Haemolytic Anaemias in dentistry

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Haemolytic Anaemias in dentistry

  1. 1. Increased lysis of RBC’s Normal RBC life span is 90-120 days In these life span of RBC’s decreases Haemolytic diseases result in anaemia if the bone marrow is not able to replenish adequately the premature destroyed RBC’s
  2. 2. CLASSIFICATION <ul><li>Due to intra-erythrocytic defects </li></ul><ul><li>CONGENITAL </li></ul><ul><li>MEMBRANE DEFECTS  hereditary spherocytosis, hereditary elliptocytosis </li></ul><ul><li>HAEMOGLOBIN DEFECTS  sickle cell anaemia, thalassaemia other abnormal haemoglobin (Hb C, Hb D) </li></ul><ul><li>ENZYME DEFECTS  G6PD deficiency, pyruvate kinase deficiency </li></ul><ul><li>ACQUIRED </li></ul><ul><li>Paroxysmal nocturnal haemoglobinuria </li></ul><ul><li>Due to extra- erythrocytic defects </li></ul><ul><li>Autoantibodies( autoimmune and alloimmune) </li></ul><ul><li>Mechanical (prosthetic heart valves, microangiopathic haemolytic anaemia) </li></ul><ul><li>Drugs(dapsone) </li></ul><ul><li>Infections (malaria) </li></ul><ul><li>Inflammatory and neoplastic diseases </li></ul>
  3. 3. COMMON CLINICAL FEATURES OF HAEMOLYTIC ANAEMIAS <ul><li>Mild jaundice </li></ul><ul><li>Symptomns of anaemia </li></ul><ul><li>Urine is normal in colour on passing but turns dark on standing due to oxidation of urobilinogen to urobilin called as “black water” </li></ul><ul><li>Acute back pain </li></ul><ul><li>Symptomns of cholelithiasis </li></ul><ul><li>Splenic pain due to rapid enlargement of spleen </li></ul><ul><li>Infections </li></ul>
  4. 4. ORAL MANIFESTATIONS <ul><li>Pallor or jaundice of oral mucosa </li></ul><ul><li>Paresthesia of mucosa </li></ul><ul><li>Hyperplastic marrow spaces in maxilla, mandible and facial bones </li></ul><ul><li>PALLOR OF ORAL MUCOSA </li></ul>
  5. 5. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA <ul><li>It is a intravascular hemolytic anaemia </li></ul><ul><li>Very rarely seen </li></ul><ul><li>Due to mutation in X – linked gene, termed PIG-A (phosphatidyl inositol glycans) </li></ul><ul><li>Defect in stem cell is a mutation affecting myeloid progenitor cells that normally is required for biosynthesis of glycosyl phosphatidyl inositol(GPI) needed to anchor red cell membrane. </li></ul><ul><li>sensitivity of RBC membrane to complement </li></ul>
  6. 8. CLINICAL FEATURES <ul><li>FATIGUE </li></ul><ul><li>PAIN </li></ul><ul><li>EOSOPHAGEAL SPASM </li></ul><ul><li>ERECTILE DYSFUNCTION </li></ul><ul><li>THROMBOSIS </li></ul><ul><li>TREATMENT </li></ul><ul><li>ALLOGNIC BONE MARROW TRANSPLATATION </li></ul><ul><li>ECULIZUMAB  MONOCLONAL ANTIBODY AGAINST C5 THAT INHIBITS OMPLEMENT ACTIVATION  REDUCE HAEMOLYSIS </li></ul>
  7. 9. GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY ANAEMIA <ul><li>INHERITED AS AN X-LINKED HEMOLYTIC ANAEMIA </li></ul><ul><li>G-6-PD ACTS VIA HMP SHUNT TO CATALYZE THE OXIDATION OF G-6-PD TO 6-PHOSPHOGLUCONATE </li></ul><ul><li>GLUCOSE </li></ul><ul><li>ATP ADP HEXOKINASE G6PD </li></ul><ul><li>GLUCOSE-6-PHOSPHATE 6-PHOSPHOGLUCONATE </li></ul><ul><li>NADP NADPH </li></ul><ul><li>PHOSPHOENOL PYRUVATE </li></ul><ul><li>ATP ADP PYRUVATE OXIDIZED REDUCED </li></ul><ul><li>KINASE GLUTATHIONE GLUTATHIONE </li></ul><ul><li>PYRUVATE HMP SHUNT </li></ul><ul><li>EMBRDEN MEYERHOF PATHWAY </li></ul>
  8. 11. <ul><li>PRECIPITATING OR AGGRAVATING FACTORS OF HAEMOLYSIS </li></ul><ul><li>DRUGS </li></ul><ul><li>Analgesics  aspirin </li></ul><ul><li>Antimalarials  primaquine , chloroquine </li></ul><ul><li>Antibiotics  sulfonamides, nalixidic acid </li></ul><ul><li>Miscellaneous  vit K, Vit C </li></ul><ul><li>VIRAL INFECTIONS </li></ul><ul><li>BACTERIAL INFECTIONS </li></ul><ul><li>DIABETIC ACIDOSIS </li></ul><ul><li>BROAD BEAN ( VICIA FABA IN MEDITTERANEAN VARIETY ) </li></ul>
  9. 12. CLINICAL FEATURES <ul><li>MAJORITY OF AFFECTED PEOPLE REMAIN CLINICALLY ASYMPTOMATIC THROUGHOUT THEIR LIVES. </li></ul><ul><li>MALAISE </li></ul><ul><li>WEAKNESS </li></ul><ul><li>ABDOMINAL OR LUMBAR PAIN </li></ul><ul><li>PERIPHERAL VASCULAR COLLAPSE </li></ul><ul><li>JAUNDICE AND DARK URINE DUE TO INTRAVASCULAR HEMOLYSIS </li></ul><ul><li>ACUTE RENAL FAILURE </li></ul><ul><li>ANAEMIA AND HAEMOGLOBINURIA ( COLA COLORED URINE) </li></ul>
  10. 13. <ul><li>DIAGNOSIS </li></ul><ul><li>BY MEASURING RBC G-6-PD ACTIVITY BY QUANTITATIVE ASSAY </li></ul><ul><li>SHOULD BE DONE AFTER THE ACUTE EPISODE . </li></ul><ul><li>TREATMENT </li></ul><ul><li>REMOVAL OF OFFENDING AGENT </li></ul><ul><li>SUPPORTIVE THERAPY FOR ANAEMIA LIKE BLOOD TRANSFUSION </li></ul><ul><li>TREATMENT OF INFECTION </li></ul>
  11. 14. ORAL HEALTH CONSIDERATIONS <ul><li>PATIENTS SHOULD MAINTAIN EXCELLENT ORAL HYGIENE AND COMPLY WITH ROUTINE RECALL VISITS AS TO PREVENT ORAL AND PERIODONTAL INFECTION. </li></ul><ul><li>PROMPT AND AGGRESSIVE TREATMENT OF ORAL INFECTION ONCE DIAGNOSED IS IMPORTANT </li></ul><ul><li>PATIENTS SHOULD AVOID THE USE OF ASPIRIN OR OYHER DRUGS KNOWN TO TRIGGER HAEMOLYSIS. </li></ul>
  12. 15. SICKLE CELL ANAEMIA <ul><li>AUTOSOMAL RECESSIVE IN INHERITENCE </li></ul><ul><li>HEMOGLOBIN GENE MUTATION </li></ul><ul><li>GLUTAMIC ACID  VALINE AT SIXTH POSITION ON THE β -HEMOGLOBIN CHAIN </li></ul><ul><li>NORMAL BICONCAVE  SICKLE SHAPED </li></ul><ul><li>DISCOID SHAPE </li></ul><ul><li>(120 DAYS) (14 DAYS) </li></ul><ul><li>RESULTING IN ANAEMIA AND HYPERTROPHIC BONE MARROW </li></ul>
  13. 17. ORAL MANIFESTATIONS <ul><li>“ STEP LADDER”TRABECULAR PATTERN </li></ul><ul><li>ENAMEL HYPOMINERALIZATION </li></ul><ul><li>CALCIFIED CANALS </li></ul><ul><li>INCREASEDOVERBITE </li></ul><ul><li>INCREASED OVERJET </li></ul><ul><li>PALLOR OF ORAL MUCOSA AND DELATED ERUPTION OF TEETH </li></ul>
  14. 18. <ul><li>PALLOR OF ORAL MUCOSA </li></ul><ul><li>INCREASEDOVERJET </li></ul><ul><li>INCREASED OVERBITE </li></ul>
  15. 19. <ul><li>INVOLVEMENT OF MAXILLOFACIAL SKELETON LEADING TO RADIOOPAQUE LESIONS CORRESPOND TO BONE INFARCTS IN THE COURSE OF KNOWN VESSEL OR IN THE APICAL REGION OF TEETH </li></ul><ul><li>SUCH LESIONS COMBINED WITH FACIAL PAIN OR SENSORY CHANGES IN THE DISTRIBUTION OF THE INFERIOR ALVEOLAR NERVE DURING SICKLE CELL CRISIS AND ABSENCE OF DENTAL PATHOLOGY SHOULD BE CONSIDERED TO BE OF POSSIBLE VASO-OCCLUSIVE ORIGIN. </li></ul><ul><li>INTERRUPTION OF BLOOD SUPPLY CAN RESULT IN AN ANAESTHESIA OF INFERIOR ALVEOLAR NERVE AND PULPAL NECROSIS OF OTHERWISE SOUND PREMOLAR AND MOLAR TEETH. </li></ul><ul><li>RADIONUCLEOTIDE SCAN OF MANDIBLE MAY DEMONSTRATE THE POSITION AND EXTENT OF INFARCTED AREA. </li></ul><ul><li>OSTEOPOROSIS OF JAWS. </li></ul>
  16. 20. <ul><li>GARRES OSTEOMYELITIS OF MANDIBLE </li></ul>
  17. 21. GNATHOPATHY MAXILLARY EXCESS OF A 28 YEAR OLD AFRICAN AMERICAN WITH SCA LEFT MOLAR BITEWING RADIOGRAPH RADIOGRAPHIC STEP LADDER APPEARANCE AND DENSE LAMINA DURA, RESULTING FROM HYPERPLASTIC MARROW
  18. 23. ORAL HEALTH CONSIDERATIONS <ul><li>ANTIBIOTIC PROPHYLAXIS OF CHILDREN WITH SCA FOR THE FOLLOWING CLINICALSOLUTIONS:- </li></ul><ul><li>DENTAL EXTRACTIONS </li></ul><ul><li>TREATMENT UNDER GA </li></ul><ul><li>STATUS POST SPLENECTOMY </li></ul><ul><li>AMOXYCILLIN IS THE MOST COMMON CHOSEN ANTIBIOTIC. </li></ul><ul><li>MAINTAINING GOOD ORAL HYGIENE </li></ul><ul><li>ROUTINE CARE DURING NO CRISIS PERIOD </li></ul><ul><li>AGGRESSIVE TREATMENT OF ORAL INFECTION </li></ul><ul><li>AVOIDANCE OF USE OF ASPIRIN </li></ul><ul><li>CAUTION WITH RESPIRATORY DEPRESSING CONCIOUS SEDATION </li></ul><ul><li>AVOIDANCE OF LONG STRESS DENTAL VISITS </li></ul><ul><li>USE OF NITROUS OXIDE FOR ANXIOLYSIS IS SAFE WITH MAINTAINENCE OF ADEQUATE FLOW RATES. </li></ul><ul><li>PREOPERATIVE TRANSFUSION OF SCA PATIENTS PRIOR TO GA MAY BE NEEDED TO INSURE ADEQUATE LEVELS OF NORMAL HbA TO PREVENT SICKLE CELL CRISIS. </li></ul><ul><li>AS COMPLETE BLOOD SUPPLY IS VERY IMPORTANT DURING APPLICATION OF BOTH INTRAORAL AND EXTRAORAL FORCES SUCH PATIENTS WHO SEEK ORTHODONTIC TREATMENT REQUIRE MULTIDISCIPLINARY MANAGEMENT. </li></ul>
  19. 24. THALASSEMIAS <ul><li>GROUP OF GENETIC DISORDERS of Hb SYNTHESIS CHARACTERIZED BY DISTURBANCE OF EITHER α OR β Hb CHAIN PRODUCTS. </li></ul><ul><li>SO CAN BE CLASSIFIED AS α THALASSEMIA </li></ul><ul><li>β THALASSEMIA </li></ul><ul><li>Hb BARTS HYDROPS </li></ul><ul><li>FURTHER CAN BE CLASSIFIED AS α THALASSEMIA MAJOR FETALIS </li></ul><ul><li>Hb H </li></ul><ul><li>α THALASSEMIA MINOR (TRAIT) </li></ul><ul><li>SIMILARLY β THALASSEMIA MAJOR (HOMOZYGOUS) </li></ul><ul><li>β THALASSEMIA MINOR (HETEROZYGOUS) </li></ul><ul><li>β THALASSEMIA IS ONE OF THE FREQUET SEEN HEMOGLOBINOPATHIES </li></ul><ul><li>COOLEY’S ASNAEMIA OR THALASSEMIA MAJOR IS THE SEVEREST FORM OF </li></ul><ul><li>β THALASSEMIA. </li></ul>
  20. 26. ORAL MANIFESTATIONS <ul><li>RADIOGRAPHICALY JAWS AND TEETH AMONG PEOPLE WITH THALASSEMIA MAJOR INCLUDE:- </li></ul><ul><li>APPEARANCE OF SPIKY SHAPED AND SHORT ROOTS </li></ul><ul><li>TAURODONTISM </li></ul><ul><li>ATTENUATED LAMINA DURA </li></ul><ul><li>ENLARGED BONE MARROW SPACES </li></ul><ul><li>SMALL MAXILLARY SINUS </li></ul><ul><li>ABSENCE OF INFERIOR ALVEOLAR CANAL </li></ul><ul><li>THIN CORTEX OF MANDIBLE </li></ul>
  21. 27. CRANIOFACIAL DEFORMITIES  CLASS 2 SKELETAL BASE RELATIONSHIP WITH SHORT MANDIBLE REDUCED POST. FACIAL HEIGHT INCREASED ANT. FACIAL PROPORTION THINNING OF CORTICAL PLATE AT MANDIBULAR ANGLE REGION
  22. 28. <ul><li>SALT AND PEPPER APPEARANCE </li></ul><ul><li>O OBLITERATED SINUSES </li></ul>
  23. 29. <ul><li>SOME WITH SEVERE FACIAL DISFIGUREMENTS (GRADE 3 OR CHIPMUNK FACES) . </li></ul><ul><li>DENTAL ARCH MORPHOLOGIC CHANGES INLUDE NARROWER MAXILLA AND SMALL INCISOR WIDTHS FOR THE MAND. AND MAX. ARCHES </li></ul><ul><li>CONSISTENT WITH GENERAL GROWTH RETARDATION, DENTAL DEV. OF PATIENTS WITH β THALASSEMIA MAJOR WAS FOUND TO BE DELAYED BY A MEAN OF 1.61 YEARS,INCREASED WITH AGE.HIGHER FOR BOYS THAN GIRLS COMPARED WITH UNAFFECTED CHILD. </li></ul><ul><li>INCREASED DENTAL CARIES EXPERIENCE </li></ul><ul><li>Ig A AND PHOSPHOROUS CONTENT OF SALIVA IS LESS. </li></ul>
  24. 30. <ul><li>FACE OFTEN DEVELOPS MONGOLOID FEATURES DUE TO PROMINENCE OF CHEEK BONES PROTRUSION OR FLARING OF MAXILLARY ANT. TEETH AND DEPRESSION OF BRIDGE OF NOSE GIVING RISE TO CHARACTERSTIC RODENT FACIES. </li></ul>
  25. 31. ORAL HEALTH CONSIDERATIONS <ul><li>PRIMARY CONCERN IS THE LEVEL OF ANAEMIA </li></ul><ul><li>HEMOGLOBIN LEVELS OF 10.9 g/dl OR LESS WERE FOUND IN 3% OF 1000 CHILDREN NEEDING MINOR DENTAL SURGERY. </li></ul><ul><li>PLANNED GENERAL ANAESTHESIA WERE UNDERTAKEN WITHOUT TRANSFUSION ALLOWING AUTHORS TO CONCLUDE THAT PREANAESTHETIC HEMATOLOGICAL ASSESMENT IS RARELY OF ANY SIGNIFICANCE. </li></ul>
  26. 32. MEGALOBLASTIC ANAEMIA <ul><li>ANAEMIA DUE TO INEFFECTIVE ERYTHROPOESIS. </li></ul><ul><li>CHARACTERIZED BY MACROCYTOSIS ASSOCIATED WITH MEGALOBLASTIC MARROW. </li></ul><ul><li>IT IS DUE TO VIT. B12 OR FOLATE DEFICIENCY. </li></ul><ul><li>CAUSES OF DEFICIENCY MOST FREQUENTLY INCLUDE FOOD-COBALAMIN MALABSORPTION SYNDROME,PERNICIOUS ANAEMIA, INSUFFICIENT DIETARY INTAKE, AND MALABSORPTION. </li></ul><ul><li>PERNICIOUS ANAEMIA IS AN AUTOIMMUNE DISEASE RESULTING FROM AUTOANTIBODIES DIRECTED AGAINST INTRINSIC FACTOR AND GASTRIC PARIETAL CELLS. </li></ul>
  27. 33. ORAL MANIFESTATIONS <ul><li>BURNING SENSATIONS IN TONGUE, LIPS, BUCCAL MUCOSA AND OTHER MUCOSAL SITES. </li></ul><ul><li>TONGUE AND MUCOSA MAY BE SMOOTH OR PATCHY AREAS OF ERYTHEMA. </li></ul><ul><li>DYSPHAGIA AND TASTE ALTERATIONS HAVE BEEN REPORTED. </li></ul><ul><li>ATROPHY OF PAPILLAE OF TONGUE RESULTING IN HUNTER’S OR MOELLER’S GLOSSITIS </li></ul><ul><li>TONGUE LOOKS BALD AND BEEFY RED IN COLOR </li></ul>
  28. 34. ERYTHEMA OF LIPS GLOSSODYNIA

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