【Yen-Ju Chu】點評問題和點評內容

用戶【Yen-Ju Chu】點評問題和點評內容

【評論主題】99. 下列關於過敏性鼻炎的敘述何者錯誤？(A)是盛行率最高的慢性過敏性疾病。(B)之後罹患氣喘的風險至少增加兩倍。(C)常伴隨著過敏性結膜炎發生。(D)輕度持續性過敏性鼻炎的治療以第一代抗組織胺為主

【評論內容】B: Childhood AR is associated with a 3-fold increase in risk for asthma at an older ageD/E:過敏性鼻炎的治療:. 減少接觸過敏原. 口服抗組織胺, Second-generation antihistamines are preferred because they cause less sedation . Intranasal corticosteroid is need in patient with persistent, severe symptoms. Pseudoephedrine 與 infant mortality 有association, 部分廠商建議小於4歲避免使用 . Intranasal decongestants 建議使用不超過5天, 每個月最多用1次, 以避免 rebound nasal congestion. Cromolyn sodium nasal spray: 抑制mast cell degranulation, 有效但需q4h使用. Anticholinergic nasal spray(ipratropium ...

【評論主題】98. 若氣喘病患之個人最佳尖峰呼氣流量(peak expiratory flow)為300L/min，則其綠色區域是指呼氣流量為多少 L/min？(A)< 150。(B)150--180。(C

【評論內容】Classification of asthma control:FEV1 or Peak flow:

【評論主題】94. 小明罹患全身性青少年性類風濕性關節炎(systemic type Juvenile Idiopathic Arthritis) ，長期規則性於小兒風濕科門診追蹤，且情況穩定。最近因發燒已五天以

【評論內容】Elevated ANA titers are present in 40-85% of children with oligoarticular or polyarticular JIA, but are rare with sJIA

【評論主題】93. 關於Wiscott-Aldrich症候群之敘述下列何者為非？(A)屬於性聯隱性遺傳(X-linkel recessive)。(B)血液抹片可見微小血小板(Microplatelet)。(C)有

【評論內容】Wiskott-Aldrich syndrome:Genetic: X-linked recessive disease mutation on Xp11.22-11.23Manifestation Atopic dermatitis Thrombocytopenic purpura with normal appearing megakaryocytes but small defective platelets Undue susceptibility to infection.Onset Prolonged bleeding from the circumcision site or bloody diarrhea during infancy. Atopic dermatitis and recurrent infections usually develop during the 1st yr of life.Immunodeficiency: Uniformly impaired humoral immune response to polysccharide antigens. Reduced T cell percentage, depressed lymphocyte response to mitogens. Variab...

【評論主題】89. 大細胞貧血(macrocytic anemia)，且網狀紅血球高的疾病？(A)葉酸缺乏。(B)溶血性貧血。(C)Vit. B12缺乏。(D)甲狀腺功能下。(E)再生不良性貧血。

【評論內容】多數macrocytic anemia都是紅血球製造不足, low/inadequate reticulocyte

【評論主題】88. 有基因異常疾病的人,比較好發癌症，下列那一項應排除在外？(A)唐氏症。(B)第一型纖維神經瘤病。(C)骨質石化症。(D)嚴重複合型免疫缺乏症。(E)Li-Fraumeni 症候群 (p53 g

【評論內容】Down syndrome: ALL, AML, Transient myeloproliferative syndrome (Nelson 20ed)NF-1: Optic pathway gliomas, astrocytoma, malignant peripheral nerve sheath tumors, neurofibromas(Nelson 20ed) pheochromocytoma, rhabdomyosarcoma, leukemia, Wilms tumorNF-2: Vestibular schwannomas, meningiomas, spinal cord ependymoma, spinal cord astrocytoma, hamartomas(Nelson 20ed)SCID: EBV induced lymphoproliferative disease, non-Hodgkin lymphoma(74%), Hodgkin lymphoma, leukemia (uptodate)Li-Fraumeni syndrome(p53 germ line mutation): Astrocytoma, primitive neuroectodermal tumor (Nelson 20ed , Table 497-1...

【評論主題】87. 一位七歲大男童患有遺傳性球型紅血球症(congenital spherocytosis)，最近三日來感覺精神不佳。理學檢查發現心跳每分鐘190下，呼吸每分鐘50下，臉色十分蒼白。實驗室檢查顯

【評論內容】Children with HS are susceptible to aplastic crises, a result of parvovirus B19 infection and hypoplastic crises associated with various other infections.

【評論主題】86. 足月產新生兒第一天血液檢查結果如下: RBC 4.95x106/mm3, Hb 14.6g/dl, MCV 88fl, RDW 14.8%, HbA 8.5%, HbF 85%, Hb Bar

【評論內容】Alpha thalassemias:Carrier : −,α/α,α ; Newborn: 1-2% Bart(gamma_4) , adult: normal ; no anemiaTrait(minor) : −,α/−,α −, −/α,α ; Newborn: 5-10% Bart(gamma_4) , adult: normal ; mild anemiaHbH(intermedia): −,−/−,α ; Newborn: Bart: 20-30% , Adult: HbH(beta_4) ; mild anemia, transfusions not requiredConstant srping : −,−/α,α^CS ; 2-3% constant spring, 10-15% HbH(beta_4) ; moderate to severe anemia, transfusion, splenectomyMajor : -,-/-,- ; Hb Barts(beta_4), Hb Portland(del...

【評論主題】79. 有關先天性心臟病－心房中膈缺損的陳述，何者為非？(A)雖然卵圓孔開放(Patent Foramen Ovale)在超音波心圖上，可發現由左心房至右心房分流，但是不屬於心房中膈缺損。(B)在成年

【評論內容】PFO left to right shunt除了newborn時期以外不常見，在左心房volume load或壓力上升時可能出現

【評論主題】78. 6歲男孩，主訴運動時稍喘且有發紺(cyanosis)現象。身體理學檢查時，發現於左上胸有一2-3度收縮噴射心雜音合併固定且寬的第二心音。其胸部X光如下，下列何者為最可能診斷？ (A)總肺靜脈回

【評論內容】Supracardiac TAPVR = Snowman signIncreased RA preload = pulmonary systolic ejection murmur(left upper sternal border), wide and fixed S2

【評論主題】75. 關於Long QT syndrome之敘述，下列何者不對？(A)corrected Q-T interval > 0.42秒即能斷定。(B)常因polymorphic ventricul

【評論內容】Diagnosis of LQTS is based on ECG and clinical criteria

【評論主題】73. 王小弟今年11歲，人中較長、上嘴唇明顯與嘴巴較大，他爸爸說他從小被診斷有supravalvular aortic stenosis，雖有智能不足 (IQ =56)，在學校卻喜歡講話且人緣很好，

【評論內容】Ans: EWilliam syndrome7q11.23 deletion, autosomal dominent , 大部份是偶發的CHD: elastin defect, 血管狹窄: aortic valve stenosis or pulmonary valve stenosisFacial dysmorphism: 眼皮腫、鼻尖朝上、人中長、嘴寬、厚唇、下巴小等。 Neonatal hypercalcemiaHypotoniaTooth: 牙齒小、牙縫大、排列不整齊、咬合差。 Feeding difficulty: 嬰兒期吸吮力弱、吞嚥不協調、餵食困難、容易嘔吐、便祕及腹絞痛Hernia: 腹股溝或臍疝氣的機率較一般正常人為高。 Eye: 斜視、遠視、星狀虹膜等。 ENT: 耳朵對某些頻率的聲音敏感，甚至會感到刺耳或痛苦。較易罹患中耳炎，有的則影響聽力。Kidney：結構異常、結石或腎功能障礙。 Thyroid: 少數...

【評論主題】72. 您在病房看到一位一個月大之法洛式四重症病嬰，合併有胸腺發育不全、顎裂、窄鼻、及低血鈣；心臟科醫師請身為總醫師的您向家屬解釋病情並作遺傳諮詢，以下何者是正確的、您該說明的事項？(A)目前只有以自

【評論內容】Digeorge syndrome( 22q11.2 deletion)dysmorphogenesis of the 3rd and 4th pharyngeal pouches典型的臉型明顯的鼻及鼻樑基部寬大、人中短、上唇薄、耳廓異常、外耳小且有凹窩、眼距寬等。另外85%患者有顎裂、且常見叉型懸庸垂及聽力障礙。心臟方面主要是Conotruncal cardiac defect ，包括：心室中隔缺損、右側動脈弓、法洛氏四重症、動脈幹、主動脈阻斷或肺動脈發育不良。

85%患者有多重的心臟異常，最常見的是B型主動脈阻斷及動脈幹。

內分泌方面副甲狀腺低下造成低血鈣，有時亦有甲狀腺低下的情形。免疫方面胸腺發育與否分成：

1.部分DiGeorge症候群佔大多數

2.完全DiGeorge症候群因胸腺完全無發育，故T細胞嚴重缺乏，易引起伺機性感染（如黴...

【評論主題】70. 五個月大的小妹妹因肺炎入院，理學檢查發現心臟擴大、低張力與肝腫大，出生史為正常，家族史發現病人之一姐一兄均於嬰兒時期因心臟疾病死亡，可能之診斷為何？(A)Mucopolysaccharido

【評論內容】Ans: CMPS: defect of Glycosaminoglycan(GAGs) metabolism, type I-VII(有些type沒有以下部分症狀)type I: α-L-iduronidaseMental deficiency, coarse facial features, corneal clouding, visceromegaly, short stature, joint contractures, dysostosis multiplex, leucocyte inclusions, mucopolysacchariduriaFabry disease: X-linked , defect of lyposomal glycosphingolipid metabolism by alpha-galactosidaseMale with classic phenotype: angiokeratomas, hypohidrosis, corneal and lenticular opacity, acroparethesias, vascular disease of kidney, heart and brain at advaced ageHeterozygous female: corneal opacities, is...

【評論主題】67. 關於兒童腦性麻痺，下列何種原因是導致雙側下肢麻痺型 (diplegia) 最常見的原因？(A)先天性巨細胞病毒感染 (congenital cytomegalovirus infection)

【評論內容】Classfication of cerebral palsy and major causesMOTOR SYNDROME (APPROX. % OF CP)NEUROPATHOLOGY/MRIMAJOR CAUSESSpastic diplegia(35%)Periventricular leukomalaciaPeriventricular cysts or scars in white matter, enlargement of ventricles, squared off posterior ventriclesPrematurityIschemiaInfectionEndocrine/metabolic (e.g., thyroid)Spastic quadriplegia (20%)Periventricular leukomalaciaMulticystic encephalomalaciaCortical malformationsIschemia, infectionEndocrine/metabolic, genetic/developmentalHemiplegia (25%)Stroke: in utero or neonatalFocal infarct...

【評論主題】66. 下列何者有關腦發展異常(CNS malformation)的描述不正確？(A)平腦迴常合併點頭癲癇。(B)腦裂(Schizencephaly)可能不會抽搐。(C)myelomeningocel

【評論內容】Chiari malformation:Chiari I: downward dysplasment of cerebellum and cerebellar tonsilChiari II(Arnold-Chiari): Associated with myelomeningoceleChiari III: meningoencephaloceleChiari IV: hypoplasia of cerebellumBetween 35 and 85 percent of children with classic lissencephaly develop infantile spasms in the first year of lifeSchizencephaly:Bilateral, open-lip : severe seziure Unilateral, closed-lip : hemiparesis, motor delayPeriventricular nodular heterotopia: If without other anormaly: seizures and learning problems are common, while more severe developmental problems are not. When mi...

【評論主題】63. 有關吉巴氏症 (Guillain-Barre syndrome)，何者敘述錯誤？(A)由下而上肢體無力發作，較為常見，但也可由上而下發作。(B)可能有感覺異常或左右不對稱性發作。(C)少數侵犯

【評論內容】Guillian-Barre syndrome 有四種subtype

【評論主題】61. 關於嬰兒的細動作發育，請問嬰兒會將物體從一隻手交換到另一隻手的發育平均年齡是？(A)4個月大。(B)5.5個月大。(C)7.5個月大。(D)9個月大。(E)10.5個月大。

【評論內容】5–6 months

【評論主題】60. 正常三歲大的小朋友，她(他)的發展里程應會出現下列何者？(A)單腳往前跳。(B)會說出四種顏色。(C)會畫三角形。(D)會畫正方形。(E)會說出年齡或性別。

【評論內容】單腳跳 : 4歲

【評論主題】59. 注意力不足過動症的診斷標準，下列何者為非？(A)以SNAP量表中，症狀持續出現至少六個月。(B)某些症狀在七歲之後才出現。(C)某些症狀須在大於或等於兩種情境下出現。(D)症狀必須有明顯證據顯

【評論內容】DSM-V diagnostic criteria

【評論主題】57. 愛滋病孕婦與疑似母子垂直感染愛滋病的新生兒 (愛滋寶寶)，在照顧上應該注意的事項，以下何者為非？(A)我國全面建議孕婦知情後同意進行愛滋病篩檢，來避免母子垂直感染。(B)若新生兒為沒有產前檢查

【評論內容】(D) : zidovudine 治療 6周

【評論主題】55. 一位罹患全身紅斑性狼瘡的12歲女童，目前已接受prednisolone 5 mg qid治療3個月。2週前同住的祖父被發現罹患開放性肺結核，該女童出生時接種過一劑卡介苗。女童所做的結核菌素測驗

【評論內容】