Effect of cervical and lumbosacral spina bifida cystica on volumes of intracranial structures in children.

PURPOSE: Spina bifida is a major disorder that occurs when the membranes of the spinal cord and medulla fail to close during the embryonic period and affects the individual for the rest of life. Some physical, mental, and social difficulties can be observed in the lives of children with spina bifida after surgery. The aim of this study is to determine what kind of volumetric changes occur in the brain when spina bifida occurs in different regions of the cord. METHODS: The volume of intracranial structures of 14 children aged 1 to 9 years (7 cervical, 7 lumbosacral) with different levels of spina bifida compared with vol2Brain. RESULTS: Spina bifida occurring in the cervical region was found to cause a greater volumetric reduction in subcortical structures, cortex and gyrus than spina bifida occurring in the lumbosacral region. CONCLUSION: We believe that our study will help clinicians involved in the management of this disorder.

A Study of the Relationship Between Cystatin C and Metabolic Bone Disease in Preterm Infants

OBJECTIVE: Cystatin C (CysC) is commonly used as a marker of renal failure in premature infants. The aim of this study was to investigate serum CysC levels in osteopenia of prematurity (OP) and determine whether CysC could be safely used as a marker of renal insufficiency in infants with OP. METHODS: Subjects were 50 preterm infants (≤32 gestational weeks). Calcium (Ca), phosphorus (P) and alkaline phosphatase (ALP) serum levels were measured in postnatal week nine, and bone density was measured concurrently by quantitative ultrasonography. Patients with a Z score of <-2 were considered to have OP. RESULTS: The mean serum CysC levels in preterm infants in postnatal week nine were 1.50±0.19 mg/L. Serum CysC levels were not correlated with speed of sound values, Z scores, serum Ca, P or ALP levels. Serum CysC levels were not significantly different between infants with OP [1.50 (1.35-1.61) mg/L] and in infants without OP [1.58 (1.28-1.70) mg/L]. CONCLUSION: The presence of OP does not affect the safety of CysC as a marker of renal insufficiency in preterm infants.

Effects of Lycopene in Hyperoxia-Induced Lung Injury in Newborn Rats.

The aim of this study was to evaluate the therapeutic effect of lycopene on a hyperoxia-induced lung injury model in rat pups. Full-term rat pups were included in the study 12-24 h after delivery. The pups were separated into 4 groups: normoxia control (NC), hyperoxia control (HC), hyperoxia + lycopene (HL), and normoxia lycopene (NL). The normoxia groups were housed in ambient air, and the hyperoxia groups in > 85% O2. HL and NL groups received 50 mg lycopene in oil/kg body weight/day delivered intraperitoneally (i.p.), the other groups received oil alone. On day 11, the rat pups were sacrificed and their lungs removed. Statistically significant injury was observed in all histological parameters measured (MLI, proliferating cell nuclear antigen (PCNA), and apoptosis) in the HC group (HC vs NC, p = 0.001). This injury could not be reversed with lycopene treatment (HC vs HL, 0.05; NC vs HL, p = 0.001). With hyperoxia, statistically significant decreases were observed in biochemical parameters in terms of SOD, MDA, and IL-6 values (HC vs NC: SOD, p = 0.02; MDA, p = 0.043; IL-6, p = 0.001). The use of lycopene did not provide any improvement in these values (HC vs HL, p > 0.05). Hyperoxia or lycopene had no effect on IL-1ß and GPx (p > 0.05). When comparing NC and NL groups, negative effects were observed in the group given lycopene in terms of MLI, PCNA, apoptosis, and IL-6 (all parameters, p = 0.001). We observed that 50 mg lycopene in oil/kg body weight/day given via i.p. had no curative effect on the hyperoxia-induced lung injury in newborn rats and may even induce adverse effects.

A Newborn With Familial Hemophagocytic Lymphohistiocytosis Complicated With Transfusion Associated Graft Versus Host Disease.

Hemophagocytic lymphohistiocytosis (HLH) is characterized by activation of cytotoxic T and natural killer (NK) cells, and macrophages related to a spectrum of hyperinflammatory disorders. The clinical findings mainly include high fever, cytopenia, splenomegaly, phagocytosis, and proliferation of histiocytes in lymphoreticular tissue. To the best of our knowledge, transfusion-associated graft versus host disease (TA-GVHD) in a 13-day old male newborn with HLH is being reported first time in the literature. The aim of this report was to emphasize the importance of blood products irradiation in the prevention of the development of graft versus host disease especially among high-risk subjects such as newborns with HLH.

The effects of thyroid function on retinopathy of prematurity.

OBJECTIVES: To assess whether TSH and fT4 have a role in the angiogenesis of vaso-obliteration and neovascularization which are the basic pathophysiology of ROP. METHODS: In this retrospective case-control study, the control group (n = 56) included preterm newborns with risk for ROP while the laser group (n = 63) was recruited from cases who developed severe neovascularization and needed laser photocoagulation therapy. Considering the first (vaso-obliteration) and second (neovascularization) phases of the disease, in this study we researched the distribution of thyroid function tests between groups. RESULTS: With regard to the first phase of the disease, TSH and fT4 showed no significant differences between the control and laser groups accordingly (P > 0.05). Likewise, in the second phase of ROP, there was no significant difference between the control and laser groups with respect to TSH and fT4 levels (P > 0.05). CONCLUSION: We found that between the study groups, the levels of thyroid function tests did not have any significant differences, either in the first or the second phases of ROP which are the principal pathophysiology of the disease. Therefore, it was concluded that thyroid hormone values were not informative markers in the course of the disease in preterm babies at risk of developing ROP.

Is it worth screening for vancomycin-resistant Enterococcus faecium colonization?: Financial burden of screening in a developing country.

BACKGROUND: The screening of critically ill patients at high risk of vancomycin resistant enterococci (VRE) colonization, to detect and isolate colonized patients, is recommended to prevent and control the transmission of VRE. Screening asymptomatic carriers brings financial burden for institutions. In this study, we performed risk analysis for VRE colonization and determined the financial burden of screening in a middle-income country, Turkey. METHODS: We retrospectively analyzed the VRE surveillance data from a pediatric hospital between 2010 and 2014. A case-control study was conducted to identify the risk factors of colonization. Total cost of VRE screening and additional costs for a VRE colonized patient (including active surveillance cultures and contact isolation) were calculated. RESULTS: During the 4-year period, 6,372 patients were screened for perirectal VRE colonization. The rate of culture-positive specimens among all patients screened was 239 (3.75%). The rate of VRE infection was 0.04% (n = 3) among all patients screened. Length of hospital stay, malignancy, and being transferred from another institution were independently associated risk factors for colonization. Annual estimated costs for the laboratory were projected as $19,074 (76,295/4) for all patients screened. Cost of contact isolation for each patient colonized in a ward and an intensive care unit was $270 and $718, respectively. CONCLUSIONS: In developing countries, institutions should identify their own high-risk patients; screening priorities should be based on prevalence of infection and hospital financial resources.

Aortic intima-media thickness in nicotine-exposed rat pups during gestation and lactation period.

There have been several studies confirming an association between maternal smoking during pregnancy and low birth weight. The detrimental effect of nicotine exposure beginning in fetal life continues during lactation, in infancy and in the early childhood period. In our previous studies, we found increased aortic intima-media thickness (aIMT) as a preatherosclerotic lesion in neonates with intrauterine growth restriction and in infants of smoking mothers. We aimed to evaluate histopathologically the effect of nicotine exposure during pregnancy and lactation period on fetal growth and aIMT at postnatal 45 days of age (end of the mid-adolescent period) in rat pups living in the same conditions. Gravid rats were assigned into three groups. In nicotine A, pregnant rats received 6 mg/kg/day nicotine intraperitoneally during pregnancy from 1 to 21 days of gestation and lactation (until postnatal day 21). Nicotine B received 3 mg/kg/day nicotine for the same period. Control pregnant rats received only saline intraperitoneally. Abdominal aIMT was studied histopathologically at postnatal 45 days of age. Nicotine exposure resulted in decreased birth weight and pregnancy weight gain. The mean aIMT values of the rat pups exposed to nicotine in both nicotine A and B groups were higher than those of the control group (103.78 ± 21.33 µm, 99.11 ± 30.12 µm, and 62.56 ± 7.18 µm, respectively). In conclusion, the detrimental effect on birth weight of nicotine exposure that began in fetal life is dose dependent. Nicotine exposure during intrauterine life and the lactation period causes increased aIMT in rat pups.

Fetal-neonatal ovarian cysts--their monitoring and management: retrospective evaluation of 20 cases and review of the literature.

OBJECTIVE: Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cysts may lead to severe complications. A standard guide to management, treatment and follow-up of NOC is not yet available. The aim of this study was to evaluate retrospectively the records of NOC patients from two medical centers. METHODS: A total of 20 newborns with NOC were included in the study. The size and localization of the cyst, the age, the signs and symptoms at presentation, and the possible maternal and fetal-neonatal etiologic factors were recorded. Follow-up procedures and treatment modalities were evaluated. RESULTS: The mean age at diagnosis was 34 gestational weeks. The cysts (mean size 53±15 mm) were predominantly in the right ovary (75%) and were evaluated as large cysts in 16 (80%) of the patients. In 5 of the patients with large cysts and in 1 of the 4 patients with small cysts, the cysts were evaluated as complex cysts. Torsion of the ovary was detected in five (25%) cases and these cases were treated surgically. Patients with simple cysts were closely followed by ultrasonography until the cysts disappeared. CONCLUSION: To date, there is no precise guide for the monitoring and treatment of NOCs. Surgical treatment should always be performed in a way to protect the ovaries and to ensure future fertility. In our NOC series, it has been possible to apply a non-invasive follow-up program and minimally invasive surgical procedures.

Cutis laxa associated with central hypothyroidism owing to isolated thyrotropin deficiency in a newborn.

Cutis laxa is an uncommon entity characterized by laxity of the skin, which hangs in loose folds, producing an appearance of premature aging. It can be subdivided into congenital and acquired forms. Genetic forms of cutis laxa include at least three forms of recessive disease, an X-linked form also termed occipital horn syndrome and an autosomal dominant form. Isolated pituitary hormone deficiency can be induced by many causes including mechanical destruction of the hypothalamo-pituitary axis, neoplasm, inflammation, and injury and genetic defects of pituitary hormone production and secretion. Isolated-thyrotropin deficiency has been considered to be a rare disease. We report a newborn with autosomal recessive form of congenital cutis laxa, who had congenital hypothyroidism owing to isolated thyrotropin deficiency. To the best of our knowledge, this is the first instance of this association to be reported in the literature.

Influence of maternal nicotine exposure on neonatal rat bone: protective effect of pentoxifylline.

Limited research in young adults and immature animals suggests a detrimental effect of tobacco on bone during growth. The aim of this study was to determine the adverse effects of maternal nicotine exposure during pregnancy and lactation on neonatal rat bone development, and to determine a protective effect of pentoxifylline (PTX). Gravid rats were assigned into four groups, one control (group I) and three experimental (groups II, III, and IV). In group II, pregnant rats received 3 mg/kg/day nicotine alone, subcutaneously, until 21 days postnatal. In group III, pregnant rats received nicotine (3 mg/kg/day) and PTX (60 mg/kg/day). In group IV, pregnant rats received PTX alone (60 mg/kg/day). Whole body mineral density (BMD), content (BMC), area (BA), and histopathologic and morphologic findings of the femur were determined at 21 days of age. The study revealed that nicotine exposure (group II) decreased birth weight, pregnancy weight gain, and length of femur compared with other groups (P < 0.01). Birth weight was higher in groups III (PTX + nicotine) and IV (PTX) than in group II (nicotine). Body weight at 21 days of age was higher (P = 0.009) in the PTX alone group (group IV) compared with the other groups. BMD was higher (P < 0.001) in the PTX-treated groups (group III and IV) compared with other groups. In addition, there were more apoptotic chondrocytes in the hypertrophic zone of rats exposed to nicotine alone (group II) compared with the other groups (P < 0.001). In conclusion, maternal nicotine exposure resulted in decreased birth weight, pregnancy weight gain, and bone lengthening, and increased apoptosis. Pentoxifylline supplementation was found to prevent the adverse effects of maternal nicotine exposure on BMD and birth weight.

Influence of maternal smoking on neonatal aortic intima-media thickness, serum IGF-I and IGFBP-3 levels.

Epidemiological studies have reported associations between a range of cardiovascular risk factors such as smoking and intima-media thickness (IMT). Some reports indicate that the maternal tobacco smoking causes disturbances of the endocrine status of the foetus. There are several potential mechanisms by which insulin-like growth factor I (IGF-I) could modify atherosclerotic processes either locally or in a systemic manner. The aim of this study was to investigate the influence of maternal smoking on neonatal aortic IMT (aIMT), serum IGF-I and IGF-binding protein-3 (IGFBP-3) levels. Aortic intima-media thickness was measured in 28 neonates whose mothers smoked during the pregnancy and 28 control neonates. Mean and weight-adjusted aIMT were significantly greater in the neonates whose mothers smoked (0.455 +/- 0.009 mm and 0.151 +/- 0.005 mm/kg, respectively) than in controls (0.403 +/- 0.029 mm and 0.118 +/- 0.014 mm/kg, respectively). Birth-weight of newborns whose mothers smoked was less than that of the controls. The decreases in serum IGF-I and IGFBP-3 observed in the infants whose mothers smoked were non-significant. Mean aIMT was negatively associated with birth-weight and IGF-I level. In conclusion, neonates whose mothers smoked have significantly increased aIMT. It might play a role in the pathogenesis of atherosclerosis in adult life.

A case of Langerhans cell histiocytosis presented with pneumothorax.

Pneumothorax (PTX) is an unusual complication of Langerhans cell histiocytosis (LCH) in childhood. Spontaneous PTX is rare in childhood, and it is very rare in infancy. There are no specific recommendations for the treatment of PTX from LCH described in the literature. We are presenting a 19-month-old boy, who suddenly developed left-sided PTX with infiltrations in both lungs. He presented with PTX and skin lesions. He had a prolonged cardiac arrest, and although resuscitation was successful he required continuing ventilatory support (intermittent positive-pressure ventilation). Because he suddenly developed right-sided PTX and died on the second day of the admission, his LCH diagnosis was made only postmortem. So, he did not receive chemotherapy. It is likely that intermittent positive-pressure ventilation during the operation induced the development of much more multiple lung bullae, which subsequently ruptured, and/or it facilitated the development of the right-sided PTX. The patients with PTX and skin lesions, including babies, most likely have LCH and specific chemotherapy should be started in emergency, even before the final diagnosis is achieved.

Effect of allopurinol supplementation on nitric oxide levels in asphyxiated newborns.

This study aimed to investigate the effect of allopurinol in the management of cerebral hypoxia-ischemia by monitoring nitric oxide levels of serum and cerebrospinal fluid. Sixty asphyxiated infants were divided randomly into two groups. Group I infants (n = 30) received allopurinol (40 mg/kg/day, 3 days) within 2 hours after birth. Group II infants (n = 30) received a placebo. Twenty healthy neonates served as control subjects. Cerebrospinal fluid and serum nitric oxide levels were measured within 0-24 hours and 72-96 hours after birth. Both serum and cerebrospinal fluid concentrations of nitric oxide were higher in severely asphyxiated infants (40.86 +/- 8.97, 17.3 +/- 3.63 micromol/L, respectively) but lower in mildly asphyxiated infants (25.85 +/- 3.57, 5.70 +/- 2.56 micromol/L, respectively) than in moderately asphyxiated infants (35.86 +/- 5.38, 11.06 +/- 3.37 micromol/L, respectively) within the first 0-24 hours after birth. Serum nitric oxide levels in control subjects were lower than those of moderately and severely asphyxiated infants. Serum nitric oxide levels of Group I infants within 72-96 hours after birth decreased significantly from their corresponding levels within 0-24 hours after birth. The asphyxiated newborns treated with allopurinol had better neurologic and neurodevelopmental outcome at 12 or more months of age.

Influence of maternal nicotine exposure on neonatal rat bone: protective effect of ascorbic acid.

Limited research in young adults and immature animals suggests a detrimental effect of tobacco on bone during growth. We aimed to determine the adverse effects of maternal nicotine exposure during pregnancy and lactation on neonatal rat bone development, and to determine a protective effect of ascorbic acid. Gravid rats were assigned into three groups: two experimental and one control (group I). In the first experimental group (group II), pregnant rats received 3 mg/kg/d nicotine subcutaneously during pregnancy from 1 to 21 days of gestation and lactation (until postnatal day 21). The second experimental group (group III) received nicotine and ascorbic acid (1 mg/kg body mass/d). Whole body mineral density (BMD), content (BMC), and area (BA) were measured on postnatal day 21. Histopathologic and morphologic findings of the femur were obtained. Maternal nicotine exposure decreased the body weight of the rat at the birth and postnatal day 21. The values of BMD, BA, and BMC of the groups were similar to each other. Width of the epiphyseal plate and the hypertrophic zone were higher in group III but lower in group II than in group I. Number of apoptotic chondrocytes was significantly increased in group II. The length of femur was higher in group I but lower in group II than in group III. Maternal nicotine exposure during gestation and lactation resulted in decreased body weight and bone lengthening. Ascorbic acid supplementation was found to prevent the adverse effects of maternal nicotine exposure on the growth plate.

Perinatal expression of HSP70 and VEGF in neonatal rat lung vessels exposed to nicotine during gestation.

We assessed the influence of maternal nicotine exposure during gestation on perinatal expression of HSP70 and VEGF in rat lung parenchyma and lung vessels. Adult white Sprague-Dawley virgin rats were mated with adult male rats over 2 days, with two females for every male. After confirming pregnancy, 30 gravid rats (dams) were then randomly assigned to two equal groups (one experimental and one control; n=15 in each). Experimental dams were treated with subcutaneus (s.c.) (-)-nicotine tartrate, 3 mg/kg body weight/day, during pregnancy from gestational days 9 through 21. After sacrifice, lungs were removed en bloc and formalin-fixed, and paraffinembedded tissue sections were evaluated by immunohistochemistry using a three-step streptavidin-biotin-peroxidase method with monoclonal antibodies directed against HSP70 or VEGF. HSP70 and VEGF expression was increased in the vascular smooth muscle cells of the experimental group (t1) compared to the control group (t(2))t(1)=7.593, t(2)=4.666, p<0.05). The number of bronchioles that stained positively with HSP70 was higher in the nicotineexposed group than in the control group (t(1)=9.274, t(2)=6.956, p<0.05). In conclusion, gestational nicotine exposure increased the expression of VEGF and HSP70 in rat lung parenchyma, especially in the airway epithelium and vascular smooth muscle cells. In vascular smooth muscle cells, these molecules may contribute to nicotine-related hypoxic pulmonary hypertension.

The effect of subcutaneously injected nicotine on achilles tendon healing in rabbits.

The objective of this study was to evaluate the effect of subcutaneously injected nicotine on transversely transected and sutured achilles tendon healing in an experimental rabbit model. Adult New Zealand rabbits (n=22) weighting 3,000-3,500 g were used in this experimental study. Rabbits were randomly divided into two groups. Achilles tendon was transversely incised and repaired in all animals. In the experiment group subcutaneous injection of Nicotine tartrate 3 mg/kg/day was done. In the control group Serum physiologic injection was done at the same dosage. The injections were made three times a day in equal dosages. Nicotine and SF injections were made until the end of the 8-week, and then all animals were euthanized. Both light microscopic and electron microscopic evaluations were made on 14 animals. In N group light microscopic evaluation showed a visible gap in repair site. The total tendon score represented in N group was less than in SF group. The statistical analysis of the groups was significantly different for total tendon scores (P=0.002). Beside this electron microscopic examination showed inactive and degenerated fibroblasts and irregular collagen fibrils around them as well as collagen synthesis interruption in N group. Biomechanical evaluation was made on eight animals. The average tensile strength values in Group N (139.47+/-44.55 N) were significantly lower than those in Group SF (265.9+/-39.01 N) (z=2.309, P=0.029). Nicotine is the major chemical component common to all cigarettes and previously has been shown to affect wound and fracture healing adversely. The results of this study show that nicotine impairs achilles tendon healing after a surgical repair.

Raine syndrome associated with cytomegalovirus infection.

Intracranial calcification, periosteal proliferation and microcephaly, which represent the clinical features of the congenital cytomegalovirus infection, can also be seen in a rare disorder named Raine syndrome. This clinical entity has been reported in eight families so far. Here, we report a new patient with clinical features of the Raine syndrome associated with cytomegalovirus infection. Although this may be a chance association only, the condition raised the question of whether early intrauterine CMV infection may contribute to the Raine phenotype.

Pancytopenia, a rare hematologic manifestation of brucellosis in children.

The records of 54 children with brucellosis were evaluated retrospectively. Among them, eight patients (14.8%) with pancytopenia were identified in a 7-year period between 1996 and 2003. Six of the eight patients with pancytopenia had Brucella melitensis isolated from blood cultures, and all eight patients had Brucella agglutination titers of at least 1:320. Agglutination test titers did not correlate with the degree of pancytopenia. Fever was the most common manifestation, followed by malaise, anorexia, sweating, weight loss, and gastrointestinal symptoms. Most patients had hepatosplenomegaly, and bone marrow aspiration specimens showed hyper-cellularity or normocellularity. Hemophagocytosis (3 patients) and histiocytic hyperplasia (4 patients) were observed in bone marrow examinations of eight patients, but bone marrow aplasia and granulomas were not detected. All children recovered completely; the pancytopenia was transient and resolved after the antibiotic treatment of Brucella infection. Brucellosis should be considered as a possible diagnosis among patients with pancytopenia.