Case 051229 "Odontogenic keratocyst"

"An 18-Year-Old Man with a One-Month History of Nontender Left Mandibular Swelling". This CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL describes an 18-year-old man who reported gradual painless swelling of the left mandible. On physical examination, there was obvious facial asymmetry, with the left side of the jaw larger than the right. And odontogenic source of infection was ruled out. The painless left posterior mandibular swelling with radiographic findings of a loculated radiolucency and an associated impacted tooth favor the diagnosis of an odontogenic cyst. However, the lack of bone perforation and the circumferential appearance of the radiolucency slightly favor the diagnosis of odontogenic keratocyst. Meredith August discusses both odontogenic and nonodontogenic processes in the differential diagnosis. August also explains management of cystic lesions of the jaw.

今回は、角化嚢胞性歯原性腫瘍。

Case 051222 "Onchocerciasis"

"A 63-Year-Old Woman with a Positive Serologic Test for Syphilis and Persistent Eosinophilia", this CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL discusses 63-year-old woman from Cameroon who was referred to infectious-disease specialists.
She had a positive serologic test for syphilis and persistent peripheral-blood eosinophilia. Routine chest radiograph had shown a dilated aorta with calcification.
On physical examination, there was a leopard-skin appearance of the skin of the upper back, upper buttocks, and upper thighs, with scattered hypopigmented lesions.
Though she had been sexually active only with her husband and had been a widow for 15 years, her husband had had five wives. In addition the patient had previously been treated filariasis. Transthoracic echocardiogram disclosed minimal thickening of the leaflets and moderate aortic regurgitation.Snips of the patient's skin were evaluated for microfilariae.
Donna Felsenstein discusses the diagnosis, staging and management of syphillis. And Edward Ryan describes infectious causes of persistent eosinophilia and management in this case. Video clips of the transthoracic echocardiogram and of this skin snip showing mobile microfilaria may be reviewed in NEJM.org. [Original Article]  

今回は、WHOが指定した１７つのNeglected Tropical Diseases(NTD)の１つ。"mobile"の聴き取りに難渋した。名詞でモウビール、形容詞でモウブル、いずれにせよ、前方にアクセントがあって、モバイルは完全に和製英語。

Case 051215 "SLE"

"A 29-Year-Old Pregnant Woman with the Nephrotic Syndrome and Hypertension", this CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL describes a 29-year-old pregnant woman with systemic lupus erythematosus who was admitted to the hospital because of renal failure at 20 weeks and 6 days of gestation.
Prior to the visit the patient was been monitored for hypertensive complications of pregnancy or worsening nephritis. Fetal growth was followed with ultrasonography. As she approached the 20th week of pregnancy, marked edema and weight gain occurred, with evidence of worsening renal function. Edema and proteinuria and rising blood pressure were present at the time of admission; this triad is the hallmark of preeclampsia, but each of these findings can also be associated with a flare of lupus nephritis. During the next three weeks, proteinuria persisted, anemia and thrombocytopenia developed and worsened, and the blood pressure continued to rise. A procedure was performed.
In discussing the differential diagnosis, Winfred Williams Jr. writes that the issue is whether she was having a flare of her lupus nephritis, preeclampsia, or both. These two possibilities require radically different management strategies and, therefore, must be sorted out as quickly and efficiently as possible. Williams stresses that this is especially the case in a pregnancy in which renal failure and hypertension are progressing rapidly.

Case 051208 "Brugada syndrome"

11:49| "A 35-Year-Old Man with Cardiac Arrest while Sleeping" This CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL describes a 35-year-old man who screamed during sleep and became unresponsive, with agonal breathing and a bluish skin color.His partner performed mouth-to-mouth "rescue" breathing, but not chest compressions.
 Technicians from emergency medical services found him in ventricular fibrillation; normal rhythm was restored, but the patient did not regain consciousness. In the emergency department an EKG revealed ST-segment elevation, and there was evidence of pseudoephedrine in the serum. The results of cardiac catheterization were normal.
 Keith Marill discusses the issues raised by this case including the management of out-of-hospital cardiac arrest due to ventricular fibrillation both at the scene and in the emergency department. Marill also discusses the differential diagnosis of cardiac arrest in a young patient.

Further Reading

• QT延長症候群（先天性・二次性）とBrugada症候群の診療に関するガイドライン

Case 121227 "Amniotic-fluid embolism"

9:53| "A 43-Year-Old Woman with Cardiorespiratory Arrest after a Cesarean Section", a CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL by Jeffrey Ecker and colleagues.
A 43-year-old woman (a multigravida) was admitted to the labor and delivery service of this hospital at 36.4 weeks of gestation because of vaginal bleeding.
Marginal placenta previa was seen on obstetrical ultrasonography, and two episodes of bleeding occurred, at 27.7 weeks and 32.7 weeks of gestation, which resolved after the patient was admitted for bed rest, hydration, and the administration of betamethasone. She was advised to maintain bed rest at home. At a routine prenatal visit 8 days before this presentation (at 35.3 weeks of gestation), obstetrical ultrasonography revealed an anterior placenta with the edge covering the internal os, a finding consistent with placenta previa.
Heavy vaginal bleeding developed on the day of admission. Plans were made for emergency cesarean delivery. A healthy boy was delivered. The placenta previa was removed.
Twenty minutes after delivery, as the abdominal fascia was being closed, the patient's systolic blood pressure fell to 70 to 80 mm Hg, the pulse to 30 to 39 beats per minute, and oxygen saturation to 70 to 80%. The patient reported chest pain, and her lips became white; apnea developed rapidly thereafter, and she became unresponsive. Examination revealed pulseless electrical activity.
In situations such as this clinicians must focus promptly on a most-likely diagnosis in order to direct problem-specific therapies.

Case 051124 "MELAS"

13:24| A CASE RECORD OF THE MASSACHUSETTS GENERAL HOSPITAL describes a 61-year-old who was admitted to the hospital because of a seizure and altered mental status.
 The patient had been in good health, with the exception of mild hypertension for which she took no medications.
 On the morning of admission, her husband awoke at 4 a.m. to find her thrashing in bed, with rhythmic movements of all four extremities, for two to five minutes. She subsequently appeared groggy but alert, with a facial droop, garbled speech, and an inability to follow verbal commands.
 For the next six months, she remained aphasic and confused. During first hospitalization, the neuroimaging findings confirmed the clinical localization of the left temporo-occipital lesion, and its characteristics suggested focal metabolic dysfunction.
This neurologic syndrome occurred in the context of a urinary tract infection, and lumbar puncture revealed only elevated lactate levels, without evidence of a central nervous system infection or inflammatory response.
 Dr. Bradford Dickerson discusses differential diagnosis and management of this patient. [Original Article]

Vocabulary:

• groggy ふらふらした（グロッキーの元の語）
• garbled 不明瞭な(「文字化け」の意味もある）

Further Reading:

• Gene Reviews Japan "MELAS"

Case 051117 "Breast Cancer"

12:14| In the CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL, a 56-year-old woman was referred to the multidisciplinary breast clinic, for management of invasive breast cancer.
She presented with a 1-cm invasive ductal carcinoma of the breast.
On examination, the patient appeared well, and her vital signs were normal. There was a palpable 1.5-cm mass at the 10-o'clock position in the anterior axillary line. There was no discharge or inversion of the nipples and no lymphadenopathy. The results of routine laboratory tests revealed no abnormalities.
One month later, a right partial mastectomy, intraoperative lymphangiography, and sentinel-lymph-node biopsy of the right axilla were performed. Immunohistochemical staining detected positive tumor cells. The tumor was positive for estrogen-receptor protein and faintly positive for progesterone-receptor protein.
The patient returned to the breast clinic one month later for discussion of further management.
 The authors discuss the staging and management of breast cancer with sentinel lymph-node involvement. [Orignal Article]

Case 051110 "TEN"

10:18| This CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL describes a 10-year-old girl who was transferred to the Shriners Hospital for Children in Boston because of a bullous skin eruption, with sloughing of the skin and respiratory failure.
The patient had been well until two months before admission, when she had a generalized tonic–clonic seizure. She was examined at different hospital and treated with diazepam.
One month before admission, during an episode of gastroenteritis, intermittent twitching occurred in both legs. A diagnosis of seizure was made, and a neurologist prescribed carbamazepine.
On examination two days after the seizure, the physical and neurologic examinations showed no abnormalities.
Nine days later, the temperature rose and a rash appeared on the patient's trunk. The next day she went to the emergency room of the hospital where she had been treated previously. A diffuse, erythematous morbilliform rash with extensive confluence was present, most intensely visible on the back, trunk, and upper arms and thighs, with blanching. A diagnosis of hypersensitivity reaction was made. The carbamazepine was discontinued.  The patient was discharged to her home.
The next day, the rash had spread to involve most of her body, and vesicles had developed. On examination, diffuse, erythematous, raised lesions involved the face, trunk, back, arms and legs, and lips. Nikolsky's sign that is ready removal of the epidermis with slight tangential pressure was present.
This case record describes ??? After a protracted course of management in the burn unit, reepithelialization occurred, and the patient fully recovered.
 Dr. Vincent Liu discusses differential diagnosis of blistering skin eruption. [Original Article]

Further Reading:

• Robert S. Stern, M.D. Exanthematous Drug Eruptions N Engl J Med 2012; 366:2492-2501

"Cabot Cases" in NEJM

NEJMで長く連載されているCPCは、MGHで研修の目的で行われているカンファレンスである。合衆国のロースクールでの"Case book method"をメディカルスクールに導入したのが、Walter Bradford Cannonであった。その後、臨床と病理を突き合わせるという形式を定めたのがRichard Clarke Cabot。その創設者に敬意を表して"Cabot cases"とも呼ばれてきた。そして、いよいよ1923年10月25日 "Case 9431"がNEJM誌に登場する。冒頭は、以下の通り。

An American student of twenty-four entered March 26, March, 1923, complaining of pain and dyspnea.

ケースのナンバリングの最後の桁は、その週の何番目のケースかを示している。つまり、連載開始以前942週に渡り、MGHでこのカンファレンスが開催されていたことになる。このナンバリングは1960年12月29日号に掲載されたCase 46522まで、平均して週に2症例ずつ続くことになる。1961年からは、年毎の連番となり、１９６１年には９２症例だったものが漸減し、1967年以降は週1症例に固定され、年間50〜53症例の時代が続く。1992年からはJerome P. KassirerのClinical Problem-Solvingの連載が始まり、1995年からは各月第1週は、この新たな症例報告形式が定期連載となり、CPCはその時は掲載されず、年間40症例の掲載となって現在に至っている。この間、Audio Summaryが2005年１１月3日から開始され、CPCも約1分間にまとめられて現在まで287症例の概要が音声を通じて紹介されている。

Serious Celiac Sprue

NEJMの"Clinical Practice"のシリーズは、全文がオーディオで提供され、今回134本目を数える。ぜひMP3プレーヤに入れておきたいシリーズである。

9:13| "Celiac Disease", a clinical practice article by Alessio Fasano from University of Maryland School of Medicine, Baltimore.
• Once considered a gastrointestinal disorder that mainly affects white children, celiac disease is now known to affect persons of different ages, races, and ethnic groups, and it may be manifested without any gastrointestinal symptoms.
• Measurement of IgA anti–tissue transglutaminase antibodies is the preferred initial screening test for celiac disease because of its high sensitivity and specificity, but it performs poorly in patients with IgA deficiency (which is more common in patients with celiac disease than in the general population).
• The diagnosis is confirmed by means of upper endoscopy with duodenal biopsy, although recent guidelines suggest that biopsy may not be necessary in selected children with strong clinical and serologic evidence of celiac disease.
• Given the undisputable role of gluten in causing celiac disease enteropathy, the cornerstone of treatment is the implementation of a strict gluten-free diet for life.
• Gluten sensitivity may occur in the absence of celiac disease, and a definitive diagnosis should be made before implementing a lifelong gluten-free diet.
Listen to the full text of this article at NEJM.org.(192 words / 93 sec = 124bpm)

 上掲本の最初の提示症例がセリアック病(Celiac disease)である。なんと１５年もの間、様々な専門医、総合医の診察を受け見逃されてきた経過が語られる。カッパドキアのアレテウスの昔に記載され、その名も、"abdominal" diseaseと呼ばれ、西洋人においては、さほど珍しくもないこの病気がである。その誤診の仕組みは、ぜひ本書に目を通して頂きたい。

備えあれば…

今度の冬至に地球が滅亡するという説があるそうですが、我が家は電気不要の灯油ストーブを購入して備えています。

19:13| "Preparing Health Systems for Future Disasters", a perspective article by Irwin Redlener from Columbia University, New York.
The smooth operation and then evacuation of NYU's hospitals during and after Hurricane Sandy were remarkable. But for the future, it's important to understand what medical and public health challenges are to be expected after such megadisasters.
The first order of business is always to identify and treat storm-related casualties requiring urgent attention.
Second, potentially serious public health complications may follow large-scale storms with extensive flooding. In fact, overflow from the toxin-filled Gowanus Canal in Brooklyn is already a concern that will require close monitoring. Prolonged lack of power and heat, a real problem in the first 2 weeks after Sandy, became dangerous for the elderly, homebound patients, and small children, especially those living in low-income housing projects. There are already signs of dangerous mold infestations in dwellings soaked by rain and floods.
Third, essential supply chains must be restored. The most obvious and critical concern is ensuring that patients receiving life-critical medications or supplies have uninterrupted access.
Finally, access to health care, often a casualty of large-scale disasters, has been a major challenge in Sandy's aftermath. Offices, clinics, and hospitals were damaged, some irreparably. (211 words / 99sec = 128 wpm) [Original Article]

"irreparably"はアクセントの移動があり、ピンとこなかったが、"repair"出来ないほどに、ということ。"repair"繋がりで、JFKの言葉に次のものがあります。

The time to repair the roof is when the sun is shining.

「屋根を直すのは晴れているうちに。 」、つまり、有事に先んじて準備せよってことです。