scholarly journals Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

2014 ◽  
Vol 53 (5) ◽  
pp. 403-412 ◽  
Author(s):  
Masayuki Matsuda ◽  
Nagaaki Katoh ◽  
Shu-ichi Ikeda
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rachael Chan ◽  
Stephanie Carpentier

Abstract Background Amyloidosis is characterized by extracellular tissue deposition of fibrils, composed of insoluble low-molecular-weight protein subunits. The type, location, and extent of fibril deposition generates variable clinical manifestations. Gastrointestinal (GI) bleeding due to amyloid deposition is infrequent. Previous literature describes upper GI bleeding (UGIB) in patients with known amyloid disease. Here, we describe a case of recurrent UGIB that ultimately led to a diagnosis of GI amyloidosis and multiple myeloma in a patient with no history of either. Case presentation A 76-year-old male presented to the emergency department with frank hematemesis, melena, and a decreased level of consciousness. Management required intensive care unit (ICU) admission with transfusion, intubation, and hemodynamic support. Upper endoscopy revealed gastritis with erosions and nodularity in the gastric cardia and antrum. Hemostasis of a suspected bleeding fundic varix could not be achieved. Subsequently, the patient underwent computerized tomography (CT) angiography and an interventional radiologist completed embolization of the left gastric artery to address potentially life-threatening bleeding. Complications included development of bilateral pleural effusions and subsegmental pulmonary emboli. Pleural fluid was negative for malignancy. He was transferred to a peripheral hospital for continued care and rehabilitation. Unfortunately, he began re-bleeding and was transferred back to our tertiary center, requiring re-admission to the ICU and repeat endoscopy. Repeat biopsy of the gastric cardial nodularity was reported as active chronic gastritis and ulceration. However, based on the unusual endoscopic appearance, clinical suspicion for malignancy remained high. He exhibited symptoms of congestive heart failure following standard resuscitation. Transthoracic echocardiogram (TTE) demonstrated a reduced ejection fraction of 35–40% and a strain pattern with apical sparing. Following discussions between the treating gastroenterologist, consulting cardiologist, and pathologist, Congo Red staining was performed, revealing submucosal amyloid deposits. Hematology was consulted and investigations led to diagnosis of multiple myeloma (MM) and immunoglobulin light-chain (AL) amyloidosis. The patient was treated for MM for four months prior to cessation of therapy due to functional and cognitive decline. Conclusions GI amyloidosis can present with various non-specific clinical symptoms and endoscopic findings, rendering diagnosis a challenge. This case illustrates GI amyloidosis as a potential—albeit rare—etiology of UGIB.


2018 ◽  
Vol 49 (1) ◽  
pp. 59-61 ◽  
Author(s):  
Manijeh Nourian ◽  
Aliehsan Heidari ◽  
Saleheh Tajali ◽  
Erfan Ghasemi ◽  
Mehdi Mohebali ◽  
...  

Visceral leishmaniasis (VL) is a neglected disease. Our retrospective study describes 38 clinical and epidemiological characteristics of VL in patients admitted to a paediatric hospital in Tehran, Iran, who came from different geographical regions, indicating that the disease has spread to most parts of the country. Some 76.3% of the children documented suffered with symptoms of the disease for two months before admission.


2022 ◽  
Vol 8 ◽  
Author(s):  
Han Zhang ◽  
Yingying Wu ◽  
Yuqing He ◽  
Xingyuan Liu ◽  
Mingqian Liu ◽  
...  

Objective: To study the differences in clinical characteristics, risk factors, and complications across age-groups among the inpatients with the coronavirus disease 2019 (COVID-19).Methods: In this population-based retrospective study, we included all the positive hospitalized patients with COVID-19 at Wuhan City from December 29, 2019 to April 15, 2020, during the first pandemic wave. Multivariate logistic regression analyses were used to explore the risk factors for death from COVID-19. Canonical correlation analysis (CCA) was performed to study the associations between comorbidities and complications.Results: There are 36,358 patients in the final cohort, of whom 2,492 (6.85%) died. Greater age (odds ration [OR] = 1.061 [95% CI 1.057–1.065], p < 0.001), male gender (OR = 1.726 [95% CI 1.582–1.885], p < 0.001), alcohol consumption (OR = 1.558 [95% CI 1.355–1.786], p < 0.001), smoking (OR = 1.326 [95% CI 1.055–1.652], p = 0.014), hypertension (OR = 1.175 [95% CI 1.067–1.293], p = 0.001), diabetes (OR = 1.258 [95% CI 1.118–1.413], p < 0.001), cancer (OR = 1.86 [95% CI 1.507–2.279], p < 0.001), chronic kidney disease (CKD) (OR = 1.745 [95% CI 1.427–2.12], p < 0.001), and intracerebral hemorrhage (ICH) (OR = 1.96 [95% CI 1.323–2.846], p = 0.001) were independent risk factors for death from COVID-19. Patients aged 40–80 years make up the majority of the whole patients, and them had similar risk factors with the whole patients. For patients aged <40 years, only cancer (OR = 17.112 [95% CI 6.264–39.73], p < 0.001) and ICH (OR = 31.538 [95% CI 5.213–158.787], p < 0.001) were significantly associated with higher odds of death. For patients aged >80 years, only age (OR = 1.033 [95% CI 1.008–1.059], p = 0.01) and male gender (OR = 1.585 [95% CI 1.301–1.933], p < 0.001) were associated with higher odds of death. The incidence of most complications increases with age, but arrhythmias, gastrointestinal bleeding, and sepsis were more common in younger deceased patients with COVID-19, with only arrhythmia reaching statistical difference (p = 0.039). We found a relatively poor correlation between preexisting risk factors and complications.Conclusions: Coronavirus disease 2019 are disproportionally affected by age for its clinical manifestations, risk factors, complications, and outcomes. Prior complications have little effect on the incidence of extrapulmonary complications.


2014 ◽  
Vol 117 (5) ◽  
pp. e356-e357
Author(s):  
Kanako Matsumoto ◽  
Fumihiko Tsushima ◽  
Jinkyo Sakurai ◽  
Masaru Sato ◽  
Atsushi Uesugi ◽  
...  

Blood ◽  
2021 ◽  
Author(s):  
Giovanni Palladini ◽  
Giampaolo Merlini

The treatment of patients with systemic light chain (AL) amyloidosis is a challenge to hematologists. Despite its generally small size, the underlying clone causes a rapidly progressing, often devastating multiorgan dysfunction through the toxic light chains that form amyloid deposits. Clinical manifestations are deceitful and too often recognized at an irreversible stage. However, hematologists are in the unique position to diagnose AL amyloidosis at a pre-symptomatic stage checking biomarkers of amyloid organ involvement in patients with monoclonal gammopathies at higher risk to develop the disease. Adequate technology and expertise are needed for a prompt and correct diagnosis, particularly for ruling out non-AL amyloidoses that are now also treatable. Therapy should be carefully tailored based on severity of organ involvement and clonal characteristics, and early and continual monitoring of response is critical. Three recent randomized clinical trials moved AL amyloidosis to evidence-based era. Above all, the daratumumab-bortezomib combination is a new standard-of-care for newly diagnosed patients inducing rapid and deep responses that translate into high rates of organ response. The availability of new effective drugs allows to better personalize the therapy, reduce toxicity, and improve outcomes. Patients should be treated within clinical trials whenever possible.


2019 ◽  
Vol 48 (4) ◽  
pp. 030006051989586
Author(s):  
Xiang Guo ◽  
Lan Sun ◽  
Wei Lei ◽  
Shuang Li ◽  
Hui Guo

Objective This study aimed to summarize clinical manifestations, and physical examination, laboratory examination, and ultrasound results of children with testicular torsion <360°. Methods We performed a retrospective study of children who were diagnosed with testicular torsion <360° between October 2007 and October 2017. Results There were 11 (19.2%) patients with testicular torsion of 90°, 33 (58.0%) with 180°, and 13 (22.8%) with 270°. The median age of onset was 5.7 years (range, 1–14 years) and the median duration of symptoms was 4.2 days (range, 0.5–5 days). Ultrasound showed low blood flow in 46 (80.8%) patients. The testis was retained in 41 (72.0%) patients and resected in 16 (28.0%). The testes appeared necrotic at 2 to 3 days after onset for patients with 270° torsion, appeared necrotic at 3 to 4 days after onset for those with 180° torsion, and were not necrotic at 4 to 5 days after onset for those with 90° torsion. The duration of symptoms in children decreased as the torsion angle increased. Conclusion Children with testicular torsion <360° are relatively young, while the duration of symptoms is relatively long. A high postoperative testicular survival rate is one of the clinical features in this study.


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