scholarly journals Are Current Normal Values of 11DOC Useful for Diagnosis of Non Classical Congenital Adrenal Hyperplasia Due to 11β- Hydroxylase Deficiency?

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A709-A709
Author(s):  
Galia Barash ◽  
Lior Drach ◽  
Larisa Naugolni ◽  
Tamar Yacoel ◽  
Tzvi Tzvi Bistritzer ◽  
...  

Abstract Background: A non-classic form of 11β-hydroxylase deficiency (NC 11β-OHD) has been reported to cause mild androgen excess, with a clinical presentation of precocious puberty, menstrual cycle abnormalities, or hirsutism during adolescence. Since genetic diagnosis of NC 11βOHD is yet not routinely available, the current gold standard for biochemical diagnosis is elevated 11 DOC levels after corticotropin stimulation test (ACTHstimT). However, there are no clear hormone level cutoffs. One of the accepted references for basal and stimulated levels for the pediatric population was published in 1991 by Lashansky et al1. Aim: To determine the correlation between 11DOC levels measured during ACTHstimT, clinical symptoms attributed to NC11βOHD and androgen levels at presentation, and long-term follow-up among children and adolescents with hyperandrogenism. Methods: a retrospective study including all patients who underwent ACTHstimT between 20072015, in one center, during which 11 DOC levels were routinely measured as part of the test. Clinical data was collected from the patients’ medical files and, by telephone calls for complete long-term follow-up. 11DOC levels before and after ACTHstimT were categorized as elevated according to both pre-defined cut-offs; greater than 1.5 times the 95th percentile according to Lashansky1 normal level for sex and age, and greater than 1.5 times the upper limit of the normal level of the commercial kit. Results: Data were complete at presentation for 136 patients, 92 females, and for long for 98 patients, 68 females, mean follow up duration of 3.1 years (1.37,5.09). There was no statistically significant difference in the number of cases with elevated 11DOC according to both cut-offs, among patients with precocious and early puberty, premature adrenarche nor acne. Higher baseline and stimulated 11 DOC levels were demonstrated in females who presented with mild hirsutism and regular menses. Long term data demonstrated no statistically significant difference in the number of cases with elevated 11DOC levels among patients with compromised final adult height, PCOS or hyperandrogenism. There was negative correlation between stimulated 11 DOC levels and basal levels of testosterone, androstenedione and DHEAS levels. Conclusions: This report demonstrates that the current interpretation of 11DOC levels, basal and ACTHstimulated in children, according to 1.5 times the highest range, of both, the Lashansky1 acceptable norms for children, and some of the laboratory’s kit, are not clinically applicable.1Lashansky G, Saenger P, Fishman K, Gautier T, Mayes D, Berg G and Reiter E. Normative data for adrenal steroidogenesis in a healthy pediatric population: Age- and sex-related changes after adrenocorticotropin stimulation. J. Clin. Endocrinol. Metab. 1991; 73(3): 674-686.

2021 ◽  
Vol 10 (7) ◽  
pp. 1336
Author(s):  
Toshifumi Takahashi ◽  
Shinya Somiya ◽  
Katsuhiro Ito ◽  
Toru Kanno ◽  
Yoshihito Higashi ◽  
...  

Introduction: Cystine stone development is relatively uncommon among patients with urolithiasis, and most studies have reported only on small sample sizes and short follow-up periods. We evaluated clinical courses and treatment outcomes of patients with cystine stones with long-term follow-up at our center. Methods: We retrospectively analyzed 22 patients diagnosed with cystine stones between January 1989 and May 2019. Results: The median follow-up was 160 (range 6–340) months, and the median patient age at diagnosis was 46 (range 12–82) years. All patients underwent surgical interventions at the first visit (4 extracorporeal shockwave lithotripsy, 5 ureteroscopy, and 13 percutaneous nephrolithotripsy). The median number of stone events and surgical interventions per year was 0.45 (range 0–2.6) and 0.19 (range 0–1.3) after initial surgical intervention. The median time to stone events and surgical intervention was 2 years and 3.25 years, respectively. There was a significant difference in time to stone events and second surgical intervention when patients were divided at 50 years of age at diagnosis (p = 0.02, 0.04, respectively). Conclusions: Only age at a diagnosis under 50 was significantly associated with recurrent stone events and intervention. Adequate follow-up and treatment are needed to manage patients with cystine stones safely.


1991 ◽  
Vol 9 (5) ◽  
pp. 736-740 ◽  
Author(s):  
L E Spitler

We conducted a long-term follow-up (median, 10.5 years) of patients included in a randomized trial of levamisole versus placebo as surgical adjuvant therapy in 203 patients with malignant melanoma. Of the patients randomized, 104 received levamisole, and 99 received placebo. The results show that there is no difference between the treatment and control groups with regard to any of the three end points analyzed. These included disease-free interval, time to appearance of visceral metastasis, and survival. Moreover, there was no significant difference between the treatment and control groups after adjusting for age, sex, or stage of disease.


2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Joanna Wojtasik-Bakalarz ◽  
Zoltan Ruzsa ◽  
Tomasz Rakowski ◽  
Andreas Nyerges ◽  
Krzysztof Bartuś ◽  
...  

The most relevant comorbidities in patients with peripheral artery disease (PAD) are coronary artery disease (CAD) and diabetes mellitus (DM). However, data of long-term follow-up of patients with chronic total occlusion (CTO) are scarce. The aim of the study was to assess the impact of CAD and DM on long-term follow-up patients after superficial femoral artery (SFA) CTO retrograde recanalization. In this study, eighty-six patients with PAD with diagnosed CTO in the femoropopliteal region and at least one unsuccessful attempt of antegrade recanalization were enrolled in 2 clinical centers. Mean time of follow-up in all patients was 47.5 months (±40 months). Patients were divided into two groups depending on the presence of CAD (CAD group: n=45 vs. non-CAD group: n=41) and DM (DM group: n=50 vs. non-DM group: n=36). In long-term follow-up, major adverse peripheral events (MAPE) occurred in 66.6% of patients with CAD vs. 36.5% of patients without CAD and in 50% of patients with DM vs. 55% of non-DM subjects. There were no statistical differences in peripheral endpoints in both groups. However, there was a statistically significant difference in all-cause mortality: in the DM group, there were 6 deaths (12%) (P value = 0.038). To conclude, patients after retrograde recanalization, with coexisting CTO and DM, are at higher risk of death in long-term follow-up.


2020 ◽  
Vol 72 (1) ◽  
Author(s):  
Mohammad Abdallah Eltahlawi ◽  
Abdel-Aziz Fouad Abdel-Aziz ◽  
Abdel-Salam Sherif ◽  
Khalid Abdel-Azeem Shokry ◽  
Islam Elsayed Shehata

Abstract Background We hypothesized that 1st generation everolimus-eluting bioresorbable vascular scaffold (BVS) stent associated with less complication and less restenosis rate than everolimus-eluting stent (EES) in chronic total occlusion (CTO) recanalization guided by intracoronary imaging. Therefore, we aimed to assess the safety and performance of BVS stent in CTO revascularization in comparison to EES guided by intracoronary imaging. Our prospective comparative cross-sectional study was conducted on 60 CTO patients divided into two groups according to type of stent revascularization: group I (EES group): 40 (66.7%) patients and group II (BVS group): 20 (33.3%) patients. All patients were subjected to history taking, electrocardiogram (ECG), echocardiography, laboratory investigation, stress thallium study to assess viability before revascularization. Revascularization of viable CTO lesion guided by intracoronary imaging using optical coherence tomography (OCT). Then, long-term follow-up over 1 year clinically and by multi-slice CT coronary angiography (MSCT). Our clinical and angiographic endpoints were to detect any clinical or angiographic complications during the follow-up period. Results At 6 months angiographic follow-up, BVS group had not inferior angiographic parameters but without statistically significant difference (p = 0.566). At 12 months follow-up, there was no difference at end points between the two groups (p = 0.476). No differences were found at angiographic or clinical follow-up between BVS and EES. Conclusion This study shows that 1st generation everolimus-eluting BVS is non-inferior to EES for CTO revascularization. Further studies are needed to clearly state which new smaller footprint BVS, faster reabsorption, magnesium-based less thrombogenicity, and advanced mechanical properties is under development. We cannot dismiss the efficacy and safety of new BVS technology. Trial registration ZU-IRB#2498/3-12-2016 Registered 3 December 2016, email: [email protected]


2021 ◽  
Vol 12 ◽  
Author(s):  
Mohit Agrawal ◽  
Kanwaljeet Garg ◽  
Raghu Samala ◽  
Roopa Rajan ◽  
Vikas Naik ◽  
...  

Background: Magnetic resonance guided focused ultrasound (MRgFUS) is a relatively novel technique to treat essential tremor (ET). The objective of this review was to analyze the efficacy and the safety profile of MRgFUS for ET.Methods: A systematic literature review was done. The post procedure changes in the Clinical Rating Scale for Tremor (CRST) score, hand score, disability and quality of life scores were analyzed.Results: We found 29 studies evaluating 617 patients. DTI based targeting was utilized in six cohorts. A significant difference was observed in the pooled standard mean difference between the pre and postoperative total CRST score (p-value < 0.001 and 0.0002), hand score (p-value 0.03 and 0.02); and the disability at 12 months (p-value 0.01). Head pain and dizziness were the most in procedure complications. The immediate pooled proportion of ataxia was 50%, while it was 20% for sensory complications, which, respectively, declined to 31 and 13% on long term follow up. A significant reduction (p = 0.03) in immediate ataxia related complications was seen with DTI targeting.Conclusion: MRgFUS for ET seems to be an effective procedure for relieving unilateral tremor. Use of DTI based targeting revealed a significant reduction in post procedure ataxia related complications as compared to traditional targeting techniques. Analysis of other complications further revealed a decreasing trend on follow up.


Author(s):  
David Zadok ◽  
Isaac Avni ◽  
Erez Bakshi ◽  
Irina S Barequet ◽  
Isaac Aizenman ◽  
...  

ABSTRACT Purpose To report refractive, topographic and safety outcomes of corneal cross-linking (CXL) in patients younger than 18 years of age with progressive keratoconus. Materials and methods In this retrospective study, we enrolled 31 eyes of 21 children aged 11 to 17 years that underwent corneal riboflavin-ultraviolet A induced CXL due to progressive keratoconus at three different ophthalmology departments in Israel. They were followed for 3 to 48 months (average 23 ± 13.6 months). Evaluated parameters were uncorrected visual acuity (UCVA), best spectacle corrected visual acuity (BSCVA), manifest refraction, pachymetry, slit- lamp examination and corneal topography at baseline and at 1,3,6,12,24 and 48 months. Results We found a nonsignificant improvement in UCVA and BSCVA with a small reduction of manifest cylinder and no significant change in spherical equivalent or K-values. Following CXL, stability of UCVA and BSCVA at the last follow-up examination was found in 71 and 77% of treated eyes, respectively. No permanent adverse events have been recorded throughout the study period. Conclusion In our series, CXL was a safe procedure in the pediatric population. Stabilization of progressive keratoconus was achieved in visual acuity, refractive and topography parameters with no improvement in corneal indices in contrary to adult CXL treatment. How to cite this article Bakshi E, Barequet IS, Aizenman I, Levinger S, Avni I, Zadok D. Corneal Corss-linking in Patients Younger than 18 Years: Long-term Follow-up in Three Israeli Medical Centers. Int J Kerat Ect Cor Dis 2014;3(2):84-87.


Neurology ◽  
2012 ◽  
Vol 79 (15) ◽  
pp. 1607-1614 ◽  
Author(s):  
A. Radtke ◽  
M. von Brevern ◽  
H. Neuhauser ◽  
T. Hottenrott ◽  
T. Lempert

2020 ◽  
Vol 65 (4) ◽  
pp. 144-148
Author(s):  
Radhakrishnan Ganesh ◽  
James Lucocq ◽  
Neville Ogbonnia Ekpete ◽  
Noor Ul Ain ◽  
Su Kwan Lim ◽  
...  

Background and aim COVID-19 pandemic has predisposed patients undergoing surgery to post-operative infection and resultant complications. Appendicitis is frequently managed by appendicectomy. After the onset of the pandemic, selected cases of appendicitis were managed with antibiotics which is a recognised treatment option. Our objective was to compare the management of appendicitis and post-operative outcomes between pre- and post-COVID-19. Methods Ninety-six patients were identified from before the onset of the pandemic (November 2019) to after the onset of the pandemic (May 2020). Data were collected retrospectively from electronic records including demographics, investigations, treatment, duration of inpatient stay, complications, readmissions and compared between pre- and post-COVID-19 groups. Results One hundred percent underwent surgical treatment before the onset of pandemic, compared with 56.3% from the onset of the pandemic. A greater percentage of patients were investigated with imaging post-COVID-19 (100% versus 60.9%; p < 0.00001). There was no significant difference in the outcomes between the two groups. Conclusion CT/MRI scan was preferred to laparoscopy in diagnosing appendicitis and conservative management of uncomplicated appendicitis was common practice after the onset of pandemic. Health boards can adapt their management of surgical conditions during pandemics without adverse short-term consequences. Long term follow-up of this cohort will identify patients suitable for conservative management.


1993 ◽  
Vol 11 (8) ◽  
pp. 1523-1528 ◽  
Author(s):  
F B Stehman ◽  
B N Bundy ◽  
G Thomas ◽  
H M Keys ◽  
G d'Ablaing ◽  
...  

PURPOSE Long-term follow-up data of a randomized trial that compared hydroxyurea and the hypoxic-cell radiosensitizer to misonidazole as adjuncts to standard radiation therapy in locally advanced carcinoma of the cervix are reported. PATIENTS AND METHODS Three hundred eight women were entered, and all 294 eligible patients are assessable as randomized. Eighty-one percent of patients have been monitored for 5 years or to death. RESULTS There was an advantage for hydroxyurea in progression-free interval and survival (P = .05 and P = .066, respectively). There was no significant difference in the distribution of sites of failure between the regimens. For the 39% of patients with stages III to IVA disease, the advantage in progression-free interval for hydroxyurea was significant (47.8% v 33.6%). More leukopenia occurred on the hydroxyurea regimen than on the misonidazole regimen. CONCLUSION In summary, these data provide stronger evidence than our previous analysis that hydroxyurea is superior to misonidazole as an adjunct to radiation therapy. For patients with locally advanced carcinoma of the cervix, hydroxyurea continues to be the adjunct of choice with radiation.


1994 ◽  
Vol 12 (9) ◽  
pp. 1778-1782 ◽  
Author(s):  
M Omne-Pontén ◽  
L Holmberg ◽  
P O Sjödén

PURPOSE To evaluate, in a long-term follow-up study of consecutive patients (N = 99), the impact of surgery--breast-conserving treatment (BCT) versus mastectomy (MT)--on psychosocial adjustment among women with breast cancer, pTNM stage I/II. PATIENTS AND METHODS Semistructured interviews were conducted at a median of 6 years (range, 5.8 to 8.1) after primary surgery. Sixty-six women were available for the long-term follow-up study. Twenty-six women had been treated with BCT and 40 with MT. RESULTS No statistically significant differences were found between the two groups concerning psychosocial adjustment, as measured by the Social adjustment Scale (SAS). In general, the levels of maladjustment were lower than at 13 months postoperatively, but 10% still showed maladjustment. Sixty percent of the women were unwilling to show themselves naked, and 22% felt that they had become less attractive because of the surgical treatment. In an explorative part of the interview, 68% of the women complained about how they had been informed of the diagnosis. A tendency toward a significant difference was found in the relation between previous maladjustment and a negative experience at the time of diagnosis (P = .07). CONCLUSION Few data are available on long-term follow-up results with regard to psychosocial adjustment among women after breast cancer surgery. This study provides the important information that there are no differences in patient psychosocial adjustment that can be ascribed to the type of surgery at 6-year follow-up evaluation.


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