scholarly journals Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity

2021 ◽  
Vol 12 ◽  
Author(s):  
Marina Caputo ◽  
Tommaso Daffara ◽  
Simonetta Bellone ◽  
Valentina Mancioppi ◽  
Paolo Marzullo ◽  
...  
Keyword(s):  
Kinase Inhibitor ◽  
Sleep Apnea Syndrome ◽  
Genetic Obesity ◽  
Cyclin Dependent Kinase Inhibitor ◽  
Imprinting Disorder ◽  
Melanocortin 4 Receptor ◽  
First Case ◽  
Syndromic Obesity ◽  
Obstructive Sleep ◽  
Clinical Conditions

Genetic obesity, including syndromic and non-syndromic forms, represents a minority of cases compared to essential obesity but gene dysregulations lead to complex clinical conditions that make their management particularly difficult. Among them, Beckwith-Wiedemann syndrome (BWS) is a multisystem human genomic imprinting disorder characterized by overgrowth. We describe the first case of liraglutide treatment in an 18-year-old boy patient affected by BWS complicated by macroglossia, cryptorchidism, nephroblastoma, organomegaly, microscopic lymphocytic colitis, pharmacologically treated arterial hypertension, obesity, and obstructive sleep apnea syndrome. He presented a normal cognitive development. Body mass index at the time of first transition visit in the adult endocrinology department at the age of 18-years-old was 40.6 kg/m2 without glucose metabolism impairment. Lifestyle interventions failed because of poor compliance. During 20 months of 3.0 mg liraglutide treatment, a weight loss of 19 kg (−13.3%) and BMI reduction of 6.8 points were registered without side effects. To date, liraglutide treatment was effective on obesity in 7 subjects with Prader Willy Syndrome and 14 with melanocortin-4 receptor mutations. The efficacy of liraglutide in BWS could be related to a crosstalk among glucagon-like peptide (GLP)-1 system, mechanisms related to the cyclin-dependent kinase inhibitor 1C (CDKN1C), and dopamine mesolimbic circuit. Clinical trials aiming at a tailored medicine in genetic obesity are needed.

scholarly journals Operative Management of OSAS in a Complex Case of Proteus Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Elena Cantone ◽  
Michele Cavaliere ◽  
Giovanni Castagna ◽  
Anna Marino ◽  
Luigi Del Vecchio ◽  
...  
Keyword(s):  
Local Anesthesia ◽  
Sleep Apnea Syndrome ◽  
Operative Management ◽  
Complex Case ◽  
Proteus Syndrome ◽  
Body Parts ◽  
Craniofacial Malformations ◽  
Bipolar Radiofrequency ◽  
First Case ◽  
Obstructive Sleep

Obstructive sleep apnea syndrome (OSAS) is a common disorder in childhood with high prevalence in syndromic subjects with craniofacial malformations. Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils. We report a case of a 12-year-old boy diagnosed with Proteus Syndrome (PS) and suffering from OSAS due to asymmetric palatine tonsillar hypertrophy, treated with partial resection of left tonsil. To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed. Recovery of the obstructive respiratory disease was obtained. To our knowledge, this is the first case reported in the literature of partial tonsillar resection performed in a patient with PS suffering from OSAS under local anesthesia.

scholarly journals Clinical Conditions and Predictive Markers of Non-Dipper Profile in Hypertensive Patients

2018 ◽  
Vol 64 (1) ◽  
pp. 10-16 ◽  
Author(s):  
Ioan Tilea ◽  
Dorina Petra ◽  
Elena Ardeleanu ◽  
Adina Hutanu ◽  
Andreea Varga
Keyword(s):  
Blood Pressure ◽  
Current Knowledge ◽  
Blood Pressure Monitoring ◽  
Target Organ Damage ◽  
Sleep Apnea Syndrome ◽  
Predictive Markers ◽  
Distribution Width ◽  
Hypertensive Patients ◽  
Obstructive Sleep ◽  
Clinical Conditions

AbstractHypertension remains one of the primary causes of premature cardiovascular mortality representing a major independent risk factor.The importance of ambulatory blood pressure monitoring in clinical evaluation of hypertensive patients, beyond diagnosis, is the identification of circadian dipping/non-dipping profile. The non-dipper pattern in hypertensive and normotensive patients is associated with significant target organ damage and worse outcomes, as an increased cardiovascular risk condition. Non-dipping pattern has been found to be associated with specific clinical conditions. Obesity, diabetes mellitus, metabolic syndrome, obstructive sleep apnea syndrome, chronic kidney disease, autonomic and baroreflex dysfunctions, salt sensitivity, hormonal changes, gender and age were extensively studied. Research efforts are focused on recognizing and exploring predictive markers of abnormal blood pressure circadian pattern. Previous studies acknowledge that red cell distribution width, mean platelet volume, fibrinogen level, C-reactive protein, serum uric acid and gamma-glutamyltransferase, are independently significant and positive associated to non-dipping pattern. Moreover, research on new biomarkers are conducted: Chitinase 3-Like-Protein 1, atrial and B-type natriuretic peptide, brain-derived neurotrophic factor, chemerin, sphingomyelin and the G972R polymorphism of the insulin receptor substrate-1 gene. This review summarizes the current knowledge of different clinical conditions and biomarkers associated with the non-dipper profile in hypertensive patients.

scholarly journals Clinical conditions and memory complaints in outpatient elderly

2008 ◽  
Vol 2 (4) ◽  
pp. 315-320 ◽  
Author(s):  
Regina Miksian Magaldi ◽  
Alexandre Leopold Busse ◽  
Venceslau Antonio Coelho ◽  
Daniel Apolinário ◽  
Leonardo da Costa Lopes ◽  
...  
Keyword(s):  
Comprehensive Evaluation ◽  
Sleep Apnea Syndrome ◽  
Vitamin B12 Deficiency ◽  
Normal Pressure ◽  
Screening Tests ◽  
Cognitive Screening ◽  
Memory Complaints ◽  
Obstructive Sleep ◽  
B12 Deficiency ◽  
Clinical Conditions

Abstract Memory complaints are common in elderly, and may be associated with many clinical problems. Objective: To identify clinical conditions and possible factors related to memory complaints in elderly outpatients presenting at a tertiary unit. Methods: Patients with memory complaints and normal cognitive screening tests were submitted to clinical and laboratorial testing. Radiological evaluation was performed as needed for diagnosis. Results: One hundred and seventy-seven subjects were initially evaluated, 12 were excluded because of poor and inconsistent memory complaints. Of the remainder, seventeen had criteria for dementia diagnosis. Ninety-two (55.4%) had one or more comorbid conditions possibly related to their complaints. Major depression was present in 26.0%. Sixteen patients (9.6%) had vitamin B12 deficiency, 8 were in use of inappropriate medications, and 7 (4.2%) had hypothyroidism. Other conditions diagnosed were: generalized anxiety disorder, obstructive sleep apnea syndrome, hyperparathyroidism, normal pressure hydrocephalus. Three patients had severe hearing loss (in 22 with hearing complaints); one had severe visual impairment (in 22 with visual complaints). Conclusions: Comprehensive evaluation was able to identify treatable conditions possibly related to memory complaints.

scholarly journals Personalized treatment of obesity complicated with obstructive sleep apnea syndrome

2014 ◽  
Vol 11 (1) ◽  
pp. 48-52
Author(s):  
N V Strueva ◽  
L V Saveleva ◽  
G A Melnichenko ◽  
M G Poluektov ◽  
N V Gegel
Keyword(s):  
Obstructive Sleep Apnea ◽  
Body Weight ◽  
Sleep Apnea ◽  
Patient Adherence ◽  
Sleep Apnea Syndrome ◽  
Obstructive Sleep ◽  
Osa Treatment ◽  
Treatment Of Obesity ◽  
Clinical Conditions ◽  
The Impact

Obstructive sleep apnea (OSA) and obesity are mutually burdening clinical conditions. Weight loss is quite effective for the control of breathing disorders during sleep, but the impact of OSA treatment on the dynamics of body weight in obese patients remains poorly studied. Presented clinical case features a significant reduction in body weight in a patient with morbid obesity complicated by severe OSA with contemporary approach and without CPAP therapy due to high patient adherence to therapy and absence of comorbidities.

Postoperative monitoring of children with obstructive sleep apnea syndrome

2014 ◽  
Vol 155 (18) ◽  
pp. 703-707 ◽  
Author(s):  
Pálma Benedek ◽  
Gabriella Kiss ◽  
Eszter Csábi ◽  
Gábor Katona
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Obstructive Sleep Apnea Syndrome ◽  
Preoperative Evaluation ◽  
Sleep Apnea Syndrome ◽  
Study Patient ◽  
Severe Obstructive Sleep Apnea ◽  
Obstructive Sleep ◽  
Significant Difference ◽  
Apnea Syndrome

Introduction: Treatment of pediatric obstructive sleep apnea syndrome is surgical. The incidence of postoperative respiratory complications in this population is 5–25%. Aim: The aim of the authors was to present the preoperative evaluation and monitoring procedure elaborated in Heim Pál Children Hospital, Budapest. Method: 142 patients were involved in the study. Patient history was obtained and physical examination was performed in all cases. Thereafter, polysomnography was carried out, the severity of the obstructive sleep apnea syndrome was determined, and the patients underwent tonsilloadenotomy. Results: 45 patients with mild, 50 patients with moderate and 47 patients with severe obstructive sleep apnea syndrome were diagnosed. There was no complication in patients with mild disease, while complications were observed in 6 patients in the moderate group and 24 patients in the severe group (desaturation, apnea, stridor, stop breathing) (p<0.000). In patients with severe obstructive sleep apnea syndrome, no significant difference was noted in preoperative apnoea-hypapnea index (p = 0.23) and in nadir oxygen saturation values (p = 0.73) between patients with and without complication. Conclusions: Patients with severe obstructive sleep apnea syndrome should be treated in hospital where pediatric intensive care unit is available. Orv. Hetil., 2014, 155(18), 703–707.

Sign in / Sign up

Export Citation Format

Share Document