The cat scratch disease-bacillary angiomatosis puzzle.
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Angiomatosis, Bacillary↗
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Management of children with prolonged fever of unknown origin and difficulties in the management of fever of unknown origin in children in developing countries.
This is Part II of a 2-part paper on fever of unknown origin (FUO) in children. It examines the aetiology and management of prolonged FUO in children and the difficulties in the management of FUO in children in developing countries. Part I of this paper discussed acute FUO in children and was published in the March 2001 issue of Paediatric Drugs. Prolonged FUO is documented fever of more than 7 to 10 days which has no apparent source and no apparent diagnosis after 1 week of clinical investigations. About 34% of cases of prolonged FUO are caused by infections, with bacterial meningitis and urinary tract infection accounting for about 6.5 and 11.4%, respectively, of cases attributable to infections. Chronic infections, particularly tuberculosis and 'old' disorders such as Kawasaki disease, cat-scratch disease and Epstein-Barr virus infection presenting with 'new' manifestations, collagen-vascular diseases and neoplastic disorders are the other issues of major concern in prolonged FUO. Overall, however, there is a trend towards an increased number of undiagnosed cases. This is due to advancements in diagnostic techniques, such that illnesses which were previously common among the causes of prolonged FUO are now diagnosed earlier, before the presentation becomes that of prolonged FUO. Clinical examination supplemented with laboratory tests to screen for serious bacterial infections should be the mainstay of initial evaluation of children with prolonged FUO. Use of scanning techniques (such as computerised tomography and ultrasound) as additional supplements to this clinical examination may allow for the earlier diagnosis of causes of prolonged FUO in children such as 'occult' abdominal tumours. A common error in management of children with prolonged FUO is the failure to perform a complete history and physical examination; repeated clinical examination and continued observation are of paramount importance in the diagnosis of difficult cases. Major difficulties in the management of FUO in children in developing countries include constraints in the availability and reliability of laboratory tests, cost, misuse of antibiotics and difficulties encountered in the diagnosis of malaria and typhoid fever. Malaria and typhoid fever are major aetiological considerations in both acute and prolonged FUO in children in developing countries. The newer quinolones may hold great promise for the treatment of serious bacterial infections, including meningitis, which are associated with prolonged FUO in developing countries.
Child↗
Optic disk edema with a macular star.
Optic disk edema with a macular star is a descriptive term encompassing a heterogeneous group of disorders. The clinical features include sudden visual loss, swelling of the optic disk, peripapillary and macular exudates that may occur in a star pattern, and cells in the vitreous. Herein we describe the clinical features, potential etiologic factors, differential diagnosis, work-up, and natural history of this entity. Although optic disk edema with a macular star is usually idiopathic, infectious causes, especially syphilis, Lyme disease, cat-scratch disease, and toxoplasmosis, should be considered. The macular exudate likely results from primary optic nerve disease, not from inflammation of the retina; therefore, we prefer the term "idiopathic optic disk edema with a macular star" for idiopathic cases rather than "neuroretinitis." When optic disk swelling and macular star are associated with focal or multifocal inflammatory lesions in the retina (retinitis), especially if an infectious cause is documented, the term "neuroretinitis" is appropriate. The prognosis for visual recovery is usually good, but residual visual loss may be severe in a few cases. Patients with a recurrent type of the disease may not experience pronounced improvement in optic nerve function. The macular exudate may not develop in cases of disk edema until 2 weeks after the patient's initial assessment; thus, patients who have acute papillitis with a normal macula should be reexamined within 2 weeks for development of a macular star. The presence of a macular star militates strongly against subsequent development of multiple sclerosis.
Diagnosis, Differential↗
Pattern of lymph node pathology in a private pathology laboratory.
Lymph node excision biopsy is commonly carried out for the investigation of lymphadenopathy. The objective of this study is to elucidate the pattern of nodal pathology seen in a private pathology practice. A total of 137 nodal biopsies for primary investigation of nodal enlargement were retrieved from the files in a private diagnostic pathology laboratory in the year 1997. Lymph nodes excised for cancer staging were excluded from this study. The histology was reviewed based on H&E stained sections, and with additional histochemical and immunoperoxidase stains when deemed necessary. Cases of malignant lymphomas were sub-classified with the aid of further immunophenotyping using a panel of monoclonal and polyclonal lymphoid antibodies. One case was excluded from this study due to inadequate tissue for further assessment. There were 58 males and 78 females, giving a ratio of 1:1.3 in the remaining 136 cases. They consisted of 13 Malays (M), 108 Chinese (C), 14 Indians (I) and 1 other ethnic group (O). The ratio of M:C:I:O was 1:8.3:1.1:0.1. The majority of the cases were in the age range of 20 to 50 years. The pathology consisted of 17 (12.5%) malignant lymphomas [6 Hodgkin's lymphoma, 11 non-Hodgkin's lymphoma], 35 (25.7%) metastatic carcinomas, 45 (33.1%) reactive hyperplasia, 19 (13.9%) tuberculosis, 11 (8.2%) Kikuchi's disease and 9 (6.6%) others (Castleman's disease 2, cat scratch disease 2, Kimura's disease 1, sarcoidosis 1, non-specific lymphadenitis 3). All categories of nodal disease showed approximately similar ratio of ethnic and gender distribution as above, except for Kikuchi's disease, for which 100% of the patients were female. The most common site of biopsy was from the head and neck region, particularly the cervical group of nodes. The most common nodal pathology seen in the private laboratory was reactive hyperplasia, followed by metastatic carcinoma. Malignant lymphoma constituted only 12.5% of the cases.
Adolescent↗
[Bartonelloses].
Bartonellae belong to less known causal agents of many human diseases. They are gram-negative bacteria growing slowly on culture media enriched with hemin or bovine serum. The genus Bartonella, which currently involves more than 15 species, is present worldwide. Bartonellae live in natural foci in dependence on the occurrence of natural host (rodents, felines, canidae, human) and insect vector (flea, tick, louse). By reservoir animals they usually cause permanent intraerythrocytic bacteraemia without system inflammation symptoms. A classical example of a human disease is cat scratch disease (CSD) caused by Bartonella henselae and characterised by regional lymphagoitis and lymphadenitis. Increasing interest is being devoted to the ability of Bartonella sp. (e.i. B. quintana) to cause the opportune infections with diverse clinical manifestation: bacillary angiomatosis, specific liver and spleen vasculitis (peliosis hepatis, splenis), endocarditis and others. The issue of Bartonella infections is relatively new and its importance is still growing with increasing knowledge in this field.
Animals↗
[Newer diagnostic procedures for chlamydial diseases (author's transl)].
Chlamydiales are bacteries showing a growth cycle unique among procaryotes. The two species Chlamydia trachomatis and Chlamydia psittaci are genetically very distant and their pathogenicity for man is very distinct. Human chlamydia infections by Chlamydia trachomatis are diseases chiefly sexually transmitted and their epidemiological importance is growing. The relationship between chlamydial infections, Reiter disease, and cat scratch disease are discussed. The various laboratory diagnostic procedures are reported, including the techniques and their indications; the method of choice is in the majority of cases the isolation of Chlamydia on cell culture.
Chlamydia↗
[Newer diagnostic procedures for chlamydial diseases (author's transl)].
Chlamydiales are bacteries showing a growth cycle unique among procaryotes. The two species Chlamydia trachomatis and Chlamydia psittaci are genetically very distant and their pathogenicity for man is very distinct. Human chlamydial infections by Chlamydia trachomatis are diseases chiefly sexually transmitted and their epidemiological importance is growing. The relationship between chlamydial infections, Reiter disease, and cat scratch disease are discussed. The various laboratory diagnostic procedures are reported, including the techniques and their indications; the method of choice is in the majority of cases the isolation of Chlamydia on cell culture.
Antigens, Bacterial↗
Identification of Bartonella henselae and B. quintana 16s rDNA sequences by branch-, genus- and species-specific amplification.
Given the controversy surrounding the aetiology of cat scratch disease and the association of both Bartonella henselae and B. quintana with bacillary angiomatosis, a method for the direct detection in clinical samples of 16S rRNA from the Proteobacteria alpha subgroup was developed. The primary structure of amplified 16S rDNA was determined by cloning and sequencing. Three sequences were identified: one corresponded exactly to GenBank accession number M73229 (B. henselae); the second was related to, but distinct from, GenBank accession number Z11684 (referred to as 'B. henselae variant'); and a third sequence was identical with GenBank accession number M73228 (B. quintana). No sequence corresponding to Afipia spp. was found. To speed identification and reduce the cost of analysis, a nested amplification method for B. henselae and B. quintana was devised. These techniques were applied to DNA extracted from 30 unfixed lymph node biopsies, two liver biopsies and 36 node pus samples from patients with suspected cat scratch disease, and from 17 skin biopsies from AIDS patients with suspected bacillary angiomatosis. B. henselae or B. henselae variant sequences were found in 42 (62%) of 68 samples from suspected cat scratch disease. B. quintana was not associated with cat scratch disease, but a B. quintana sequence was found in seven (41%) of 17 samples from suspected bacillary angiomatosis patients. B. henselae 16S rDNA sequences were not found in bacillary angiomatosis specimens.
Bartonella henselae↗
[Bacillary angiomatosis].
Bacillary angiomatosis is a bacterial disease which affects mainly immunosuppressed patients. It may compromise any tissue, especially the skin, presenting papules, nodules or angiomatous tumors. We studied three young men with AIDS, all of them with 1-2 papules, nodules or subcutaneous tumors suggesting telangiectatic granuloma, sarcoma and lipoma. Microscopically, they were misdiagnosed as telangiectatic granuloma, Kaposi's sarcoma and "angioma with secondary inflammation". After reviewing the histopathology, we saw them to be composed by vessels with prominent endothelium and stroma rich in leukocytoclastic polymorphonuclears. Fibrinoid deposits were observed in the neighborhood of vessels as well as minute eosinophilic granular interstitial masses corresponding to Bartonella aggregates, criteria which answer to the diagnosis of bacillary angiomatosis with HE staining. The Warthin-Starry stain was not useful; using resin embedded tissue from paraffin-embedded material, bacterial clusters, both in semithin section stained with toluidine blue and in thin sections observed under the electron microscope, were clearly seen, confirming bacillary angiomatosis diagnosis. Patients were successfully treated with surgery and either erythromycin or doxycycline. We reviewed the entity as well as its differential diagnoses with telangiectatic granuloma, Kaposi's sarcoma, Carrión's disease, and cat-scratch disease. In conclusion, we showed the presence of bacillary angiomatosis in three patients, illustrated its typical histopathological appearance with HE staining and demonstrated the causal bacteria in thick sections and with the electron microscope. It is essential to recognize bacillary angiomatosis, as it can be cured with antibiotics.
AIDS-Related Opportunistic Infections↗
A chemically defined liquid medium that supports primary isolation of Rochalimaea (Bartonella) henselae from blood and tissue specimens.
Rochalimaea (Bartonella) henselae is a fastidious, slowly growing, gram-negative bacillus that is an etiologic agent of bacillary angiomatosis, cat scratch disease, and related syndromes. Accumulation of direct microbiologic evidence of the relationship between the organism and the syndromes compatible with cat scratch disease has been hindered by the difficulties in the primary isolation of the organism from infected tissue specimens. A chemically defined liquid medium was developed to support the growth of Rochalimaea species to facilitate study of the organism. This medium was also used successfully to isolate R. henselae from clinical specimens from infected patients and a domestic cat. Recovery of R. henselae in this was more successful than when recovery was attempted on solid agar. This cell-free, extract-free, defined medium additionally supported the growth of Rochalimaea quintana and Afipia felis.
Angiomatosis, Bacillary↗
An unusual presentation of cat scratch encephalitis.
Cat scratch disease is an infectious illness that has been recognized since the 1880s; however, our understanding and knowledge of it is still evolving (1). It was not until 1991 that the etiologic species, Rochalimaea, was finally confirmed (2,3). Only recently have the breadth of its clinical spectrum and the population at risk been appreciated. We now realize that signs and symptoms that had been considered cardinal for diagnosis may be absent. Cat scratch disease was known to afflict primarily children and adolescent; however, the incidence of CSD is increasing in immunocompromised groups, such as AIDS and transplant patients (3,4). The recent discovery of the infectious agent and improved understanding of the disease process have led to new approaches in diagnosis and treatment. We present a report of a patient with cat scratch disease who presented with seizure and altered mental status secondary to encephalitis.
Bartonella henselae↗
Ocular manifestation of systemic infections.
Patients with various systemic infections can often be diagnosed on the basis of their presenting ocular signs and symptoms. Recognition of the ocular manifestations of systemic infections will expedite diagnosis and allow rapid institution of appropriate therapy thereby reducing morbidity and mortality. Recent literature discussing ophthalmologic findings in the following diseases are reviewed: Whipple's disease, endogenous endophthalmitis, cat-scratch disease, toxoplasmosis, and hepatitis C.
Bartonella henselae↗
Bacillary angiomatosis.
The acquired immunodeficiency syndrome and other causes of immunosuppression have ushered in a variety of opportunistic infections. One of these is bacillary angiomatosis, a vasoproliferative lesion whose principal causative agent is Rochalimaea henselae. Bacillary angiomatosis, while preponderantly a cutaneous affliction, can be systemic, including involvement of the head and neck mucous membranes. Molecular technology and epidemiologic studies used to identify the bacterial agent of bacillary angiomatosis have also uncovered R henselae as the organism responsible for most cases of cat-scratch disease. Why the same organism promotes two different histopathologic lesions, as seen in bacillary angiomatosis and cat-scratch disease, is unknown.
Angiomatosis, Bacillary↗
Ocular bartonellosis.
PURPOSE: To review recent advances in the basic and clinical biology of Bartonella-related eye disease. METHOD: A review of the pertinent medical literature was performed. RESULTS: A number of novel Bartonella species have been identified over the past decade. Of these, Bartonella henselae, the etiologic agent in cat scratch disease, is most often associated with ocular complications, which may include Parinaud oculoglandular syndrome, neuroretinitis, and focal retinochoroiditis. Although cat and flea exposure appear to be the main risk factors for contracting cat scratch disease, the diagnosis of ocular bartonellosis relies primarily on the recognition of suggestive clinical signs in conjunction with positive serologic testing. B. henselae-associated ocular complications are usually self-limited but may be treated with doxycycline or erythromycin, with or without rifampin, when the infections are severe or sight-threatening. CONCLUSIONS: B. henselae infection is common and should be considered in patients with Parinaud oculoglandular syndrome, neuroretinitis, or focal retinochoroiditis, particularly when there is a history of cat or flea exposure.
Animals↗
Acute clinical disease in cats following infection with a pathogenic strain of Bartonella henselae (LSU16).
Bartonella henselae is the causative agent of human cat scratch disease as well as several serious sequelae of infections, including bacillary angiomatosis and bacillary peliosis. Conflicting reports describe the pathogenesis of B. henselae in the cat. In this study, we characterized a strain of B. henselae termed LSU16. This strain was isolated on rabbit blood agar from a naturally infected 10-month-old female cat during a recurrent episode of bacteremia. The bacterial species was confirmed by PCR-restriction fragment length polymorphism analysis. Nine cats were infected intradermally with 5 x 10(7) CFU of LSU16, and clinical signs, antibody responses, and bacteremia were monitored. All nine cats developed raised, erythematous areas at the site of inoculation within 72 h postinoculation; the swelling peaked at 14 days postinfection and was not palpable by 28 days postinfection. Fever developed in all nine cats between 6 and 16 days postinfection and lasted for 1 to 8 days. Between 6 and 16 days postinfection, all nine cats experienced lethargy which persisted 5 to 18 days. Seven of nine cats were bacteremic by day 7, and all nine cats had become bacteremic by 14 days postinfection. Bacteremia peaked at 14 to 28 days postinfection in all cats. In six of the nine infected cats, bacterial numbers reached nondetectable levels during the 7th week postinfection; however, a single animal maintained bacteremia to 18 weeks postinfection. All nine cats developed strong antibody responses to B. henselae, as determined by Western blot analysis and enzyme-linked immunosorbent assay. Subsequently, three naive cats were injected intradermally with blood from cats infected with LSU16 from a pure culture, and five naive cats were injected with feces from fleas which had been feeding on cats infected with a pure culture of LSU16. These cats developed signs similar to those described in the previous experiment and were euthanized at 5 weeks postinfection. We conclude that B. henselae LSU16 is a virulent strain of B. henselae in cats and propose that the virulence of B. henselae in cats is strain dependent.
Acute Disease↗
Cat-scratch encephalopathy presenting as status epilepticus and lymphadenitis.
A five-year-old male was admitted to the hospital with generalized seizures. Enlarged lymph nodes raised the suspicion of cat-scratch disease. The diagnosis was confirmed by a positive history of a cat bite, typical histopathologic findings in the biopsy of the lymph nodes, and a positive skin test. Brain CT scan and LP were repeatedly normal. The clinical course was remarkable for recurrent episodes of status epilepticus refractory to usual anticonvulsant therapy and prolonged encephalopathy consisting of mental confusion, hemiparesis, tremor, chorea, and vomiting. All neurologic symptoms gradually resolved within nine months, without sequelae. Cat-scratch encephalopathy should be suspected in a child presenting with status epilepticus and enlarged lymph nodes. Aggressive and prolonged anticonvulsant therapy is strongly recommended.
Brain Diseases↗
[An oculoglandular syndrome and ophthalmia nodosa].
A case of ophthalmia nodosa associated with an oculoglandular syndrome is described. The patient, scratched by a cat six-and-a-half months earlier, presented a positive cat-scratch disease antigen test. The possibility that the oculoglandular syndrome is due to the reactivation of a latent virus, the cause of cat-scratch disease, is discussed.
Cat-Scratch Disease↗
Bartonellosis: light and shadows in diagnostic and therapeutic issues.
Cat-scratch disease involves a prolonged and/or complicated course, and lymph node drainage is usually required. Culture and molecular techniques often yield negative results, but immunofluorescence assays may give early information, and elevated antibodies may persist for months. Cat-scratch disease should be suspected in patients with prominent swelling of lymph nodes draining from the upper limbs, limited systemic involvement, and typical epidemiological-clinical features. The temporal antibody response during the sub-acute course remains unknown. Although biomolecular assays are available, the time between onset and investigation is an obstacle to positive results. The role of surgical debridement and the unpredictable activity of antimicrobial agents warrant further investigation.
Animals↗