Clinico-pathologic profile and outcomes of pediatric endocrine patients managed by endocrine surgeons: Experience over three decades in a tertiary center in India.

BACKGROUND: Pediatric endocrine disorders requiring surgical intervention are rare and so are experienced surgeons dealing with these. The aim of the current study was to investigate disease profile and perioperative outcome of pediatric patients with surgical endocrine disorders in an endocrine surgery unit. METHODS: This retrospective study (Sep 1989-Aug 2019) consisted of pediatric endocrine surgery patients (<18 years) who were managed by a team of pediatric endocrinologists and endocrine surgeons at our center. Patients were divided into three cohorts consisting of a decade each. Clinico-pathologic variables, perioperative events operative and follow-up details were recorded. RESULTS: A total of 332 children were included and their mean age was 14.6 ± 3.9 years (M:F = 1:1.6). Thyroid disorders were most prevalent (59.8%), followed by adrenal (28.2%), parathyroid (10.4%), and pancreas (1.5%). Incidence of benign, malignant, and congenital/developmental disorders were 65.4, 28.1 and 8.3, respectively. Familial association was observed in 8.9% children, which is highest among pheochromocytoma patients. Overall, 201 thyroidectomies + associated procedures, 35 parathyroidectomies, 96 adrenal and paraganglioma resections, and 5 pancreatic procedures were performed. Median hospital stay was 5.6 ± 4.1 days. The number of cases increased significantly over 3 decades. Clinical profile and outcome did not vary except for significant decrease in incidence of malignant pathology (p = 0.04) and increase in VHL cases (p = 0.04) in the last decade though overall increase in familial cases was nonsignificant (p = 0.11). No perioperative mortality was observed except for 3% after adrenalectomy. CONCLUSION: A team of dedicated endocrine surgeons and pediatric endocrinologists is effective in management of pediatric endocrine surgery.

Total vs less than total thyroidectomy for benign multinodular non-toxic goiter: an updated systematic review and meta-analysis.

BACKGROUND: We have performed an updated meta-analysis of randomized controlled trials (RCT) comparing total thyroidectomy (TT) with less than total thyroidectomy (LTT) for benign multinodular non-toxic goiter (BMNG). OBJECTIVES: The objective was to evaluate the effects and outcomes of TT as compared to LTT. METHODS: Eligibility criteria: RCTs comparing TT vs LTT. INFORMATION SOURCES: PubMed, Embase, Cochrane Library and online registers were searched for articles comparing TT with LTT. Risk of bias: Articles were assessed for risk of bias using the Cochrane's revised tool to assess risk of bias in randomized trials (RoB 2 tool). SYNTHESIS OF RESULTS: The main summary measures were risk difference using a random effects model. RESULTS: Five randomized controlled trials were included in the meta-analysis. Recurrence rate was lower for TT compared to LTT. Adverse events like temporary or permanent recurrent laryngeal nerve (RLN) palsy and permanent hypoparathyroidism were similar in both groups except for the rate of temporary hypoparathyroidism which was lower in the LTT group. DISCUSSION: All studies had unclear risk of bias for blinding of the participants and personnel and high risk of bias for certain selective reporting. This meta-analysis did not show any clear benefit or harm of either procedure (TT vs LTT) for goiter recurrence and re-operation rates (for both recurrence and incidental thyroid cancer). However, re-operation for goiter recurrence was significantly higher in the LTT group based on a single RCT. Evidence suggests increased rates of temporary hypoparathyroidism with TT but there was no difference in the rate of RLN palsy and permanent hypoparathyroidism between the two methods. The overall quality of evidence was low to moderate.

Safe and Standard Thyroid Cancer Surgery, or Lack Thereof: Patterns and Correlates of Patient Referral to Tertiary Care Centre for Revision Thyroid Surgery in a LMIC.

Saroj Kanta MishraBackground A surgeon's characteristics such as volume and practice setup are essential elements in outcome of thyroid cancer. However, little information is available from the developing world regarding qualities of primary surgeon, such as level of knowledge, skill, and proper documentation while referring to higher center. Methods Records of 164 patients of differentiated thyroid cancer (DTC) from January 1990 to December 2018 undergoing revision thyroid surgery following primary surgery elsewhere were retrospectively analyzed. Results Out of 164 patients with postoperative diagnosis of DTC, referral patterns were as follows: low volume (LV) to high volume (HV) ( n = 120, 73.2%), followed by HV to HV ( n = 44, 26.8%). The primary surgery assessed by the extent of residual disease was in agreement with the documentation in only 55%. The type of thyroidectomy performed was not mentioned in 9.8%. The status of the parathyroid glands was mentioned only in 15.8% and recurrent laryngeal nerve in 12.2%. Less than recommended surgery was performed in 52.5% patients. Despite less than recommended surgery, 44.5% patients were directly referred for radioactive iodine ablation (RAIA). Thirty two percent patients were referred for RAIA after hemithyroidectomy. Central or lateral compartment lymphadenectomy, even after indication, was less likely at LV centers (risk ratio [RR], 0.71; 95% confidence interval [CI], 0.64-0.77). Similarly, for DTC patients, the relationship between LV center surgery and subsequent referral for RAIA was RR, 0.71 (95% CI, 0.48-1.02). Conclusions Most patients referred from LV surgeons are less likely to have proper thyroidectomy, have inadequate documentation of the primary surgery, and are referred for RAIA after less than total thyroidectomy. Our study highlights the lacunae in the approach to and understanding of thyroid cancer surgery by secondary care physicians in our country. We believe that there is an urgent necessity of educational reform and training to rectify this problem.

Truncal Ligation of Inferior Thyroid Artery Does Not Affect the Incidence of Hypocalcaemia After Central Compartment Lymph Node Dissection.

Preserving all parathyroids with intact blood supply in situ is a desirable goal while performing total thyroidectomy (TT) and central compartment dissection (CCLND), but the same is not feasible in presence of bulky and/or matted lymph nodes in the central compartment. The aim of this study was to investigate the difference in incidence of postoperative hypocalcemia between the groups of papillary thyroid carcinoma (PTC) patients who had truncal ligation (TL) versus ligation of peripheral branches (PL) of inferior thyroid artery (ITA) during TT and CCLND. Retrospective review of prospectively kept data of PTC patients undergoing TT and CCLND. Patients were divided in two groups: Group 1-(n = 57) patients who had to undergo TL and Group 2-(n = 99) patients having PL. Clinico-pathologic profile, operative details and follow-up events were noted. Clinico-pathologic profile of both groups was comparable except for higher incidences of tumor multicentricity (p = 0.014) in Group 1. There was no significant difference in the number of parathyroids identified between the Groups (p = 0.556) but more parathyroids were auto-transplanted in Group 1 (p = 0.001). The incidence of temporary (77.2% vs 83.8, p = 0.304) and permanent hypocalcemia (7% vs 8.1%, p = 0.810) was not significantly different between the groups and neither was need for intravenous calcium. At discharge, Group 1 patients received lower dose of calcium (p = 0.001) but not of vitamin D (p = 0.769). TL of ITA during CCLND does not result in increased temporary or permanent hypocalcemia rate.

Circumferential Laryngotracheal Resection in Thyroid Cancer: Experience and Outcome in a Single Center.

There is limited experience of laryngotracheal resection in patients with differentiated thyroid carcinoma (DTC). The aim of this study was to report our experience of circumferential laryngotracheal resection in DTC and its long-term outcome. In this retrospective study, 10 patients of locally invasive DTC who underwent circumferential laryngotracheal resection between January 2000 and December 2015 were included. Clinicopathologic profile and follow up was noted. Mean age of the cohort was 50.1 ± 7.8 years (M:F = 1: 2.3). Papillary carcinoma was the commonest pathology (60%) followed by follicular carcinoma (20%), 10% each had Hurthle cell and poorly differentiated thyroid carcinoma. Sixty percent patients presented with recurrent or persistent disease and 20% with distant metastases. Vocal cord palsy was observed in 30%. Fifty percent patients underwent tracheal resection with end to end anastomosis and remaining laryngotracheal resection. Carotid artery resection and anastomosis was performed in one patient. Shin stage IV invasion was observed in 80% and stage III in remaining. There was no perioperative mortality. All patients received adjuvant radioiodine therapy and 40% external beam radiotherapy. Median follow up was 48 months. One patient who earlier had tracheal resection underwent total laryngectomy due to recurrent disease after 24 months. Forty percent patients developed distant metastases during follow-up. Mean survival was 77.8 months (CI = 63.0-92.5). Five-year overall survival was 60% and was significantly high in those without distant metastases (p = 0.006). The outcome of circumferential larygotracheal resections for DTC is excellent in terms of local disease control and long term survival.

Outcome of 18f-Fluorodeoxyglucose Positron Emission Tomography (PET)-Directed Resections in Differentiated Thyroid Carcinoma (DTC): Experience in a Developing Country.

The aim of this study was to assess the accuracy of 18F-fluorodeoxyglucose positron emission tomography (18-FDG-PET scan) in localizing the disease in differentiated thyroid carcinoma patients undergoing re-operations. This is a retrospective analysis of a prospectively maintained data (December 2007 to December 2016). The patients included had elevated serum thyroglobulin (Tg) levels and negative iodine uptake (TENIS) and planned for re-operation with one or more accessible site of metastasis detected on FDG-PET scan. Clinical details, FDG-PET/CT findings, operative findings, histology, pre-, and post-operative Tg levels were recorded. Thirty-two patients were included. The mean age of the patients was 46.8 ± 15.8 years (M:F = 1:1.6) and mean pre-operative Tg value was 247.6 ± 92.3 ng/ml. FDG-PET disclosed a total of 77 hot spots in these 32 patients, 56 of which were surgically explored and resections performed. Patient- and lesion-based positive predictive value (PPV) of FDG-PET in detecting recurrent/metastatic DTC lesions was 87.5 and 71%, respectively. Remaining cases had granulomatous or nonspecific inflammatory lesions. A total of 12.5% of recurrent DTC patients explored could achieve biochemical cure. All these had disease confined to neck. Remaining patients continued to have high serum Tg level, though it fell substantially in majority of patients. False positive scans are frequent in regions with high prevalence of inflammatory diseases. Hence, FDG-PET directed re-operations should be taken up judiciously.

Medial Approach for Identification of Recurrent Laryngeal Nerve in Locally Advanced Differentiated Thyroid Carcinoma: Revisited.

Recurrent laryngeal nerve injury is a well-recognized and potentially catastrophic complication of thyroidectomy. In literature, four approaches have been described for identification of RLN, the superior, lateral, medial, and inferior approaches. But medial approach is lesser known amongst surgeons. Locally infiltrative thyroid cancer with extensive lymph nodal metastasis in central and lateral compartment sometimes makes it difficult to identify RLN by any of these standard approaches described above. A medial approach was described where RLN is identified as it extends up to interdigitate with the trunk or the branches of the inferior thyroid artery. We present here a new alternative technique for identification of recurrent laryngeal nerve through modified medial approach that was used in difficult thyroidectomies.

Clinical, Biochemical, Tumoural and Mutation Profile of VHL- and MEN2A-Associated Pheochromocytoma: A Comparative Study.

OBJECTIVE: To compare clinical, biochemical, tumoural and mutational characteristics of Von Hippel Lindau Syndrome (VHL)-associated pheochromocytoma (PCC) to multiple endocrine neoplasia 2A (MEN2A)-associated pheochromocytoma. DESIGN: Retrospective study design in a tertiary health care centre in Northern India. METHODS: A total of 47 patients with biochemical and histologically proven pheochromocytoma/paraganglioma (PCC/PGL): 29 associated with VHL and 18 with MEN2A, were divided in two cohorts, respectively. Analysis of their medical records along with a prospective follow-up was done. RESULTS: There were more children <19 years in VHL group (13 vs 1). Despite majority of VHL-PCC showing elevation of normetanephrine (NMN) (93%) as compared to MEN2A-PCC (22.2%), 75.8% presented with hypertension as compared to MEN2A (33.3%). The average size of VHL-PCC tumours was 5.66 cm. VHL-PCC as compared to MEN2A-PCC were multifocal (75% vs 61.1%), bilateral synchronous (72.4 vs 61.1%) and extra-adrenal (17.2% vs 0%). Both VHL (24%) and MEN2A-PCC (27.7%) showed multiple nodules, but more MEN2A PCC showed extra-tumoural hyperplasia (44.4% vs. 6.8%). In VHL, the commonest mutation (n = 17) was missense mutation with a hot spot on exon 3, while in MEN2A-PCC majority (66.6%) had 634 mutation in exon 11 and only 2 patients had the rare 611 mutation in exon 10. CONCLUSION: In contrast to world literature, our study suggests Indian VHL-PCC can be symptomatic in spite of noradrenergic phenotype, large in size and multifocal. Multiple nodules in VHL-PCC could increase risk of recurrence following subtotal adrenalectomy.

Telemedicine: History and Success Story of Remote Surgical Education in India.

Initiation of telemedicine in medical education in India was at par with developed countries but acceptance and progress have been slow. However, the recent coronavirus disease-19 (COVID-19) pandemic leading to disruption of Halstedian model of surgical teaching has changed the traditional dynamics of perception of this mode of education. Sanjay Gandhi PostGraduate Institute of Medical Sciences (SGPGIMS), has been a pioneer and introduced the telemedicine system into surgical education as early as in year 2001. In this article, we reviewed the literature on tele-education in surgical field in Indian scenario, with particular emphasis on tele-education activities at the SGPGIMS, with respect to current thinking and future prospects on surgical training.

Thyroid dysfunctions and autoimmunity in breast cancer patients: a prospective case-control study.

OBJECTIVE: The relationship of thyroid dysfunction and autoimmunity with breast cancer (BC) continues to be contentious. The primary aim of this study was to estimate the prevalence of thyroid dysfunctions and autoimmunity in BC patients, and the secondary aims were to investigate the relationship of thyroid dysfunction with the clinicopathological profile of and therapy received by BC patients. METHODS: This was a single-center prospective case-control study (March 2015-May 2017). Women with BC (n = 191), age-matched healthy controls (n = 166) and malignant controls (patients with cervical cancer, n = 87) were enrolled. Basal serum free thyroxin (fT4), thyrotropin (TSH) and anti-thyroid peroxidase (TPO) antibody levels were measured in all three groups; fT4, TSH and TPO measures were repeated after chemotherapy and at the 1-year follow-up (one year after diagnosis) in the BC patients. RESULTS: The prevalence of overall hypothyroidism and autoimmunity (p = 0.106) did not differ significantly between the three groups, but the rate of clinical hypothyroidism was significantly higher in the BC group than in the healthy control group and the malignant control group (12.2% vs. 3.0% vs. 4.6%, respectively; p = 0.001). BC patients had significantly lower mean basal TSH concentrations than the healthy controls (p = 0.017). The postchemotherapy TSH concentrations were significantly lower (p = 0.001), and the fT4 concentrations were higher, albeit not significantly (p = 1.00), than the respective basal concentrations. The reverse was true for the follow-up values, in which the TSH (p = 1.00) values were higher and the fT4 (p = 0.03) concentrations were lower than the respective basal concentrations. An additional 6% of the BC patients developed clinical hypothyroidism during follow-up. Hypothyroid (p = 0.02) and TPO-positive (p = 0.004) patients had significantly smaller tumors, but their other clinicopathological features were comparable to those without thyroid dysfunction. CONCLUSION: The prevalence of clinical hypothyroidism requiring thyroxine replacement was significantly high in BC patients and increased further during follow-up. Hence, BC patients should be considered a high-risk group that should receive routine screening for hypothyroidism.

Hereditary Medullary Thyroid Carcinoma: Genotype, Phenotype and Outcomes in a North Indian Cohort.

BACKGROUND: Aggressiveness of hereditary medullary thyroid carcinoma (hMTC) has been conventionally described to correlate with American Thyroid Association (ATA) risk groups based on RET mutations. Recent evidence increasingly contradicts this notion. We studied the RET genotype and its correlation with disease phenotype and survival outcomes in a cohort of hMTC patients. METHODS: In a retrospective cohort of 55 hMTC patients from 23 families treated at a north Indian tertiary care institute over 15-years, RET genotype was correlated with disease phenotype (clinical, biochemical, and pathological attributes) and outcomes in terms of biochemical cure (normalization of serum calcitonin), structural cure, overall survival (OS) and disease specific survival (DSS). RESULTS: Forty-nine patients had Multiple Endocrine Neoplasia (MEN)-type 2A syndrome, 02 had MEN-2B, and 4 had familial MTC. Two patients belonged to highest ATA risk, 41 to high-risk, and 12 to moderate risk categories. Age of the patients or stage of disease at presentation did not differ significantly between the ATA risk groups. Though the baseline serum calcitonin was significantly higher in highest risk category, the biochemical cure rates were not significantly different. At a median follow up of 48 months (Inter-quartile range 18-84, range 12-192) structural cure rates in ATA moderate and high risk groups were significantly higher than highest risk group (p = 0.04). No significant difference in OS between the three ATA groups of hMTC among the patients who underwent surgical treatment was observed (p = 0.098). CONCLUSIONS: The ATA moderate and high risk groups have better structural cure rates compared to ATA highest risk group. The biochemical cure and overall survival rates did not significantly differ between ATA risk-groups, and were impacted by the disease stage at presentation. The current ATA risk-groups do not reliably predict the outcomes in terms of biochemical cure and survival in hMTC patients.

Pediatric Primary Hyperparathyroidism: Experience in a Tertiary Care Referral Center in a Developing Country Over Three Decades.

BACKGROUND: There is limited experience in managing pediatric primary hyperparathyroidism (PHPT). The aim of this study was to analyze the clinical presentation and outcome of surgery in children with PHPT managed at a tertiary referral center. METHODS: This retrospective study (September 1989-August 2019) consisted of 35 pediatric PHPT patients (< 18 years) who underwent parathyroidectomy. Clinico-pathologic profile and outcome were noted. RESULTS: The mean age of cohort was 15.2±2.9 years and girls outnumbered boys (M:F = 1:1.9). Familial and symptomatic disease was noted in 8.5 and 94.3% cases, respectively. Skeletal manifestations (83%) were the commonest followed by renal (29%). Fifty-four percent children had skeletal fractures, and 23% were bed-ridden. Among rare manifestations, hypercalcemic crisis, recurrent pancreatitis and stigmata of rickets were observed in 2.8, 11.4 and 14.2% children, respectively. Mean calcium concentration was 12.1 ± 2.0 mg/dl and PTH 91.8 ± 66.5 pmol/L. The sensitivity of preoperative imaging in parathyroid localization was 91.4%. Minimally invasive parathyroidectomy (MIP) was performed in 40% cases. Parathyroid adenoma was observed in 91.4% patients, whereas remaining had hyperplasia. Thirty-four percent suffered from Hungry bone syndrome in postoperative period. The cure rate following primary surgery was 97%. One child with persistent PHPT had successful re-operation. Median follow-up was 5 (1-17) years, and no recurrence or familial disease was revealed during this period. CONCLUSION: Majority of pediatric patients present with symptomatic PHPT. Despite relatively high incidence of familial disease select pediatric patients can undergo successful MIP.

Thyroid dysfunctions and autoimmunity in breast cancer patients: a prospective case-control study

ABSTRACT Objective: The relationship of thyroid dysfunction and autoimmunity with breast cancer (BC) continues to be contentious. The primary aim of this study was to estimate the prevalence of thyroid dysfunctions and autoimmunity in BC patients, and the secondary aims were to investigate the relationship of thyroid dysfunction with the clinicopathological profile of and therapy received by BC patients. Materials and methods: This was a single-center prospective case-control study (March 2015-May 2017). Women with BC (n = 191), age-matched healthy controls (n = 166) and malignant controls (patients with cervical cancer, n = 87) were enrolled. Basal serum free thyroxin (fT4), thyrotropin (TSH) and anti-thyroid peroxidase (TPO) antibody levels were measured in all three groups; fT4, TSH and TPO measures were repeated after chemotherapy and at the 1-year follow-up (one year after diagnosis) in the BC patients. Results: The prevalence of overall hypothyroidism and autoimmunity (p = 0.106) did not differ significantly between the three groups, but the rate of clinical hypothyroidism was significantly higher in the BC group than in the healthy control group and the malignant control group (12.2% vs. 3.0% vs. 4.6%, respectively; p = 0.001). BC patients had significantly lower mean basal TSH concentrations than the healthy controls (p = 0.017). The postchemotherapy TSH concentrations were significantly lower (p = 0.001), and the fT4 concentrations were higher, albeit not significantly (p = 1.00), than the respective basal concentrations. The reverse was true for the follow-up values, in which the TSH (p = 1.00) values were higher and the fT4 (p = 0.03) concentrations were lower than the respective basal concentrations. An additional 6% of the BC patients developed clinical hypothyroidism during follow-up. Hypothyroid (p = 0.02) and TPO-positive (p = 0.004) patients had significantly smaller tumors, but their other clinicopathological features were comparable to those without thyroid dysfunction. Conclusions: The prevalence of clinical hypothyroidism requiring thyroxine replacement was significantly high in BC patients and increased further during follow-up. Hence, BC patients should be considered a high-risk group that should receive routine screening for hypothyroidism.

Endoscopic versus Conventional Thyroid Surgery: A Comparison of Quality of Life, Cosmetic Outcomes and Overall Patient Satisfaction with Treatment.

BACKGROUND: Our aim was to investigate whether the surgical approach, i.e. endoscopic (ET) versus conventional/open thyroidectomy (ConT), contributes towards the improvement in quality of life (QoL), cosmetic outcomes and overall patient satisfaction with treatment. METHODS: We conducted a prospective study (October 2016-August 2018) consisting of patients undergoing hemithyroidectomy for euthyroid, non-malignant solitary thyroid nodules (STNs). Patients were divided into two groups: Group I (ET)-41 patients and Group II (ConT)-52 patients. ET was performed via bilateral-axillo breast approach (BABA). A thyroid disease-specific questionnaire "ThyPRO-39hin" was used to evaluate pre- and post-operative QoL. Visual analogue scale (VAS) was used for post-operative pain. Cosmetic satisfaction, paraesthesia and overall satisfaction with treatment were evaluated using Likert-type verbal response scales. Pre- and post-operative scores (>6 months) were compared, and p value < 0.05 was considered significant. RESULTS: Mean age (p = 0.26), gender distribution (p = 0.07), mean tumour size (p = 0.74) and preoperative scores of QoL were comparable between the two groups. Post-operatively scores of symptom domain (p = 0.03), tiredness (p = 0.03), impaired social life (p = 0.03), cosmetic complaints (p = < 0.001) and overall QoL (p = < 0.001) were significantly better in Group I. Also, post-operative pain perception at first follow-up visit (p = 0.001) was lower in patients undergoing ET/Group I, with higher scar satisfaction scores (p = < 0.001) and overall satisfaction with treatment (p = < 0.001). Post-operative paraesthesia perception was comparable (p = 0.06) amongst the two groups. CONCLUSION: In this cohort study, patients undergoing endoscopic thyroid surgery reported superior post-operative QoL, cosmetic and overall satisfaction with treatment as compared to conventional/open thyroidectomy.

Distribution and Prognostic Significance of Estrogen Receptor α (ERα), Estrogen Receptor ß (ERß), and Human Epidermal Growth Factor Receptor 2 (HER-2) in Thyroid Carcinoma.

PURPOSE: The primary aim of this study was to determine the incidence of estrogen receptor α (ERα), estrogen receptor ß (ERß), and human epidermal growth factor receptor 2 (HER-2) expression in various subtypes of thyroid carcinoma (TC) of follicular origin and the secondary aim was to correlate the expression with various clinicopathologic prognostic factors. METHODS: Immunohistochemistry analysis was performed on archival paraffin-embedded tissue sections (1991-2016). ERα, ERß, and HER-2 expressions were correlated with clinicopathologic prognostic factors, disease recurrence, and overall survival (OS). RESULTS: A total of 264 TC patients were included in the study. Incidences of ERα, ERß, and HER-2 were 8.1 vs 16.3 vs 13.9% (p=0.15), 26.6 vs 11.5 vs 36.1% (p=0.002), and 12.9 vs 2.9 vs 0% (p=0.003) in papillary thyroid carcinoma (PTC), follicular thyroid carcinoma (FTC), and poorly differentiated thyroid carcinoma (PDTC), respectively. Overall ERα had significant correlation with distant metastases (0.038) and in case of PDTC with multicentricity (p=0.037). ERß had significant correlation with lymph node metastases (p=0.023) in FTC. HER-2 correlated with tumor size (p=0.027) only on univariate analysis. OS did not correlate with expression of any receptor. CONCLUSION: ERα, ERß, and HER-2 have differential expression and prognostic implications in different TC subtypes.

Primary hyperparathyroidism in developing world: a systematic review on the changing clinical profile of the disease.

While the developed world is focusing on laying guidelines for selecting out cases of Asymptomatic primary hyperparathyroidism (PHPT) for surgical intervention and promoting minimal access surgery, the developing world is observing a change in disease spectrum from advanced symptomatic to lesser degree of symptomatic disease and not many with associated Vitamin D deficiency. Few studies from the developing countries of the world have focused on the changing clinical spectrum of PHPT. Objective of this study is to review the changing profile of PHPT in developing world. A systematic literature search was done in December 2017 focussing on publications from the developing world. All studies pertaining to the epidemiology of PHPT published after 1st January 2000 and published in English language were included for analysis. Most of the studies published from developing countries report a predominance of symptomatic disease (79.6% of all included patients) with musculoskeletal disease present in the majority of patients (52.9%). The combined mean serum total calcium (11.9 ± 1.4 mg/dL), serum PTH (668.6 ± 539 pg/mL), serum alkaline phoshpatase (619 ± 826.9 IU/L) and weight of excised parathyroid glands (4.4 ± 3.8 grams) are much higher than those reported from the western studies. Despite this, we found that there is a distinct trend towards a milder form of disease presentation and biochemical profile noticeable in more recent times. Although there is a striking difference in all aspects of PHPT disease epidemiology, clinical presentation and biochemical profile of developing and developed countries, there is a distinct trend towards a milder form of disease presentation and biochemical profile in more recent times.

Primary hyperparathyroidism in developing world: a systematic review on the changing clinical profile of the disease

ABSTRACT While the developed world is focusing on laying guidelines for selecting out cases of Asymptomatic primary hyperparathyroidism (PHPT) for surgical intervention and promoting minimal access surgery, the developing world is observing a change in disease spectrum from advanced symptomatic to lesser degree of symptomatic disease and not many with associated Vitamin D deficiency. Few studies from the developing countries of the world have focused on the changing clinical spectrum of PHPT. Objective of this study is to review the changing profile of PHPT in developing world. A systematic literature search was done in December 2017 focussing on publications from the developing world. All studies pertaining to the epidemiology of PHPT published after 1st January 2000 and published in English language were included for analysis. Most of the studies published from developing countries report a predominance of symptomatic disease (79.6% of all included patients) with musculoskeletal disease present in the majority of patients (52.9%). The combined mean serum total calcium (11.9 ± 1.4 mg/dL), serum PTH (668.6 ± 539 pg/mL), serum alkaline phoshpatase (619 ± 826.9 IU/L) and weight of excised parathyroid glands (4.4 ± 3.8 grams) are much higher than those reported from the western studies. Despite this, we found that there is a distinct trend towards a milder form of disease presentation and biochemical profile noticeable in more recent times. Although there is a striking difference in all aspects of PHPT disease epidemiology, clinical presentation and biochemical profile of developing and developed countries, there is a distinct trend towards a milder form of disease presentation and biochemical profile in more recent times.

Smartphone-Based Application for Tele-follow-up of Patients with Endocrine Disorders in Context of a LMIC: A Compliance, Satisfaction, Clinical Safety and Outcome Assessment.

BACKGROUND: Remote video consultations are increasingly used in clinical practice, and a lot of data are emerging on its feasibility and acceptability. This study aims to bring out qualitative and quantitative data which will enhance our understanding of strengths and limitations of this media in the setting of a low- and middle-income country (LMIC). MATERIALS AND METHODS: This was a prospective study conducted from January 2017 to May 2018, at the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, India. A total of 107 patients were selected who chose remote follow-up care through social media (WhatsApp). Data were analyzed on feasibility, clinical safety, satisfaction and economic burden. RESULTS: A total of 107 postoperative patients were followed up using a social media tool. A total of 396 tele-sessions were held. The average number of tele-consultations per patient was 3.7 (range 2-6). Reasons for tele-follow-up included: confirmation of histology report (n = 92), medication dosage adjustments (n = 148), wound evaluation (n = 102), reporting of serum TSH and serum calcium levels (n = 296) and medical fitness certificate (n = 13). Wound evaluation through tele-follow-up was on par with the outpatient department (OPD) follow-up as no patient had to report to OPD for wound infection. Satisfaction level was excellent in 55% of patients and very good in 25%. 20% of the patients reported an average satisfaction level. If all of these 107 patients would have come to our OPD follow-up, they would have traveled 613.2 miles (908 km) per patient on an average, apart from losing work hours. Average cost and workdays saved per visit were $78 and 5.4 days, respectively. CONCLUSION: Video consultation using social media tools is clinically safe and cost effective. Economic benefits far outweigh the risk of missing an adverse event, especially in the setting of low- and middle-income countries.

Genetic Profile of Indian Pheochromocytoma and Paraganglioma Patients - A Single Institutional Study.

BACKGROUND AND AIMS: Pheochromocytomas (PCCs) and Paragangliomas (PGL) are rare catecholamine producing tumors that may present in sporadic or familial settings. Despite vast strides in understanding of PCC/PGL genetics in the last two decades, there is a dearth of information from India. The aim here is to study the prevalence of genetic mutations in Indian PCC/PGL patients. SETTINGS AND DESIGN: Tertiary care academic hospital; prospective study. METHODS: 50 histopathologically diagnosed PCC/PGL patients formed the study group. Clinical, biochemical, pathological attributes and outcomes were documented and the phenotype was compared to the genotype. Succinyl dehydrogenase (SDH), Re-Arranged during Transfection (RET), Von-Hippel-Lindau (VHL) and NeuroFibromatosis-1 (NF1) mutations were studied. Additionally, immunohistochemisty for SDHB was also done, and the results compared to mutational analysis of SDH by MLPA (Multiplex Ligation-dependent Probe Activation). STATISTICAL ANALYSIS: Independent samples t-test and Fisher's exact test were used as appropriate. P values ≤0.05 were considered statistically significant. RESULTS: The mean age was 34.3 years. Of the 50 patients, 27 were males and 23 females. 10 patients (20%) in all were detected to have a genetic mutation. 6 patients possessed a RET mutation, while two had VHL mutations. No patient presented with a NF1 mutation. 2 patients had a SDH mutation, and Immunohistochemistry for SDHB correlated with mutational analysis for these patients. CONCLUSIONS: The proportion of patients with a familial variant of PCC/PGL is more than what the historic "Rule of Ten" suggests. Our study found that one in five patients have a genetic mutation. PCC/PGL patients with genetic mutations not only require more stringent follow-up, but also screening of family members.

Genetic Alterations in Anaplastic Thyroid Carcinoma.

INTRODUCTION: Anaplastic thyroid cancer (ATC) is rare but fatal thyroid cancer responsible for majority of thyroid cancer related mortality. ATC may originate de novo or from preexisting differentiated thyroid cancer. Complex interaction between different gene mutation has been suggested to be the main causative factor for origin of ATC in both pathways. Mostly affected pathways are MAP kinase and PI3CA kinase. Hence, we decided to study the frequent alterations in both the pathways in ATC patients. METHODOLOGY: Clinico-pathological data of 34 ATC patients were collected retrospectively and Formalin Fixed Paraffin Embedded (FFPE) blocks were taken out for genetic analysis. DNA and RANA were isolated from FFPE tissues. BRAF V600E mutations were screened by RFLP PCR method and confirmed by sequencing. RAS, PI3CA and p53 mutations were checked by sequencing. RET/PTC translocations were screened by Real Time PCR. RESULTS: A total of 34 patients were studied: Mean age 58.6+ 11.6 years with F:M- 1.8:1, 60% had history of goiter. Most common presenting symptom was rapidly growing thyroid mass followed by dyspnea, dysphasia and hoarseness of voice. Extent of disease was local, locoregional and metastatic in 32%, 35% and 33% respectively. 57.6% were euthyroid, 20.5 % were hyperthyroid while functional status were not available in 11.7%. FNAC was suggestive of ATC only in 52.9% cases. 15 (44%) were operated. BRAF V600E mutations were observed in 10/34 (29.4%). Interestingly, all three ATC patients with DTC components had previous history of goiter with rapid increase in size and BRAF V600E mutation, while BRAF was positive only in 7/31 (22.5%) of patients with no DTC component. Mean survival of 3.5 months in BRAF positive cases in comparison to 5.5 months in BRAF negative ATC. RAS mutations were found to be positive in 5.8%, and none had RET-PTC/PI3CA mutations. P53 mutation was positive in 7 patients. 3 patients presented with history of rapid increase in size of previous goiter while rest 4 patients presented with rapidly increasing thyroid swelling of 1 to 3 months. At presentation 2 patients has disease localized to thyroid, 4 has loco-regional disease and one patient presented with metastasis. 5 out of these 7 patients were operated (Total thyroidectomy:3, thyroidectomy with neck dissection:2). Mean survival was 4 months (1-6 months). CONCLUSION: BRAF V600E was the commonest mutation followed by p53 of the 5 genes tested and BRAF was more common in patients with previous history of longstanding goiter or differentiated thyroid cancer. This provides an indirect evidence of neoplastic transformation of PTC to ATC.