1993년 가톨릭 의과대 본과에 들어간 세 명의 젊은 의학도는 같은 의문을 품었습니다. 우리가 만든 의학교과서는 왜 없을까? 우리는 왜 외국서적으로만 공부해야 할까? 외국의 의대생이 우리가 만든 의학교과서로 공부하게 할 순 없을까? 이런 의문에 답을 내고, 또 그 답을 실현시키기까지 15년의 세월이 흘렀습니다. 20대 청년에서 이제 중년의 의사가 된 지금, 그 15년의 이야기를 15분의 메시지로 전합니다.
J& 2 Cutaneous Hematologic Vascular al J O Systemic et cu R onj cle nd 20 el et u ri ro 0 la C is sy in nc ti m Anemia (of chronic ds) Thrombosis in vessels sk r Malar rash al ti s ia lo ep ca lg †Hemolytic anemia ‡ Lupus anticoagulant (LA) and Fatigue, malaise va val cu Si Discoid rash ya sc / c us † Leukopenia (<4000/µL) ‡ Anti-cardiolipin (aCL) ab Fever, Nausea ly os y M ul Photosensitivity itiM po er th a , Lymphopenia (<1500/ µL) : associated with clotting. Anorexia s on pa gi Oral ulcers N yo ral th e † Thrombocytopenia <100,000/ µL) Cerebral emboli Weight loss tis Alopecia M rth ar iv ri e Splenomegaly A Vasculitis Neurologic (CNS lupus) Cardiopulmonary Cognitive dysfunction (mc) Mood disorder Pleurisy, Pericarditis Myocarditis, arrhythmia Endocarditis, CAD (MI) Systemic Lupus Erythematosus Headache, Seizure Psychosis, CVA Polyneuropathy Pleural effusion Autonomic dysfunction Lupus pneumonitis Optic neuritis, SAH Heart failure, Pulmonary hypertension *The most common cause of pulmonary infiltrate in Neonatal lupus SLE in pregnancy Lumbar puncture 2001년 함께 꿈을 꾸다! patients with SLE is infection Caused by transmission (when the Dx of CNS lupus is in doubt of maternal anti-Ro Fertility rates : normal or when infection is a possible cause across the placenta Spontaneous abortion and still birth are frequent (10-30%) of symptoms) Transiet skin rash and ; Tx : low-dose heparin (e.g., 5000U S.C. twice a day) MRI with contrast Renal (lupus nephritis) (rarely) permanent heart * if contraindicated to heparin, low-dose aspirin or (acute and chronic lesion of SLE) heart block low-to moderate dose glucocorticoid may be used. CT (R/O bleeding or mass lesion) Proteinuria >500mg/24hr Transient thrombocytopenia Glucocorticoid : do not cause fetal abnormality because Neurologic problems Cellular casts from maternalanti-PLT ab. corticosteroid are inactivated by placental enzymes, usually improve with Nephrotic syndrome immunosuppressive tx Renal failure : recurrence in 1/3 of patients Hematuria † (with the exception of deficits resulting Hemolysis is usually responsive to high-dose GC from large infarcts) * resistant case may respond to splenectomy Leukopenia is common but is rarely associated A slow rise in serum Rapidly deteriorating renal function with infection : does not require treatment Cr. To 3mg/dL and active urine sediment require Severe thrombocytopenia with bleeding or Gastrointestinal : should be biopsied prompt aggressive tx without biopsy purpura should be treated with high-dose GC * If the platelet count fail to reach acceptable Lupus peritonitis (nausea, diarrhea, level in 2 weeks, cytotoxic drugs, cyclosporins, vague discomfort) If they fail danazole, and/or splenectomy should be considered. Pathology to respond Vasculitis of intestine ‡ (the most dangerous) LA is recognized by prolongation of PTT and : presenting with acute crampy abd. pain,Mesangial or mild focal proliferative nephritis maintain failure of added normal plasma to correct the prolongation vomiting, diarrhea,good renal function (do not require treatment) LA, aCL : thrombocytopenia, recurrent venous or arterial : Intestinal perforation (immediate surgery)Diffuse prolierative nephritis develop renal failure if untreated clotting, recurrent fatal loss, vascular heart disease. Pseudoobstruction (require tx with high-dose glucocorticoid and cytotoxic agents) LA is associated with hypoprothrombinemia or thrombocytopenia. (*surgery shoud be avoided unless frank*A high proportion of sclerotic glomeruli : suggest that these Bleeding may occur. obstruction is present) patients are unlikely to respond to immnosuppressive tx and *Bleeding syndrome usually respond to GC. Acute pancreatitis are candidates for dialysis ro trnasplantation but clotting syndrome do not. Elevated serum transaminase levels
2002년 다시 시작된 작업 ! Reticulocyte production index Risk factors for osteoporosis fracture Factors affecting peak bone mass Nonmodifiable Gender Fracture hx as an adult Race, Genetic factor, Gonadal steroids Hx of fracture in first degree relative Index ≥ 2.5 Growth hormone, Timing of puberty Index < 2.5 Female, Advanced age Caucassian race, Dementia Caclium intake, exercise Search bleeding focus and treat Hemolysis or Hemorrhage Modifiable IDA Anemia of chronic Current cigarette smoking, Alcoholism disease Low body weight, Impaired eyesight Peak Bone mass : at age of 30-35 years Hemorrhage Serum iron Estrogen def, Recurrent falls Findings of acute Serum total iron-binding capacity Low normal - or chronic blood Low calcium intake, Inadequate physical activity ※ If estrogen deficiency : Transferrin saturation Low normal - loss on history or Poor health physical examiantion IL-1, IL-6, TNF ↑ → osteoclast mediated bone resorption ↑ Serum ferritin Normal - MCV Serum soluble transferrin receptor No symptoms or Bone marrow iron stores signs of blood loss Iron-containing normoblasts Management of Type I osteoporosis : Estrogen ↓ ⇒ Ca resorption ↑ ⇒ PTH ↓ 1,25(OH)2D3 ↓ in the bone marrow [ General measures of hemolysis ] the fractures Type II osteoporosis : 1,25(OH)2D3 ↓ ⇒ intestinal Ca absorption ↓ ⇒ PTH↑ ⇒ Ca resorption ↑ Reduction in serum haptoglobin Differential diagnosis Hemolysis Presence of urine hemoglobin 2ndary causes (secondary osteoporosis) [ Further evaluation ] and/or urine hemosiderin Fracture Serum iron, TIBC, ferritin, IDA (severe) Increased LDH and serum Osteoporosis Hypogonadal state : Turner syndrome, Anorexia nervosa, Kleinfelter unconjugated bilirubin Endocrine dis: Cushing’s syndrome soluble transferrin receptor ; Vertebra fracture Anemia of chronic disease Drug induced osteoporosis upper lumbar vertabra : most common Hyperparathyroidism (± bone marrow iron stain) (some cases) Acquired + Thyrotoxicosis [ Further evaluation ] Hip fracture Thalassemia trait Immune hemolysis Colles’ fracture IDDM Hemoglobin electrophoresis, Low Direct Coombs’ test, : Early ambulation is recommended. Diagnosis Acromegaly (reticulocyte count may be elevated) -Autoimmune quantitation of red blood hemoglobin A2 and Adrenal insufficiency hemoglobin F levels, -Drug-induced cell surface antibodies, Sideroblastic anemia Nutritional and GI dis : Malnutrition globin DNA alalysis, -Alloimmune cold agglutinin titer (some cases) Parenteral nutrition globin chain systhesis ratios gastrectomy Lead poisoning (rare in adults) Traumatic (microangiopathic and FDA-Approved Indications for BMD tests Direct and indirect Reumatologic dis: RA, AS macroangiopathic) Hemolysis Coombs’ test, - TTP/HUS/HELLP Estrogen deficient women at clinical risk of osteoporosis Hematologic : MM. Lymphoma, leukemia quantitation of red blood - DIC cell surface antibodies, Verterbral abnormalities on X-ray suggestive of osteoporosis PLT count, WBC count with - Vasculitis cold agglutinin titier Glucocoriticoid treatment equivalent to ≥7.5mg of prednisolone, differential, BM aspirate and biopsy - Eclampsia or duration of therapy >3months Drgus Bone marrow aplasia/hypoplasia Primary hyperparathyroidism - Malignant hypertension : Glucocortuciud Renal function test Renal insufficiency - Prosthetic heart valves Monitoring response to an FDA-approved medication for osteoprosis Cyclosporin History and physical exam. - Arterial grafts of hypertension, pregnancy, Repeat BMD evaluation at >23-month intervals, or more frequently, Cytotoxic drugs BM biopsy with stains Pure red cell aplasia prosthetic heart valves or if medically justified. Anticonvulsants Hypersplenism vascular grafts, for collagen (trichrome stain) Myelofibrosis Excessive alcohol systemic vasculitis, and reticulin (siliver stain) Excessive thyroxine Myelophthisis Normal Membrane abnormalities neurologic changes, fever?; DEXA(BMD) : T score <-2.5 - Acanthocytes (spur cells) Schistocytes, anemia, and Aluminum destructive thrombocytopenia; Biochemical markers : monitoring the responsiveness Heparin BM aspirate and biopsy Myelodysplasia (most cases) - Echinocytes (burr cells) BUN/Cr; Urinalysis; (including Prussian blue DIC study to treatment GnrH agonist - PNH stain of iron), Anemia of chronic disease (most cases) - Thermal injury (burns) Bone formation markers Lithium00 karyotype analysis Acid hemolysis (HAM) or serum bone-specific alkaline phosphatase Mixed microcytic and macrocytic Infection sucrose hemolysis test, anemias flow cytometry analysis of serum osteocalcin - Malaria, Babesiosis, Iron deficiency (mild to moderate) Bartonellosis, Clostridia toxin GPI-anchored cell serum propeptide of type I procollagen surface proteins Bone resorption markers. Glucocorticoid induced osteoporisus Physiologic anemia Osmotic damage (e.g., CD55, 59) : inhibit osteoblast fuction, increase osteoblast apoptosis - Fresh water drowning stimulation of bone resorption, increase of urinary Treatment recommedations calcium loss impairment of the absorption of calcium (from National Osteoporosis Foundation) across the intestineSerum vit. B12 level, Inherited/Congenital : Bone loss – rapid loss first 6-12 months after start of Treatmenturine (± serum) methylmalonic 1. Women with no risk factors should begin therapy glucocorticoidacid level, RBC membranopathies Cobalamine (vit B 12 )def. if their T score is -2 or below; and Tx :Risk facotrs reduction, ExerciseBM aspirate, Schilling tests -Spherocytosis Folate def. -Eliptoctyosis 2. Women with risk factors should begin therapy Bisphosphonate (residronate) if their T score is -1.5 or below. Calcium (1000mg) + calcitriol (0.5 – 1.0µg)Red blood cell folate level, Treatment with drugs that interfere with -Stomatocytosis Calcitoninserum folate level, BM aspirate DNA sysnthesis and cell division RBC Enzymatopathies Prior cancer chemotherapy High -G6PD deficiency -Pyruvate kinase def. Myelodysplasia (some cases) Thyroid function test Hemoglobinopathies Pharmacologic treatment Hypothyroidism Risk factors -Thalassemias Liver function test Liver disease Reduction Antiresoptive drug Formation stimulator -Hemoglobin S,C,D,E HRT(ERT)† Lowdose intermittent PTH Exercise SERM Fluoride J&J Caclium : 1000=-1200mg of elemental caclium Anabolic steroid Bisphhosphonate (mnostly derivatives of testosterone Vit D 20µg or 800 IU primarly as antiresorptive agents J&J Calcitonin : might have analgesic effect may also stimulate osteoblst acitivity)
2003년 도전의 시작! ‘ 꿈은 매 우 구체적이 어 야한다!’1. 아마존닷컴 에 꽂힌 다.2. 하바드의 대 학생들이 우리 책으로 공부하게 한다.