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[Parinaud's oculoglandular syndrome. A rare differential diagnosis of "red eye"].

Two cases of Parinaud's oculoglandular syndrome, which represents an ocular manifestation of cat-scratch disease, are reported. The symptoms are subacute and include unilateral conjunctivitis and pre-auricular lymphadenopathy. Diagnosis primarily relies on the recognition of suggestive clinical signs in conjunction with positive serologic testing. In most cases, therapy is not necessary.

Cat-Scratch Disease↗

The agent of bacillary angiomatosis. An approach to the identification of uncultured pathogens.

BACKGROUND: Bacillary angiomatosis is an infectious disease causing proliferation of small blood vessels in the skin and visceral organs of patients with human immunodeficiency virus infection and other immunocompromised hosts. The agent is often visualized in tissue sections of lesions with Warthin-Starry staining, but the bacillus has not been successfully cultured or identified. This bacillus may also cause cat scratch disease. METHODS: In attempting to identify this organism, we used the polymerase chain reaction. We used oligonucleotide primers complementary to the 16S ribosomal RNA genes of eubacteria to amplify 16S ribosomal gene fragments directly from tissue samples of bacillary angiomatosis. The DNA sequence of these fragments was determined and analyzed for phylogenetic relatedness to other known organisms. Normal tissues were studied in parallel. RESULTS: Tissue from three unrelated patients with bacillary angiomatosis yielded a unique 16S gene sequence. A sequence obtained from a fourth patient with bacillary angiomatosis differed from the sequence found in the other three patients at only 4 of 241 base positions. No related 16S gene fragment was detected in the normal tissues. These 16S sequences associated with bacillary angiomatosis belong to a previously uncharacterized microorganism, most closely related to Rochalimaea quintana. CONCLUSIONS: The cause of bacillary angiomatosis is a previously uncharacterized rickettsia-like organism, closely related to R. quintana. This method for the identification of an uncultured pathogen may be applicable to other infectious diseases of unknown cause.

Acquired Immunodeficiency Syndrome↗

High-resolution and color Doppler ultrasonography of cervical lymphadenopathy in children.

PURPOSE: To assess the frequency of high-resolution and color Doppler sonographic findings in the most common diseases of childhood manifested with cervical lymphadenopathy at initial presentation. MATERIAL AND METHODS: High-resolution and color Doppler US were performed in 103 and 43 children respectively, with cervical lymphadenopathy at initial presentation. Sonomorphology and intranodal vascularity were assessed. Final diagnoses, based on biopsy or clinical and sonographic follow-up, included: Reactive hyperplasia (n=34), infectious mononucleosis (n=20), lymphoma (n=11), bacterial (n=28), tuberculous (n=5) and cat-scratch disease (CSD, n=5) lymphadenites. RESULTS: Round shape (L/S<2) was common both in lymphoma (78%), infectious mononucleosis (85%) and bacterial lymphadenitis (73%) while 91% of reactive nodes had L/S>2. Wide hilum conforming to nodal shape characterized reactive hyperplasia (94%) and infectious mononucleosis whereas absent or narrow hilum was frequent in lymphoma (100%) and bacterial lymphadenitis (60%). Central irregular hyperechogenic areas, blurred margins and central necrosis were most frequent in bacterial, tuberculous and CSD lymphadenites. On color Doppler US, hyperplastic nodes more frequently exhibited a solitary hilar vessel (48%), whereas infectious mononucleosis nodes had a central radial pattern (75%). Bacterial lymphadenitis presented with a variety of vascular patterns. CONCLUSION: Although individual sonographic signs are not specific, the categorization and combination of findings might be highly suggestive of diagnosis of the underlying disease presenting with cervical lymphadenopathy.

Adolescent↗

White spots syndromes.

The different types of white spots occurring in the fundus are analysed. A. Acute white spots, vanishing later on. 1. Multiple Evanescent White Dot Syndrome. 2. Cat scratch disease. 3. AIDS microangiopathy. 4. Cotton-wool spots. 5. Acute vitelliform maculopathy. B. Acute white spots with coalescence and diffuse scarring. 1. Acute posterior multifocal placoid pigment epitheliopathy. 2. Serpiginous choroïditis (geographic choroïditis). 3. Herpes retinitis. C. Acute white spots becoming white scars with variable pigmentation. 1. Multifocal choroiditis--classical form. 1a. Punctate inner choroidopathy. 1b. Diffuse subretinal fibrosis. 2. Toxoplasmic retinochoroiditis. 3. Tuberculous chorioretinitis. 4. Syphilitic chorioretinitis. 5. Lyme disease. 6. Sarcoidosis. 7. Sympathetic ophthalmia. 8. Vogt-Koyanagi-Harada disease. 9. Bacterial retinochoroiditis. 10. Fungal retinochoroiditis--Candida. 11. Pneumocystis carinii choroiditis. D. Late white spots with or without initial white-orange spots. Birdshot chorioretinitis.

AIDS-Related Opportunistic Infections↗

Chromophobe renal cell carcinoma in a pediatric living-related kidney transplant recipient.

Renal cell carcinoma can occur in children who have received renal allografts from adults. Chromophobe renal cell carcinoma is a rare variant of renal carcinoma with distinct histochemical, ultrastructural, and genetic characteristics. We describe the incidental finding of a chromophobe renal cell carcinoma in a 13 1/2-year-old boy 5 years after receiving a living-related renal transplant. This tumor was found by serendipity during the evaluation of fever and inguinal lymphadenopathy, with the presumptive diagnosis of posttransplantation lymphoproliferative disorder. The patient was found to have cat-scratch disease. A renal cell carcinoma should be considered in the differential diagnosis of a pediatric recipient of an adult kidney with an incidental finding of a tumor in the graft.

Adolescent↗

Rochalimaea antibodies in HIV-associated neurologic disease.

Rochalimaea henselae, a recently described pathogen thought to cause syndromes as varied as bacillary angiomatosis, parenchymal bacillary peliosis, fever with bacteremia, and cat-scratch disease, is associated with CNS diseases including cerebral and retinal bacillary angiomatosis, as well as cat-scratch-related encephalitis, myelitis, cerebral arteritis, and retinitis. We used a newly developed enzyme immunoassay and the polymerase chain reaction to investigate the association of R henselae infection with HIV-related CNS disease and found that whereas seroprevalence rates in HIV-positive patients unselected for neurologic disease were 4% to 5.5%, those with neurologic disease had seroprevalence rates of 32%. The ratio of organism-specific antibodies in CSF compared with serum suggested intra-blood-brain-barrier synthesis of these antibodies. CSF specimens containing only R henselae IgM had 16S rDNA specific for R henselae. Stored serum from one of these patients indicated he had developed R henselae-reactive IgM antibodies 10 months prior to the onset of neurologic disease. In the 14 patients for whom clinical data were available, evidence of CNS invasion by R henselae was accompanied by acute and subacute mental status changes including hallucinations, disorientation, and rapidly progressive dementia.

AIDS-Associated Nephropathy↗

[Severe retinal phlebitis in ocular bartonellosis].

PURPOSE/METHODS: To report the clinical case of a 34-year-old male with atypical ophthalmic manifestations of cat-scratch disease (ocular bartonellosis), including an extensive retinal phlebitis, as well as the evolution of the clinical picture with treatment. RESULTS/CONCLUSIONS: The clinical diagnosis of ocular bartonellosis was serologically confirmed. Oral treatment with ciprofloxacine healed the phlebitis and the neuroretinitis, with a residual optic disk pallor. This case exemplifies the diversitiy of ocular manifestations of this disease. The authors recommend considering this condition in the differential diagnosis of posterior uveitis in young patients.

Adult↗

Optic disk edema with a macular star.

A 13-year-old boy presented with acute loss of vision in his right eye of 2 weeks' duration. He had a high fever and was ill for several days, then improved but suffered recurrent episodes of sweating and a high fever. Ophthalmoscopy of the right eye showed optic disk edema, mild vitreous cells, and minimal exudates in the macula. Bartonella henselae titers were positive. A diagnosis of optic disk edema with a macular star secondary to cat-scratch disease was made. The patient was treated with doxycycline and made a dramatic improvement to visual acuity of 20/30 with a minimal residual relative central scotoma. The optic disk edema and macular star resolved, and the patient was left with mild optic atrophy in the right eye.

Adolescent↗

[Warthin's tumor of the parotid. The signs of a case].

The authors report a case of Warthin's tumor associated with cat scratch disease placed in 2 nodes. Both arose at the same time and evolved rapidly. Diagnosis was made by histology after their removal. We underline the rarity of the pathology, the frequent diagnosis made after operation and recommend enuclouresection of neoplasm as the first choice, considering the benignity of the lesion and the modest aggression allowed.

Adenolymphoma↗

Bartonella henselae invasion of feline erythrocytes in vitro.

Bartonella henselae, the causative agent of cat scratch disease, establishes long-term bacteremia in cats, in which it attaches to and invades feline erythrocytes (RBC). Feline RBC invasion was assessed in vitro, based on gentamicin selection for intracellular bacteria or by laser confocal microscopy and digital sectioning. Invasion rates ranged from 2 to 20% of the inoculum, corresponding to infection of less than 1% of the RBC. Invasion was a slow process, requiring >8 h before significant numbers of intracellular bacteria were detected. Pretreatment of the bacteria with trypsin, or of the RBC with trypsin or neuraminidase, had no effect, but pronase pretreatment of RBC resulted in a slight increase in invasion frequency. The ability to model B. henselae invasion of feline RBC in vitro should permit identification of bacterial surface components involved in this process and elucidate the significance of RBC invasion to transmission and infection in cats.

Animals↗

Reactive immunoblastic proliferations.

The purpose of this report is to emphasize the criteria for recognizing the benign diseases such as viral lymphadenitis, necrotizing lymphadenitis, cat scratch disease, etc, in which a proliferation of immunoblasts is evident, and to discuss the criteria for distinguishing these diseases from neoplastic and atypical proliferations of immunoblasts. In this report, we use the term "immunoblasts" for transformed lymphoid cells of medium and large size. These cells may or may not have abundant quantities of cytoplasm, which may or may not be deeply staining. These immunoblasts may be of the B or the T cell type, and they may be mononuclear, binucleated, or multinucleated. The distinction between benign and malignant immunoblastic proliferations is not made on the basis of cytologic features, but by assessment of all histologic parameters including the architectural pattern, the cellular composition of the proliferation, the relationship of different cell types to one another, and the cytologic features of the immunoblasts.

Diagnosis, Differential↗

Neurologic complications of Bartonella henselae infection.

Bartonella henselae can cause cat scratch disease and bacillary angiomatosis, a multisystem disorder seen primarily in patients with the acquired immunodeficiency syndrome. Both of these diseases are associated with neurologic complications, particularly encephalopathy. B. henselae may also cause bacteremia and endocarditis, and has been associated with aseptic meningitis and with dementia in patients also infected with the human immunodeficiency virus. Recent advances in identification of this difficult-to-culture organism will lead to recognition of more neurologic complications.

AIDS Dementia Complex↗

Recent advances in pediatric infectious diseases and their impact on dermatology.

The past five years have seen numerous advances in the field of pediatric infectious diseases, and many of these have a substantial impact on the practice of dermatology. We review some of these advances and discuss their implications on etiology, diagnosis, therapy and complications of some relatively common conditions. The etiologic agent of exanthum subitum (roseola infantum) has been clearly implicated as a herpesvirus-6. Although in the classically described situation high fever in a young child is followed by defervescence and rash, two new scenarios have been described associated with this virus. The first is fever without rash and the second is rash without fever. The etiologic agent of erythema infectiosum ("slapped cheek") has been shown to be a human parvovirus B19. The virus has also been associated with aplastic crises (in hemoglobinopathies), hydrops fetalis, and a syndrome of subacute arthralgias in women. The etiologic agent in cat-scratch disease has recently been shown to be a small pleomorphic bacillus that also can produce pyogenic granuloma-like lesions in patients with acquired immunodeficiency syndrome. The number of cases of congenital syphilis, particularly in large cities, is increasing tremendously. Many of these infants have received no prenatal care because of drug abuse problems in their parents. Finally, we describe the changing etiology of impetigo that is predominantly associated with Staphylococcus aureus. We further describe the growing resistance to erythromycin and several new erythromycin drug-drug interactions.

Cat-Scratch Disease↗

Infection-associated vascular lesions in acquired immunodeficiency syndrome patients.

Several reports have recently appeared in the literature describing "unique" non-neoplastic vascular lesions in patients with the acquired immunodeficiency syndrome (AIDS). These lesions may be mistaken clinically and histologically for Kaposi's sarcoma. The terms epithelioid angiomatosis, epithelioid or histiocytoid hemangioma, and pyogenic granuloma have all been used to describe a similar entity in which cat scratch disease bacillus (CSDB) was subsequently identified. Lesions closely resembling this entity occur in patients with bartonellosis. We report a case of a cutaneous vascular lesion on the hand of an AIDS patient in which cytomegalovirus (CMV) and organisms consistent with CSDB were both found. Simultaneous infections with CMV and CSDB have not been previously described. The presence of these organisms in and around endothelial cells may provide the common stimulus for the formation of these reactive vascular proliferations.

Acquired Immunodeficiency Syndrome↗

[Peripheral lymphadenopathy in childhood--recommendations for diagnostic evaluation].

BACKGROUND: Enlargement of peripheral lymph nodes most commonly caused by a local inflammatory process is frequently seen in childhood. The aim of the present study was to analyze the most common causes of peripheral lymphadenopathy and to develop a simple algorithm for the primary diagnostic evaluation of peripheral lymph node enlargement in this age group. PATIENTS: Between April and September 1999 87 unselected children (median age: 5 1/2 years) with peripheral lymphadenopathy were referred to the Department of Pediatrics, University of Graz, for further investigation. RESULTS: EBV infection was diagnosed in 20 (23.0%) children. 19 (21.8%) patients had acute bacterial lymphadenitis. In 21 (24.1%) patients lymph node enlargement was classified as "post/parainfectious (viral)". Four patients each had toxoplasmosis and cat scratch disease. In 11 (12.6%) patients neither physical nor laboratory examinations revealed pathologic results. Among the remaining 8 children sarcoidosis and Hodgkin disease was diagnosed in one patient each. Small, soft, mobile, nontender, cervical, axillary or inguinal lymph nodes do not require further investigations. In case of enlarged, tender lymph nodes with overlying skin erythema and fever diagnostic evaluation should include complete blood count, erythrocyte sedimentation rate and/or c-reactive protein level, supplemented by appropriate antibody testing (EBV, CMV, Toxoplasma gondii, Bartonella henselae). Firm, enlarged, painless lymph nodes which are matted together and fixed to the skin or underlying tissues necessitate a more detailed diagnostic evaluation in order to exclude malignant or granulomatous diseases. CONCLUSIONS: Our study demonstrated that primary diagnostic evaluation of childhood peripheral lymphadenopathy is mainly based on clinical grounds. In most cases a small number of additionally performed laboratory tests allow to correctly identify the cause of the peripheral lymph node enlargement.

Adolescent↗

Bartonella henselae infection associated with neuroretinitis, central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss.

PURPOSE: To report a case of Bartonella henselae infection. DESIGN: Observational case report. METHODS: Review of the clinical, laboratory, photographic, and angiographic records of a patient with cat scratch disease associated with central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss. RESULTS: A 21-year-old man had no light perception in the left eye secondary to concurrent central retinal artery and vein occlusion believed to have resulted from infection with Bartonella henselae. Forty days later, he developed neovascular glaucoma in the left eye. CONCLUSION: Ocular complications associated with Bartonella henselae infection may include central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss.

Adult↗

Sonicated diagnostic immunoblot for bartonellosis.

Two simple Bartonella bacilliformis immunoblot preparation methods were developed. Antigen was prepared by two different methods: sonication of whole organisms or glycine extraction. Both methods were then tested for sensitivity and specificity. Well-defined control sera were utilized in the development of these diagnostic immunoblots, and possible cross-reactions were thoroughly examined. Sera investigated for cross-reaction with these diagnostic antigens were drawn from patients with brucellosis, chlamydiosis, Q fever, and cat scratch disease, all of whom were from regions where bartonellosis is not endemic. While both immunoblots yielded reasonable sensitivity and high specificity, we recommend the use of the sonicated immunoblot, which has a higher sensitivity when used to detect acute disease and produces fewer cross-reactions. The sonicated immunoblot reported here is 94% sensitive to chronic bartonellosis and 70% sensitive to acute bartonellosis. In a healthy group, it is 100% specific. This immunoblot preparation requires a simple sonication protocol for the harvesting of B. bacilliformis antigens and is well suited for use in regions of endemicity.

Bartonella↗