Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange

2009 ◽  
Vol 24 (1) ◽  
pp. 25-27 ◽  
Author(s):  
Mehmet Hursitoglu ◽  
Osman Kara ◽  
Mehmet A. Cikrikcioglu ◽  
Tahsin Celepkulu ◽  
Sengul Aydin ◽  
...  
2021 ◽  
Vol 5 (2) ◽  
pp. 39-44
Author(s):  
Elham Zare ◽  
Zahra Mahbubi ◽  
Maryam Panahi

We report a short-statured, 39-year-old male presenting with recurrent kidney stones, history of refractory rickets, and bone deformity. He had been consuming multiple doses of calcium supplements and multiple courses of vitamin D over past 30 years beforeprior to reporting in our clinic without any significant laboratory or clinical improvement. The patient was diagnosed as having Fanconi’s syndrome attributable to Wilson’s disease. This patient highlighted that in case of resistant rickets, a high index of uncertainty must be invoked for Wilson’s disease. Appropriate timely recognition of this entity results in prompt ministrations and prevention of disability. We also presented and discussed reviews on Wilson’s disease from literature.


Kanzo ◽  
1995 ◽  
Vol 36 (10) ◽  
pp. 589-593 ◽  
Author(s):  
Toru ISHIKAWA ◽  
Naoki ISHIKAWA ◽  
Hironobu OOTA ◽  
Toshiaki YOSHIDA ◽  
Akira HONMA ◽  
...  

2018 ◽  
Vol 5 (3) ◽  
pp. 583
Author(s):  
S. Subash ◽  
D. Umesh ◽  
Prathyusha Mervala

Background: Therapeutic plasma exchange (TPE) is the separation and removal of plasma from whole blood with replacement by a crystalloid/colloid solution (typically albumin or plasma). The DGHS has established guidelines and recommendations for application of therapeutic apheresis in clinical practice. Guillain-Barré syndrome (GBS) is considered category I indications for TPE. This study was undertaken to establish the effectiveness and safety of therapeutic plasma exchange in GBS which is one of the common indication for TPE at our tertiary care teaching hospital.Methods: A retrospective study of 30 patients admitted to a tertiary care teaching hospital, from January 2014 to December 2016 with clinical signs of Guillain-Barre syndrome (GBS) and/or GBS variants were evaluated for performing TPE. A total of 104 procedures were performed for 30 patients. Replacement of crystalloids and plasma was used. Medical Research Council scale was used to assess the clinical improvement by measuring the grade of muscle power. Information was collected in a structured proforma and statistical analysis was performed using SPSS software (version 20). P value less than 0.05 was considered statistically significant.Results: During the study period, 104 procedures were performed on 30 patients on an average of three procedures per patient. The average age of the patients was 41.4±10.4 years. The mean period of illness at admission was 14.5±5.4 (range 4-32) days. In 23 out of 30 patients, more than three TPE procedures were done, out of which 21 patients clinically improved. The common complications during the procedure were chills (16%), hypotension (10%) and non-hemolytic febrile transfusion reaction (10%) and they were managed accordingly. Two (6.7%) patients who were not ambulatory at discharge had significantly (p <0.05) lower grade of power in lower limbs at admission and all patients recovered fully on follow up.Conclusions: GBS is one of the most commonly occurring clinical paralytic disorders. 76.7% of patients underwent three or more cycles of TPE with 70% had excellent clinical improvement which was comparable with various other studies. Based on results published by various other studies, therapeutic plasma exchange is a comparatively safe and effective procedure.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2093347 ◽  
Author(s):  
Philip Keith ◽  
Matthew Day ◽  
Carol Choe ◽  
Linda Perkins ◽  
Lou Moyer ◽  
...  

The COVID-19 pandemic has brought about an urgent need for effective treatment, while conserving vital resources such as intensive care unit beds and ventilators. Antivirals, convalescent plasma, and biologics have been used with mixed results. The profound “cytokine storm” induced endotheliopathy and microthrombotic disease in patients with COVID-19 may lead to acute respiratory distress syndrome, sepsis, and multi-organ failure. We present a case of SARS-COV2 pneumonia with septic shock and multi-organ failure that demonstrated significant clinical improvement after therapeutic plasma exchange. A 65-year-old female with multiple comorbidities presented with progressive dyspnea and dry cough. She was found to be COVID-19 positive with pneumonia, and developed progressive hypoxemia and shock requiring vasopressors, cardioversion, and non-invasive positive pressure ventilation. Given her worsening sepsis with multi-organ failure, she underwent therapeutic plasma exchange with rapid clinical improvement. Her case supports the theory that plasma exchange may help abate the “cytokine storm” induced endotheliopathy and microthrombosis associated with COVID-19. Further studies are needed to identify markers of this pathway and the potential role of plasma exchange in these critically ill patients.


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