scholarly journals Growth Outcome and Metabolic Profile of PWS Patients Treated With GH and Differences Between AGA and SGA Group

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A677-A678
Author(s):  
Ju Young Yoon ◽  
Chong Kun Cheon ◽  
Seok Dong Yoo
Keyword(s):  
Glucose Level ◽  
Uniparental Disomy ◽  
Metabolic Dysfunction ◽  
Duration Of Treatment ◽  
Gh Treatment ◽  
Laboratory Findings ◽  
Growth Impairment ◽  
Growth Profile ◽  
Metabolic Dysfunctions ◽  
Mean Glucose

Abstract Objective: Prader-Willi syndrome (PWS) is a complex genetic disease associated with growth impairment, severe obesity and metabolic dysfunctions. High proportion of PWS patients are born small for gestational age (SGA), which also increase the risk of growth impairment and metabolic dysfunction. The aim of this study was to describe growth outcome and and metabolic profiles in GH treated PWS patients. We also investigated the differences in clinical outcomes between AGA and SGA group Methods: Data of 55 children and adults with genetically verified PWS aged more than 2 years old (32 male and 23 female, age 2-18.8 years) from single center were studied. Only patients who were treated with GH were included. The clinical characteristics and laboratory findings were reviewed retrospectively. Results: Among 55 subjects, 39 had 15q11-13 deletion and 16 had uniparental disomy (UPD). Twenty (36.3%) were born SGA. All patients received GH treatment, and 11 (20%) discontinued GH treatemnt. Mean age at GH treatment initiation was 2.5 (range 0.3-12.4) years, and mean duration of treatment was 6.3 (range 1.0-11.3) years. Current height-SDS (-0.36 vs -0.16) and BMI-SDS (1.44 vs 1.33) did not differ between AGA and SGA group. Two patients in SGA group, but none in AGA group had diabetes mellitus. Mean glucose level was also higher in SGA group (100.1 vs 114.4 mg/dL) Conclusions: Our report gives an overview of growth profile and metabolic dysfunctions recorded in GH treated PWS patients. Growth profile did not differ between AGA and SGA group. Glucose level was higher in SGA group, so more careful monitoring and prevention for DM will be required in SGA group.

Safety and effectiveness of growth hormone therapy in infants with Prader-Willi syndrome younger than 2 years: a prospective study

2019 ◽  
Vol 32 (8) ◽  
pp. 879-884 ◽  
Author(s):  
Raquel Corripio ◽  
Carla Tubau ◽  
Laura Calvo ◽  
Carme Brun ◽  
Núria Capdevila ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Prospective Study ◽  
Mixed Model ◽  
Standard Deviation Score ◽  
Uniparental Disomy ◽  
Chromosome 15 ◽  
Prader Willi Syndrome ◽  
Gh Treatment ◽  
Maternal Uniparental Disomy ◽  
A Prospective Study

Abstract Background There is little evidence of the effects of early treatment with growth hormone (GH) in infants with Prader-Willi syndrome (PWS). A prospective study was conducted to assess the safety of GH therapy in infants younger than 2 years of age with PWS. Methods A total of 14 patients with PWS started treatment with GH under the age of 2 years and were followed over a 2-year period. A deletion of chromosome 15 was present in nine infants (64.3%) and maternal uniparental disomy 15 in five infants (35.7%). The median age at start of GH treatment was 9.6 months (interquartile range [IQR] 9.0–18.3 months). Changes in height standard deviation score (SDS), body mass index (BMI) SDS and subcapsular and tricipital skinfolds in the follow-up period were evaluated with a mixed-model regression analysis using the Package R. Results There were no fatal adverse events. A significant decrease (p < 0.001) in tricipital and subcapsular skinfold thickness, with an upward trend of height SDS and a downward trend of BMI SDS, was observed. Infants who started GH before 15 months of age started walking at a median of 18.0 [17.0–19.5] months vs. 36.6 [36.3–37.8] months for those who began treatment with GH after 15 months of age (p = 0.024). Conclusions GH treatment in infants with PWS less than 2 years of age is safe and improved body composition. Infants who received GH before the age of 15 months started to walk earlier.

scholarly journals 249. Evaluation of 99 Radiologically-proven Osteomyelitis Cases

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S232-S232
Author(s):  
Olcay Buse Kenanoğlu ◽  
Gunel Quliyeva ◽  
Tansu Yamazhan ◽  
Bilgin Arda ◽  
Meltem Taşbakan ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Clinical Response ◽  
Clinical Laboratory ◽  
Clinical Signs ◽  
Surgical Debridement ◽  
Purulent Discharge ◽  
Resonance Imaging ◽  
Duration Of Treatment ◽  
Laboratory Findings

Abstract Background Herein we aimed to evaluate osteomyelitis cases in our setting. Methods We evaluated the hospital records of patients with osteomyelitis between January 2013 and December 2020 retrospectively. Osteomyelitis was confirmed by direct radiography or magnetic resonance imaging or pathology. Demographic features, risk factors, clinical/laboratory findings, treatment response and mortality rates were evaluated. Clinical response was defined as (resolution of clinical signs including fever and purulent discharge and other symptoms) and/or negative culture at the end of antimicrobial therapy. Results Patients were 33 female, aged 29–85 years (mean 59±12.6). Fourty nine of the patients were diabetic foot infection, 30 were spondylodiscitis, eight were primary, seven were post-traumatic, and five were post-surgical osteomyelitis. Overall 62 patients had diabetes mellitus and 16 patients had chronic renal failure. Peripheral arterial disease, neuropathy, diabetic retinopathy and venous insufficiency rate in the DM subgroup is shown in table. Fever was present in 24.2% of the cohort. İncreasing of CRP was in 95,9%, erythrocyte sedimentation rate in 83,9%, and leukocytosis in 37.3%. The radiological findings of osteomyelitis were detected via magnetic resonance imaging in 73 patients. Etiology in biopsy cultures were elucidated in 59.5% and the most common pathogen was S. aureus (30%) Table1. The most common empirical treatment regimens were tigecycline in 27 patients, ampicillin/sulbactam in 19 patients and ceftriaxone+teicoplanin in 12 cases. Duration of treatment was 36,2±17.3 days (range 6-104 days). Overall, clinical response was obtained in 91.9%. Fifty patients were performed surgical procedure + antibacterial treatment, clinical response was 96% (p:0.091). Surgical debridement could be performed in 22 patients, clinical response was obtained in all (p:0.193). Thirteen patients developed recurrence within one year. Sixty-seven patients received oral consecutive treatment after discharge. In hospital mortality rate was 2/99 (2,02%). Conclusion Despite surgical debridement and/or developed antimicrobial treatment, approximately 1/5 of osteomyelitis cases required further treatment Further interventions seem to be needed to reach better outcomes. Disclosures All Authors: No reported disclosures

scholarly journals Dysregulation of macrophage PEPD in obesity determines adipose tissue fibro-inflammation and insulin resistance

2020 ◽  
Author(s):  
Vanessa Pellegrinelli ◽  
Segio Rodriguez-Cuenca ◽  
Christine Rouault ◽  
Hanna Schilbert ◽  
Sam Virtue ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Adipose Tissue ◽  
Inflammatory Responses ◽  
Vital Role ◽  
Metabolic Dysfunction ◽  
Collagen Turnover ◽  
Metabolic Complications ◽  
Genetic Ablation ◽  
Metabolic Dysfunctions ◽  
Inflammatory Macrophages

Abstract Fibrosis is a hallmark of adipose tissue (AT) dysfunction and obesity-associated insulin resistance that results from an impaired collagen turnover. Peptidase D (PEPD) plays a vital role in collagen turnover by degrading proline-containing dipeptides. Nevertheless, its specific function and importance in AT is unknown. GWAS identified the rs731839 variant in the locus near PEPD that uncouples obesity from insulin resistance and dyslipidaemia, thus indicating that defective PEPD might impair AT remodelling and exacerbate metabolic complications. Here we show that in human and murine obesity, PEPD expression and activity decrease in AT, coupled to the release of PEPD systemically. Both events, in turn, are associated with the accumulation of fibrosis in AT and insulin resistance. Using pharmacologic and genetic animal models of PEPD down-regulation, we show that whereas dysfunctional PEPD activity provokes AT fibrosis, it is the PEPD secreted by AT the main contributor to inflammation, insulin resistance and metabolic dysfunction. Also, PEPD originated in inflammatory macrophages (Mɸ), plays an essential role promoting fibro-inflammatory responses via activation of EGFR in Mɸ and preadipocytes. Using genetic ablation of pepd in Mɸ that prevents obesity-induced PEPD release, also averts AT fibro-inflammation and obesity-associated metabolic dysfunctions. Taking advantage of factor analysis, we have identified the coupling of prolidase decreased activity and increased systemic levels of PEPD as the essential pathogenic triggers of AT fibrosis and insulin resistance. Thus, PEPD produced by Mɸ qualifies as a biomarker of AT fibro-inflammation and a therapeutic target for AT fibrosis and obesity-associated insulin resistance and type 2 diabetes.

scholarly journals Differences in glucose level between right arm and left arm using continuous glucose monitors

2020 ◽  
Vol 6 ◽  
pp. 205520762097034
Author(s):  
Nicole Kim ◽  
Kevin Pham ◽  
Allen Shek ◽  
Jeremy Lim ◽  
Xiaohan Liu ◽  
...  
Keyword(s):  
Glucose Level ◽  
Skin Irritation ◽  
Glucose Monitoring ◽  
Target Range ◽  
Glucose Levels ◽  
Significant Difference ◽  
Freestyle Libre ◽  
The Right ◽  
Mean Glucose ◽  
Time In Target Range

Background Continuous glucose monitoring (CGM) measures interstitial glucose levels through a sensor with a thin filament inserted under the skin. It is customary for patients to rotate sensor application sites between arms to minimize skin irritation. However, there is limited data regarding the degree of inter-arm differences with CGM technology. Methods Self-proclaimed right-handed (n = 5) and left-handed (n = 5) participants, regardless of concurrent comorbidities, were enrolled for CGM. Participants wore a FreeStyle Libre Pro sensor on each arm for a maximum of 14 days. Muscle mass and body fat analysis was conducted using a multi-frequency segmental body composition analyzer. Glucose levels from both arms were time-matched with the first 12 hours eliminated from analysis. Mean glucose and time in target range were compared between readings from the right and left arm. Results A total of 9830 paired glucose levels were included for analysis. In all participants (n = 10), mean glucose on the right arm was 89.1 mg/dL (SD, 19.9) and 85.3 mg/dL (SD, 19.3) on the left arm (P < 0.001). Glucose was out of target range (70-180 mg/dL) for 12.7% of the time in the right arm compared to 18.5% in the left arm (P < 0.001). Conclusions In a group of 10 nondiabetic and diabetic adults, there was a statistically significant difference in CGM readings between the right and left arms. Time in target range may differ based on arm selection when using a CGM. Arm dominance did not explain the inter-arm glucose level discordance.

Brucellosis: A Retrospective Evaluation

2003 ◽  
Vol 33 (3) ◽  
pp. 151-153 ◽  
Author(s):  
Meltem Işikgöz Taşbakan ◽  
Tansu Yamazhan ◽  
Deniz Gökengin ◽  
Bilgin Arda ◽  
Mahmut Sertpolat ◽  
...  
Keyword(s):  
Diagnostic Techniques ◽  
Test Results ◽  
Duration Of Treatment ◽  
Laboratory Findings ◽  
Treatment Regimens ◽  
Unpasteurized Milk ◽  
Clinical Presentations ◽  
History Of ◽  
Almost All ◽  
Antimicrobial Regimen

One hundred and sixty-six presumed brucellosis patients were included in the study. These patients were classified as primary (91), relapse (18) and suspected (57) cases according to their clinical presentations, and serologic and microbiologic test results. Primary and relapse cases were evaluated retrospectively according to age, sex, residence, routes of transmission, clinical and laboratory findings, treatment regimens, duration of treatment, and relapse rates. Of the 109 primary and relapse patients, 57 were male and 52 female. The ages of the patients ranged between 16–75 (mean age 40.2). The percentages of the urban and rural residence of the patients were 41.3% and 58.7%, respectively. The most common mode of transmission was consumption of unpasteurized milk and milk products (67.9%). Malaise, fever and sweating were the most frequently observed symptoms (96.3%, 95.4%, 91.7%, respectively). The most common signs were fever (97.2%), splenomegaly (59.6%), and hepatomegaly (37.6%). The liver was the most frequently involved organ (21.1%). Almost all (99.1%) patients were serologically positive. However, the positivity rate of culture was low (15.6%). The most frequently preferred antimicrobial regimen was rifampin and doxycycline combination. The relapse rate was 8.3%. Brucellosis is still prevalent in Turkey as in many other countries in the Mediterranean basin. The clinical presentation of the disease may show regional variations. Patients with a history of occupational or nutritional contact with the bacterium and with a compatible clinical picture should be examined using appropriate diagnostic techniques before any attempt to prescribe an antimicrobial.

scholarly journals Impact of growth hormone treatment on children’s height

2014 ◽  
Vol 54 (6) ◽  
pp. 318
Author(s):  
Nur Rochmah ◽  
Muhammad Faizi
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Growth Hormone Treatment ◽  
Body Height ◽  
Hormone Treatment ◽  
Post Treatment ◽  
Hormone Deficiency ◽  
Duration Of Treatment ◽  
Height Measurement ◽  
Gh Treatment

Background The use of growth hormone (GH) is a routinetreatment for growth hormone deficiency (GHD), small forgestational age (SGA), and Turner syndrome (TS). During thetreatment, height measurement at regular intervals is a vital stepto assess success. To date, there have been no previous studieson GH treatment in Dr. Soetomo Hospital, Surabaya, the referralhospital in East Indonesia.Objective To compare body height between pre- and post-growthhormone treatment in pediatric patients.Method This study was a non-randomized, pre-post clinical trialperformed at Dr. Soetomo Hospital, Surabaya. The prospectivecohort was accessed during January 2008-June 2013. Theinclusion criteria was GH treatment for more than 3 months.Clinical data on GH treatment, including diagnosis, age, heightpre-and post-treatment, height gain, duration of treatment, andparental satisfaction were collected. Two-tailed, paired T-test andPearson’s test were used for statistical analyses.Result Nineteen patients underwent GH treatment during thestudy period, but only twelve patients had complete data and wereincluded in the study. Eight subjects were female. Subjects’ meanage was 11 (range 8-15) years. Nine patients had GHD, 2 hadTS, and 1 had SGA. Mean pre-treatment height was 121.05 cm,while mean post-treatment height was 130.5 cm. Mean durationof treatment was 10.5 (range 3-30) months. Mean height gainwas 0.8 cm/month in GHD and SGA cases, and 0.78 cm/monthfor the TS cases. Eleven parents reported satisfaction with theresults of GH treatment in their children. There is significantdiffrent between pre- and post-treatment (P=0.001). Pearson’scorrelation test (r=0.90) revealed a strong correlation betweengrowth hormone treatment and height gain.Conclusion Growth hormone treatment has impact on heights inGH defficiency, Turner syndrome, and small for gestational age.

Blood Glucose Variability: A Strong Independent Predictor of Neurological Outcomes in Aneurysmal Subarachnoid Hemorrhage

2016 ◽  
Vol 33 (3) ◽  
pp. 189-195 ◽  
Author(s):  
Tomoya Okazaki ◽  
Toru Hifumi ◽  
Kenya Kawakita ◽  
Hajime Shishido ◽  
Daisuke Ogawa ◽  
...  
Keyword(s):  
Blood Glucose ◽  
Subarachnoid Hemorrhage ◽  
Glucose Level ◽  
Neurological Outcome ◽  
Independent Predictor ◽  
Aneurysmal Subarachnoid Hemorrhage ◽  
Glucose Variability ◽  
Independent Effect ◽  
Neurological Outcomes ◽  
Mean Glucose

Purpose: In patients with aneurysmal subarachnoid hemorrhage (SAH), increased glucose variability (GV) is associated with increased mortality and cerebral infarction; however, there are no reports demonstrating an association between GV and neurological outcome. This study investigated whether GV had an independent effect on neurological outcomes in patients with SAH in the intensive care unit. Materials and Methods: Consecutive adult patients hospitalized with SAH between January 1, 2009, and May 31, 2015 (N = 122) were retrospectively reviewed. Univariate/multivariate analyses were performed to identify independent predictors of poor neurological outcome. Patients were divided according to the mean glucose level (80-139 vs 140-200 mg/dL) and further subdivided using quartiles (Q) of the standard deviation (SD, representing variability) of the glucose level (Q1, Q2 + 3, and Q4). Results: Unfavorable neurological outcomes occurred in 44.2% of the patients. On multiple regression analysis, age, Hunt and Kosnik grade, SD of glucose (odds ratio [OR], 1.09; 95% confidence interval [CI], 1.02-1.17; P < .01), and minimum blood glucose level (OR, 0.95; 95% CI, 0.91-0.99; P < .01) were significantly associated with unfavorable neurological outcomes. Both groups (mean glucose levels: 80-139 and 140-200 mg/dL groups) had increasing unfavorable neurological outcomes with increasing SD of glucose (Q1, 15.0%; Q2 + 3, 40.0%; Q4, 52.4% and Q1, 44.4%; Q2 + 3, 50%; Q4, 88.9% in the 80-139 and 140-200 mg/dL groups, respectively). Patients with minimum glucose of <90 mg/dL comprised >50% of unfavorable neurological outcome. Conclusion: Increased GV was an independent predictor of unfavorable neurological outcomes in patients with SAH.

scholarly journals Beneficial Effects of Growth Hormone Treatment on Cognition in Children with Prader-Willi Syndrome: A Randomized Controlled Trial and Longitudinal Study

2012 ◽  
Vol 97 (7) ◽  
pp. 2307-2314 ◽  
Author(s):  
Elbrich P. C. Siemensma ◽  
Roderick F. A. Tummers-de Lind van Wijngaarden ◽  
Dederieke A. M. Festen ◽  
Zyrhea C. E. Troeman ◽  
A. A. E. M. (Janielle) van Alfen-van der Velden ◽  
...  
Keyword(s):  
Randomized Controlled Trial ◽  
Longitudinal Study ◽  
Cognitive Functioning ◽  
Cognitive Skills ◽  
Controlled Trial ◽  
Block Design ◽  
Uniparental Disomy ◽  
Prader Willi Syndrome ◽  
Gh Treatment ◽  
Randomized Controlled

Abstract Background: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited. Methods: Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores. Results: During the randomized controlled trial, mean sd scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest sd scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean sd scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P &lt; 0.0001) sd scores. Conclusions: Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment.

scholarly journals Preweaning Growth Hormone Treatment Ameliorates Adipose Tissue Insulin Resistance and Inflammation in Adult Male Offspring Following Maternal Undernutrition

Endocrinology ◽  
2013 ◽  
Vol 154 (8) ◽  
pp. 2676-2686 ◽  
Author(s):  
C. M. Reynolds ◽  
M. Li ◽  
C. Gray ◽  
M. H. Vickers
Keyword(s):  
Gene Expression ◽  
Growth Hormone ◽  
Adipose Tissue ◽  
Insulin Receptor ◽  
Glucose Uptake ◽  
Saline Control ◽  
Low Grade ◽  
Adult Offspring ◽  
Metabolic Dysfunction ◽  
Gh Treatment

Abstract It is well established that early-life nutritional alterations lead to increased risk of obesity and metabolic disorders in adult life. Although it is clear that obesity gives rise to chronic low-grade inflammation, there is little evidence regarding the role of inflammation in the adipose tissue of undernourished (UN) offspring. GH reduces fat mass and has antiinflammatory properties. The present study examined the effect of maternal UN on adipose inflammation in adult offspring and whether GH treatment during a critical period of developmental plasticity could ameliorate metabolic dysfunction associated with a poor start to life. Sprague Dawley rats were assigned to chow (C) or UN (50% ad libitum; UN) diet throughout gestation. Male C and UN pups received saline (control saline [CS]/UN) or GH (2.5 μg/g/d; control growth hormone [CGH]/undernourished growth hormone [UNGH]) from days 3–21. Postweaning males were further randomized and fed either chow or high-fat diet until day 160. An ex vivo glucose uptake assay demonstrated adipose tissue from UN offspring displayed attenuated insulin-stimulated glucose uptake compared with CS, CGH, and UNGH. This was associated with increased insulin receptor, glucose transporter 4, and insulin receptor substrate 1 gene expression. Furthermore, UN demonstrated enhanced TNFα and IL-1β secretion from adipose explants and stromal vascular fraction cultures accompanied by increased adipose tissue gene expression of several key proinflammatory genes and markers of macrophage infiltration. Overall, UN offspring displayed a more potent immunophenotype, which correlated with decreased insulin sensitivity. Preweaning GH treatment negates these detrimental effects, indicating the potential for reversing metabolic dysfunction in UN adult offspring.

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