scholarly journals Therapeutic Response of Wilson’s disease to D-Penicillamine in Paediatric Population: A one year follow-up study

1970 ◽  
Vol 18 (1) ◽  
pp. 37-43
Author(s):  
NC Saha ◽  
A Sultana ◽  
MAH Mollah ◽  
T Begum ◽  
AKMM Rahman ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Paediatric Population ◽  
Wilson's Disease ◽  
Response To Treatment ◽  
College Hospital ◽  
Medical College ◽  
Urinary Copper ◽  
F Ring ◽  
One Year

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

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
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Autoimmune Hepatitis ◽  
Wilson’S Disease ◽  
Combined Therapy ◽  
Paediatric Population ◽  
Wilson's Disease ◽  
Girl Child ◽  
Early Benefit

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

scholarly journals Preliminary Report of Encephalitis Surveillance Study in Rajshahi Medical College Hospital

1970 ◽  
Vol 17 (2) ◽  
pp. 75-79
Author(s):  
Sultana M Hussain ◽  
MJ Hossain ◽  
ARMS Ekram ◽  
ES Gurley ◽  
MM Alam ◽  
...  
Keyword(s):  
Japanese Encephalitis ◽  
Nipah Virus ◽  
Medical College Hospital ◽  
Serum Samples ◽  
Recent Infection ◽  
College Hospital ◽  
Medical College ◽  
Control And Prevention ◽  
One Year

More than 100 different viruses, bacteria, toxins and drugs can cause acute encephalitis. A prospective, hospital-based study to define the causes of encephalitis in Bangladesh began in June 2003. At Rajshahi Medical College Hospital in one year 105 out of 391 suspected encephalitis patients were enrolled. The specimens were collected from these patients and were tested for a variety of pathogens at twelve laboratories at the Centers for Disease Control and Prevention (CDC) in Atlanta and Ft. Collins, USA. Among the first 105 patients enrolled, the median age of the patient was 18 years; 55% were male. Twenty-four percent (n-25) died in hospital or before completing the follow-up. Thirteen out of 105 (12.38%) patients had Japanese encephalitis; three of the encephalitis patients had a positive test result of having recent infection with dengue virus and one had encephalitis due to alpha virus, another patient had encephalitis due to echovirus. Three (2.8%) were polymerase chain reaction (PCR) or culture positive for Streptococcus pneumoniae, and 4 for Mycoplasma pneumoniae. No Nipah virus infection was identified among the first 105 serum samples tested. These data suggest that Japanese encephalitis virus is an emerging cause of encephalitis in northern part of Bangladesh. doi: 10.3329/taj.v17i2.3449 TAJ 2004; 17(2): 75-79  

scholarly journals Kayser-Fleischer Ring evaluation in Wilson's Disease in a tertiary eye care centre of Nepal

2015 ◽  
Vol 4 (3) ◽  
pp. 17-20
Author(s):  
A K Sharma ◽  
S Sitaula ◽  
M Thapa ◽  
G S Shrestha ◽  
B P Gajurel ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Neuropsychiatric Symptoms ◽  
Positive Family History ◽  
Measurement Data ◽  
Copper Metabolism ◽  
Cost Effective ◽  
Wilson's Disease ◽  
Medical College ◽  
The Status ◽  
F Ring

Wilson’s disease is a hereditary disorder of copper metabolism which is characterized by neuropsychiatric and hepatic manifestations as well as appearance of Kayser-Fleischer ring. This is a retrospective review of the records of the patients of Wilson’s disease who attended Neuro-ophthalmic clinic for the identification of Kayser-Fleischer (K-F) ring from January2010 to June 2012. Detailed eye examination included visual acuity assessment, slit lamp biomicroscopy and intra-ocular pressure measurement. Data regarding clinical features, laboratory investigations and the status of K-F ring was recorded. Seven cases of Wilson’s disease with age range of 9-15 years were included in the study. Among them four (57.1%) had neuropsychiatric symptoms, two (28.5%) had hepatic disease and one (14.3%) was asymptomatic, diagnosed by positive family history and laboratory tests. Among four subjects having K-F ring, three (75%) subjects had neuropsychiatric symptoms and one subject had hepato-billiary disease. Besides K-F rings, other ophthalmic findings were sunflower cataract (14.3%) and vernal keratoconjuctivitis (14.3%). The identification of K-F ring is a simple and cost effective screening test for the diagnosis of Wilson’s disease. K-F is present in majority of the patients with neurological manifestations. DOI: http://dx.doi.org/10.3126/jcmc.v4i3.11934Journal of Chitwan Medical College 2014; 4(3): 17-20 

scholarly journals Value of Kayser-Fleischer ring as a diagnostic tool for Wilson’s disease in children

2021 ◽  
Vol 3 (Number 1) ◽  
pp. 20-23
Author(s):  
Sadika Kadir ◽  
S M Bazlul Karim ◽  
Mohammed Ashraful Haque ◽  
Rafiqul Islam ◽  
Asif Imran ◽  
...  
Keyword(s):  
Liver Disease ◽  
Diagnostic Tool ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Female Ratio ◽  
Copper Excretion ◽  
Group I ◽  
Urinary Copper ◽  
F Ring ◽  
Urinary Copper Excretion

The Kayser-Fleischer(K-F) ring is the hallmark of Wilson’s disease (WD). In most adults or older children, the diagnosis of Wilson’s disease may be made easily when K-Frings and low ceruloplasmin levels are present. In this study presence of K-F ring has been evaluated among children with liver disease in Bangladesh to improve the management of Chronic liver disease due to WD and reduce complications. This cross-sectional study was carried out at the Department of Paediatric Gastroenterology and Nutrition, BSMMU, Dhaka on 60 children presented with liver disease. Thirty children over three years of age considered as cases (Group-I) and thirty children with non- Wilsonian liver disease as control (Group-II). Slit lamp examination for K-F ring and twenty-four hour urinary copper excretion after giving one gram d-penicillamine 12-hour apart were done in each patient. The efficacy of K-F ring was studied. Mean age of WD patients was 8.9± 2.78 years, with a male female ratio of 1.3: 1. There was significant low level of serum ceruloplasmin in 93.33% of cases (p<.001). After penicillamine challenge, 24-hour urinary copper excretion was found significantly higher in patients with WD (median 3626.5±1698 μg/24h, range 1262- 195000) than non-Wilsonian liver disease (median 450±278.09 μg/24-h, range 47- 2062 μg/24h), (p<.001). K-F ring was found in 15 (50%) patients, absent in all patients of non-Wilsonian liver disease group and the difference was statistically significant (p<.001). Evaluation of Kayser-Fleischer ring is still a very essential diagnostic tool and is a non-invasive, affordable way to assist in the diagnosis of a potentially fatal disease.

A COMPARATIVE STUDY OF TB MENINGITIS IN HIV+ve AND HIV-ve PATIENTS

2021 ◽  
pp. 51-53
Author(s):  
Saiyed Rana ◽  
Manotosh Sutradhar ◽  
Meghdeepa Sengupta ◽  
Ritabrata Mitra ◽  
Sisir Chakraborty ◽  
...  
Keyword(s):  
Prospective Study ◽  
Blood Sugar Level ◽  
Clinical Presentation ◽  
Age Distribution ◽  
Response To Treatment ◽  
Advanced Stage ◽  
College Hospital ◽  
Medical College ◽  
The Difference

Aim:To study the difference in clinical presentation, baseline laboratory parameters, radiological parameters, CSF picture, response to treatment, in-hospital outcome, nal outcome in follow up & any parameters signifying poor outcome in advance in patients of TBM both in HIV+ve& HIVve group. Material and methods:This comparative non-randomised prospective study was conducted from 16th June,2012 to 15th June,2013 in Medical College Hospital. Total 53 patients were referred where 23 patients had TB Meningitis with HIVand 30 patients had TB Meningitis without HIV. Result: In our study we have found no statistically signicant differences in age distribution, and distribution of symptoms at presentation. The classical symptoms like fever, headache, and vomiting, altered sensorium all are present in both groups and the differences are not statistically signicant. Conclusion:HIV+ve patients show relatively less intense reduction of CSF sugar level in relation to blood sugar level estimated simultaneously.Two factors with high mortality are advanced stage of TB meningitis at presentation, low CD4 count.

scholarly journals Dysphagia in Wilson’s Disease: A Case Report of One-Year Follow-Up

2021 ◽  
Vol 11 (1) ◽  
pp. 82-86
Author(s):  
Doyoung Kim ◽  
Yoon Ghil Park ◽  
Jung Hyun Park ◽  
Jinyoung Park
Keyword(s):  
Case Report ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
One Year

Outcome of Percutaneous Nephrostomy in Children With Ureteropelvic Junction Obstruction

2020 ◽  
Vol 20 (2) ◽  
pp. 61-64
Author(s):  
Mohammad Mahfuzur Rahman Chowdhury ◽  
Rifat Zaman ◽  
Md Amanur Rasul ◽  
Akm Shahadat Hossain ◽  
Shafiqul Alam Chowdhury ◽  
...  
Keyword(s):  
Renal Function ◽  
Low Dose ◽  
Ureteropelvic Junction Obstruction ◽  
Percutaneous Nephrostomy ◽  
Ureteropelvic Junction ◽  
College Hospital ◽  
Medical College ◽  
Split Renal Function

Introduction and objectives: Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis. Management protocols are based on the presence of symptoms and when the patient is asymptomatic the function of the affected kidney determines the line of treatment. Percutaneous nephrostomy (PCN) became a widely accepted procedure in children in the 1990s. The aim of the study was to evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with UPJO and split renal function (SRF) of less than 10% in the affected kidney, because the management of such cases is still under debate. Methods:This prospective clinical trial was carried out at Dhaka Medical College Hospital from January 2014 to December 2016. Eighteen consecutive patients who underwent PCN for the treatment of unilateral UPJO were evaluated prospectively. In these children, ultrasonography was used for puncture and catheter insertion. Local anesthesia with sedation or general anesthesia was used for puncture. Pig tail catheters were employed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose cephalosporin prophylaxis. Repeat renography was done after 4 weeks. When there was no significant improvement in split renal function (10% or greater) and PCN drainage (greater than 200 ml per day) then nephrectomies were performed otherwise pyeloplasties were performed. The patients were followed up after pyeloplasty with renograms at 3 months and 6 months post operatively. Results: All the patients had severe hydronephrosis during diagnosis and 14 patients with unilateral UPJO were improved after PCN drainage and underwent pyeloplasty. The rest four patients that did not show improvement in the SRF and total volume of urine output underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF was increased to 26.4% ±8.6% (mean± SD) after four weeks and pyeloplasty was performed. At three and six months follow-up, SRF value was 29.2% ±8.5% and 30.8.2% ±8.8% respectively. Conclusion: Before planning of nephrectomy in poorly functioning kidneys (SRF < 10%) due to congenital UPJO, PCN drainage should be done to asses improvement of renal function. Bangladesh Journal of Urology, Vol. 20, No. 2, July 2017 p.61-64

Treatment of Pilonidal Sinus by Limberg Flap-Only Hope of Cure (Study of 50 Cases)

2020 ◽  
Vol 22 (2) ◽  
pp. 89-94
Author(s):  
Nasir Uddin Mahmud ◽  
ABM Khurshid Alam ◽  
Mohammad Altaf Hossain Chawdhury ◽  
Mohammad Abul Khair
Keyword(s):  
Pilonidal Sinus ◽  
Skin Barrier ◽  
Limberg Flap ◽  
College Hospital ◽  
Medical College ◽  
Flap Technique ◽  
Unpleasant Smell ◽  
Lower Morbidity ◽  
Computer Operator

Background : Pilonidal sinus was first described by Dr. A.W. Anderson in 1847 and is often seen in the intergluteal region. It is a disease of mainly younger male and rarely affects after 40. Main causes for the formation of this sinus are hirsutism, sweating in the area, repeated maceration due to trauma, leading to breakage of the skin barrier, attracting hair inside which initiates a foreign body reaction leading to infection with abscess or sinus formation. Most common embarrassing situation is discharging sinus. The diagnosis is mainly on clinical ground. Though there are a lot of modalities of treatment but recurrence is still a challenge. We have adopted Limberg flap technique since 2012 with a least recurrent chance. Objectives : To prove that this procedure will be end up with lower morbidity & least chance of recurrence Methods : This prospective study was done jointly by the Department of Surgery of Central Medical College and Comilla Medical College Hospital. In this series 50 patients were selected from July 2012 to June 2017. Results : Out of 50 patients operated by Limberg flap technique 42(84%) were males and 8(16%) were females. Mean age was 27.3 years (range 18–37years). Of them,12(24%) were driver, 6(12%) police, 6(12%) teacher, 6(12%) student, 5(10%) worker, 4(8%) millitary army, 4(8%) housewife, 4 (8%)service holder, 2(4%) computer operator, 1(2%) doctor and 1(2%) were barber. In our study most common mode of presentation were chronic discharging sinuses 15(30%), beside this 12(24%) had multiple sinuses, 10(20%) had multiple pits,7(14%) had acute abscess and 6(12%) had unpleasant smell. Associated hirsutism were present in 30(60%) and absent in 20(40%) patients. One (2%) male patient had wound gap & discharge and six(12%) patients had flap edema. We have found no recurrence in follow up period. Conclusion : Pilonidal sinus is a notoriously recurrent disease, so treatment by this flap technique is the only hope of cure with lower morbidity. Journal of Surgical Sciences (2018) Vol. 22 (2) : 89-94

scholarly journals A206 EVOLUTION OF PEDIATRIC AUTOIMMUNE CHOLANGITIS AND PRIMARY SCLEROSING CHOLANGITIS INTO ADULTHOOD

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 234-236
Author(s):  
P Willems ◽  
J Hercun ◽  
C Vincent ◽  
F Alvarez
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Response To Treatment ◽  
Similar Proportion ◽  
Autoimmune Cholangitis ◽  
Significant Difference ◽  
One Year ◽  
Liver Tests

Abstract Background The natural history of primary sclerosing cholangitis (PSC) in children seems to differ from PSC in adults. However, studies on this matter have been limited by short follow-up periods and inconsistent classification of patients with autoimmune cholangitis (AIC) (or overlap syndrome). Consequently, it remains unclear if long-term outcomes are affected by the clinical phenotype. Aims The aims of this is study are to describe the long-term evolution of PSC and AIC in a pediatric cohort with extension of follow-up into adulthood and to evaluate the influence of phenotype on clinical outcomes. Methods This is a retrospective study of patients with AIC or PSC followed at CHU-Sainte-Justine, a pediatric referral center in Montreal. All charts between January 1998 and December 2019 were reviewed. Patients were classified as either AIC (duct disease on cholangiography with histological features of autoimmune hepatitis) or PSC (large or small duct disease on cholangiography and/or histology). Extension of follow-up after the age of 18 was done for patients followed at the Centre hospitalier de l’Université de Montréal. Clinical features at diagnosis, response to treatment at one year and liver-related outcomes were compared. Results 40 patients (27 PSC and 13 AIC) were followed for a median time of 71 months (range 2 to 347), with 52.5% followed into adulthood. 70% (28/40) had associated inflammatory bowel disease (IBD) (78% PSC vs 54% AIC; p=0.15). A similar proportion of patients had biopsy-proven significant fibrosis at diagnosis (45% PSC vs 67% AIC; p=0.23). Baseline liver tests were similar in both groups. At diagnosis, all patients were treated with ursodeoxycholic acid. Significantly more patients with AIC (77% AIC vs 30 % PSC; p=0.005) were initially treated with immunosuppressive drugs, without a significant difference in the use of Anti-TNF agents (0% AIC vs 15% PSC; p= 0.12). At one year, 55% (15/27) of patients in the PSC group had normal liver tests versus only 15% (2/13) in the AIC group (p=0.02). During follow-up, more liver-related events (cholangitis, liver transplant and cirrhosis) were reported in the AIC group (HR=3.7 (95% CI: 1.4–10), p=0.01). Abnormal liver tests at one year were a strong predictor of liver-related events during follow-up (HR=8.9(95% CI: 1.2–67.4), p=0.03), while having IBD was not (HR=0.48 (95% CI: 0.15–1.5), p=0.22). 5 patients required liver transplantation with no difference between both groups (8% CAI vs 15% CSP; p=0.53). Conclusions Pediatric patients with AIC and PSC show, at onset, similar stage of liver disease with comparable clinical and biochemical characteristics. However, patients with AIC receive more often immunosuppressive therapy and treatment response is less frequent. AIC is associated with more liver-related events and abnormal liver tests at one year are predictor of bad outcomes. Funding Agencies None

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