内分泌英文病历讨论(学生版)

1、Case Discussi onA 35-year-old pregnant woman (gravida 2 胎次,para 1) was admitted to this hospital at 19 weeks and 6 days of gestatio n 怀孕期 because of the recent on set of hyperte nsion and diabetes.Three weeks before admission, at a routine prenatal visit, her blood pressure was 150/100(150 over 100)

2、 mm Hg(millimeters hydragyrim). On the same day she saw her primary care physicia n, who recorded a blood pressure of 172/102 mm Hg. The results of a physical exam in ati on were normal. Urinalysis showed glucose (4+). The results of other laboratory tests are shown in Table 1. The n ext day, the bl

3、ood pressure was 180/100 mm Hg. The blood glucose level 1 hour after the oral adm ini strati on of glucose (50 g) was 346 mg per deciliter ?desili:t?分升(19.2 mmol per liter). Treatment with labetalol 拉贝洛尔，glyburide 格列本脲(优降糖) ，and potassium p?si?m乍钾 suppleme nts was in itiated. The results of fetal ul

4、traso und exam in ati on were normal for the gestational age of the fetus ?fit? s. Three weeks later, despite increasing doses of labetalol, the patients blood pressure remained in the range of 180/110 mm Hg and her fasting blood glucose level ran ged betwee n 140 and 180 mg per deciliter (7.8 and 1

5、0.0 mmol per liter); the patie nt was admitted to the hospital.The patient had gained 6.8 kg in weight during the pregnancy. She had recently had polyuria and polydipsia and in creased facial puffin ess 虚胖;her complexi on 面色 was chroni cally ruddy 红润. She did not have headaches, proximal muscle weak

6、ness, bruising 挤压伤,flushing 激动脸红, abdominal pain, edema, palpitations 心悸 , diaphoresis ,dai?f?ri:sis发汗(sweat), edema, or cha nges in visi on. Her men ses had bee n regular before preg nancy, and she had had no difficulty conceiving 怀胎 with either this pregnancy or a pregnancy 3 years earlier, during

7、 which she had mild, diet-c on trolled gestati onal diabetes. She had bee n mildly overweight, with a body-mass index ( BMI) (the weight in kilograms divided by the square of the height in meters) of approximately 25 for several years. She did not smoke, drink alcohol, or use illicit ?l?s?t非法的drugs.

8、 She was married, with a 2-year-old daughter. Her mother and maternal m ?:?:nl母亲方面 的 grandmother had type 2 diabetes mellitus, and many family members had hypertension.Q1: What sthe possible cause of the patient? Give 3 or more diseases for hypertension duri ng preg nan cy.1. preeclampsia or eclamps

9、ia2. chr onic hyperte nsion probable diag no sis3. preeclampsia or eclampsia superimposed on chronic hyperte nsion4. gestati onal hyperte nsionWhen I saw this patient during her first admission, she had marked hypertension, poorly controlled diabetes mellitus, and hypokalemia. The four hypertensive

10、disorders that are recognized during pregnancy are preeclampsia ?pri?Kl?mpsi ?先兆子痫 or eclampsia ek?l?mpsi? 子痫惊厥,chronic hypertension (including essential hypertension and secondary hypertension), preeclampsia or eclampsia superimposed ?sju:p?rim?p/&zd on chronic hypertension , and gestational hypert

11、ension . Although this patient had proteinuria, it was not severe enough to warrant 正当理由 a diagnosis of preeclampsia; in addition, the onset of preeclampsia would be unlikely this early in the pregnancy. Gestational hypertension WOukLe unlikely this early in pregnancy. Thus, I was left with a probab

12、le diagnosis of chronic hypertension.In a patient with newly diagnosed chronic hypertension, the major question is whether it is essential hypertension or associated with another condition . A pregnant patient with chronic hypertension is at increased risk for superimposed preeclampsia, intrauterine

13、子宫 内的 growth restriction(grow slowly), abruption 分裂 placentae pl?会ent?胎盘(胎盘早剥 正常 20week to birth), premature birth, and perinatal ?peri?neitl围产期 death. Efforts to control blood pressure with labetalol or methyldopa 甲基多巴 to reduce the incidence of preeclampsia and its associated perinatal morbidity 发

14、病率 have been disappointing; thus, a search for a secondary cause in a case such as this is mandatory 必要的强制的 .In this patient, the presence of hypokalemia increased my suspicion that the problem was secondary hypertension.Q2: Did the patie nt have preexist ing, un diag no sed diabetes? Why?YesThe gly

15、cated hemoglob in value of 8.2% at 16 weeks and 6 days gestatio n led me to suspect that she had had hyperglycemia for some time before her preg nancy bega n.The broad definition of gestational diabetes includes the coincidental development of type 1 during pregnancy as well as the presence of preex

16、isting, undiagnosed type 2.Diabetes in PregnancyThis patient also had carbohydrate intolerance, with glycosuria at 16 weeks and 6 days gestation, as well as gestational diabetes (defined as carbohydrate intolerance of any degree of severity, with an onset or first recognition during pregnancy). The

17、glycated hemoglobin value of 8.2% at 16 weeks and 6 days gestation led me to suspect that she had had hyperglycemia for some time before her pregnancy began. The broad definition of gestational diabetes includes the coincidental 巧合的 development of type 1 during pregnancy as well as the presence of p

18、reexisting, undiagnosed type 2. The vast majority of patients who receive a diagnosis of gestational diabetes have a relatively mild degree of carbohydrate intolerance that develops late in pregnancy and is associated with the insulin resistance of pregnancy. It seemed fairly clear to me that this w

19、oman had preexisting, undiagnosed type 2 diabetes.The results of additional laboratory tests (Table 2) led me to suspect that she had Cushings syndrome and to request a consultation with an endocrinologist.Q3: What is keypo int in the n ext physical exam in ati on?BMI and weight gainblood pressure a

20、nd pulseedema (face orbital peripheral) extraocular moveme nts and visual fields thyroidsupraclavicular or dorsal adipose tissue, hirsutism, bruising 痤疮 abdome n striaeProximal muscle stre ngth and reflexesCushings Syndrome in PregnancyIn a case that is suggestive of Cushings syndrome, the goals are

21、 to confirm the presence of a pathologic excess of endogenous cortisol, to determine its source, and to remove the source to prevent illness and death. Both the diagnosis and management in this case were further complicated by the patients pregnancy. The diagnosis of Cushings syndrome in pregnancy i

22、s confounded by the normal hormonal and biochemical changes of pregnancy the management is confounded by the profoundly 极度的 leterious effect of hypercortisolemia on both mother and fetus, the side effects of medications, and the technical problems involved in undertaking surgical resection. The comp

23、lications of pregnancy for women with Cushings syndrome include hypertension, diabetes, preeclampsia, and infection. Fetal complications include prematurity and intrauterine growth retardation 延迟.This patient had no signs or symptoms of Cushings syndrome before pregnancy. Consideration of the diagno

24、sis of Cushings syndrome is typically based on clinical features. However, many features of this disease are similar to those of normal pregnancy, including weight gain, amenorrhea ei?men?:?无月经,striae 条纹,fatigue f?i q劳累,back pain, mood changes, and plethora ?ple r?过量过剩.In this patient, clinically si

25、gnificant hypertension and abnormal glucose tolerance were present; although these findings are common in Cushings syndrome, they are nonspecific. Objective signs favoring a diagnosis of Cushings syndrome such as weakness, particularly proximal weakness, spontaneoussp a n?teni?自然发生的 ecchymoseseki?m?

26、jsis瘀斑,and wide striae were not seen in this case, probably because the development of hypercortisolemia had been rapid. The single finding that appropriately prompted an evaluation of this patient for Cushings syndrome was unexplained hypokalemia.On physical exam in ati on, the patie nts weight was

27、 80 kg and height 170 cm, with a body-mass in dex of 28. The blood pressure was 180/100 mmHg, and the pulse was 88 beats per minu te; other vital sig ns were no rmal. Her face was slightly roun ded, there was mild per orbital 眼窝的 edema, and her facial complexion was ruddy. Extraocular ?ekstr?jul ?眼外

28、的 movements and visual fields were in tact 未受损伤.The thyroid was no rmal in size, with no palpable no dules. There was no in crease in supraclavicular 锁骨上的 or dorsal adipose 脂肪 tissue, hirsutism 多毛症,or evidenee of bruising 擦伤.The abdomen was gravid 怀孕的,nontender, and without striae. Proximal muscle s

29、tre ngth and reflexes were no rmal. There was no peripheral edema.Reiulti of Serum And Urine Cbemicil Twtt between WMki 6 nd6*VariablaNormat RangpValues in P両ntSodium (mmollirer)L1S-14S13$Fotinium3 I S2.6Chlofdf (iTirtnol/l4t#T)100-1OB99Cifbon din Kidc (mm： 11 ttr)23.0-31.93L2Um Httrofcn (mg/dl)1-2S

30、K0.6Glucotc70-110240Cikiitm15-10.59.2Protrirt6.0-K06.7Albumin (g/dl)LI J3.4Globulin fg/dl)2HJJAirline phqsphatiip (U/Mrr)3010040atttnolrdnslfcrdlf9-2512Alinmc a mine transferase 4U/htrr|7-JO22LKtit*dehyttrogiHAte (U/li(er)UCkJlC40Qilirubir (mg d )Total0-1,004Ccrijugited0-0.401Uf nary ratal proleH fr

31、rig 24 hr)0-115241hetnogiobin(?t|扎40.2* To convert the viluei for urea nilTogm a rrnlLmoles per liter, mulvpl/ by0. J57. To convert th乜far crejtminf to micromol#! per liter, multiplyb, 8$ 4. To convert ihe values fcr glucote to millimoles per liter multiply t O.OSSl To corid the 诃Ium for cikium to r

32、nillimolet per liter, mutbpljr by 0.250. To cornrert the Far total 4nd conjugated bdirubm (a micro- ctiqIuy ptr lilf仃 muhiply by 17,1.Q4: The n what is your diag no sis? Why?chronic hyperte nsiongestational diabetes (type 2 diabetes)hypokalemiaCush ing syn drome?Q5: Are there any other exam in ati o

33、ns we should take? Why?Confirmation of a pathologic excess of cortisol is based on one or more of three methods: assessme nt of total cortisol product ion over a 24-hour period with a determ in ati on of 24-hour urinary free cortisoldocumentation of the loss of normal diurnal variation in cortisol s

34、ecretion on the basis of a late-ni ght salivary cortisol measureme ntdocumentation of the loss of feedback inhibition of cortisol on the hypothalamic-pituitary -adrenalaxis with dexamethas one suppressi on testi ngTo rule out an aldoster on e-secret ing tumor as a cause of hypokalemiaaldoster one le

35、vel and elevated plasma renin activityto find the location of the lesion(adrenal, pituitary or ectopic)magn etic resonance imagi ng (MRI)to determ ine if the Cush in gs syn drome caused by excess corticotrop in or notan undetectable corticotropin level or a 9 a.m. level below 10 pg per milliliter wi

36、th a two-site immuno radiometric assayQ6: What is your prescription for this patient?Labetalol( un til the blood pressure became no rmal)硝苯地平in suli n and potassium suppleme ntati onIn this patient, rapidly escalating hypertension and insulin-requiring diabetes made surgical cure an immediate goal.

37、If surgery cannot be performed in a casesuch as this, the use of interim medical therapy to block cortisol production should be considered. In this case, metyrapone 美替拉酉同,a drug that blocks the conversion of 11-deoxycortisol 脱氧可的松 to cortisol, was used briefly while surgery was being scheduled. The

38、use of metyrapone during pregnancy has been reported in a few cases15,16,17,18; however, definitive surgery usually should not be delayed until the cortisol level is normalized. Other drugs more commonly used to treat hypercortisolemia, such as ketoconazole 酉同康唑,cross the placenta, inhibit progester

39、one production, and may be both teratogenic ?ter?t?u?dzfenik产生畸形 and associated with fetal loss.One week later, the patient was seen in the neuroendocrine clinic. The results of additional laboratory tests were showed in Table 2. A repeated 24-hour urinary cortisol measureme nt showed that the level

40、 was 1805 g. 卩Q7:How to confirm the prese nee of a pathologic excess of cortisol ?Confirmation of a pathologic excess of cortisol is based on one or more of three methods: assessme nt of total cortisol product ion over a 24-hour period with a determ in ati on of 24-hour urinary free cortisol, docume

41、ntation of the loss of normal diurnal variation in cortisol secretion on the basis of a late-ni ght salivary cortisol measureme nt, and docume ntati on of the loss of feedbackinhibition of cortisol on the hypothalamic -pituitary -adrenal axis with dexamethasone suppression testi ng. The biochemical

42、diag no sis of hypercortisolemia in preg nancy is complicated by two factors.Detecting Pathologically Excessive Cortisol ProductionConfirmation of a pathologic excess of cortisol is based on one or more of three methods: assessment of total cortisol production over a24-hour period with a determinati

43、on of 24-hour urinary free cortisol, documentation of the loss of normal diurnal variation in cortisol secretion on the basis of a late-night salivary cortisol measurement, and documentation of the loss of feedback inhibition of cortisol on the hypothalamic -pituitary -adrenal axis with dexamethason

44、e suppression testing. The biochemical diagnosis of hypercortisolemia in pregnancy is complicated by two factors. First, cortisol production rates markedly increase during pregnancy, so that urinary free cortisol levels in the second and third trimesters may overlap with levels seen in Cushings synd

45、rome. Second, levels of corticotropin 促肾上腺皮 质激素 rise despite increasing cortisol levels, which is consistent with the occurrence of decreased feedback on corticotropin secretion. Therefore, unless cortisol levels are markedly elevated, the results of these tests may be difficult to interpret.In heal

46、thy people, cortisol secretion peaks in early morning and reaches a nadir as midnight approaches; the difference in cortisolsecretion between such people and those with Cushings syndrome is maximal at approximately 11 p.m.4 However, although this difference provides an excellent screening test for C

47、ushings syndrome, normal late-night salivary cortisol levels are not well established during pregnancy.Low-dose dexamethasone suppression testing can be used to diagnose Cushings syndrome despite the occurrence of false negativeresults. During pregnancy, however, false positive results can occur.Pos

48、sible reasons for false positive results include an estrogen-inducedelevation in the cortisol-binding globulin; the impaired suppressibility of the hypothalamic -pituitary -adrenal axis; placental production ofcorticotropin and corticotropin-releasing hormone, which is not regulated by negative feed

49、back control; tissue refractoriness toglucocorticoids; and possible antiglucoc次rticoid effects of progesterone.4g 2In this case, urinary free cortisol levels that were more than 1000g above the upper limit of the norm4-houg e during two 2periods in the absence of glucocorticoid therapy clearly estab

50、lished the diagnosis of Cushings syndrome. The hypokalemia reflected the magnitude of this excess cortisol. The normal aldosterone level and elevated plasma renin activity were consistent with pregnancy, ruling out an aldosterone-secreting tumor as a cause of hypokalemia.Q8: How to determ ine the so

51、urce of a pathologic excess of cortisol?Corticotrop in-i ndepe ndent Cush in gs syn drome is due to an adre nal lesi on, whereas the corticotropin-dependent form of the disease can be traced to either a pituitary 垂体的 or an ectopic source.In Cush in gs syn drome caused by excess corticotrop in from a

52、ny source, corticotrop in levels are typically in the no rmal range which is in appropriate give n the level of cortisol or they areelevated. In corticotrop in-i ndepe ndentCush in gs syn drome, corticotrop in levels should besuppressed.In the sett ing of hypercortisolemia, an un detectable corticot

53、rop in level or a 9 a.m. level below 10 pg per milliliter (2 pmol per liter) with a two-site immuno radiometric 免疫放射测定assay iscon sidered to be suggestive of corticotrop in-i ndepe ndent Cush in gs syn drome.Determining the Cause of Excess Cortisol ProductionOnce pathologic hypercortisolemia has bee

54、n identified, the next step is to determine whether the hormone excess is corticotropin-dependent. Corticotropin-independent Cushings syndrome is due to an adrenal lesion, whereas the corticotropin-dependent form of the disease can be traced to either a pituitary 垂体的 or an ectopic source. The most c

55、ommon cause of endogenous Cushings syndrome is overproduction of corticotropin by a benign pituitary corticotropic tumor. The vast majority of such tumors are microadenomas (1 cm in diameter); approximately one third are too small to be visualized on sensitive high-resolution magnetic resonance imag

56、ing (MRI). Ectopic 易位的 Cushings syndrome is caused by a neoplasm outside of the pituitary gland that produces corticotropin or, in rare cases, corticotropin-releasing hormone. Many tumors have been reported to cause ectopic Cushings syndrome,In Cushings syndrome caused by excess corticotropin from a

57、ny source, corticotropin levels are typically in the normal range which is inappropriate given the level of cortisol or they are elevated. In corticotropin-independent Cushings syndrome, corticotropin levels should be suppressed. In the setting of hypercortisolemia, an undetectable corticotropin lev

58、el or a 9 a.m. level below 10 pg per milliliter (2 pmol per liter) with a two-site immunoradiometric 免疫放射测定 assay is considered to be suggestive of corticotropin-independent Cushings syndrome. However, because corticotropin levels may be higher in pregnant women than in nonpregnant women, this measure may be misleading. Therefore, unless corticotropin levels are lower than normal, a corticotropin-dependent tumor may be erroneously diagnosed.In this case, the corticotropin level of 3 pg per millilite