1. Thalassemia is a group of inherited blood disorders caused by a defect in the synthesis of the globin chains that make up hemoglobin. There are two main types - alpha and beta thalassemia.
2. Symptoms range from mild anemia to life-threatening conditions depending on the type and severity. Diagnosis involves blood tests and family screening. Treatment involves lifelong blood transfusions and iron chelation therapy for severe cases.
3. Complications include iron overload, organ damage, bone changes and endocrine abnormalities which require monitoring and additional management. While transplantation offers a cure, compliance with treatment and managing complications long-term is important to maximize outcomes for patients.
Get here,
1. WHAT IS THALASSEMIA?
2. Molecular Basis of Thalassemia.
3. Types of Thalassemia.
4. - Thalassemia.
5.Types of - Thalassemia.
6. 휷- Thalassemia.
7. Types of 휷- Thalassemia.
8. Thalassemia Syndrome.
9.Treatment
• Thalassemia
• Hemoglobinopathies
• Incidence of thalassemia in Thailand
• Mode of inheritance
• How to name thalassemia ?
• Common types of thalassemia
• Alpha Thalassemia
• Symbolism Alpha Thalassemia
• Classification & Terminology Alpha Thalassemia
• Types of a-thalassemia
• Compound heterozygotes
• Comparison of α Thalassemias
• Beta thalassemias (β thalassemias)
• Types of β Thalassemia
• β Thalassemia Symptoms Include
• Diagnosis of Beta Thalassemia
• Prevention
• What Is The Treatment For Thalassemia
A presentation made about Sickle cell disease by Yara Mostafa, Yasser Osama, Yaser Mostafa ,Ain shams university, Medicine faculty, first year students.
Get here,
1. WHAT IS THALASSEMIA?
2. Molecular Basis of Thalassemia.
3. Types of Thalassemia.
4. - Thalassemia.
5.Types of - Thalassemia.
6. 휷- Thalassemia.
7. Types of 휷- Thalassemia.
8. Thalassemia Syndrome.
9.Treatment
• Thalassemia
• Hemoglobinopathies
• Incidence of thalassemia in Thailand
• Mode of inheritance
• How to name thalassemia ?
• Common types of thalassemia
• Alpha Thalassemia
• Symbolism Alpha Thalassemia
• Classification & Terminology Alpha Thalassemia
• Types of a-thalassemia
• Compound heterozygotes
• Comparison of α Thalassemias
• Beta thalassemias (β thalassemias)
• Types of β Thalassemia
• β Thalassemia Symptoms Include
• Diagnosis of Beta Thalassemia
• Prevention
• What Is The Treatment For Thalassemia
A presentation made about Sickle cell disease by Yara Mostafa, Yasser Osama, Yaser Mostafa ,Ain shams university, Medicine faculty, first year students.
Chemistry Investigatory Project Class 12 - Green Chemistry - Bio Diesel And B...Dhananjay Dhiman
Chemistry investigatory project for class 12 CBSE on the topic Green chemistry - bio diesel and bio petrol. It includes all the necessary formats and the content is relevant for the CBSE practical examination.
Contents :
General features of genes and chromosomes
General features of genetics
General features of chromosomes
Barr body
Genes
Aneuploidy
Chromosomes
Cytogenetics and DNA recombinant technology
General features of cytogenetic studies
Polymerase chain reaction
Restriction fragment length polymorphism
DNA fingerprinting
Fluorescence and luminence
Karyotyping
Blotting
Hybridoma technology
Genetic studies
Chromosomal studies
Transgenic animals
Gene incorporation
Recombinant technology
Gene therapy
Mode of inheritance of diseases
Inheritance of diseases
Features of inheritance
Mitochondrial inheritance
Genetic disorders
General features of genetic disorders
Rearrangement of transcription factors
Down’s syndrome
Trisomy 13
Trisomy 18
Trisomy 22
Turner syndrome
Klinefelter syndrome
Noonan syndrome
Disorders of DNA repair mechanism
For more details, visit www.medpgnotes.com
You can send your queries to medpgnotes@gmail.com
Thalassemia Unveiled: Insights into Diagnosis, Treatment, and Care.pptxNoorulainMehmood1
Thalassemia, a group of inherited blood disorders, presents a complex interplay of genetic mutations and clinical manifestations. This presentation delves into the intricacies of thalassemia, exploring its genetic underpinnings, clinical spectrum, diagnostic modalities, and therapeutic approaches. Through comprehensive analysis and case studies, attendees will gain a deeper understanding of thalassemia's impact on patients' lives and the latest advancements in management strategies.
Keywords:
Thalassemia
Genetic Disorders
Hemoglobinopathies
Blood Disorders
Anemia
Genetic Mutations
Clinical Spectrum
Diagnosis
Treatment Modalities
Transfusion Therapy
Iron Chelation Therapy
Genetic Counseling
Patient Care
Hematological Disorders
Research Advancements
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
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
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
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
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
5. Thalassaemias are group of the
haemoglobin
disordersin which the
production of normal haemoglobin is
partly or completely suppressed as a result
of the defective synthesis of one or more
globin chains
5
13. • β-Thalassaemia major is an inherited blood
disorder presenting with anaemia at 4 – 6
months of age.
• The carrier rates of α-thalassaemia and
Haemoglobin E (HbE) are 1.8-7.5% and 5-46%
respectively
• Interaction between a β-thalassaemia carrier
with a HbE carrier may result in the birth of a
patient with HbE/β-thalassaemia or
thalassaemia intermedia with variable clinical
severity
13
14. 2. Types
14
THALASSEMIA
α
- Silent Carrier
- Trait (Minor)
- Hemoglobin H Disease
(Intermediate)
- Major (Hemoglobin Bart’s)
- Hemoglobin Constant Spring
β
- Trait (Minor)
- Intermedia
- Major (Cooley anemia)
15. Alpha Thalassemia
• Deficient/absent alpha subunits
▫ Excess beta subunits
▫ Excess gamma subunits newborns
• Encoding genes on chromosome 16
• Each cell has 4 copies of the alpha globin gene
▫ Each gene responsible for ¼ production of alpha
globin
16. • Possible mutation states:
▫ Loss of ONE gene silent carrier
▫ Loss of TWO genes thalassemia minor (trait)
▫ Loss of THREE genes Hemoglobin H disease
Accumulation of beta chains
Association of beta chains in groups of 4 Hemoglobin H
▫ Hb constant spring similar to HbH but no microcytosis
▫ Loss of FOUR genes Hemoglobin Barts
NO alpha chains produced ∴ only gamma chains present
Association of 4 gamma chains Hemoglobin Barts
GENE
CHROM
OSOME
CELL
DNA
PROTEIN
18. Beta Thalassemia
• Encoding genes on chromosome 11
• Each cell contains 2 copies of beta globin gene
• Suppression of gene more likely than deletion
▫ β0 refers to the complete absence of production of β -
globin on the affected allele
▫ β+ refers to alleles with some residual production of ‚
β -globin (around 10%)
▫ β++ ;the reduction in β -globin production is very mild
19. β-Thalassaemia
An absence or deficiency of β-chain synthesis of adult HbA
β Chain synthesis
Hb-A α2β2
γ and δ chain
20. • Loss of ONE gene thalassemia minor (trait)
▫ ↑HbA2
▫ HbA normal
▫ HbF normal
• Loss of BOTH genes
▫ Thalassemia intermedia β+ β+ or β0 β+
▫ Thalassemia major β0β0
20
21. HbE α2βE
2
• Haemoglobin E disorder is the most common
structural variant resembling thalassemia
disorders
• HbE results from a mutation (GA) at codon 26 of
the ‚ β -globin gene
• HbE/ β thalassemia
21
22. 3. Diagnosis
• Clinical features
▫ History
▫ Physical examinations
• Lab investigations
• Screening family members
22
23. Clinical Outcomes of α Thalassemia
• Silent carriers
• Asymptomatic
• Alpha Thalassemia minor (trait)
• No anemia
• Microcytosis
• Alpha Thalassemia intermedia (Hemoglobin H)
• Anemia and microcytosis
• Bone deformities
• Splenomegaly
24. • Hemoglobin Constant Spring
• Similar to HbH but no microcytosis
• Anemia
• Growth delay
• Alpha Thalassemia major
• Hb Bart’s
• Fatal hydrops fetalis
30. ↑Haemolysis ↑demands of phagocytic
function hyperplasia of phagocytes
Hepatosplenomegaly
To compensate anaemia extramedullary
haemopoiesis in liver, spleen & brain
Organomegaly
34. 34
The red blood cells here are normal, happy RBC's. They have a zone of central
pallor about 1/3 the size of the RBC. The RBC's demonstrate minimal variation in
size (anisocytosis) and shape (poikilocytosis). A few small fuzzy blue platelets are
seen. In the center of the field are a band neutrophil on the left and
asegmented neutrophil on the right.
35. 35
The RBC's here appear smaller than normal and have an increased zone of central
pallor. This is indicative of a hypochromic (less hemoglobin in each RBC) and
microcytic (smaller size of each RBC) anemia. There is also increased anisocytosis
(variation in RBC size) and poikilocytosis (variation in RBC shape).
38. 5. Complications and management
• Complications of disease
• Complications of treatment
38
39. Management
Baseline investigations
• Full blood count, Peripheral blood film
• Hb analysis by electrophoresis / High Performance Liquid
Chromatography (HPLC)
• Serum ferritin.
• Red cell phenotyping (ideal) before first transfusion.
• DNA analysis (ideal)
• Liver function test.
• Infection screen: HIV, Hepatitis B & C, VDRL screen (before
first transfusion).
• HLA typing (for all patient with unaffected siblings)
39
41. β Thalassemia major
When to start blood transfusion?
• After completing blood investigations for
confirmation of diagnosis.
• Hb < 7g/dl on 2 occasions > 2 weeks apart (in
absence other factors e.g. infection).
• Hb > 7g/dl in β+-thalassaemia major/severe forms
of HbE-β-thalassaemia if impaired growth, severe
bone changes, enlarging liver and spleen.
41
42. Transfusion targets?
• Maintain pre transfusion Hb level at 9 -10 g/dl.
• Keep mean post-transfusion Hb at 13.5-15.5g/dl.
• Keep mean Hb 12 - 12.5 g/dl.
• The above targets allow for normal physical activity and
growth, abolishes chronic hypoxaemia, reduce
compensatory marrow hyperplasia which causes
irreversible facial bone changes and para-spinal masses.
42
44. Transfusion interval?
• Usually 4 weekly interval (usual rate of Hb
decline is at 1g/dl/week).
• Interval varies from individual patients (range: 2
- 6 weekly).
Transfusion volume?
• Volume: 15 - 20mls/kg (maximum) packed red
cells (PRBC).
44
45. • In the presence of cardiac failure or Hb < 5g/dl,
use lower volume PRBC (< 5ml/kg) at slow
infusion rate over > 4 hours with IV Frusemide 1
mg/kg (20 mg maximum dose).
• It is recommended for patients to use
leucodepleted (pre-storage, post storage or
bedside leucocyte filters) PRBC < 2 weeks old.
• Leucodepletion would minimize non-haemolytic
febrile reactions and alloimmunization by
removing white cells contaminating PRBC.
45
46. Example
• Beta thalassemia major
• Wt 16 kg
• Hb 4
Calculations:
Total PC: (12-4)(16)(3.5) = 448 cc
1st tx 5cc/kg = (5)(16) =80 cc
2nd tx 10cc/kg= (10)(16) = 160 cc
Balance 384- 80 -160=208 cc
Max possible tx 20cc/kg = 320 cc
46
47. α Thalassemia (HbH disease)
• Transfuse only if Hb persistently < 7g/dl and/or
symptomatic.
47
49. DFO: Desferrioxamine (Desferal®)
When to start? • Usually when the child is > 2 - 3 years old.
• When serum ferritin reaches 1000 μg/L.
• Usually after 10 – 20 blood transfusions.
Dosage, route • Average daily dose is 20 – 40mg/kg/day.
• By subcutaneous (s.c.) continuous infusion using a
portable pump over 8-10 hours daily, 5 - 7 nights a week.
Complications • Local skin reaction
• Yersinia infection
• Ocular/auditory toxicity
• Skeletal lesion i.e. vertebral growth retardation
49
50. DFP DFX
• An alternative if iron chelation is
ineffective or inadequate despite
optimal Desferal® use, or if
Desferal® use
is contraindicated.
• Deferiprone is given 75 – 100
mg/kg/day in 3 divided doses.
• Can also be used in combination
with Desferal®, using a lower dose of
50mg/kg/day.
• Risks of GI disturbance, arthritis and
rare occurrence of idiopathic
agranulocytosis.
• Stop if neutropenic
(<1,500/mm³).
• Can also be used for transfusional
iron overload in patients 2 years or
older
• Expensive.
• The dose is 20-30 mg/kg/day in
liquid dispersible tablet, taken once
daily.
• There are risks of transient skin rash,
GI disturbance and a reversible rise in
serum creatinine.
50
55. Splenectomy
Indications
• Blood consumption volume of pure RBC > 1.5X
normal or >200-220 mls/kg/year in those > 5
years of age to maintain average haemoglobin
levels.
• Evidence of hypersplenism.
55
57. Example of calculation
(volume pure RBC/kg/yr)
▫ Wt 16 kg
▫ Average HCT of pack RBC given 50-55% (0.55)
• Total PC transfused in a yr
(300cc)(12)=3600cc
• Annual blood requirement/kg
3600cc/16kg=225cc/kg/yr
• Annual pure red cells requirement/kg
(225)(0.55)=123.75cc/kg/yr
57
58. Note:
• Give pneumococcal and HIB vaccinations 4-6 weeks
prior to splenectomy.
• Meningococcal vaccine required in endemic areas.
• Penicillin prophylaxis for life after splenectomy.
• Low dose aspirin (75 mg daily) if thrombocytosis >
800,000/mm³ after splenectomy.
58
65. Diet and supplements
• Oral folate at minimum 1 mg daily
• Low dose Vitamin C at 3 mg/kg augments iron excretion for those on
Desferral only. Dose: <10 yrs, 50mg daily; >10yrs, 100mg daily given
only on desferral days
• Avoid iron rich food such as red meat and iron fortified cereals or
milk.
• Tea may help decrease intestinal iron absorption.
• Dairy products are recommended as they are rich in calcium.
• Vitamin E as antioxidant.
• Calcium and zinc.
65
66. Bone marrow transplantation
• Potential curative option when there is an HLA-compatible
sibling donor.
• Results from matched unrelated donor or unrelated cord blood
transplant are still inferior with higher morbidity, mortality and
rejection rates.
• Classification of patients into Pesaro risk groups based on the
presence of 3 risk factors: hepatomegaly > 2cm, irregular
iron chelation and presence of liver fibrosis.
• Best results if performed at the earliest age possible in Class 1
patients.
66
69. 5. Take home messages
• What is thalassemia?
• Genetic transmission and variations
• Family screening
• Patient education and compliance
• Complications
69
70. References:
1. Pediatric Protocol 3rd ed
2. Illustrated Textbook of Pediatrics of 3rd ed
3. Nelson Essential of Pediatrics 6th ed
4. Malaysian CPG Management of Transfusion Dependent Thalassemia
November 2009
5. Guidelines for the Clinical Management of Thalassemia 2nd Revised
ed by Thalassemia International Federation 2008
6. www.mytalasemia.net.my
7. Molecular basis of thalassemia by Chris Chan, Louis Chiu, Lok Tin Liu
and Janet Lui
8. http://library.med.utah.edu/WebPath
9. CDC
70
Editor's Notes
Introduction-definition, genetic transmission, pathophysiology and epidemiology
Diagnosis-clinical features, investigations process