scholarly journals Wilson’s Disease with Superimposed Autoimmune Features: A Case Report

2017 ◽  
Vol 5 (1-2) ◽  
pp. 72-74
Author(s):  
Md Wahiduzzaman Mazumder ◽  
ASM Bazlul Karim ◽  
Nazma Begum ◽  
Md Nurullah ◽  
Lina Florance Karmoker

The association of Wilson’s disease (WD) with autoimmune hepatitis (AIH) has rarely been documented. The difficulties in differential diagnosis of WD and AIH have seen specially in the paediatric population. Children with WD specially in younger ones, may have clinical features indistinguishable from autoimmune hepatitis. We report a case of 8-year old girl child who presented with jaundice, anaemia, leg oedema and arthritis. Ultimately the patient was diagnosed as a case of WD with AIH after meticulous investigation. Treatment was started with prednisolone, d-penicillamine and subsequently prednisolone was tapered with addition of azathioprine. D-penicillamine was stopped, with clinical and biochemical improvement and zinc was added to continue life-long. Follow up after 8 months of initial treatment she was non anaemic and non-icteric. Her leg oedema and arthritis also subsided. Liver biochemistry returned to normal level. It is important therefore to recommend strongly a thorough screening for AIH in patients with initial diagnosis of WD and also screening for WD for patients presenting with AIH. So a combined therapy may be of early benefit. Ibrahim Card Med J 2015; 5 (1&2): 72-74

1970 ◽  
Vol 18 (1) ◽  
pp. 37-43
Author(s):  
NC Saha ◽  
A Sultana ◽  
MAH Mollah ◽  
T Begum ◽  
AKMM Rahman ◽  
...  

Objectives: The objective of this study was to observe the outcome of patients treated with penicillamine. Design: Intervention type of study Setting: Department of Paediatrics, Dhaka Medical College Hospital Study period: January 2007 to December 2008. Study subjects: Sixteen diagnosed cases of Wilson's disease as per inclusion criteria. Intervention: D-penicillamine was started in a low dose, which was titrated gradually. The clinical and biochemical parameters were evaluated to look for the response to treatment. Results: A total of 16 cases were included. Among them 12 were male and 4 were female. The mean (± SD) of age of the patients was 10 (± 2.34) years. Consanguinity between parents was present in 44% (n=7). The hepatic and neurological variety of WD were 56 % (n=9) and 44% (n=7) respectively. The K-F ring was present in 75% (n=12/16) of WD cases. The excretion of 24 hrs urinary copper was steadily increased from discharge till second follow-up in response with increasing dose of penicillamine, thereafter the value was declining gradually till final follow-up at 1 year. Regarding outcome, 7 patients improved of which 4 were in hepatic and 3 in neurological group, 3 of hepatic WD expired and 2 developed neurological manifestations. One patients developed proteinuria while penicillamine treatment .About half of patients with WD were improved. Adequate cupriuresis occurred at three months. All the symptoms and biochemical markers WD improved gradually. No significant side effect was seen. Key words: Wilson's disease; penicillamine; urinary copper. DOI: 10.3329/jdmc.v18i1.6304 J Dhaka Med Coll. 2009; 18(1) : 37-43


Author(s):  
Harun Özmen ◽  
Bahar Aydınlı

Wilson's disease is an autosomal recessive inherited chronic disease that occurs as a result of the deposition of copper in organs and tissues with impaired biliary excretion. With this case report, we aimed to share our experience in cesarean anesthesia in a pregnant woman with Wilson disease who had irregular medical follow-up and underwent chelation therapy.


2019 ◽  
Vol 14 (0) ◽  
pp. 120-124
Author(s):  
N.Yu. Zavhorodnia ◽  
O.Yu. Lukianenko ◽  
N.O. Zhigir

2019 ◽  
Vol 9 (1) ◽  
pp. 5-6
Author(s):  
Ouidad Louachama ◽  
Aicha Bourrahouat ◽  
Ibtissam Khattou ◽  
Imane Ait Sab ◽  
Mohamed Sbihi

Wilson disease (WD) is a disorder of copper metabolism. Liver and brain disorders are the main presentations, hemolytic anemia in WD is a rare inaugural symptom. We report a case of a child who developed recurrent hemolytic anemia associated with liver failure in the second hemolysis episode as the first manifestation of WD. Wilson's disease is not exceptional in children with hemolytic anemia, but another differential diagnosis must be excluded.


2015 ◽  
Vol 15 (6) ◽  
Author(s):  
Naghi Dara ◽  
Farid Imanzadeh ◽  
Ali Akbar Sayyari ◽  
Peiman Nasri ◽  
Amir Hossein Hosseini

2021 ◽  
Vol 11 (1) ◽  
pp. 82-86
Author(s):  
Doyoung Kim ◽  
Yoon Ghil Park ◽  
Jung Hyun Park ◽  
Jinyoung Park

2004 ◽  
Vol 14 (1) ◽  
pp. 172-175 ◽  
Author(s):  
A. R. Di Gilio ◽  
G. Cormio ◽  
L. Resta ◽  
C. Carriero ◽  
V. Loizzi ◽  
...  

Smooth muscle tumors arising in the vulva are rare. Leiomyosarcoma is the most common variant of vulvar sarcoma, and very few cases have been reported during pregnancy. A 36-year-old woman presented with a progressively enlarging vulvar mass during pregnancy, diagnosed as a Bartholin's gland cyst. The lesion was resected at 38 weeks of gestation during cesarean section and diagnosis of myxoid leiomyosarcoma of the vulva was made. Six weeks later the patients were referred to our center and submitted to wide vulvar excision with groin lymph node dissection that revealed the presence of a small residual focus of leiomyosarcoma. At 30 months of follow-up the patient was well without any sign of recurrent disease. Leiomyosarcoma should be included in the differential diagnosis of vulvar masses; progressively enlarging vulvar lesion should be biopsied even during pregnancy. Leiomyosarcoma should be considered in the differential diagnosis of vulvar mass.


2018 ◽  
Vol 8 (1) ◽  
pp. 61-66
Author(s):  
Yoshinosuke Shimamura ◽  
Takuto Maeda ◽  
Yufu Gocho ◽  
Yayoi Ogawa ◽  
Kunihiko Tsuji ◽  
...  

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