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症例は62歳の男性. 輸血歴なく, 飲酒歴なし. 昭和61年, 肝障害で近医に入院歴あり. このときHBs抗原は陰性であった. 肝庇護療法のみで肝機能は改善し, 以後7年間自他覚症状なく経過していた. 通院観察中もトランスアミナーゼは正常値を持続した. 平成6年2月, GPT164IU/lと肝障害再燃を認め当科入院. HBs抗原陰性, HCV抗体 (第二世代) 陰性で, 抗核抗体は20, 480倍と高力価を, Ig-Gは3, 458mg/dlと高値を示した. 腹腔鏡下肝生検では肝表面は軽度不整を認め, 組織像では形質細胞浸潤を伴う慢性肝炎, 活動性の像を呈した. 上記より自己免疫性肝炎と診断し, プレドニゾロン投与にて肝機能は速やかに改善した. 本症は当初, 非B型肝障害と診断され, 無治療にも拘らず7年もの長期間, 無症候性に経過した後に再燃したと考えられた. 自己免疫性肝炎の発症様式を考える上で, また男性例であることから示唆に富むと思われ報告した.

16歳の若年女性でtoxic epidermal necrolysis (TEN) 治療中に, 高度な急性肝内胆汁うっ滞を発症. 種々内科的治療に抵抗性にて, 難治性慢性肝内胆汁うっ滞へ移行し, 約3年の短期間に続発性胆汁性肝硬変症に進展し, 死亡した極めて稀な例を経験した. 本症例はSherlockらの提唱するthe disappearing intrahepatic bile ductsの範躊にはいるが, 臨床的にprimary biliary cirrhosis (PBC), Alagille病, Byler病などは否定的であった. アセトアミノフェンの服用歴とLST陽性やTEN様の皮疹, ステロイドの中止後胆汁うっ滞の悪化を認めたことより, 薬剤アレルギーが病態の背景にあり, 肝内胆管の潜在的なhypoplasiaやprimary screlosing cholangitis (PSC) の存在が病態の増悪因子となった可能性が示唆された, 残された治療選択は肝移植のみと考えられたが, 高度な胆汁うっ滞を主とした病態においては, 経過中にその適応および時期の適切な判断を下すことは難しく, 類似症例の参考となると考えられ報告した.

A 63-year-old woman with multiple disturbances of the cranial nerves is reported. She initially complained of bilateral hearing loss and otorrhea, followed by right facial palsy and vertigo and was hospitalized on August 25, 1983. Despite steroid therapy, her condition deteriorated and finally complete bilateral deafness and complete bilateral facial palsy developed.Histologial examination of the mastoid mucosa, obtained by exploratory tympanotomies in both ears, showed non-specific inflammation. Noteworthy findings or laboratory examinations were extremely high levels of ESR and CRP, positive RA test, and negative tuberculin test.Tracheotomy was performed because of recurrent bilateral nerve palsy. Scleritis occurred in March, 1984, and the disturbances of eye movement, ptosis and atrophy of tongue developed in September, 1984. Long-term steroid therapy did not improve cranial palsy. There were no neurological signs such as pathological reflex in the limbs, involuntary movement or meningeal irritation symptom. There were no remarkable findings on CT or MRI.Although treatment by a local hospital continued, the III, IV, VI, VII, VIII, X and XII cranial nerves remained paralyzed. She finally died of systemic weakness in March, 1989.This was a rare case that began with bilateral otitis media and proceeding to paralysis of initially occurred and cranial nerves in order of VII, VIII, X, XII, III, IV and VI nerves. The background of this case included systemic vascular disease, especially Cogan's syndrome, because of otologic and ocular symptoms and multiple disturbances of the cranial nerves.

A 52-year-old housewife with a chief complaint of vertigo and right tinnitus, suddenly fell into a coma 12 days after admission and died in 24 hours. Autopsy revealed massive hemorrhagic necrosis of both adrenal cortices, disseminated intravascular coagulation in various organs, and otic meningitis.These findings suggested the possibility that otic meningitis caused WaterhouseFriderichsen syndrome.The purpose of this case report is to describe Waterhouse-Friderichsen syndrome due to otic meningitis and to call attention to the early signs of impending neurotologic complications.

The basal cell nevus syndrome (Gorlin-Goltz syndrome) is an autosomal dominant disorder. It is characterized by multiple basal cell nevi, multiple odontogenic keratocysts and skeletal abnormalities (ex. bifurcated rib, scoliosis).Few reports of its clinical features have been published in the oto-rhino-laryngological literature. The diagnostic criteria are not yet clear, except in patients with co-existing skin anomalies and keratocysts. A 20-year-old male visited our clinic with a chief complaint of pain and swelling around the right bucca. He had multiple jaw cysts with an impacted tooth, cysts in both maxillary sinuses with an impacted tooth, protrusion of the frontal bone, a broad nasal root, increased distance between the inner canthi of the eyes, and mental retardation.The histologic diagnosis of multiple jaw cysts and maxillary cysts was odontogenic keratocysts. So far, there has been no recurrence since the operation.

We report a case of pleomorphic adenoma which was assessed pre-operatively as arising from the accessory parotid gland. Computed tomography (CT)-sialography provided information about the relationship between the tumor and the remaining normal accessory parotid tissue, and fine-needle aspiration of the tumor provided additional information as to the histologic features of the tumor, i.e. it was identified as of salivary origin. Zygomatic and buccal branches of the facial nerve extended over the accessory parotid gland tumor and the parotid duct was located just beneath the tumor. Therefore, pre-operative assessment of the tumor's nature was important for prevention of facial nerve damage and salivary fistula. The normal accessory parotid gland tissue associated with the accessory parotid tumor was histologically different from that of normal parotid gland tissue dissected in other patients with pleomorphic adenoma of the parotid gland. It is suggested that the difference in cell composition between these glands affects the development of the associated tumors.

Nasal and paranasal papillomas are rare benign tumors and are known with frequent local recurrence and occasional malignant change.The pathogenesis of these papillomas is not known, but recently a viral etiology has been proposed.Only a few ultrastructural observations of these papillomas have been reported by Gaito, R.A. et al. (1965), Jahnke V. (1971) etc.We studied papilloma in two patients ultrastructurally.Both patients were complaining of bilateral nasal obstruction, and a 68-year-old male had undergone removal of nasal papilloma twenty years ago and a 64-year-old male had nasal polypotomy three times in the past. Both were operated by Denker's method, and the specimens were taken at the time of biopsy and operation. Light microscopic findings were compatible of inverted papilloma. Specimens were immediately fixed in ice-cold buffered glutaraldehyde (Karnovsky) solution and post-fixed in 2 % osmium tetroxide solution buffered with cacodylate, then dehydrated in alcohol and embedded in Epon 812.Sections were cut and stained with toluidine blue for light microscopy.Ultrathin sections for electron microscopy were cut on a Porter-Blum Ultrotome. These were stained with uranyl acetate and lead acetate.Hitachi HU 12 type microscope (100 kV), and HU 11 B (75 kV) were used for examination. Electron microscopic findings were as follows:Case 1 : Superficial layer of flat cells and in some regions ciliated columnar cells were observed. Junctions between adjacent epithelial cells were consisted of cytoplasmic processes with desmosomes.Intermediated cells were polygonal in shape and had large nuclei and two or three nucleoli. The deeper layer of these cells were elongated and showed many mitosis and rested on discontinuous or obscure basement membrane. Peg like cytoplasmic process protruded into stroma. The intercellular space was gradually dilated in deeper layer.The cytoplasms of these tumor cells contained mitochondria, rough surfaced endoplasmic reticulum and abundant tonofilaments and a dense body in some regions.We could not demonstrate intranuclear virus like particles.Case 2 : Superficial layer was covered with flat cells, and intermediated cells had no atypia, mitosis, virus like particles, and the basement membrane was intact.It was thought that peg like process of basal cells indicated easy recurrence of this tumor, and slight atypia, mitosis and discontinuous basement membrane suggested precancerous condition. So we emphasized the utility of electron microscopical observation for differentiating nasal and paranasal papillomas either precancerous or not.

The etiology of vocal cord dyskinesia was studied histopathollogically in 70 specimens with laryngeal carcinoma.The results revealed that in 62.5% of the cases with vocal cord immobilisation, it was caused by the invasions of the cancer into intrinsic laryngeal muscles and in 37.5%, it was caused by the destructive changes of the arytenoid cartilage and the cricoarytenoid joint. In the cases with invasion to the intrinsic laryngeal muscles, the tumors were advanced to the oblong fossa of the arytenoid cartilage. The perichondrium of the arytenoid generally showed fibrotic hypertrophy. The marked fibrosis and the poor exudative and vascular reactions were observed in the stroma at the tumor apex.When the tumor expanded backward, it was found that the anterior aspect of the cricoarytenoid joint was first destructed, and that the posterior aspect of the arytenoid cartilage was first destructed.

The clinical effects of MS-A nebulizer therapy were evaluated in 23 patients with nasal hypersensitivity: 13 with nasal allergy (NA) and 10 with vasomotor rhinitis (VMR). The patients received 2 ampules once or twice a week for a total of 8 times.The efficacy rate was 61.5% for NA and 60.0% for VMR. The rate of improvement in nasal congestion was 46.2% for NA and 60.0% for VMR. Rhinorrhea decreased in 61.5% of these with NA and 70.0% of those with VMR. Percentages of patients showing a decrease in the number of sneezing attacks was 61.5% for NA and 57.1% for VMR. In half of the patients, the effects of MS-A nebulizer therapy were noted by the 5th application. There was no side effect observed in any subject.MS-A nebulizer therapy was considered to be effective for the treatment of nasal hypersensitivity.