The Comprehensive Examination Cage S Johnson M D
- Slides: 34
The Comprehensive Examination Cage S. Johnson, M. D. Professor Emeritus of Medicine Director, Sickle Cell Center Keck School of Medicine University of Southern California
Adult Health Care • Preventive Health Maintenance: • Psychosocial Assessment: school achievement, occupational Hx, depression, anxiety, financial resources • Education: tobacco, alcohol, recreational drugs, safe sex techniques, sickle cell related education – fever, dyspnea, pain management • Surveillance for complications of SCDz, as well as for disorders of adulthood • Referral network, early treatment of complications
General Internal Medicine • • Thyroid Cholesterol Diabetes Mellitus Hypertension
General Internal Medicine • • Thyroid Cholesterol Diabetes Mellitus Hypertension
Immunization • • • Pneumovax Hib Hepatitis B HPV Hepatitis A (confounder)
Age appropriate cancer screening • • PAP smear Mammography PSA Occult blood
Malignancy in SCD, No HU • Incidence of malignancy at Howard Univ. is 1. 74 per 1000 pt-yrs Vs. 3. 82 per 1000 ptyrs in the Afro-American population. Am J Hematol 55: 188, 1997 • 2 malignancies in 144 autopsies in Hb SS Br J Haematol 123: 359, 2003 • “CSSCD reported 16 malgnancies in over 4000 patients during a 15 y follow-up” Cited in Am J Hematol 74: 249, 2003
IASCNAPA: ~n= 16, 613 Am J Hematol 74: 249, 2003 • • All: 6 cases, 1 on HU AML: 2 cases CML: 1 case Brain, Breast, Bladder, Bone, Gall bladder, GI tract, Lymphoma – HD & NHL, Myeloma, Pancreatic, Testis
Manifestations of Sickle Cell Disease 1. 2. 3. 4. 5. Chronic Hemolysis Intermittent “crises” Frequent Infections End organ dysfunction Disorders unrelated to the Hbopathy
Acute & Chronic Manifestations of Sickle Cell Disease • • Pain : Brain: Eye: Bone: ischemic, neuropathic, collapsed bone TIA, ischemic Stroke, hemorrhage retinopathy remodeling by marrow hyperplasia, osteonecrosis, collapse, osteomyelitis • Lung: ACS, chronic restrictive lung disease (hypoxia) • Heart : pulmonary hypertension • Liver : gallstones, hepatitis, iron overload, sequestration • Spleen : auto-infarction, sequestration, bacterial sepsis • GU: infection, proteinuria, renal insufficiency, obstetrical, priapism • Skin: leg ulcers
Surveillance: (repeated annually) • H/O sickling related events: Pain-acute & chronic, CVA, PNA/ACS (ICU), Hepatitis, Cholelithiasis, Priapism, Retinopathy, Pregnancy, Leg Ulcer, Hematuria/Infection, Bone infections/AVN, Transfusion • Lab: CBC, Bun/Creatinine, Urine protein, Iron/Ferritin, Hepatitis markers, B 12, Folate, RBC phenotype, pulse oximetry, allantibodies, EKG, additional assays based upon Hx/Px • X-ray: Chest, Hips, Shoulders, T/L Spines, etc.
Br Med J 295: 234, 1987
Hepatic Diseases • Cholelithiasis • Chronic active hepatitis B/C RUQ ultrasound (biopsy) Viral load & genotype Rx: Interferon & Ribivarin • Iron overload
Pulmonary Disease (CSSCD) • N = 318, > 18 y of age (mean 31 10), 41 % male • 90 % abnormal Restrictive - 74 % Obstructive - 1% Mixed O/R - 2% DLCO - 13 % • Association between lung & kidney disease Am J Resp Crit Care Med epublished 23 Oct 2005
J Clin Epidemiol 45: 893, 1992
Prevalence rates of proteinuria and renal insufficiency per decade of life Int J Artif Organs 13: 231, 1990
Proteinuria microalbuminuria: > 20 mg/g creatinine macroalbuminuria: > 300 mg/g creatinine Histology: Progressive focal segmental glomerular sclerosis – – – effacement of foot processes thickening of basement membrane Fe+++ deposition Differential diagnosis: – – – post streptococcal glomerulonephiritis acute diffuse proliferative glomerulonephritis acute diffuse mesangial glomerulonephritis membranoproliferative glomerulonephritis renal vein thrombosis Treatment: – ACE inhibitor
Hematuria • Papillary necrosis • Glomerulonephritis • Medullary Carcinoma
Renal Insufficiency • Anemia: EPO + HU • Acidosis - ineffective erythropoiesis - Shohl’s Solution • Blood Pressure
Management of Hypertension • Rx if BP > 130/80 • Assess for renal disease • Rx if BP rises from baseline by 20 mm Hg systolic or 10 mm Hg diastolic • With proteinuria: Rx if BP > 120/75 or with > 300 mg/24 h
Management of Hypertension • ACE inhibitor – hyperkalemia (spurious) • Calcium channel blocker – dose adjustment in hepatic or renal disease • Beta-blocker – asthma, CHF, thrombocytopenia, bradycardia • Diuretic – dose adjustment for hyposthenuria/naturesis
Air Travel • • • Identify a source of care at the destination Oxygen if Sp. O 2 < 88% Non-caffeinated fluids Walk every hr or more often VTE prophylaxis with h/o prior VTE
CHALLENGES in MANAGEMENT of the ADULT PATIENT: • Shorten the duration of acute complications • Reduce the frequency/severity of chronic complications • Improve survival • Enhance the Quality of Life
Options • Stem Cell Transplantation • Hydroxyurea, L-Glutamine • Crizanlizumab • Transfusion/Chelation • Gene Therapy
Mortality: Hydroxyurea • 40 % reduction (JAMA 290: 756, 2003) • Unclear (Blood 105: 545, 2005)
Leukemia in SCD on HU • ALL, Ph+: 10 y F SS, 7 weeks of Rx Arch Dis Child 81: 437, 1999; Blood 98: 2878, 2001 • • AML: 42 y F SS, 6 yrs of Rx Ann Intern Med 133: 925, 2000 AML: 2 yrs of Rx Saudi Med J 23: 1411, 2002 APL(m 3 v): 21 y F SS, 8+ yrs of Rx Br J Haematol 123: 368, 2003 ANLL: 27 y F SO-Arab, 8 yrs of Rx on a background of MDS Blood 94: 415 a, 1999
Leukemia in SCD, No HU • ALL: 4 cases Clin Pediatr 11: 183, 1972 Cent Afr J Med 34: 158, 1988 Cancer Genet Cytogenet. 21: 267, 1986 Arch Pathol Lab Med 123: 745, 1999 • AML: 5 cases Ann Intern Med 39: 920, 1953 Am J Med 73: 268, 1982 N Engl J Med 311: 780, 1984 Am J Hematol 21: 223, 1986 Indian Pediatr 16: 453, 1979 • CML, Ph+: 6 cases (plus 1 from USC) Hum Genetik 30: 13, 1975 N Engl J Med 298: 72, 1978 Am J Med 85: 567, 1988 J Assoc Physicians India 34: 821, 1986 Am J Hematol 31: 302, 1989 Arch Pathol Lab Med 129: 423, 2005
Long Term Follow-up of SCD on HU • MSH: no malignancy at 9 yrs (n=156 on HU>1 y) JAMA 289: 1645, 2003 • MCG: no malignancy in 236 adults at 15 yrs Blood 195: 545, 2005 • HUG-KIDS: no malignancy in 84 children at 2+ yrs Blood 94: 1550, 1999 • France: 1 ALL in 101 children at 2+ yrs Arch Dis Child 81: 437, 1999 • Belgium: 1 AML (M 3 v) in 141 children with 598 pt-yrs of F/U Br J Haematol 123: 368, 2003
Summary: Goal of reducing morbidity & mortality • More aggressive use of Hydroxyurea: www. ahrq. gov/clinic/tp/hydscdtp. htm • • Additional therapies Regular Surveillance Prompt referral Individualize therapy to the patient
Gene Therapy • Clinical Trial in 5 Hb SS & 5 Thal (Paris) • Self-inactivating lentiviral vector • A-T 87 Q Ann NY Acad Sci 1054: 308, 2005 ,
Gene Therapy • Blue. Bird Bio
- Credo
- Jjeds employee directory
- Johnson background
- Johnson and johnson organizational structure
- Dreikurs classroom management theory
- Johnson and johnson bcg matrix
- Johnson and johnson botnet infections
- Johnson and johnson md&d
- Brad and laurie johnson
- Johnson and johnson swot analysis
- John cage water walk
- Find my cage code
- They cage the animals at night
- Why does the tiger ignore the visitors
- Tianeptin
- Fontana mix john cage
- Squirrel cage rotor
- Bengal stackable cage system
- Kyle cage
- How to measure ap diameter of chest
- The old witch locked hansel in a cage and set gretel
- Cage distance framework
- Cage full form
- Speed control of squirrel cage induction motor
- It is a bony cage enclosing vital human organs
- Cage alcohol
- Cage framework
- Rib head articulation
- Synex cage
- Ribs in human body
- Embrasses
- John cage is the proponent of chance music
- Lucas lira soares
- Jacking cage
- Cage questionaire