菅原 斉

We present a case of diffuse aspiration bronchiolitis (DAB) with a false‐positive Gaffky score. “Tree‐in‐bud” opacities detected after aspiration should be considered DAB rather than mycobacterial infection.

It is important to suspect intracranial hypotension based on distinctly frequent orthostatic headaches and diffuse dural hyperplasia. Lumbar puncture is a procedure prone to complications, especially in patients with already existing intracranial hypotension.

An 82‐year‐old man with untreated diabetes mellitus (DM) had anterior chest wall swelling and ulcers 2 years following blunt chest trauma. Contrast‐enhanced computed tomography revealed sternal fracture with osteolytic change and subcutaneous abscess. Blood and sternal cultures were positive for methicillin‐susceptible Staphylococcus aureus (MSSA). Transesophageal echocardiogram showed vegetation on the right coronary cusp and moderate aortic regurgitation. The patient received a diagnosis of infective endocarditis associated with chronic sternal osteomyelitis complicated by subcutaneous abscess because of MSSA. This case report showed that trivial trauma in patients with uncontrolled DM can cause chronic sternal osteomyelitis resulting in infective endocarditis.

Peritonsillar abscess should be considered as a differential in patients presenting with fever, sore throat, and other cold‐like symptoms. Point‐of‐care transcutaneous cervical ultrasound is effective for diagnosing peritonsillar abscess in place of computed tomography (CT) imaging. Tongue movement during ultrasound examination will help confirm the presence of anatomical lesions on tonsils, and comparing the affected and unaffected sides will improve proper diagnosis.

A 49‐year‐old‐Japanese woman visited us with a 1‐year history of verrucous lesions on the oral mucosa and fingers. Her past medical history was unremarkable except for chilblain in winter. She had no family history of collagen disease. Physical examination showed a well‐defined, hyperkeratotic erythematous plaque with ulceration on the palate and verrucous nodules on the fingers (Fig. 1a,b). She had no butterfly rash or discoid lesions on any other sites. Histology from both oral mucosa and finger revealed similar features; marked hyperkeratosis and acanthosis with perivascular lymphocytic infiltrates in the dermis. Liquefaction degeneration with a few necrotic keratinocytes was also found (Fig. 1c,d). We regarded the patient as verrucous lupus erythematosus (LE). Then, leukopenia (2,820/μl) and thrombocytopenia (67,000/μl) were observed as well as positive antinuclear antibody (ANA). However, the diagnosis of systemic lupus erythematosus (SLE) was not confirmed by the negative finding of anti‐DNA antibody, and the lack of requirements in 1997 updated ACR criteria for SLE validated back at that time. She developed skin ulcers on the left lower leg in the next two months (Fig. 2a). Four months later, laboratory examination revealed leukopenia (2,850/μl), thrombocytopenia (89,000/μl), low C3/C4 level (27 mg/dl, 3 mg/dl, respectively), positivity for ANA (1 : 640, speckled type), and anti‐Sm antibody (1 : 2). Histology of the leg ulcer exhibited no vasculitis but the occlusion of the vessels (Fig. 2b). Direct immunofluorescence revealed IgM, C1q, and C3 depositions on vessel walls as well as linear IgM deposition at basement membrane zone (Fig. 2c). Seven months later, she had seizure attacks. Then, SLE was diagnosed based upon ACR (1997) and SLICC criteria. Even under 2019 EULAR/ACR classification criteria,1 she was classified as SLE from total 24 points scored by the findings of fever (38.9 °C), leukopenia, thrombocytopenia, seizure, low C3/C4, and anti‐Sm antibody. Then, the patient was transported to us for dyspnea and high fever. Methylprednisolone pulse therapy was performed. Chest roentgenogram revealed extensive lung infiltrates. Aspergillus fumigatus was isolated from the sputum. Invasive aspergillosis was diagnosed. She died of multiple organ failure 8 months after the first visit.

A 66-year-old woman complaining of painless bulging in the right flank abdomen exhibited redness with belt-like scab formation in her right lower quadrant (Th11-12 level).

A 77-year-old man visited the hospital with a chronic cough persisting for 2.5 months accompanied with night sweats, weight loss (3.5 kg) and elevated C-reactive protein level. Chest CT of the lung field was normal, but aortic wall thickening accompanied by a contrast effect was noted. Positron emission tomography–CT (PET–CT) showed that the aorta and subclavian artery were inflamed, suggesting large-vessel vasculitis. Ultrasonography showed thickening of the superficial temporal artery wall (macaroni sign). Biopsy revealed lymphocytic infiltration in the tunica media and foreign-body giant cell reaction with the elastic lamina, resulting in a diagnosis of giant cell arteritis (GCA). The cough was considered a symptom of GCA as it resolved following prednisolone administration. Cough may rarely be an initial GCA symptom. However, for chronic cough accompanied with elevated inflammatory findings but with a normal lung field, imaging studies such as PET–CT are useful for the differential diagnosis.

A 66-year-old man complained of lower limb edema was diagnosed with mesenteric panniculitis because of fat ring sign and tumoral pseudocapsule sign on contrast-enhanced CT. Patient had edema due to protein-losing enteropathy associated with mesenteric panniculitis.

Background: Brucellosis, an important zoonotic disease, is endemic in various parts of the world. Patients diagnosed with brucellosis in developed countries are often travelers and immigrants from endemic areas. This pathogen is listed as biosafety level 3, which means that it is highly contagious and therefore a risk to clinical laboratory technicians. Case presentation: A 43-year-old Chinese man, who could not understand Japanese, visited our hospital because of an intermittent fever that had persisted for 5 months. Associated symptoms included muscle pain whenever he had a fever. He reported currently working as a welder in Japan. However, his previous employment working in animal husbandry in Heilongjiang, mainland China was not determined at the initial visit owing to language barriers. Two sets of blood culture showed nonfermenting gram-negative bacilli, initially misidentified as Ochrobactrum anthropi and subsequently identified as Brucella abortus. Six-week doxycycline and rifampicin were administered, with intravenous gentamicin for the initial 1 week. The patient recovered without relapse, confirmed by the negative result of a Brucella agglutination test. The patient’s wife and three laboratory technicians were required to undergo blood examinations, which revealed no evidence of infection; however, they received prophylaxis with 3 weeks’ doxycycline and rifampicin. Conclusion: In nonendemic countries, immigrants with imported brucellosis can be treated, to prevent secondary brucellosis infection, an occupational hazard among laboratory technicians. Greater attention is needed for positive findings of blood cultures, which may initially be misidentified as O. anthropi. When providing medical care for immigrants with fever of unknown origin, it is especially important for primary care physicians to overcome language barriers so as to assess pertinent information regarding their home country, such as previous employment, to prevent the spread the imported zoonoses in the era of a dramatically increasing number of immigrants and foreign travelers.

Essential thrombocythaemia (ET) is characterised by elevated platelet count by a clonal stem cell disorder of megakaryocytes. Although thrombosis is a common complication of ET, splenic infarction (SI) is extremely rare. Here, we present the case of a 31-year-old Japanese man who presented with sudden-onset severe pain at the left hypochondrium on the day before admission. Enhanced abdominal CT revealed SI. The laboratory test results revealed a normal platelet count (439×10⁹/L). Subsequently, the patient was diagnosed with ET because the platelet count gradually increased to 50.0×10⁴/μL, and <italic>JAK2 V617F</italic> mutation was identified. Accordingly, low-dose aspirin was initiated, and no thrombotic episode occurred. Nevertheless, 6 months postdischarge, the platelet count gradually increased to &gt;650 × 10⁹/L, and anagrelide was initiated. This case demonstrates an unusual complication of acute SI due to ET under the rare situation of the normal platelet count.

BACKGROUND: Systemic vasculitides constitute heterogenous conditions affecting many organs and systems through blood vessel inflammation. Although there are some classifications for vasculitis, several vasculitides are “unclassified” because they cannot be clearly assigned to one of the known entities. CASE REPORT: We report an autopsy case of a 67-year-old Japanese man who presented with fever, ocular pain, erythema, chest pain, and headache. The disease caused episcleritis, thrombophlebitis, extensive deep vein thrombosis, multiple pulmonary nodules and masses, hypertrophic pachymeningitis, and hyper-intensity areas in brain parenchyma on magnetic resonance images. Histopathology of the pulmonary nodule confirmed vasculitis affecting medium-to-small veins and arteries without necrotizing vasculitis or granulomatous inflammation. We diagnosed the patient with unclassified vasculitis based on the clinicopathological characteristics. Steroids in combination with immunosuppressants were used, but the disease was refractory and relapsing. The disease activity was eventually controlled with rituximab, but the patient died of bronchopneumonia. On autopsy, lung and brain findings indicated healed vascular inflammation. CONCLUSIONS: This is the first case report of unclassified vasculitis, which is characterized as medium-to-small-sized arteritis and phlebitis, causing episcleritis, thrombophlebitis, deep vein thrombosis, pulmonary vasculitis, and intracranial vasculitis. The clinical conditions share some similarities with granulomatosis with polyangiitis and Behçet’s disease; however, they meet no classification criteria of any specific vasculitis. More cases need to be analyzed to confirm our findings.

Our case patient was a 38-year-old pregnant Japanese woman who underwent emergency Caesarean section because of massive vaginal bleeding due to a low-lying placenta. Immediately after delivery, she presented with rapidly progressive dyspnea. Contrast-enhanced computed tomography revealed bilateral pleural effusion, lung nodules, multiple liver tumors, and multiple osteolytic lesions. Accordingly, epidermal growth factor receptor-mutant advanced lung adenocarcinoma was diagnosed. This report highlights the occurrence of rapid progression of lung cancer following delivery that led to postpartum acute respiratory failure, rather than due to pulmonary thromboembolism associated with the existing deep venous thrombosis of the inferior vena cava.

We herein report a case of a 31-year-old Japanese man who simultaneously had a positive influenza A virus antigen test result and Vogt-Koyanagi-Harada disease (VKHD), demonstrated by both diffuse multiple early hyperfluorescent points on fluorescein fundus photography and serous retinal detachments on optical coherence tomography. He had meningitis. It was difficult to determine whether the main cause of meningitis was influenza A or VKHD. After initial treatment with peramivir for influenza A and then methylprednisolone pulse with subsequent corticosteroid therapy for VKHD, his symptoms improved gradually. These findings suggest that influenza A virus infection contributes to the onset or exacerbation of VKHD.

Common adverse effects of serotonin-norepinephrine reuptake inhibitors are nausea, dry mouth, dizziness and headache. We describe the case of a patient with dysosmia and subsequent dysgeusia associated with duloxetine. A 68-year-old Japanese woman with a history of type 1 diabetes mellitus, hypertension, insomnia and reflux esophagitis presented to a local hospital with bilateral leg pain; she was treated with duloxetine. However, after 4 weeks, she sensed rotten egg smell, experienced nausea and vomiting and was admitted to our hospital. We diagnosed dysosmia using the T&T olfactometer threshold test and dysgeusia using filter paper disk method. Taste was assessed using electrogustometry. We suspected that dysosmia and dysgeusia were adverse effects of duloxetine. After stopping duloxetine, her symptoms gradually subsided and the above test results improved, despite continuing the other ongoing medication. To the best of our knowledge, this is the first case report of dysosmia and dysgeusia associated with duloxetine.

Objectives: Symptomatic cerebral infarction (CI) can occur in patients without main cerebral artery stenosis or occlusion. This study investigated the unique features of carotid artery plaque and white matter disease (WMD) in patients with symptomatic CI and transient ischemic attack (TIA) but without stenosis or occlusion of a main cerebral artery. Patients and methods: We studied 647 patients who underwent both carotid ultrasound examination and brain magnetic resonance images. Plaque score (PS), plaque number, maximal plaque intima-media thickness and grades of WMD were examined. Subjects were divided into four groups, the CI group, TIA group, myocardial infarction (MI) group and risk factor (RF) group. Plaque and WMD were analyzed in cerebral ischemia group (CI and TIA), compared to non-cerebral ischemia groups and to a high PS group and a high WMD grade group from the RF group. Results: Both of each value of plaque and grades of WMD in the cerebral ischemia group were significantly higher than those in other groups. Grades of WMD in the cerebral ischemia group were significantly higher than those in the high PS group, although there was no significant difference of the each value of plaque between the two groups. The each value of plaque in the cerebral ischemia group was also significantly higher than those in the high WMD grade group, although there was no significant difference of grade of WMD between the two groups. Conclusion: Simultaneous increases in carotid artery plaque and WMD are associated with symptomatic CI, which is not caused by stenosis or occlusion of a main cerebral artery.

A 54-year-old Japanese man noticed painful swelling and redness of his left leg. He was admitted for treatment of cellulitis, which was accompanied with increased anti-streptolysin O and anti-streptokinase titers in his clinical course. After Piperacillin/Tazobactam administration, the skin lesion resolved. However, the patient then developed arthritis, palpable purpura, and intermittent abdominal pain, later found to be secondary to a severe duodenal ulcer. He was diagnosed with cellulitis-associated anaphylactoid purpura and was given prednisolone, which dramatically improved his symptoms. The anaphylactoid purpura was likely caused by<italic> Streptococcus</italic>-induced cellulitis, which was successfully treated with prednisolone. Association between these diseases is rare.

The causes of chronic unilateral leg swelling include common manifestations such as venous insufficiency, varicosis, deep vein thrombosis, and persistent cellulitis, less commonly are secondary lymphedema (tumor, radiation, and surgery), pelvic tumors or lymphoma causing pressure on the veins, and reflex sympathetic dystrophy, and rarely congenital venous malformation, pregnancy, and iliac vein compression syndrome. Here, we describe a 78‐year‐old nonsmoking man with history of type 2 diabetes mellitus, dyslipidemia, and hypertension presented with left leg swelling that had been getting worse over several months.

Background: When an internal carotid artery (ICA) occludes, a patient may develop cerebral infarction (CI). We investigated whether CI caused by ICA occlusion (ICAO) is associated with collateral flow through the anterior and posterior communicating arteries (ACoA and PCoA). Methods: In 100 patients with ICAO, we investigated CI and white matter disease by performing an MRI and the anatomy of the ACoA and PCoA were investigated by performing magnetic resonance angiography. All patients were divided into the symptomatic CI group or the no-CI group. The collateral flow pathway was estimated by the anterior cerebral artery (ACA)-PCoA score and the collateral flow volume after ICAO was estimated by the middle cerebral artery (MCA) flow score, based on how well the MCA was visualized. Results: Of 100 patients with ICAO, the symptomatic CI group included 36 patients. ACA-PCoA score and white matter disease grades were significantly higher in the CI group (indicating poor collateral flow). More than 80% of patients with an ACA-PCoA score of 4 (poor collateral) experienced symptomatic CI. Thirty-one symptomatic CI patients (86%) had an MCA flow score of 1 or 2 (decreased MCA flow). Conclusion: The ACA-PCoA score and white matter disease grade may suggest an increased risk of CI following ICAO.

In pancreatic β-cells, glucose-induced closure of the ATP-sensitive K+ (KATP) channel is an initial process triggering glucose-stimulated insulin secretion (GSIS). This KATP-channel dependent pathway has been believed to be a central mechanism for GSIS. However, since the resting membrane potential of cells is determined by the balance of the net result of current amplitudes in outward and inward directions, it must be taken into consideration that not only KATP channel inhibition but also inward current via the basal opening of non-selective cation channels (NSCCs) plays a crucial role in membrane potential regulation. The basal activity of NSCCs is essential to effectively evoke depolarization in concert with KATP channel closure that is dependent on glucose metabolism. The present study summarizes recent findings regarding the roles of NSCCs in GSIS and GTP-binding protein coupled receptor-(GPCR) operated potentiation of GSIS.

We report the case of a 71-year-old Japanese woman with adult-onset Still’s disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and<italic>Pneumocystis jirovecii</italic>pneumonia. After these were resolved, AOSD-associated MAS was well controlled. She was discharged on hospital day 87. Although biologics such as TCZ are becoming established for the treatment of AOSD, there is no recommended therapy for AOSD-associated MAS. Several biologics have been tried for this complication, but their efficacy and safety remain controversial. We reviewed reported cases of AOSD-associated MAS successfully treated with various biologics. TCZ initiation after adequate nonselective immunosuppressive therapy, such as methylprednisolone pulse therapy or a prednisolone-based combination of immunosuppressants, can be an effective treatment for AOSD-associated MAS. On the other hand, biologics given after insufficient immunosuppressive therapy may cause MAS. A strategy combining adequate immunosuppression and a biologic could be safe if special attention is given to adverse events such as opportunistic infections or biologic-associated MAS.

An autopsy of a 70-year-old man with multiple bone metastases from a malignancy of unknown origin (MUO) and renovascular hypertension revealed an aortic intimal sarcoma (AIS) in the right renal artery accompanied by atherosclerotic changes. AIS appeared as aggregated mutton fat-like translucent particles arising from the intima of the branching portion of the right renal artery and was composed of undifferentiated, fine spindle cells with thicket-like proliferation. AIS was confirmed by immunohistopathology, showing the loss of the lumen lined by CD31-positive endothelium and the expression of CD31, keratin, and vimentin in the viable part of the tumor. In patients with MUO presenting with both bone metastases and an acute or sub-acute onset of renovascular hypertension, AIS in the renal artery may be responsible.

A D-shaped left ventricle (D-LV), predominantly during systole, on a short axis view of two-dimensional echocardiography indicates pulmonary hypertension(PH). Here we describe a rare case involving a 35-year-oldmale smoker who developed typical D-LV secondary to fatal pulmonary Langerhans cell histiocytosis(PLCH)-related PH (PLCH-PH). To the best of our knowledge, there is no article to exhibit the image of D-LV caused by PLCH -PH.

42歳の男性。15歳時に管後型大動脈縮窄症で胸部下行大動脈の人工血管置換術を受けた。胸部造影CTで人工血管置換部周囲に低吸収域を認め，人工血管感染の疑いで転院搬送された。ガリウム・シンチグラフィで人工血管置換部周囲に集積を認めた。前医の血液培養からブドウ球菌が検出された。メチシリン耐性黄色ブドウ球菌や嫌気性菌の複合感染も考慮し，血液培養提出後にバンコマイシン，ピペラシン・タゾバクタム，ゲンタマイシンを開始した。原因菌がメチシリン感受性黄色ブドウ球菌と判明したため，セファゾリンへ変更した。転院時の血液培養は陰性であった。人工血管再置換術を施行せず，計８週間の抗菌薬静脈内投与実施後に退院し，さらにセファクロル内服を６か月間継続し終了した。治療終了後６か月間以上の経過観察で再燃を認めていない。大動脈人工血管感染症の死亡率は高く，再置換術を要したとの報告が多い。本症例のような大動脈人工血管感染症であっても，適切な抗菌薬の長期治療で人工血管再置換術を回避できる。

A 51-year-old Japanese woman developed candidemia as an outpatient secondary to a Candida albicans upper urinary tract infection complicated by previously undiagnosed type 2 diabetes mellitus with poor glycemic control and ureterolithiasis. The patient did not have any risk factors typically associated with candidemia, such as an indwelling vascular catheter, parenteral nutrition or broad-spectrum antibiotic use. During the clinical course, her condition was complicated by unilateral candida endophthalmitis, which progressed despite the administration of systemic antifungal agents and ultimately required vitreous surgery. The etiology of candidemia in this patient and the reason she developed progressive ocular symptoms after starting antifungal treatment are reviewed.

症例は54歳の男性。41歳の時に糖尿病と診断され，51歳からインスリン治療が行われていた。血糖コントロールは不良で糖尿病性網膜症，足壊疽を併発していた。右趾壊疽の治療中に，脱力感，左下腿と右手関節の腫脹と疼痛のため緊急入院した。左下肢の深部軟部組織感染病巣と血液培養からＡ群溶血性レンサ球菌を検出し，ショックを伴っていたため，Streptococcal Toxic Shock-like Syndrome（STSS）と診断した。その後，壊死性筋膜炎，腸腰筋膿瘍，化膿性脊椎炎を順次合併した。しかし本症例では，総合診療科のコーディネートによって，多職種との連携を逐次迅速に行うことができた。その結果，救急部からの迅速な受け入れ，専門医による観血的検査・治療の適否と実施タイミングの決定や適切な呼吸循環管理，適切な抗菌薬の選択と血糖コントロールを行うことができ，救命・救肢および自宅退院を可能とした。このように，糖尿病患者に発生したSTSSの治療にあたっては，総合診療科の存在が患者の予後を改善する可能性がある。

症例は73歳女性，右利き。以前より軽度の記銘力低下があったが，生活は自立していた。既往に大動脈弁置換と心房細動があったが，ワーファリンを怠薬していた。ふらつきと視覚異常を主訴に来院した。上方水平半盲，色覚異常，体幹失調を認め，頭部MRIで両側後頭葉下部と左小脳虫部に梗塞像を認めた。保存的加療で体幹失調と色覚異常は改善した。第８病日よりリハビリテーション室で歩行訓練を開始したが，一人で帰室できなかった。高次脳機能評価で相貌失認を伴わない街並失認が示唆された。 街並失認が改善するまでの外出時付き添いを家族に指導したところ，退院後６ヶ月で自宅付近では独歩 可能となった。街並失認はベッドサイド診察で気付くのは困難であり，特に軽度記銘力低下例では見過 ごされている可能性がある。右後頭葉病変を有する患者では積極的にその存在を疑い，適切な評価と助 言を行うことが重要である。

(1)LDHは多くの組織に存在し疾患特異性は低い(2)LDHアイソザイムを測ることで肝臓、心筋、悪性腫瘍などどの組織由来かの推測が可能となる(3)LDH以外の検査データも参照して、病態を考える(4)LDHがほかの検査値に比較して極端に上昇している場合やLDH増加スピードが速い場合には悪性腫瘍を強く示唆する(著者抄録)

In pancreatic beta-cells, closure of the ATP-sensitive K+ (K-ATP) channel is an initial process triggering glucose-stimulated insulin secretion. In addition, constitutive opening of background nonselective cation channels (NSCCs) is essentially required to effectively evoke depolarization as a consequence of K-ATP channel closure. Thus, it is hypothesized that further opening of NSCC facilitates membrane excitability. We identified a class of NSCC that was activated by exendin (ex)-4, GLP-1, and its analog liraglutide at picomolar levels. This NSCC was also activated by increasing the glucose concentration. NSCC activation by glucose and GLP-1 was a consequence of the activated cAMP/EPAC-mediated pathway and was attenuated in TRPM2-deficient mice. The NSCC was not activated by protein kinase A (PKA) activators and was activated by ex-4 in the presence of PKA inhibitors. These results suggest that glucose- and incretin-activated NSCC (TRPM2) works in concert with closure of the K-ATP channel to effectively induce membrane depolarization to initiate insulin secretion. The current study reveals a new mechanism for regulating electrical excitability in beta-cells and for mediating the action of glucose and incretin to evoke insulin secretion, thereby providing an innovative target for the treatment of type 2 diabetes.

Parsonage-Turner syndrome (PTS), also known as neuralgic amyotrophy or brachial neuritis, consists of a complex set of symptoms including sudden onset shoulder pain, usually unilaterally, followed by progressive neurologic deficits such as motor weakness, dysesthesia and numbness. We report a case of a 45-year-old Japanese man with PTS, who was initially suspected of having orthopedic diseases, such as cervical spondylosis and suprascapular nerve entrapment syndrome. The patient presented with acute-onset severe pain in his right arm and neck, followed by muscle weakness. We diagnosed PTS by taking a detailed medical history, ruling out other differential diagnoses, and by considering the characteristic clinical symptoms and the denervation pattern identified by needle electromyography. High-dose corticosteroid therapy prednisolone (PSL) relieved the initial sharp pain and oral amitriptyline successfully relieved the residual nighttime pain. Doses of PSL and amitriptyline were tapered, pain was alleviated, and the patient returned to full-time work after discharge in one-and-a-half years. For prompt diagnosis, physicians should consider the possibility of PTS in patients with acute onset of severe arm pain or subsequent muscle weakness and muscle atrophy in the arm or shoulder girdle.