should be considered in the differential diagnosis of infants who present with skin and mucosal lesions.

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.

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.

Intravenous Immunoglobulin Use in Hemolytic Disease Due to ABO Incompatibility to Prevent Exchange Transfusion.

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion. Materials and Methods: This study evaluated the data of infants with ABO HDN in the Turkish Neonatal Jaundice Online Registry. The infants with ABO HDN who met the total serum bilirubin level inclusion criteria (within 2-3 mg/dL of exchange transfusion or even above exchange transfusion level) were included in the study according to the guidelines from the American Academy of Pediatrics and the Turkish Neonatal Society. All patients were managed according to the unit protocols recommended by these guidelines and received light-emitting diode (LED) phototherapy. Infants who only received LED phototherapy, and who received one dose of IVIG with LED phototherapy were compared. Results: During the study period, 531 term infants were included in the study according to inclusion criteria. There were 408 cases in the phototherapy-only group, and 123 cases in the IVIG group. The demographic findings and the mean bilirubin and reticulocyte levels at admission were similar between the groups (p > 0.05), whereas the mean hemoglobin level was slightly lower in the IVIG group (p = 0.037). The mean age at admission was earlier, the need for exchange transfusion was higher, and the duration of phototherapy was longer in the IVIG group (p < 0.001, p = 0.001, and p < 0.001, respectively). The rate of re-hospitalization and acute bilirubin encephalopathy (ABE) was higher in the IVIG group (p < 0.001 and p = 0.01, respectively). Conclusion: In this study, we determined that one dose of IVIG did not prevent an exchange transfusion nor decrease the duration of phototherapy in infants, who had bilirubin levels near or at exchange transfusion level, with hemolytic disease due to ABO incompatibility.

Vitamin D Deficiency Prevalence in Late Neonatal Hypocalcemia: A Multicenter Study

Objective: Late neonatal hypocalcemia (LNH) is a common metabolic problem associated with hypoparathyroidism, high phosphate intake and vitamin D deficiency, often presenting with seizures. In this cross-sectional study, we aimed to evaluate the role of vitamin D deficiency in LNH in Turkey and to describe the characteristics of affected newborns. Methods: Conducted with a cross-sectional design and with the participation of 61 neonatal centers from December 2015 to December 2016, the study included term neonates with LNH (n=96) and their mothers (n=93). Data were registered on the FAVOR Web Registry System. Serum samples of newborns and mothers were analyzed for calcium, phosphate, magnesium, albumin, alkaline phosphatase, intact parathyroid hormone (iPTH) and 25 hydroxyvitamin D [25(OH)D] levels. Results: The median (range) onset time of hypocalcemia was 5.0 (4.0-8.0) days of age, with a male preponderance (60.4%). The median (range) serum 25(OH)D levels of the neonates and their mothers were 6.3 (4.1-9.05) and 5.2 (4.7-8.8) ng/mL, respectively. The prevalence of vitamin D deficiency (<12 ng/mL) was high in both the neonates (86.5%) and mothers (93%). Serum 25(OH)D levels of the infants and mothers showed a strong correlation (p<0.001). While the majority (93.7%) of the neonates had normal/high phosphorus levels, iPTH levels were low or inappropriately normal in 54.2% of the patients. Conclusion: Vitamin D deficiency prevalence was found to be high in LNH. Efforts to provide vitamin D supplementation during pregnancy should be encouraged. Evaluation of vitamin D status should be included in the workup of LNH.

A Multicentered Study on Epidemiologic and Clinical Characteristics of 37 Neonates With Community-acquired COVID-19.

BACKGROUND: Coronavirus disease 2019 (COVID-19) primarily affects adults and spares children, whereas very little is known about neonates. We tried to define the clinical characteristics, risk factors, laboratory, and imagining results of neonates with community-acquired COVID-19. METHODS: This prospective multicentered cohort study included 24 neonatal intensive care units around Turkey, wherein outpatient neonates with COVID-19 were registered in an online national database. Full-term and premature neonates diagnosed with COVID-19 were included in the study, whether hospitalized or followed up as ambulatory patients. Neonates without severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) via reverse transcriptase-polymerase chain reaction testing or whose mothers had been diagnosed with COVID-19 during pregnancy were excluded. RESULTS: Thirty-seven symptomatic neonates were included. The most frequent findings were fever, hypoxemia, and cough (49%, 41%, 27%, respectively). Oxygen administration (41%) and noninvasive ventilation (16%) were frequently required; however, mechanical ventilation (3%) was rarely needed. Median hospitalization was 11 days (1-35 days). One patient with Down syndrome and congenital cardiovascular disorders died in the study period. C-reactive protein (CRP) and prothrombin time (PT) levels were found to be higher in patients who needed supplemental oxygen (0.9 [0.1-8.6] vs. 5.8 [0.3-69.2] p = 0.002, 11.9 [10.1-17.2] vs. 15.2 [11.7-18.0] p = 0.01, respectively) or who were severe/critical (1.0 [0.01-8.6] vs. 4.5 [0.1-69.2] p = 0.01, 11.7 [10.1-13.9] vs. 15.0 [11.7-18.0] p = 0.001, respectively). CONCLUSIONS: Symptomatic neonates with COVID-19 had high rates of respiratory support requirements. High CRP levels or a greater PT should alert the physician to more severe disease.

The relationship between mother and infant plasma trace element and heavy metal levels and the risk of neural tube defect in infants.

OBJECTIVE: To determine levels of trace elements [copper (Cu), zinc (Zn), selenium (Se), and cobalt (Co)] and heavy metals [arsenic (As), mercury (Hg), lead (Pb), and cadmium (Cd)] in the plasma of mothers and infants and investigate the relationship between those levels and neural tube defects (NTD). METHODS: A total of 100 neonates diagnosed with NTD and placed in the Neonatal Intensive Care Unit of Yuzuncu Yil University, Turkey between May 2013 and December 2016 comprised the study group. The control group consisted of 70 healthy neonates not diagnosed with NTD or any other congenital anomalies. For both the groups, mother and infant plasma levels of Cu, Zn, Co, Cd, Se, Hg, As, and Pb were measured and compared. Plasma levels of Cu, Zn, Co, Cd, Se, Hg, As, and Pb were measured and compared between two groups of mothers and infants. FINDINGS: Mother and infant plasma levels of trace elements Zn and Se were determined to be significantly lower in the study group compared with the control group, while Cu levels were significant elevated in the study group (all p values < .05). Plasma levels of heavy metals As, Pb, and Cd were found to be significantly higher in the NTD control group (p < .05 for all). There was no association between maternal infection, maternal smoking status, history of miscarriage, or history of NTD with the development of NTD (p > .05). Differences in maternal age, birth weight, length of gestation, and infant gender for the two groups were also determined not to be statistically significant. RESULTS: High plasma levels of heavy metals As, Pb, and Cd and trace element Cu were identified as risk factors for the development of NTD. At the same time, low plasma levels of trace elements Zn and Se were also found to be risk factors for NTD. However, no association between Hg and Co plasma levels and increased risk for the development of NTD was observed. This study, while being the most comprehensive case study to date investigating the relationship between heavy metals and trace element levels and increased risk of NTD, nonetheless highlights the need for further research in order to make definite statements regarding this relationship.

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.

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.