scholarly journals Two cases of special POEMS syndrome without monoclonal protein expression: a case report and literature review

2021 ◽  
Vol 49 (4) ◽  
pp. 030006052199096
Author(s):  
Hua Ping Du ◽  
Ye Zhang ◽  
Lei Min Sun ◽  
Liang Luo

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is rare, with polyneuropathy and monoclonal plasma cell disorder generally considered as essential diagnostic symptoms. We report two cases of POEMS syndrome without monoclonal protein expression. The first case was a 72-year-old man who had experienced recurrent edema of the lower limbs for 2 years and abdominal distention for 2 months. The other case was a 62-year-old man with a 5-year history of recurrent numbness of the extremities and muscle weakness, which had become serious over the preceding 3 months. Both patients had various symptoms that matched those of POEMS syndrome, but neither had monoclonal protein expression. However, a diagnosis of POEMS syndrome was made in each case. Both patients were treated with lenalidomide and dexamethasone, after which their symptoms improved and laboratory test results normalized. The findings in these two cases suggest the possibility that POEMS syndrome may occur without monoclonal protein expression. The diagnostic criteria of POEMS syndrome may thus need further investigation.

2021 ◽  
pp. 1-5
Author(s):  
Walaa A. Kamel ◽  
Ismail Ibrahim Ismail ◽  
Jasem Yousef Al-Hashel

Objective: Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy that is often related to a previous infectious exposure. GBS emerged as a potentially serious complication of coronavirus disease 2019 (COVID-19) since its declaration as a global pandemic. We report the first case from Kuwait, to the best of our knowledge. Clinical Presentation: A 72-year-old male presented with 3 weeks history of acute progressive and ascending lower limbs weakness. He developed these symptoms 3 weeks after testing positive to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Electrophysiological studies showed acute demyelinating polyradiculoneuropathy and cerebrospinal fluid showed protein-cell dissociation. He was successfully treated with intravenous immunoglobulins (IVIGs). Conclusion: Neurologists should be aware of GBS as a potentially serious complication associated with CO­VID-19. Our patient had a favorable outcome with IVIG with no autonomic or respiratory affection.


2021 ◽  
Vol 84 (4) ◽  
pp. 680-681
Author(s):  
C Wu ◽  
S Wang ◽  
B Hu

Question: A 29-year-old Chinese male was admitted to our department with a history of right upper quadrant pain for two months, anorexia, weight loss about 5 kg and without fever. The pain radiated to the back and it got worst lately. He didn’t have a history of tuberculosis. The sclera was mild icteric. Laboratory test results showed total bilirubin level increased to 58 umol/L (normal level <28 umol/L) and tumor markers were normal. Chest X-ray was normal. Abdominal contrastenhanced CT showed a 25 x 30 mm cyst-solid mass in the head of pancreas (Figure1. A) and the lesion was mild enhancement in arterial phase. Pancreatic tumor was considered. Endoscopic ultrasonography confirmed the mass with uneven echo in the pancreatic head and the boundary of the mass was not clear (Figure1. B). The lesion may invade the portal vein. As the patient was young and the operation was very traumatic, the patient refused surgery unless the lesion was proved to be a tumor.


Author(s):  
Georgia Garani ◽  
Canan Eren Atay

A clinical data warehouse (CDW) can be an important tool for the purposes of analysis and critical decision making in the medical field. Such a data repository integrates heterogeneous health data, including clinical, treatment and diagnostic data and laboratory test results from a variety of sources. Accurate data need to be stored and processed in a CDW with adequate computation capabilities and thus, time plays a crucial factor. A slowly changing dimension (SCD) is a dimension that changes slowly over time, either gradually or intermittently. This article introduces a new SCD type, Type BTA, where both valid time and transaction time are supported for providing a complete history of the dimensional data. With Type BTA, the history of an object can be captured through the changes as reflected in the CDW. Consequently, for the first time, the full history of retroactive and post-active changes can be preserved in a CDW. Specifically, Type BTA is implemented for a Clinical Data Warehouse using real cancer data, for which the advantages of this methodology are demonstrated and advocated.


1978 ◽  
Author(s):  
N. G. Reed ◽  
J. M. Wilson

The engineering aspects of a new high efficiency 16,200-hp (12,080-MW) gas turbine using the Spey gas generator are presented. The history of the Spey and its evolution as an industrial gas generator are given. The design philosophy, aerodynamic features, and mechanical aspects of the new power turbine are discussed. Full-load laboratory test results of the new unit are reviewed. The first field installation, including the conversion of an existing natural gas pipeline unit from an Avon gas generator, is described. The first year of field operating experience is also summarized.


2020 ◽  
Author(s):  
Nuvia Mackenzie ◽  
Eva Lopez-Coronel ◽  
Alberto Dau ◽  
Dieb Maloof ◽  
Salvador Mattar ◽  
...  

Abstract Background: During the COVID-19 pandemic, different neurological manifestations have been published. However, few cases of Guillain-Barre Syndrome and COVID-19 have been reported. We describe a concomitant Guillain-Barre Syndrome and COVID-19 patient.Case presentation: a 39 years old woman was admitted in a teaching hospital in Barranquilla, Colombia with a history of progressive general weakness with lower limb dominance. A previous symptom as ageusia, anosmia and intense headache was reported. On admission, facial diplegia, quadriparesis with lower limbs predominance and Medical Research Council Scale 2/5 in lower limbs and 4/5 in upper limbs was reported. During clinical evolution, due to general areflexia, hypertensive emergency and progressive diaphragmatic weakness, the patient was admitted to intensive care unit. Cerebrospinal Serum Fluid revealed protein-cytologic dissociation and electromyography test were compatible with Guillain-Barre Syndrome. By symptoms before hospitalization, SARS-CoV2 diagnostic testing was performed with positive result in second test. Management to COVID-19 and Guillain Barre Syndrome was performed and patient was discharged after 20 days of hospitalization with clinical improvement.Conclusions: Few cases have been published reporting COVID-19 and Guillain-Barre Syndrome. We report the first confirmed case of COVID-19 with concomitant Guillain-Barre Syndrome in Colombia. In patients with Guillain-Barre Syndrome, several viral and bacterial pathogens have been found in case-control studies but there are do not clarity in what triggers the immune-mediated destruction of nerves. More studies are needed to determine possible association among COVID-19 exposure and Guillain-Barre Syndrome.


2017 ◽  
Vol 4 (1) ◽  
pp. 35
Author(s):  
Tosia Nisar ◽  
David G. Parr

POEMS syndrome is a rare multi-system condition that arises from an underlying plasma cell disorder. We report a case of a 69-year-old lady who presented with symptoms of exertional breathlessness and leg swelling, and a recent history of peripheral neuropathy, borderline diabetes mellitus and monoclonal gammopathy of undetermined significance (MGUS).  The development of worsening right heart failure, which remained refractory despite diuretic therapy, pulmonary hypertension, organomegaly, hypothyroidism, chronic kidney disease, right pleural effusion and ascites, did not lead to a definitive diagnosis until cutaneous abnormalities were recognised.  A presumptive diagnosis of POEMS was subsequently confirmed with the finding of raised serum levels of VEGF and bone marrow histopathology. Our case highlights the complex nature of this multi-system syndrome, the potential for delayed diagnosis and the importance of an integrated sub-specialty approach to management.


Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 563-566
Author(s):  
Ziao Wang ◽  
Huijie Jiang ◽  
Hao Jiang ◽  
Ruoshui Zheng ◽  
Ru Yi

Abstract2019 novel coronavirus disease (COVID-19, previously known as novel coronavirus pneumonia) was first discovered in December 2019 and spread widely in China and all over the world in 2020. The initial symptoms of most patients include fever, cough, and fatigue. Dyspnea may occur with the progress of the disease, and acute respiratory distress syndrome may occur in severe cases. The CT manifestations of this disease are mainly ground-glass opacity (GGO) in the lung, which may be accompanied by patchy consolidation, and fibrous changes may appear in the lung at the later stage of the disease. Combined with typical clinical and imaging findings and positive nucleic acid test results, the disease can be diagnosed. We report the first case of novel coronavirus disease (COVID-19) in Heilongjiang Province, China. The patient was seriously ill, who felt that he suffered from fever, fatigue, cough, and expectoration and sought medical treatment, with a history of contact with Wuhan. The leukocyte count was normal, and the lymphocyte count was decreased. CT imaging showed large GGO and partial patchy consolidation in both lungs. The patient recovered and was discharged after 26 days of treatment. This study is helpful for early diagnosis and timely clinical management by mastering the typical imaging of novel coronavirus disease (COVID-19).


Author(s):  
Joana Alves Vaz ◽  
Lilia Frada ◽  
Maria Manuela Soares ◽  
Alberto Mello e Silva

POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. We report a case of a man with Castleman disease and monoclonal gammopathy, with a history of chronic diarrhoea and asthenia. Gastrointestinal involvement in POEMS syndrome is not frequently referred to in the literature and its physiopathology is not fully understood. Diagnostic criteria were met during hospitalization but considering the patient’s overall health condition, therapeutic options were limited. Current treatment for POEMS syndrome depends on the management of the underlying plasma cell disorder. This report outlines the importance of a thorough review of systems and a physical examination to allow an attempted diagnosis and appropriate treatment.


2021 ◽  
Vol 62 (11) ◽  
pp. 1553-1559
Author(s):  
Jungyul Park ◽  
Hee-young Choi

Purpose: To demonstrate the efficacy of subcutaneous tocilizumab (TCZ-SC) treatment for recalcitrant thyroid eye disease (TED) refractory to intravenous methylprednisolone (MP) and oral methotrexate (MTX).Case summary: (Case 1) A 52-year-old man, smoker, with hyperthyroidism presented with a 3-months history of TED. The initial clinical activity score (CAS) was 5 (total score of 7). High-dose intravenous MP and oral MTX failed to improve the symptoms. He was treated with four doses of 162 mg TCZ-SC at an interval of 2 weeks. Pre-treatment laboratory test results were within normal limits. CAS was reduced to 2 weeks after the final injection, and there were no recurrences during the 6-month follow-up. (Case 2) A 37-year-old woman, non-smoker, with a 3-months history of hyperthyroidism presented with conjunctival injection and upper eyelid erythema. The initial CAS was 4, and thyroid-stimulating immunoglobulin level was raised (475% of normal). The symptoms did not improve with 7.5 g intravenous MP and oral MTX. The patient was treated with 162 mg TCZ-SC. Pre-treatment laboratory test results were within normal limits. CAS was reduced to 1 after the final injection, and there were no recurrences during the 6-months follow-up.Conclusions: TCZ-SC improves symptoms of corticosteroid-resistant TED, and may be a reasonable option in recalcitrant TED cases. However, further studies are required to justify the use of TCZ-SC for TED.


2021 ◽  

Isolated superior mesenteric artery dissection (ISMAD) is a relatively rare disease and often lacks specificity in its clinical presentation, diagnosing it in emergency departments quickly and accurately is a challenge. The study was conducted to understand the basic characteristics of ISMAD including age of onset, risk factors, gender difference and whether D-dimer can be used as a biomarker for its detection. We retrospectively analyzed patients with ISMAD admitted to the emergency department of Xiangya Hospital of Central South University from September 1, 2017 to September 30, 2020. The data included the patient’s basic information and the first laboratory test results after admission, including routine blood, liver function, renal function and coagulation function tests. Statistical analysis of results was done using GraphPad Prism 5. There were a total of 17 (15 male and 2 female) patients with a mean age of 52.53 ± 7.11 years diagnosed with ISMAD. Out of these, 7 (41%) patients had history of hypertension, 7 (41%) had history of smoking and/or alcohol intake, and almost all patients experienced significant abdominal pain and fullness. Four patients (24%) were initially misdiagnosed. The laboratory test results of renal, liver function tests and myocardial enzymology were in their normal ranges. In coagulation results, the positive rates of fibrinogen degradation products (FDP) and D-dimer were 29% and 35%, respectively. We compared these ISMAD results with our previous results for acute aortic dissection (AAD). D-dimer and FDP levels were lower in the ISMAD than in the AAD patients (P = 0.0004, P = 0.0325, respectively), and negative rates of D-dimer and FDP in ISMAD (65%, 71%) were higher than in AAD patients (14%, 24%) (P < 0.0001, P = 0.0003, respectively). In our study, ISMAD mostly occurred in middle-aged male patients with known hypertension or active smoking status. Misdiagnosis was common (24% of cases). Since D-dimer and FDP levels proved to be of limited diagnostic value, an abdominal Computed Tomography (CT) scan should be conducted in patients with unclear abdominal pain at an early stage of their diagnostic workup.


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