5th Decade Research Articles

Quality of Life Assessment in Patients of Stage V Chronic Kidney Disease

Introduction: Despite ongoing advances in the treatment of chronic kidney disease (CKD), the mortality rate, and level of health-related quality of life (HRQOL) for the CKD population remain significantly higher than for the general population. The importance of measuring end-stage renal failure (ESRF) patients' quality of life in relation to healthcare lies in not only providing absolute survival but also the quality of that survival. Due to cost constraints, the profile of chronic kidney disease patients and their treatment in Bangladesh is almost identical to that of India, with patients frequently requesting a reduction in the frequency of dialysis sessions, the use of less expensive dialyzers, dialyzer reuse, and the absence of erythropoietin therapy. Hence, augmenting the QOL may perhaps be a challenge and an observable fact of specific interest for renal healthcare teams. This study is intended to assess KDQOL among patients receiving hemodialysis for 8 hours, and 12 hours per week, patients receiving CAPD, and patients of CKD stage V who decline any form of renal replacement therapy, and remained on conservative treatment. The aim of the study was to assess the quality of life among patients of CKD Stage V. Methods: This cross-sectional study was carried out at the Department of Nephrology, Dhaka Medical College Hospital and BIRDEM general hospital. The patients who received consultation at the Outdoor Department of selected hospital from November 2010 to October 2011. The study assess the of Quality of Life, and Cost Effectiveness on different modalities of treatment among the patients of chronic kidney disease stage V, and also to find out the best modality of dialysis. A total number of 134 consecutive patients were enrolled in this study, out of which 42 patients who were advised to commence renal replacement therapy, and after counseling opted to remain in conservative treatment were considered as GROUP I, 39 patients who received hemodialysis 8 hours per week were considered as GROUP II, 30 patients who received hemodialysis 12 hours per week in one or more centers were considered as GROUP III, and 23 patients who received Continuous Peritoneal Dialysis at least 3 exchanges per day were considered as GROUP IV. Result: Mean age was almost similar in all four groups, and most of the patients were 5th decade, and above. Male was predominant in all four groups, and the male-female ratio was almost 2:1 in the whole study patients. Diabetic nephropathy and glomerulonephritis were more common etiology of CKD in all four groups. Monthly expenditure was significantly (p<0.05) higher in group IV, followed by group III, group II, and group I in all three follow-ups, however, monthly expenditure was almost similar between group III, and group IV (p>0.05) but the mean monthly expenditure was higher in group IV patients. Mean serum Creatinine was lowest in group IV followed by group III, group II, and group I in descending order. Serum albumin was low in all the groups but almost parallel in all follow-ups in group II, groups I, and group IV, whereas the lowest was in group II followed by group I and group IV, but declined in group III during the 2nd, and 3rd follow-up from 1st follow-up. Hemoglobin level consistently remained within the target range in group IV in all follow-ups but below the target range in group I followed by group II, and group III in ascending order. The physical component score increased significantly in Group III, and Group IV at consecutive follow-ups, however, it was higher in Group IV. Similarly, the mental component score was recorded highest in Group IV, followed by group III, group II, and group I. Regarding mortality, it was observed that more than half of the patients were expired in group I, one-third in group II, and 16.7% in group III, and only 8.7% in group IV during final follow-up. Conclusion: Patients receiving continuous ambulatory peritoneal dialysis achieved the best clinical parameters in terms of control of blood pressure, and volume overload. On the other hand, parameters were a lot away from the desired target in patients receiving hemodialysis for 8 hours per week, and they did not have significantly better parameters in comparison to those who were only on conservative treatment. The scenario of serum albumin, and serum creatinine, though complicated by the existence of malnutrition, were in best approximation to the desired level in these patients. According to the age-sex-matched risk categorization of the patients, it was found that the majority of patients on conservative treatment were in more than the average risk category.

Pigmented Lesions of the Oral Cavity: A Review

The term “pigmentation of oral mucosa” is applied to a wide range of lesions or conditions featuring a change of color of oral tissues. Common causes of mucosal coloration including petechiae, purpura, ecchymoses, hematomas, vascular tumors and exogenous substances are not true pigmented lesions. In contrast, melanin which is synthesized by melanocytes, is a true pigment and usually imparts a brown, blue or black appearance of the mucosa. Oral pigmentation affects about 3% of the total population and most likely in those with dark skin, more prevalent in females than males during the 3rd or 4th decade of life. In this review, we are describing the oral manifestation and dental considerations associated with common pigmented disorders which will allow the practitioner to have a holistic approach in diagnosis and management of these patients.

PSAT283 Sclerosing Mucoepidermoid Cancer – A Unique Entity

Abstract Introduction - Sclerosing mucoepidermoid thyroid cancer is a rare entity with approximately 60 cases reported in the literature. Initially described by Chan. Et al in 1991,1 it was included as a separate entity by WHO in 2004.2 Previously considered to have indolent nature, recent evidence has reported aggressive nature ranging from local invasion to metastasis. Case - 66-year-old female with complaints of difficulty in swallowing, breathing, and sensation of a lump in her throat for last 6 months, was noted to have left thyroid mass on ultrasound. Thyroid ultrasound showed solid hypoechoic nodule, 8.2×5.3×5.9 cm replacing left thyroid lobe with solid hypoechoic nodule 2.2×1.1x 1.6 cm at the right end of the isthmus. The neck ultrasound was negative for lymph nodes. Past medical history was significant for long-standing hypothyroidism, and recently diagnosed invasive ductal carcinoma of the breast. CT chest showed an enlarged thyroid gland with substernal extension and mass effect on the trachea. FNA thyroid showed Bethesda category 5, suspicious for papillary thyroid cancer. The patient underwent a total thyroidectomy. Pathology for left nodule showed sclerosing mucoepidermoid carcinoma with eosinophils, 8.6×6.9×5.8 cm, p T4a N×Mx, with positive margins for tumor, no angiolymphatic invasion with co-existing right-sided papillary microcarcinoma (follicular variant) with chronic lymphocytic thyroiditis. Levothyroxine was dosed to aim goal TSH <0.1, considering the high risk of recurrence. Post-op Thyroglobulin was 1 ng/ml (< 2 ng/ml) with elevated Thyroglobulin antibodies 6.4 U/ml (<0.4). The patient further had radioiodine ablation with 150 mCi I-131, 3 months later. Post therapy whole-body scan showed multiple foci of increased uptake in thyroid bed consistent with thyroid tissue/ residual disease. No distant metastasis was noted. Discussion – Sclerosing mucoepidermoid cancer is thought to be derived from the ultimobranchial body.1 It is more common in females in age groups 3rd - 8th decade.3 Most common presentation is unilateral painless thyroid mass. Coexistent chronic lymphocytic thyroiditis and papillary thyroid cancer are usually found. On literature review of 56 cases, the gross extrathyroidal extension was seen in 26% of cases with distant metastasis was most commonly seen in lungs.3 No established treatment strategy exists due to a small number of reported cases, however, treatment ranges from only Thyroid surgery to Thyroid surgery with radiation +/- chemotherapy and radioactive iodine. References Shah AA, La Fortune K et al. Thyroid sclerosing mucoepidermoid carcinoma with eosinophilia: a clinicopathologic and molecular analysis of a distinct entity. Mod Pathol. 2017;30: 329–339. Pantola C, Kala S, Athar M, et al. Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid: a cytological dilemma. J Cytol 2016; 33: 37. Chambers M, Nosé V et al. Salivary-Like Tumors of the Thyroid: A Comprehensive Review of Three Rare Carcinomas. Head Neck Pathol. 2021 Mar;15(1): 212-224. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

SACROCOCCYGEAL CHORDOMA: A RARE CASE REPORT DIAGNOSED ON CYTOLOGY

Chordomas are rare malignant tumors of notochordal origin. They commonly arise in 5th and 6th decade with a male predominance of 3:1. They have indolent nature and rarely metastasize. We report a case of 71 year old male presenting with lower backache diagnosed as Chordoma on FNAC and later conrmed on histopathology.

A study on role of DSA in intracerebral vascular anomaly comparison with computed tomography angiography/magnetic resonance angiography

Background: Brain imaging techniques provide the ability to noninvasively map the structure and functions of the brain. Brain vascular malformation mainly affects people in the 5th decade followed by 4th and 3rd decade. Aims and Objectives: The aim of the study was to compare the diagnostic supremacy of computed tomography angiography (CTA)/magnetic resonance angiography (MRA) and Digital subtraction angiography (DSA) for the detection of intracranial vascular anomalies. Materials and Methods: An observational descriptive study with cross-sectional design was performed among 50 patients of both sexes undergoing DSA test at Medical College and Hospital, Kolkata with acute stroke syndrome or any other symptoms suggesting intracranial vascular lesion, who were investigated with one or more index tests and a reference standard diagnosed by computed tomography (CT) or MR scanning or other parameters. DSA served as the standard of reference for presence of intracranial vascular anomalies. Results: Out of the 50 patients included in the study, 41 were diagnosed with vascular malformations by DSA. Moya moya disease was diagnosed in three and distal AV fistula in six patients. In the 41 patients with vascular malformations, CTA could correctly identify 17 (41.5%) cases whereas MRA could identify 73.2% cases. Conclusion: DSA can be used for both diagnostic and interventional angiography. Its high spatial and temporal resolution have maintained DSA as a very important tool. The study reveal DSA is more superior to accurate angioarchitectural delineation of different intracerebral vascular malformation.

STUDY OF PATTERN OF ANTERIOR UVEITIS IN RURAL SET UP

Introduction:Anterior uveitis includes iritis, iridocyclitis and anterior cyclitis. Aim of the study is to evaluate clinical profile of anterior uveitis in tertiary care centre in Western Maharashtra. Methodology:An observational ,descriptive , cross sectional study was conducted at tertiary care hospital in rural set up. A total 35 eyes of 27 patients diagnosed with anterior uveitis were evaluated. All patients underwent complete ophthalmic evaluation and systemic examination. Results:This study included 35 eyes of 27 patients. Anterior uveitis affected most commonly in people of age group of 5th and 6th decade. No gender predilection was seen. In majority of patients etiology was idiopathic (19 cases /70.37%) while of Rheumatoid Arthritis 2 cases were seen (7.40%). Majority of patients had non- granulomatous type of uveitis 91.42%. Unilateral presentation was seen in 70.37% of cases. Most common complication seen was complicated cataract seen in 7 eyes (20%).). Most of the eyes with anterior uveitis had cells of grade grade 2+( 37.14 %) and flare 1+ (28.57%). Conclusion:Anterior uveitis was most commonly observed in 5th and 6th decade of life. In most of the patients etiology was idiopathic. Acute anterior uveitis was most common common case presentation. Majority of patients of anterior uveitis had unilateral involvement and complicated cataract was the most common complication seen.

The role of age, sex, and multimorbidity in 7-year change in prevalence of limitations in adults 60–94 years

Recent data suggest a temporal trend in decline in functional limitations in older adults but whether this trend extends to the period after the 8th decade of life remains unclear. We examined change in prevalence of limitations in activities and instrumental activities of daily living (ADL and IADL) between 2008 and 2015 among adults of 60–94 years and the role of age, sex, multimorbidity; we also examined changes in severity of limitations. Data were drawn from two nationally representative surveys in 2008 (n = 13,593) and 2015 (n = 13,267). The 6-item scales of ADL and IADL were each categorized first as ≥ 1 limitations, and then to examine severity as 0, 1–2, and ≥ 3 limitations. Weighted logistic and multinomial regressions were used to estimate prevalence of limitations; the difference between surveys were extracted every 5 years between 60 and 90 years. The prevalence of ≥ 1 ADL declined between 2008 and 2015, from age 75 (− 1.2%; 95%CI = − 2.0, − 0.4%) to age 90 (− 8.8%; 95%CI = − 12.7, − 5.0%). This decline was more pronounced in men than women (p-value for interaction = 0.05) and observed primarily in those with multimorbidity (p-value for interaction = 0.06). Up to 2 ADL limitations declined from age 75 (− 1.0; 95%CI = − 1.7, − 0.3) to 90 (− 6.7; 95%CI = − 9.9, − 3.6) and from age 80 (− 0.6; 95%CI = − 1.1, 0.1) to 85 (− 1.2; 95%CI = − 2.2, − 0.1) for ≥ 3 ADL limitations. There was no substantial change in IADL limitations. These data from a high-income country with universal health care show improvement in ADL even after the 8th decade of life despite increase in multimorbidity.

Frequency of Prediabetes in patients of HIV Infection Presenting at Tertiary Care Hospital

Objective: To find out the frequency of prediabetes in patients of HIV presenting at tertiary care hospital. Study design: Cross sectional study. Place and duration: Department of Medicine, D.G Khan Hospital, D.G Khan. (January 2020 to December 2020) Methodology: Total 187 patients of HIV infection, age 20 years to 60 years both male or female with duration of HIV 10 years were selected and pre-diabetes was studies. Results: Total 187 patients of HIV infection was recruited for this study and prediabetes was assessed. Mean age and mean HIV infection duration was 37.81 ± 12.81 years and 5.31 ± 2.86 years. Prediabetes was noted in 63 (34%) patients. Male patients and female patients were 122 (65.24%) and 65 (34.76%) respectively. Prediabetes was found in 58 (47.54%) males and in 5 (7.69%) females. Prediabetes was significantly (P=0.000) associated with gender. Conclusion: This study showed a higher percentage of prediabetes among HIV infected patients. Most of the cases were belonged to 3rd and 4th decade of life. Males were more victim of HIV infection as compared to females and most of the males were prediabetics. No association of development of prediabetes with duration of HIV infected was noted. Keywords: Prediabetes, HIV infection, diabetes mellitus, IGT

Clinical Profile of Acute Exacerbation of Chronic Obstructive Pulmonary Disease Patients Admitted in Tertiary Care Hospital

Background: COPD is one of the most widespread noncommunicable diseases, and its incidence is on the rise in developing countries. Acute COPD exacerbation is linked to a higher financial burden on the health system, as well as a negative impact on patients’ health in terms of lost working days, functional capacity, and mortality. COPD exacerbation is characterized by a worsening of symptoms such as cough and dyspnea, as well as a considerable risk of type-II respiratory failure. An understanding of the symptoms and signs, as well as the causes linked to acute COPD exacerbation in our population, is thought to aid in the prevention of such exacerbations, reducing the burden on patients and the community. Aim: This study is meant to bring forth the clinical profile and variables related with acute exacerbation of COPD among our community, taking into account the specific symptoms preceding an exacerbation of COPD and the variety of factors associated with it in different parts of the world. This study characterizes the clinical signs and symptoms of COPD acute exacerbations, as well as investigates the involvement of a respiratory tract infection in COPD exacerbation establishing a relationship between smoking and COPD exacerbation. Method: This is a hospital-based cross-sectional observational study. The study was conducted at the Dhaka Medical College Hospital’s Department of Medicine (DMCH). A method of purposive sampling was applied. The study took place from November 2012 until June 2013. The sample size computed for this study is 75. Result: Age above 7th decade increases mortality risk by 2.32 times. Meanwhile, comorbidity, low BMI (<18.5 kg/m2), smoking and frequent acute exacerbation increases risk by 1.29 times, 1.16 times, 0.03 times and by 2.07 times respectively in patients having COPD. Conclusion: Chronic obstructive pulmonary disease (COPD) is more frequent in the 6th decade of life and is male predominant. Recovery period has been found longer in the presence of dyspnea or symptoms of a common cold at exacerbations onset, higher PaCO2 and lower pH has been found in the patients admitted in ICU. Low BMI, RTI, Smoking and Comorbidity are more frequent in patients with chronic obstructive pulmonary disease (COPD). Sir Salimullah Med Coll J 2022; 30: 161-167

Loss of function variants in DNAJB4 cause a myopathy with early respiratory failure.

DNAJ/HSP40 co-chaperones are integral to the chaperone network, bind client proteins and recruit them to HSP70 for folding. We performed exome sequencing on patients with a presumed hereditary muscle disease and no genetic diagnosis. This identified four individuals from three unrelated families carrying an unreported homozygous stop gain (c.856A > T; p.Lys286Ter), or homozygous missense variants (c.74G > A; p.Arg25Gln and c.785T > C; p.Leu262Ser) in DNAJB4. Affected patients presented with axial rigidity and early respiratory failure requiring ventilator support between the 1st and 4th decade of life. Selective involvement of the semitendinosus and biceps femoris muscles was seen on MRI scans of the thigh. On biopsy, muscle was myopathic with angular fibers, protein inclusions and occasional rimmed vacuoles. DNAJB4 normally localizes to the Z-disc and was absent from muscle and fibroblasts of affected patients supporting a loss of function. Functional studies confirmed that the p.Lys286Ter and p.Leu262Ser mutant proteins are rapidly degraded in cells. In contrast, the p.Arg25Gln mutant protein is stable but failed to complement for DNAJB function in yeast, disaggregate client proteins or protect from heat shock-induced cell death consistent with its loss of function. DNAJB4 knockout mice had muscle weakness and fiber atrophy with prominent diaphragm involvement and kyphosis. DNAJB4 knockout muscle and myotubes had myofibrillar disorganization and accumulated Z-disc proteins and protein chaperones. These data demonstrate a novel chaperonopathy associated with DNAJB4 causing a myopathy with early respiratory failure. DNAJB4 loss of function variants may lead to the accumulation of DNAJB4 client proteins resulting in muscle dysfunction and degeneration.

Relationship of age and gender with cytopathological findings of thyroid nodules diagnosed by FNAC: a retrospective study.

In Afghanistan, there are no reliable epidemiological data available about thyroid cytopathology and its possible relationship with the age and gender of patients. Therefore, we conducted this study to outline this relationship and the importance of fine needle aspiration cytology in this regard. A retrospective review study was conducted including 686 consecutive medical records of thyroid nodules diagnosed by fine needle aspiration cytology in a span of five years. Out of 686 consecutive patients with thyroid nodules included in this study, 566 were females and 120 were males. Most of the thyroid nodules diagnosed were benign lesions with female predominance. These nodules were commonly arising in between 3rd and 6th decades of life in both the genders with mean age of 42 ± 13years in females and 52 ± 15years in males. Thyroid malignancy was commonly diagnosed in middle-age females, however in males, it was frequently diagnosed in older age with a peak at 7th decade. Most of the malignant nodules were solid and complex with no purely cystic nature. In both the genders, thyroid nodules were commonly arising in the right thyroid lobe (52.3%), followed by left (35.7%), isthmus (8.7%) and bilateral (3.2%). Fine needle aspiration cytology remains the modality of choice for the diagnostic evaluation of nodular lesions of thyroid. In contrast to the data reported around the world, thyroid malignancies, in our study, were diagnosed in relatively older age groups with peak incidence in the 5th decade of life for females and 7th decade for males.

“CLINICAL PRESENTATION OF HYPERTENSIVE EMERGENCY AND URGENCY PATIENTS”

Arterial hypertension affects approximately a billion people worldwide and approximately 30 % of them remain undiagnosed.1% - 2% of known hypertensive patients will have hypertensive emergency at some time in their life Though hypertensive crisis contributes one fourth of all emergency visits and trend is increasing in last few years still there is lack of data over the actual incidence of hypertensive crisis The present study aims to know the incidence and clinical presentation of the hypertensive crisis ( hypertensive emergency and urgency ) in a tertiary hospital and will help us to know the incidence and pattern of clinical presentation with respect to different aspects. Methods; This is a prospective observational study of 100 cases of hypertensive crisis (hypertensive emergency and hypertensive urgency) presenting to the emergency department. Study conduct during 2016 to 2018. Results; The incidence of hypertensive crisis was found to be 20%.Most of the patients were belonged to the 5th and 6th decades of life and Male patients were more than female patients Majority of the patients were known hypertensives for 6 to 10 years and 22% of the known hypertensive patients has discontinued their anti hypertensive medications Cardiovascular symptoms in the form of dyspnoea & chest pain were the most common accompanying symptoms Acute coronary syndrome was the most common clinical manifestation followed by neurological decit. Conclusion; As the trend of hypertensive crisis is increasing efforts should be made to reduce the occurrence of hypertension Preventive measures should be taken in the form of community education for dietary modication and routine exercise Routine blood pressure check ups compliance to the anti hypertensive treatment and control of associated comorbidities is essential to reduce the risk of hypertensive crisis.

Moyamoya disease incidentally discovered in head trauma

Moyamoya disease is a chronic cerebral vascular disease of undetermined origin, characterized by stenosis and progressive occlusion of the distal portion of the internal carotid arteries and the proximal portion of the middle and anterior cerebral arteries with resulting, abnormal vascular network development at the base of the skull, realizing a smoke volute appearance or moyamoya. The disease is most common in East Asia. It has two peaks of incidence: around the age of 05 years and during the 4th decade with a female predominance. In children, it leads more frequently to repeated ischemic strokes, while in adults, cerebral hemorrhages are observed. Cerebral MRI and arteriography are the imaging modalities allowing the positive diagnosis of the disease as well as the planning of the management which is based on a revascularization surgery which must be indicated at the optimal period during the follow-up of the disease.

Clinico etiological spectrum of unilateral vocal cord paralysis - A prospective study

Unilateral vocal cord palsy is relatively common in Otorhinolaryngology clinics. Due to reasons more than one, the exact incidence of the etiology behind unilateral vocal cord palsy has remained a mystery difficult to unfold. The present study was undertaken to determine the clinical presentation and etiopathology of unilateral vocal cord paralysis. The present study was a prospective study undertaken in the Department of Otorhinolaryngology at KIMS Hospital, Secunderabad from June 2018 to May 2020 after the approval of the Ethical Committee among 50 adult patients with a confirmed diagnosis of Unilateral Vocal cord palsy. Patients with the fixity of the vocal cord, laryngeal malignancies, and bilateral Vocal cord palsy/paresis were excluded from the study. A thorough history and ENT, head & neck examination followed by a radiological examination to make an etiological diagnosis of Vocal cord palsy were done. For patients with no cause found, a CT/ MRI imaging was done before labeling them as idiopathic. Most of the patients presented in the 4th and 5th decades of life, with male predominance. The most common presenting symptom was hoarseness of voice (80%) and Left-side vocal cord palsy (56%) is more common. The most common cause was idiopathic (34%) followed by post-surgical trauma, neoplastic, central cause, cardiac, granulomatous disorders, and neurological and post-intubation trauma respectively. In conclusion, our study shows thorough evaluation and investigation of cases with unilateral vocal cord palsy unveils interesting and sometimes rare and probably life-threatening underlying pathologies, that could be managed appropriately.

Incidence of in-hospital all-cause mortality, resource utilization and complications in patients with adult congenital heart disease undergoing TAVR-a national inpatient sample study

Abstract Background The prevalence of congenital heart disease (CHD) in adults in the United States is approximately 1.4 million. (1) With the advancement in diagnostic modalities and advanced treatments, including minimally invasive techniques, the life expectancy of individuals with CHD has greatly improved. (2) As these patients enter the 8th decade of their lives, the risk of calcification and aortic stenosis increases like the population without CHD. Current evidence supports transcatheter aortic valve replacement (TAVR) over surgical aortic valve replacement in individuals with moderate to high surgical risk. (3) Adults with acyanotic CHD (ACHD) with a higher risk for surgical complications are candidates for consideration of TAVR. There are sparse data about the cardiovascular outcome in these patients. Purpose With this National inpatient sample (NIS) study, the authors have shown the incidence of in-hospital all-cause mortality, resource utilization, and complications in adult patients with ACHD undergoing TAVR. Methods NIS 2016–2018 were utilized to conduct the study. Analyses were performed using STATA, version 16.0. Using appropriate ICD-10-PCS codes, authors identified adult patients with ACHD undergoing TAVR. The primary outcome of the study is to identify the impact of ACHD on all-cause in-hospital mortality and complications. Secondary outcomes of interest were resource utilization. Results 134,170 patients were identified who had TAVR done between 2016–2018. Patients aged ≤18 years were excluded (N=25). Out of 134,170 patients that underwent TAVR, 1,170 (0.87%) were noted to have ACHD. Using the greedy algorithm, 1,115 matched pairs were generated. The ACHD group had a higher burden of co-morbidities including atrial fibrillation (46.2% vs. 38.8%, p=0.016), pulmonary hypertension (27.4% vs. 17.5%, p<0.001), metabolic syndrome (1.3% vs. 0.3%, p=0.005), peripheral vascular disease (29.5% vs. 24.1%, p=0.049), alcohol use disorder (3.0% vs. 1.3%, p=0.018), coagulation disorder (22.7% vs. 12.8%, p<0.001), drug abuse (1.3% vs. 0.4%, p=0.043), liver disease (7.3% vs. 3.1%, p<0.001) and electrolyte disturbances (20.5% vs. 14.9%, p=0.017). We also noted a possible trend towards higher complication odds (cardiac complications such as the need for pericardial drain or cardiac implantable electronic device and cardiac arrest) in patients with ACHD undergoing TAVR without statistical significance based on multivariate analysis. On propensity matching, no difference was found in the incidence of overall cardiac complications between patients with ACHD and patients without ACHD, except STEMI (OR 4.16, 95% CI, 1.08–16.00, p=0.038). Conclusion(s) The study points towards the possible safety of pursuing TAVR in ACHD patients provided adequate technical support and operator competency. Funding Acknowledgement Type of funding sources: None.

S3521 A Rare Case of Duodenal Leiomyoma

Introduction: Only 1–5% of gastrointestinal neoplasms are found in the small intestine. Adenocarcinomas, lymphomas, and neuroendocrine tumors are the most common malignant tumors seen in the small intestine, while gastrointestinal stromal tumors, leiomyosarcomas, and leiomyomas are uncommon. The jejunum, ileum, and duodenum are the three most common locations for leiomyomas. We are presenting a rare case of a female with duodenal leiomyoma. Case Description/Methods: A 70-year-old female presented to the hospital with complaints of fatigue and weakness. Laboratory evaluation showed iron deficiency anemia with hemoglobin 7.5g/dl and hematocrit 25.4%. The patient reported an episode of black tarry stool about a week before the presentation. The patient received one packed red blood cell transfusion, and upper gastrointestinal endoscopy (EGD) was performed to evaluate anemia. EGD revealed non-erosive gastritis and gastric erosions without any current or recent bleeding stigmata. A single large pedunculated polyp was found in the duodenum's first portion, extending into the second portion of the duodenum(a). Biopsy from the polyp revealed submucosal leiomyoma without any evidence of dysplasia or malignancy(b). The next day, a colonoscopy showed significant diverticulosis and non-bleeding internal hemorrhoids. The patient remained hemodynamically stable and was discharged home the next day. The patient presented about a month later with iron deficiency anemia again. Repeat EGD and colonoscopy performed this time showed no obvious source of bleeding. We also conducted a pill cam study, which also came back negative. (Figure) Discussion: It is unusual for leiomyomas, which are benign smooth muscle tumors, to develop in the gastrointestinal tract. Males are more likely than females to be diagnosed with duodenal leiomyomas in the 6th and 7th decades of life. The origin and pathology of these tumors in the gastrointestinal tract remain a mystery. Leiomyomas of the duodenum seldom cause symptoms. When a duodenal tumor is larger, it might cause more severe symptoms, such as bleeding, nausea, vomiting, and weakness. In our patient described above, we believe the leiomyoma caused bleeding, as no other definitive source of bleeding was found even after a complete evaluation. The case describes a rare duodenal tumor with an unusual presentation.Figure 1.: a. EGD showing a large pedunculated polyp extending from the first portion to the second portion of the duodenum. b. H&E stain showing submucosal benign spindle cell proliferation. No atypia, pleomorphism or mitotic activity.

S3414 Goblet Cell Adenocarcinoma Presenting as Appendicitis

Introduction: Goblet Cell Adenocarcinomas (GCAs) are a very rare subtype of appendiceal tumors, with an estimated incidence of 1 per 2 million people. They are not only rare but also unique in terms of their origin. Whereas most cancers arise from one cell line, goblet cell adenocarcinomas are a hybrid of neuroendocrine cells and epithelial cells. The aim of this abstract is to provide an overview of this unique clinical entity. Case Description/Methods: We present a case of a 62-year-old female with past medical history of hypertension, diabetes mellitus and hyperlipidemia who presented to the ED with a one day history of right lower quadrant abdominal pain that had migrated from the periumbilical area, associated with non-bloody vomiting. On arrival, she was stable and her physical examination was significant for tenderness in the right lower quadrant and a positive Rovsing and obturator sign. Labs were remarkable for leukocytosis with left shift. CT scan of the abdomen was done which revealed an inflamed appendix and subsequently patient was taken for a laparoscopic appendectomy. Pathology of the surgical specimen revealed a well differentiated T1NxMx GCA with negative margins and no lymphovascular or perineural invasion. A decision was made to proceed with a right hemicolectomy for the purpose of staging and treatment. Pathology sent after right hemicolectomy revealed no residual GCA with 21 lymph nodes negative for metastasis and negative surgical margins (Figure). Discussion: First described in 1969, GCAs mostly occur in the 5th and 6th decade of life with equal prevalence in both genders. GCAs most commonly present as acute appendicitis but can also present with abdominal pain associated with abdominal mass and weight loss. They can metastasize to the peritoneum, omentum, abdominal wall, and ovaries. Majority of GCAs are diagnosed incidentally on pathological examination of inflamed appendix after appendectomy. The mainstay of treatment is surgical resection with the addition of adjuvant chemotherapy for node-positive (stage III) disease or higher. Due to the rarity of this condition, there is lack of consensus regarding the extent of surgical resection. For stage I disease appendectomy alone maybe sufficient, but higher stages may require hemicolectomy. Prognosis depends on the grade and stage at diagnosis, with one retrospective analysis reporting 5-year survival rates of 100%, 76%, 22%, and 14% for stages I, II, III, and IV, respectively.Figure 1.: 1 and 2 - CT scan of the abdomen showing distended appendix with appendiceal wall thickening and hyperemia, as well as trace periappendiceal edema. 3 - Histologically conventional Goblet cell adenocarcinoma has a “crypt cell pattern” characterized by small round clusters of goblet cells in acinar configuration, resembling colonic crypts. [Pathology slide from Mod Pathol. 2016;29(10):1243-1253. DOI: 10.1038/modpathol.2016.105]).

The role of Anti-VEGF agents in treatment of neovascular glaucoma.

Aim: The aim of this study was to show the efficacy of intravitreal treatment with Bevacizumab (Avastin) in patients with secondary neovascular glaucoma, in different stages of the disease. Method: A retrospective study was performed on 67 patients with neovascular glaucoma. The main parameters evaluated were the patients' history, slit lamp examination, visual acuity, ocular tonometry, fundus examination, gonioscopy, and visual field. Results: It was observed that the pathology had a preponderance in males of the 6th decade, with frequently unilateral damage. Patients were referred to an ophthalmologist when the diseases reached an advanced stage, usually when the visual acuity had no light perception and the intraocular pressure was over 45 mmHg. However, the treatment with Avastin intravitreal showed a good evolution, with regression of neovessels in the first 4-7 days and maintenance of intraocular pressure within normal limits in about 60% of cases, 3 months after injection. Conclusion: The most effective treatment in secondary neovascular glaucoma is the correct therapy of the main disease. The association of Avastin and laser photocoagulation leads to regression in iris and retinal neovessels. Abbreviations: anti-VEGF = anti-Vascular Endothelial Growth Factor, PDGF = Platelet Derived Growth Factor, bFGF = basic Fibroblast Growth Factor.

S3411 Extensive Hepatic Lesions From a Primary Duodenal Neuroendocrine Carcinoma in a Young Male

Introduction: Neuroendocrine neoplasms (NEMs) are relatively rare, accounting for 0.5% of newly diagnosed malignancies. NEMs can be found throughout the body with two-thirds arising in the GI tract and incidence increasing among duodenal NEMs. These malignancies have a female predominance and generally occur in the 6th decade of life. This is a case of advanced metastatic disease from an unexpected duodenal neuroendocrine carcinoma in a healthy young male. Case Description/Methods: A 29-year-old male, with no PMH, acutely developed sharp RUQ abdominal pain. Prior to this, he was in his usual state of health. In the ED, the patient was hemodynamically stable with tenderness to palpation in the RUQ. Initial labs showed WBC 12.10 × 103/uL, Hgb 11.7 g/L, AST 55 U/L, ALT 173 U/L, ALP 127 U/L, and TBIL 0.8 mg/dL. CT A/P showed multiple well-circumscribed liver lesions, largest measuring 8.6 × 7.8 cm, with portal venous invasion (Figure A). CA 19-9 and CEA were negative. There was elevation of AFP 1,577 ng/mL and LDH 817 U/L, but negative hCG. A1AT, ANA, AMA, ASMA, ALKM, and ceruloplasmin were negative. Liver biopsy showed a poorly-differentiated malignant neoplasm of unknown primary. EGD was performed revealing a fungating mass in the second portion of the duodenum (Figure B). Pathology favored a high-grade poorly-differentiated neuroendocrine carcinoma (Figure C). He was discharged with palliative cisplatin/irinotecan initiation. Discussion: Gastrointestinal NEMs have been increasing in prevalence due to improved awareness. When carcinoid symptoms are absent, detection is dependent on imaging and endoscopy after the patient presents with complications from metastasis or mass effect. Following biopsy, GI NEMs are classified by WHO 2010 criteria into 3 groups based on mitotic count and Ki-67 index. Group 3 tumors are classified as poorly-differentiated neuroendocrine carcinomas with Ki-67 >20% with higher rates of angioinvasion and metastatic potential. Lymph node metastasis occurs in 60% of duodenal NETs, while liver mets occur in < 10%. In our case, the patient’s Ki-67% index was >90%, characterizing it as a Group 3 NEM. Characterization is important given the impact on prognosis and treatment. Group 3 NEMs have the overall worst prognosis. Since >90% of duodenal NEMs do not cause a clinical syndrome and are not associated with metastatic disease, surgical or endoscopic resection is preferred. However, if there is metastatic disease or high-grade histologic features, chemotherapy is the mainstay of treatment.Figure 1.: A, CT A/P with multiple large heterogeneously enhancing liver lesions concerning for metastatic malignancy of unknown primary (B) Large fungating mass located in the second portion of the duodenum (C) Duodenal mass, H&E stain, 20×, high-grade poorly differentiated epithelial malignancy composed of nested cells with indistinct nucleoli, moderate eosinophilic cytoplasm, and brisk mitosis.

REMOTE MONITORING OF CYCLE TRENDS IN PATIENTS OF ADVANCED MATERNAL AGE - AN OPPORTUNITY TO STREAMLINE ACCESS TO IVF

Advanced maternal age (AMA) is associated with a decline in both ovarian reserve and oocyte competence. Yet, the proportion of individuals delaying childbearing until the 3rd-early 4th decade of life has greatly increased. In AMA patients, an infertility work-up is recommended after 6 months of regular unprotected intercourse. We looked to compare quantitative hormone data and cycle trends among AMA and non-AMA patients to inform patient management.