Search NEL Online

Comparative Study 2014; 35(3): 242-247 PubMed PMID: 24977976 Citation  Keywords:  Adolescent, Body Height, Child, Dwarfism, Pituitary:drug therapy, Female, Hormone Replacement Therapy, Human Growth Hormone:therapeutic use, Humans, Male, Poland, Prognosis, Recombinant Proteins:therapeutic use, Severity of Illness Index,.   

OBJECTIVES: The main goal of growth hormone therapy is to reach the height in the population ranges. The aim of the study was the comparison of selected methods for predicting final height in Polish patients with severe (sGHD) and partial (pGHD) growth hormone deficiency.

METHODS: 149 children with growth hormone deficiency treated with rhGH in the Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology Developmental Age, PUM, in Szczecin, in 2000-2010 have been evaluated. Patient were divided into two groups: sGHD and pGHD. Two methods of final height prediction have been used: Roche-Weiner-Thissen (RWT) and target height (TH), results were compared to the final height (FH). 117 children finished therapy in the analysed period and reached final height.

RESULTS: The mean FH was similar in both groups. There was no significant difference between the accuracy of prediction methods of TH and RWT between groups of pGHD v. sGHD. Further analysis revealed, that in the group of boys with sGHD the prediction error of RWT was significantly lower than of the TH method (p < 0.05).

CONCLUSIONS: It seems that in the group of boys with sGHD RWT is a more accurate method than TH....

Read abstract  Full text PDF

Journal Article 2014; 35(3): 213-217 PubMed PMID: 24977971 Citation  Keywords:  Adult, Aged, Child, Crime Victims:psychology, Female, Humans, Life Change Events, Male, Middle Aged, Oxytocin:blood, Stress, Psychological:blood, Suicide, Attempted:psychology, Violence:psychology, Young Adult,.   

OBJECTIVE: Some studies have reported an inverse relationship between childhood adversity and oxytocin levels. The purpose of this study was to assess the relationship between CSF and plasma oxytocin levels and lifetime trauma history in suicide attempters. We hypothesised lower CSF and plasma oxytocin levels in suicide attempters with high exposure to interpersonal violence and negative childhood emotional climate.

METHODS: 28 medication free suicide attempters participated in the study. CSF and plasma morning basal levels of oxytocin were assessed with specific radioimmunoassays. The Karolinska Interpersonal Violence Scale (KIVS) was used to elicit lifetime trauma history and revictimization status and the childhood emotional climate factor was derived from the socialization subscale of the Karolinska Scales of Personality.

RESULTS: Correlations between exposure to interpersonal violence as a child and as an adult and CSF and plasma oxytocin levels were not significant. Revictimized suicide attempters had significantly lower plasma oxytocin levels and more negative childhood emotional climate compared to non-revictimized suicide attempters.

CONCLUSIONS: Our results indicate a complex relationship between life time trauma and the oxytocin system....

Read abstract  Full text PDF

Case Reports 2014; 35(2): 137-141 PubMed PMID: 24878975 Citation  Keywords:  Central Nervous System:physiopathology, Child, Glycogen Storage Disease:complications, Humans, Intellectual Disability:complications, Male, Microcephaly:complications, Mutation, Missense, Phenotype, Phosphoglucomutase:genetics,.   

OBJECTIVES: A 10-year-old boy presented with cleft palate, hepatopathy, cholecystolithiasis, myopathy, coagulopathy, hyperlipidemia, hypoglycemia, hyperuricemia, short stature, obesity, hypothyroidism, microcephaly and mild intellectual disability. The multi-systemic manifestation involving certain distinct clinical features prompted us to search for a subtype of congenital disorders of glycosylation (CDG).

METHODS: The patient was screened for CDG by examining the distribution of transferrin (TRF) and apolipoprotein C-III (ApoC-III) sialylated isoforms using isoelectric focusing of serum. This was followed by spectrophotometric measurement of phosphoglucomutase 1 (PGM1) activity in fibroblasts and molecular analysis including sequencing and PCR-RFLP of PGM1 gene. Selected bioinformatics tools were used to evaluate the data.

RESULTS: Increased relative levels of di-, mono- and asialotransferrin reflected a defect of N-glycosylation in the patient. Markedly decreased activity of PGM1 corresponding to less than 5% of control´s was found. Sequencing of PGM1 gene revealed the presence of two heterozygous missense mutations c.1010C>T (p.T337M) and c.1508G>A (p.R503Q), whose pathogenicity was confirmed by in silico analysis.

CONCLUSION: We report the first Czech patient with a glycosylation disorder due to PGM1 deficiency. Compared to the described cases, no dilated cardiomyopathy was noted in our patient. However, he suffered from a mild neurological impairment, which is an uncommon feature that extends the phenotype associated with PGM1-CDG. Lactose-rich diet, which was previously reported to have ameliorated the clinical symptoms in some PGM1-CDG patients, did not result in any improvement in our patient....

Read abstract  Full text PDF

Journal Article 2014; 35(2): 110-115 PubMed PMID: 24878974 Citation  Keywords:  AIDS-Related Opportunistic Infections:immunology, Amino Acid Sequence, Antigens, Bacterial:chemistry, Blotting, Western, Cambodia, Child, Cohort Studies, HIV Antigens:chemistry, HIV Infections:complications, HIV-1:immunology, Humans, Intestines:immunology.   

OBJECTIVES: Human immunodeficiency virus type 1 is widely accepted as the cause of AIDS (Acquires Immunodeficiency Syndrome) but it is necessary to consider other factors, not only HIV, which may be involved in AIDS process. It is apparent that a viral reservoir persists in virtually all infected individuals receiving HAART. Reservoirs were detected in macrophages and other cells of the blood system, in which even very effective HAART was not able to eliminate the virus. Over the last period of time AIDS research has been focused on the gut and other mucosal tissue as the major site of HIV infection and CD4+ T cells loss. Intestinal bacteria and cells associated with GIT are in close vicinity and so has been raised the idea that bacteria may be involved in AIDS pathogenesis. Matherial/Methods: Bacteria and yeast isolated from a cohort of 67 Cambodian and Kenyan HIV positive children and from a cohort of 62 Slovak and American AIDS patients were analyzed for detection of expression of HIV-1 antigens p17, p24, p55, gp41 and gp120 (Abcam, UK).

RESULTS: By monoclonal antibodies against HIV-1 proteins p17 and p55 was detected protein with molecular weight of 45-55 kDa. In samples of Cambodian and Kenyan HIV positive children was found 35 kDa protein using MAb against HIV-specific protein p17. By using MAbs against p24 was found protein of 55-60 kDa in Cambodian and Kenyan samples but, suprisingly, no proteins were detected in bacterial extracts of American and Slovak AIDS patients by this MAbs. Using monoclonal antibodies against HIV-1 specific protein gp41 was positive signal identified in 30-35% of samples from both cohorts of patients from Kenya and Cambodia and in 75% of samples from American and Slovak patients. The protein of about 75-85 kDa was detected by MAbs against gp120 only in protein extracts obtained from yeasts Candida sp. of Cambodian and Kenyan HIV positive children.

CONCLUSION: The molecular weight of 55 kDa protein was detected by MAbs anti HIV p24, p17+p55. Its molecular weight is comparable to gag-encoded Pr55Gag precursor. Surprisingly, such proteins were not found in bacterial extract from samples of American and Slovak patients by using the MAbs against HIV-specific protein p24. The protein of about 75-85 kDa was detected only in Candida species protein extracts of Cambodian and Kenyan HIV positive children by the MAbs against gp120. In Slovak and American samples, protein reacting with MAbs anti gp120 was not found. These results suggest that there are specific differences between Slovak and American HIV positive patients bacterial proteins on one side and Cambodian and Kenyan on the other. These differences may suggest a diverse bacterial evolution in various geographical areas....

Read abstract  Full text PDF

Journal Article 2014; 35(1): 1-6 PubMed PMID: 24625909 Citation  Keywords:  Attention Deficit Disorder with Hyperactivity:complications, Central Nervous System Stimulants:adverse effects, Child, Child Development:drug effects, Growth:drug effects, Humans,.   

: Attention deficit hyperactivity disorder (ADHD) is one of the most commonly diagnosed childhood psychiatric disorders. It is manifested in every part of an affected child's behavior, with multiple symptomatology and heterogenous etiology. Published studies report that ADHD children may show changes in growth and development. Most of the studies on ADHD have been focused on connections between medication and growth changes and describe growth delays associated with medication. However, recent research results point to the low significance of the changes accompanying pharmacological treatment. Changes in growth may not only be a secondary effect of the treatment, but may also be specific characteristics of ADHD....

Read abstract  Full text PDF

Journal Article 2013; 34(5): 388-394 PubMed PMID: 23922046 Citation  Keywords:  Adolescent, Child, Child, Preschool, Female, Ghrelin:blood, Humans, Leptin:blood, Male, Nephrotic Syndrome:blood,.   

OBJECTIVE: The aim of the present study was to evaluate the serum and urinary levels of leptin and ghrelin in children with primary idiopathic nephrotic syndrome (NS), to compare these results between patients during the relapse and remission phase and to evaluate the possible role of leptin and ghrelin in the pathogenesis of NS.

PATIENTS AND METHODS: Forty-nine children with primary idiopathic NS (25 children with relapse and 24 children in remission), who were followed up at the Pediatric Nephrology Unit, enrolled. Twenty-eight age- and sex-matched healthy children served as controls. Serum and urinary leptin levels were determined by immunoenzymatic ELISA, and serum and urinary ghrelin levels were determined by the RIA method.

RESULTS: The serum leptin levels were significantly lower in the children with NS during the relapse phase than in the children with NS during remission or in the controls (1.42±0.34 ng/dl and 3.60±0.70 ng/ml; p<0.01, 1.42±0.34 ng/ml and 5.27±4.67 ng/ml; p<0.001, respectively). The urinary leptin excretion levels were significantly higher in the relapse group than in the controls (0.40±0.11 ng/ml and 0.12±0.06 ng/ml, p<0.01, respectively). The serum ghrelin levels were similar between the study groups (p>0.05). The urinary ghrelin excretion levels were significantly higher in the relapse group than in the remission group and the controls (965.0 pg/ml [93-3711] and 679.7 pg/ml [93-3783], p<0.05; 965.0 pg/ml [93-3711] and 387.7 pg/ml [114-1214], p<0.001, respectively). The urinary ghrelin levels were also significantly higher in the remission group than in the controls (679.7 pg/ml [93-3783] and 387.7 pg/ml [114-1214]), p<0.01, respectively). The serum leptin levels were positively correlated with the serum albumin levels (r=0.440, p<0.05) and were negatively correlated with the serum triglyceride levels during the relapse phase. The urinary leptin and ghrelin levels were positively correlated with proteinuria in the relapse group.

CONCLUSIONS: We propose that leptin plays a role in the pathophysiology of NS and is associated with proteinuria, hypoproteinemia and hyperlipidemia. The significant urinary excretion of ghrelin in children with NS is possibly due to underlying pathophysiological changes, and normal serum ghrelin levels might be associated with an unknown compensatory mechanism....

Read abstract  Full text PDF

Clinical Trial 2013; 34(3): 222-228 PubMed PMID: 23685421 Citation  Keywords:  Adolescent, Body Height:drug effects, Child, Drug Monitoring:methods, Female, Growth Disorders:drug therapy, Human Growth Hormone:blood, Humans, Insulin-Like Growth Factor Binding Protein 3:blood, Insulin-Like Growth Factor I:metabolism, Male, Predictive.   

BACKGROUND: Insulin-like growth factor-I (IGF-I) generation test has been introduced for the assessment of growth hormone (GH) sensitivity, however, its significance in predicting growth response to GH therapy has also been brought up. The molar ratio of IGF-I to its binding protein-3 (IGFBP-3) determines IGF-I bioavailability.

AIMS: Evaluation of usefulness of IGF-I and IGFBP-3 generation test in predicting the effectiveness of rhGH therapy in children with short stature.

PATIENTS AND METHODS: The analysis comprised 60 children with short stature, normal results of GH stimulating tests but decreased IGF-I secretion. In all the patients, GH insensitivity was excluded on the basis of IGF-I and IGFBP-3 generation test. Next, GH therapy was administered and height velocity (HV), together with IGF-I and IGFBP-3 secretion, was assessed every year, during 3 years. The comparative group consisted of 30 children with partial GH deficiency (pGHD).

RESULTS: Both IGF-I secretion and IGF-I/IGFBP-3 molar ratio increased significantly during generation test (p<0.05) and - further - during GH therapy (however insignificantly), together with at least doubling of pretreatment HV. There was no significant difference between the studied group of patients and children with pGHD.

CONCLUSIONS: Significant increase of IGF-I in generation test speaks for GH therapy effectiveness in short children, despite normal results of GH stimulating tests.

...

Read abstract  Full text PDF

Journal Article 2013; 34(3): 212-216 PubMed PMID: 23685419 Citation  Keywords:  Adolescent, Asthma:blood, Body Mass Index, Child, Cross-Sectional Studies, Disease Progression, Female, Humans, Hypersensitivity:epidemiology, Male, Resistin:blood, Risk Factors, Severity of Illness Index, Sex Distribution,.   

OBJECTIVES: There are limited data on the role of adipokines in atopic asthma.

DESIGN AND SETTING: To determine serum levels of resistin in asthmatic children in relation to body weight, asthma severity and gender, serum resistin (RES) levels were measured using ELISA in 89 asthmatic children (61 boys and 28 girls, aged 7.0-17.0 years) and in 33 healthy children. Among examined asthmatics 59 (19 girls and 40 boys) had normal weight (ANW) and 30 (9 girls and 21 boys) were obese (AO).

RESULTS: The mean serum levels of resistin were significantly (p<0.01) higher in all non-obese asthmatic children (4.11±0.1 ng/mL) than in healthy children (3.83±0.1 ng/mL). After stratifying by gender only ANW boys and AO boys had significantly higher RES levels than boys from control group. Both AO (4.4±0.2 ng/mL) and ANW girls (4.38±0.2 ng/mL) as well as girls from control (4.09±0.1) group showed significantly higher mean RES serum concentrations than boys from corresponding groups (3.99±0.1 ng/ml, 3.83±0.17 ng/ml and 3.44±0.06 ng/ml, respectively). No relationship between examined adipokine levels and asthma severity, spirometric parameters, degree of allergic sensitization, BMI, BMI-SDS was stated.

CONCLUSION: Increased serum RES in children with atopic asthma suggest that this adipokine may be implicated in its pathogenesis....

Read abstract  Full text PDF