RESUMEN
BACKGROUND Neonatal hypoxic-ischemic encephalopathy (HIE) is a significant cause of perinatal and postnatal morbidity and mortality worldwide. Catalase (CAT) activity detection is used to determine levels of inflammation and oxidative stress. Glutathione (GSH) is the most critical non-enzymatic endogenous antioxidant. Lipid peroxidation levels marked after hypoxia can be detected based on the level of malondialdehyde (MDA). Ischemia-modified albumin (IMA) is considered a biomarker for cardiac ischemia and is known to increase in the liver, brain, and kidney in states of insufficient oxygenation. We aimed to explain the results and relations between the oxidant and antioxidants to detail oxidant-antioxidant balance and cellular mechanisms. MATERIAL AND METHODS Serum levels of IMA and MDA, as an oxidative stress marker, and CAT and GSH, as antioxidant enzymes, were measured in first blood samples of 59 neonates diagnosed with HIE, with pH <7, base excess >12, and APGAR scores. RESULTS Neonates who were ≥37 weeks of gestation and had hypoxia were included. Compared with healthy newborns (n=32), CAT was statistically significantly lower in the hypoxia group (P=0.0001), while MDA serum levels were significantly higher in neonates with hypoxia (P=0.01). There was no difference between hypoxic and healthy neonates in GSH and IMA measurements (P=0.054, P=0.19 respectively). CONCLUSIONS HIE pathophysiology involves oxidative stress and mitochondrial energy production failure. Explaining the pathways between oxidant-antioxidant balance and cell death, which explains the pathophysiology of HIE, is essential to develop treatment strategies that will minimize the effects of oxygen deprivation on other body organs, especially the brain.
Asunto(s)
Antioxidantes , Biomarcadores , Hipoxia-Isquemia Encefálica , Malondialdehído , Estrés Oxidativo , Humanos , Recién Nacido , Hipoxia-Isquemia Encefálica/metabolismo , Hipoxia-Isquemia Encefálica/sangre , Hipoxia-Isquemia Encefálica/fisiopatología , Biomarcadores/sangre , Biomarcadores/metabolismo , Antioxidantes/metabolismo , Femenino , Masculino , Malondialdehído/sangre , Malondialdehído/metabolismo , Glutatión/sangre , Glutatión/metabolismo , Albúmina Sérica Humana/metabolismo , Catalasa/sangre , Catalasa/metabolismo , Peroxidación de LípidoRESUMEN
Gaucher disease type 2 is the most progressive and the rarest form of Gaucher disease, defined as the acute neuronopathic type. We presented two GD2 patients who died before three months of age due to severe septicemia, respiratory and liver failure. One was homozygous for a novel GBA variant c.590 T > A (p.197 K), and the second homozygous for the known GBA mutation c.1505G > A (p.R502H). Ichthyosis, hydrops fetalis, apnea, myoclonic seizures, and hepatosplenomegaly occurred in both patients, but hypertrophic cardiomyopathy was observed only in the second and unilateral cataract in the first patient. Due to the disease's early and rapid neurological progression, we did not administer ERT to our patients. It is strongly believed that early diagnosis is essential, and prenatal diagnosis makes genetic counselling possible for future pregnancies.
Asunto(s)
Enfermedad de Gaucher , Femenino , Enfermedad de Gaucher/complicaciones , Enfermedad de Gaucher/diagnóstico , Enfermedad de Gaucher/genética , Homocigoto , Humanos , Hidropesía Fetal/etiología , Hidropesía Fetal/genética , Mutación/genética , EmbarazoRESUMEN
Our aim was to determine characteristics of children with chronic critical illness (CCI) admitted to the pediatric intensive care unit (PICU) of a tertiary care children's hospital in Turkey. The current study was a multicenter retrospective cohort study that was done from 2014 to 2017. It involved three university hospitals PICUs in which multiple criteria were set to identify pediatric CCIs. Pediatric patients staying in the ICU for at least 14 days and having at least one additional criterion, including prolonged mechanical ventilation, tracheostomy, sepsis, severe wound (burn) or trauma, encephalopathy, traumatic brain injury, status epilepticus, being postoperative, and neuromuscular disease, was accepted as CCI. In order to identify the newborn as a chronic critical patient, a stay in the intensive care unit for at least 30 days in addition to prematurity was required. Eight hundred eighty seven (11.14%) of the patients who were admitted to the PICU met the definition of CCI and 775 of them (87.3%) were discharged to their home. Of CCI patients, 289 (32.6%) were premature and 678 (76.4%) had prolonged mechanical ventilation. The total cost values for 2017 were statistically higher than the other years. As the length of ICU stay increased, the costs also increased. Interestingly, high incidence rates were observed for PCCI in our hospitals and these patients occupied 38.01% of the intensive care bed capacity. In conclusion, we observed that prematurity and prolonged mechanical ventilation increase the length of ICU stay, which also increased the costs. More work is needed to better understand PCCI.
Asunto(s)
Enfermedad Crítica/epidemiología , Adolescente , Enfermedades del Sistema Nervioso Central/epidemiología , Enfermedades del Sistema Nervioso Central/patología , Niño , Preescolar , Enfermedad Crítica/economía , Enfermedad Crítica/mortalidad , Femenino , Mortalidad Hospitalaria , Humanos , Incidencia , Lactante , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico , Tiempo de Internación , Masculino , Nacimiento Prematuro , Modelos de Riesgos Proporcionales , Respiración Artificial , Estudios Retrospectivos , Factores de Riesgo , Sepsis/epidemiología , Sepsis/patología , TurquíaRESUMEN
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.
Asunto(s)
Antibacterianos/farmacología , Infecciones Relacionadas con Catéteres/prevención & control , Defectos del Tubo Neural/cirugía , Rifampin/farmacología , Sepsis/prevención & control , Infección de la Herida Quirúrgica/prevención & control , Infecciones Urinarias/prevención & control , Administración Tópica , Antibacterianos/administración & dosificación , Cefotaxima , Femenino , Humanos , Recién Nacido , Enfermedades del Recién Nacido , Infecciones/tratamiento farmacológico , Infecciones/etiología , Masculino , Defectos del Tubo Neural/complicaciones , Paraplejía/etiología , Rifampin/administración & dosificación , Resultado del Tratamiento , Derivación Ventriculoperitoneal/efectos adversosRESUMEN
INTRODUCTION AND PURPOSE: The Urinary Neutrophil-gelatinase associated lipocalin (NGAL) levels which are a biomarker for early diagnosis of kidney damage that may develop in patients with Polycystic Ovary Syndrome (PCOS) were investigated in the study. MATERIAL AND METHODS: The 30 patients diagnosed with Polycystic Ovarian Syndrome between the ages of 13 and 18 who applied to the Yuzuncu Yil University General Children's Outpatient Clinic were included in the PCOS group and 30 healthy adolescents without any known acute or chronic illness and drug use were included in the control group. FINDINGS: Urine NGAL value was 842.204 ± 21.561 in PCOS group and 775.379 ± 23.98 in control group. NGAL level in PCOS group was statistically significantly higher than control group (p: .045). When we examine the relationship between dyslipidemia and PCOS; While dyslipidemia was positive in 10 (33.7%) patients in the PCOS group, it was negative in 20 (66.7%) patients. While 1 patient had dyslipidemia, 29 patients did not have dyslipidemia in the control group. A significant relationship was found between dyslipidemia and PCOS (p: .005). CONCLUSION: We found that subclinical kidney dysfunction started in early stage patients in PCOS in our study. The urine NGAL level was thought to increase in response to increased oxidative stress in PCOS. We found no relationship between, insulin resistance and urea, BUN, creatinine and NGAL levels. However, we found a negative correlation between NGAL level and LDL. In addition, dyslipidemia, insulin resistance and ALT elevation were detected in the PCOS group.
Asunto(s)
Lipocalina 2/orina , Síndrome del Ovario Poliquístico/complicaciones , Insuficiencia Renal/etiología , Adolescente , Estudios de Casos y Controles , Femenino , Humanos , Síndrome del Ovario Poliquístico/orinaRESUMEN
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.
Asunto(s)
Sangre Fetal/química , Metales Pesados/sangre , Defectos del Tubo Neural/inducido químicamente , Oligoelementos/sangre , Adulto , Estudios de Casos y Controles , Femenino , Edad Gestacional , Humanos , Recién Nacido , Embarazo , Factores de Riesgo , Adulto JovenAsunto(s)
Antagonistas Adrenérgicos alfa/envenenamiento , Antidepresivos Tricíclicos/envenenamiento , Antagonistas de los Receptores Histamínicos H1/envenenamiento , Letargia/inducido químicamente , Mianserina/análogos & derivados , Carbón Orgánico/uso terapéutico , Preescolar , Femenino , Humanos , Mianserina/envenenamiento , Mirtazapina , Resultado del Tratamiento , TurquíaRESUMEN
AIM: To evaluate the clinical findings, risk factors, therapy and outcome in 946 children with nutritional rickets. PATIENTS AND METHODS: This retrospective study included a review of medical records of patients with nutritional rickets between March 2004 and 2009. Patients who displayed both the biochemical inclusion criteria and the clinical signs/symptoms or radiological signs of rickets were included in the study. RESULTS: The present study included 946 patients aged between 4 months and 15 years. Distribution of the cases showed a density between December and May. The age at diagnosis, showed two peaks and most of the patients were in the age range 0-23 months and 12.0-15 years. In infants and young children, most of the patients had been admitted to the hospital due to infectious diseases. In older children, short stature and obesity were the most common complaints. CONCLUSION: Children aged between 0-23 months and 12.0-15 years were under most risk for nutritional rickets, especially in winter and spring and vitamin D should be given to them as supplementation dose.
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Raquitismo/epidemiología , Raquitismo/etiología , Raquitismo/terapia , Deficiencia de Vitamina D/complicaciones , Adolescente , Niño , Preescolar , Comorbilidad , Femenino , Humanos , Lactante , Masculino , Estado Nutricional/fisiología , Admisión del Paciente/estadística & datos numéricos , Estudios Retrospectivos , Raquitismo/complicaciones , Factores de Riesgo , Resultado del Tratamiento , Turquía/epidemiología , Deficiencia de Vitamina D/epidemiologíaRESUMEN
Tarka® is a combination antihypertensive medication composed of verapamil hydrochloride and trandolapril. A 3.5-year-old female was brought to our hospital due to a sleepy condition 7 hours after an accidental ingestion of six tablets of Tarka® containing 240 mg verapamil hydrochloride and 4 mg trandolapril in each tablet. Five hours after hospitalization, her condition deteriorated and arterial pressure progressively decreased despite the treatment. Finally, a temporary pacemaker was implanted, after which the vital findings began to return to normal values. The pacemaker was removed 13 hours after implantation as normal heart rhythm was observed. There are no reports of intoxication with fixed-dose combination products, especially Tarka®, in young children in the literature. Therefore, we believe that our report can provide an insight on the toxic dose of this drug in younger children. Clinicians should keep in mind that lethargy can be the first symptom of a possible clinical deterioration, even in normotensive and normorhythmic individuals.
Asunto(s)
Indoles/envenenamiento , Verapamilo/envenenamiento , Preescolar , Combinación de Medicamentos , Sobredosis de Droga/diagnóstico , Sobredosis de Droga/terapia , Electrocardiografía , Femenino , Humanos , Indoles/administración & dosificación , Marcapaso Artificial , Resultado del Tratamiento , Verapamilo/administración & dosificaciónRESUMEN
The authors examined clinical outcome and cranial magnetic resonance imaging (MRI) findings in infants with hypoglycemia to determine the effects of hypoglycemia on the developing brain. A total of 110 infants with hypoglycemia were included in the study. Of the patients, 36 were females and 74 were males. The age of the infants was between 1 day and 22 months. Of the 110 infants, 47 were preterm neonates, 40 were term neonates, and 23 were older than 28 days. No difference in serum glucose level was noted between symptomatic and asymptomatic infants. The most common observed abnormal findings were hyperintense lesions, encephalomalacia, and cerebral atrophy. Abnormal MRI findings were found in 4% of preterm infants, in 32.5% of term infants, and in 43.5% of older infants. Abnormal MRI findings were statistically significantly more common in symptomatic infants than in asymptomatic infants. Of the infants, 45.5% of hypoglycemic infants had cerebral palsy and/or cerebral palsy plus epilepsy.
Asunto(s)
Encéfalo/patología , Parálisis Cerebral/etiología , Epilepsia/etiología , Hipoglucemia/complicaciones , Hipoglucemia/patología , Análisis de Varianza , Parálisis Cerebral/patología , Epilepsia/patología , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Resultado del TratamientoRESUMEN
Cerebral venous sinus thrombosis (CVST) is an uncommon disease in childhood. In the largest study carried out so far, the incidence was 0.67 case per 100,000 children per year. A number of etiologies and risk factors have been so far identified for CVST in childhood, including head trauma, local and systemic infectious diseases, malignancies, and autoimmune diseases. Celiac disease (CD) is a disease of the small intestine caused by an immune response to ingested gluten. Epilepsy, bilateral occipital calcification, cerebellar ataxia, degenerative central nervous system disease, peripheric neuropathy, myopathy, and rarely stroke were defined as neurologic disorders. In this presentation, we report 2 cases (16-year-old boy and 2-year-old boy) with CD and CVST. We emphasized that CD can be investigated in patients with CVST even with the absence of gastrointestinal symptoms. Finally, we suggest that algorithm of CVST can be involved in the investigation of CD.
Asunto(s)
Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico , Adolescente , Algoritmos , Enfermedad Celíaca/epidemiología , Preescolar , Humanos , Incidencia , Masculino , Factores de Riesgo , Trombosis de los Senos Intracraneales/epidemiologíaRESUMEN
OBJECTIVE: The aim of this study was to estimate the prevalence of overweight and obesity in school children in Eastern Turkey. METHODS: This study included 9048 school children aged 6-18 years. The subjects were classified as overweight and obese, according to the International Obesity Task Force. RESULTS: We found prevalence of overweight of 11.1% in the studied population. It was detected that 2.2% of the population in the study was obese; 2.1% of males and 2.3% of females. While the prevalence of obesity was extremely low before 9 ages and after 15, it reached to high values at puberty and just before pubertal period in boys. The prevalence of overweight was higher in girls and reached to peak point at pubertal ages. Generally, the prevalence of obesity and overweight was slightly higher in girls than in boys, although the boys were more obese in prepubertal ages. CONCLUSION: Overweight and obesity are concerns for children and adolescents in low socio-economic status regions as well.
Asunto(s)
Obesidad/epidemiología , Sobrepeso/epidemiología , Adolescente , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Prevalencia , Pubertad , Turquía/epidemiologíaRESUMEN
Varicella-associated purpura fulminans is a rare syndrome associated with substantial morbidity and mortality. General supportive care, heparinization, and plasma infusions are the mainstays of treatment. A patient aged 8 years and 8 months with purpura fulminans and multiple deep vein thromboses after varicella infection because of deficiencies of proteins C and S is presented in this case report.