Candidemia, look at the eyes.

Two patients with disseminated candidiasis are presented. The diagnosis was established by fundoscopy. Candida endophthalmitis can be the only manifestation of disseminated candidiasis. The role of fundoscopy in the management of candidemia is emphasized because it can provide evidence of disseminated disease. Every positive blood culture requires repeated fundoscopic examination. Early recognition is essential to prevent irreversible loss of vision.

Properties of the residual alpha-galactosidase activity in the tissues of a Fabry hemizygote.

The properties of the residual alpha-galactosidase activity in kidney, liver, spleen, fibroblasts and urine of a Fabry hemizygote have been studied using p-nitrophenyl-alpha-galactoside and 4-methylumbelliferyl-alpha-galactoside as substrates. In addition, alpha-galactosidase activity in urine has been determined with ceramidetrihexoside as substrate. The residual alpha-galactosidase activity of Fabry, measured with artificial substrate, is stimulated (6-35%) by myo-inositol and only slightly inhibited by melibiose (7-17%) in all the materials used. In contrast, the alpha-galactosidase of normal tissues and urine is inhibited (36-48%) by myo-inositol and inhibited to a much greater extent (40-50%) by melibiose. The KM for artificial substrate of the residual activity of Fabry is higher than that of the alpha-galactosidase in normal kidney, liver, spleen, fibroblasts and urine. The residual activity of Fabry is generally more stable to heating than the activity in the normal materials, although exceptions were noted. When these properties are compared with those of the alpha-galactosidase isoenzymes in normal tissues and body fluids, the residual activity of Fabry material seems to be very similar to the minor component of normal tissue (alpha-galactosidase B). Moreover, the pH optimum curve of this minor component and of the Fabry alpha-galactosidase in urine are similar, whereas the major isoenzyme (alpha-galactosidase A) shows a curve much more like that of normal urine. The findings with ceramidetrihexoside as substrate indicate a possible discrepancy. Alpha-Galactosidase A hydrolyses ceramidetrihexoside, Fabry urine preparation does not. However, alpha-galactosidase B of normal urine shows a slight but definite ceramidetrihexosidase activity. No contamination of the B preparation with alpha-galactosidase A could be detected. The minimum hypothesis, supported by most of the experimental evidence, is that the residual activity of Fabry and normal alpha-galactosidase B are identical.

Physico-chemical and immunological properties of acid alpha-glucosidase from various human tissues in relation to glycogenosis type II (Pompe's disease).

The physico-chemical and immunological properties of acid alpha-glucosidase from various human tissues have been studied. Heat stability of acid alpha-glucosidase from heart, liver and skeletal muscle is identical, but for kidney some different results are obtained. Identical isoelectrofocussing patterns are found for heart, liver and skeletal muscle. Furthermore, the effect of antiserum against human liver acid alpha-glucosidase on the activity of acid alpha-glucosidase from various tissues is studied. The results are discussed in relation to glycogenosis type II (Pompe's disease).

Disseminated Penicillium marneffei infection as an imported disease in HIV-1 infected patients. Description of two cases and a review of the literature.

Two cases of disseminated Penicillium marneffei infection, as an imported disease, in HIV-1-infected patients with a severe immunodeficiency are reported. These patients had a history of travel in Southeast Asia where P. marneffei is endemic. Fever, cough, malaise, hepatosplenomegaly, anaemia, skin lesions and mucosal ulcers are the main clinical characteristics. Differentiation from histoplasmosis and leishmaniasis might be difficult. Treatment with amphotericin B was successful. Anti-fungal maintenance therapy is most likely indicated.

PIP: A 33-year-old, HIV-1 positive, white, homosexual man was hospitalized in May, 1991, because of fever, cough, skin eruptions, anorexia, and weight loss during the previous 2 months. In October, 1990, he had traveled in Sumatra. On examination he was ill, tachypneic, normotensive with a temperature of 39.1 degrees Celsius. The spleen was substantially enlarged. Laboratory investigations showed: ALAT 72 U/I (normal 23 U/1), LDH 508 U/1 (normal 275 U/1). A bronchoscopy with bronchoalveolar lavage revealed yeast cells. Gastroscopy showed an ulcer in the hypopharynx and an erosion in the stomach. Biopsies of this ulcer demonstrated the presence of Penicillium marneffei. Biopsies of the liver showed the same organism. The patient was treated with amphotericin B induction therapy (1 dd 0.5 mg/kg for 21 days, total dose of 730 mg) in combination with flucytosine (3 dd 2500 mg, total dose 142 g in 19 days). In the following 2 weeks the temperature became normal, and the dyspnea and the skin eruptions disappeared, except for the mollusca contagiosa. The spleen diminished by 50%. LDH and ALAT became normal. Oral maintenance therapy followed with fluconazole (the first 3 months 400 mg daily, followed by 200 mg a day). 24 months later, no recurrence had been observed. Case 2 was a 28-year-old, HIV-infected, homosexual man, born in Suriname, who was hospitalized in October, 1991, with prolonged fever, dyspnea, and a painful throat. In March, 1991, he had traveled in rural Thailand. AIDS was diagnosed on the basis of cerebral toxoplasmosis in August, 1991. A biopsy of the ulcer in the oropharynx showed an active aspecific inflammation and also P. marneffei. Treatment with amphotericin B intravenously (0.5 mg/kg, total dose 1052 mg in 32 days) was commenced. The lesions in the oral cavity and throat, the lymph nodes, and the shortness of breath disappeared within a few days. Ten months later he died from emaciation caused by cryptosporidiosis.

[Mycobacterium haemophilum as the cause of lymphadenitis in the neck in an otherwise healthy boy]. / Mycobacterium haemophilum als veroorzaker van lymphadenitis colli bij een niet-zieke jongen.

In an 8.5-year-old boy with severe recurring unilateral swelling of the neck, infection with Mycobacterium haemophilum was established. In addition to the more usual causative agents, cervical lymphadenopathy in children can, in rare cases, be caused by M. haemophilum. The skin tests did not differentiate between Mycobacterium avium and Mycobacterium tuberculosis infection. There was no evidence of specific immune deficiencies. The swelling did not respond to treatment with four tuberculostatic drugs (rifampicin, isoniazid, pyrazinamide and ethambutole), nor to repeated surgical excision. Finally, M. haemophilum grew on the culture despite sub-optimal culture conditions. Thereupon the patient was treated with co-trimoxazole and recovered in two weeks. This is the first patient with cervical lymphadenopathy caused by M. haemophilum to be described in the Netherlands. M. haemophilum grows on an iron-rich medium at a relatively low temperature (30-32 degrees C). In cases of cervical lymphadenopathy, it is advisable to consider M. haemophilum as a cause and to adjust the culture conditions accordingly.

[Extra-intestinal infections caused by Vibrio spp. in The Netherlands]. / Extra-intestinale infecties door Vibrio spp. in Nederland.

Six patients with serious cellulitis or ear infection due to Vibrio vulnificus, V. parahaemolyticus or V. alginolyticus are described. Four patients were infected during the summer in the Netherlands and two outside the Netherlands. Contact with seawater was the most probable source in four patients. In two patients the infection, caused by V. vulnificus, was transmitted by eel. This is a new way of transmission.

[Reducing the risk of blood-transmitted infections of HIV, hepatitis B or C virus in a teaching hospital in Amsterdam--evaluation of a protocol for needlestick accidents among hospital staff during the period 1997-2001]. / Risicoreductie van bloedoverdraagbare besmetting met HIV, hepatitis-B- of -C-virus in een opleidingsziekenhuis in Amsterdam--evaluatie van een protocol voor 'prikaccidenten' bij ziekenhuismedewerkers, 1997-2001.

OBJECTIVE: To evaluate a protocol for hospital staff aimed at reducing their risk of exposure to blood-transmitted infections. DESIGN: Prospective. METHOD: In August 1997 a protocol was introduced to the Onze Lieve Vrouwe Gasthuis Hospital in Amsterdam, with procedures to be followed after percutaneous or mucocutaneous blood contact in which there was a chance of transmission of hepatitis B (HBV) or C (HCV), as well as guidelines for the prescription of post-exposure prophylaxis (PEP) after accidents with an HIV risk. In the period 1 August 1997-30 June 2001 data were collected from registration forms that reported accidents and the ensuing events. RESULTS: A total of 403 accidents were reported by 138 (34.2%) physicians, 135 (33.5%) ward nurses, 46 (11.4%) operation assistants, 30 (7.4%) co-assistants, 21 (5.2%) analysts and 33 (8.2%) persons with another position. There was a constant increase in the number of reports over the period. The seroprevalence of the source patients was: 6.9% (25/360) HIV, 8.1% (6/74) HBV and 6.3% (23/363) HCV. PEP was prescribed on 46 occasions: 15 times for an HIV positive source and 31 times for what was initially an unknown HIV source. Following the introduction of a rapid HIV test in September 1999, there was a reduction in the number of unnecessary PEP prescriptions from 4 in 1997, 7 in 1998 and 16 in 1999 to 3 in 2000 and 1 in 2001. All 15 persons who were correctly started on a PEP treatment took the medication for a period of 28 days despite many side effects. No seroconversions were established during the follow-up period of 6 months.