Interleukin-6 and C-reactive protein load in pre-storage and post-storage white blood cell-filtered red blood cell transfusions in premature infants.

OBJECTIVE: Leukocyte contamination during blood transfusion can cause many adverse effects. Filtration can be performed either at bedside during the transfusion or as pre-storage filtration. Pre-storage filtration is superior to bedside filtration because leukocytes are removed prior to storage, thus preventing further adverse effects associated with the storage of these cells. METHODS AND MATERIALS: One hundred and six infants were randomised into two groups: pre-storage filtration (group 1, n = 53) and bedside filtration (group 2, n = 53). C-reactive protein (CRP) and interleukin-6 (IL-6) levels were analysed within 24 h prior to the transfusion and 24 h after completion of the transfusion. RESULTS: In group 1, pre-transfusion median CRP and IL-6 levels were 2·95 (0·73-10·25) mg L(-1) and 8·59 (3·45-20·55) pg L(-1) , respectively, and post-transfusion median CRP and IL-6 levels were 2·28 (0·44-12·87) mg L(-1) and 6·62 (2·18-27·87) pg L(-1) , respectively. In group 2, pre-transfusion median CRP and IL-6 levels were 1·30 (0·40-7·84) mg L(-1) and 4·40 (2-17·12) pg L(-1) , respectively, and post-transfusion median CRP and IL-6 levels were 3·50 (0·50-7·85) mg L(-1) and 8·30 (3·48-23·75) pg L(-1) , respectively. There were no differences between pre-storage and post-storage leukoreduction average IL-6 and CRP levels in either group (P > 0·05 for both). Packed red blood cell (PRBC)-related necrotizing enterocolitis was detected in one infant in group 2. CONCLUSIONS: Because leukocytes in PRBC transfusions can be associated with many undesirable effects, leukoreduction is the best choice to prevent those effects. However, this method is still controversial. We demonstrated that using pre-storage and post-storage leukoreduction methods in erythrocyte transfusions did not change CRP or IL-6 levels, which are indicators of acute-phase response.

Effect of maternal cervical bacterial colonization on neonatal outcome in high-risk pregnancies: results from a tertiary maternity center in Turkey.

PURPOSE: To evaluate and compare the morbidity and mortality of neonates born to pregnant women with positive and negative cervical cultures. MATERIALS AND METHODS: The demographic and clinical features of mothers included in this study, along with details of the microorganisms isolated on maternal cervical cultures and the number of days between a positive cervical culture and delivery were recorded. Neonates were stratified into two groups based on cervical culture results of their mothers--Group 1, positive cervical culture; Group 2, negative cervical culture. RESULTS: A total of 216 women who delivered 242 infants were included in the study. Group 1 consisted of 90 neonates while Group 2 had 152 newborns. The difference between the groups with demographic characteristics was statistically insignificant. Mean levels of the acute phase reactants, CRP, and IL-6, obtained six hours after delivery were significantly higher in Group 1 compared to Group 2 (p < 0.05 for C-reactive protein (CRP) andp < 0.001 for IL-6). Although there was no difference between groups in terms of duration of respiratory support, mean duration of hospitalization, as well as mortality rate were significantly higher in Group 1 (p < 0.001, p < 0.05, respectively). CONCLUSIONS: Women diagnosed with a high-risk pregnancy should be treated with antibiotics immediately after a positive cervical culture result, and delivery should be delayed until the success of antibiotic treatment can be evaluated. Early initiation of maternal antibiotic therapy is associated with shorter durations of hospital stay for newborns. Close follow-up of mothers with high-risk pregnancies and extension of treatment duration are critical for determining prognosis in newborn infants.

Case report: partial trisomy 4q27q35 syndrome.

Partial trisomy 4q is a rare chromosomal abnormality which results in variable clinical features, often including growth and developmental delay, mental retardation and dysmorphic features. We herein report a newborn with the diagnosis of partial trisomy 4q with Hirschsprung's disease who was monitored in neonatal intensive care unit. This is the first report of partial trisomy 4q27q35 with accompanying Hirschsprung's disease.

Fryns anophthalmia-plus syndrome: two rare cases.

Fryns anophthalmia-plus syndrome is a rare syndrome with clinical diversity primarily including anophthalmia/microphthalmia, facial clefts, cleft lip/palate, ear and nasal deformities. Here we present two different cases of APS with anopthalmia/microphthalmia, cleft palate, low set ears, ventriculomegaly and one of which had intestinal non-fixation anomaly not described in the literature before.

Is metoclopramide safe for the premature infant?

BACKGROUND: Gastroesophageal reflux disease (GERD) may occur with poor weight gain, esophagitis, hematemesis and respiratory problems in an infant. Common treatment strategies include positioning, feeding thickeness, histamine2 receptor antagonists, antiacids, and prokinetics. Metoclopramide is a prokinetic drug used to treat GERD and it has been reported to be a most commonly prescribed medication in neonatal intensive care unit (NICU). This research involves a patient that was born at 30 weeks' gestation age and on the twentieth day of his admission, vomiting and gastric residuals were observed. All diseases which are related these symptoms were excluded. With no improvement observed following non-pharmacological interventions and metoclopramide was started with a dosage of 0.1 mg/kg, per dose 12 hours. After the second dose of metoclopramide, dystonic reactions occured. The premature infant was evaluated for differential diagnosis of the abnormal movements. No abnormal findings were reported. The dystonic reactions didn't recur after metoclopramide was stopped. CONCLUSIONS: The observed adverse effects of metoclopramide in the preterm infant might be due to an excessive serum concentration of the drug as a result of its prolonged plasma clearance in this age group. Attention is drawn to the serious adverse effects of metoclopramide in the neonate, particularly premature infant.

First and second trimester biochemical markers in familial mediterranean fever.

OBJECTIVES: Our aim was to investigate whether the maternal serum concentrations of first and second trimester serum analytes are altered in familial Mediterranean fever (FMF) pregnancies. MATERIALS AND METHODS: The screening tests were compared in a series of 16 serum samples from FMF pregnancies and in a cohort of 48 pregnant women with normal pregnancy. Serum samples were obtained between 11 and 13 weeks; 16 and 18 weeks gestation. RESULTS: Serum pregnancy-associated plasma protein-A (PAPP-A) levels, expressed as multiples of the median (0.9 ± 0.45 MoM) in the control group, were significantly higher than FMF patients (0.6 ± 0.3 MoM) (p = 0.027). Analyses of alpha-fetoprotein, human chorionic gonadotropin and oestriol levels showed no significant differences between FMF and normal pregnancies. CONCLUSIONS: Our study revealed that low levels of PAPP-A are associated with FMF.

Evaluation of treatment with hydrocortisone on oxidant/antioxidant system in preterm infants with BPD.

AIM: Evidence that oxidative stress plays a role in the development of bronchopulmonary dysplasia (BPD). There is a close relationship between oxidative stress and inflammation. In this study, it is aimed to investigate influences of hydrocortisone used in the treatment of BPD on anti-oxidant system in preterm infants with BPD. PATIENTS AND METHODS: The study enrolled 33 infants with severe BPD who were undergone inpatient treatment in neonatal intensive care unit (NICU) of our Hospital and received therapy with hydrocortisone. Total oxidant status (TOS) and total anti-oxidant capacity (TAC) levels of infants enrolled to the study before and one week after the hydrocortisone therapy were studies and oxidative stress index levels were calculated. Pre- and post-treatment TOS, TAC and OSI index levels were statistically compared. RESULTS: In preterm infants with BPD, who were enrolled into the study, TOS and OSI index were found high, whereas TAC values were low. Following the treatment with hydrocortisone, statistically significant decrease in TOS and OSI index and statistically significant elevation in TAC levels were found in comparison with pre-treatment levels. CONCLUSIONS: The treatment with hydrocortisone, which is used for BPD, improves anti-oxidant system and reduces oxidative stress in infants with BPD. There is need for further studies in order to clarify the physio-pathogenesis.

Compassionate use of voriconazole in newborn infants diagnosed with severe invasive fungal sepsis.

BACKGROUND: Invasive fungal sepsis has become the third most common cause of late-onset infections in many neonatal intensive care units (NICU). The prevalence rate ranges from 2.6% to 16.7% among very-low-birth-weight infants and from 5.5% to 20% among extremely low-birth-weight infants. Despite the development of several new antifungal agents in the past few years, the management of serious fungal infections in the newborn continues to be problematic. MATERIALS, METHODS AND RESULTS: Voriconazole treatment was given to 17 newborns with invasive fungal sepsis, in initial doses of 2-3 mg/kg twice daily. In spite of the complications of cholestasis and liver function abnormality in 2, the dose regimen was 4-6 mg/kg (loading dose), followed by initial doses of 2-3 mg/kg twice daily. Drug cessation did not occur, and no permanent side effects were observed. In the end, 12 patients had been cured with antifungal treatment. CONCLUSIONS: Voriconazole at this dosage common with other antifungal agents or alone appears to be a safe and effective antifungal agent for neonatal invasive fungal sepsis. Based on relevant literature, to the best of our knowledge, ours is the largest case series to underline this issue. However, further studies are required to determine the pharmacokinetics (e.g. serum half-life, concentration, time of peak concentration, and distribution) of voriconazole use in newborns.  

The effects of water birth on neonatal outcomes: a five-year result of a referral tertiary centre.

OBJECTIVE: As scant information is present about the effect of water birth on newborns, we aimed to detect the fetal outcomes of water birth. MATERIALS AND METHODS: A hundred and ninety one among totally 220 newborns who were born by water birth were enrolled. The demographic and clinical features of the patients, birth complications, infection rates and rates of neonatal intensive care unit attendance were evaluated. RESULTS: The mean gestational week and birth weight were 39.2±1.3 weeks and 3326±409 g. 26% of the mothers was primiparous. Birth trauma was observed in three patients (1.6%) as one brachial nerve paralysis, one cord rupture and one cephal hematoma. Six of the patients (3.1%) were admitted to neonatal intensive care unit (NICU); four of whom had respiratory tract problems. CONCLUSIONS: Water birth is a safe method of delivery for the neonates when certain criteria are met.  

Trends of hepatitis B notification rates in Turkey, 1990 to 2012.

Turkey is a country with intermediate endemicity for hepatitis B, and approximately 4% of the population are HBsAg-positive. A number of measures have been implemented to prevent hepatitis B infection. In 1998, hepatitis B antigen was included in the national immunisation programme, and infants have since been vaccinated with three doses. Catch-up strategies, vaccination for high risk groups and screening measures were also adopted. The aim of this study was to evaluate the impact of the prevention and control strategies on hepatitis B notification rates in Turkey in the period from 1990 to 2012, using data from the national surveillance system. Secular trends revealed that rates showed an initial increasing trend, followed by a steady decline from 2005. The most dramatic decline occurred among children younger than 15 years, highlighting the benefits of vaccination and catch-up strategies. However, vaccination cannot fully explain the decrease in this age group. Socioeconomic development, through interrupting the horizontal transmission may also have contributed. After 2005, a steady decline was achieved also among those 15 years and older. The rates in adults were higher, which indicates that stronger prevention measures are needed to target this group, particularly men.

Partial trisomy 3p and partial monosomy 11q associated with double outlet right ventricle and septum pellucidum et vergae: a case report.

Su Partial trisomy 3p and partial monosomy 11q are rare chromosomal disorders with a deletion of part of chromosome 11 combined with a duplication of part of chromosome 3. These are usually inherited from a parent who carries a balanced translocation involving chromosome 3, which can result in the unbalanced translocation trisomy 3p in a child. In this paper, we report a newborn who has dysmorphic facial features, double outlet right ventricle, hypotonia, hypospadias, neonatal thrombocytopenia, hydroureteronephrosis, talipes equinovarus and septum pellucidum et vergae. Cytogenetic investigation revealed 46,XY,der(11)t(3;11)(p22.2;q23.3) and the karyotype of his father showed a balanced translocation, 46XY,t(3;11)(p22.2;p23.3).

Jarcho-Levin syndrome presenting with severe hydrocephalus: a report of three cases.

Jarcho-Levin syndrome (JLS) is a genetic disorder characterized by defects of spine formation, abnormal fusion of the ribs at the costovertebral and costochondral junction due to abnormal vertebral and costal segmentation. There are two subtypes associated with different distribution and severity of defects in JLS. While vertebral segmentation abnormalities are frequent in spondylo-thoracic subtype costal segmentation and fusion abnormalities are frequent in spondylo-costal subtype. Neural tube defects with severe hydrocephalus are rare findings in this syndrome. Herein we report three infants with severe hydrocephalus associated with Jarcho-Levin syndrome.

Transfusion-associated necrotising enterocolitis in very low birth weight premature infants.

OBJECTIVE: Our aim was to determine the relationship between red blood cell transfusion and necrotising enterocolitis (NEC) in all admitted very low birth weight (VLBW) infants with or without transfusion. STUDY DESIGN: All VLBW neonates were categorised into five groups: (i) subjects that developed NEC <48 h after transfusion (n = 15); (ii) subjects that developed NEC >48 h after transfusion (n = 31); (iii) subjects that were never transfused but developed NEC, (n = 50); (iv) subjects that were transfused but did not develop NEC, (n = 250) and (v) subjects that were neither transfused nor developed NEC (n = 301). RESULTS: A group of 647 infants were enrolled in the study. Mean gestational age and birth weight of the patients were 29 ± 3.1 weeks and 1157 ± 237 g, respectively. The mean age at the onset of NEC in the NEC groups were 20 ± 2.3 days, 12 ± 3 days and 11 ± 2.6 days, respectively (P < 0.05). The mean interval from the last transfusion to the onset of NEC was 16.8 ± 8.8 h in group 1 and 240 ± 50 h in group 2 (P < 0.05). CONCLUSION: In this study, we sought to evaluate all VLBW infants, whether they received a transfusion or not. We suggest that transfusion associated NEC exists, but many other factors influence this multifactorial disease. The age of NEC onset was later in transfused vs non-transfused patients, whereas the interval between transfusion and NEC was shorter in transfused vs non-transfused patients.

Urgent surgical management of a prenatally diagnosed midgut volvulus with malrotation.

Midgut volvulus is a life-threatening condition that commonly presents during the first year of life rarely antenatally. Here we report successful urgent surgical management of an unusual case with malrotation exhibiting the sonographic findings of volvulus in utero that leads to premature birth. This 34-wk, 2700 g infant was born via spontaneous vaginal delivery. Prenatal ultrasound showed polyhydramnios and 30 x 40 mm cystic lesion showing whirlpool sign in abdomen that made us think midgut volvulus. The patient had distended abdomen with skin discoloration. An emergency surgical management was performed showing malrotation, volvulus and a 15 cm ischemic necrotic region of terminal ileum.

A preterm infant with prolonged respiratory problems due to Ritscher-Schinzel syndrome.

Ritscher-Schinzel also known as cranio-cerebello-cardiac (3C) syndrome is a very rare clinical entity. The striking features of this syndrome are cerebellar, cardiac and craniofacial abnormalities. Life threatening features of this syndrome are generally associated with cardiac abnormalities. We here present prolonged respiratory problems due to pulmonary hypertension in a preterm baby with Ritscher-Schinzel syndrome.

Congenital mirror hand deformity.

Ulnar dimelia or commonly called mirror hand is a rare congenital anomaly of upper extremity characterized by duplication of ulna, absence of the radius with symmetrical polydactyly. This anomaly may be associated with shoulder dislocation, fibular dimelia, idiopathic scoliosis, cirrhosis, pyloric hypertrophy or polycystic kidney. We report a new case of this rare congenital anomaly which was not associated with any other malformation.

A case of fetal carbamazepine syndrome with right hemihypoplasia of the entire body.

Anticonvulsant drugs taken by pregnant women to prevent seizures are among the most common causes of potential harm to the fetus. It has been suggested that carbamazepine was less teratogenic than the other drugs. Here, we report a case of fetal carbamazepine syndrome presenting with facial dysmorphism, congenital heart defect, skeletal abnormalities, renal agenesis, ambiguous genitalia, anal atresia, and right hemihypoplasia of the entire body. To the best of our knowledge this is the most severe case of fetal carbamazepine syndrome in the literature. This case can provide useful data about teratogenicity of carbamazepine therapy during the pregnancy.

A case of Keutel syndrome diagnosed in the neonatal period: associated with Binder phenotype.

Keutel syndrome is a rare autosomal recessive disorder, characterized by brachytelephalangia (short, broad distal phalanges), midfacial hypoplasia, abnormal cartilage calcifications, peripheral pulmonary stenosis and hearing loss. Binder profile is a well known maxillonasal dysplasia composed of midfacial hypoplasia with absence of anterior nasal spine and facial dysmophism (short nose, flat nasal bridge, perialar flatness, convex upper lip). Here we report a Keutel syndrome presenting with Binder phenotype, abnormal calcifications, hearing loss and respiratory insufficiency in the newborn period. Keutel syndrome should be considered in the differential diagnosis of children with tracheobronchial calcifications, midfacial hypoplasia and stippled epiphysis.

Severe semilobar holoprosencephaly and lissencephaly associated with cebocephaly in a newborn.

Holoprosencephaly is frequently accompanied by midline facial abnormalities such as hypotelorism, cyclopia, etmocephaly and cebocephaly. Cebocephaly is a very rare congenital anomaly combining with semilobar holoprosencephaly. Chromosomal analysis shows normal karyotyping. Lissencephaly and holoprosencephaly are rare associations, that have not been reported yet with cebocephaly. Herein we present the first case of cebocephaly with severe semilobar holoprosencephaly and lissencephaly.

CD31 and Factor VIII in angiogenesis of normal and pre-eclamptic human placentas.

We aimed to evaluate normal and pre-eclamptic placental vasculature by using CD31 and Factor VIII. We evaluated placentas of 37 pre-eclamptic women by using immunohistochemical staining with CD31 and Factor VIII antibodies. Individual microvessels in the placental tissues were counted at × 100 and × 400 magnification. Microvessel counts were lower in placentas of pre-eclamptic patients compared with healthy controls after staining with CD31 (26.5 ± 5.7 vs 103.9 ± 8.2, p < 0.001) and Factor VIII (24.8 ± 6.7 vs 98.7 ± 10.3, p < 0.001). Balance between proangiogenic and antiangiogenic factors seems to be shifted in favour of anti-angiogenic factors in pre-eclampsia.