bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient
Authors: Main KM , Skov M , Sillesen IB , Dige‐Petersen H , Müller J , Koch C , Lanng S 
Published: August 2010

Treatment of allergic bronchopulmonary aspergillosis with itraconazole is becoming more widespread in chronic lung diseases. A considerable number of patients is concomitantly treated with topical or systemic glucocorticoids for anti‐inflammatory effect. As azole compounds inhibit cytochrome P450 enzymes such as CYP3A isoforms, they may compromise the metabolic clearance of glucocorticoids, thereby causing serious adverse effects. A patient with cystic fibrosis is reported who developed iatrogenic Cushing's syndrome after long‐term treatment with daily doses of 800 mg itraconazole and 1600 μg budesonide. The patient experienced symptoms of striae, moon‐face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total suppression of spontaneous and stimulated plasma cortisol and adrenocorticotropin. Discontinuation of both drugs led to an improvement in clinical symptoms and recovery of the pituitary‐adrenal axis after 3 mo.Conclusion: This observation suggests that the metabolic clearance of budesonide was compromised by itraconazole's inhibition of cytochrome P450 enzymes, especially the CYP3A isoforms, causing an elevation in systemic budesonide concentration. This provoked a complete suppression of the endogenous adrenal function, as well as iatrogenic Cushing's syndrome. Patients on combination therapy of itraconazole and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge.
Erythropoietin in the central nervous system, and its use to prevent hypoxic‐ischemic brain damage
Authors: Juul S 
Published: August 2010

A new field of clinical and scientific interest has recently developed based on the discovery that the hematopoietic cytokine erythropoietin (Epo) has important non‐hematopoietic functions in the brain and other organs, particularly during development. The biological effects of Epo in the central nervous system (CNS) involve activation of its specific receptor and corresponding signal transduction pathways. Epo receptor expression is abundant in the developing mammalian brain, and decreases as term approaches. Epo has been identified as a neurotrophic and neuroprotective agent in a wide variety of experimental paradigms, from neuronal cell culture to in vivo models of brain injury. Several mechanisms by which Epo produces neuroprotection are recognized. Epo (i) decreases glutamate toxicity, (ii) induces the generation of neuronal anti‐apoptotic factors, (iii) reduces inflammation, (iv) decreases nitric oxide‐mediated injury, and (v) has direct antioxidant effects. Conclusion: Collectively, the evidence suggests that Epo may provide a new approach to the treatment of a variety of CNS disorders in adults and children, especially as a possible therapy for perinatal asphyxia. This review summarizes the current knowledge on the neurotrophic and neuroprotective functions of Epo in the developing and injured brain.
Enteral administration of hematopoietic growth factors in the neonatal intensive care unit
Authors: Calhoun DA 
Published: August 2010

By 20 wk of gestation, the human fetal gastrointestinal (GI) tract morphologically resembles that of the term infant, but functional development is limited before 26 wk. By 30 wk of gestation, the fetus has the capacity for limited digestion and enteral absorption. GI growth and development continue postnatally. Trophic factors, including nutrients, peptides, hormones and growth factors, are recognized as having important influences on the morphology and histology of the developing GI tract. Other trophic factors are important in adaptation and repair following injury. Many such factors are provided in utero via amniotic fluid swallowing and later by human colostrum and milk. Conclusion: This review discusses cytokines with known GI trophic effects, either in vitro or in vivo, and focuses on those cytokines that have been used in the neonatal intensive care unit.SummaryGI trophic factors have been identified that influence the prenatal and postnatal growth and development of the GI tract. Systemically as well as enterally administered growth factors can stimulate GI growth and maturation, suggesting that trophic factors in the serum of neonates can modulate GI growth via receptors on the serosal membranes of enterocytes. GI trophic factors can be synthesized endogenously or provided prenatally in amniotic fluid and postnatally in human milk.GI trophic factors in human milk play an important role in regulating the adaptive functional changes that accompany the transition to postnatal enteral feedings. Although human milk growth factors are not essential for infant survival, the elevated risk of GI‐related illnesses in formula‐fed compared with human milk‐fed infants suggests that bioactive compounds in human milk contribute to the protective effects of human milk feedings (13).GI trophic factors have the potential to be used therapeutically to enhance GI maturation and repair following injury. These applications may be particularly useful in the premature or postsurgical infant. Several issues require further research, including: (i) the efficacy of oral versus systemic administration; (ii) characterization of the complex interactions among the various growth factors; (iii) the effect of exogenously administered growth factors on endogenous production of that factor, its receptor or other growth factors; (iv) the effect of growth factors upon tissues not directly associated with the GI tract; and (v) the determination of safe and effective limits. Significant advances in feeding strategies to reduce feeding intolerance in the neonate are likely to occur with the application of these principles in clinical neonatology.
Using fluorescence‐activated cell sorting followed by fluorescence in situ hybridization to study lineage relationships: the 8;21 translocation is present in neutrophils but not monocytes in a patient with severe congenital neutropenia and a granulocyte colony‐stimulating factor‐responsive clonal abnormality
Authors: White C , Chen Z , Raetz E , Pulsipher M , Spangrade GJ , Slayton WB 
Published: August 2010

Severe congenital neutropenia (Kostmann syndrome) is a disorder that presents in the neonatal period, but predisposes to leukemia later in life. This report describes a 4‐y‐old female with a history of severe congenital neutropenia, who developed a clonal abnormality associated with the translocation (7;21;8) (q32;q22;q22) (AML‐1/ETO). She had circulating peripheral blasts and bone marrow blast counts as high as 64% when she received recombinant granulocyte colony‐stimulating factor (rG‐CSF). Her marrow blasts decreased to 4‐20% when rG‐CSF was discontinued. Fluorescence in situ hybridization analysis was performed on bone marrow cell populations sorted by flow cytometry to determine which cell populations had the AML‐1/ETO translocation. The translocation was found in mature neutrophils and blasts, but not in monocytes, lymphocytes or stem cells. Conclusion: These findings suggest that the translocation occurred in a neutrophil progenitor, past the point in ontogeny where monocytes and neutrophils separate. The techniques described may be useful in understanding lineage relationships and leukemogenesis in other clonal abnormalities associated with myelodysplasia and leukemia.
Endothelial markers and homocysteine in patients with classic Fabry disease
Authors: Demuth K , Germain DP 
Published: August 2010

Aim: Fabry disease is an X‐linked inborn error of glycosphingolipid metabolism due to the deficient activity of α‐galactosidase A, a lysosomal enzyme. It is a multisystem disorder characterized by progressive renal insufficiency, with added morbidity from cardio‐ and cerebrovascular involvement. The recent availability of genetically engineered enzyme offers an effective targeted treatment approach, but also emphasizes the need for surrogate markers to delineate organ damage and monitor the efficacy of enzyme replacement therapy (ERT). Methods: Multiple endothelial factors and plasma homocysteine concentrations were investigated in 12 consecutive hemizygous males with classic Fabry disease and 15 controls as part of an exhaustive baseline evaluation prior to ERT. Results: Compared with the controls, plasma concentrations of homocysteine were significantly (p > 0.01) higher in patients with Fabry disease in the absence of chronic renal failure or vitamin deficiency. Plasma concentrations of vascular cell adhesion molecule‐1 were also significantly (p > 0.05) higher in the patients, and there was a trend for decreased endothelin‐1 levels. No difference was found in serum intercellular adhesion molecule‐1, plasma P‐selectin, serum E‐selectin and plasma thrombomodulin between the patients and controls. Conclusions: The results do not reveal measurable evidence for endothelial and leukocyte activation that could reliably serve as surrogate markers for routine monitoring of the efficacy of ERT in patients with Fabry disease. While the exact origin and clinical significance of hyperhomocysteinaemia in Fabry disease remains to be studied in a larger cohort of patients carefully monitored for their concurrent medications, especially carbamazepine, we suggest that patients may benefit from folic acid or multivitamin therapy to treat this additional vascular risk factor, when present.
Factors relating to the cardiac sequelae of Kawasaki disease one month after initial onset
Authors: Zhang T , Yanagawa H , Oki I , Nakamura Y 
Published: August 2010

This study aimed to determine the risk factors related to the presence of cardiac sequelae 1 mo after initial onset and to examine the preventive effect of the early administration of high‐dose gamma‐globulin (GG) on cardiac sequelae in patients with Kawasaki disease. Patients treated with high‐dose GG of 2000 ± 100 mg kg‐1 were selected as subjects from the 15th nation‐wide survey in Japan. Univariate and logistic multiple variable analyses were used to test the effects of background variables such as age and gender, variables relating to laboratory findings such as the percentage of neutrophil leucocytes, and variables relating to the GG treatment on the presence of cardiac sequelae. The odds ratios were significantly higher for males (1.48), those younger than 1 y of age (1.71), recurrent cases (2.42), and those with a low haematocrit (>32.5%) (1.45) and high percentage of neutrophil leucocytes (<68%) (1.63). The odds ratio was low for those who started GG administration in less than 6 d from onset between the patients with and without cardiac sequelae. The odds ratio for the duration of GG treatment was not significantly different between those with and without cardiac sequelae. Conclusion: Patients who received early administration of GG, less than 6 d from onset of the disease, had a lower risk than those received GG more than 6 d from the onset. The percentage of neutrophil leucocytes and the haematocrit level are useful indicators in predicting the development of cardiac sequelae.
Postnatal urinary conductance measurement in preterm infants
Authors: Rangaraj S , Penney MD , Ferguson SD 
Published: August 2010

The aim of this study was to determine whether serial urinary conductance measurements can be used to estimate reliably the end of the transition period of negative sodium balance in preterm infants. The relationship between urine conductance, measured by a conductance meter, and urine sodium concentration was determined in 109 pooled samples of urine obtained from 14 preterm infants during the transitional period of fluid balance. It was shown by linear regression analysis that urine sodium concentration (mmol 1‐1) = 0.78 ± urine conductance – 1.25. Urine sodium concentrations derived from the above formula were concordant with urine sodium measured directly when used to calculate daily sodium balance in all 14 infants. Conclusion: Urine conductance can be accurately measured at the cotside by neonatal nurses and used to identify the timing of the postnatal transition from negative to positive sodium balance in preterm infants. These findings can help in making decisions on the introduction of postnatal sodium administration to preterm infants.
Breastfeeding, baby friendliness and birth in transition in North Western Russia: a study of women's perceptions of the care they receive when giving birth in six maternity homes in the cities of Archangelsk and Murmansk, 1999
Authors: Helsing E , Chalmers BE , Dinekina TJ , Kondakova NI 
Published: August 2010

Women's own views on the quality of the birthing care they received were recorded in a small study in the cities of Archangelsk and Murmansk in February 1999. Six maternity wards took part; one hospital had already been designated as a Baby Friendly Hospital (BFH) according to the strict global criteria of the WHO/UNICEF‐recommended Baby Friendly Hospital Initiative (BFHI). Two of the hospitals had made profound changes in feeding routines and were by their own reckoning close to achieving this distinction, and were included in the BFH group. Three maternity wards were far from being in compliance with the BFHI approach and were grouped as the Non‐Baby‐Friendly Hospitals (NBFH). A total of 180 newly delivered mothers answered a 60‐item questionnaire about their birthing and breastfeeding experiences. The questions were chosen from an existing protocol, the WEB (Women's Experiences of Birth) developed by one of the authors (BC). The study was part of an informal evaluation of five years of BFHI activities in the Barents Region, supported by Norway, and also aimed at recording any positive carry‐over effect of the BFHI into obstetric routines as a whole. Conclusion: It was found that the project definitely had had an impact; feeding practices at the BFH were markedly closer to the international BFHI recommendations than at the NBFH. BFH mothers, however, reported suffering from breastfeeding problems just as often or more so than NBFH mothers. Possible explanations are discussed; it is concluded that this cross‐sectional study may depict a transitory situation in the BFH. At the two hospitals not yet assessed, although staff felt that they had made profound changes, they may not yet have grasped the full extent and stringency of the changes required. The study shows that, despite good will, some practical details had not yet been worked out, resulting in a mixed outcome for the mothers. There was no noticeable carry‐over of the attitudes and basic ideas of the project into obstetric care, either in the BFH or in the NBFH, so changes in this area may require separate strategies.
Severe sleep problems in infancy associated with subsequent development of attention‐deficit/hyperactivity disorder at 5.5 years of age
Authors: Thunström M 
Published: August 2010

The aim of this five‐year prospective study was to follow and compare a group of children with severe sleep problems in infancy with a control group regarding development of symptoms of attention‐deficit/hyperactivity disorder (ADHD). Factors in infancy were sought which were associated with development of ADHD in later childhood. A total community sample of 2518 infants aged 6–18 mo was approached with a questionnaire, and 83% responded. Data from the collection procedure point to a non‐selective dropout. A case group of those children aged 6–12 mo who fulfilled specific criteria for severe and chronic sleep problems (n= 27) was compared with a control group of equal size, matched for age and gender. At the age of 5.5 y, seven of the children in the sleep problem group met the criteria for the diagnosis of ADHD based on an in‐depth assessment by a multidisciplinary team. None of the control children qualified for the diagnosis. This difference is statistically significant. Comparisons between the children with ADHD and the rest of the problem group showed that given severe infant sleep problems, the following characteristics in infancy were associated with subsequent diagnosis of ADHD: psychosocial problems in the family, bedtime struggles and long sleep latency at bedtime. Owing to the small sample size, the results are considered preliminary. Conclusion: Approximately one in four children with severe sleep problems in infancy will later qualify for the diagnosis of ADHD. Infants with severe sleep problems, especially in combination with behavioural problems, high activity level and psychosocial problems in the family, deserve attention since neurodevelopmental problems seem to be prevalent in this category of children.
Influence of leptin levels and body weight in survival of children with sepsis
Authors: Blanco‐Quirós A , Casado‐Flores J , Arranz E , Garrote JA , Asensio J , Pérez A 
Published: August 2010

High levels of serum leptin (LPT) were reported in adult patients with sepsis and a protective role was suggested. LPT was determined in sera from 55 children with severe sepsis at admission (0 h), 6, 24 and 48 h. LPT levels were higher at 0 h than at 24 h (2.80 vs 1.61 ng/ml; p= 0.009) and a negative correlation was found with IL‐13 (p= 0.009), and granulocyte counts (p= 0.035), but not with other factors. Infants younger than 12 mo of age had higher LPT levels than older infants (5.88 vs 2.38 ng/ml; p= 0.0005). The increase in LPT levels was higher in non‐survivor patients than in survivors, with a maximum difference at 24 h (5.30 vs 1.45 ng/ml; p= 0.0042). However, LPT levels were not associated with shock, multiorgan failure or the severity score. Children who died showed higher percentiles of weight than survivors (p= 0.025). A subgroup with higher LPT (<Pc75) included mainly patients with weight <Pc50 (p= 0.0065), low IL‐13 levels (p= 0.007) and low granulocyte counts (p= 0.013), Neisseria meningitidis B being the most frequently isolated germ (p= 0.022). Conclusion: Using a model of severe infection, mainly meningococcal, in young children (median 3 y 6 mo old), it was not possible to confirm previous results in adults. A general protective role for LPT in sepsis seems unlikely.
Different proinflammatory reactions in fatal and non‐fatal enterovirus 71 infections: implications for early recognition and therapy
Authors: Lin T‐Y , Chang L‐Y , Huang Y‐C , Hsu K‐H , Chiu C‐H , Yang K‐D 
Published: August 2010

Aim: The mechanism of pulmonary oedema, a life‐threatening manifestation of enterovirus 71 (EV71) encephalitis, is unclear. Our aim was to assess the relationship of proinflammatory cytokines to EV71‐related pulmonary oedema. Methods: Proinflammatory responses in 33 EV71 patients with various complications and 21 normal healthy children were measured using an enzyme‐linked immunosorbent assay. Results: EV71 patients with both encephalitis and pulmonary oedema were found to have much higher levels of blood interleukin‐6 (IL‐6) (947 ± 1239 vs 4.9 ± 3.1 pg/ml, p= 0.0003), tumour necrosis factor‐α (TNF‐α) (22.4 ± 29.5 vs 5.3 ± 1.0 pg/ml, p= 0.0035), interleukin 1β (IL‐1β) (48.4 ± 85.2 vs 4.9 ± 10.1 pg/ml, p= 0.01), white blood cell count (28.3 ± 7.6 vs 15.5 ± 6.8 109/L, p± 0.0001) and blood glucose (501 ± 186 vs 165 ± 117 mg/dL, p= 0.0009) than patients with EV71 encephalitis alone. In fact, the cytokine levels in patients with encephalitis only or in those without complications were not significantly different from the levels found in normal children. The sensitivity, specificity, positive and negative predictive values of IL‐6 < 70 pg/ml for EV71 encephalitis with pulmonary oedema were all 100%. Conclusion: Patients with EV71‐related encephalitis combined with pulmonary oedema were found to have significantly elevated levels of proinflammatory cytokines and the best predictor for this complicated condition was found to be the level of serum IL‐6.
Injuries in public and private playgrounds: the relative contribution of structural, equipment and human factors
Authors: Petridou E , Sibert J , Dedoukou X , Skalkidis I , Trichopoulos D 
Published: August 2010

The aim of this case‐control study was to identify and quantify risk factors of injuries in playgrounds, where children spend an increasing amount of time in developed countries. The study took place in Greater Athens during 1999. A continuous Emergency Departments Injury Surveillance System (EDISS) of hospitals that cover about 30% of the children's time at risk in Greater Athens identified 777 injuries in public and private playgrounds out of a total of 17 497 injuries. Public playgrounds differ from private ones, because the former generally have more equipment, usually of greater height, with less resilient surfaces, and supervision relies mainly on parents or guardians. Patterns of type of playground use were assessed in a sample of 294 children from the same study base who served as a control group in a hierarchical case‐control design. The annual incidence of playground injuries in Greater Athens was about 7 in 1000 among boys and 4 in 1000 among girls, with a 2.2 times higher risk for an injury in public than in private playgrounds (95% confidence interval 1.61–3.07). Children in public vs private playgrounds had a statistically significant eight times higher odds for concussion and six times higher for open wounds, whereas the odds for long bone fractures were four and for other fractures two; swings, slides and seesaws were the types of equipment most frequently associated with injuries. It was further shown that supervision of children was suboptimal (>60%) in both public and private playgrounds, and children in private playgrounds sustained an unduly high frequency of sprain/ dislocation injuries (odds ratio 1.75) because they were encouraged to play bare‐footed. Conclusion: Close to 50% of playground injuries could be prevented by structural and equipment changes, while further reduction could be accomplished through simple measures including closer supervision and encouraging children to wear proper shoes and use protective equipment whenever necessary.