Ultrastructure of Kaposi's sarcoma in acquired immune deficiency syndrome (AIDS)

We used electron microscopy to examine the ultrastructural morphology of Kaposi's sarcoma (KS) of the oral mucosa in patients with acquired immune deficiency syndrome (AIDS). The tumors manifested endothelial vascular proliferation and neoplastic spindle cell formations. The endothelial tumor cells contained several multivesicular bodies and a large number of tubuloreticular structures within the endoplasmic reticulum. Ultrastructural changes in the other cytoplasmic organelles included defective cell junctions that seemed to facilitate the migration and extravasation of erythrocytes through endothelial gaps, after which erythrophagocytosis occurred. Tumor cells contained viral particles which were 100-120 nm in diameter and contained dense cylindrical cores. We believe that this is the first time these particles have been identified in KS cells of the oral mucosa in patients with AIDS. These viral particles are also ultrastructurally identical to the human T-cell lymphotropic retroviruses subgroup HTLV-III. Our observations are discussed in the light of recent immunological findings.     

Ultrastructure of Kaposi's sarcoma in acquired immune deficiency syndrome (AIDS)

Summary We used electron microscopy to examine the ultrastructural morphology of Kaposi's sarcoma (KS) of the oral mucosa in patients with acquired immune deficiency syndrome (AIDS). The tumors manifested endothelial vascular proliferation and neoplastic spindle cell formations. The endothelial tumor cells contained several multivesicular bodies and a large number of tubuloreticular structures within the endoplasmic reticulum. Ultrastructural changes in the other cytoplasmic organelles included defective cell junctions that seemed to facilitate the migration and extravasation of erythrocytes through endothelial gaps, after which erythrophagocytosis occurred. Tumor cells contained viral particles which were 100–120 nm in diameter and contained dense cylindrical cores. We believe that this is the first time these particles have been identified in KS cells of the oral mucosa in patients with AIDS. These viral particles are also ultrastructurally identical to the human T-cell lymphotropic retroviruses subgroup HTLV-III. Our observations are discussed in the light of recent immunological findings.This work was supported by the Austrian Foundation for Cancer Research (Fonds Österreichische Krebsforschungsinstitute) and by the Tumor Board of the Medical Faculty of the University of Vienna (Krebsausschuß der Medizinischen Fakultät der Universität Wien)     

Characterization of a chemotactic defect in patients with Kartagener syndrome

Kartagener syndrome (KS) is an autosomally inherited recessive condition characterized by situs inversus, bronchiectasis, and chronic sinusitis. Ciliary dynein, the mechanochemical force generator in ciliary movement, is deficient in patients with KS. We examined blood samples from two patients and tissue biopsy specimens from five patients and found: (1) no significant defect in neutrophil or monocyte chemotaxis in response to formylpeptide chemoattractant; (2) no alterations in centriolar structure, but significantly more centriole-associated microtubules in KS neutrophils and monocytes than in control leukocytes; and (3) a marked reduction in KS fibroblast chemotaxis in response to fibronectin compared with control fibroblasts. The significance of these cellular defects in KS is described.     

Virus-like particles in human vestibular ganglion cells

We found intracytoplasmic aggregates of virus-like particles in human vestibular ganglion cells. These particles were always observed in the peripheral area of the cytoplasm. Morphological characteristics of the cytoplasm are similar to those of other ganglion cells. The inclusion bodies are round and measure about 1.7 micron in maximum diameter. They consist of a porous convoluted dense material and virus-like particles. The overall diameter of 118 randomly selected particles varies from 36 nm to 73 nm, and the mean value is 53 nm. Most of the particles are spherical while a few possess a hexagonal or semicircular profile. The particles exhibit a double external membrane or vesiculated external layer. Most of the particles are empty. There are, however, some particles which show vesicular structures in their content. Although our present data are insufficient to determine these particles as viral, their hexagonal shape and size are similar to true virus. With these data in mind, we suggest that these particles might be a dormant form of virus and may possibly produce infectious disease in the inner ear.     

Normal sense of smell in Kallmann syndrome. A case report

BACKGROUND: Kallmann's syndrome (KS) was first mentioned in 1944 as an association of anosmia and hypogonadotropic hypogonadism. Causes are multiple genetic defects the most common of which is the x-linked KS appearing mostly in men. However, autosomal dominant and autosomal recessive forms have also been described. PATIENT: We present a case of KS with normosmia (male, 39 years of age). All symptoms of hypogonadotropic hypogonadism were present. RESULTS: Psychophysical olfactory testing revealed left-sided anosmia with right-sided normosmia which was confirmed by electrophysiological measures of olfactory function. Magnetic resonance imaging indicated aplasia of the left olfactory tract and bulb, whereas the right-sided structures appeared to be normal. CONCLUSIONS: As indicated in this case with lateralized anosmia and contralateral normosmia, overall olfactory function strongly depends on the "best" nostril. Therefore, in many clinical situations, lateralized olfactory testing appears to be extremely important.     

Oropharyngeal kaposi sarcoma in related persons negative for human immunodeficiency virus

OBJECTIVES: Kaposi sarcoma (KS) is a vascular tumor that can affect the mucosa of the upper aerodigestive tract. Although KS is the most common malignancy in patients with acquired immunodeficiency syndrome, it is rare in immunocompetent persons. We describe an unusual presentation of KS in 2 related individuals and describe our attempts to determine whether oropharyngeal KS is associated with human herpesvirus 8 (HHV-8). METHODS: All relevant clinical and surgical information, including information on tumor histopathologic and human immunodeficiency virus (HIV) serologic tests, was abstracted from the patient charts and electronic databases. HHV-8 immunohistochemistry was performed on paraffin-fixed specimens. RESULTS: Both patient 1 and patient 2 (the nephew of patient 1) were referred for workup of a tonsillar mass that was pathologically confirmed to be KS. In each case, HIV serologic testing was negative, and a screening immunologic workup, including a quantitative natural killer cell count, a B- and T-lymphocyte count, and immunoglobulin analysis, also yielded findings that were within normal limits. Immunohistochemistry performed on 1 pathological specimen showed positive staining for the presence of HHV-8, the etiologic agent of KS. CONCLUSIONS: The presence of oropharyngeal KS in 2 related HIV-negative individuals supports a role for genetic factors in susceptibility to KS, a common exposure to an infectious agent such as HHV-8, or both. Whereas most KS cases in industrialized countries are associated with immunodeficiency, clinical and laboratory data do not suggest that either of the patients described in this report are immunodeficient. Their susceptibility to KS may be secondary to a subtle inherited defect in host resistance to HHV-8, or another unknown factor.     

Virus-like particles in human vestibular ganglion cells

Summary We found intracytoplasmic aggregates of virus-like particles in human vestibular ganglion cells. These particles were always observed in the peripheral area of the cytoplasm. Morphological characteristics of the cytoplasm are similar to those of other ganglion cells. The inclusion bodies are round and measure about 1.7 m in maximum diameter. They consist of a porous convoluted dense material and virus-like particles. The overall diameter of 118 randomly selected particles varies from 36 nm to 73 nm, and the mean value is 53 nm. Most of the particles are spherical while a few possess a hexagonal or semicircular profile. The particles exhibit a double external membrane or vesiculated external layer. Most of the particles are empty. There are, however, some particles which show vesicular structures in their content. Although our present data are insufficient to determine these particles as viral, their hexagonal shape and size are similar to true virus. With these data in mind, we suggest that these particles might be a dormant form of virus and may possibly produce infectious disease in the inner ear.     

Synthesis of glycocalyx and associated structures in vestibular sensory cells

The ultrastructure of the glycocalyx with special reference to the synthesizing process was studied in the guinea pig vestibular sensory cells using the tannic acid staining technique. The glycocalyx emerged from the outer layer of the plasma membrane covering the entire length of the cilia. This glycocalyx also interconnected the ciliary structures tightly, such that a structural continuity was established between actin-membrane links and the glycocalyx. Interconnections between the actin filaments themselves were also noticed in the stereocilia as well as interconnections between individual actin filaments and the plasma membrane. These findings indicate that the glycocalyx and the ciliary interconnections may be closely related to the sensory hair transduction system. In the cellular cytoplasm, vesicles seemingly related to the synthesis of the glycocalyx were observed. These coated vesicles, which were synthesized by the Golgi complex and endoplasmic reticulum, interacted with the plasma membrane forming a coated pit. The lysosomal-like bodies also observed in the cell were closely related to the glycocalyx as well. Thus the glycocalyx seems to be synthesized by the endoplasmic reticulum and Golgi complexes and transferred through the coated vesicles or lysosomal-like bodies to the apical plasma membrane.     

Imaging by Atomic Force Microscopy of the Plasma Membrane of Prestin-Transfected Chinese Hamster Ovary Cells

The high sensitivity of mammalian hearing is achieved by amplification of the motion of the cochlear partition. This cochlear amplification is thought to be generated by the elongation and contraction of outer hair cells (OHCs) in response to acoustical stimulation. This motility is made possible by a membrane protein embedded in the lateral membrane of OHCs. Although a fructose transporter, GLUT-5, was initially proposed to be this protein, a later study identified the gene of the motor protein distributed throughout the OHC plasma membrane. This protein has been named “prestin.” However, although previous morphological studies by electron microscopy and atomic force microscopy (AFM) found the lateral wall of OHCs to be covered with 10-nm particles, believed to be motor proteins, it is unknown whether such particles consist only of prestin or are a complex of GLUT-5 and prestin molecules. To determine if the 10-nm particles are indeed constituted only of prestin, plasma membranes of prestin-transfected and untransfected Chinese hamster ovary (CHO) cells, which do not express GLUT-5, were observed by AFM. First, the cells attached to a substrate were sonicated so that only the plasma membrane remained on the substrate. The cytoplasmic face of the cell was observed by the tapping mode of the AFM in liquid. As a result, particle-like structures were recognized on the plasma membranes of both the prestin-transfected and untransfected CHO cells. Comparison of the difference in the frequency distribution of these structures between those two cells showed approximately 75% of the particle-like structures with a diameter of 8–12 nm in the prestin-transfected CHO cells to be possibly constituted only by prestin molecules. Our data suggest that the densely packed 10-nm particles observed on the OHC lateral wall are likely to be constituted only of prestin molecules.     

与人类免疫缺陷病毒相关性耳鼻咽喉部卡波济肉瘤21例报告

报告２１例与人类免疫缺陷病毒（ＨＩＶ）相关性耳鼻咽喉部卡波济肉瘤，其主要局部表现是鼻出血、鼻塞、咽喉干燥、异物感及结节状新生物，并对ＨＩＶ感染与卡波济肉瘤的关系、病例特点及诊断依据进行讨论。     

Is there a relation between mastoid aeration and Körner’s septum?

The purpose of this study is to investigate the prevalence of Körner’s septum (KS) in temporal bones with varying degrees of pneumatization and to evaluate any relationship between the degree of pneumatization and the presence of KS. Data were obtained retrospectively from 356 temporal bone high-resolution computed tomography of 178 patients who underwent tympanoplasty. Mastoid bone pneumatization was classified as aerated, diploic and sclerotic. The presence of Körner’s septum was also investigated. The relationship between the presence of Körner’s septum and the type of mastoid pneumatization was assessed. KS was encountered in 98 out of total 356 ears. Prevalence of KS was not statistically different between aerated (31%, n = 36), diploic (24.7%, n = 27) and sclerotic mastoids (26.9%, n = 35; p > 0.05). In conclusion, there is no relationship between the presence of KS and the degree of pneumatization. The presence of KS does not necessarily corroborate the presence of poor pneumatization.     

基于NK公式和KS公式预测ICL术后早期拱高的一致性研究

目的 探讨基于CASIA OCT的NK和KS公式预测有晶状体眼人工晶体术后拱高的一致性.方法 前瞻性病例研究.选取植入ICL矫正中高度近视患者25例(50眼).采用CAISA OCT分析术后1d、1周、1个月拱高(Vault)变化情况以及NK、KS公式的预测拱高值和实际拱高值的差异以及一致性.各时间点拱高变化数据采用单...     

Primary Kaposi’s sarcoma of the nasal cavity not associated with AIDS

Kaposi’s sarcoma (KS) is an unusual vascular tumor characterized by multiple reddish blue nodules, which usually present on the skin of the lower and upper extremities. KS may also involve mucosal sites, lymph nodes and visceral organs. During the last two decades, with the large increase in the incidence of this tumor associated with the acquired immune deficiency syndrome (AIDS), there have been increasing number of cases with KS presenting on the skin or mucosa of the head and neck. A review of the literature revealed that only six cases of primary KS of the nasal cavity have previously been published and only one of them presented in a patient not associated with AIDS. We report the case of a 59-year-old woman who presented 4 years ago with nasal obstruction and intermittent minor epistaxes. Physical examination revealed the presence of a fleshy tumor arising from the left nasal septum, which was excised. Histological examination of the tumor showed morphological and immunohistochemical features of KS. A complete physical and laboratory examination revealed no other pathological findings. The patient received no further treatment and 4 years later, she is in excellent condition. In the present study, we report the second case where the primary manifestation of the KS was in the nasal cavity in a patient with an adequate immune system.     

Kartagener's syndrome. A blinded, controlled study of cilia ultrastructure

We investigated respiratory mucosa cilia ultrastructure in patients homozygous for the gene for Kartagener's syndrome (KS) and patients apparently phenotypic for KS who had bronchiectasis and sinusitis but without situs inversus. Parents, as obligate carriers of the recessive KS gene, were also evaluated among other control groups. The four patients with KS had significantly fewer cilia outer dynein arms than normal subjects or parents of patients with KS. Two of five patients apparently phenotypic for KS demonstrated distinctive ultrastructural changes. No other subjects demonstrated explicit ultrastructural abnormalities. Internal control specimens showed that the number of outer dynein arms was consistent within a subject compared with variation between subjects. The outer dynein arm serves as a dependable ultrastructural marker. Carriers of KS do not demonstrate distinctive morphologic cilia abnormalities. Not every patient with chronic bronchiectasis and sinusitis demonstrates abnormal cilia ultrastructure.     

Electron microscopy of hyalin bodies in the human intraosseous endolymphatic sac

Hyalin bodies are amorphous, eosinophilic masses protruding from the subepithelial connective tissue into the lumen of the intraosseous endolymphatic sac (ES). We studied hyalin bodies at the electron microscopic level. Celloidin- embedded temporal bone sections known to have hyalin bodies were re-embedded into plastic and cut into thin sections appropriate for electron microscopy. The results revealed that the hyalin bodies are composed predominantly of thick bundles of collagenous fibers arranged in various directions. Fibroblasts and disintegrated macrophages were occasionally observed among the collagen fibers. Concentric calcific structures found within the hyalin bodies were composed of multiple smaller, concentric, lamellar calcifications. The results of this study support the hypothesis that the hyalin bodies are a repository of membranous cellular debris phagocytized by the macrophages.     

Ultrastructural abnormalities of the cilia in human nasal epithelia

Nasal mucosal cilia were observed with electron microscope in 14 patients with immotile cilia syndrome (ICS), 9 with nasal papilloma (NP), 23 with sinobronchial syndrome (SB), 2 with sinusitis combined with dextrocardia (SC), 1 with Kartagener's syndrome (KS), and 5 normal controls (C). Abnormalities such as complex cilia, cilia with abnormal axonemes and cilia with randomly oriented central microtubules were frequently found in the groups of ICS (8.1%) and NP (10.4%) while less in other groups: SB (4.9%), SC (5.3%), KS (4.7%) and C (3.9%). The percentage of cilia with defective dynein arms (DA) was the highest in the ICS group (94.0%), followed by the groups of SC (53.7%), SB (47.5%), NP (41.2%), C (35.8%) and KS (33.3%). The ICS group was found to be the largest in the number of defective DA per a cilium (4.1), followed by the groups of NP (1.0), SB (0.6), SC (0.7), KS (0.4) and C (0.4). Increased rates of defective DA were also recognized in cilia of tracheal mucosa and flagella of sperm in 7 patients with ICS examined. In conclusion, neither abnormal cilia nor defective DA of cilia are specific findings for ICS. However, when we observe these findings in high percentage in nasal mucosa as well as in other organs, we may define this condition as ICS.     

Local treatment of AIDS-associated bulky Kaposi's sarcoma in the head and neck region

Kaposi’s sarcoma (KS) is frequently seen in the head and neck regions of HIV-infected patients. We report two cases of patients with AIDS who consulted the ENT clinic. One patient came to our clinic complaining of abnormal sensations in the pharynx, and dysphasia due to a gross KS in the oropharynx. The excision of the tumor improved the difficulty of swallowing. The other patient complained of masticatory problems and tongue pain due to a bulky KS on the dorsal side of the tongue. We treated the tongue lesion with intralesional chemotherapy. The administration of intralesional vinblastine resulted in a partial response. Unless systemic chemotherapy is effective enough to improve a functional disorder, it is thought that local therapy employing excision or intralesional chemotherapy is one of the common therapeutic option of the otolaryngologist, because this treatment avoids severe side effects caused by systemic chemotherapy or radiotherapy.     

Presence of surfactant lamellar bodies in normal and diseased sinus mucosa

BACKGROUND: Pulmonary surfactant originates from phospholipid lamellar bodies secreted from the type II epithelial cell of the alveolus. In the lower airway, surfactant optimizes surface tension and oxygen exchange, decreases mucus viscosity and aids in mechanical elimination of inhaled pathogens. In addition to the lung, lamellar bodies have been identified in many other cell types throughout the human body. However, no prior studies have identified lamellar bodies in human sinus mucosa. OBJECTIVES: We performed ultrastructural studies to assess whether lamellar bodies are present in the human sinus in a variety of diseased and normal epithelium. METHODS: We biopsied sinus mucosa from 5 subjects, 1 each with allergic fungal sinusitis, eosinophilic mucin rhinosinusitis, cystic fibrosis, frontal sinus mucocele, and cerebrospinal fluid leak (healthy control). Mouse lung served as a positive control. Specimens were prepared using ferrocyanide-reduced osmium tetroxide and thiocarbohydrazide for fixation (R-OTO method) to avoid extraction of phospholipids during dehydration and were viewed with transmission electron microscopy. RESULTS: We identified lamellar bodies in the sinus mucosa of all patients. Additionally, preservation of mouse lung lamellar bodies confirms that the R-OTO method is a valid technique to preserve these structures. CONCLUSIONS: We describe a simpler, faster technique for identification of cellular phospholipid components than those used previously. Definitive identification of these lamellar bodies within ciliated pseudostratified epithelium of the upper airway indicates that surfactant may have a role in sinus function and pathophysiology.     

Three-dimensional visualizations of the inner ear hair cell of the guinea pig. A rapid-freeze, deep-etch study of filamentous and membranous organelles

The fine structure of the filamentous and membranous organelles in the stereocilia and in the cuticular plate of sensory hair cells from the guinea pig was examined using a rapid-freeze, deep-etch method. In fixed and unfixed tissue the outer surface of the plasma membrane of the stereocilia had numerous surface protrusions of various sizes and shapes, while the protoplasmic fractured face of the membrane had rather sparse intramembrane particles. Many tiny cross links were present between the adjacent actin filaments and between actin filaments and the plasma membrane of the stereocilia. Numerous fibrils radiating from the hair rootlet were attached to the peripheral actin filaments in the cuticular plate. The radiating fibrils differed from the tiny cross links which interconnected the adjacent, randomly-oriented actin filaments in the cuticular plate. These complex structures consisting of actin filaments in the hair rootlets, radiating fibrils, and peripheral actin filaments may play an important role in regulating stereociliary bending.     

Kaposi's sarcoma and community-acquired immune deficiency syndrome

Kaposi's sarcoma (KS), or idiopathic multiple hemorrhagic sarcoma, has heretofore been considered an indolent disease of the elderly, which also occurs in immunosuppressed hosts. Within the last two years, an epidemic of a community-acquired immune deficiency syndrome (AIDS) and disseminated KS has been reported in various population groups across the United States. The head and neck manifestations of KS in AIDS and our experience at UCLA are reviewed. Of 45 patients with AIDS, 18 (40%) had initial disease in the head and neck region. Most commonly, dermal lesions (44%), oropharyngeal lesions (39%), and cervical lymphadenopathy (33%) were noted. One patient had coexistent lymphoma. Six patients (33%) died of opportunistic infections. Head and neck lesions often herald more extensive disease. Early recognition of AIDS is important for complete patient evaluation as well as for personal safety.