Wilson disease is an inherited disorder that causes copper to accumulate in the body's tissues. It primarily affects the liver and brain. Symptoms can include liver disease, neurological or psychiatric issues. It is caused by a genetic defect that prevents the body from properly processing copper. Diagnosis involves tests of copper levels in blood and urine as well as occasionally a liver biopsy. Treatment aims to reduce copper intake and use chelating agents like penicillamine to remove excess copper from the body. With proper treatment, prognosis is good though neurological symptoms may persist in some cases.
What is bronchiolitis and its definition, the age group, signs and symptoms and clinical presentation The clinical practice guidelines, how to diagnosis, clinical criteria, what are the severity degrees and How to assess the severity, what are the investigations that may be needed, Is there any diagnostic test, what is the prognosis
What is the management,
What is bronchiolitis and its definition, the age group, signs and symptoms and clinical presentation The clinical practice guidelines, how to diagnosis, clinical criteria, what are the severity degrees and How to assess the severity, what are the investigations that may be needed, Is there any diagnostic test, what is the prognosis
What is the management,
Epideiology of Wilson’s Disease
Genetics of Wilson’s Disease
Pathophysiology of Wilson’s Disease
Clinical features
Investigations
Diagnostic criteria
Treatment
Prognosis
Wilsons disease- A brief medical study. martinshaji
this brief study describes all the basic aspects of Wilsons disease , including management. as this is a congenital abnormality associated with severe complications on the future proper diagnosis management , and lifestyle modifications , …..surgical options are also needed ,if necessary .
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Wilson's disease is an autosomal recessive disease causing progressive
copper overload. It is an initially hepatic affection which can evolve
towards a multi-systemic attack with an accumulation of copper in
the brain, the eye, the kidney, the heart. The diagnosis is carried out
on a bundle of clinical, biological and radiological findings. Treatments associate a diet low in copper, copper chelators or zinc salts.
Liver transplantation is the treatment for fulminant liver forms. This
rare genetic disease has a good prognosis if treatment is started early
and continued for life. It is therefore important to know the clinical manifestations of the disease and the diagnostic tests to evoke it
quickly in order to ensure regular clinical and biological monitoring
of patients. In this context, we report a familial case of Wilson's disease with its neurological, psychiatric, hepatic and ophthalmological
manifestations
Similar to pediatrics.Wilson disease.(dr.bakr) (20)
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
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2 Case Reports of Gastric Ultrasound
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Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
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Acute scrotum is a general term referring to an emergency condition affecting the contents or the wall of the scrotum.
There are a number of conditions that present acutely, predominantly with pain and/or swelling
A careful and detailed history and examination, and in some cases, investigations allow differentiation between these diagnoses. A prompt diagnosis is essential as the patient may require urgent surgical intervention
Testicular torsion refers to twisting of the spermatic cord, causing ischaemia of the testicle.
Testicular torsion results from inadequate fixation of the testis to the tunica vaginalis producing ischemia from reduced arterial inflow and venous outflow obstruction.
The prevalence of testicular torsion in adult patients hospitalized with acute scrotal pain is approximately 25 to 50 percent
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
2. -Wilson's disease is named after Samuel Alexander Kinnier
Wilson (1878–1937), the British neurologist who first described
the condition in 1912.
-Wilson disease is inherited autosomal recessive disease.
-A single abnormal copy of the gene is present in 1 in 100
people.
-The incidence is 1-4/100000
-The abnormal gene for welsion disease is located on the long
arm of chromosome -13-.
-Because of the abnormal gene there is defect in the biliary
copper excretion , and copper incorporation into ceruloplasmin.
-Wilson disease is a multisystem disorder it involves
liver,brain,eye,blood,kidney, and endocrine glandes.
3.
4. Pathogenesis
-Absence or malfunction of the gene results in
decreased biliary copper excretion and diffuse
accumulation of copper in the cytosol of hepatocyte.
-When liver cells become overloaded, copper is
redistributed to other tissues including the brain and
kidneys, to which it is toxic,primarly as a potent
inhibitor of enzymatic processes.
5. Pathology
-There is degenerative changes in the brain, liver and
kayser-Fleischer rings in the cornea.
-All grades of hepatic injury occure,there is progressive
parenchymal damage,fibrosis,and cirrhosis.
-Ultrastractural changes primarily involves
mitochondria.
6.
7.
8. CLINICAL MANIFESTATION
Wilson disease can be presents with any form of liver disease
after the age of -5-years,but mutation that completely knock out
gene function are associated with disease onset as early as ( 2-
3)years of age.
Wilsonian hepatic disease include:
-Asymptomatic hepatomegaly with or without splenomegaly.
-Sub acute or chronic hepatitis.
-Fulminant hepatic failure, three times more common in female
-Liver cirrhosis with portal hypertention,ascites
edema,varecial bleeding.
-Other effects of hepatic dysfunction like:
delayed puberty,amenorrhea,coagulation defect.
9. CLINICAL MANIFESTATION
Neurological manifestations:
After-20-years of age, neurologic symptoms predominate.
Neurologic disorders may present with:
-Intention tremor.
-Dysarthria.
-Dystonia.
-Lack of motor coordination.
-Deterioration in school performance.
-Behavior changes.
-Kayser-Fleischer ring always present in patient with neurological
manifestation.
- Wilson's disease is also associated with sunflower cataracts exhibited
by brown or green pigmentation of the anterior and posterior lens
capsule. Neither cause significant visual loss.
11. Renal manifestation:
Wilson disease may present with renal fanconi syndrome
which include:
-Aminoaciduria.
-Glycosuria.
-phosphaturia.
-Renal tubular acidosis.
-Progressive renal failure.
*Unusual manifestation include:
arthritis,cardiomyopathy,endocrinopathy.
12. DIAGNOSIS
Wilson disease should be in the differential diagnosis
of children or teenager presenting with:
1-Unexplained acute or chronic liver disease.
2-Neurologic symptoms of unknown cause.
3-Acute hemolysis.
4-Psychiatric illness.
5-Behaiveral changes.
6-Fanconi syndrome.
13. LABORATORY INVESTIGATIONS
1-Cerulopasmin:the best screening test is to measure
the serum ceruloplasmin level, most patients have
decreased cerulopasmin level,caution must be used in
interpreting ceruloplasmin level as they may be
elevated in acute inflammation and in states of
elevated estrogen such as pregnancy, estrogen
supplimentation,or oral contraceptive use.
2-Serum copper:
The serum copper level may be elevated in early
wilson disease.
14. 3-Urinary copper: urinary copper excretion is normally
<40ug/day,in patient with Wilson disease is increased
to >100ug/day,and often up to 1000 ug /day or more.
In equivocal cases urinary copper output in response
to chelation may be of diagnostic help.
15. 4-Liver biopsy:
Liver biopsy is for diagnosis, and for determining the
extent and severity of liver disease, normal copper
content of liver is( <10ug/gm dry weight).In Wilson
disease hepatic copper content exceeds 250ug/gm dry
weight.
16. 5-Genetic screening:
There are more than 250 mutations in the gene have been
identified, making diagnosis by DNA mutational analysis a
difficult task unless a proband mutation is known.
--Family members of patient with proven cases of Wilson
should be screened by:
-Kayser-Flescher ring.
-Serum ceruloplasmin level.
-Urinary copper excretion.
If these result are abnormal or equivocal, liver biopsy
should be carried out.
17. TREATMENT
Wilson disease is progressive and fatal ,but effective
treatment is available.
1-Copper intake:
Copper intake should be restricted to <1mg/day.
Foods such as liver,shellfish,nuts,and chocolate should
be avoided.
If copper content of drinking water exceed 0.1mg/L ,it
may be necessary to demineralize.
18. 2-copper chelating agent:
Administration of copper chelating agent leads to
rapid excretion of excess deposited copper.
Best chelating agent is oral D-penicillamine in dose of
20mg/kg/day.
Patient with neurological manifestation 10-50% of
them when initially receive D-penicillamin have a
worsening thir symptomes.
19. Side effects of D-penicillamin include:
1-Goodpature syndrome.
2-Systemic lupus erythematosis.
3-Polymyositis.
4-Aplastic anemia.
5-Nephrosis.
6-Deficiency of other elements like zinc.
7-Vitamin B6 deficiency.
20. For those patient who can not tolerate D-
penicillamin,other chelating agent can be use like:
1-Triethylene tetramine dihydrochloride in dose of
20mg/kglday.
2-Ammonium tetrathiomolybdate is a new agent
under investigation for patient with neurological
manifestation.side effectes
include:anemiam,lucopenia,thrombocytopenia.
21. 3-Zinc therapy:
Zinc has been used as adjuvant therapy,maintenance
therapy,or primary therapy.
Mecanism of action:it impair the gastrointestinal
absorbtion of copper.
Dose : 25mg/8hr in children above the age of five years.
4-Liver transplantation: is indicated for patient with
fulminant liver failure,decompensated cirrhosis,Liver
transplantation is curative with survival rate of 85-90%.
22. PROGNOSIS
Untreated patient with Wilson disease die of
hepatic,neurologic,renal or hematologic complication.
The prognosis for patients receiving penicilammin
variable depends on time of initiation and the
individual response to chelation.
In asymptomatic patient ,early initiation of chelation
therapy prevent expression of the disease.