scholarly journals MULTIPLE SURGERIES AND LONG-TERM ENDOCRINE FOLLOW-UP IN MEN 2A SYNDROME

2015 ◽  
Vol 2 (4) ◽  
pp. 204-209
Author(s):  
Rene Baloescu ◽  
Mara Carsote ◽  
Simona Elena Albu ◽  
Ana Valea

MEN2A (Multiple Endocrine Neoplasia type 2) syndrome, an autosomal dominant condition, requires multiple surgeries in order to prevent or treat different tumours that are related to RET proto-oncogene mutations. Early intervention is most useful in cases with prior genetic confirmation. This is a case report presenting a long medical history of a female suffering of MEN2A syndrome. A 44-year-old woman, with a large family history of different endocrine cancers but no gene testing, is diagnosed at the age of 31 with a thyroid nodule of 1.7 centimeters (cm) which is removed by a partial thyroidectomy. Medullary thyroid cancer (MTC) is confirmed and calcitonin is found high. Total thyroidectomy is performed without consecutive normalization of calcitonin and secondary persistent post-operatory hypoparathyroidism. 6 months later a right laparoscopic adrenalectomy is performed for a pheochromocytoma (Pheo) of 5.2 cm. Despite normal levels of metanephrines and normetanephrines, a left adrenal tumor of 1.5 cm is discovered one year later. One decade later, the blood calcitonin progressively increased and 41 lymph nodes from latero-cervical areas and central neck compartment were removed. 14 of them had MTC metastases. 8 months later, typical endocrine profile suggested a Pheo and laparoscopic left adrenalectomy is performed with chronic adrenal insufficiency needing lifelong hydrocortisone and fludrocortisone replacements. The MEN2A syndrome-related multiple surgeries are correlated with long-term endocrine follow-up in challenging cases such as ours requiring a multi-disciplinary approach. The lack of gene mutation identification during childhood or at close relatives delays the diagnosis and thus the potential curative surgical removal of the thyroid and adrenal tumours with a more severe prognosis.

Author(s):  
Shinwan Kany ◽  
Johannes Brachmann ◽  
Thorsten Lewalter ◽  
Ibrahim Akin ◽  
Horst Sievert ◽  
...  

Abstract Background Non-paroxysmal (NPAF) forms of atrial fibrillation (AF) have been reported to be associated with an increased risk for systemic embolism or death. Methods Comparison of procedural details and long-term outcomes in patients (pts) with paroxysmal AF (PAF) against controls with NPAF in the prospective, multicentre observational registry of patients undergoing LAAC (LAARGE). Results A total of 638 pts (PAF 274 pts, NPAF 364 pts) were enrolled. In both groups, a history of PVI was rare (4.0% vs 1.6%, p = 0.066). The total CHA2DS2-VASc score was lower in the PAF group (4.4 ± 1.5 vs 4.6 ± 1.5, p = 0.033), while HAS-BLED score (3.8 ± 1.1 vs 3.9 ± 1.1, p = 0.40) was comparable. The rate of successful implantation was equally high (97.4% vs 97.8%, p = 0.77). In the three-month echo follow-up, LA thrombi (2.1% vs 7.3%, p = 0.12) and peridevice leak > 5 mm (0.0% vs 7.1%, p = 0.53) were numerically higher in the NPAF group. Overall, in-hospital complications occurred in 15.0% of the PAF cohort and 10.7% of the NPAF cohort (p = 0.12). In the one-year follow-up, unadjusted mortality (8.4% vs 14.0%, p = 0.039) and combined outcome of death, stroke and systemic embolism (8.8% vs 15.1%, p = 0.022) were significantly higher in the NPAF cohort. After adjusting for CHA2DS2-VASc and previous bleeding, NPAF was associated with increased death/stroke/systemic embolism (HR 1.67, 95% CI 1.02–2.72, p = 0.041). Conclusion Atrial fibrillation type did not impair periprocedural safety or in-hospital MACE patients undergoing LAAC. However, after one year, NPAF was associated with higher mortality. Graphic abstract


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
S Kany ◽  
J Brachmann ◽  
T Lewalter ◽  
I Akin ◽  
H Sievert ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Stiftung für Herzinfarkforschung Background  Non-paroxysmal (NPAF) forms of atrial fibrillation (AF) have been reported to be associated with an increased risk for systemic embolism or death compared with paroxysmal AF (PAF). This study investigates the procedural safety and long-term outcomes of left atrial appendage closure (LAAC) in patients with different forms of AF. Methods  Comparison of procedural details and long-term outcomes in patients (pts) with PAF against controls with NPAF in the prospective, multicentre observational registry of patients undergoing LAAC in Germany (LAARGE).  Results  A total of 638 pts (PAF 274 pts, NPAF 364 pts) were enrolled. NPAF consisted of 31.6% patients with persistent AF and 68.4% with longstanding persistent AF or permanent AF. In both groups, a history of PVI was rare (4.0% vs 1.6%, p = 0.066). The PAF group had significantly less history of heart failure (19.0% vs 33.0%, p < 0.001) while the current median LVEF was similar (60% vs 60%, p = 0.26). The total CHA2DS2-VASc score was lower in the PAF group (4.4 ± 1.5 vs 4.6 ± 1.5, p = 0.033), but no difference in the HAS-BLED score (3.8 ± 1.1 vs 3.9 ± 1.1, p = 0.40) was observed. The rate of successful implantation was equally high (97.4% vs 97.8%, p = 0.77) in both groups. In the three-month echo follow-up, device-related thrombi (2.1% vs 7.3%, p = 0.12) and peridevice leak >5 mm (0.0% vs 7.1%, p= 0.53) were numerically higher in the NPAF group. Overall, in-hospital complications occurred in 15.0% of the PAF cohort and 10.7% of the NPAF cohort (p = 0.12). In the one-year follow-up, unadjusted mortality (8.4% vs 14.0%, p = 0.039) and combined outcome of death, stroke and systemic embolism (8.8% vs 15.1%, p = 0.022) were significantly higher in the NPAF cohort. After adjusting for CHA2DS2-VASc and previous bleeding, NPAF was associated with increased death/stroke/systemic embolism (HR 1.67, 95%-CI: 1.02-2.72). Conclusion  Atrial fibrillation type did not impair periprocedural safety or in-hospital MACE of patients undergoing LAAC. However, after one year, NPAF was associated with higher mortality and combined outcome of death, stroke and systemic embolism.


1998 ◽  
Vol 4 (2) ◽  
pp. 151-157 ◽  
Author(s):  
Y. Niimi ◽  
U. Ito ◽  
O. Tone ◽  
K. Yoshida ◽  
S. Sato ◽  
...  

We present a rare case of multiple spinal perimedullary arteriovenous fistulae associated with the Parkes-Weber (PW) syndrome. A 31-year-old male known to have the PW syndrome involving the left leg since birth, presented with a 7-month-history of progressive myelopathy of the lower extremities and dysfunction of the bladder and bowel. Myelography demonstrated dilated intradural vessels. Angiography demonstrated two distinct single hole perimedullary arteriovenous fistulae near the conus at two different metameres. They were supplied by the left posterior spinal artery. The patient was treated by transarterial embolisation using polyvinyl alcohol particles, which resulted in venous side occlusion of the fistulae. After the treatment, the patient developed transient worsening of the spasticity of the lower extremities, and was treated by heparinization. After heparinization, the patient partially recovered from the pre-embolisation status of his myelopathy. The follow-up angiogram one year after the embolisation demonstrated persistent obliteration of both fistulae. At long-term follow-up, the patient can ambulate without assistance and work as a farmer.


1996 ◽  
Vol 13 (4) ◽  
pp. 145-148 ◽  
Author(s):  
Steven D. Baerg ◽  
David A. Russell ◽  
Laura M. LeVan ◽  
Carl A. Kirker-Head

A 22 year old thoroughbred gelding was presented for evaluation and treatment of chronic dental disease. The horse had a history of quidding and abnormal bit behavior. Intraoral examination revealed signs of chronic generalized gingival inflammation and severe dental caries affecting the maxillary and mandibular incisor teeth. Treatment was provided on two separate visits over an interval of four months. The first visit consisted of the surgical extraction of three unrestorable incisor teeth and restoration of six carious maxillary incisor teeth. The second visit consisted of conventional endodontic therapy on the remaining mandibular incisor teeth and the surgical removal of a chronic suppurative osteomyelitic lesion. Immediate and long term improvements in eating habits were noted. Three month follow-up examinations following completion of treatment have shown the teeth to be in functional position, the restorations intact, and the surgical site well healed.


1994 ◽  
Vol 07 (04) ◽  
pp. 173-176 ◽  
Author(s):  
J. C. Yovich ◽  
R. A. Read

SummaryAcute traumatic fracture of the me-dial coronoid process occurred in two adult dogs. The fracture was the sole injury in one dog, but was associated with further elbow trauma in the other. Surgical removal of the fractured portion of medial coronoid process resulted in a rapid return to normal elbow function which was sustained on long-term follow-up. Radiographs showed some osteophyte formation within the elbow joints at one year postoperatively. Two years later, repeat radiography of the elbow of one of the dogs did not show any further osteophyte production. In contrast, the surgical removal of fragmented coronoid processes frequently results in continuing lameness, reduced range of elbow movement and progressive osteoarthritis.Removal of a traumatically fractured portion of the medial coronoid process in two adult dogs resulted in a rapid return to soundness. Elbow function in both dogs remained normal on long-term follow-up.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nicolas Lellouche ◽  
Raphaele Arrouasse ◽  
Julien Ternacle ◽  
Romain Gallet ◽  
Jean-Sylvain Hermida ◽  
...  

Abstract Background Percutaneous left atrial appendage (LAA) closure is an alternative to oral anticoagulation (OAC) for atrial fibrillation (AF) patients with high thromboembolism risk, particularly with contraindications to OAC. The LAA itself could possess proarrhythmogenic properties. As patients undergoing LAA closure could be candidates for cardioversion or ablation, we aimed to evaluate AF disease progression following LAA closure and the outcome of patients undergoing a rhythm control strategy after the procedure. Methods The prospective multicenter French Nationwide Observational LAA Closure Registry (FLAAC) comprises 33 French interventional cardiology departments. Patients were included if they fulfilled the following criteria: history of non-valvular AF, successful LAA closure and long-term ECG follow-up. Results A total of 331 patients with successful LAA closure were enrolled in the study. Patients mean age was 75.4 ± 0.5 years. The study population was characterized by a high thromboembolic risk (CHA2DS2-VASc score: 4.5 ± 0.1) and frequent comorbidities. The median follow-up was 11.9 months. One hundred and nineteen (36.0%) patients were in sinus rhythm (SR) at baseline. Among SR patients, documented AF was observed in 16 (13.4%) patients whereas 15 (7.1%) patients in AF at baseline restored SR, at the end of follow up. Finally, only 13 patients (4%) underwent procedures to restore SR without complications during the follow-up. Conclusions The vast majority of patients undergoing LAA closure have the same AF status at baseline and one year after the index procedure. During the follow-up, a very small proportion (4%) of our population underwent procedures to restore SR without complications whatever the post-procedural antithrombotic strategy was.


2001 ◽  
Vol 87 (2) ◽  
pp. 104-108 ◽  
Author(s):  
Soichi Oya ◽  
Takamitsu Fujimaki ◽  
Akio Asai ◽  
Takaaki Kirino

We report on a patient with ependymoma who had a recurrence after long-term remission. The patient developed frontoparietal ependymoma at the age of one year and ten months. The tumor was radically removed and postoperative radiation therapy was performed. A calcified area adjacent to the area of surgical removal remained unchanged until the patient was 18 years old. The patient was healthy except for mild hemiparesis until an MRI scan performed when he was 25 years old showed regrowth of the tumor. The patient underwent surgery with additional radiation therapy and was discharged. The 23-year interval until tumor recurrence in this case is far beyond the so-called risk period of “Collins’ law”. Immunohistochemical study with MIB-1 and anti-p53 antibody showed a high proliferative potential of the primary and recurrent tumors and possible p53 mutation in the primary tumor. This is the first report to describe the detailed clinical course and histological features of a recurrent infantile ependymoma that progressed after Collins’ risk period. It seems that follow-up of ependymoma patients after initial treatment should be performed regularly for a longer period in cases showing radiological evidence of a residual lesion.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
L Fauchier ◽  
C Semaan ◽  
G Fauchier ◽  
J Herbert ◽  
T Genet ◽  
...  

Abstract Background Diabetes mellitus (DM) is a factor of increased mortality in patients with acute myocardial infarction (AMI). DM is also associated with a higher risk of heart failure (HF) in patients with coronary artery disease as in the general population. The aim of the present study was to assess the incidence of HF developing at the acute stage of MI and of HF occurring in the year following hospital discharge, according to presence of DM. We also assessed the association between DM, HF and long-term mortality in this AMI population. Methods We used the French administrative hospital-discharge database, including all patients without history of HF admitted for AMI between 2010 and 2019 (n=797,212, mean age 69 years, 66% male). Among them, 520,258 patients (65%) had ST-segment elevation myocardial infarction (STEMI), 276,954 (35%) had non-STEMI, 192,456 patients (24%) had a history of DM. Occurrence of HF during the initial hospital stay was analysed in the whole population. In patients without HF during the index hospitalisation, discharged and alive at day 8 (n=535,813), we collected all hospitalisations for HF occurring during the year after discharge and analysed subsequent long-term mortality in those alive at one year (n=270,534) (length of follow-up 2.0±2.5 years, median 0.9, IQR 0.1–3.5). Results Overall, DM patients were older than non-DM patients (71±12 vs 67±15 years) and had more frequent comorbidities. At the acute stage, DM was associated with a higher risk of HF (28.7% vs 20.5% adjusted OR 1.40, 1.38–1.42, p<0.0001). In patients without HF at the acute stage and discharged alive at day 8, DM was associated with a higher risk of being hospitalised with HF in the first year (5.6% vs 2.8%, adjusted HR 1.52, 1.49–1.56, p<0.0001). In patients alive at one year, rates of all-cause death per year during subsequent follow-up were 2.2% in those without DM or HF during the first year (reference), 3.4% in those with DM and no HF during the first year (adjusted HR 1.22, 1.18–1.25, p<0.0001), 7.7% in those without DM and with HF during the first year (adjusted HR 1.92, 1.83–2.02, p<0.0001) and 8.9% in those with DM hospitalised with HF during the first year (adjusted HR 2.23, 2.09–2.37, p<0.0001) (see figure). Conclusion After AMI, patients with diabetes are at increased risk of heart failure both at the acute stage and in the year following myocardial infarction, compared with non-diabetic patients. Non-fatal HF developing in the year following discharge is associated with noticeably higher subsequent mortality, and the combination of DM and HF is particularly at risk. Improved management is needed in diabetic patients following an AMI to avoid development of heart failure and its longer-term consequences. FUNDunding Acknowledgement Type of funding sources: None.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 47-48
Author(s):  
Ang Li ◽  
Hanqing Shang ◽  
Rohit Gupta ◽  
Chris Davis ◽  
Stephanie J. Lee ◽  
...  

Introduction: Thrombotic microangiopathy (TMA) is a known complication of allogeneic hematopoietic cell transplantation (HCT). Post-transplant TMA has been associated with acute kidney injury (AKI) and early mortality. However, the long-term kidney outcomes and survival in patients who recover from the disease have not been well characterized. Methods: We performed a retrospective cohort study of adult allogeneic HCT recipients transplanted during 2006-2015 who survived to one-year (index date) and had follow-up at the Long-Term Follow-Up (LTFU) clinic at the Fred Hutchinson Cancer Research Center (FHCRC). Patients were classified as TMA or non-TMA based on whether a diagnosis was made within one-year post-transplant (BBMT 2019;25:570). Outpatient creatinine values obtained during LTFU visits were assessed over time and averaged at the distinct years post-transplant. Estimated glomerular filtration rate (eGFR) was calculated using the CKD-EPI formula. Chronic kidney disease (CKD) was defined as eGFR <60 mL/min/1.73m2. Potential confounders included pre-HCT eGFR, prior autologous HCT, older age, female sex, black race, myeloablative conditioning (including high-dose total body irradiation), calcineurin/mTOR inhibitor exposure, development of AKI within 6 months, acute graft versus host disease (GVHD) within 6 months, and chronic GVHD within 12 months post-transplant. Pre-transplant hypertension and diabetes were not considered as confounders because they had no known association with TMA development. To assess the association between history of TMA and CKD over time among post-transplant survivors, generalized estimating equation (GEE) was used with exchangeable correlation, binomial family, and logit link, after adjustment for pre-index variables. GEE was chosen to model the longitudinal creatinine outcomes at discrete intervals and to help account for interval missingness. The adjusted odds ratio (OR), 95% confidence interval (CI), robust standard error (SE), and P-values were presented. Unadjusted Kaplan Meier (KM) analysis with landmark at 1 year was used to compare long-term overall survival. Results: Among 2091 patients that underwent first allogeneic HCT, we identified 1151 patients who had survived at least one-year and had available long-term follow-up data (Figure 1). Fifty-seven patients were survivors who had a history of TMA within one-year post-transplant and 1094 did not. Outpatient creatinine data were available in decreasing number of patients each year for the first 5 years post-transplant. The median eGFR over time for the two groups was shown in Figure 2. At one-year post-transplant, 52% of TMA survivors had CKD versus 27% of non-TMA survivors. After adjusting for other potential confounders, a history of TMA was associated with an odds ratio of 2.62 (95% CI 1.25-5.52) for CKD at one-year post-transplant (Table 1). There was no appreciable change in CKD status over time (non-significant interaction for TMA x year). The adjusted covariates had the expected magnitude and significance of association with CKD development, whereas age, pre-transplant eGFR, acute GVHD, and early AKI had the strongest association. While TMA was significantly associated with short-term mortality, there was no association between history of TMA and long-term overall survival in KM analysis landmarked beyond year one, where the conditional 5-year survival was 71% in the TMA survivors and 74% in the non-TMA survivors (log rank P= 0.113). Conclusions: In this study of 1151 post-transplant long-term survivors, we found that TMA survivors had higher risk of CKD post-transplant despite adjusting for key potential confounders. The overall eGFR had the largest decrease between pre-transplant and year-one post-transplant, with non-appreciable variation in subsequent years. While TMA patients were more likely to die early, in those who survived to one-year, their long-term mortality was similar to non-TMA patients. Limitations in the study include the lack of uniform follow-up for all transplant survivors and potentially unobserved confounders. Overall, our data suggest that TMA appears to be a time-limited systemic insult; although its damage to the kidney requires continued monitoring and management. Disclosures Lee: Amgen: Research Funding; Novartis: Research Funding; AstraZeneca: Research Funding; Kadmon: Research Funding; Incyte: Consultancy, Research Funding; Syndax: Research Funding; Pfizer: Consultancy, Research Funding; Takeda: Research Funding.


2010 ◽  
Vol 23 (02) ◽  
pp. 124-127 ◽  
Author(s):  
K. Hurter ◽  
N. Medl

SummaryTwo adult Domestic Shorthaired cats were presented with acute forelimb lameness. In one case, there was a history of trauma. A fracture of the anconeal process was diagnosed on the flexed mediolateral radiographs of the elbow in both cats. The fracture was accompanied by a bony avulsion of the tricipital tendon in one animal. Both cats underwent surgical removal of the fractured anconeal process. Follow-up radiographic evaluation was available for one cat six months after treatment; radiographs showed evidence of osteoarthritis. The long-term clinical outcome, based on owner telephone follow-up conducted four and nine years postoperatively, was considered to be satisfactory.


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