Retinal emboli in patients with mitral valve prolapse

Small fibrin-platelet emboli caused by prolapsed mitral valves may cause retinal occlusive disease with a wide range of ophthalmic manifestations, including amaurosis fugax in young people, retinal or choroidal arteriolar occlusion, and retinal neovascularization (atypical Eales' disease). Six patients with retinal occusive disease underwent extensive noninvasive cardiac and systemic tests and were found to have prolapsed mitral valves. Patients with unexplained ocular emboli should be examined by a cardiologist for possible mitral valve prolapse.     

Eye findings in patients with phenylketonuria

Untreated phenylketonuria (PKU) is characterized by excretion of phenylpyruvic acid in the urine and mental retardation. Reported eye abnormalities include photophobia (probably due to the albinoid iris), cataracts, and corneal opacities. Eleven patients in an institutionalized population of approximately 1,000 mentally retarded patients had PKU. Cataracts were found in six patients. One patient had bilateral phthisis bulbi and one patient had monocular phthisis. No corneal opacities were found otherwise. All patients with cataracts were middle-aged or had a history of severe self-induced trauma or treatment with high dosages of thioridazine hydrochloride. Albinism is associated with anomalous chiasmal routing of optic fibers and abnormal, extraocular motility. The ocular hypopigmentation of patients with PKU did not have the same results; all seeing patients were orthophoric. Although eye abnormalities are common in PKU except for hypopigmentation, none can be considered a direct effect of the inborn error of metabolism.     

Laryngeal Kaposi's sarcoma in patients with AIDS

Over a period of 10 years 17 human immunodeficiency virus(HIV)-infected patients with laryngeal Kaposi's sarcoma were seen and treated at University College London Hospitals. All patients had advanced HIV disease. Their presentation was with symptoms of upper airway obstruction in the majority of cases and the diagnosis was made by fibreoptic examination of the larynx. Biopsy was associated with brisk haemorrhage in one patient, who required a temporary tracheostomy, and was not performed in the other 16 cases. The commonest site of laryngeal involvement was the supraglottis in 11 patients, with glottic lesions noted in eight patients: subglottic lesions were seen in only three. Treatment of laryngeal Kaposi's sarcoma was, in general, conservative, five patients received low dose radiotherapy to the larynx and 10 were treated with systemic chemotherapy for disseminated Kaposi's sarcoma. Laryngeal Kaposi's sarcoma did not contribute to patient mortality.     

Multifocal varicella-zoster virus leukoencephalitis in a patient with AIDS: MR findings

We describe a patient with AIDS who presented with an acute encephalitis caused by infection with varicella-zoster virus. The hemorrhagic, necrotizing encephalitis had an unusual MR appearance, with innumerable discrete, small, targetlike lesions in the right cerebral hemisphere, which were coalescent in the posterior temporal, parietal, and occipital regions. Of the several known disease patterns of varicella-zoster viral infection in the CNS, this histopathologic pattern of multifocal leukoencephalitis is rare. It is important to recognize, as effective antiviral drug treatments are available.     

Central venous catheters in patients with AIDS

Central venous catheters (CVCs) for patients with AIDS are at risk of a number of complications including bacterial infections. A 6-year retrospective review was undertaken of the records of the 33 patients (42% infected by injection drug use (IDU)) who received intravenous therapy both in hospital and at home via CVCs. Twenty-eight per cent of 53 insertions suffered a complication, the commonest of which was a pneumothorax (8%). The post insertion complication rate was 0.98/100 catheter days (cd). Thrombotic occlusion (0.15/100 cd) was the commonest non septic event while sepsis was overall the commonest event (0.69/ 100 cd) of which half were considered serious (0.33/100 cd). The most frequently isolated organisms were Staphylococci spp. (71%). The median time to an exit site infection was 59 days and to serious catheter sepsis 86 days. Infection did not differ significantly with age, gender, transmission risk activity or catheter type although Portacaths had the lowest rate of infection (0.33/100 cd). The median survival of the 53 CVCs was 88 days although if the temporary catheters were excluded it was 118 days. Kaplan-Meier estimates of survival analysis revealed 55%, 32% and 19% of all the CVCs surviving 3, 6 and 12 months respectively. Our experience suggests that home intravenous therapy and previous IDU does not preclude the use of CVCs although further research is needed on reducing the infection-related complications of such therapy.     

T-lymphocytopaenia, opportunistic infections and pathological findings in Ghanaian AIDS patients and their sexual partners

Ninety-nine patients at Center for Disease Control (CDC) clinical stage IV were studied. Twelve (12.12%) of these patients turned out to be HIV seronegative. Ten out of the 12 HIV negative patients were immunocompetent whereas the other two had proportional decreases in both CD4+ and CD8+ T-lymphocytes. HIV-1, HIV-2, and dual infection, were detected in 51.5%, 2%, and 22.2% respectively of clinical AIDS patients. The other 12.12% of clinical AIDS patients were indeterminate for HIV antibodies. All HIV positive patients with the exception of two, were immunocompromised with respect to CD4+ and CD8+ T-lymphocyte counts. Two healthy spouses and three children of patients who died from the disease were seronegative for HIV antibodies. Herpes simplex virus type 2 (HSV-2) and cytomegalovirus (CMV) antibody titres were higher in HIV infected than uninfected blood. Patients with chronic diarrhoea, lymphadenopathy, pneumonia, and tuberculosis, either alone or in combination of two or more of such symptoms, were found to be more likely to be confirmed by serology and immunology as definitive AIDS patients in Ghana. In postmortem studies on 20 patients, pneumonia due to tuberculosis constituted the major cause of death. Toxoplasmosis, cytomegaloviral eosophagitis and enteritis, and cryptococcosis were the major opportunistic infections detected. Programmed cell death (apoptosis) was found by the DNA gel electrophoresis method to be an unlikely major mechanism of accelerated culture induced death of PBMCs from CDC stage IV AIDS patients.     

Ocular findings in patients with neutrophil dysfunction

Of 32 patients (19 females and 13 males, ranging in age from 2 to 44 years), nine had ocular problems related to neutrophil dysfunction. Four patients had blepharokeratoconjunctivitis and pannus formation. In one of these, severe visual loss secondary to corneal thinning and scarring occurred. Five patients had inactive chorioretinal scars without visual loss. Although some of the other 23 patients had minor ocular abnormalities, we could not demonstrate that they were related to the neutrophil dysfunction. However, none of the control subjects (20 patients with systemic lupus erythematosus and 20 randomly selected eye clinic patients) had lesions resembling those of the patients with neutrophil dysfunction. Fisher's exact test (one-tailed) gave the following values: P less than .03 for keratitis; P less than .01 for chorioretinal scars; and P less than .001 for keratitis and chorioretinal scars. Abnormal neutrophil function probably interferes with the control of normal eyelid flora and predisposes the eye to the development of marginal keratitis. Eyelid hygiene and topical administration of antibiotics and corticosteroids during inflammatory episodes may prevent the progression of corneal vascularization. The chorioretinal scars do not appear to be progressive.     

Unique monocyte subset in patients with AIDS dementia

BACKGROUND: 15-30% of patients infected with HIV will develop a debilitating dementia. Whilst HIV enters the brain soon after infection, presumably within monocyte-derived macrophages, not all patients with HIV become demented. Blood monocytes probably cross the blood-brain barrier and give rise ultimately to parenchyma macrophages. We looked for a specific monocyte subset in AIDS patients with dementia. METHODS: Peripheral blood monocytes from three groups were compared: AIDS patients with (n = 12) and without (n = 11) dementia, and ten HIV seronegative healthy controls. We used flow cytometry to analyse monocytes, and cell lysis and apoptosis assays to examine monocyte effects on human brain cells in vitro. FINDINGS: We found a unique subset of monocytes in patients with AIDS dementia. These monocytes were more dense and granular and expressed CD14/CD16 and CD14/CD69. Means (SD) for CD14/CD16 in HIV-negative controls and in AIDS non-dementia and AIDS dementia patients were 6.5% (4), 16% (13), and 37% (21), respectively (p = 0.008 between the two groups of patients). The corresponding means for CD14/CD69 were 7% (6), 8% (10), and 69% (18) (p < 0.0001). INTERPRETATION: CD69 is a member of the natural-killer-cell gene complex that is expressed after activation. Supernatants from cultures containing these dense cells can trigger apoptosis of human brain cells in vitro. The monocyte subset we found in patients with AIDS dementia might enter the brain and expose neural cells to toxic factors.     

Cytomegalovirus retinitis in patients with AIDS in Europe. AIDS in Europe Study Group

The incidence of cytomegalovirus (CMV) retinitis and risk factors associated with the condition were studied in patients with the acquired immune deficiency syndrome (AIDS) in a multicenter retrospective cohort study of 6458 patients from 52 centers in 17 countries in Europe. Cytomegalovirus retinitis was diagnosed in 154 patients (2.4%) at the time of AIDS diagnosis, the probability of this diagnosis being significantly higher for those with CD4+ cell counts of < 100/mm3 (3.4%) than with counts of 100-200/mm3 (1.3%) or > 200/mm3 (0.8%). The rate of developing CMV retinitis after AIDS diagnosis was 9.4 per 100 patient years of follow-up. Multivariate analysis showed that risk behavior was significantly associated with the risk of developing CMV retinitis: lower for intravenous drug users [relative risk (RR) 0.47] and those engaged in "other risk behavior" (RR 0.58) than for homosexual men. The risk of developing CMV retinitis after AIDS diagnosis was significantly associated with CD4+ cell count at the time of AIDS diagnosis: for counts < 100/mm3 (RR 2.90) and from 100 to 200/mm3 (RR 2.13), there was a higher risk than for counts > 200/mm3. Patients with Pneumocystis carinii pneumonia, toxoplasmosis, or extraocular CMV infection at time of AIDS diagnosis exhibited an increased risk of developing CMV retinitis. Patients treated with zidovudine exhibited an increased rate of CMV retinitis: RR was 1.75 during and 2.87 after the second year of treatment as compared to those who had not received zidovudine. Median survival after CMV retinitis at time of AIDS diagnosis was eight months.     

CNS cryptococcosis in AIDS: spectrum of MR findings

Cryptococcus is a ubiquitous saprophytic fungus that may become pathogenic, particularly in immunosuppressed patients. In the CNS of AIDS patients, the incidence of this fungal infection is 5% of all the opportunistic infections. In this study, we review the MR findings in nine AIDS patients affected by CNS cryptococcosis. All MR studies were performed before and after intravenous administration of a single dose of gadopentetate dimeglumine and again after an additional dose. Autopsy was performed in one case. Magnetic resonance imaging revealed evidence of meningitis or meningoencephalitis, dilated Virchow-Robin spaces, cyst-like structures (gelatinous pseudocysts), and granulomas (cryptococcomas) of the choroid plexuses. The last two findings (pseudocysts and choroidal ependymal granulomas) are relatively specific for cryptococcosis.     

Etiology of lymphadenopathy in patients with AIDS in Taiwan

From 1986 to 1995, we retrospectively reviewed the records of 40 of 125 patients (32.0%) with acquired immunodeficiency syndrome (AIDS) who presented with extrainguinal lymphadenopathy. Most of the patients had an advanced stage of HIV infection with a mean CD4 lymphocyte count of 44/mm3. AIDS-defining opportunistic infections and malignancies were present in most patients and the neck region was the most common site of involvement. The etiology of lymphadenopathy was established in 26 patients. Tuberculous lymphadenitis was the most common cause, followed by lymphadenopathic Kaposi's sarcoma, benign reactive hyperplasia, cryptococcal lymphadenitis and disseminated Mycobacterium avium complex infection. Characteristic histopathologic findings were detected in 19 patients and 7 had presumptive tuberculous infections. The remaining 14 patients had no definitive etiology for their lymphadenopathy. As the causes are variable and the number of HIV/AIDS cases is increasing in Taiwan, more patients with lymphadenopathy, especially in the early stages of HIV infection will be encountered. Therefore, it is essential that diagnostic histopathologic and microbiologic studies be performed for appropriate and timely treatment.     

Electroencephalographic findings in patients with mucolipidosis type IV

To evaluate their prognostic value, the expressions of CD44v and sialyl LeX (SLX) in colorectal cancers were studied immunohistochemically. Tissue specimens were reacted with monoclonal antibodies (mAb) CD44-1V and CSLEX-1. Of the 145 colorectal cancer patients undergoing curative resection, 59 (40.7%) were positive for mAb CD44-1V, and 40 (27.6%) were positive for mAb CSLEX-1. There was a significant correlation between the combined expression of SLX and CD44v8-10 and lymph node metastasis. The patients with tumors negative for CD44v8-10 and SLX had the most favorable prognoses. Conversely, the patients with tumors positive for both CD44v8-10 and SLX had a high recurrence rate and the poorest prognoses. In a multivariate analysis using the Cox regression model, the combined expression of SLX and CD44v8-10 emerged as an independent prognostic indicator. These results suggested that the combined expression of CD44v8-10 and SLX may be a biologic marker of prognostic significance.     

Hepatobiliary diseases in patients with AIDS: focus on AIDS cholangiopathy and gallbladder disease

Since there have been very few studies on nucleolar signaling, an attempt was made to establish nucleolar signal pathways which link the cell membrane to the nucleolus for the transfer of extracellular signals. Two pathways were studied. One was the G alpha s mediated cAMP pathway where two signal molecules were yielded, including RII and protein kinase A. The other was the G alpha q mediated DAG/IP3 pathway which yields two signals including protein kinase C and IP3/Ca2+. By the studying isolated nucleoli from resting liver, regenerating liver or weak carcinogen thioacetamide treated liver, it was possible to detect protein kinase A (PKA), protein kinase C (PKC) and RII subunits. In addition, CK2 was detected. It was found that external signals transmitted through G protein coupled receptors could reach into the nucleolus and that physical translocation of signal molecules was an integral step involved in membrane-nucleolus linked pathways. When an in vitro assay of the above signal molecules was carried out using [gamma-32P]-ATP, most kinase dependent phosphorylation was via the major CK2 (more than 95%). Therefore, it is suggested that the major CK2 dependent pathway is involved in 'house keeping' for nucleolar integrity and the minor pathways, dependent on PKA, PKC and others, are involved in subtle regulatory mechanisms such as 'extra-house-keeping' activities by nucleolar chromosomal remodeling.     

Neurological complications caused by cytomegalovirus in patients with AIDS

In patients with HIV infection, disease due to CMV depends on reactivation of the virus. Such reactivation usually occurs at an advanced stage of the disease, when there is severe immunodepression and the CD4+ leukocyte count is < 100/ml. CMV infection is seen clinically as three syndromes: 1) encephalitis with or without associated meningitis and/or ventriculitis. 2) polyradiculomyelitis affecting the lumbosacral roots, and 3) multifocal senso-motor neuropathy. Diagnosis depends on showing the virus to be present in the CSF, by detecting the early CMV antigen or by conventional culture. There are marked differences between encephalitis, polyradiculomyelitis and multifocal neuropathy in the rentability of viral culture. Whilst in encephalitis CMV culture is negative in most patients, in polyradiculomyelitis the sensitivity of viral culture may be 50-60% and in multifocal neuropathy 15%. The treatment indicated is with ganciclovir or foscarnet. Results depend on the type of neurological disease, degree of involvement when treatment is started and an history of extracerebral CMV infection previously treated with these drugs.