Infants with macrosomia and infants of diabetic mothers have increased carotid artery intima-media thickness in childhood.

Incidence of diabetes during pregnancy is increasing worldwide, and intrauterine hyperglycemia exposure may have long-term adverse effects on the cardiovascular health of children. We investigated prospectively the risk of atherosclerosis and carotid intima-media thickness (CIMT) in infants born macrosomic and in infants of diabetic mothers (IDM) at the age of 8-9 years in 2021. A total of 49 infants of diabetic mothers (IDM group) and 13 macrosomic infants (macrosomic group) were included in the study. They were compared with 26 age-matched healthy children with birth weight appropriate for gestational age born to non-diabetic mothers (control group). Anthropometric measurements, atherosclerosis risk factors, and CIMT measurements were performed. There was no significant difference between the groups in terms of age, gender, actual anthropometric measurements, blood pressure measurements, laboratory parameters, or atherosclerosis risk factors. Gestational age was lower in the IDM group (p < 0.001), while birth weight was higher in the macrosomic group (p < 0.001). High-density lipoprotein cholesterol level was lower in the IDM group than the other groups. Duration of exclusive and total breastfeeding was lower in IDM group than in the control group (p < 0.001 for both). Body mass index, skinfold thickness, waist-to-hip ratio, and waist-to-height ratio were higher in those breastfed for less than 6 months in the IDM group. The CIMT values were statistically higher in IDM [0.43 ± 0.047 (0.34-0.60)] and macrosomic [0.40 ± 0.055 (0.33-0.50)] groups than control group [0.34 ± 0.047 (0.26-0.45)]. CONCLUSION: CIMT values were higher in IDM and macrosomic groups at 8-9 years old age compared to children born with normal birth weight. This indicates intrauterine exposure in both groups. And also, breastfeeding seems very important for IDMs. WHAT IS KNOWN: • Intrauterine hyperglycemia exposure has long-term adverse effects on the cardiovascular health of children. • Infants of diabetic mothers have higher carotid artery intima-media thickness at birth. WHAT IS NEW: • Both infants of diabetic mothers and infants with macrosomia have increased carotid artery intima-media thickness at the age of 8-9 years. • Duration of breast feeding is important especially in infants of diabetic mothers as body mass index, skinfold thickness, waist to hip and height ratio were higher in those breastfed less than 6 months.

Increased serum malondialdehyde level in neonates with hypoxic-ischaemic encephalopathy: prediction of disease severity.

Increased serum level of malondialdehyde (sMDA) in neonates with hypoxic-ischaemic encephalopathy (HIE) was evaluated as a possible criterion for determining HIE severity. Mean body weight and gestational age in a healthy control group of neonates (n = 63) and in neonates with HIE (n = 69) were statistically similar. Apgar scores at 1 and 5 min for the HIE group were significantly lower than for the control group. The mean sMDA level for the HIE group was significantly higher than the control group. Within the HIE group, the sMDA level for neonates with Sarnat's grade II and III was significantly higher than for those with Sarnat's grade I. There was a significant correlation between Sarnat's grading and the sMDA level. The sMDA level was significantly higher for neonates who died (n = 20) compared with those who survived (n = 49). In conclusion, the sMDA level was highest in neonates with HIE and correlated with HIE severity. The sMDA concentration could, therefore, be used as a criterion for predicting disease severity.

A Turkish newborn infant with cerebellar agenesis/neonatal diabetes mellitus and PTF1A mutation.

Classical neonatal diabetes mellitus is defined as hyperglycemia that occurs within the first month of life in term infants. It can be either permanent or transient. Cerebellar agenesis and permanent neonatal diabetes has been previously reported as a new autosomal recessive disorder. Pancreas Transcription Factor 1 Alpha (PTF1A) mutations have been related with this constellation of abnormalities. Here we report a new case of cerebellar agenesis and neonatal diabetes mellitus whose parents are PTF1A mutation carriers.

The effects of indomethacin on caspases, glutathione level and lipid peroxidation in the newborn rats with hypoxic-ischemic cerebral injury.

Activation of phospholipase A(2), degradation of membrane phospholipids resulting in tissue accumulation of arachidonic acid, and the activation of cyclooxygenase that leads to the formation of prostaglandin and free radicals may occur after hypoxic-ischemic damage. The aim of this study was to investigate the effects of indomethacin, a nonselective cyclooxygenase inhibitor, on caspase activity, glutathione levels and lipid peroxidation in newborn rats with hypoxic-ischemic encephalopathy. The effects of indomethacin were evaluated by measuring caspase-3 and caspase-8 activities and glutathione levels. Lipid peroxidation was evaluated by measuring concentrations of malondialdehyde in rat brains. Seven-day-old rat pups with the Levine-Rice model of hypoxic-ischemic cerebral injury were randomly divided into three study groups. In the indomethacin-treated group, rats were administered three doses of indomethacin, at a dose of 2 mg/kg every 12 h. Sham and the hypoxic-ischemic group of rats were given physiologic saline. The sham group underwent all surgical procedures except for arterial ligation. After 72 hours, the rats were decapitated and brain tissues were evaluated. Caspase-3 and caspase-8 activities and glutathione and malondialdehyde levels were evaluated in all groups. There was an obvious decrease in caspase-3 and caspase-8 activities and depleted glutathione levels were reversed in the indomethacin-treated group compared to the hypoxic-ischemia group (p<0.001). As indomethacin was unable to prevent lipid peroxidation, malondialdehyde concentrations increased to ischemia-induced levels. In conclusion, indomethacin administration after hypoxic-ischemic encephalopathy injury has a neuroprotective effect since it inhibits caspase activity and reverses the depletion of glutathione. However, it also aggravates lipid peroxidation-induced ischemia.

Bruck syndrome: osteogenesis imperfecta and arthrogryposis multiplex congenita.

Bruck syndrome is characterised by osteogenesis imperfecta and arthrogryposis multiplex. In some patients, mutations in the lysyl hydroxylase 2 gene (PLOD2, 3q23-q24) have been demonstrated. A male newborn with Bruck syndrome is reported.

Rubella seroprevalence in children in Dogankent, a rural area of Adana province in Turkey, January-February 2005.

A cross-sectional study was performed to determine the rubella seroprevalence in 331 children aged between 0 and 59 months in Turkey who were not vaccinated for rubella and lived in the area covered by Dogankent Health Center, a rural area with a large proportion of residents of low socioeconomic status. Rubella seropositivity was found to be low, with 17.5%, increased with age and low socioeconomic level, and was particularly high in children who live in a household with one member going to school, and in children of uneducated parents (p<0.05). The asymptomatic infection rate was 98.3%. There was no significant difference in seropositivity with regards to the gender, history of rubella infection, size of the household, or number of children at home (p>0.05). Rubella vaccine has only been included into the national vaccination programme in the form of the measles-mumps-rubella (MMR) vaccine since 2006 and is performed at the age of 12 months, in the first year of primary school and at the age of about 15 years. In order to eliminate rubella and congenital rubella syndrome, it is necessary that use of MMR vaccine is expanded to include the children born before 2006.

Growth hormone and insulin-like growth factor 1 levels and their relation to survival in children with bacterial sepsis and septic shock.

OBJECTIVES: Despite improved supportive care, the mortality of sepsis and septic shock is still high. Multiple changes in the neuroendocrine systems, at least in part, are responsible for the high morbidity and mortality. A reduced circulating level of insulin-like growth factor and an elevated level of growth hormone are the reported characteristic findings early in the course of sepsis and septic shock in adults. The aim of this study was to evaluate the changes of growth hormone/insulin-like growth factor 1 axis in sepsis and septic shock and investigate the relationship between these hormones and survival. METHODS: Fifty-one children with sepsis (S), 21 children with septic shock (SS) and 30 healthy, age- and sex-matched children (C) were enrolled in this study. Growth hormone, insulin-like growth factor 1 and cortisol levels of the sepsis and septic shock groups were obtained before administration of any inotropic agent. RESULTS: Growth hormone levels were 32.3 +/- 1.5 microIU/mL (range 4-56), 15.9 +/- 0.6 microIU/mL (range 11-28) and 55.7 +/- 2.7 microIU/mL (range 20-70) in S, C and SS groups, respectively. The difference between the growth hormone levels of the S and C groups, S and SS groups, and C and SS groups were significant (P < 0.001). Non-survivors (54.7 +/- 1.6 microIU/mL) had significantly higher growth hormone levels than survivors (29.4 +/- 1.5 microIU/mL) (P < 0.001). Insulin-like growth factor 1 levels were 38.1 +/- 2.1 ng/mL (range 19-100), 122.9 +/- 9.6 ng/mL (range 48-250) and 22.2 +/- 1.9 ng/mL (range 10-46) in the S, C and SS groups, respectively, and the difference between the insulin-like growth factor 1 levels of the S and C, S and SS, and C and SS groups were significant (P < 0.001). Non-survivors (8.8 +/- 1.1 micro g/dL) had significantly lower cortisol levels than survivors (40.9 +/- 2.1 microg/dL) (P < 0.001). We detected a significant difference between the levels of cortisol in non-survivors (19.7 +/- 1.8 microg/dL) and survivors (33.9 +/- 0.9 microg/dL) (P < 0.01). CONCLUSIONS: There were elevated levels of growth hormone with decreased levels of insulin-like growth factor 1 in children during sepsis and septic shock compared to healthy subjects. In addition, there were even higher levels of growth hormone and lower levels of insulin-like growth factor 1 in non-survivors than in survivors. We think that both growth hormone and insulin-like growth factor 1 may have potential prognostic value to serve as a marker in bacterial sepsis and septic shock in children. As there is insufficient data in the paediatric age group, more studies including large numbers of patients and additionally evaluating cytokines and insulin-like growth factor binding proteins are needed.

Correlation of simultaneously obtained capillary, venous, and arterial blood gases of patients in a paediatric intensive care unit.

AIMS: To investigate the correlation of pH, partial pressure of oxygen (PO2), partial pressure of carbon dioxide (PCO2), base excess (BE), and bicarbonate (HCO3) between arterial (ABG), venous (VBG), and capillary (CBG) blood gases. METHODS: Patients admitted to the paediatric intensive care unit (PICU) in Cukurova University between August 2000 and February 2002 were enrolled. RESULTS: A total of 116 simultaneous venous, arterial, and capillary blood samples were obtained from 116 patients (mean age 56.91 months, range 15 days to 160 months). Eight (7%) were neonates. Sixty six (57%) were males. pH, PCO2, BE, and HCO3 were all significantly correlated in ABG, VBG, and CBG. Correlation in PO2 was also significant, but less so. Correlation between pH, PCO2, PO2, BE, and HCO3 was similar in the presence of hypothermia, hyperthermia, and prolonged capillary refilling time. In hypotension, correlation in PO2 between VBG and CBG was similar but disappeared in ABG-VBG and ABG-CBG. CONCLUSIONS: There is a significant correlation in pH, PCO2, PO2, BE, and HCO3 among ABG, VBG, and CBG values, except for a poor correlation in PO2 in the presence of hypotension. Capillary and venous blood gas measurements may be useful alternatives to arterial samples for patients who do not require regular continuous blood pressure recordings and close monitoring of PaO2. We do not recommend CBG and VBG for determining PO2 of ABG.

Schwartz-Jampel syndrome: three pediatric case reports.

Schwartz-Jampel syndrome is a heterogeneous autosomal recessive syndrome defined by myotonia, short stature, bone dysplasia and growth retardation. Three types have been described: type 1A, usually recognized in childhood, with moderate bone dysplasia; type 1B similar to type 1A but recognizable at birth, with more prominent bone dysplasia and type 2, a rare, more severe form with increased mortality in the neonatal period. In this paper we report three pediatric cases, one with neonatal manifestation.

A newborn infant with sternal malformation/vascular dysplasia association.

Sternal malformation/vascular dysplasia complex was described by Hersch et al. in 1985. The principle findings include cleft of the sternum covered by an atrophic skin, a midline abdominal raphe and hemangiomatosis. The inheritance pattern seems to be sporadic. We report a newborn baby with sternal defect, cleft lip and palate, supraumbilical raphe and hemangiomas.

Congenital Hypofibrinogenemia: A Newborn Infant with Cord Bleeding.

The genetic disorders causing decreased fibrinogen synthesis may be caused by heterozygous (hypofibrinogenemia) or homozygous (afibrinogenemia) deficiency. The consequences of the disorders are gastrointestinal bleeding, cord bleeding, eccymoses, subcutaneous hematomas and hemarthroses especially due to traumatic delivery in the neonatal period. Laboratory evaluation of the patient with hypofibrinogenemia reveals prolongation of thrombin time, partial thromboplastin time, prothrombin time and decreased fibrinogen level. We report a 21 days old, congenital hypofibrinogenemia case with cord bleeding.