Microsoft Word - 内分泌・代謝分野.doc
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- ゆめじ やたけ
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1 TRH, TRH TSH, a. GH, PRL b.acth, FSH, LH, TSH GH PRL Kallmann KAL LHRH Laurence-Moon-Biedl AR Frohlich
2 Prader-Willi Hypotonia Hypogonadism Hypomentia Obesity Sheehan DIC T4 GH 80% Laron GH IGF-I GH GH acromegaly) GH
3 J-15 GH 10 ng/ml 5 ng/ml double floor ballooning heel pad thickness GH IGF-I -C IGF-I GH GH TRH, GnRH, CRH GH GH GH GH IGF-I P Ca FSH, LH Hardy II Chiari-Frommel Argonz del Castillo Frobes-Albright
4 10 mm MRI T2 10mm, anorexia nervosa 30-20% GH T3 reverse T3 low T3 T.Chol ADH ADH DI; diabetes insipidus)
5 ADH DI MRI T1 DI ADH ADH ADH receptor Ca, Li, K Na Na, Na, SIADH ADH ADH ADH Na K T3, T4, rt3 T3 80% T4 low T3
6 Basedow Af) Stellwag Mobius Grafe TSH C, C,C Chol CK-MM ChE TSH-R R ) R ) ChE ChE Plummer I123- hot nodule
7 C, C, L Chol CK-MM LDH TSA T3, T4 17-OHCS, 17-KS TSH T4 ESR,CRP CRP, ESR
8 CK-MM Chol LDH 70-80% PBC RA, SjS PBC B-42 SjS SS-A, SS-B F-69 T4 R ) Schmidt Addison TSH C
9 CEAMEN2A,2B MEN1 (PPP) Parathyroid, Pituitary, Pancreas MEN2A (PTA) Parathyroid, Thyroid, Adrenal MEN2B (NTA) Nerve, Thyroid, Adrenal 1,2 PPPTA PTA) 1 Ca Ca PTH V.D 3 ) PTH V.D 3 Ca, P PTH Ca P V.D3 Ca P Ca P Ca (ADH ADH Ca Li K Ca, P Ca, P QT Ca PTH camp II RTA PTH HCO3 K, P,Cl
10 K, P II RTA PTH II RTA I RTA I I RTA PTH PTH II RTA I RTA ALP3 MEN I MEN II I HAM HAM PTH Addison Ca PTH Ca, P
11 II ACTH K ACTH ACTH 17-KS 17-OH DHEA 17-OHP DOC 11- OHCS ACTH OHCS I II ACE
12 JG apparatus ADH ACTH, GH Cushing Cushing ACTH microadenoma) Ca Na K K 2mg) Cushing 8mg) 17-OHCS Cushing ACTH MRI/CT
13 17KS ACTH MRI/CT ACTH Cushing CRH ACTH Nelson ACTH ACTH Barter-Gitelman Barter Liddel 17-OHCS, 17-KS Na K Ca K Li K Barter Na-K-2Cl
14 Gitelman Mg Ca Na-Cl Barter Liddel Na Addison Schmidt Addison
15 ACTH Addison Basedow Waterhouse-Friderichsen PC; pheochromocytoma 5H disease: Hypertension Hypermetabolism Hyperglycemia Headache Hyperhydrosis VMA, 17-OHP
16 MEN1 (PPP) Parathyroid, Pituitary, Pancreas MEN2A (PTA) Parathyroid, Thyroid, Adrenal MEN2B (NTA) Nerve, Thyroid, Adrenal 1,2 PPPTA PTA) 1 Zollinger-Ellison Langerhans 80% K Ca PTHMEN1 ZE Wipple's triad 50mg/ml hypervasculr
17 5-20 U/ml triad WDHA Watery Diarrhea, Hypokalemia K, Achlorhydria GHACTH) DM DM
18 DM DM ICA: islet cell antibody GAD 1 DM MODY 75% C-1 20 g/day 200 mg/dl FBS (fasting blood sucrose) 126 mg/dl 75gOGTT (oral glucose tolerance test) 200mg/dl HbA1c 6.5% 75gOGTT HbA1c %) DM DM 25
19 25kcal/kg 30kcal/kg 35 kcal/kg (kcal/day) 1000+(age-1)x kcal FBS>250mg/dl HbA1c>9%) SU BG MODY: maturity-onset diabetes of the young) 25 DM DM DM 1 DM DM DM
20 Kussmaul ph HCO3 BE PCO2 0.9% K K HCO3 GI glucose-insulin K DM 0.45% DKA K K Ca GI Ca
21 330mOsm/l 4ml x (kg) x (%) ml/kg) K
22
23 I, III, IV, VI, VIII II, III, V, VII I von Gierke I, III II Pompe Wilson IV I GAG) Hurler, Hunter, Morquio Hunter Hunter Morquio
24 TG TC: TG: 150 LDL: 160 TC>220, LDL>160, TG>150 CM, VLDL, IDL, LDL, HDL origin, pre-, pre- +, origin, pre =LDL pre V CM, VLDL, IDL, LDL, HDL TG Chol B-100 B-100 B-100 E E E C C A-1 CM VLDL IDL LDL HDL HDL-Chol HDL
25 TC>220, LDL>160, TG>150 HDL 40 R ) TG IIa IIb III IV V I LDL, LDL VLDL, IDL, VLDL, VLDL CM, CM C C,T C,T T T T PBC IIa)IV CM VLDL TG LDL C LDL: VLDL: CM: Friedewald LDL=TC-HDL-1/5TG AIP) -ALAPBG
26 SIADH Wilson AR Kayser-Fleischer Fanconi Wilson D- Menkes Cu Cu Cu Wilson, Menkes, PBC Wilson Cu Cu Menkes Cu Cu PBC Cu Cu 25%) Wilson
27 CT MRI (T2) low PRPP HGPRT de novo salvage de novo PRPP salvage HGPRT 3-7 mg/dl; mg/ml 8 mg/dl Ca ACE Lesch-Nyhan HGPRT
28 J Hz; pill rolling) Wilson base excess (B.E. 0 2 meq/l anion gap (AG) = [Na + ]-([Cl - ] +[HCO 3- ]) 12 2 meq/l [HCO - 3 ] 24 2 meq/l Ca 8-10 mg/dl K4 meq/l
29 AG KUSSMAL K DKA U uremia S S sepsis M A L lactic K K T QRS K T U Ca QT Ca QT K Ca GI Ca Na Na CPM; central pontine myelinolysis) Na Ca Mg Ca hyperventilation ADH Ca, Li, K Ca Na Na DEKA A: D:
30 E: K: II, IX, VII, X B1 Wernicke B2 B6INH B12 C:
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Clinical Question 2017 年 5 月 15 日 分野 : 内分泌テーマ : 治療 19 14 15 18 sick contact1l 170cm/68.8kg, GCSE1V1M5, HR: 120/min, BP: 104/60mmHg, RR: 30/min, SpO 2 : 96%, BT: 38.0 WBC Hb PLT CRP Cr BUN 23400 15.0 35.5
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