Resident: Adam J. Bottrill
Date: 09MAR11
Region: Providence
Article title: Sickle Cell Anemia: A Review for the Pediatric Dentist
Author(s): Fonseca, Marcio A. et al.
Journal: Pediatric Dentistry
Page #s: 159-169
Vol:No Date: 2007
Major topic: Sickle Cell Anemia
Minor topic(s): None
Type of Article: Topic Summary and Discussion
Main Purpose: Provide a comprehensive review of Sickle Cell Anemia
Key points in the article discussion:
I. General:
A. Sickle Cell Anemia (SCA)is an inherited defect that affects the structure and synthesis of hemoglobin.
B. Alpha and Beta Thalassemia and SCA are the most common types of hemoglobinopathies worldwide.
C. SCA: most frequent genetic disorder. Found often in African, Mediterranean, Middle Eastern, Indian, Caribbean, Central American and South American.
D. Comprises sickle cell trait and sickle cell disease (SCD)
E. SCD: hemolysis, chronic organ damage, unpredictable acute life-threatening complications.
G. Trait: Benign. 8% of African Americans and 10-30% of equatorial Africans.
H. HbS (sickle hemoglobin) carriers protected from Malaria.
II. Pathophysiology:
A. Polymerization of deoxygenated HbS... "single, indispensable event in the path..."
B. Lifespan of sickle RBC=12-17 days (normal=120).
C. Sickle cells caught in slow-flowing venular side of microcirculation.... increased adhesion to endothelial walls and local hypoxia increased HbS polymer formation and spread of occlusion to adjacent vasculature. Increased inflammation.
D. Problem becomes "erythrocitic sticking" rather than simple "sticking". Other problems include adhesion, hemolysis, deformation.
E. Symptoms arise from chronic endothelial damage instead of acute erythrocyte formation. "chronic inflammatory vascular disease"
III. Systemic manifestations:
A. Symptoms begin within 1st 6 mo of life: evolving organ damage with intermittent pain and pulmonary complications.
B. Genotype most effects the clinical severity.
C. "Crisis" usually occur due to other illnesses, dehydration, temperature changes, hypoxia, stress and menstruation.
D. Other symptoms: Short stature, deformed bones, PAIN, PAIN, PAIN, splenic sequestration crisis, cardiomegaly, systolic murmors, CHF, osteomylitis, osteonecrosis, marrow hyperplasia, acute chest syndrome (leading cause of death and hospitalization among SCD patients), cerebrovascular events, impaired psychosocial function, altered inter-intrapersonal relationships, altered sexuality, poor body image, social withdrawal, poor academic functioning, marital problems.
IV. Prevention and Tx:
A. Hydroxyurea (marrow suppression) has been shown to decrease crisis occurrences and pain. Remains unlicensed in most countries due to unknown long term side effects.
B. Being studied: Vascular lubricants, xanthine oxidase inhibitors, nitric oxide enhancers, gardos channel inhibitors, monoclonal AB's, statins, gene therapy.
C. ONLY current available curative therapy is hematopoietic stem cell transplantation which is needed if organ dysfunction occurs.
D. Pain usually managed at home with opioid and non-opioid meds, massage, heating/cooling, electric nerve stimulation.
E. Splenic sequestration: correction of hypovolemia with a transfusion. If it occurs 2-3 yo, spenectomy. Lifetime of AB prophylaxis.
F. Blood Transfusions:
1. increase oxygen-carrying blood
2. improve end-organ perfusion
3. Indications: severe symptoms, splenic sequestration, ACS, acute organ damage, surgery
4. NOT indicated: stable compensated anemia, infections without aplastic crisis, minor surgeries, uncomplicated acute painful crisis.
V. SCD and GA:
A. Dental restorations and simple extractions may be performed on low-risk patients. Moderate risk and high risk must go to OR.
B. NIH recommends simple transfusion prior to all but low risk procedures such as dental, optha etc....
C. Goal of GA management remains meticulous attention to the basic principles of safe anesthesia.
VI. Oral/Dental/Craniofacial Manifestations.
A. Jaundice mucous membranes, glossitis, pallor of tongue, delayed tooth eruption, decreased radiodensity, abnormal radiographic bone presentation, radioluscent teeth, bimaxillary protrusion, flared incisors, tower skull, hair-on-end appearance, granular appearance of skull, mandibular osteomylitis (men),
B. Possible oral pain in the absence of odontogenic pain due to occlusive crisis in the microvasculature of facial muscles.
C. Crisis MAY be precipitated by perio infection, pericoronitis.
D. Possibly a lower caries rate due to long term antibiotic use by those with the disease. Lowered caries rate falls off later in life due to stopped AB use.
VII. Dental Management:
A. HISTORY HISTORY HISTORY
1. Complications, pain episodes, med tx, catheters, blood-born viruses due to transfusions, growth and development.
2. Social and coping issues.
B. Removal of any potential infections must be performed.
C. Painful teeth should be treated seriously.
D. No conclusions have been drawn WRT best pulp therapy.
E. Restorations preferable to extraction.
F. Facial swelling or celulitis may warrant hospitalization.
G. Operative during NON crisis periods.
H. CBC should be done before any invasive dental procedure if the patient is taking HU.
I. No official rec regarding altering Tx due to SCA pts taking bisphosphonates.
J. No evidence supporting using anesthesia withOUT vasoconstrictor.
K. N2O is ok... but care to AVOID hypoxia. Max 50% and pt should breathe 100% O2 for a few minutes following.
L. Need for AB proph remains controversial (some use for endocartditis while others use to prevent systemic infection).
M. Elective surgeries should be avoided.
N. No contra-indications for ortho... unless non-compliance with OH.
O. PHYSICIAN SHOULD BE CONSULTED!!!
Assessment of Article: QUALITY article. Good scientific review, medical considerations, dental considerations, GA considerations!! The ULTIMATE SCA REFERENCE ARTICLE!!!!! Don't think I've ever been this enthusiastic about an article. Don't worry, it won't happen again.
Showing posts with label 03/09/2011. Show all posts
Showing posts with label 03/09/2011. Show all posts
Wednesday, March 9, 2011
Tuesday, March 8, 2011
03/09/2011 Oral Manifestations of Vitamin B12 Deficiency: A Case Report
Resident: J. Hencler
Date: 03/09/2011
Article title: Oral Manifestations of Vitamin B12 Deficiency: A Case Report
Author: Pontes, Neto, Ferreira, Fonseca, Vallinoto, Pontes, Pinto Jr
Journal: JCDA Sept 2009, Vol. 75, No. 7
Major topic: B12 deficiency and megaloblastic anemia
Type of Article: Case report
Main Purpose: Report a case of megaloblastic anemia in which oral manifestations were significant.
Background:
Megaloblastic anemias are a subgroup of macrocytic anemias caused by impaired DNA synthesis that results in macrocytic RBCs, abnormalities in leukocytes and platelets, and epithelial changes, particularly in the rapidly dividing epithelial cells of the mouth and GI tract. The most common causes of megaloblastic anemias are cobalamin (vitamin B12) and folate (vitamin B9) deficiency. Clinically, megaloblastic anemia progresses slowly (takes 2-5 years to develop) and symptoms include weakness, fatigue, shortness of breath, and neurologic abnormalities. Oral signs and symptoms including glossitis, angular chelitis, recurrent oral ulcer, oral candidiasis, diffuse erythematous mucositis, and pale oral mucosa, offer the dentist an opportunity to diagnosis of this condition.
Findings:
41 year old female with cc of difficulty in eating certain types of food (banana and tomato) because of a burning sensation and the presence of red stains on her cheek and tongue. She has been a strict vegetarian for 2.5 years and had not consumed milk, cheese, fish, meat or eggs during that time. Clinical evaluation and patient reports include paleness and dry lips, disturbance of taste, fatigue after simple daily activities, paresthesia of structures innervated by mandibular division of the trigeminal nerve, and disturbance of memory and slowing mental faculty. Oral exam revealed pale oral mucosa, glossitis with papillary atrophy and multiple areas of painful erythema on the dorsal surface and lateral borders of the tongue and buccal mucosa. Hematologic tests were done and a diagnosis of megaloblastic anemia was made. Treatment comprised doses of B12 (1000mg/wk) and intramuscular hydroxocobalamin and 1mg folic acid daily. After 14 days, the lesions had healed and all symptoms disappeared.
Key points in the article discussion:
Vitamin B12 is only found in bacteria, eggs, and foods of animal origin. Most B12 is stored in the liver. Megaloblastic anemia occurs when the body’s stores fall below 0.1mg. B12 or B9 deficiency are the most important cofactors necessary for normal maturation of all cells and B12 is necessary for DNA synthesis, as it’s deficiency prevents cell division in the bone marrow. When either of these factors is deficient, RBCs become large erythroblasts with nuclear or cytoplasmic asynchrony. A wide range of oral signs and symptoms may appear in anemic patients as a result of basic changes in the metabolism of oral epithelial cells. Abnormalities in cell structure and keratinization pattern of the oral epithelium can lead to a beefy, red, and inflamed tongue with erythematous macular lesions on the dorsal and border surfaces. Differential diagnosis of patients with similar signs and symptoms include iron deficiency, diabetes, allergy, autoimmune disease, physical and chemical injury, atrophic candidiasis, and anemia of chronic disease. Although B12 deficiency is almost always associated with strict vegetarians, the condition can also result from gastrectomy, bacterial overgrowth in small intestine, diverticulitis, celiac disease, Crohn’s disease, alcoholism, HIV, and certain medications. Careful investigation of clinical history and clinical exam will often lead to the cause of megaloblastic anemia.
Summary of conclusions:
Megaloblastic anemia has a complex pathogenesis. As oral lesions are among the most common initial symptoms, the dentist has a responsibility alongside medical specialists to contribute to the diagnosis.
Assessment of article:
Interesting case report, but on a 40 year old vegan. Not really our patient population. The article was well written and included a very detailed discussion on the pathogenesis, oral signs and symptoms, diagnosis, and treatment of megaloblastic anemia (B12 deficiency). I found it interesting that this patient had paresthesia of CN V3 innervated structures. So asked myself…self, what is the mechanism behind such an unfortunate nuerological deficit. The mechanism is not fully elucidated but may be due to impaired synthesis of myelin phospholipids or from a deficit of succinyl-CoA that leads to the generation of odd chained fatty acids which may get incorporated into the myelin and cause the neurological syndrome of Vitamin B12 deficiency. Good shtuff.
Date: 03/09/2011
Article title: Oral Manifestations of Vitamin B12 Deficiency: A Case Report
Author: Pontes, Neto, Ferreira, Fonseca, Vallinoto, Pontes, Pinto Jr
Journal: JCDA Sept 2009, Vol. 75, No. 7
Major topic: B12 deficiency and megaloblastic anemia
Type of Article: Case report
Main Purpose: Report a case of megaloblastic anemia in which oral manifestations were significant.
Background:
Megaloblastic anemias are a subgroup of macrocytic anemias caused by impaired DNA synthesis that results in macrocytic RBCs, abnormalities in leukocytes and platelets, and epithelial changes, particularly in the rapidly dividing epithelial cells of the mouth and GI tract. The most common causes of megaloblastic anemias are cobalamin (vitamin B12) and folate (vitamin B9) deficiency. Clinically, megaloblastic anemia progresses slowly (takes 2-5 years to develop) and symptoms include weakness, fatigue, shortness of breath, and neurologic abnormalities. Oral signs and symptoms including glossitis, angular chelitis, recurrent oral ulcer, oral candidiasis, diffuse erythematous mucositis, and pale oral mucosa, offer the dentist an opportunity to diagnosis of this condition.
Findings:
41 year old female with cc of difficulty in eating certain types of food (banana and tomato) because of a burning sensation and the presence of red stains on her cheek and tongue. She has been a strict vegetarian for 2.5 years and had not consumed milk, cheese, fish, meat or eggs during that time. Clinical evaluation and patient reports include paleness and dry lips, disturbance of taste, fatigue after simple daily activities, paresthesia of structures innervated by mandibular division of the trigeminal nerve, and disturbance of memory and slowing mental faculty. Oral exam revealed pale oral mucosa, glossitis with papillary atrophy and multiple areas of painful erythema on the dorsal surface and lateral borders of the tongue and buccal mucosa. Hematologic tests were done and a diagnosis of megaloblastic anemia was made. Treatment comprised doses of B12 (1000mg/wk) and intramuscular hydroxocobalamin and 1mg folic acid daily. After 14 days, the lesions had healed and all symptoms disappeared.
Key points in the article discussion:
Vitamin B12 is only found in bacteria, eggs, and foods of animal origin. Most B12 is stored in the liver. Megaloblastic anemia occurs when the body’s stores fall below 0.1mg. B12 or B9 deficiency are the most important cofactors necessary for normal maturation of all cells and B12 is necessary for DNA synthesis, as it’s deficiency prevents cell division in the bone marrow. When either of these factors is deficient, RBCs become large erythroblasts with nuclear or cytoplasmic asynchrony. A wide range of oral signs and symptoms may appear in anemic patients as a result of basic changes in the metabolism of oral epithelial cells. Abnormalities in cell structure and keratinization pattern of the oral epithelium can lead to a beefy, red, and inflamed tongue with erythematous macular lesions on the dorsal and border surfaces. Differential diagnosis of patients with similar signs and symptoms include iron deficiency, diabetes, allergy, autoimmune disease, physical and chemical injury, atrophic candidiasis, and anemia of chronic disease. Although B12 deficiency is almost always associated with strict vegetarians, the condition can also result from gastrectomy, bacterial overgrowth in small intestine, diverticulitis, celiac disease, Crohn’s disease, alcoholism, HIV, and certain medications. Careful investigation of clinical history and clinical exam will often lead to the cause of megaloblastic anemia.
Summary of conclusions:
Megaloblastic anemia has a complex pathogenesis. As oral lesions are among the most common initial symptoms, the dentist has a responsibility alongside medical specialists to contribute to the diagnosis.
Assessment of article:
Interesting case report, but on a 40 year old vegan. Not really our patient population. The article was well written and included a very detailed discussion on the pathogenesis, oral signs and symptoms, diagnosis, and treatment of megaloblastic anemia (B12 deficiency). I found it interesting that this patient had paresthesia of CN V3 innervated structures. So asked myself…self, what is the mechanism behind such an unfortunate nuerological deficit. The mechanism is not fully elucidated but may be due to impaired synthesis of myelin phospholipids or from a deficit of succinyl-CoA that leads to the generation of odd chained fatty acids which may get incorporated into the myelin and cause the neurological syndrome of Vitamin B12 deficiency. Good shtuff.
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