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Infantile acute hemorrhagic edema and rotavirus infection.

Infantile acute hemorrhagic edema (AHE) is a benign condition characterized by a dramatic onset of inflammatory edema and ecchymotic purpura in a target or cockade pattern. It is considered an uncommon form of cutaneous vasculitis occurring in children younger than 2 years of age. The outbreak is frequently preceded by an immunization or various infections. We describe an 11-month-old girl with rosette-shaped purpuric plaques on the face and limbs, clinically consistent with a diagnosis of AHE of the skin, associated with fever and diarrhea. Laboratory investigations showed a rotavirus infection, which has not previously been reported in association with AHE of the skin. The disease had a benign course without relapses. Appropriate microbiologic investigations are advisable to confirm the possible etiologic role of rotavirus.

Diagnosis, Differential↗

Unusual presentation of necrotizing fasciitis in a patient who had achieved long-term remission after irradiation for testicular cancer.

We report a case of a 60-year-old man with necrotizing fasciitis complicated by streptococcal toxic shock syndrome. The patient had received high-dose chemotherapy and radiotherapy to the pelvis for relapsed seminoma 7 years previously. He had been in long-term remission. He was admitted to the Tsukuba University Hospital, Tsukuba-City, Ibaraki, Japan, with complaints of fever and localized erythema over the foreskin. The patient suffered from septic shock and multiple organ failure. Despite intensive care, he died 18 h after admission. Streptococcus pyogenes was isolated from both the wound and blood culture. To our knowledge, this is the first description of necrotizing fasciitis primarily affecting the penile skin.

Antineoplastic Agents↗

Fluconazole therapy for pulmonary mucormycosis complicating acute leukemia.

A 29-year-old woman with acute myelogenous leukemia in relapse at the end of a nine-month period of remission was admitted to hospital where intensive antileukemic therapy was started. Antibiotics were given when she developed a fever and, when oral thrush appeared, intravenous amphotericin B was initiated. After 16 days, the amphotericin B (at a total dose of 295 mg) was discontinued because of side effects and 12 days later, when a lung biopsy had revealed mucormycotic hyphae in infarcted tissue in the left upper lobe, fluconazole (300 mg daily by intravenous infusion over a period of two hours) was substituted. This was continued for one month with clinical and radiologic improvement in the lung condition and no attributable adverse effect. At this juncture the patient died of intractable heart failure. We suggest that fluconazole may be an acceptable alternative to amphotericin B in the treatment of pulmonary mucormycosis.

Adult↗

Viridans streptococcal endocarditis.

Although responsible for a declining proportion of cases of infective endocarditis, viridans streptococci remain the commonest cause and accounted for 30% of our cases seen in Huntsville, Alabama. Usually viridans streptococcal endocarditis is associated with dental manipulation or infection and underlying heart disease but both conditions were not common in our series. Similar to the experience of other investigators, our patients exhibited a subacute course with fever, dyspnea, weight loss and heart murmur. Echocardiography, reported useful in detecting vegetations in one series, was not a sensitive diagnostic tool in our patients. We confirmed the low relapse rate and mortality rate associated with earlier reports of viridans streptococcal endocarditis but observed a high rate of complications, as noted in a recent series.

Adult↗

Progressive disseminated histoplasmosis in patients with the acquired immune deficiency syndrome: a report of 12 cases and a literature review.

Progressive disseminated histoplasmosis is an increasingly common cause of infection in patients with acquired immune deficiency syndrome (AIDS) from areas endemic for histoplasmosis. We report 12 cases of progressive disseminated histoplasmosis associated with AIDS and review 20 previously reported cases. The clinical presentation of progressive disseminated histoplasmosis is nonspecific with persistent fever, weight loss, and splenomegaly. Frequently progressive disseminated histoplasmosis was the initial clue to the presence of AIDS. Bone marrow culture is the best method for establishing a diagnosis. Relapses were common both with ketoconazole and after a 2.0 to 2.5 g course of amphotericin B. This suggests a 2.0 to 2.5 g course of amphotericin B followed by long term ketoconazole suppression may be the best therapeutic regimen in these patients.

Acquired Immunodeficiency Syndrome↗

Five-day treatment of non-severe, community-acquired pneumonia with josamycin.

This study assessed the efficacy of oral josamycin 1 g bd for five days as treatment for non-severe, community-acquired pneumonia in patients less than 60 years of age who were not at obvious risk of developing respiratory tract infection caused by aerobic Gram-negative bacilli. Of the 84 patients (43 male, 41 female) with a mean age of 33 years who were enrolled during a 14-month period, the clinical outcome was invariably favourable. All patients became afebrile within three days of starting therapy; the mean duration of fever after initiating treatment was 1.7 days. Therefore, according to the study protocol, josamycin therapy was discontinued on day five. A chest X-ray performed four to six weeks after completing treatment was normal in every case and no relapses were observed during a six-week follow-up period. We conclude that a five-day course of josamycin is effective monotherapy for community-acquired pneumonia in patients without the clinical features of severe infection.

Adolescent↗

Successful treatment of refractory visceral leishmaniasis in India using antimony plus interferon-gamma.

Fifteen Indian patients with relapsing or drug-refractory visceral leishmaniasis were retreated for 30 days with antimony plus interferon-gamma (IFN-gamma). All 15 had failure of an initial course of antimony and at least one additional course of antimony or pentamidine; 7 patients had failure of three or four prior courses of therapy. During the study, treatment was discontinued in 2 patients because of anemia and congestive heart failure in 1 and intractable vomiting in the other; both subsequently died. In the remaining 13 patients, IFN-gamma plus antimony treatment was associated with daily fever but no other adverse reactions. After 30 days of therapy, 9 (69%) of the 13 patients were apparently cured. Six months after treatment, all 9 were healthy, had parasite-free bone marrow aspirate smears, and were considered cured. None have relapsed during a mean follow-up of 15.9 +/- 1.7 months. These results support the use of antimony plus IFN-gamma as an immunochemotherapeutic alternative for kala-azar patients who have repeated failures of conventional treatment.

Adolescent↗

Toxic shock syndrome in menstruating women.

Eight adult women became severely ill with an acute, toxic erythroderma during menses. The syndrome was characterized by fever, generalized erythema, profound shock, multiple organ dysfunction, and desquamation occurring several days after the rash had faded. Gastrointestinal and cardiovascular abnormalities were present in all patients; three patients required ventilatory support; dialysis was performed on two; and one patient died. Mild relapse occurred in two patients during subsequent menses; the other patients have recovered without sequelae during follow-up of 6 to 42 months. Cervical colonization or local infection with Staphylococcus aureus is associated with this syndrome.

Acute Kidney Injury↗

Head and neck manifestations of relapsing polychondritis: review of 29 cases.

This review of 29 patients with relapsing polychondritis seen at the Mayo Clinic between 1962 and 1976 emphasizes the head and neck manifestations of the disease and the role of the otolaryngologist in its diagnosis and treatment. The major clinical features included inflammation of the pinna, eye involvement, nasal cartilage involvement, laryngotracheal involvement, arthropathy, hearing loss, costal chondritis, and fever. The erythrocyte sedimentation rate was often elevated, and mild anemia was not uncommon.

Adolescent↗

Childhood brucellosis in north-western Greece: a retrospective analysis.

UNLABELLED: Fifty-two cases of childhood brucellosis which occurred in north-western Greece during the 15-year period 1979-1993, are reviewed. It is believed that they represent very closely the total incidence of the disease in the region which has a population of 100,000 children aged 0-14 years old. Brucellosis-affected children were almost exclusively from goat- or shepherd families and of both sexes and all age groups. A broad spectrum of clinical manifestations ranging from malaise only to brain abscess was observed. Fever and arthralgia were the most common manifestations followed by malaise, myalgia, sweating, rash, cough, and gastro-intestinal, cardiac and CNS involvement. Splenomegaly was found more often than hepatomegaly and lymphadenopathy. Laboratory findings included anaemia, leukopenia, neutropenia, lymphocytopenia, monocytosis, eosinophilia, thrombocytopenia and pancytopenia. Leukocytosis and lymphocytosis were extremely rare and ESR and serum C-reactive protein levels were mildly elevated. All patients had positive Rose Bengal slide agglutination tests and standard tube agglutination titres of 1:160 or more. When performed, blood culture was often diagnostic. The children were treated with streptomycin for 2 weeks plus either tetracyclines or trimethoprim-sulphamethoxazole for 3 weeks. Treatment was well tolerated. Relapse was observed in one case. CONCLUSION: Brucellosis nowadays affects children in an occupational pattern. As symptoms, signs and first-line laboratory findings are not characteristic, agglutination tests and blood culture should be performed in any child with prolonged fever. Treatment is effective, but prevention of the disease by animal testing and education of high risk families is indicated.

Adolescent↗

Treatment with ciprofloxacin in children with typhoid fever.

We investigated the use, treatment results and safety of ciprofloxacin in the treatment of childhood typhoid and paratyphoid infections in an industrialized country. The study was retrospective, and the material consisted of children admitted to Hvidovre or Glostrup University Hospitals from 1991 to 1995, and treated with ciprofloxacin for a culture proven diagnosis of typhoid fever. 21 children were included, 18 had positive cultures for Salmonella typhi and 3 had positive cultures for S. paratyphi A. All isolates were fully susceptible to ciprofloxacin. The median duration of treatment was 10 d, median oral dose was 24 mg/kg/d and the median intravenous dose was 15 mg/kg/d. Within 4 d after start of treatment all subjects had a normal body temperature. No subjects had clinical or microbiological relapse and all stool cultures after end of treatment were negative. Adverse events were rare, but in 2 children a transient limb/ataxia or a period of confusion were recorded. Both children recovered within a few days without sequelae. We conclude that ciprofloxacin was effective and well tolerated for treatment of typhoid fever in children. The few adverse events that were recorded left no permanent sequelae, and were likely to be caused by the disease itself.

Adolescent↗

[A "retinoic acid syndrome" observed in two cases of acute promyelocytic leukemia].

Two cases of acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) developed fever, dyspnea and chest pain. A chest roentgenogram showed bilateral pleural effusion (case 1) and bilateral interstitial infiltration (case 2). The first case was a 50-year-old female in her first relapse, who was initially diagnosed as having pleuritis tuberculosa and was treated with anti-tuberculotic agents. Her symptoms continued for 44 days and complete remission was achieved 53 days after commencing ATRA therapy. The second case was a previously untreated 46-year-old male. His case had been diagnosed as adult respiratory distress syndrome and he had been treated with prednisolone. His symptoms rapidly improved and complete remission was achieved 38 days after the ATRA therapy. This was the first report of patients in Japan considered to have developed "retinoic acid syndrome (RAS)". In our five APL cases treated with ATRA, the syndrome was not always accompanied by peripheral blood leukocytosis even though the two cases with RAS showed higher leukocyte counts than the other two cases without RAS and also had DIC. We should pay attention to the severe respiratory symptoms that develop in APL patients after ATRA treatment and immediate steroid therapy is required for such patients.

Adult↗

Rapid laboratory confirmation of human brucellosis by PCR analysis of a target sequence on the 31-kilodalton Brucella antigen DNA.

We developed a PCR-based assay for the rapid and specific laboratory diagnosis of human brucellosis directly from whole blood. Specimens were collected in EDTA tubes from 17 patients with acute serologic brucellosis and 3 patients with chronic relapsing brucellosis as determined by serologic tests and the patient's clinical picture. DNA was extracted from peripheral mononuclear cells obtained from the blood of patients with brucellosis and control individuals. Specific primers for the PCR amplification of a 223-bp region on the sequence encoding the 31-kDa immunogenic Brucella abortus protein (BCSP 31) were used. All amplicons had the expected size of 223 bp. The specificity of amplification was determined by Southern hybridization and restriction endonuclease analysis. DNA extracted from blood taken from 30 healthy individuals as well as from 9 patients with typhoid fever did not show any amplification with the primers used. The test proved to be rapid and specific for the laboratory confirmation of acute human brucellosis. Further studies must be conducted to assess the utility of this test on additional patients with chronic relapsing brucellosis as well as patients under treatment.

Acute Disease↗

[Total body irradiation, vincristine in continuous infusion and high-dose melphalan with transplant of autologous bone marrow in the treatment of neuroblastoma].

High dose chemo-radiotherapy followed by autologous bone marrow transplantation (ABMT) is known to be an effective treatment in stage IV neuroblastoma (NB). Since October '84, 19 children with NB (12 relapsed or resistant: Group A; 7 in first CR: Group B) received ablative therapy (AT) consisting of VCR (4 mg/mg), L-PAM (140 mg/mg) and fractionated TBI (1000 Rads). Induction strategy at diagnosis or at relapse included high dose Peptichemio, 2-3 cycles of Vincristine-Cyclophosphamide--high dose Platinum and surgery. Bone marrow was harvested after 2 evaluation proved negative by cytomorphology, histology and immunofluorescence. Mononuclear cells (median 6.7 x 10(7)/kg) were cryopreserved and reinfused without purging. At the time of AT in Group A8 children were in CR, 4 had minimal diseases; in Group B 6 were in CR and one in PR. One toxicity-related death occurred on day 7 in a child in first CR; median duration of granulocytopenia 0.5 x 10(9)/l and thrombocytopenia less than 50 x 10(9)/l were 20 days (R: 9-40) and 27 days (R: 11-51) respectively. Persistent immune thrombocytopenia occurred in 4 children. Fever higher tha 38 degrees C developed in all patients: sepsis was documented in 6 patients. Extramedullary toxicity was moderate: GI tract was the most affected. Two out of 5 children who received AT having residual disease achieved CR; relapse or progression of disease occurred in all these patients. Four out of 8 children in second or subsequent CR and 4 out of 5 in first CR are alive and well at 3-12 months (median 7).(ABSTRACT TRUNCATED AT 250 WORDS)

Antineoplastic Combined Chemotherapy Protocols↗

Indomethacin treatment of eighteen patients with Sweet's syndrome.

BACKGROUND: The standard treatment for Sweet's syndrome (acute febrile neutrophilic dermatosis) is oral corticosteroids. Despite a good initial response, the disease is characterized by frequent relapses. OBJECTIVE: Our purpose was to test the therapeutic effect of the nonsteroidal antiinflammatory drug indomethacin on Sweet's syndrome. METHODS: All patients with Sweet's syndrome observed during a 4-year period were given indomethacin, 150 mg/day for the first week and 100 mg/day for two additional weeks. The therapeutic response was assessed on days 4, 7, 14, 30, and 180. RESULTS: Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours and eruptions cleared between 7 and 14 days. The remaining patient's cutaneous lesions continued to develop and were successfully treated with prednisone (1 mg/kg/day). The only side effect of indomethacin treatment was epigastric pain in two patients. No patient had a relapse after discontinuation of indomethacin (mean followup, 20.1 months). CONCLUSION: Indomethacin is a safe and effective treatment for Sweet's syndrome.

Adult↗

Improved results of allogeneic bone marrow transplantation for advanced hematologic malignancy using busulfan, cyclophosphamide and etoposide as cytoreductive and immunosuppressive therapy.

Twenty-four patients between the ages of 8 and 48 years (median 27.5) with high-risk for relapse hematologic malignancy received a marrow transplant from an HLA and MLC compatible sibling donor after chemotherapy with busulfan, 4 mg/kg/day for 4 days by mouth, cyclophosphamide 60 mg/kg/day i.v. for 2 days, and etoposide 60 mg/kg i.v. over 4 h on the first day of cyclophosphamide treatment (BU/CY/VP). Toxicity consisted of mucositis, skin rash, and nausea and vomiting in all patients, transient fever thought to be due to etoposide administration in 16/24 (67%) patients, and clinical veno-occlusive disease (VOD) of the liver in 4/24 (17%). There were nine deaths from causes other than recurrent disease in the first 100 days after transplant and two deaths after day 100, a total transplant mortality of 11/24 (46%). Three patients relapsed, but 10/24 (40%) remain alive and disease free 26-182 weeks (median 60 weeks) from transplant. These results compare favorably with results in a group of 12 similar risk patients treated with total body irradiation (TBI) containing regimens during an overlapping time period. Six of the TBI patients have had persistent or recurrent disease and only two (17%) are currently alive and disease free. The probability of disease persistence or relapse is 67% in the TBI group and 20% in the BU/CY/VP group (p less than 0.02).

Adolescent↗

Four varied cases of intravascular lymphomatosis and a literature review.

BACKGROUND: Intravascular lymphoma (IVL) is an uncommon malignancy defined pathologically by neoplastic proliferation of lymphoid cells within the lumens of capillaries, small veins, and arteries, with little or no adjacent parenchymal involvement. The nature of IVL has been the subject of considerable controversy. Recent immunohistochemical studies have demonstrated clearly that the tumors bear the immunophenotype of neoplastic lymphoid cells of either B-cell or T-cell lineage. IVL commonly affects the central nervous system, resulting in progressive dementia and multiple neurologic deficits; skin is the second most common site of involvement, in the form of cutaneous plaques and nodules. METHODS: In a retrospective review of all cases of non-Hodgkin's lymphoma seen at our institution, four cases of IVL were identified. Case 1 was fixed in methacarin, and Cases 2-4 were fixed in 10% formalin. Standard fixation, tissue processing, sectioning, and hematoxylin and eosin staining were used. Immunophenotypic studies were performed using a modified avidin-biotin complex technique. The specimen in Case 1 was stained by Giemsa stain using standard techniques. RESULTS: Four cases of IVL are presented. One patient experienced hypoxemia and fevers of unknown origin; the second, dementia and a vasculitislike illness; the third rapidly progressive dementia, nonlocalizing neurologic deficits, and panhypopituitarism; the fourth Kaposi-like skin lesions. Case 1 relapsed shortly after completing chemotherapy. Salvage chemotherapy has yielded good initial response. Autopsy findings in cases 2, 3 and 4 confirmed IVL in many vessels, including the brain, lung, liver, heart, gastrointestinal tract, glomerulus, central nervous system, and skin. Malignant lymphoid cells marked as B-cells in all cases. CONCLUSIONS: Unusual and interesting clinical presentations may occur in patients with IVL. The medical literature indicates that few cases are diagnosed ante mortem. Although isolated patients may respond favorably to chemotherapy, most will deteriorate rapidly and the diagnosis of IVL not contemplated until necropsy.

Adult↗

Adjuvant treatment of locally advanced renal cancer with autologous virus-modified tumor vaccines.

We report on 208 patients with locally advanced renal-cell carcinoma who received a surgical adjuvant vaccination with autologous, Newcastle disease virus (NDV)-modified, and lethally irradiated tumor cells in combination with low-dose recombinant interleukin-2 and interferon-alpha. The pathological stage was defined as pT2-3a, N1-2, MO (n = 107); pT3b-4 NO, MO (n = 68); and pT3b-4, N1-2, MO (n = 23). The follow-up of 203 evaluable patients showed a median disease-free survival of 21+ months (range, 2-64+ months). In all, 18 relapses (9%) occurred in spite of initial vaccination therapy. Those patients presented with local relapse (n = 3), lymph node metastases (n = 10), and/or distant organ metastases (n = 9). All patients relapsing during the first 6 months after the onset of treatment had primary lymph node involvement of the disease. An analysis of the patient subgroup with a follow-up of more than 22 months showed 10 relapses among 56 patients (18%) along with a median follow-up of 39 months (range, 23-64 months). Toxicity was very mild, manifesting as flu-like symptoms and fevers of up to 38 degrees C. At 8 and 24 weeks after the start of vaccination, anti-NDV serum antibodies were detectable in 70% and 100% of the patients tested, respectively. In comparison with historical data based on the natural course of patients with locally advanced renal-cell cancer, our results demonstrate an improvement of the disease-free survival after surgical adjuvant treatment with autologous, NDV-modified tumor vaccines in combination with low-dose cytokines.

Adjuvants, Immunologic↗