Clinical outcome in a series of pediatric patients with congenital generalized lipodystrophies treated with dietary therapy

2018 ◽  
Vol 31 (1) ◽  
pp. 77-83 ◽  
Author(s):  
Laura Papendieck ◽  
María B. Araujo

AbstractBackground:Congenital generalized lipodystrophy (CGL) produces clinical features with severe metabolic consequences. Research has focused on measuring the response to the drugs. Nevertheless, there are no studies on the response to dietary therapy. The aim of this study was to show the clinical response to early nutritional intervention to modify or reverse metabolic effects in pediatric patients with CGL.Methods:A retrospective study was conducted on patients with CGL followed up between January 2003 and June 2017. After diagnosis, patients were indicated a diet with a total calorie intake according to the reference daily intake (RDI), without fast sugars. Clinical feature and laboratory tests (insulin, glucose, A1C, lipid panel, liver and kidney function tests), and complementary studies (abdominal ultrasonography, echocardiogram), at the first visit and during follow-up were recorded. Statistics, version 10, was used for analysis.Results:Eight patients were included. The median age at the first visit was 1.1 years (range, 0.28–9.9 years) and the median time of follow-up was 3.9 years (range, 0.4–9.47 years). The median initial triglyceride level was 516 mg/dL (range, 327–3590 mg/dL) p=0.014; median low-density lipoprotein (LDL) was 157 mg/dL (range, 94–370 mg/dL) p=0.03; median glycemia was 84 mg/dL (range, 63–126 mg/dL) p=0.02; median insulin was 28.84 µUI/mL (range, 3.9–116); median homeostatic model assessment-insulin resistance (HOMA-IR) was 5.3 (range, 1.21–23.2). After 3–6 months of diet, the median percentage of triglyceride decrease was 79.5% (range, 47–97%), LDL 44% (range, 20.5–59%), glycemia 8.8% (range, 0–53.1%), insulin 67.8% (range, 0–92.8%) p=0.02 and HOMA-IR was 81.5% (range, 50–98%) p=0.05. As of the last follow-up visit, in none of the patients, liver ultrasonography or echocardiogram had deteriorated.Conclusions:In these pediatric patients with CGL, a diet adjusted to RDI with restriction of fast sugars led to a marked improvement in metabolic parameters.

2019 ◽  
Vol 37 (05) ◽  
pp. 475-482 ◽  
Author(s):  
Brian M. Casey ◽  
Madeline Murguia Rice ◽  
Mark B. Landon ◽  
Michael W. Varner ◽  
Uma M. Reddy ◽  
...  

Abstract Objective The main purpose of this article is to evaluate whether identification and treatment of women with mild gestational diabetes mellitus (GDM) during pregnancy affects subsequent maternal body mass index (BMI), anthropometry, metabolic syndrome, and risk of diabetes. Study Design This is a follow-up study of women who participated in a randomized controlled treatment trial for mild GDM. Women were enrolled between 5 and 10 years after their index pregnancy. Participants underwent blood pressure, height, weight, and anthropometric measurements by trained nursing personnel using a standardized approach. A nurse-assisted questionnaire regarding screening and treatment of diabetes or hypercholesterolemia, diet, and physical activity was completed. Laboratory evaluation included fasting serum glucose, fasting insulin, oral glucose tolerance test, and a lipid panel. Subsequent diabetes, metabolic syndrome, obesity, and adiposity in those diagnosed with mild GDM and randomized to nutritional counseling and medical therapy (treated) were compared with those who underwent routine pregnancy management (untreated). Multivariable analyses were performed adjusting for race/ethnicity and years between randomization and follow-up visit. Results Four-hundred fifty-seven women with mild GDM during the index pregnancy were included in this analysis (243 treated; 214 untreated) and evaluated at a median 7 years after their index pregnancy. Baseline and follow-up characteristics were similar between treatment groups. Frequency of diabetes (9.2 vs. 8.5%, p =0.80), metabolic syndrome (32.2 vs. 34.3%, p =0.63), as well as adjusted mean values of homeostasis model assessment for insulin resistance (2.5 vs. 2.3, p =0.11) and BMI (29.4 vs. 29.1 kg/m2, p =0.67) were also not different. Conclusion Identification and treatment of women with mild GDM during pregnancy had no discernible impact on subsequent diabetes, metabolic syndrome, or obesity 7 years after delivery.


2013 ◽  
Vol 18 (4) ◽  
pp. 292-297 ◽  
Author(s):  
Brooke L. Honey ◽  
Lourdes Ramos ◽  
Nancy C. Brahm

OBJECTIVES The aim of this retrospective study was to identify the frequency of recommended metabolic monitoring and follow-up in pediatric patients on second-generation antipsychotic (SGA) medications from a pediatric clinic. METHODS A retrospective review of electronic medical records of all patients on antipsychotics from an academic medical center pediatric clinic was conducted. Inclusion criteria required patients to be established members of the pediatric clinic, < 19 years of age, and on ≥ 1 SGA for at least 1 year, regardless of medical diagnosis. Data collection consisted of patient demographic information and frequency of family history, vital signs, and recommended laboratory monitoring. RESULTS A total of 67 patients on antipsychotics were identified. After the application of inclusion criteria, 32 patients qualified for review. The average age was 13.5 ± 4 years and gender distribution included 72% males. Only 4 (13%) patients had documented baseline monitoring that included weight, blood pressure, and fasting lipid panel. No patient had a fasting plasma glucose recorded at any point during antipsychotic therapy. Follow-up monitoring decreased over time, with the exception of quarterly weight and annual blood pressure. CONCLUSIONS The results of this study highlight the lack of baseline and periodic monitoring that occur when pediatric patients are prescribed antipsychotic medications, putting the patient at risk for adverse events. The marked increase in antipsychotic prescribing and concerns related to their safety emphasize the need for improvement in monitoring of antipsychotic medications. This gap in patient care and safety opens an excellent opportunity for a clinical pharmacy team to provide education and assistance with SGA monitoring for the purpose of providing optimal patient care.


2016 ◽  
Vol 11 (2) ◽  
pp. 284-293 ◽  
Author(s):  
Magdalena Gibas-Dorna ◽  
Monika Szulińska ◽  
Piotr Turkowski ◽  
Justyna Kupsz ◽  
Anna Sowińska ◽  
...  

The aim of the current study was to examine the liposuction-induced metabolic changes with regard to release of major adipokines and insulin sensitivity in overweight male patients. Seventeen overweight male patients aged 37.15 ± 9.60 years (6 with diabetes type 2, 11 without comorbidities) and 10 age-matched healthy lean controls were enrolled in the study. Using Vibration Amplification of Sound Energy at Resonance System, ultrasound assisted liposuction was applied onto the deep layers of abdominal subcutaneous adipose tissue. The mean volume supranatant fat was 2208 ± 562 ml. To eliminate the confounding effects of postsurgical inflammation and to evaluate delayed metabolic effects, fasting blood was collected on the day of liposuction, within 1 to 2 months and more than 6 months after surgery. Serum leptin, soluble receptor for leptin, adiponectin, insulin, and glucose concentrations were tested and insulin sensitivity was calculated using updated model Homeostasis Model Assessment 2. Both treatment groups (diabetic and nondiabetic patients) experienced similar postsurgical weight reduction with concomitant lowering of body mass index value at 1 to 2 months follow-up, which was sustained after 6 months from surgery. Improvement in insulin sensitivity at 1 to 2 months follow-up was observed ( p = .017 and p = .002, for diabetics and nondiabetics, respectively) and this change persisted over the next 4 months. At the same time, no significant changes in adipokines and soluble leptin receptor were found. These data demonstrate that in terms of metabolic consequences, Vibration Amplification of Sound Energy at Resonance abdominal liposuction might have beneficial effects in overweight diabetic and nondiabetic males by improving their insulin sensitivity.


2015 ◽  
Vol 8 (1) ◽  
pp. 6-11 ◽  
Author(s):  
M Abu Sayeed ◽  
Akhter Banu ◽  
Khaleda Khatun ◽  
Parvin Akter Khanam ◽  
Tanjima Begum ◽  
...  

The prevalence of non-communicable diseases (NCD) like diabetes, hypertension, dyslipidemia and atherosclerotic cardiovascular diseases (CVD) are on the increase globally and predominantly in the South East Asian Region (SEAR). The increasing NCD and its complications burdened the health cost of Bangladesh. The available literatures suggest that edible mushrooms are effective in controlling metabolic risks like hyperglycemia and hypercholesterolemia.The study addressed the metabolic effects of edible oyster mushroom (Pleurotus ostreatus) in diabetic individuals and to assess the undesirable effects of mushroom.A total of 5000 newly registered diabetic women were screened for eligible participants (urban housewives, age 30 – 50y, BMI 22 – 27, FBG 8 – 12 mmol/l; free from complications or systemic illnesses and agreed to adhere to the study for 360 days). The investigations included weight and height for BMI, waist- and hip-girth for WHR, BP, FBG, 2ABF, T-chol, TG, HDL, LDL, ALT and Creatinine starting from the day 0 (baseline) and each subsequent follow-up days: 60, 120, 180, 240, 300 and 360 for comparison between placebo and mushroom groups and also within group (baseline vs. follow up days), individually for placebo and mushroom. The daily intake of mushroom was 200g for the mushroom group and an equivalent calorie of vegetables for the placebo group.Overall, 73 diabetic housewives (mushroom / placebo = 43 /30) volunteered. The mean (with SEM) values of BMI, WHR, BP, FBG, 2ABF, T-chol, TG, HDL, LDL, ALT and Creatinine of the placebo group were compared with the mushroom group. Compared with the placebo, the mushroom group showed significant reductions of FBG (p<0.001), 2ABF (p<0.001), T-chol (p<0.001), TG (p=0.03) and LDL (p<0.001); whereas, no difference was observed for BMI, SBP, DBP, HDL, Hb, creatinine and ALT. The comparison within groups (baseline vs. follow-up) there were significant reduction of these variables in mushroom but not in the placebo group.Mushroom was found to have significant effect in reducing blood glucose, T-chol, TG and LDL. No impaired function was observed for liver, kidney and hemopoeitic tissue in taking mushroom for 360 days of the study period.Ibrahim Med. Coll. J. 2014; 8(1): 6-11


2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.


Author(s):  
Madeline B. Karsten ◽  
R. Michael Scott

Fusiform dilatation of the internal carotid artery (FDCA) is a known postoperative imaging finding after craniopharyngioma resection. FDCA has also been reported following surgery for other lesions in the suprasellar region in pediatric patients and is thought to be due to trauma to the internal carotid artery (ICA) wall during tumor dissection. Here, the authors report 2 cases of pediatric patients with FDCA. Case 1 is a patient in whom FDCA was visualized on follow-up scans after total resection of a craniopharyngioma; this patient’s subsequent scans and neurological status remained stable throughout a 20-year follow-up period. In case 2, FDCA appeared after resection and fenestration of a giant arachnoid cyst in a 3-year-old child, with 6 years of stable subsequent follow-up, an imaging finding that to the authors’ knowledge has not previously been reported following surgery for arachnoid cyst fenestration. These cases demonstrate that surgery involving dissection adjacent to the carotid artery wall in pediatric patients may lead to the development of FDCA. On very long-term follow-up, this imaging finding rarely changes and virtually all patients remain asymptomatic. Neurointerventional treatment of FDCA in the absence of symptoms or significant late enlargement of the arterial ectasia does not appear to be indicated.


1997 ◽  
Vol 73 (2) ◽  
pp. 75-79
Author(s):  
Sandra Maria Gonçalves Vieira ◽  
Carlos Kieling ◽  
Elza D. Mello ◽  
Carmen M. Santos ◽  
Carla Silveira ◽  
...  

Author(s):  
Tran Anh Quynh ◽  
Pham Duy Hien ◽  
Le Quang Du ◽  
Le Hoang Long ◽  
Nguyen Thi Ngoc Tran ◽  
...  

AbstractRobotic surgery offers three-dimensional visualization and precision of movement that could be of great value to gastrointestinal surgeons. There were many previous reports on robotic technology in performing Soave colonic resection and pull-through for Hirschsprung’s disease in children. This study described the follow-up of the Robotic-assisted Soave procedure for Hirschsprung’s disease in children. Robotic-assisted endorectal pull-through was performed using three robotic arms and an additional 5-mm trocar. The ganglionic and aganglionic segments were initially identified by seromuscular biopsies. The rest of the procedure was carried out according to the Soave procedure. We left a short rectal seromuscular sleeve of 1.5–2 cm above the dentate line. From December 2014 to December 2017, 55 pediatric patients were operated on. Age ranged from 6 months to 10 years old (median = 24.5 months). The aganglionic segment was located in the rectum (n = 38), the sigmoid colon (n = 13), and the left colon (n = 4). The mean total operative time was 93.2 ± 35 min (ranging from 80 to 180 min). Minimal blood was lost during the surgery. During the follow-up period, 41 patients (74.6%) had 1–2 defecations per day, 12 patients (21.8%) had 3–4 defecations per day, and 2 patients (3.6%) had more than 4 defecations per day. Fecal incontinence, enterocolitis, and mild soiling occurred in three (5.4%), four (7.3%), and two pediatric patients, respectively. Robotic-assisted Soave procedure for Hirschsprung’s disease in children is a safe and effective technique. However, a skilled robotic surgical team and procedural modifications are needed.


2021 ◽  
Vol 23 ◽  
pp. 100159
Author(s):  
Zemenay Ayinie Mekonnen ◽  
Debas Yaregal Melesse ◽  
Habitamu Getinet Kassahun ◽  
Tesera Dereje Flatie ◽  
Misganaw Mengie Workie ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Adam Jacobson ◽  
Carin Rojas ◽  
Brenda L. Bohnsack

Abstract Background Limited data exists on the effectiveness of the collagen matrix, Ologen, on increasing Ahmed glaucoma valve (AGV) success in childhood glaucomas. Methods Ocular examination and surgical details of pediatric patients who underwent AGV placement ± Ologen augmentation between 2012 and 2020. Complete success was defined as intraocular pressure (IOP) between 5 and 20 mmHg without glaucoma medications and additional IOP-lowering surgeries. Qualified success was defined as above, except IOP control maintained with or without glaucoma medications. Results Twenty-two eyes of 16 patients underwent AGV placement of which 6 eyes had Ologen-augmentation (OAGV) and 16 eyes had conventional surgery (CAGV). Average age was 6.4 ± 5.1 years with 4.2 ± 2.5 follow-up years. There was no difference in age, number of previous surgeries, and preoperative IOP and glaucoma medications. At final follow-up, success rate was 100% (5 eyes complete, 6 eyes qualified) in the OAGV group compared to 31% (0 eyes complete, 5 eyes qualified) in the CAGV group. One and two-year survival rates were 100% for OAGV compared to 62 and 38% for CAGV. Postoperative IOP was significantly lower at 1-month and final follow-up (p = 0.02) as was the number of glaucoma medications at 3, 6, 12-months and final follow-up (p < 0.05) in the OAGV group. Conclusions Ologen-augmentation increased the success and survival rates of AGVs in childhood glaucomas. Further, Ologen mitigated the hypertensive phase and decreased medication dependency. Longer follow-up with a greater number of eyes is required to fully evaluate the effectiveness of OAGV.


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