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Recurrent viral associated hemophagocytic syndrome in a child with Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) with subsequent viral-associated hemophagocytic syndrome (VAHS) or secondary hemophagocytic lymphohistiocytosis (HLH) is extremely rare. A 15-month-old girl with disseminated LCH experienced three episodes of VAHS during maintenance therapy. Viral infection, with influenza A, herpes simplex, and adenovirus, respectively, was documented at each episode. She recovered each time after interruption of maintenance therapy. The occurrence of fever and pancytopenia in patients with chemotherapy-treated LCH can be associated with VAHS and not with relapsing LCH.

Adenoviruses, Human↗

[Duration of treatment of nosocomial pneumonia. What judgement criteria to use?].

AN UNRESOLVED ISSUE: Inappropriate duration of antibiotic treatment is one of the factors explaining the high mortality of nosocomial pneumonia. There are however few data on the ideal duration of treatment. An improvement in the radiological image is not a good criterion. The right duration would be one that is necessary and sufficient to achieve cure and avoid recurrence and relapse and also one that avoids the drawbacks of prolonged treatment. LIMITATIONS OF CLINICAL CRITERIA: High-grade fever, an alveolar image on the chest x-ray, and a high white cell count are synonymous with bacterial pneumonia in only 40 to 60% of the cases. PRIMARY AND SECONDARY INFECTION: Using reliable microbiological methodology is has been possible to demonstrate that the causal germs of primary infections disappear by day 3 of an adapted treatment but that early secondary infection occurs in 14% of the cases. PROSPECTS FOR PROGRESS: An open multicentric randomized study is being conducted in France to compare 7 day versus 14 day treatment against identified germs (irrespective of the strain isolated) using reliable microbial sampling techniques.

Cross Infection↗

Application of Transmission Electron Microscopy for Analysis of Ultrastructural Organization of Circulating Immune Complexes in Patients with Typhoid Fever and Chronic Carriers of Salmonella typhi.

Transmission electron microscopy was used to study the ultrastructural organization of circulating immune complexes (CIC), isolated from patients with typhoid fever in the different periods of acute infectious process (febrile period, period of an early and late reconvalescence), relapse and from acute and chronic carriers of Salmonella typhi in the period of pathogen excreting. It has been shown that preparations of CIC from healthy donors consisted of amorphous mean electron density material, including a cell-like detritus. At acute and chronic infectious process there were bacterial cells in a structure of the CIC. Depending on the period of disease, bacteria had different ultrastructural organization in the CIC. In the febrile period, in the period of an early reconvalescence and in the period of formation of acute carriage of S. typhi, and also in relapse, bacteria had a typical structure, with reference to gram-negative microorganisms. In the period of formation of acute and chronic carriers of S. typhi, in a period of excretion of S. typhi, bacteria in the CIC had ultrastructural organization, relevant to the forms of bacteria with a defective cell wall. Immunocytochemistry research made with the purpose of visualization the O-antigen of S. typhi in bacteria has demonstrated positive immunolabeling on the O-antigen in the bacterial forms and forms with a defective cell wall, and on amorphous mean electron density material. The analysis of ultrastructural organization of the circulating immune complexes and immunolabeling on the O-antigen of S. typhi have allowed to conclude that S. typhi, both typical bacterial form, and the forms with a defective cell wall were the main structural component of circulating immune complexes in acute and chronic forms of infectious process.

Journal Article↗

A single cycle of 2-chlorodeoxyadenosine results in complete remission in the majority of patients with hairy cell leukemia.

Twenty-six patients with hairy cell leukemia (HCL) were treated with 2-chlorodeoxyadenosine (2-CdA), a purine analogue resistant to adenosine deaminase, at 0.1 mg/kg/d for 7 days by continuous intravenous infusion. Fifteen patients were previously untreated, while 11 patients had received prior treatment with splenectomy alone (three patients), interferon alpha alone (four), splenectomy, then interferon alpha (two), or splenectomy, interferon alpha, then 2-deoxycoformycin (2-DCF) (two). Sixteen (80%) of 20 patients evaluable at 3 months achieved complete remission (CR), and four (20%) achieved partial remission (PR) following a single cycle of therapy. All four patients in PR had complete recovery of their peripheral blood counts (except one patient whose platelet count remained 84,000/microL), but had residual HCL in the bone marrow (three patients) or residual splenomegaly (one). Patients with bulky adenopathy, massive splenomegaly, and severe pancytopenia responded as well as those with only modest marrow involvement. The three patients with residual marrow disease received a second cycle of 2-CdA, and two have attained CR. Therefore, 18 of 20 (90%) achieved CR with either one or two cycles of therapy. No patient achieving CR has relapsed at a median follow-up of 12 (+/- 2.1) months. Toxicities included myelosuppression and culture-negative fever. A community-acquired pneumonia was the only infectious complication. Since a single cycle of 2-CdA induces sustained CR in the vast majority of patients with minimal toxicity, this agent is emerging as the treatment of choice for all patients with HCL.

Bone Marrow↗

[Five cases of de novo acute myeloid leukemia with trilineage myelodysplasia (AML/TMDS) achieved CR with the continuous drip infusion of low-dose etoposide and low-dose cytosine arabinoside combined with mitoxantrone (MEtA)].

From 1998 to 2001, 5 consecutive cases of AML/TMDS entered our hospital and achieved complete remission (CR) with continuous drip infusion of low-dose etoposide and low-dose Ara-C combined with mitoxantrone (MEtA regimen). The ages of the 5 patients (4 males and 1 female) ranged 32 to 50 years-old, respectively. WBCs were 1,560-45,150/microl, blasts were 12-62%. Bone marrow aspirates revealed trilineage myelodysplasia with various number of blasts. These patients had an acute onset and no preceding hematologic disorders. They were diagnosed M2/TMDS or M4/TMDS. Continuous drip infusion of etoposide (50 mg/body/day) and Ara-C (30 mg/body/day) were given for 11-14 days and a bolus injection of mitoxantrone (8 mg/m2) was added for 2-3 days. Patient 5 was given additional MIT (6.7 mg/m2 on day 6). All cases achieved CR in 21-24 days after the end of the therapy. Toxicities were nausea, vomiting, stomatitis, alopecia and fever due to infection. All were well tolerable, however. Two patients are alive more than 4 years without relapse. MEtA regimen is available for AML/TMDS.

Adult↗

[Treatment of atypical pneumonia with josamycin].

In the present study the clinical efficacy and tolerance of josamycin (1 g every 12 hours) was prospectively evaluated for the empirical therapy of patients below 70 years with community acquired pneumonia of atypical presentation (AP), without respiratory failure, radiological cavitation or risk factors of pharyngeal colonization by gram-negative bacilli. During a 28-month period 168 patients adequate for the study were included. The etiological diagnosis was established in 56 cases (33.3%); in 55 by seroconversion (28 Mycoplasma pneumoniae, 22 Legionella pneumophila, 1 Chlamydia psittaci and 4 Coxiella burnetii) and in only one case by positive blood culture (Streptococcus pneumoniae). The mean duration of therapy was 9 days and that of fever 1.4 days. Five patients (3%) had mild transient gastrointestinal complaints. No patient required a change of therapy. Relapses were not observed in the 45 days of follow up. In conclusion, josamycin (1 g/12 h p.o.) is an effective and well tolerated antibiotic for the therapy of pneumonia by M. pneumoniae or L. pneumophila, and it represents a good empirical treatment of AP in patients below 70 years without risk factors of GNB infection, respiratory failure or radiological cavitation.

Adolescent↗

Juvenile relapsing periarteritis nodosa and streptococcal infection.

Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, HIV) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased. His condition improved following the administration of oral steroids but he experienced relapses 5 and 12 years later when penicillin withdrawal was attempted. The flares were accompanied by a major increase of antistreptolysin titre and response to corticosteroid was obtained. He is currently 38 years old and he remains well on prophylactic penicillin. Polyarteritis nodosa in children may occur after a streptococcal infection. It may be prudent to consider penicillin prophylaxis in patients with periarteritis nodosa when a streptococcal etiology is documented or highly suspected.

Adolescent↗

Once daily ceftriaxone vs. chloramphenicol for treatment of typhoid fever in children.

In a prospective, randomized, open study ceftriaxone was compared with chloramphenicol for treatment of 59 children who had bacteriologically confirmed acute typhoid fever. Ceftriaxone was administered intramuscularly in a once a day dose of approximately 80 mg/kg body weight for 5 days. Chloramphenicol was given orally for 3 weeks in a daily dosage of 50 to 100 mg/kg body weight divided into 4 doses. In the ceftriaxone group 23 of 29 patients were cured, 2 improved, 1 failed and 2 relapsed. For those who received chloramphenicol 22 of the 30 patients were cured, 5 improved, 1 failed and 2 relapsed. In none of the patients in either group were adverse effects recorded. The overall results of this comparative study suggest that short term treatment of typhoid fever with ceftriaxone is as effective and safe as conventional treatment with chloramphenicol.

Acute Disease↗

[Retroperitoneal liposarcoma. Clinico-pathological analysis of 6 cases].

Six patients with retroperitoneal liposarcoma are reported. Their mean age was 58,66 years. There were four males and two females. The symptoms or signs at presentation were: abdominal mass (5 cases); abdominal pain (3 cases); sustained fever (2 cases), and lower limb edema (1 case). Abdominal computed tomography (CT) showed large masses with different density. Distant metastases were not documented. All patients underwent laparotomy, and the tumor was not resectable in 2 cases. In the histological study, liposarcoma was shown to be myxoid in 4 cases, pleomorphic in one and round cell type in the remaining case. In 3 patients local relapse was observed. The longest survival was 38 months and the shortest one 3 months. It was concluded that the leading cause for consultation in these patients was an abdominal mass. Remarkably, two patients had the uncommon feature of sustained tumoral fever. CT was shown to be the most useful diagnostic method. The good general condition of the patients with large tumoral masses was remarkable. The most common variety was myxoid, local relapse was frequent and the survival was poor.

Adult↗

Difficulties in the prevention, diagnosis, and treatment of imported malaria.

BACKGROUND: Imported malaria is quite common in the United States. Increasing antimalarial drug resistance and changes in travel patterns may have important implications for the prevention, clinical presentation, and management of this disease. METHODS: Medical records were reviewed for 121 patients with microscopically confirmed malaria diagnosed at 2 university-affiliated hospitals in San Francisco, Calif, between 1988 and 1997. RESULTS: Among 57 travelers from the United States, only 13 (23%) had been compliant with an appropriate chemoprophylactic regimen. No patients developed falciparum malaria after consistent chemoprophylactic therapy with mefloquine hydrochloride. However, 12 (19%) of US residents with imported malaria developed Plasmodium vivax or Plasmodium ovale infections despite an appropriate chemoprophylactic regimen, generally with a late onset suggestive of relapsing disease. Clinical presentations were similar between foreign residents and American travelers and between patients with falciparum and nonfalciparum infections; 98% of patients had a history of fever. Sixteen percent of patients had received previous evaluations during which the diagnosis of malaria was not considered. In 9% of patients, there were errors in treatment. Only 1 patient developed severe malaria. CONCLUSIONS: Our results suggest that a standard chemoprophylactic regimen is highly effective in preventing falciparum malaria, but that many American travelers do not receive it. Also, relapsing P vivax or P ovale infection despite appropriate chemoprophylactic therapy was not uncommon among our cases. The presentation of imported malaria is nonspecific, highlighting the need to consider the diagnosis in any febrile patient who has been in a malaria-endemic area. Although errors in diagnosis and treatment were quite common in our study population, patient outcomes were good once the appropriate therapy was initiated.

Adolescent↗

Effects of two different bisphosphonates on Paget's disease of bone: ICTP assessed.

Clodronate and alendronate were compared in 27 patients with active Paget's bone disease. Carboxyterminal crosslinked telopeptide of type I collagen (ICTP) was evaluated as a marker of bone turnover in Paget's bone disease. Group 1. Nineteen patients received clodronate infusions (300 mg/daily) on 5 consecutive days. After 1 year, 12 patients (63%) were still in remission; urinary hydroxyproline (64.8%) and serum alkaline phosphatase (59.4%) were significantly reduced and had returned to normal in 30%. Patients in remission had significantly higher basal values of urinary hydroxyproline. No adverse side effects were observed. Group 2. One year after clodronate, seven relapsing patients retrospectively underwent five consecutive infusions of alendronate (5 mg/daily). Within 12 months, urinary hydroxyproline fell by 74.7%, alkaline phosphatase dropped by 75.2%, osteocalcin by 47.3%, and ICTP by 56.4%. In all patients, urinary hydroxyproline and alkaline phosphatase returned to normal within 3 months and remained within the normal range during the 12-month follow-up. Most patients had mild, short course fever and arthromyalgia. Group 3. Eight newly diagnosed pagetics, received alendronate alone (5 mg/daily for 5 days). All patients responded well to alendronate within the first month. None suffered a relapse during the follow-up. At month 12, urinary hydroxyproline was down by 71.4%, alkaline phosphatase by 75.3%, osteocalcin by 58.1%, and ICTP by 67.4%. In all patients, markers of bone remodeling were in the normal range at the end of the follow-up. Moderate, transitory arthromyalgia, and fever (high and lasting for 7 days in only one case) were observed in half of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Infections in acute lymphoblastic leukaemia.

We did a retrospective study of all acute lymphoblastic leukaemia (ALL) patients on United Kingdom ALL protocol who were admitted for febrile neutropenia. The aim of the study was to document the types of infections and aetiological agents associated with febrile neutropenia and to document the factors affecting mortality. Over the 8 1/2-year period from 1986 to June 1995, there were 77 episodes in 32 children with a mean of 2.4 episodes. Morbidity due to infection was 61%; unknown causes of fever contributed 39%. Of the microbiologically documented infections, majority were Gram-negative bacteraemia. There were 7 deaths (22%) during the study period, 3 (9%) of which were due to overwhelming sepsis, with 4 contributed by the relapse status of the leukaemia. Mortality was increased by prolonged neutropenia, relapse of the leukaemia and invasive fungal infection.

Antineoplastic Combined Chemotherapy Protocols↗

Successful treatment of typhoid fever in children with parenteral ceftriaxone.

Eight children, seriously ill with bacteriologically proven Salmonella typhi septicemia, were successfully treated with a single daily intramuscular injection of 2 g ceftriaxone given for 5-7 days. All children improved clinically within 48 h of starting therapy and all except 1 became afebrile within 5 days. None relapsed during the 4-week follow-up study.

Ceftriaxone↗

Azithromycin versus ceftriaxone for the treatment of uncomplicated typhoid fever in children.

A total of 108 children aged 4-17 years were randomized to receive 7 days of azithromycin (10 mg/kg/day; maximum, 500 mg/day) or ceftriaxone (75 mg/kg/day; maximum, 2.5 g/day), to assess the efficacy of the agents for the treatment of uncomplicated typhoid fever. Salmonella typhi was isolated from the initial cultures of blood samples from 64 patients. A total of 31 (91%) of the 34 patients treated with azithromycin and 29 (97%) of the 30 patients treated with ceftriaxone were cured (P>.05). All 64 isolates were susceptible to azithromycin and ceftriaxone. Of the patients treated with ceftriaxone, 4 subsequently had relapse of their infection. No serious side effects occurred in any study subject. Oral azithromycin administered once daily appears to be effective for the treatment of uncomplicated typhoid fever in children. If these results are confirmed, the agent could be a convenient alternative for the treatment of typhoid fever, especially in individuals in developing countries where medical resources are scarce.

Adolescent↗