Barttin mutations in antenatal Bartter syndrome with sensorineural deafness.
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Bartter Syndrome↗
Biomedical subjects
Nejat Narli
Publications and source records attributed to Nejat Narli.
The effect of human growth hormone on superoxide dismutase activity, glutathione and malondialdehyde levels of hypoxic-ischemic newborn rat brain.
OBJECTIVES: We investigated the effect of human growth hormone (GH) on newborn rat brain superoxide dismutase, glutathione and malondialdehyde (MDA) levels in hypoxic-ischemic (H-I) newborn rats. METHODS: Fourty-eight 7 days old newborn rats were randomized to a healthy (n: 15), H-I (n: 18) and GH administered H-I (GH-H-I, n: 15) group. Permanent, left common carotid ligation was performed in the H-I groups. In the GH-H-I group, 50 mg/kg human GH (Norditropin Simplex, Novo Nordisk A/S) was administered subcutaneously just before carotid artery ligation. Two hours after ligation, rats were subjected to 2 h of hypoxemia and then were decapitated. Right and left cerebral hemispheres (CHs) and cerebellum-brain stem (C-BS) were separated. RESULTS: Glutathione levels of each region were not statistically different from each other in and between the groups. Superoxide dismutase levels were higher in C-BSs compared to CHs (for each comparison p < 0.01). CHs and C-BS MDA levels were similar in the control and H-I groups but MDA levels of both CHs of the GH-H-I group were significantly higher than the levels of the H-I group (p = 0.01; p = 0.024, respectively). Left CH MDA level of GH-H-I group was higher compared to left CH MDA of the control group (p = 0.045) while there was no difference between right CHs. In the GH-H-I group, left CH MDA level was higher than the C-BS (p = 0.03). MDA levels of the C-BSs did not differ between the groups (p > 0.05). CONCLUSION: Although we have not evaluated the effect of GH histopathologically, increased lipid peroxidation especially in the H-I (left) hemisphere of the GH treated rats might suggest that GH treatment may be harmful in H-I encephalopathy.
Animals↗
Ultrasonographic measurement of subarachnoid space in normal term newborns.
This study was conducted in order to assess the normal range of subarachnoid space width in healthy term newborns. A total of 230 healthy newborns were evaluated within the first 28 days of life. Measurements were correlated with body weight, height and head circumference. Mean measurements for falx-cortex and craniocortical widths and the correlations are given in tables. Subarachnoid space widths increased as weight, height and head circumference increased and the correlation was statistically significant.
Body Height↗
Neuroprotective effects of indomethacin and aminoguanidine in the newborn rats with hypoxic-ischemic cerebral injury.
Nitric oxide (NO) and prostaglandins (PG) play important roles in delayed mechanisms of brain injury. While NO disrupts oxidative metabolism, prostaglandins are responsible for free radical attack in reperfusion interval. Relatively little is known about neuroprotection exerted at this level in perinatal models. The aim of this study was to investigate the effect of indomethacin and aminoguanidine on endogenous inducible nitric oxide synthase (iNOS) biosynthesis and neuroprotection in the newborn rats with hypoxic ischemic cerebral injury.Seven-day old rat pups with model of hypoxic-ischemic cerebral injury were randomly divided into four study groups. Group C (n=18; served as a control) pups were given physiologic saline (SF). Group I (n=18) pups were treated with indomethacin at a dose of 0,2 mg/kg per 12 h. Group A (n=20) pups were treated with aminoguanidine at a dose of 300 mg/kg per 8 h. Administration of drugs and SF were begun half an hour after hypoxic-ischemic insult in these groups. Group I+A (n=18) pups were treated with indomethacin at a single dose of 0.2 mg/kg 1 h before hypoxia-ischemia followed by aminoguanidine as in group A. Drugs and SF were administered for three consecutive days. On the tenth day, rat pups were decapitated and coronal sections at the level of dorsal hippocampal region of brains were evaluated. In the histopathologic examination; the mean infarcted area in group I+A was significantly lower than the control group (P<0.05). Although there was no statistically significant difference between treatment groups in terms of iNOS expression, the risk of iNOS expression was 7 times less for group I (CI: 1.6-30.8, P=0.01), 19.8 times less for group A (CI: 3.8-104, P=0.001) and 12.3 times less for group I+A (CI: 2.5-59, P=0.002) compared to group C. In conclusion, only indomethacin administration before hypoxic ischemia and followed by aminoguanidine was more effective to reduce infarct area, but we did not find any difference between treatment groups and control group for iNOS expression. So we suggest that this neuroprotection may not be related to depression of iNOS expression.
Animals↗
Pyruvate kinase activity in cerebral hemispheres and cerebellum-brainstem of normal and hypoxic-ischemic newborn rats.
Energy metabolism is affected in hypoxia-ischemia. Changes in the tissue concentrations of the high-energy phosphate reserves occur early during the course of the metabolic insult and with concurrent increases in cellular ADP and AMP leading glycolysis. It has been shown that enzymes of glycolysis tend to be regulated in hypoxia and ischemia. In this study we determined pyruvate kinase (PK) activity, one of the main enzymes in glycolysis, in brain tissues of healthy (n = 15) and hypoxic-ischemic (n = 18) 7-day-old newborn rats. Left common carotid artery was ligated in the hypoxic-ischemic group and after 2 hours rats were exposed to hypoxia in a chamber at 34-36 degrees C with 8% oxygen in nitrogen. The rats were decapitated after 2 hours of hypoxia and right and left cerebral hemispheres (CH) and cerebellum-brain stem (C-BS) were removed. Pyruvate kinase activity was significantly higher in C-BSs than CHs in both groups (p < 0.00005). There was no significant difference in enzyme activities of either CHs or C-BS of hypoxic-ischemic group compared to control healthy group (p > 0.05). In conclusion, brain pyruvate kinase activity did not change in hypoxia-ischemia and suggests that PK of brain differs from other tissues where it usually increases in hypoxiaischemia.
Animals↗
Serum insulin-like growth factor 1 and growth hormone levels of hypoxic-ischemic newborns.
A number of growth factors, their binding proteins, and their receptors have been shown to be induced in the hypoxic-ischemic (HI) brain. In this prospective study, we aimed at determining the levels of insulin-like growth factor 1 (IGF-1), growth hormone (GH), and cortisol in HI babies and at identifying whether they differ from the levels of control infants. The serum IGF-1 levels were measured after the first 12-24 h of life, and the measurements were repeated on the 5th and 10th days of life for babies with HI encephalopathy (n = 18) and on the 10th day of life for controls (n = 19). Blood samples for measurement of cortisol and GH from both HI and control groups were collected after the first 12-24 h of life. There were 11 babies in the mild-to-moderate (stages I and II) group and 7 babies in the severe (stage III) group according to Sarnat and Sarnat. The IGF-1 levels of the HI group measured after 12-24 h [78.5 +/- 27.9 (range 9-123.4) ng/ml] and on the 10th day [72.2 +/- 36.8 (range 29.7-159.2) ng/ml] of life were statistically significantly lower than the IGF-1 levels of the control group [121.5 +/- 50.4 (range 74.4-280.5) ng/ml and 133.1 +/- 34.4 (range 65.9-202) ng/ml, respectively] (p = 0.002 and p = 0.001, respectively). But there was no statistically significant difference between mild-to-moderate HI group and severe HI group in terms of IGF-1 levels after 12-24 h and 5 and 10 days of life (p > 0.05). Also there was no statistically significant difference in IGF-1 values after the first 12-24 h and after 10 days of life between HI subjects who died or survived (p > 0.05). The GH levels of the HI group after the first 12-24 h of life [34.6 +/- 32.3 (range 0.1-120) mIU/l] were statistically significantly higher than those in the control group [10.4 +/- 4.5 (range 3.7-16.9) mIU/l] (p = 0.005). There was no statistically significant difference in the serum cortisol levels between HI and control groups after the first 12-24 h of life [18.7 +/- 17.0 (range 1.6-65.1) microg/dl vs. 10.8 +/- 5.4 (range 3.0-23.2) microg/dl] (p > 0.05). No statistically significant correlation was found between IGF-1 levels and GH and cortisol levels of the HI encephalopathy group [r = -0.113 (p > 0.05) and r = 0.108 (p > 0.05), respectively]. In conclusion, this study showed decreased levels of serum IGF-1 and increased levels of GH which may be secondary to serum IGF-1 influx from the circulation to the brain as a protective mechanism or may be due to some cytokines which alter the GH/IGF axis, inhibit the action of IGF-1, and stimulate IGF-binding protein 1.
Blood Glucose↗
Intraventricular streptokinase for the treatment of posthaemorrhagic hydrocephalus of preterm.
Posthaemorrhagic hydrocephalus following intraventricular haemorrhage is still one of the most serious complications of premature birth. Small premature babies are not suitable for shunt surgery because of high cerebrospinal fluid protein and risk of obstruction. For this reason there is a great need for alternative approaches for treatment of posthaemorrhagic hydrocephalus. The objective of this study was to investigate if intraventricular streptokinase treatment reduces the need for ventriculoperitoneal shunt in posthaemorrhagic hydrocephalus. A case-control trial was carried out in 12 premature babies with posthaemorrhagic hydrocephalus. Six of them were treated with intraventricular streptokinase and 6 premature babies were in the control group. While 5 babies in the study group needed ventriculoperitoneal shunt, 3 of the control patients needed shunt surgery. There were no rebleeding, ventriculitis or meningitis in either groups. In conclusion on the basis of our results we do not recommend routine use of intraventricular streptokinase in posthaemorrhagic hydrocephalus.
Apgar Score↗
Sonographic measurements of the liver, spleen and kidney dimensions in the healthy term and preterm newborns.
This study was conducted in order to assess normal liver, spleen and kidney dimensions in premature and term newborns and determine the acceptable range. A total of 253 (99 preterm and 154 term) healthy newborns were evaluated within the first week of life by sonography. Gestational age ranged from 24 to 41 weeks, weight ranged from 638 to 4800 g. Measurements were compared with gestational age, weight and height of the infants. Normal ranges for kidney, liver and spleen measurements according to gestational age and weight were obtained. We found that weight showed the best correlation with any one of the mentioned organ dimensions.
Body Weight↗
The activity and kinetics of pyruvate kinase in hypoxic newborns.
Pyruvate kinase (PK) plays a key role in erythrocytes, which obtain most of their energy from glycolysis. This study investigated erythrocyte energy metabolism in hypoxic newborns, measuring pyruvate kinase activity, kinetic, and ATP levels in hypoxia. Forty-nine babies who had cord pH value lower than 7.2 and Apgar scores lower than 7 in the first minute were accepted as the hypoxic group, and 48 babies who had cord pH value higher than 7.2 and an Apgar score higher than 7 in the first minute were taken as controls. The erythrocyte mean PK activity was found to be lower (16.9+/-8.5 [5.8-47.9] EU/gHb) in the hypoxic group than the control group (21.3+/-10.9 [3.9-44.3] EU/gHb) (p<.05). The mean ATP value of hypoxic group was higher (19.2+/-11.3 [3.9-37.6] mM) compared to control group (13.8+/-7.16 [3.9-28.7] mM). In the kinetic study, with different ADP concentrations in the control group, the substrate amount (Km) that is needed to reach the half-maximum of enzyme activity (Vmax=27.7 Eu/gHb) was found to be 2.70 mM, but it was 1.47 mM to reach Vmax (22.7 Eu/gHb) in the hypoxic group. Vmax was 41.67 Eu/gHb and Km was 8.33 mM in the control group at different PEP concentrations, whereas Vmax was 21.7 Eu/gHb and Km was 0.89 mM in the hypoxic group. Increase in the ATP level while ePK activity decreases, suggesting that glycolysis increases in hypoxia. In the kinetic study, the substrate amount needed for reaching the half-maximum of enzyme activity was less in the hypoxic group, probably suggests that pyruvate kinase increases glycolysis by increasing its affinity to the substrates. In this way, erythrocytes may gain the energy required for oxygen delivery to tissues and maintaining ion gradient. This arrangement possibly proceeds from sygmoidal structure of pyruvate kinase.
Adenosine Diphosphate↗
A newborn infant with generalized glutathione synthetase deficiency.
Pyroglutamic aciduria (5-oxoprolinuria) is a rare autosomal recessive disorder caused by either glutathione synthetase deficiency (GSSD) or 5-oxoprolinase deficiency. The severe form of the disease, generalized GSSD, is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. We report a female infant who had a severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia. High level of 5-oxoproline was detected in her urine and a diagnosis of generalized GSSD was made. She died of severe metabolic acidosis and sepsis at the age of six weeks.
Acidosis↗
Is oral indomethacin effective in treatment of preterm infants with patent ductus arteriosus?
Twenty-one preterm infants (with a mean gestational age and birth weight of 29.3 weeks and 1288.6 g) and nine pretem infants (with a mean gestational age and birth weight of 29.6 weeks and 1153.1 g) were treated with an enteral preparation of indomethacin and with intravenous indomethacin, respectively, for the closure of hemodynamically significant ductus arteriosus. The patients received three doses of either oral indomethacin capsule (Endol, Deva, Turkey) or intravenous indomethacin (Confortid, Dumex GmBH, Germany) in a dose of 0.2 mg/kg at 12-hour intervals. The ductus was closed in 17 (81%) and 7 (77%) of the babies in the orally and intravenously treated groups, respectively (p > 0.05). There was no significant difference in blood urea nitrogen, creatinine levels or thrombocyte counts in either group before and after treatment with indomethacin (p > 0.05). No side effect was reported in the oral indomethacin group. Oral indomethacin may be an alternative to the intravenous preparation in developing countries if the intravenous form is not available or not affordable.
Administration, Oral↗
A neonate with malignant ectomesenchymoma.
Malignant ectomesenchymoma is a rare tumor reported in head-neck, abdomen and perineal regions. It consists of mesenchymal and neuroectodermal elements. In this tumor group, neoplastic cells are differentiated into neuronal cells. It also has at least one malignant mesenchymal element, generally rhabdomyosarcoma. In this report we present a neonate with ectomesenchymoma.
Facial Neoplasms↗