The mitochondrial copper concentrations and cytochrome C oxidase activity of the fibroblasts from the patients with Menkes syndrome were investigated. Both the mitochondrial copper concentrations and cytochrome C oxidase activity of fibroblasts from patients with Menkes syndrome were lower than those of the control fibroblasts. These data indicate that the ...

A 4 1/2-year-old girl with acquired immunodeficiency syndrome and prolonged varicella zoster virus skin infection developed multiple ischemic strokes and radiologic and histopathologic evidence of central nervous system vasculitis. Typical features of acquired immunodeficiency syndrome encephalitis were not present and there was no evidence of vasculitis outside the nervous system. ...

A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with Kasabach-Merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and ...

The combination of pyrimethamine and sulfadoxine (Fansidar) has been reported to cause severe skin reactions including erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. Recently, this drug combination has been used for prophylaxis of Pneumocystis carinii pneumonia in patients with the acquired immunodeficiency syndrome. After two months of weekly prophylaxis ...

We describe a case of disseminated penicilliosis in a patient with the acquired immunodeficiency syndrome. Penicillium marneffei was cultured from the blood, bone marrow, sputum, stool, and skin; the yeast forms were demonstrated in skin and bone marrow biopsy specimens. To our knowledge, this is the first reported case of ...

We have summarized the findings in selected congenital and acquired immunodeficiency disorders that frequently display blood and bone marrow abnormalities. Many other immunodeficiency syndromes were not discussed, as bone marrow abnormalities are infrequently seen in them. Examination of the bone marrow is not usually critical for diagnosing most immunodeficiency disorders. ...

We report a case of a 25-year-old woman affected by the acquired immunodeficiency syndrome in whom the neuropathologic examination disclosed the presence of diffuse axonal swellings in the brain stem and in selected areas of cerebral hemispheres. These lesions, although microscopically similar to those previously described as focal pontine leukoencephalopathy, ...

The acute retinal necrosis (ARN) syndrome has recently been associated with intraocular infections with one or more members of the herpesvirus family. There have been 14 cases in the literature linking ARN with a preceding or subsequent herpetic dermatitis. We report the development of bilateral ARN (BARN) after unilateral Herpes ...

Teenage schoolchildren are often sexually active and first experiments with drugs can occur at this age. A questionnaire survey of the knowledge and source of information about the acquired immune deficiency syndrome was carried out on 232 15-year-olds at a school in Fife. The results showed that this younger age ...

Visceral Kaposi's sarcoma is a common manifestation of the acquired immune deficiency syndrome (AIDS). Most lesions are clinically silent, detected only by radiographic or endoscopic studies. We report the first instance of AIDS-related jejunal Kaposi's sarcoma presenting with small intestinal obstruction due to intussusception. Gastrointestinal Kaposi's sarcoma is a clinical ...

A 32-year-old male intravenous drug abuser was admitted to our institution with constitutional symptoms and sputum smears containing acid-fast bacilli, but no parenchymal disease on chest radiograph. He subsequently developed massive haemoptysis and required an emergency lobectomy. The pathology specimen revealed miliary tuberculosis with extensive caseation, schistosomiasis and cytomegalovirus. This ...

A 32-year-old man had a history of acquired Brown's syndrome associated with diplopia, proptosis, downward displacement of the globe, and lid edema. A CT scan of the orbit revealed an osteoma arising from the left frontal sinus and extending into the left orbit. After surgical extirpation the proptosis and diplopia ...

We describe 2 patients who developed psoriatic arthritis during the course of acquired immunodeficiency syndrome. Both patients developed skin and articular involvement characteristic of psoriatic arthritis; these manifestations were refractory to conventional therapy. Our findings suggest that psoriatic arthritis should be added to the expanding spectrum of musculoskeletal manifestations of ...

Cytomegalovirus (CMV) is an opportunistic organism known to cause significant gastrointestinal pathology in patients with the acquired immune deficiency syndrome (AIDS). Hepatobiliary involvement has previously been documented. In this report, we discuss the rare entity of acalculous cholecystitis associated with CMV intranuclear inclusion bodies in an AIDS patient. The issue ...

Campylobacter pylori has been associated with gastritis, duodenitis, and duodenal ulceration in the immunocompetent individual. It has been described within the superficial mucus layer, in interepithelial junctions, and occasionally in the microcanaliculi of epithelial cells, but never in the lamina propria. We describe a case of invasive C. pylori in ...

We report a case of symptomatic cytomegalovirus cystitis associated with the acquired immune deficiency syndrome related complex. The diagnosis of the acquired immune deficiency syndrome was established by bladder biopsy. The hematuria resolved after systemic acyclovir therapy. The other known urological manifestations of the acquired immune deficiency syndrome are discussed.

Cryptococcosis is a recognized opportunistic pathogen in the acquired immune deficiency syndrome. Although central nervous system infection and disseminated cryptococcosis is common in acquired immune deficiency syndrome, localized infection is rare. We present a case of massive retroperitoneal and mesenteric adenopathy in a male homosexual patient with acquired immune deficiency ...

With the passage of time and the refinement of laboratory techniques, the ability to recognize disease related to human immunodeficiency virus infection has improved. Currently, a number of clinical conditions can be identified as "pre-AIDS" syndromes. These include the syndrome of persistent generalized lymphadenopathy, immune thrombocytopenic purpura, the wasting syndrome, ...

An hypothesis is presented to explain the link between acquired immunodeficiency syndrome (AIDS) and Kaposi's sarcoma (KS). According to this hypothesis, AIDS involves all four components of the integrated lymphatic system--lymphatics, lymph nodes, lymphocytes, and lymph--and thereby resembles various congenital and acquired lymphologic syndromes characterized by one or more of ...

The etiologic role of Clostridum perfringens type A in the acute abdominal syndrome characterized by abomasal and rumen tympany, abomasitis, and abomasal ulceration was investigated in neonatal calves. Eight calves, 4 to 12 days old, were inoculated intraruminally with toxigenic C perfringens type A. Before and after C perfringens inoculation, ...

We describe a patient in whom the diagnosis of transfusion-associated acquired immunodeficiency syndrome (AIDS) was delayed because her clinical symptoms were similar to those of systemic lupus erythematosus and Sjögren's syndrome and because of a false-negative result on a Western blot test for human immunodeficiency virus. The importance of using ...

The peripheral blood leukocytes of 6 children with clinical data suggestive of primary cellular immunodeficiencies were studied in an attempt to define the cellular basis of these disorders. The phenotype and function of T and B cells were investigated. According to the clinical and laboratory features, the patients were classified ...

An acute midbrain syndrome III/IV developed twice after tetanus immunization. The occurrence of nearly identical episodes was remarkable, as well as the relatively rapid return to normal consciousness and neurological status after deep coma. Special emphasis is placed upon the chronological relationship of the coma to the immunization and upon ...

Stevens-Johnson syndrome is both a physically and psychologically devastating disease. This paper primarily deals with the physiological complications of the disease process, but the psychological trauma often associated with such an initially disfiguring disease leaves wounds that are not visible. Constant support of both the patient and the nursing staff ...

Liver abnormalities are common in patients with acquired immune deficiency syndrome. These relate to 1) coincident exposure to hepatotropic viruses, 2) complications, either infectious, neoplastic, or iatrogenic, of the immunosuppressed state, or 3) nonspecific changes associated with chronic debilitating illness. We review the hepatobiliary manifestations of acquired immune deficiency syndrome, ...

Cavitation is a most unusual radiological feature of Pneumocystis carinii pneumonia (PCP). Autopsy evidence of cavities in PCP is poorly documented. We describe a case of fatal PCP occurring in a patient with the acquired immune deficiency syndrome; the PCP was associated with radiological as well as autopsy evidence of ...

We describe the identification, experimental transmission, and pathogenesis of a naturally occurring powerfully immunosuppressive isolate of feline leukemia virus (designated here as FeLV-FAIDS) which induces fatal acquired immunodeficiency syndrome (AIDS) in 100% (25 of 25) of persistently viremic experimentally infected specific pathogen-free (SPF) cats after predictable survival periods ranging from ...

A palatal lesion was the initial manifestation of disseminated cryptococcosis in a patient with acquired immunodeficiency syndrome (AIDS). The diagnosis was initially verified with cytologic smears in situ and later confirmed with pathologic findings from an excisional biopsy specimen. As cryptococcosis is one of the major opportunistic infections in persons ...

Data on 54 persons who died of acquired immunodeficiency syndrome were reviewed for lesions of the heart. These persons met the criteria of the Centers for Disease Control (Atlanta) for acquired immunodeficiency syndrome. Pathologic changes were seen in 30 patients (55%). Changes were seen in the endocardium (17%), myocardium (83%), ...

A 24-year-old East Indian woman presented with an inflammatory swelling in the supranasal quadrant of the left orbit and vertical diplopia. The motility disorder was of typical acquired Brown's syndrome. Histopathological examination of the lesion revealed Cysticercus cellulosae, a parasitic cyst known to produce a severe inflammatory reaction. To our ...

To distinguish the clinical features of amputation due to congenital constriction band syndrome from those of transverse deficiency, 42 cases of constriction band syndrome and 27 cases of transverse deficiency were analysed. All the transverse deficiencies were unilateral. Two cases of transverse deficiency were associated with pectoral muscle absence, whereas ...

The case of a 29-year-old former parenteral drug abuser who presented with a 3rd nerve palsy and contralateral ataxia is reported. The patient was found to have a positive HLTV-III titer and acquired immune deficiency syndrome was diagnosed. Computed tomography demonstrated two ring-enhancing lesions in the brain which were presumed ...

This case report documents a perforation of the terminal ileum in a 40-year-old white male homosexual with the acquired immunodeficiency syndrome. The perforation occurred at a site that had severe cytomegalovirus infection and was in close proximity to multiple nodules of Kaposi's sarcoma. The ileum showed multiple deep ulcers with ...

We describe 2 patients with unrecognized acquired immunodeficiency syndrome who presented with acute urological conditions. One patient had inapparent obstructive anuric renal failure and the other had a testicular mass. The etiology in each case was large cell lymphoma. Recognition of the acquired immunodeficiency syndrome by urologists requires an awareness ...

Gastrointestinal involvement by Kaposi's sarcoma in patients with cutaneous or lymph node involvement is common. Since the advent of the acquired immune deficiency syndrome in 1981, primary gastrointestinal involvement, i.e., without skin or lymph node involvement, has not been adequately documented. We describe a patient with acquired immune deficiency syndrome ...

Esophagitis due to an opportunistic infection in an immunocompromised host is not unusual. This is frequently attributed to Candida albicans. Torulopsis glabrata is similar however taxonomically distinct from C. albicans. Although found to be pathogenic in various organs, to date there have been only four reported cases of T. glabrata ...

The acquired immune deficiency syndrome (AIDS) is a syndrome requiring unique knowledge of its versatile manifestations for accurate diagnosis and skillfull management of its numerous complications for successful treatment. The human T-cell lymphotropic virus type III (HTLV-III), a replication-complete virus, is now reported as the etiologic agent. The neurologic complications ...

A patient with macroamylasaemia in association with the acquired immunodeficiency syndrome is described. Patients with the acquired immunodeficiency syndrome commonly have gastrointestinal symptoms that often prompt serum amylase determination. Macroamylasaemia has been found to occur in a variety of diseases including various autoimmune disorders. The marked immunological abnormalities and increased ...

A young infant with Wilms' tumour was found to have acquired von Willebrand's syndrome but no bleeding symptoms. Neither parent of the infant had evidence of von Willebrand's syndrome. Postoperatively, after removal of the tumour, factor VIII complex levels returned to normal. The mechanism of the acquired von Willebrand's syndrome ...

The Stevens-Johnson syndrome is a relatively uncommon, severe form of erythema multiforme. A case is presented in which Mycoplasma pneumoniae infection was established as the causative agent. Clinicians need to be aware of this uncommon association. We emphasize the need to consider M. pneumoniae infection as one of the many ...

A 62-year-old man and his maternal uncle had a selective vitamin E deficiency without generalized fat malabsorption. A progressive neurological disorder comprising ataxia, areflexia, and loss of proprioception developed in their sixth and seventh decades. The vitamin E deficiency is thought to be due to abnormally accelerated utilization, excretion, or ...

Pathological changes in the liver have been described in patients with acquired immune deficiency syndrome; however, lesions of the bile ducts have not been noted. We report 2 patients with intrahepatic bile duct abnormalities, one having sclerosing cholangitis of the large ducts. We discuss the possibility that these changes are ...

Cutaneous Kaposi's sarcoma developed eight months after initiation of prednisone treatment in a 58-year-old man with systemic rheumatoid disease (rheumatoid arthritis, Felty's syndrome, rheumatoid vasculitis, and myositis). This patient did not have the acquired immune deficiency syndrome. Review of the literature suggests that the onset of his Kaposi's sarcoma may ...

Nocardial cerebral abscess is an unusual neurologic manifestation of the acquired immunodeficiency syndrome. A 20-year-old woman with the acquired immunodeficiency syndrome presented with headache and fever. Nocardia asteroides was cultured from a stereotaxic brain biopsy specimen. Despite antibiotic therapy to which the pathogen was sensitive, resolution of the abscess followed ...

The widespread emergence of chloroquine-resistant Plasmodium falciparum led to the formulation of an effective, fixed combination of two antimalarial agents, pyrimethamine and the long-acting sulfonamide sulfadoxine, for prophylaxis and treatment. These drugs act at sequential steps to inhibit the formation of tetrahydrofolate in the parasite. Recently, their use for malaria ...

Two new deficient variants of glucose-6-phosphate dehydrogenase (G6PD) causing hereditary nonspherocytic hemolytic anemia (HNSHA) are described. Both of these are unique and they have been named G6PD Wayne and G6PD Huron. Patients with G6PD Wayne underwent splenectomy and no objective improvement was noted. The patients with G6PD Huron were under ...

Bacteremic infection with Shigella species is extremely rare in adults. We report five cases of Shigella bacteremia in adult men that occurred at our hospital over a 2-yr period. Four of these cases occurred in patients with the acquired immune deficiency syndrome. The fifth patient had evidence of cell-mediated immunodeficiency, ...

Kaposi's sarcoma is a well-known manifestation of the acquired immune deficiency syndrome. Involvement of the gastrointestinal tract, including the colon, by this tumor usually causes no gastrointestinal symptoms. However, Kaposi's sarcoma has never been reported in association with ulcerative colitis in patients with acquired immune deficiency syndrome. Herein, the second ...

Pleural cryptococcosis is extremely rare. We report the first case of cryptococcal pleural effusion in association with the acquired immune deficiency syndrome. Pleural effusion without the evidence of pulmonary parenchymal involvement was the initial and only clinical finding leading to the diagnosis of disseminated cryptococcosis. The pleural effusion resolved spontaneously ...

The first case, to our knowledge, of an integumentary form of botryomycosis is reported in a homosexual man with acquired immunodeficiency syndrome. Anal fistula and ischiorectal and gluteal abscesses developed following severe cryptosporidial diarrhea. Grains composed of gram-positive cocci were identified in the suppurative exudate. The grains had attached to ...