Your browser doesn't support javascript.
loading
: 20 | 50 | 100
1 - 20 de 83
1.
Neuropsychology ; 38(2): 134-145, 2024 Feb.
Article En | MEDLINE | ID: mdl-37883034

OBJECTIVE: Alexithymia, a deficit in identifying and describing feelings, is prevalent in traumatic brain injury (TBI). Sometimes referred to as "emotional unawareness," we sought to investigate whether alexithymia after TBI was related to, or distinct from, impaired self-awareness (ISA) and whether the two predicted differentiable emotional and aggression profiles. Further, the mediating role of frontal system behaviors (disinhibition, dysexecutive function, apathy) was explored. METHOD: Participants with TBI (N = 40) from diverse backgrounds completed self-report measures of alexithymia, emotional distress, aggression, and frontal system behaviors. For the assessment of ISA, significant other ratings were obtained to identify discrepancies from self-ratings. Data were analyzed quantitatively using independent samples t tests, correlations, partial correlations, and simple mediation. RESULTS: There was a negative correlation between alexithymia and ISA. Alexithymia, but not ISA, was associated with higher expressions of emotional distress and aggression even after controlling for the effects of ISA via partial correlations. Exploratory analyses found that frontal system behaviors mediated the relationships between alexithymia and aggression and alexithymia and emotional distress. CONCLUSIONS: Alexithymia is more accurately conceptualized as an emotional processing deficit than an awareness deficit. Indeed, self-awareness may be a prerequisite for the ability to identify alexithymic tendencies. Negative psychological effects of alexithymia are compounded by poorer executive function and disinhibition and call for the development of TBI-specific alexithymia screening tools and interventions. Alexithymia interventions are best delivered in conjunction with rehabilitation of emotion regulation and executive function. (PsycInfo Database Record (c) 2024 APA, all rights reserved).


Brain Injuries, Traumatic , Psychological Distress , Humans , Affective Symptoms/diagnosis , Affective Symptoms/etiology , Aggression , Emotions , Brain Injuries, Traumatic/complications , Brain Injuries, Traumatic/psychology
2.
Curr Opin Otolaryngol Head Neck Surg ; 32(2): 125-133, 2024 Apr 01.
Article En | MEDLINE | ID: mdl-38116795

PURPOSE OF REVIEW: The aim of this study was to provide a timely and relevant review of the latest findings and explore appropriate management of aggressive variants of papillary thyroid cancer (AVPTC). RECENT FINDINGS: In general, AVPTCs tend to exhibit more invasive characteristics, a lack of responsiveness to radioiodine, increased occurrences of regional spreading, distant metastases and higher mortality rates. Meanwhile, each variant showcases unique clinical and molecular profiles. SUMMARY: Given the elevated risk of recurrence postsurgery, a more aggressive strategy may be necessary when suspected preoperatively, particularly for those presenting with invasive features. Decision on the extent of surgical treatment and adjuvant therapy is individualized and made by experienced clinicians and multidisciplinary teams based on the clinical presentation, presence of aggressive features and molecular profile. Future studies on development of personalized medicine and molecular target therapy may offer tailored treatment options.


Carcinoma, Papillary , Thyroid Neoplasms , Humans , Thyroid Cancer, Papillary/therapy , Thyroid Neoplasms/therapy , Thyroid Neoplasms/pathology , Prognosis , Carcinoma, Papillary/therapy , Carcinoma, Papillary/pathology , Iodine Radioisotopes/therapeutic use
3.
Acta otorrinolaringol. esp ; 74(4): 203-210, Julio - Agosto 2023. ilus, tab
Article En | IBECS | ID: ibc-223478

Objective: To review the clinical presentation, diagnosis, pathology and management strategies in a modern cohort of patients with thyroglossal duct cyst carcinoma. Study design: Retrospective case series following PROCESS Guidelines. Setting Comprehensive cancer centre. Methods: Data recorded included: gender, age at diagnosis, clinical presentation, thyroid function, diagnostic investigations, cytological results, final histology, staging and follow up status. The risk of malignancy in cytological analysis was stratified according to the Royal College of Pathologists classification in United Kingdom. Results: Twelve patients were included. The majority of patients (66.7%) presented with an isolated thyroglossal duct cyst. Only 4 patients had preoperative cytological suspicion of carcinoma (sensitivity: 33.3%). At the time of presentation all patients were euthyroid. Following diagnosis of malignancy, a total thyroidectomy was performed in all patients, with the exception of 2, who had a thyroglossal duct cyst carcinoma of less than 10mm. Among the 10 patients who underwent total thyroidectomy, 7 (70%) patients had proven carcinoma in the thyroid gland, 3 with deposits of less than 10mm. The average size of the thyroid cancer deposits was 7.2mm (1–20mm). With a mean follow-up of is 44 months (5–120), all patients were alive and free of recurrence at the end of the study period. Conclusion: Thyroglossal duct cyst carcinoma is a rare condition and its management should be discussed in a multidisciplinary meeting. As with differentiated thyroid cancer originating in the thyroid gland, it bears extraordinary survival rates. Accordingly, the management of these cancers has shifted towards a more conservative approach although its peculiarities must be taken into account: ease of extracystic invasion and possible different lymph node invasion. (AU)


Objetivo: Revisar la presentación clínica, el diagnostico, la histología y las estrategias de tratamiento en una cohorte moderna de pacientes con carcinoma del conducto tirogloso. Diseño del estudio: Serie de casos retrospectiva utilizando PROCESS Guidelines. Localización: Unidad de cáncer de cabeza y cuello. Métodos: Los datos incluidos fueron: sexo, edad al diagnóstico, presentación clínica, función tiroidea, investigaciones diagnósticas, resultados citológicos, histología final, estadificación y estado durante el seguimiento. El riesgo de malignidad en el análisis citológico fue estratificado de acuerdo con la clasificación del Royal College of Pathologists del Reino Unido. Resultados: Se incluyeron 12 pacientes. La mayoría de ellos (66,7%) presentaron solamente un quiste del conducto tirogloso al diagnóstico. Solamente 4 pacientes tuvieron sospecha de malignidad de acuerdo con los resultados de la citología preoperatoria. En el momento de la presentación, todos los pacientes tenían función tiroidea normal. Después del diagnóstico, se realizó tiroidectomía total a todos los pacientes menos dos, que tuvieron carcinoma del conducto tirogloso menor de 10mm. Entre los 10 pacientes que recibieron tiroidectomía total, 7 (70%) sufrieron carcinoma en la glándula tiroides, 3 de ellos con depósitos menores de 10mm. El tamaño medio de los depósitos de carcinoma en la glándula tiroides fue de 7,2mm (1-20mm). Con una media de seguimiento de 44meses (5-120), todos los pacientes estaban vivos y libres de recidiva al final del periodo estudiado. Conclusión: El carcinoma del conducto tirogloso es una entidad poco frecuente y su manejo debe ser realizado por un equipo multidisciplinario. Igual que en el carcinoma diferenciado de tiroides que se origina en la glándula tiroides, las tasas de supervivencia son excelentes. ... (AU)


Humans , Male , Female , Adult , Middle Aged , Aged , Thyroglossal Cyst/diagnostic imaging , Thyroglossal Cyst/diagnosis , Thyroglossal Cyst/history , Thyroglossal Cyst/pathology , Thyroglossal Cyst/therapy , Head and Neck Neoplasms/diagnosis , Head and Neck Neoplasms/history , Head and Neck Neoplasms/surgery , Head and Neck Neoplasms/therapy , Cohort Studies , Age and Sex Distribution , Thyroid Function Tests , Neoplasm Metastasis , Cell Biology
4.
Article En | MEDLINE | ID: mdl-37479461

OBJECTIVE: To review the clinical presentation, diagnosis, pathology and management strategies in a modern cohort of patients with thyroglossal duct cyst carcinoma. STUDY DESIGN: Retrospective case series following PROCESS Guidelines. SETTING: Comprehensive cancer centre. METHODS: Data recorded included: gender, age at diagnosis, clinical presentation, thyroid function, diagnostic investigations, cytological results, final histology, staging and follow up status. The risk of malignancy in cytological analysis was stratified according to the Royal College of Pathologists classification in United Kingdom. RESULTS: Twelve patients were included. The majority of patients (66.7%) presented with an isolated thyroglossal duct cyst. Only 4 patients had preoperative cytological suspicion of carcinoma (sensitivity: 33.3%). At the time of presentation all patients were euthyroid. Following diagnosis of malignancy, a total thyroidectomy was performed in all patients, with the exception of 2, who had a thyroglossal duct cyst carcinoma of less than 10mm. Among the 10 patients who underwent total thyroidectomy, 7 (70%) patients had proven carcinoma in the thyroid gland, 3 with deposits of less than 10mm. The average size of the thyroid cancer deposits was 7.2mm (1-20mm). With a mean follow-up of is 44 months (5-120), all patients were alive and free of recurrence at the end of the study period. CONCLUSION: Thyroglossal duct cyst carcinoma is a rare condition and its management should be discussed in a multidisciplinary meeting. As with differentiated thyroid cancer originating in the thyroid gland, it bears extraordinary survival rates. Accordingly, the management of these cancers has shifted towards a more conservative approach although its peculiarities must be taken into account: ease of extracystic invasion and possible different lymph node invasion.


Carcinoma, Papillary , Carcinoma , Thyroglossal Cyst , Thyroid Neoplasms , Humans , Thyroglossal Cyst/surgery , Thyroglossal Cyst/diagnosis , Thyroglossal Cyst/pathology , Retrospective Studies , Carcinoma, Papillary/pathology , Thyroid Neoplasms/surgery
5.
Curr Opin Otolaryngol Head Neck Surg ; 31(2): 83-88, 2023 Apr 01.
Article En | MEDLINE | ID: mdl-36912220

PURPOSE OF REVIEW: To highlight recent advances in our understanding of the epidemiology, incidence, evaluation, management and outcomes of primary thyroid lymphoma (PTL), and highlight the indications and limitations of surgery. RECENT FINDINGS: The differential diagnosis of a rapidly enlarging thyroid mass with or without obstructive symptoms should include PTL and anaplastic thyroid cancer. When PTL is suspected, initial investigations should include blood tests and ultrasound-guided biopsy preferably core need biopsy to allow tissue typing and immunohistochemistry analysis. Systemic imaging with FDG PET-CT is required for staging. Surgery is not recommended for treatment purposes and should be reserved for diagnosis and airway management. Treatment includes chemotherapy and radiotherapy and offer an excellent prognosis. SUMMARY: PTL is a rare malignancy making diagnosis and management challenging. Initial investigations of suspected PTL should include blood tests and ultrasound-guided biopsy, preferably core needle biopsy and systemic imaging is required for staging. Surgery is reserved for diagnosis and airway management. Chemotherapy and radiotherapy are the treatment of choice.


Lymphoma , Thyroid Neoplasms , Humans , Positron Emission Tomography Computed Tomography , Thyroid Neoplasms/pathology , Lymphoma/diagnosis , Lymphoma/pathology , Diagnostic Imaging
6.
Thorac Surg Clin ; 32(4): 553-563, 2022 Nov.
Article En | MEDLINE | ID: mdl-36266040

The optimal management of Siewert Type II or Junction AEG II adenocarcinoma remains a point of debate. Surgical options include an extended total gastrectomy or esophagectomy. Accurately identifying the location of the esophagogastric junction (GEJ) is important as the epicenter of the lesion is defined in reference to the GEJ. Type II tumors, in the most recent iteration of the AJCC, describe these lesions as being within 1 cm cephalad and 2 cm caudal to GEJ. Accurate staging of the location and identification of nodal metastasis is vital to guide the optimal surgical approach. Endoscopy, endosonography, CT, and PET help guide decision-making as to what junctional subtype is present. The extent of resection and lymphadenectomy remains contestable. Both surgical approaches remain viable, as each has its own advantages and issues. The key to the management of these cancers is that the surgeon has the capability to operate on both sides of the diaphragm to manage these oftentimes challenging malignancies.


Adenocarcinoma , Esophageal Neoplasms , Stomach Neoplasms , Humans , Esophagectomy , Stomach Neoplasms/surgery , Stomach Neoplasms/pathology , Esophageal Neoplasms/surgery , Esophageal Neoplasms/pathology , Gastrectomy , Esophagogastric Junction/surgery , Esophagogastric Junction/pathology , Adenocarcinoma/surgery , Adenocarcinoma/pathology , Lymph Node Excision , Retrospective Studies
7.
J Foot Ankle Surg ; 61(4): 680-685, 2022.
Article En | MEDLINE | ID: mdl-35562304

Magnetic resonance imaging (MRI) is commonly used to evaluate soft tissue pathology of the foot and ankle. Prior investigations have reported limitations of this modality, however, in evaluation of pathologies related to the peroneal tendons. This article investigates the correlation of pre-operative MRI studies with intraoperative findings. Five board-certified radiologists interpreted MRIs of 80 ankles that subsequently underwent surgical procedures performed by one board-certified foot and ankle surgeon, after which comparison was made between their findings. Statistically significant disagreement was found between radiologist and surgeon findings of a normal peroneus brevis (PB), PB and peroneus longus (PL) tendinosis, PB and PL hypertrophy, PB and PL partial linear tears, PB and PL flattening, PB longitudinal split tears, and the PB attritional spectrum (combined analysis of flattening, partial linear tearing, and longitudinal split tears). These results suggest that given the disconcordance between MRI and intraoperative findings, surgeons should remain cautious in their reliance upon this imaging modality when evaluating this anatomic region.


Tendon Injuries , Ankle Joint , Humans , Magnetic Resonance Imaging/methods , Retrospective Studies , Rupture , Tendon Injuries/diagnostic imaging , Tendon Injuries/pathology , Tendon Injuries/surgery , Tendons/surgery
8.
Int J Endocrinol ; 2022: 7360282, 2022.
Article En | MEDLINE | ID: mdl-35465075

Objective: The insulin tolerance test (ITT) is the gold standard endocrine test used to assess the integrity of the growth hormone (GH) and cortisol axes. The ITT has potential risks, and severe hypoglycaemia may necessitate intravenous glucose rescue. There is no clear consensus as to the optimal insulin dose for the ITT. Therefore, we sought to compare the standard dose (0.15 U/kg) and a low-dose ITT (0.1 U/kg). Design: Single-centre audit of ITT data (2012-2021). Patients and Measurements. Patients who underwent an ITT to assess possible GH deficiency/adrenal insufficiency were included. Glucose, GH, and cortisol were measured at baseline and 30, 45, 60, 90, and 120 minutes following I.V. insulin bolus (0.15 U/kg or 0.10 U/kg). Results: Of the ITTs performed, only 3/177 (1.7%) did not achieve adequate hypoglycaemia (≤2.2 mmol/L) with a single insulin dose. In total, 174 patients (43.5 ± 12.1 yrs, mean ± standard deviation) were included for analysis (0.15 U/kg: n = 113, 0.10 U/kg: n = 61). All 174 subjects had adequate hypoglycaemia regardless of baseline fasting blood glucose level or insulin dose. Neither nadir glucose nor glucose delta (i.e., baseline minus nadir) differed between insulin doses. Trends in both cortisol and GH responses over time were similar between groups, and a greater proportion of patients receiving the standard dose had an adequate cortisol response (77/106 (72.6%) vs. 32/60 (53.3%), p=0.01). The rates of glucose rescue did not differ in a subset of 79 patients, with on-demand glucose rescue in 4/35 (11%) for the standard dose and 2/44 (5%) for the low dose (p=0.25). Conclusions: Our results suggest that the low-dose ITT produces comparable glucose, cortisol, and GH responses to the higher dose. Given the risks associated with hypoglycaemia, the low dose appears to be preferable to the standard dose ITT in most circumstances.

9.
Transplant Direct ; 8(4): e1284, 2022 Apr.
Article En | MEDLINE | ID: mdl-35317006

Background: Genetically determined hypoparathyroidism can lead to life-threatening episodes of hypocalcemia and, more rarely, to end-stage kidney disease at a young age. Parathyroid allotransplantation is the only curative treatment, and in patients already receiving immunosuppression for kidney transplantation, there may be little additional risk involved. We report the first such case in a child. Methods: An 11-y-old girl, known to have hypoparathyroidism secondary to an activating pathogenic variant in the calcium-sensing receptor, developed end-stage kidney disease and was started on intermittent hemodialysis. Since the age of 2.5 y, she had been receiving treatment with exogenous synthetic parathyroid hormone (PTH). In June 2019, at the age of 11.8 y, she received a living-donor kidney and simultaneous parathyroid gland transplant from her father. The kidney was implanted into the right iliac fossa, followed by implantation of the parathyroid gland into the exposed rectus muscle. Results: The kidney graft showed immediate function while the intrinsic serum PTH level remained low at 3 ng/L. Exogenous PTH infusion was reduced on day 6 posttransplantation to stimulate PTH production by the new gland, which resulted in improving intrinsic PTH concentrations of 28 ng/L by day 9. Twelve months after transplantation, PTH levels remain in normal range and the kidney graft function is stable with a serum creatinine of 110 µmol/L. Conclusions: Simultaneous living donation and transplantation of a kidney and a parathyroid gland into a child is safe and feasible and has the potential to cure primary hypoparathyroidism as well as kidney failure.

10.
Abdom Radiol (NY) ; 47(4): 1414-1424, 2022 04.
Article En | MEDLINE | ID: mdl-35157102

Bladder paragangliomas (bPGL) are rare neuroendocrine tumors arising from the sympathetic paraganglia present in the bladder wall. Bladder PGLs are typically submucosal or intramural but when subserosal may not be readily visible at cystoscopy. The average size at presentation is 3.9 cm (range 1.0-9.1 cm). When small, bPGL are usually spherical, well-marginated and homogeneous. Larger bPGL are typically more complex with peri- and intra-tumoral neovascularity and central necrosis. On ultrasound, increased color Doppler signal is typical. The increased soft tissue resolution of MRI enables localization of bPGL within the bladder wall more accurately than CT. Restricted diffusion and avid contrast enhancement help differentiate small bPGLs from leiomyomas, which have similar appearances on ultrasound and CT. Nuclear medicine techniques identify bPGLs and their metastases with high specificity, 68Ga-DOTATATE PET/CT having largely replaced 123I-mIBG SPECT/CT as the first line functional investigation. Imaging is essential to aid surgical planning, as endoscopic resection is often not possible or incomplete due to tumor location. For patients with advanced disease, 68Ga-DOTATATE PET/CT and 123I-mIBG SPECT/CT assess suitability for peptide receptor radionuclide therapy. Up to 63% of bPGL patients have a germline mutation, most commonly in the SDHB subunit gene, increasing their risk of developing pheochromocytomas and further paragangliomas; lifelong annual biochemical and periodic imaging screening from skull base to pelvis is therefore recommended.


Adrenal Gland Neoplasms , Organometallic Compounds , Paraganglioma , Pheochromocytoma , Humans , Paraganglioma/diagnostic imaging , Paraganglioma/genetics , Pheochromocytoma/genetics , Positron Emission Tomography Computed Tomography/methods , Positron-Emission Tomography , Radionuclide Imaging , Urinary Bladder
11.
Endocr Connect ; 11(2)2022 Feb 16.
Article En | MEDLINE | ID: mdl-35060925

OBJECTIVE: Succinate dehydrogenase subunit (SDHx) pathogenic variants predispose to phaeochromocytoma and paraganglioma (PPGL). Lifelong surveillance is recommended for all patients to enable prompt detection and treatment. There is currently limited evidence for optimal surveillance strategies in hereditary PPGL. We aim to detail the clinical presentation of PPGL in our cohort of non-index SDHB and SDHD pathogenic variant carriers. METHODS: Retrospective analysis of medical and genetic records from a single tertiary referral centre identified SDHB or SDHD pathogenic variants in 74 non-index cases (56 SDHB and 18 SDHD). Surveillance screening for asymptomatic relatives consisted of annual plasma metanephrine measurement and whole-body MRI with contrast at 3-5 yearly intervals. RESULTS: Twenty-three out of 74 non-index patients (10 SDHB and 13 SDHD) were diagnosed with PPGL, 17 patients through surveillance screening (24 tumours in total) and 6 diagnosed prior to commencement of cascade screening with symptomatic presentation. MRI with contrast identified PPGL in 22/24 screen-detected tumours and 5/24 tumours had elevated plasma metanephrine levels. Penetrance in non-index family members was 15.2 and 47.2% for SDHB carriers and 71.6 and 78.7% for SDHD carriers at age of 50 and 70 years, respectively. CONCLUSION: Surveillance screening with combined biochemical testing and imaging enables early detection of PPGL in asymptomatic relatives with SDHx pathogenic variants. The presence of disease at first screen was significant in our cohort and hence further multi-centre long-term data are needed to inform counselling of family members undergoing lifelong surveillance.

12.
Pituitary ; 25(1): 152-158, 2022 Feb.
Article En | MEDLINE | ID: mdl-34518996

OBJECTIVE: Immune checkpoint inhibitors can cause various immune-related adverse events including secondary hypophysitis. We compared clinical characteristics of immunotherapy-induced hypophysitis (IIH) and primary hypophysitis (PH) DESIGN: Retrospective multicenter cohort study including 56 patients with IIH and 60 patients with PH. METHODS: All patients underwent extensive endocrine testing. Data on age, gender, symptoms, endocrine dysfunction, MRI, immunotherapeutic agents and autoimmune diseases were collected. RESULTS: Median time of follow-up was 18 months in IIH and 69 months in PH. The median time from initiation of immunotherapy to IIH diagnosis was 3 months. IIH affected males more frequently than PH (p < 0.001) and led to more impaired pituitary axes in males (p < 0.001). The distribution of deficient adenohypophysial axes was comparable between both entities, however, central hypocortisolism was more frequent (p < 0.001) and diabetes insipidus considerably less frequent in IIH (p < 0.001). Symptoms were similar except that visual impairment occurred more rarely in IIH (p < 0.001). 20 % of IIH patients reported no symptoms at all. Regarding MRI, pituitary stalk thickening was less frequent in IIH (p = 0.009). Concomitant autoimmune diseases were more prevalent in PH patients before the diagnosis of hypophysitis (p = 0.003) and more frequent in IIH during follow-up (p = 0.002). CONCLUSIONS: Clinically, IIH and PH present with similar symptoms. Diabetes insipidus very rarely occurs in IIH. Central hypocortisolism, in contrast, is a typical feature of IIH. Preexisting autoimmunity seems not to be indicative of developing IIH.


Hypophysitis , Cohort Studies , Humans , Hypophysitis/chemically induced , Immunotherapy/adverse effects , Ipilimumab , Male , Retrospective Studies
13.
Clin Endocrinol (Oxf) ; 97(4): 448-459, 2022 10.
Article En | MEDLINE | ID: mdl-34870338

OBJECTIVES: Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours with malignant potential and a hereditary basis in almost 40% of patients. Germline genetic testing has transformed the management of PPGL enabling stratification of surveillance approaches, earlier diagnosis and predictive testing of at-risk family members. Recent studies have identified somatic mutations in a further subset of patients, indicating that molecular drivers at either a germline or tumour level can be identified in up to 80% of PPGL cases. The aim of this study was to investigate the clinical utility of somatic sequencing in a large cohort of patients with PPGL in the United Kingdom. DESIGN AND PATIENTS: Prospectively collected matched germline and tumour samples (development cohort) and retrospectively collected tumour samples (validation cohort) of patients with PPGL were investigated. MEASUREMENTS: Clinical characteristics of patients were assessed and tumour and germline DNA was analysed using a next-generation sequencing strategy. A screen for variants within 'mutation hotspots' in 68 human cancer genes was performed. RESULTS: Of 141 included patients, 45 (32%) had a germline mutation. In 37 (26%) patients one or more driver somatic variants were identified including 26 likely pathogenic or pathogenic variants and 19 variants of uncertain significance. Pathogenic somatic variants, observed in 25 (18%) patients, were most commonly identified in the VHL, NF1, HRAS and RET genes. Pathogenic somatic variants were almost exclusively identified in patients without a germline mutation (all but one), suggesting that somatic sequencing is likely to be most informative for those patients with negative germline genetic test results. CONCLUSIONS: Somatic sequencing may further stratify surveillance approaches for patients without a germline genetic driver and may also inform targeted therapeutic strategies for patients with metastatic disease.


Adrenal Gland Neoplasms , Paraganglioma , Pheochromocytoma , Adrenal Gland Neoplasms/pathology , Genetic Predisposition to Disease , Germ-Line Mutation/genetics , Humans , Paraganglioma/pathology , Pheochromocytoma/diagnosis , Retrospective Studies
14.
Ir J Med Sci ; 191(2): 831-837, 2022 Apr.
Article En | MEDLINE | ID: mdl-33728528

INTRODUCTION: The emergence of the novel coronavirus Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) and the coronavirus disease COVID-19 has impacted enormously on non-COVID-19-related hospital care. Curtailment of intensive care unit (ICU) access threatens complex surgery, particularly impacting on outcomes for time-sensitive cancer surgery. Oesophageal cancer surgery is a good example. This study explored the impact of the pandemic on process and short-term surgical outcomes, comparing the first wave of the pandemic from April to June in 2020 with the same period in 2019. METHODS: Data from all four Irish oesophageal cancer centres were reviewed. All patients undergoing resection for oesophageal malignancy from 1 April to 30 June inclusive in 2020 and 2019 were included. Patient, disease, and peri-operative outcomes (including COVID-19 infection) were compared. RESULTS: In 2020, 45 patients underwent oesophagectomy, and 53 in the equivalent period in 2019. There were no differences in patient demographics, co-morbidities, or use of neoadjuvant therapy. The median time to surgery from neoadjuvant therapy was 8 weeks in both 2020 and 2019. There were no significant differences in operative interventions between the two time periods. There was no difference in operative morbidity in 2020 and 2019 (28% vs 40%, p = 0.28). There was no in-hospital mortality in either period. No patient contracted COVID-19 in the perioperative period. CONCLUSIONS: Continuing surgical resection for oesophageal cancer was feasible and safe during the COVID-19 pandemic in Ireland. The national response to this threat was therefore successful by these criteria in the curative management of oesophageal cancer.


COVID-19 , Esophageal Neoplasms , Esophageal Neoplasms/epidemiology , Esophageal Neoplasms/surgery , Humans , Ireland/epidemiology , Pandemics , SARS-CoV-2
15.
Clin Endocrinol (Oxf) ; 96(4): 499-512, 2022 04.
Article En | MEDLINE | ID: mdl-34558728

OBJECTIVE: Phaeochromocytomas and paragangliomas (PPGL) are rare, but strongly heritable tumours. Variants in succinate dehydrogenase (SDH) subunits are identified in approximately 25% of cases. However, clinical and genetic information of patients with SDHC variants are underreported. DESIGN: This retrospective case series collated data from 18 UK Genetics and Endocrinology departments. PATIENTS: Both asymptomatic and disease-affected patients with confirmed SDHC germline variants are included. MEASUREMENTS: Clinical data including tumour type and location, surveillance outcomes and interventions, SDHC genetic variant assessment, interpretation, and tumour risk calculation. RESULTS: We report 91 SDHC cases, 46 probands and 45 non-probands. Fifty-one cases were disease-affected. Median age at genetic diagnosis was 43 years (range: 11-79). Twenty-four SDHC germline variants were identified including six novel variants. Head and neck paraganglioma (HNPGL, n = 30, 65.2%), extra-adrenal paraganglioma (EAPGL, n = 13, 28.2%) and phaeochromocytomas (PCC) (n = 3, 6.5%) were present. One case had multiple PPGLs. Malignant disease was reported in 19.6% (9/46). Eight cases had non-PPGL SDHC-associated tumours, six gastrointestinal stromal tumours (GIST) and two renal cell cancers (RCC). Cumulative tumour risk (95% CI) at age 60 years was 0.94 (CI: 0.79-0.99) in probands, and 0.16 (CI: 0-0.31) in non-probands, respectively. CONCLUSIONS: This study describes the largest cohort of 91 SDHC patients worldwide. We confirm disease-affected SDHC variant cases develop isolated HNPGL disease in nearly 2/3 of patients, EAPGL and PCC in 1/3, with an increased risk of GIST and RCC. One fifth developed malignant disease, requiring comprehensive lifelong tumour screening and surveillance.


Adrenal Gland Neoplasms , Carcinoma, Renal Cell , Gastrointestinal Stromal Tumors , Kidney Neoplasms , Paraganglioma , Pheochromocytoma , Adrenal Gland Neoplasms/genetics , Female , Germ-Line Mutation/genetics , Humans , Male , Membrane Proteins/genetics , Middle Aged , Paraganglioma/genetics , Paraganglioma/pathology , Pheochromocytoma/genetics , Pheochromocytoma/pathology , Retrospective Studies , Succinate Dehydrogenase/genetics , Succinate Dehydrogenase/metabolism , United Kingdom
16.
Child Psychiatry Hum Dev ; 53(6): 1349-1358, 2022 12.
Article En | MEDLINE | ID: mdl-34216333

In this study, a community sample of parents attending free 7-week Positive Discipline parenting workshops were recruited, as well as a non-randomized community control. Both samples consisted of primarily Hispanic parents with similar demographic information and attrition rates (initial N = 91), as well as children of similar age (mean age 6.89 and 6.95 years) and gender. Parenting stress, parenting style, and parent-reported child adaptive behavior were assessed at baseline and after three months. Longitudinal analysis was performed using mixed-effects regression modeling. Results indicate that attendance in Positive Discipline parenting workshops was related to a decrease in authoritarian parenting style, a decrease in permissive parenting style, and a decrease in parental stress. It was also related to an increase in child academic competence, and a decrease in externalizing-hyperactive behavior (both parent-report). These results suggest that positive discipline parenting workshops may alter parenting style and may positively impact children of parents who attend.


Parent-Child Relations , Parenting , Adaptation, Psychological , Child , Child Behavior , Humans , Parents
17.
Pituitary ; 24(4): 611-621, 2021 Aug.
Article En | MEDLINE | ID: mdl-33709288

PURPOSE: Data on the safety of growth hormone (GH) replacement therapy during pregnancy are limited. We report a combined analysis of data from pregnant women treated with GH while enrolled in two non-interventional, multicenter studies: NordiNet® International Outcome Study (IOS) and the American Norditropin® Studies: Web-Enabled Research (ANSWER) Program. METHODS: Pregnancy data were pooled from NordiNet® IOS and the ANSWER Program. Data were collected during routine clinic visits by participating physicians using a web-based system. Patients exposed to GH replacement therapy during pregnancy were included in the analysis. RESULTS: The study population included 40 female patients with typical causes of adult GH deficiency (GHD). Overall, there were 54 pregnancies. Of these, 47 were exposed to GH between conception and delivery. In 48.9% of pregnancies exposed to GH, the dose was > 0.6 mg/day. GH was continued past conception and then stopped during the first, second, and third trimester, in 27.7%, 17.0%, and 2.1% of pregnancies, respectively. In 29.8%, GH was continued throughout pregnancy, with an unchanged dose in most cases. Of the 47 GH-exposed pregnancies, 37 (78.7%) progressed to normal delivery. There were three adverse events reported in two pregnancies. CONCLUSION: These real-world data suggest that there were no new safety signals related to GH exposure in women with GHD during pregnancy. These results are consistent with findings from previous studies reporting data in pregnancies exposed to GH at conception or throughout pregnancy. This observational study in additional pregnancies provides further evidence that GH exposure does not adversely affect pregnancy outcome. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT00960128 (date of registration: August 13, 2009) and NCT01009905 (date of registration: November 5, 2009).


Hormone Replacement Therapy , Dwarfism, Pituitary , Female , Growth Hormone/therapeutic use , Human Growth Hormone/therapeutic use , Humans , Insulin-Like Growth Factor I , Outcome Assessment, Health Care , Pregnancy , Pregnancy Outcome , United States
18.
Ann Thorac Surg ; 111(6): 1812-1819, 2021 06.
Article En | MEDLINE | ID: mdl-33031780

BACKGROUND: There is a need to compare the proportions, risk factors, and natural histories of postesophagectomy paraconduit hernias in minimally invasive and open esophagectomies. METHODS: This is a single-center, retrospective cohort study of esophageal cancer surgery performed between 2007 and 2017. Postesophagectomy paraconduit hernias were identified on cross-sectional imaging. Patient charts were reviewed to describe the management and natural history. RESULTS: Between 2007 and 2017, 391 esophagectomies were performed. After exclusions, 347 patients remained, 135 of whom were total minimally invasive esophagectomies (MIEs) (39%). Postoperative paraconduit hernias developed in 10% of patients. Median time to diagnosis was 258 days. Of 135 MIEs, 20 had a paraconduit hernia (15%) compared with 16 of 212 open or hybrid esophagectomies (8%; P = .03). Hernias were symptomatic in 13 patients (36%) and asymptomatic in 23 (64%), which were detected radiographically. Repair was performed in 11 of 13 symptomatic patients (85%), compared with 3 of 23 asymptomatic patients (13%). In the asymptomatic group, only 1 required emergency repair (4.3%). There was a trend toward a greater proportion of symptomatic paraconduit hernias compared with asymptomatic patients (77% versus 43%; P = .08) in MIE patients. Factors associated with the development of paraconduit hernias on univariate analysis were younger age (P = .02) and not receiving neoadjuvant chemotherapy (P = .01) or neoadjuvant radiation (P = .03). CONCLUSIONS: Postesophagectomy paraconduit hernia is more common after totally minimally invasive esophagectomy compared with open or hybrid techniques. One third are symptomatic and the remainder are detected only radiographically. Repair of asymptomatic hernias consider the patient's cancer prognosis.


Esophageal Neoplasms/surgery , Esophagectomy/adverse effects , Esophagectomy/methods , Hernia, Hiatal/etiology , Laparoscopy , Postoperative Complications/etiology , Adult , Aged , Aged, 80 and over , Cohort Studies , Female , Hernia, Hiatal/diagnosis , Hernia, Hiatal/epidemiology , Humans , Male , Middle Aged , Postoperative Complications/diagnosis , Postoperative Complications/epidemiology , Retrospective Studies , Risk Factors
19.
Ther Adv Endocrinol Metab ; 11: 2042018820945855, 2020.
Article En | MEDLINE | ID: mdl-33133491

Disorders of thyroid function are common in pregnancy and have implications for foetal and maternal health. Thyroid autoimmunity, as evidenced by the presence of elevated levels of anti-thyroid antibodies (anti-TPO and anti-Tg antibodies) is associated with an increased risk of miscarriage, though the mechanism remains poorly understood. There has been considerable focus on the implications and optimal management of pregnant women with thyroid disease, especially those undergoing assisted reproduction. Pregnancy results in significant changes in thyroid physiology and these need to be understood by clinicians involved in the care of pregnant women. Guidelines for the use of thyroxine and target thyroid function tests have been produced by international bodies but it is recognised that these predominantly reflect expert opinion rather than established evidence-based practice. Importantly a number of key clinical trials have been performed to aid understanding, particularly of the consequences of hypothyroidism for mother and baby, and the effectiveness of thyroid hormone use in autoimmune and subclinical hypothyroidism. This review summarises the current knowledge base and guidance for practice relating to thyroid disorders in pregnancy and subfertility.

20.
Trials ; 21(1): 638, 2020 Jul 13.
Article En | MEDLINE | ID: mdl-32660526

BACKGROUND: Advances in peri-operative oncological treatment, surgery and peri-operative care have improved survival for patients with oesophagogastric cancers. Neoadjuvant cancer treatment (NCT) reduces physical fitness, which may reduce both compliance and tolerance of NCT as well as compromising post-operative outcomes. This is particularly detrimental in a patient group where malnutrition is common and surgery is demanding. The aim of this trial is to assess the effect on physical fitness and clinical outcomes of a comprehensive exercise training programme in patients undergoing NCT and surgical resection for oesophagogastric malignancies. METHODS: The PERIOP-OG trial is a pragmatic, multi-centre, randomised controlled trial comparing a peri-operative exercise programme with standard care in patients with oesophagogastric cancers treated with NCT and surgery. The intervention group undergo a formal exercise training programme and the usual care group receive standard clinical care (no formal exercise advice). The training programme is initiated at cancer diagnosis, continued during NCT, between NCT and surgery, and resumes after surgery. All participants undergo assessments at baseline, post-NCT, pre-surgery and at 4 and 10 weeks after surgery. The primary endpoint is cardiorespiratory fitness measured by demonstration of a 15% difference in the 6-min walk test assessed at the pre-surgery timepoint. Secondary endpoints include measures of physical health (upper and lower body strength tests), body mass index, frailty, activity behaviour, psychological and health-related quality of life outcomes. Exploratory endpoints include a health economics analysis, assessment of clinical health by post-operative morbidity scores, hospital length of stay, nutritional status, immune and inflammatory markers, and response to NCT. Rates of NCT toxicity, tolerance and compliance will also be assessed. DISCUSSION: The PERIOP-OG trial will determine whether, when compared to usual care, exercise training initiated at diagnosis and continued during NCT, between NCT and surgery and then during recovery, can maintain or improve cardiorespiratory fitness and other physical, psychological and clinical health outcomes. This trial will inform both the prescription of exercise regimes as well as the design of a larger prehabilitation and rehabilitation trial to investigate whether exercise in combination with nutritional and psychological interventions elicit greater benefits. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03807518 . Registered on 1 January 2019.


Esophageal Neoplasms/therapy , Exercise Therapy , Neoadjuvant Therapy , Physical Fitness , Preoperative Exercise , Stomach Neoplasms/therapy , Esophageal Neoplasms/surgery , Humans , Multicenter Studies as Topic , Postoperative Care , Quality of Life , Randomized Controlled Trials as Topic , Reference Standards , Stomach Neoplasms/surgery , Treatment Outcome
...