Laryngotracheobronchial Amyloidosis: Patterns of Presentation and Management

2021 ◽  
pp. 000348942110427
Author(s):  
Sarah M. Dermody ◽  
Erica L. Campagnaro ◽  
Robbi A. Kupfer ◽  
Norman D. Hogikyan ◽  
Robert J. Morrison

Objective: To evaluate the pattern of presentation and management of laryngotracheobronchial amyloidosis at a tertiary care academic center over a 27 year period. Methods: In a retrospective review, the electronic medical record at a tertiary care academic center was queried for encounters with 3 laryngologists between 1996 and 2019 which included the ICD-9 or ICD-10 diagnosis of amyloidosis. Demographics, clinical presentation, referral diagnoses, medical history, family history, laboratory values, radiology studies, and treatment modalities of subjects were collated. Results were analyzed using standard univariate descriptive statistics. Results: Seventeen subjects were identified with an average age at diagnosis of 58 years (range 26-76 years). The most common amyloid type on biopsy was immunoglobulin light chain (AL) subtype. The most common location of laryngeal amyloid at diagnosis was the glottis and disease was more likely to be bilateral at the time of diagnosis in this location. Supraglottic disease more often had a unilateral presentation and had a tendency to spread to additional laryngeal subsites. Nearly 25% of subjects had associated systemic disease, including multiple myeloma, auto-immune disease, and familial ATTR mutation. Conclusions: The overall rate of associated systemic disease was low in our study cohort; however, it is higher than typically referenced in extant literature. Our cohort demonstrates that while laryngeal amyloidosis is a chronic condition, the behavior is generally indolent with a low treatment burden.

2019 ◽  
Vol 6 (7) ◽  
pp. 2556 ◽  
Author(s):  
Sreeramulu P. N. ◽  
Srinivasan Dorai Swamy ◽  
Vikranth Suresh N. ◽  
Suma S.

Background: Liver abscess is a disease of frequent occurrence which is important in the differential diagnosis of upper abdominal and right lower respiratory tract diseases. Liver abscess are space occupying lesion in liver which has a higher incidence of mortality and morbidity. The aim is to study the clinical presentation and compare the outcomes of various treatment modalities.Methods: A Retrospective Study was conducted over a period of 3 years from November 2016 to October 2019, in tertiary care centre, R. L. Jalappa hospital, Tamaka, Kolar, Karnataka, India. 46 cases of liver abscesses were studied. Complete clinical details about the clinical presentation of the cases, investigative work up and treatment modalities adopted were collected. The associated morbidity and mortality of all patients were reviewed.Results: In our study, the mean age was 49.5 years which included male patients most commonly. Pyogenic liver abscess was more common than amoebic liver abscess. Right lobe of the liver was most commonly involved. The common treatment modality was continuous drainage of the abscess cavity by the percutaneous insertion of a pig tail catheter. Surgical intervention for the rupture was done in one patient.Conclusions: In our experience of managing liver abscess, pyogenic liver abscess involving right lobe of the liver was common with the presentation of upper abdominal pain, high grade fever with chills and tender hepatomegaly. Ultrasound abdomen is very useful investigative tool in diagnosis and also in intervention and in the follow up of the condition and to evaluate progression or resolution.


Author(s):  
Ahsan Ali Laghari ◽  
Ghulam Akbar Khaskheli ◽  
Aijaz Ahmed Shaikh ◽  
Ambreen Munir ◽  
Tufail Ahmed Baloch ◽  
...  

Objective: To determine the clinical presentation of carcinoma of thyroid and its treatment modalities at tertiary care Hospital. Study Design: Prospective observational study. Place and Duration: This study was conducted in Liaquat university of Medical and health sciences Jamshoro/Hyderabad, Pakistan, during two years from July 2017 to June 2020. Patients and Methods: All the patients with age range between 20 to 80 years having swelling in front of neck according to clinical examination with hard inconsistency along with change of voice lymphadenopathy, difficulty in swallowing and breathing, patients diagnosis with malignancy according to FNAC and CT, and either of gender were included. FNAC was done in each patient except toxic goiter, diffuse goiter and multinodular goiter with dominant nodules. Patients those were diagnosed with stage I and II, underwent surgical treatment and remaining were referred to oncology department for new adjuvant therapy and then surgeries were done if possible. All the data was recorded in study proforma and data analysis was done by using SPSS data analysis software version 20. Results: Out of all cases of suspicious of carcinoma thyroid, 70% were females. Neck swelling was in all of the cases, followed by pain, cervical lymphadenopathy, recurrent laryngeal nerve palsy with change of voice, swallowing difficulty, cyanosis and pathological fracture. According to tumor staging, most of the cases were found with benign tumor, followed by 5 cases with stage I, three cases with stage II, three patients presented with stage IV and one was seen with secondary’s from malignant melanoma. Most of the cases 70.0% underwent subtotal and near total thyroidectomies Eight patients were initially operated having stage I and II of malignancy and remaining 7 patients of stage III and IV were referring to oncology department for further management. Conclusion: In the conclusion of this study the neck swelling was the commonest clinical presentation. Surgical decision making and the operative planning is one of the new challenges. Mostly patients underwent subtotal and near total thyroidectomies having benign presentation and patients those had stage I and II of malignancy were initially operated, while patients of stage III and IV including secondary were referred to oncology.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-14
Author(s):  
Ankita Sen ◽  
Tuphan Kanti Dolai ◽  
Tuhin Suvra Gayen ◽  
Riya Roy ◽  
Aditi Sen ◽  
...  

Introduction:Sickle cell disorders were originally found in the African regions, Arabian Peninsula and parts of India. However, in today's age of globalization patients with homozygous or compound heterozygous Sickle cell disorders can be found all over the world. The objective of our study was to assess the distribution and clinical presentation of patients with Sickle homozygous or heterozygous diseases in the Eastern part of India. Methods:Patients who attended the Thalassemia Clinic in our tertiary care center, between 1st January 2018 to 31st May 2020 (2 years and 4 months) were retrospectively analysed and the ones with a component of Sickle haemoglobin(HbS), either in the form of Sickle cell anemia/homozygous Sickle cell disorders(SCA) or compound heterozygous diseases, like Sickle cell/β thalassemia(HbS/β), Sickle cell/Delta thalassemia(HbS/D), Sickle cell Haemoglobin/E thalassemia(HbS/E), were included in the study. People having Sickle cell trait (HbS trait), have also been included. Thorough history of painful crises, blood transfusions, family history and treatment history was elicited and every patient was clinically examined. The patients were diagnosed by High Pressure Liquid Chromatography (HPLC) or Thalassemia Mutation analysis by Polymerase Chain reaction (PCR). Results:A total of 95 patients with a component of HbS were considered as our study cohort, with HbS/β thalassemia patients being the majority (53.7%), followed by SCA (30.5%). Age of the study cohort ranged between 2-50 years age. HbS/β thalassemia patients presented at a later age (median 17.5 years) than SCA patients (median 12 years). Their demographic distribution is depicted in Table 1. The most common clinical presentation was painful crisis (32,33.7%), be it abdominal pain (11,11.6%) or bone pain (13,13.7%). Other presenting complaints were pallor (26,27.4%), jaundice (12,12.6%) and fever (4,4.2%). Some rarer presenting manifestations were fatigue (4,4.2%), splenic infarction (1,1%), convulsions (1,1%), Raynaud's phenomenon (1,1%), headache (1,1%) or itchy skin lesions (1,1%). Few patients (4,4.2%) had recurrent pregnancy loss, and one patient was diagnosed incidentally during an antenatal check-up. Most patients had more than one complaint. Very occasionally patients required hospital admission, the reasons being, chest pain, fever, convulsions or abdominal pain. HbS trait was diagnosed incidentally during evaluation for other illnesses, most commonly during evaluation of pallor (3,60%): one patient was later diagnosed with iron deficiency anemia. Most patients who attended our center were from within the state or neighbouring states. The patients were treated with Hydroxyurea, with/without blood transfusions, chelation therapy with Deferasirox as required and Folic acid supplementation. People with HbS Trait continued to receive Folate supplementation. Discussion and Conclusions:This study highlights the varied distribution of HbS among the population attending a tertiary care center, irrespective of a specific area-based population. Till date most studies conducted in India have highlighted the prevalence of Sickle cell disorders among specific focused populations. HbS/β thalassemia was the most common sickle cell disorder in our study. This is in contrast to most findings in published literature from other countries, where SCA is the commonest. Only one other study conducted in eastern India, has depicted a finding similar to ours. The median age of disease presentation was at a later age in our study, in contrast to findings in published literature from other countries. There is a variation in the severity of disease manifestation in our study cohort. The most common painful crisis was bone pain, followed by abdominal pain. Pallor was also one of the commonest presenting symptoms. Stroke, a common manifestation of SCA in other countries, was rare in our study cohort. Disclosures No relevant conflicts of interest to declare.


2020 ◽  
Vol 8 (1) ◽  
pp. 159
Author(s):  
Rajiv Sonarkar ◽  
Giriraj Gajendra ◽  
T. R. V. Wilkinson ◽  
Shreyas Sonawane

Background: Liver is a common site for space occupying lesions. The objective of this study was to determine the aetiologies of space occupying lesion of liver and their clinical presentation and correlate imaging; to evaluate the treatment modalities of space occupying lesion of liver and their outcome.Methods: A 2-year tertiary care teaching hospital based longitudinal study with 63 patients was done. Selection criteria was defined and a prestructured proforma was made to assess and note the findings.Results: The mean age of occurrence of space occupying lesions of liver was 47.19±14.53 years. Space occupying lesions of liver was commonly observed in 5th decade of life. Males were affected more than females. Metastatic lesion of liver were the most common cause of liver SOL (34.9%) followed by liver abscesses.Conclusions: Majority of space occupying lesions are metastatic lesion followed by liver abscesses. Majority of space occupying lesions were surgically managed.


2021 ◽  
Author(s):  
Rashid Usman ◽  
Duaa Ajaz Hussain ◽  
Muhammad Jamil Malik ◽  
Muhammad Waseem Anwar ◽  
Kishwar Ali

Abstract Background Peripheral arteriovenous malformations (AVMs) are congenital defects resulting in abnormal connections between veins and arteries. We investigated a group of patients with peripheral AVMs to determine whether there were any gender differences in disease presentation and the response of AVMs to various modalities of treatment. Methodology The patients in this cross-sectional study were divided into two groups based on gender and their demographic data, clinical presentation at the time of diagnosis and response to treatment was assessed. Both surgical and nonsurgical treatment options were used. Nonsurgical options employed in the study included immunotherapy, embolotherapy and ultrasound-guided foam sclerotherapy (UGFS) Results Out of 43 patients, 74.4% were females with a male to female ratio of 1:3. The mean age at presentation in males was 27 years and 17 years in females. 60% of the male patients presented with high-flow AVMs while 81% of the female patients presented with low-flow lesions instead. Half of the AVMs in males were on the trunk whereas, in females, 93.9% were on the extremities. UGFS alone was used in 95.3% of patients while 32.5% of patients underwent UGFS followed by surgical excision. One patient was treated with sirolimus. In 4.6% of cases, embolization followed by surgical excision was performed. Recurrence was recorded in 20.9% of cases. Conclusions The clinical presentation of AVMs is notably different among the two genders. Sclerotherapy and embolotherapy proved to be effective treatment options. Larger studies, however, are needed to substantiate these claims. Keywords arteriovenous malformations, vascular malformations, sclerotherapy, embolotherapy, sirolimus


Author(s):  
Naorem Sunanda Chanu ◽  
Vinodkumar Suresh Basavaradder ◽  
Cibi Darsani ◽  
Ahanthembi Sanaton

Background: Ectopic pregnancy is one of the most common life-threatening emergencies in early trimester of pregnancy. The aim of this study was to determine the incidence, age group, gravidity, parity, risk factors, clinical presentation, treatment modalities associated with ectopic pregnancy in the current scenario.Methods: We conducted a prospective study for the period of two years starting from Aug 2017 to Aug 2019 at JNIMS OBG Department.Results: A total of 94 patients who were diagnosed as ectopic pregnancy and they were analysed for clinical presentation, risk factors, operative findings and treatment modality. Majority of patients were in the age group of 25 to 30 years. Ectopic pregnancy was more commonly associated with history of prior abortions, prior LSCS and PID. Commonly presented with amenorrhea of 6-8 weeks with abdominal pain and bleeding PV, most common site being ampulla followed by isthmus. Most of the patients had ruptured pregnancy at presentation. Majority of the patients underwent salpingectomy and salpingectomy with contralateral tubectomy.Conclusions: Ectopic pregnancy diagnosis is a grey zone and challenging. Early diagnosis by keeping suspicion in first trimester with PV bleeding and pain abdomen with history of amenorrhea about ectopic pregnancy, which helps in management to reduce maternal morbidity and even mortality.


2008 ◽  
Vol 2008 ◽  
pp. 1-5 ◽  
Author(s):  
Mirna H. Farhat ◽  
Ali I. Shamseddine ◽  
Kassem A. Barada

Introduction. Small bowel cancers are rare. Accumulation of data regarding their clinical presentation, pathologic features, prognostic factors, treatment modalities, and outcome is difficult.Methods. This is a retrospective study of the medical records of 33 patients with small bowel cancers treated at the American University of Beirut-Medical Center over a 20-year period.Results. The study included 25 males (76%) and 8 females (24%). Median age at presentation was 56 years. Most common symptoms were abdominal pain (66.7%) and weight loss (57.6%). Thirteen patients presented with abdominal emergencies (39.3%). Lymphoma was the most common malignant tumor (36.4%), followed by adenocarcinoma (33.3%), leiomyosarcoma (15.2%), gastrointestinal stromal tumors (12.1%), and neuroendocrine tumors (3.0%). Tumors were located in the duodenum in 30% of patients, jejunum in 33%, and ileum in 36%. Resectability rate was 72.7% and curative R0 resection was achieved in 54.1% (13/24) of patients. 5-year survival of the 33 patients was 24.2%.Conclusion. Small bowel cancers are difficult to diagnose because of the nonspecific symptoms. Most patients present with advanced disease and have poor prognosis. Adenocarcinoma and duodenal location have the worst 5-year survival in contrast to stromal tumors and those with ileal location which have the best survival.


2020 ◽  
Vol 41 (S1) ◽  
pp. s453-s454
Author(s):  
Hasti Mazdeyasna ◽  
Shaina Bernard ◽  
Le Kang ◽  
Emily Godbout ◽  
Kimberly Lee ◽  
...  

Background: Data regarding outpatient antibiotic prescribing for urinary tract infections (UTIs) are limited, and they have never been formally summarized in Virginia. Objective: We describe outpatient antibiotic prescribing trends for UTIs based on gender, age, geographic region, insurance payer and International Classification of Disease, Tenth Revision (ICD-10) codes in Virginia. Methods: We used the Virginia All-Payer Claims Database (APCD), administered by Virginia Health Information (VHI), which holds data for Medicare, Medicaid, and private insurance. The study cohort included Virginia residents who had a primary diagnosis of UTI, had an antibiotic claim 0–3 days after the date of the diagnosis and who were seen in an outpatient facility in Virginia between January 1, 2016, and December 31, 2016. A diagnosis of UTI was categorized as cystitis, urethritis or pyelonephritis and was defined using the following ICD-10 codes: N30.0, N30.00, N30.01, N30.9, N30.90, N30.91, N39.0, N34.1, N34.2, and N10. The following antibiotics were prescribed: aminoglycosides, sulfamethoxazole/trimethoprim (TMP-SMX), cephalosporins, fluoroquinolones, macrolides, penicillins, tetracyclines, or nitrofurantoin. Patients were categorized based on gender, age, location, insurance payer and UTI type. We used χ2 and Cochran-Mantel-Haenszel testing. Analyses were performed in SAS version 9.4 software (SAS Institute, Cary, NC). Results: In total, 15,580 patients were included in this study. Prescriptions for antibiotics by drug class differed significantly by gender (P < .0001), age (P < .0001), geographic region (P < .0001), insurance payer (P < .0001), and UTI type (P < .0001). Cephalosporins were prescribed more often to women (32.48%, 4,173 of 12,846) than to men (26.26%, 718 of 2,734), and fluoroquinolones were prescribed more often to men (53.88%, 1,473 of 2,734) than to women (47.91%, 6,155 of 12,846). Although cephalosporins were prescribed most frequently (42.58%, 557 of 1,308) in northern Virginia, fluoroquinolones were prescribed the most in eastern Virginia (50.76%, 1677 of 3,304). Patients with commercial health insurance, Medicaid, and Medicare were prescribed fluoroquinolones (39.31%, 1,149 of 2,923), cephalosporins (56.33%, 1,326 of 2,354), and fluoroquinolones (57.36%, 5,910 of 10,303) most frequently, respectively. Conclusions: Antibiotic prescribing trends for UTIs varied by gender, age, geographic region, payer status and UTI type in the state of Virginia. These data will inform future statewide antimicrobial stewardship efforts.Funding: NoneDisclosures: Michelle Doll reports a research grant from Molnlycke Healthcare.


2021 ◽  
Vol 15 ◽  
pp. 117955652110216
Author(s):  
Parisa Oviedo ◽  
Morgan Bliss

Objective: Masses of the sternoclavicular area are rare, and are not well described in the literature. We aim to present a series of patients with masses in this location and to review all reported English language cases of sternoclavicular masses in pediatric patients. Methods: This is a case series of pediatric patients with masses of the sternoclavicular area presenting to a tertiary care pediatric hospital from 2010 through 2017. Data was collected by using ICD-9 and ICD-10 codes to query the electronic medical record. Chart review included age at presentation, mass characteristics, medical and surgical interventions, and pathology results. A review of the literature was then performed. Results: Ten patients with masses overlying the sternoclavicular area were identified. Four patients presented with abscess and were treated with incision and drainage. Three of these patients were then treated with staged excision once infection cleared. Two additional patients were treated with primary excision. Four patients were treated with observation. The most common histopathologic finding was epidermoid. One patient was found to have a dermoid cyst, and 1 had a congenital cartilaginous rest. Conclusion: Epidermoids and dermoids are the most common masses overlying the sternoclavicular area. Controversy remains regarding the embryologic origin of sternoclavicular masses. The differential for masses in this area also includes branchial remnants, bronchogenic cysts, ganglion cysts, or septic arthritis.


Author(s):  
Essam Abdelhameed ◽  
Ahmed Ali Morsy

Abstract Background Primary intradural spinal arachnoid cysts are rare pathologies of uncertain etiology and variable presentation from no symptoms to myelopathy or radiculopathy according to cord or root compression. MRI with diffusion and contrast differentiates them from many pathologies. There is a lot of debate regarding when to treat and how to treat such rare pathologies. Objective We present a series of 10 primary intradural arachnoid cysts and evaluate outcome after surgery. Methods This retrospective study includes patients having primary intradural spinal arachnoid cysts operated in two tertiary care centers from October 2012 till October 2019. Symptomatic cysts were subjected to microsurgical resection or outer wall excision and inner wall marsupialization under neurophysiological monitoring. The Japanese Orthopedic Association Score was used for clinical evaluation while MRI with contrast and diffusion was used for radiological evaluation before and after surgery. Results This series included 10 patients, 4 males and 6 females, with mean age of 40 years. Pain was the most common presentation. The most common location was dorsal thoracic region. Total excision was achieved in 2 cases and marsupialization in 8 cases. All symptoms improved either completely or partially after surgery. No neurological deterioration or recurrence was reported during the follow-up period in this series. Conclusion Treatment of symptomatic primary intradural spinal arachnoid cysts should be microsurgical resection, when the cyst is adherent to the cord, microscopic fenestration can be safe and effective.


Sign in / Sign up

Export Citation Format

Share Document