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2015年10月31日 星期六

[醫如往常] Junior Clerkship Day 38: Ileal atresia

20151026

Morning Meeting: Polyhydramnios & Ileal atresia

R2 林醫師 A曹醫師

Symptoms & Signs

Polyhydramnios (20-35%)
Orogastric bile
Bilious vomiting
Abdominal distension



UGI (吳醫師補充)

1:3 Urografin enema 避免 induced NEC 
Microcolon:五種疾病
MIMCA
M: Meconium
I: Ileal atresia
M: Megacystic
C: colon atresia
A: aganglionic Ileal atresia


UGI 彭醫師

Suspect Meconium plug syndrome
Defecation 後排除

CVC culture cons

Staphylococcus
給Vancomycin

外科林醫師開腹手術

Lig. Trazi 開始都是脹的
Ileal atresia type 3a

推測可能是Meconium plug induced
破掉之後癒合後造成Ileal  atresia


一些比較

Real Meconium plug 是白色的
這次排出來的沒有那麼白,比較偏向Ileal  atresia

  • Meconium peritonitis可以找到Calcification
  • Meconium plug主要在Colon
  • Meconium atresia主要在Small intestine
Iliealcecal valve的保存與預後有關(Small bowel syndrome)


黃主任補充:

最近流行Enterovirus D68
NHI給付腸病毒做一次PCR


Fever

High fevers between 103 F (39.4 C) and 106 F (41.1 C)

Infants


  • < age 3 months and has a rectal temperature of 100.4 F (38 C) or higher.
  • Between ages 3 to 24 months and has a temperature up to 102 F (38.9 C)
  • A newborn and has a lower than normal temperature — less than 97 F (36.1 C).

2015年10月24日 星期六

[醫如往常] Junior Clerkship Day 29: Pediatrics; lymphadenopathy; Thalassemia; Neonatology

20151013 lymphadenopathy in Children
BCG: axillary node, neck nodeFever persist >5 days Kawasaki diz

PE 記得vital sign

Dental problem

Neoplasm pain: nerve infiltration
Neoplasm ipsilateral
Adhesion: scm 拉著跑
Lymphoma不只一顆 其他可能是reactive hyperplasia


Malignancy 驗
LDH
Ca
p
k
Uric acid

Lymphoma is most common malignancy that presents as a neck mass in children.

Dental problem

Deep neck infection
Rotation severe pain
Or cannot rotation
嘴巴打不開: 咀嚼肌,nerve
Dysphagia without oral ulcer, dysphonia

Saliva
Drainage, max abx
Contrast CT deep neck infection


Viral:

diffuse bilateral, fixation, soft
Liver function
EBV test


Bacteria:
single big

Streptococcus viridans: 口水菌

DM kp gram negative

Neck mass with air tract TB: Tense and big


NTM smear, and culture
HIV

Rickettsia立克次
Plasma

Autoimmune
Juvenile rheumatic
Kawasaki 5 sign


Kikuchi-Fujimoto disease (SLE spectrum)
Cervical and supraclavicular adenopathy, fever, fatigue, weight loss, anemia, and leukopenia

Castleman disease
Fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia

Drugs
  1. Aspirin
  2. Allopurinol
  3. Atenolol
  4. Barbiturates
  5. Captopril
  6. Carbamazepine
  7. Cephalosporins
  8. Gold
  9. Hydralazine
  10. Iodides
  11. Isoniazid
  12. Phenytoin
  13. Pyrimethamine 
  14. Primidone
  15. Phenylbutazone
  16. Phenobarbital 
  17. Penicillin
  18. Quinidine
  19. Sulindac 
  20. Sulfonamides
  21. Tetracycline


Approach to Masses of the Head and Neck
Kristi Chang, MD Associate Professor
Department of Otolaryngology-Head and Neck Surgery
University of Iowa Hospitals and Clinics
Neck mass etiologies (History)
Age
Peds ~ 80-90% benign;
     Inflammatory disease is most common cause of neck masses in children • Cervical adenitis

Adult ~ 80% neoplastic    (except thyroid masses)
80% of those are malignant (except thyroid and parotid masses)
LOCATION
New lateral neck mass in an adult > 40 yrs old is likely to be malignant.
Painless neck mass: UADT (upper aerodigestive tract) cancers
Duration: timing of onset –eg present at birth?
          > 2-4 wks
Growth pattern: worrisome if growing
Pain
Recent infection : cervical lymphadenitis more likely
Other similar masses?
Exposures: sick contacts, travel, pets/animals , h/o TB
Prior trauma or surgery in that area

Associated symptoms?
 Dysphagia
 Odynophagia/sore throat
 Hoarseness
 Otalgia
 Dyspnea/stridor
 Constitutional symptoms
fever/chills
fatigue
weight loss
night sweats

flushing
palpitations
elevated BP

* Must r/o malignancy in any patient with facial or parotid mass
with an associated facial nerve weakness or paralysis.

Long, et al. Principles and practice of pediatric infectious disease, 2 nd  ed. 2003
Torsiglieri , et al. Int J Pediatr Otol 1988;16(3);199-210
Dulin, et al. Am Fam Physician 2008;78(9);1097-98

Lymphadenopathy in children: refer or reassure?
David King, Jagadeesh Ramachandra and Daniel Yeomanson
Arch Dis Child Educ Pract Ed 2014;99:101–110. doi:10.1136/archdischild-2013-304443


Thalassemia 

Prof. Lin

北賽普勒斯不能進南部賽普勒斯Republic of Cyprus
要飛伊斯坦堡->雅典->南賽普勒斯
賽普勒斯可以進北賽普勒斯

骨髓移植:是目前唯一能完全治癒 thalassemia 的方法
TW 1984第一例
越南 1995第一例
七歲以前做就不會長不高
(至於為何 thalassemia 患者身高會矮?可能是由於大量輸血後過多鐵沉積於pituitary gland,破壞其功能。或者是嚴重貧血而輸血仍不足以平衡其貧血的狀態。)

免疫抑制劑Cyclosporine 眉毛粗 
J Dermatol Sci. 1994 Jul;7 Suppl:S47-54. Hair growth-stimulating effects of cyclosporin A and FK506, potent immunosuppressants.Yamamoto S1, Kato R.
"選擇性的immuniphilin ligands如cyclosprin A(CsA)及FK506,它們不僅是免疫抑制劑(immunosuppressants),也能調節頭髮的生長。在老鼠的身上局部使用CsA及FK506可以刺激頭髮的生長,也能抑制因cyclophosphamide引起的掉髮6。在另一個試驗中,局部使用CsA可以預防老鼠因合併etoposide、cyclophosphamide、cytarabine及doxorubicin引起的掉髮11。Cyclosporine機轉不明,但被認為可能藉由抑制p-glycoprotein做為在化學治療期間的毛囊角質細胞抑制劑(keratinocytes against),也會增加IL-1受體的表現,因此也許可以增加預防掉髮的能力。"
Ref. 化療引起的掉髮
奇美醫院柳營分院藥劑科藥師 張雅筑、鄭淨黛、蘇郁仁、陳憲煜
http://www.taiwan-pharma.org.tw/JTP/100/066-071.html


原住民海洋性貧血調查

MCV調查



Neonatology: The High Risk Newborn 

Prof. Hsieh

新生兒的定義爲出生一個月以內的嬰兒。 
周產期(Perinatal period):從懷孕滿 28 周到出生後七天。 
產下早產兒或低出生體重新生兒的比率為:原住民>本籍配偶>外籍配偶



2015年10月23日 星期五

[醫如往常] Junior Clerkship Day 37: AHT: Abusive head trauma

20151023 Morning meeting
OHCA; Out-of-Hospital Cardiac Arrest
ROSC: Return of spontaneous circulation
Doll eye sign; Oculocephalic reflex在腦幹正常的病患可觀察到眼球水平向右移動。若腦幹已經受損,則眼球不會移動,或是移動不對稱。

Brain echo→Subdural injury

Shaken baby syndrome改名→
AHT: Abusive head trauma/injury
  • Usually<3y/o
  • Brain 含水量較多
  • Bridging vein tear
  • Multiple layers Retinal hemorrhage 60-85%重要sign
  • No visible Trauma 69%
  • Mortality 比unintentionally高
CT: Subdural injury
軟硬相接處
Vein - sinus

Retinal hemorrhage
pattern很特殊Ophthalmologist 看比較準
Skeletal survey
Fractures

Life long injury
Coma 29%
Cerebral palsy
Vegetation

113線上通報家暴
https://ecare.mohw.gov.tw/


司法相驗才能開立死亡診斷書






2015年10月22日 星期四

[醫如往常] Junior Clerkship Day 36: Henoch-Schönlein purpura, Child seizure

20151022Morning meeting: Henoch-Schönlein purpura

1.  過敏性紫斑症,是一種 vasculitis syndrome
2.  主要的症狀有三種,為此病的 clinical triad
(1)  Abdominal pain:由於mesenteric artery ischemic所產生的劇痛,
又可能伴隨 vomiting 和 bloody stool。
(有可能會痛到被認為是盲腸炎,結果小兒外科抓去開刀,隔天冒出了不會痛的疹子,才發現是此病) ischemic pain
(2)  Skin rash:在下肢和臀部有「壓了不會退」的疹子,代表是因血管炎而起
(3)  Arthritis
3.  How to rule out:
(1)  病史詢問時並沒有問到曾發生過痛到不行的肚子痛。
(2)  PE  時下肢也沒有出現壓不退的出血點。
(3)  以上兩個都是 HSP  在小孩發生時常見的症狀,因此若沒有的話則 HSP  的可能性不高。

Dr. Chen (intern)
秋冬常見
男多於女
也許跟infection有關
Unblemished, macule, big, purpura
Intussusception比較特別是小腸套小腸

Diagnostic Criteria

American College of Rheumatology, 1990

Presence of two or more of the following:
• Palpable purpura without thrombocytopenia 
• Patient 20 years or younger at disease onset 
• Bowel angina (diffuse abdominal pain or diagnosis of bowel ischemia)
• Biopsy showing granulocytes in the walls of small arterioles or venules

International Consensus Conference, 2006

Palpable purpura in the presence of one or more of the following:
• Diffuse abdominal pain
• Any biopsy showing predominant immunoglobulin A deposition
• Arthritis (acute, any joint) or arthralgia
• Renal involvement (any hematuria or proteinuria)

Symptoms & Signs

Mild leukocytosis
Elevated IgA levels 
Skin or renal biopsy for IgA deposition
Thrombocytopenia suggests alternate diagnosis
microscopic or gross hematuria

Potential Complications

Severe scrotal edema may cause testicular torsion
 myocardial infarction, pulmonary hemorrhage, or central nervous system involvement with seizures and hemorrhage


DDX

Thrombocytopenic purpura: end-organ ischemia, rash也長得不一樣


Ref. 
October 1, 2009  ◆  Volume 80, Number 7     www.aafp.org/afp      American Family Physician  699-703

Child seizure 

Chief. Lee AP

Epileptic seizure

At least 2 seizures occurring >24 hr apart
治療3-5年才能停藥
十年不復發才稱痊癒

Focal(partial)seizures
Simple,  Complex跟預後無關,主要依 consciousness 有無分



2015年10月21日 星期三

[醫如往常] Junior Clerkship Day 35: Pediatrics: LGMD; Periorbital cellulitis

20151021 Morning meeting: LGMD; Limb-girdle muscular dystrophy

Dr. Chu (R3)

Case

Heart failure->DCMP, Ascites->Abdominal pain
-> Renal infarction-> hematuria
Arrhythmias-> AF; atrial fibrillation-> Cardiogenic shock


DDX: Dyspnea

DDX: Abdominal pain

 LGMD Cardiac Involvement

全部LGMD約有10% heart failure
Heart rate 控制在120以內 可以改善
但有DCMP的話很難
DMSA follow
Anticoagulation half year


動物模式2F用β-blocker可能會惡化

可做Parvo B19 test
可能加重症狀


LGMD基因

特別與heart有關之基因
1B: Lamin A/C.
2I: FKRP; fukutin-related protein
2F: Sarcoglycanopathies 青少年發病
測一次八千元

LGMD基因
臨床表徵此症的臨床表現可以依遺傳模式分為以下幾種
表1. 體染色體隱性遺傳肢帶型肌肉失養症:臨床表現(請點選)
肌聚糖病(Sarcoglycanopathies),包含 (肢帶型肌肉失養症2C,D, E, F型) 。病程從兒童發病的嚴重型到成人發病輕型都可能。小腿肥大很常見。
表2. 體染色體顯性遺傳肢帶型肌肉失養症:臨床表現(請點選)
Ref.
http://www.genes-at-taiwan.com.tw/genehelp/article.html?articleID=Limb-girdle%2520muscular%2520dystrophy%2520type%25202A%2C%25202B%2C%25202D&submenuIndex=0




Periorbital cellulitis


Purple-red eyelid discoloration occurs (called heliotrope eyelids or “raccoon eyes”紫色
小心是Hib; Haemophilus influenzae type b or non B type, HIV
一般細菌是紅色
Ex: Staphylococcus, Streptococcus
台灣MRSA很多,要小心用藥

這次Case是單純趴在地上眼睛Trauma造成Periorbital cellulitis
蠻特別的

兒童呼吸道感染 

Prof. Lee

感冒是診斷
不是症狀

Benjamin Franklin表示:「感冒是傳染自他人的疾病,並不是冷所引起的」 

「重感冒」其實指的是 influenza virus 所引起的感冒,會全身非常的不舒服。 
BMI>35建議打流感疫苗 

台灣有H5N2
沒有H5N1
原因是台灣跟菲律賓禁止大陸農產品進口
幸好候鳥帶病毒能力不強,不然台灣跟菲律賓也會有

Macrolide resistance rate

Macrolide resistance rate

Germany 3%
France 9.8%
Japan 13.2%
Taiwan 20%
Italy 26%
China 90%


兒科胸腔討論會

Prof. Liu
Weaning突然seizure
懷疑EBV encephalitis
不過比較可能原因是inflammation造成
EBV仍然是lytic


臨床症候群診斷

Prof. Tsai AP


achondroplasia rhizomelic
mesomelic dwarfism

Reye syndrome
congenital rubella syndrome

Risk Factor
low social economic family

1 major 2 minor chromosome檢查

90%初生兒會表現三種以上的 minor anomalies 會和某種
major malformation 相關,在台灣統計資料是 10.1%。 

down syndrome 鳳眼Mongoloid slant of eyes
william syndrome 八字眼Skin that covers the inner corner of the eye (epicanthal folds)

prader willi syndrome almond shaped palpebral fissure

Dr. McKusick
Father of modern genetic medicine

Array CGH 限制 
(一) 平衡性轉位(Balanced translocation) 
(二) 小的 DNA 突變(Small DNA mutations) 
1.  如點突變,  小的缺失或擴增 
(三) 低比例的鑲嵌(low level of mosaicism) 
(四) Probe 沒有設計的區域 
Ex: Philadelphia chromosome

VACTERL/VATER association  沒屁眼 

CHARGE association -> CHARGE Syndrome

OMIM (Online Mendelian Inheritance in Man)  
罕見遺傳疾病一點通 
http://www.genes-at-taiwan.com.tw/genehelp/ 

[醫如往常] Junior Clerkship Day 34: Pediatrics: Acute bronchiolitis

20151020 Morning Meeting: Acute bronchiolitis

Intern Dr. Lee.

DDx

Hypersensitivity airway : bronchodilator才有用
(tachycardia with bronchodilator)

Heart failure: Poor appetite, vomit, prolonged wheezing



Acute bronchiolitis

< 2 y/o with weezing for 3-7 days, cough for 1-2 weeks.
After URI 48-72hr  

Virus infection: 50% RSV
Mycoplasma 比較看不到cluster, 跟3-5 y/o同住的比較有可能

Zithromax, 主要功能anti-inflammation,也可用在Pertussis, Chlamydia
"Azithromycin作用機轉主要是和細菌的50s核醣體結合,阻止胜肽之移位作用進而抑制細菌蛋白質的合成。http://www.ktgh.com.tw/Medicament_tbDrug_Look.asp?CatID=124&ModuleType=Y&NewsID=207"

Clinical Presentation:

Rare in 1st month, peaks at 2-5 months
 Upper Respiratory Infection
o Rhinitis
 Lower Respiratory Disease
o Tachypnea
o Wheezing 考慮吃個dilator再測
o Cough
o Crackles
o Use of accessory muscles
o Nasal flaring
 Fever in approximately 30%

Pneumothorax, atelectasis, interstitial infiltration


應該給O2 tent

不過這次缺貨



RSV TX
Ribivirin
hyperimmune RSV IVIG
I.M. palivizumab (anti-Protein F)
  1. preterm <28 weeks
  2. chronic lung disease (6 month) with medication
  3. congenital heart disease

補充

Loss of stool跟diarrhea不太一樣

Ddehydration可以看urine specific gravity
"A specific gravity greater than 1.035 is consistent with frank dehydration. In neonates, normal urine specific gravity is 1.003. Hypovolemic patients usually have a specific gravity >1.015."
https://www.wikiwand.com/en/Urine_specific_gravity

Ribs count
"The anterior end of approximately 5-7 ribs should be visible above the diaphragm in the mid-clavicular line."
http://radiologymasterclass.co.uk/tutorials/chest/chest_home_anatomy/chest_anatomy_page11.html
Over flextion
Flat Diaphragm
Atelectasis

  • Bronchodilators:
  1. Short-Acting Beta-Agonists
  2. Racemic Epinephrine (No evidence for repeated dosing or prolonged use in inpatients)
  3. Albuterol (No routine use )
  • Corticosteroids (Not recommended)
  • Leukotriene Receptor Antagonists (Not currently recommended) Hypertonic Saline (May be useful in inpatient setting, likely not useful in emergency department)
  • Antibiotics (UTI, AOM; acute otitis media)
  • High-Flow Nasal Cannula
  • Continuous Positive Airway Pressure (CPAP) (Insufficient evidence)
  • Helium-Oxygen (Heliox)(Insufficient evidence)
  • Surfactant(Insufficient evidence)
  • Home Oxygen Therapy (Insufficient evidence)
Ref.
APA建議
Management of Bronchiolitis: A Clinical Update
https://www.meadjohnson.com/pediatrics/us-en/sites/hcp-usa/files/PCOWebinar-Bronchiolitis_Final.pdf 

Cholestasis in infancy


Prof. Chen

α-fetoprotein: 後來的albumin
>2 weeks prolonged jaundice
Bile acid: liver氧化傷害 apoptosis

biliary cirrhosis: portal area extension
HBV cirrhosis不一樣,是post necrosis cirrhosis

CMV 是最常見造成新生兒肝炎的病原。 
其他像是Medical history: TPN(全靜脈營養), Ischemia, Sepsis, Drugs 

Chronic Cholestasis 要補充MCT(中鏈脂肪酸), Vit. ADEK


Allergy

Dr. Lin
Asthma

Reliever

β2-agonist可隔 15 到 20 分鐘再使用一次。 
若使用了三次,仍覺不適,或最大吐氣流量數值仍是平常的百分之八十以下,就應到醫院治療。
出院後六小時吸一次, 吸三天

Controller:預防性的藥物 

七天內都算同一次, 一個月內發作兩次就要給controller
連用三個月沒有發作可以停藥

Allergic rhinitis

Most common
child 2/3, adult 1/3
 症狀  :  超過七天的流黃膿或綠膿鼻涕、黃膿或綠膿鼻涕倒流(鼻塞、咳嗽、清喉嚨的聲音)

α agonis噴鼻型血管收縮劑  :  只在已使用噴鼻型類固醇者,當呼吸道感染或鼻竇炎時嚴重鼻塞時使用,一次使用不可超過五天

real cough 超過三聲
清喉嚨一聲



Viral Hepatitis in Children 

Prof. Chang

HBV

Prof. Beasley發現越年輕感染越容易變成帶原者。 
吳嘉峰教授發現在宿主免疫能力方面,體內高濃度 IL-10,IL-12 較容易發生HBeAg seroconversion。以醫師的角度來說Seroconversion 能越早出現越好,能降低病毒的表現。 
倪衍玄教授發現 HBV B-type較 C-type 容易轉換進入 inactive phase。
HCC 80% has cirrhosis
張美惠教授發現5%出生就有Anti-HBsAg: intrauterine infection
陳慧玲教授發現Viral load 跟傳染力的關係
Operation room針扎很多要小心

Maurice Ralph HillemanAdjunct Professor of Pediatrics at the University of Pennsylvania in Philadelphia.
Maurice Ralph Hilleman (August 30, 1919 – April 11, 2005) was an American microbiologist who specialized in vaccinology and developed over 40 vaccines, an unparalleled record of productivity. Of the 14 vaccines routinely recommended in current vaccine schedules, he developed eight: those for measlesmumpshepatitis Ahepatitis Bchickenpox,meningitispneumonia and Haemophilus influenzae bacteria.  He also played a role in the discovery of the cold-producing adenoviruses, the hepatitis viruses, and the cancer-causing virus SV40. 
https://www.wikiwand.com/en/Maurice_Hilleman

2015年10月19日 星期一

[醫如往常] Junior Clerkship Day 30: Pediatrics: Salmonella infection; PID; UTI

20151014 Morning Meeting: Salmonella Infection

Case 1

Prof. Lin補充:

問診要問
  1. 長幾顆牙?
  2. Caregiver教育水平

Toxic sign

  • Prolonged fever > 3 day
  • Immunocompromised < 3 month 
Fever >5 days 偏向細菌性
會有Leukocytosis
Salmonella group D 最多 (O antigen分類)

是Salmonella group Group F跟人沒太大關係

桃園因為用地下水,夏天常爆發疫情

動物飼料造成Salmonella resistance
Resistance要報最新的
千萬不要報十年前的

Stool culture negative, 不代表沒有bacteremia


Case 2

三歲小朋友第一次Wheezing考慮mycoplasma。

美帝最近爆發Enterovirus type 68 
特色:Asthma with no fever.


Asthma V.S. Croup

Croup or laryngotracheobronchitis

哮吼是一種上呼吸道感染(也就是感染發生在喉部以上),大多數的原因是病毒感染造成的。
約有一半的病例會fever,嚴重時可能會使小朋友呼吸衰竭或呼吸道完全阻塞,面臨生命危險需要急救。
常在半夜突然發作,好的快但也容易復發。
超過三分之二為副流感病毒 (parainfluenza virus) 感染,其他像是呼吸道融合病毒 (RSV)、流感病毒 (influenza virus)、麻疹病毒 (measles virus)、腺病毒 (adneovirus) 等。少數哮吼是由細菌或黴漿菌 (mycoplasma) 所引起。 
哮吼多發生於 6 個月 ~ 6 歲的兒童,高峰期在兩歲左右。男生比女生多。一年中以較冷的季節發生率較高,像是秋天和早冬。 
Croup:
  1. Hoarseness
  2. Barking cough
  3. Stridor吸氣】時高頻的喘氣聲
必須和呼吸道異物吸入、會厭炎、咽喉膿瘍或深頸部感染作鑑別診斷。

和氣喘或細支氣管發炎不同處在於:

  • 哮吼有聲音沙啞和狗吠聲,這是上呼吸道感染、阻塞所產生。氣喘或細支氣管發炎為下呼吸道感染、阻塞,不會有這些症狀。
  • 喘鳴聲發生時機不同: 哮吼是吸氣時,其他是吐氣時。

Asthma(哮(氣)喘)

哮(氣)喘則是發生在下呼吸道的疾病(指喉部以下的疾病)

  • wheezing吐氣】末端高頻的喘氣聲

Ref. 



Primary immunodeficiency (PID) 

Prof. Wang AP

X-linked agammaglobulinemia (Bruton Disease)

Predominant Antibody Deficiencies的代表
Clinical manifestation:

  1. 小孩 5~6 個月時(B cell),常有反覆的細菌感染,因為是 X-linked,所以好發在小男孩。 
  2. 常見感染細菌:Streptococcus pneumonia, Haemophilus influenza 
  3. Lack of adenoid tissue (LN, tonsil) 

Lab:
1.  Low IgG, IgA, IgM
2.  Few peripheral B cells
Treatment:缺抗體,就補抗體 IVIG every 3~4 weeks, Prophylactic antibiotics
Prognosis:有輸 Ab 可活到 20~30 歲左右

WAS; Wiskott-Aldrich Syndrome (T, B 都有問題)

Etiology: WASP(Wiskott-Aldrich syndrome protein) mutation on
Xp11.22-11.23:合成actin filament

Clinical manifestation:
1.  Combined immunodeficiency
2.  Thrombocytopenia
3.  Eczema
Lab exams: Low platelet count, and small platelet
雖然 IgG 量正常,但是功能是異常的,所以治療上還是要IVIG

Congenital Defects of Phagocyte: Chronic Granulomatous Disease (CGD) 

Etiology: NADPH mutation,因為生產過氧化物的過程中有問題,所以無法產生過氧化物去破壞病原體,突變的基因如下圖左表,除了第一個(gp91phox)是 X-linked,其餘皆是 autosomal recessive。

Clinical manifestation:
1.  兩歲前發病
2.  Lymphadenitis, hepatosplenomegaly, pneumonia, osteomyelitis, abscess
3.  Staphylococcus aureus, Staph epidermidis, Serratia marcescens, E.Coli, Burkholderia, Salmonella, Candida albicans, Aspergillus
 Lab exams: NBT, chemilluminescence, DCF, bactericidal test
Treatment:
1.  Prophylactic Abx
2.  IFN-γ
3.  BMT



UTI; urinary tract infection

Prof. Tsao

Acute lobar nephronia(急性大葉性腎盂腎炎;ALN) 

ALN 屬於間質組織的發炎,一般認為其病理介於 acute pyelonephritis(APN)與 renal abscess 之間。
超音波看到nephromegaly就可以診斷!
主要致病原:papG II gene(+) P-fimbrae E. coli。

APN: 在CT下看不太到東西
ALN: CT下只看到腎腫大,加入顯影劑後卻看到類似膿瘍的東西。
Renal abscess: 未加顯影劑就看得到膿瘍。

APN在有效抗生素治療下約 2 天內會退燒,治療期間約 10 天。

Simple ALN 的 prolonged fever 平均持續 3天,complicated ALN 則平均持續 5-7 天,治療期間約 2~3 星期。
Renal abscess則要4-6 weeks。

預防性抗生素通常選用表下方的三種藥物,劑量則是治療劑量的三分之一至四分之一,一天一次。預防性抗生素投藥是否可以降低感染機率。

抗生素預防原理:減少adhesion。而非bacterialcidal
Ex: erythromycin之於Pseudomonas也是如此

Pyonephrosis

左邊圓圈wall有一些白白的debris
右邊central debris, 需要surgical drainage

[醫如往常] Junior Clerkship Day 33: Pediatrics: Pineal tumor, Biliary atresia, DDx

20151019  Morning meeting

Water view
Acute sinusitis

Hydrocephalus
Pineal tumor

Dr. Liao 

Germ cell tumor
Geninoma
ICE therapy
Prognosis重要表格

不同腫瘤治療差異

神外 Prof. Kuo

Pineal tumor

  1. 3rd ventriculostomy (第三腦室造口)
  2. BP shunt may seeding
  3. VAD; Ventricular access device
  4. EVD; External ventricular drain
RT放療需考慮堵塞問題

Vice Superintendent Chief. Prof. Ni

Suspect Biliary atresia

Clay color stool
Total bilirubin >4 (<4幾乎不可能是Biliary atresia)
Liver Biopsy negative 也很尷尬
直接做intraoperative cholangiography (IOC)+ biopsy
Negative -> Cholestasis


過完節 bilirubin>7
又要懷疑BA


不過就算不是BA也不能掉以輕心
很多也要換肝

現在也可做gene array

Dr. Chang-Jen (R4)

出生Vit. K1
2000g 能打B肝疫苗
2500g 才能打BCG


Prof. Wang

台灣的兩大代表性X-linked疾病:   

(一) Vitamin D resistant hypocalcemia rickets:幼兒的 osteoid  無法鈣化,合併骨質生長異常。   
(二) Incontinentia pigmenti:一出生就有皮膚變色,也有 mental retardation,合併癲癇,且極難治療。 

Dr. Chou

Pulmonary embolism sign

待補...

Lesch Nyhan syndrome

發病期約在嬰兒3~6個月大時,是因次黃嘌呤-鳥嘌呤磷醣基核甘轉換酵素(hypoxanthine-guanine phosphoribosyl transferase, HPRT)的先天性缺乏所引起之高尿酸血症,且會導致舞蹈手足徐動症、智力遲鈍和自身摧殘行為的產生。
http://www.genes-at-taiwan.com.tw/genehelp/database/disease/Lesch_Nyhan_syndrome_940801.htm

Marker

  1. negative finding  的重要性更高,它可以直接排除某些疾病
  2. routine Lab通常是不必要的
  3. Lab data  的判讀:「正常就是不正常」 

Chronic cough

幾種可能(咳嗽的 pattern會不盡相同) 
(1)  過敏性鼻炎、鼻竇炎 
(2)  氣喘:常有夜咳,吃完冰後、季節交替時候咳 
(3)  Neoplasm:成年人一定要考慮造成久咳的原因 
(4)  Bacteria pneumonia:不太會造成久咳,故比較不屬於此鑑別診斷
(5)TB  所造成的 arthritis,多發性的機會不是很高,常是 1~2  個關節,基本上不會超過 5 個

Orthopnea

  1. 鼻塞
  2. 氣喘
  3. Carditis


[醫畫開天] [韓劇] D-day 9, 10

[醫畫開天] [韓劇] D-day 9, 10

還不錯的災難片
已經到第10集了
https://www.wikiwand.com/zh-tw/D-Day_(%E9%9B%BB%E8%A6%96%E5%8A%87) 

第九集

BCLS手臂伸直得還算可以,不過應該要整個人到床上壓更正確

朴智娜(尹周熙)急診室專科護士EMT

???

停電時早產兒保溫法

殷昭律(金晶和)精神健康科專科醫生


鄭乭美(庭沼珉)整形外科住院醫生R3



安大吉(李成烈)實習醫生


韓宇鎮(河錫辰)一般外科副教授

第十集


李海成(金英光)一般外科醫生