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[An uncommon case of lupus].

A 44-year-old patient was referred with weight loss of some 6 kg in two months, weakness and diarrhoea. According to the criteria of the American College of Rheumatology (ACR), systemic lupus erythematosus (SLE) was diagnosed: photodermatosis, nephropathy, and pancytopenia with positive antinuclear antibodies and antibodies against native DNA. In addition, adrenal failure was diagnosed with hyponatraemia, relapsing fever, low baseline cortisol and impaired response to ACTH stimulation. Clinical features of SLE may obscure signs of adrenal insufficiency, and hence, diagnosis is jeopardized. SLE combined with Addition's disease is rare. In some patients with both disorders, antiphospholipid antibodies, as found in our patient, are considered responsible for the development of Addison's disease. Possible pathogenetic mechanisms such as adrenal haemorrhage or (micro)thrombosis are discussed. The patient's condition significantly improved under steroid therapy. The progression of renal insufficiency (histology: mesangioproliferative glomerulonephritis), however, required additional immunosuppression with cyclophosphamide.

Adult↗

[An epidemiological analysis of ixodid tick-borne borreliosis (ITBB) morbidity in Kirovo-Chepetsk District, Kirov Province in 1996-1998].

The epidemiological and some clinical features of tick-borne relapsing fever (TBRF) in the Kirovo-Chepetsk District, Kirov Province were studied and the data were statistically processed. The incidence rates of the disease in the region were compared with those of other Russian regions by sex, age, and occupation. The study revealed where and when infection had occurred, the duration of tick's attachment to the patient with TBRF, determined the duration of its incubative period, assessed whether a short-term prognosis of the expected number of patients can be made by the number of tick-bitten persons. The Kirovo-Chepetsk District is an active highly endemic focus of TBRF. Its incidence is tens of times higher that of other natural and focal infections.

Adolescent↗

[Diagnosis and treatment of hepatic artery thrombosis after liver transplantation].

The aim of this study was to examine the clinical presentation and time of hepatic artery thrombosis (HAT) after orthotopic liver transplantation (OLT), stressing the role of imaging modalities. Therapeutic options are described, such as retransplantation (Re-OLT), hepatic resections and revascularization procedures, focusing on complications and outcome in a consecutive series of 687 OLT. Over the period from 1986 to 1999, 687 OLT were carried out in 601 patients, 592 of whom were adults and 95 pediatric subjects. Of these operations 601 were primary OLT and 86 Re-OLT (71 I Re-OLT, 14 II Re-OLT and 1 III Re-OLT). In this retrospective study, we reviewed rejection episodes, time of HAT (early or late), possible cause of HAT, day of suspected diagnosis of HAT and day of confirmation of diagnosis. Clinical presentation, management, complications, outcome, survival rates and the need for Re-OLT were also recorded. The incidence of HAT was 2.47% (17/687). Early HAT (n = 9, < 30 days) was diagnosed 15.6 days after OLT (range: 3-25 days), whereas late HAT (n = 8, > 30 days) occurred 295.1 days after OLT (range: 38-1830 days). In two asymptomatic patients (2/17: 11.7%), HAT was discovered incidentally. Most of the patients (11/17: 64.7%) presented with increased liver function test values and fever. Relapsing bacteremia occurred in 7/17 cases (41.1%), whereas a biliary stricture and biliary leak were diagnosed in 3/17 (17.6%) and in 1/17 patients (5.8%), respectively. Fulminant hepatic failure was the clinical presentation in 2/17 cases (11.7%). In one case the clinical presentation was acute and chronic rejection (1/17: 5.8%). Intrahepatic abscesses were diagnosed in one case (1/17: 5.8%), as well as an intrahepatic haemorrhage (1/17: 5.8%). Doppler ultrasound (DUS) correctly revealed HAT in 9 of the 17 patients (52.9% sensitivity). In 8 of the 9 patients (88.8%) in whom HAT was diagnosed by DUS, angiography was also performed to confirm the diagnosis. Overall, angiography detected HAT in 14/17 patients (82.3% sensitivity). HAT management consisted of immediate Re-OLT in 6 patients 6.8 days (range: 3-12 days) after diagnosis. Delayed Re-OLT was performed in 6 patients 529.1 days (range: 68-1920 days) after diagnosis. The overall retransplantation rate was 70.5% (12/17). Two patients died despite undergoing intraarterial urokinase treatment. Three grafts were salvaged, but suffered biliary stricture due to ischemic cholangitis and underwent hepatico-jejunostomy. A II Re-OLT was carried out in 4 of 12 patients (33.3%). The overall mortality rate was 41.1% (7/17). One-year and 3-year overall survival rates were 58.8% (10/17) and 47.0% (8/17), respectively. Both 5- and 10-year overall survival rates were 11.7% (2/17). Although the results of OLT have improved dramatically over the past few years, HAT is still associated with substantial morbidity, a high incidence of graft failure and high mortality rates. The use of DUS to screen for HAT has permitted earlier diagnosis, but early angiographic evaluation of the hepatic arteries is still needed for accurate diagnosis of HAT and remains the gold standard. Retransplantation is the definitive solution for HAT in the majority of cases, though it is essentially the patient's clinical condition that dictates the form of management.

Adolescent↗

Field trial of the RTM dipstick method for the rapid diagnosis of malaria based on the detection of Plasmodium falciparum HRP-2 antigen in whole blood.

The performance of the Quorum RapidTest Malaria (RTM) dipstick method that detects Plasmodium falciparum histidine-rich protein-2 (PfHRP-2) antigen in whole blood was evaluated in a malaria endemic area. Results were compared with conventional Giemsa-stained blood films. Of 306 people tested 37.9% (116/306) were found to be parasitaemic; of these 66.4% (77/116) were P. vivax and 32.8% (38/116) were P. falciparum infections. There was only one (0.9%) mixed P. falciparum plus P. vivax infection. The RTM test was positive in 35/36 patients with P. falciparum identified on blood smear examination, resulting in a sensitivity of 97.2% [95% confidence interval (CI): 91.6-102.8%]. Specificity was 96.3% (95% CI: 93.9-98.6%). The RTM test had a positive predictive value of 77.8% (95% CI: 65.7-89.9%) and a negative predictive value of 99.6% (95% CI: 98.4-100.8%). Of the 10 false positives, seven reported recent malaria episode and treatment, indicating persistence of antigenaemia. If these were assumed truly infected, the positive predictive value is increased to 93.3% (95% CI: 85.8-100.8%). The RTM test was positive in all seven P. falciparum infections with gametocytes and one mixed infection, but was negative in all falciparum gametocytes and relapsing fever cases. All but one P. vivax infection gave negative result on the RTM test. The RTM test missed one patient with parasitaemia. The test is highly sensitive and specific requiring no instrument or trained personnel. It appears to be a very useful tool for rapid diagnosis of malaria, especially in the rural health institutions with limited diagnostic facilities.

Adolescent↗

[Antibiotic therapy in secondary peritonitis: towards a definition of its optimal duration].

BACKGROUND: The optimal time period of antibiotic treatment in secondary peritonitis is still unknown. AIM: To prospectively evaluate: 1) The preferred time period of antibiotic treatment in secondary peritonitis by means of a survey applied to a sample of surgeons, and 2) The outcome of a series of patients with secondary peritonitis in whom the length of antibiotic treatment was based on clinical and laboratory indicators. PATIENTS AND METHODS: A survey presenting the case of a patient with acute appendicitis and secondary peritonitis was applied to 100 Mexican surgeons. The optimal length of antibiotic treatment in that particular patient was asked. Results were tabulated and analyzed. Subsequently, 35 patients with secondary peritonitis were prospectively evaluated. Antibiotics were discontinued when fever relapsed and the white blood count normalized. Clinical characteristics, diagnosis, surgical treatment and outcome were analyzed. Recurrence of sepsis was investigated as the main outcome variable. RESULTS: The survey revealed that 96% surgeons recommended antibiotic treatment for a period longer than one week. In our study group median age was 33 years, 21 were male and 14 female. All patients had a surgical abdominal condition associated to secondary peritonitis. Median period of antibiotic treatment was 3 days. In a follow-up of one month there was no persistent or recurrent sepsis in any patient. CONCLUSIONS: This information may impact in determining the total length of antibiotic therapy if our results are reproduced in studies with more patients.

Adolescent↗

Erdheim-Chester disease. A case report.

A 63-year old man had a history of diabetes insipidus, arthralgias and myalgias, weight loss, relapsing fever and malaise. Increased uptake of Tc-99m was found predominantly in distal antebrachia, in distal femurs and in both trochanters and tibias on the bone scintigraphy. The chest radiograph showed reticulonodular pattern and the high resolution computed tomography (HRCT) scans revealed diffuse infiltrative lung disease with small multiple nodules and widening of interlobular septs. Videothoracoscopic lung biopsy and biopsy of tibial lesion were perfomed. The histopathologic examination proved non-Langerhans cell histiocytosis-Erdheim-Chester disease. Treatment with prednisone reduced the pain and fever and improved the vital capacity of the lungs while the changes in the lungs and bones remained unchanged.

Biopsy↗

[Edmond Sergent (1876-1969) and the Pasteur Institute of Algeria].

Edmond Sergent, supported by a distinguished team of colleagues, directed the Pasteur Institute of Algeria for over 60 years, from 1900 to 1963. As a student of Emile Roux, Sergent had received a Pasteurian training. His institute devoted extensive study to malaria. Sergent defined the concept of prevention and extended it to other pathologies. For many years, the Institute persevered in carrying out successful antimalarial campaigns such that Algeria was freed of the disease. In 1916-1917, Sergent and his brother were called upon to organise anti-malarial efforts for the Armée d'Orient. By way of systematic and energetic curative and prophylactic measures, they were able to eradicate the disease. In 1908, the Sergent brothers were the first to discover the role of the louse in the transmission of another disease, relapsing fever. The Pasteur Institute team also discovered the sand fly vector which transmits the parasite causing leishmaniasis. The Sergents found a new form of oculonasal myiasis, called "Thimni". In addition, they led effective campaigns against tuberculosis based on BCG vaccination administered throughout Algeria. The Pasteur Institute of Algeria conducted important research in plant and animal diseases. For example, they detected a trypanosome agent causing dromedary "debab", as well as its vector, the horsefly. They also studied in depth bovine piroplasmosis, which causes widespread and destructive disease, and demonstrated the role of the tick in promoting transmission generally. Their work in botany included the discovery that a Fusarium-type fungus was the causal agent for "baïoudh", the main disease of date palms. They also demonstrated the basic role of the fruit fly in alcoholic grape fermentation.

Academies and Institutes↗

[The laboratory breeding of lice].

The breeding of lice started in Poland as R. Weigl used to prepare a typhus vaccine from the guts of infected lice. Hélène Sparrow introduced this breeding technique in the Institut Pasteur of Tunis in 1927. Lice will feed only on human blood. They were put in small wooden boxes with a thin cloth bottom through which they could bite and feed. Once a day the boxes were placed against the skin of human feeders whose role was essential in the process. The breeding of lice at the Institut Pasteur of Tunis lasted until 1980 and allowed significant advances in the study of typhus and relapsing fever.

Animals↗

[Long-term results of mitral-aortic valve replacement in 1,154 patients with rheumatic valvular disease].

OBJECTIVE: To analyze the early and long-term results after mitral-aortic valve replacement for rheumatic valvular disease and the determinant factors involved and subsequent therapies. METHODS: 1 154 patients receiving combined mitral-aortic valve replacement for rheumatic valvular disease from May 1981 to May 2001 were reviewed. The mean age of the patients was 41.48 +/- 10.00 years. Concomitant valve plasty was performed for associated tricuspid organic or significant functional lesions. Lateral tilting disc or bileaflet valve prostheses were used for replacement. New York Heart Association functional status showed Class III or IV in 91.77% of the patients. Moderate to severe pulmonary hypertension occurred in 29.38% of the patients. The duration of follow-up varied from 8 months to 20 years. RESULTS: The hospital mortality was decreased from 6.50% to 4.45%. The 5-, 10- and l5-year survival rates were 89.46% +/- 1.35%, 86.50% +/- l.91% and 67.86% +/- 6.16%, respectively. The 5-, 10- and l5-year thromboembolic event free rates were 97.80% +/- 0.74%, 88.31% +/- 2.20% and 94.08% +/- 2.29%, respectively. the 5-, 10- and l5-year anticoagulant related bleeding free rates were 94.80% +/- 1.09%, 89.32% +/- 2.10% and 83.12% +/- 3.57% respectively. Cardiac functional status returned to Class II in 98% patients and to Class III in 2% during follow-up. CONCLUSIONS: Both left and right ventricular functions may be impaired as a result of rheumatic valvular disease. Tricuspid valve should be explored during surgery and any significant tricuspid annular enlargement and regurgitation showed be corrected in concomitance. Long-acting penicillin regimen is needed for 3 - 5 years for the prevention of rheumatic fever relapse. A low intensity anticoagulant regimen after valve replacement with prothrombin time targeting at 1.5 - 2.0 times is advisable in lessening anticoagulant related bleeding yet optimizing sufficient prevention against thromboembolic complications.

Adolescent↗

[Long-term results of prosthetic mitral valve replacement with home-made tilting disc valve: a report of 125 cases].

OBJECTIVE: To analyze the early and long-term results after mitral valve replacement for rheumatic valvular disease by using home-made tilting disc valve, and the determinant factors involved and subsequent therapies. METHODS: One hundred and five patients, including 31 patients with rheumatic mitral stenosis, 92 patients with mixed mitral stenosis and regurgitation, and 2 patients with bacterial endocarditis, underwent prosthetic mitral valve replacement with home-made tilting disc valve from September 1978 to June 1982. Three patients had a history of mitral commissurotomy, and 5 patients had concomitant functional tricuspid regurgitation. All patients were operated on under cardiopulmonary bypass with implantation of 25 - 29 mm size home-made tilting disc valve prosthesis. The associated functional tricuspid lesions were treated at the same time with modified DeVega's valvuloplasty or Kays bicuspidate valvuloplasty. RESULTS: Eleven patients died during the hospital stay with an early operative mortality of 8.8%. The major causes of the early death were low cardiac output syndrome (4 patients), respiratory failure (2), acute renal failure (2), extrinsic prosthesis dysfunction (1), ventricular arrhythmia (1), and left ventricular rupture (1). Ninety-eight survivors were followed up (total 1,162.2 years) for mean duration of 12.8 years. Eighty-nine patients (78%) survived over 10 years after operation, 58 (51%) over 15 years, and 55 (48%) over 20 years. There were 16 late deaths due to heart failure, anticoagulation related bleeding, thromboembolism and recurrence of rheumatic fever. The survival rates at 10 and 20 years were 82.3% and 51.1% respectively. Among the patients who survived over 20 years, 37 patients had the cardiac functional status returned to Class II, 13 Class III, and Class IV. CONCLUSIONS: Severe post-rheumatic valve deformity may occur in younger patients in China. Long-acting penicillin regimen given for 3 - 5 years for the prevention of rheumatic fever relapse is advocated. A low intensity anticoagulant regimen after mitral valve replacement is advisable in lowering the incidence of anticoagulant related bleeding, while optimizing sufficient protection against thromboembolic complication. Proper operative timing (e.g. when the patient is in sinus rhythm and in NYHA functional class II) is of great importance in achieving satisfied long-term results.

Adolescent↗

The Jarisch-Herxheimer reaction in ocular syphilis.

A patient with ocular syphilis is presented. She experienced deterioration in vision following the commencement of treatment due to a Jarisch-Herxheimer reaction. This is a transient febrile illness that can occur in patients after the first adequate dose of an anti-microbial drug to treat infectious diseases such as syphilis, Lyme disease and relapsing fever. However, a Jarisch-Herxheimer reaction occurring in a patient receiving treatment for ocular syphilis can be serious, resulting in the rapid loss of vision.

Adult↗

Nosocomial infection of Aeromonas hydrophila presenting as necrotizing fasciitis.

Aeromonas hydrophila is infrequently reported as a causative organism of necrotizing fasciitis. We report a case of necrotizing fasciitis due to A. hydrophila in a 44-year-old man with Marfan syndrome who underwent valve replacement surgery twice. He was admitted due to a 2-day history of fever. The fever was attributed to hepatitis, and ingestion of herbal medication was suspected to be the cause. The fever relapsed on the 28th day of hospitalization with rapidly progressive erythematous patches on the bilateral lower extremities. Septic shock developed within a few hours, and 2 small diagnostic incisions on the skin lesions suggested necrotizing fasciitis. Surgical exploration further revealed extensive necrosis of the subcutaneous tissue and fascia, but the muscle was spared. Blood cultures and cultures of the debrided tissue all yielded A. hydrophila. Pathological examination showed necrosis and degeneration of the soft tissue. Although appropriately managed with broad-spectrum antibiotics, fasciotomies and debridement, the patient's condition deteriorated rapidly and resulted in death 11 hours after the surgery. This case indicates that A. hydrophila can be a causative organism of nosocomial necrotizing fasciitis.

Adult↗

[Tick borne zoonosis: selected clinical and diagnostic aspects].

Tick-borne zoonotic infections are among the most diffuse vector borne diseases: these large group of infections is caused by different microorganisms: Babesia spp., Borrelia spp., Rickettsia spp., Ehrlichia spp., Francisella tularensis, Coxiella burnetii) and tick-borne encephalitis virus. Babesiosis is caused by the protozoa (sporozoa) Babesia microti and it is quite rare in humans in Europe. The ixodids ticks are the competent vectors. A few symptomatic cases have been reported, mainly in splenectomized patients. The laboratory diagnosis is made by the microscopic identification of the parasites within the red blood cells in blood smears. The serologic diagnosis, based mainly upon IFA and WB techniques has only an epidemiological interest. Lyme borreliosis (Lyme disease) has been recognized as the most frequent vector borne disease in mild climate areas. The etiologic agent is a spirochete, belonging to the Borrelia burgdorferi sensu lato complex: B. burgdorferi sensu stricto, B. garinii and B. afzelii. Several additional species of this geno-complex have been identified but their pathogenic capability for humans still needs to be elucidated. Lyme borreliosis is clinically divided into three different clinical stages: the early disease, the disseminated infection and the persistent infection. Individual stages are caused by the diffusion of the spirochetes to different anatomic districts of the body. The main clinical symptoms are, for each stage: the erythema chronicum migrans in the early infection, the peripheral nerves and joint involvement in disseminated diseases and the acrodermatitis chronica atrophica (ACA) with central nervous system involvement in the late disseminated infection. The microbiological diagnosis is achieved by serologic techniques (IFA, EIA, WB) and by isolation of the spirochetes (in vitro culture and DNA amplification methods). Tick-borne relapsing fever (TBRF) is occasionally transmitted to humans by the soft ticks Ornithodorus and is caused by Borrelia spp. Different borreliae are responsible for TBRF in various geographic areas. The laboratory diagnosis is based upon the identification of spirochetes in peripheral blood by microscopic observation of Giemsa stained smears. Rickettsiosis diseases are caused worldwide by the obligate intracellular bacteria belonging to the genus Rickettsia. In the Mediterranean area the most frequently identified rickettsia is R. conorii, that causes the so called Mediterranean spotted fever. The serologic detection of a specific antibody response by IFA techniques is the most prominent tool for the diagnosis. In addition, the PCR method can be applied. Bacteria of the genus Ehrlichia are well known pathogens in veterinary medicine. Since the last decade their zoonotic capability has emerged and E. chafeensis, E. canis and the so called human granulocytic agent (HGE) have been identified in human diseases following a tick bite. The ehrlichiosis is characterized, in human, by a mild fever associated with lymphoadenopathy. The diagnosis is made on the identification of morulae (the intracytoplasmatic inclusion of the growing rickettsiae) in the white cells of peripheral blood. In addition the molecular diagnosis is also possible by PCR. Tick-borne encephalitis (TBE) is the only viral arthropod-borne encephalitis in Europe: it is caused by a flavivirus and it can also be transmitted by the ingestion of goat raw milk. The more relevant epidemiological figure is limited to the Alps, in particular to the Northern side (Austria). Isolated cases have been reported also in Italy. TBE is a benign self-limiting illness that usually recovers without any reliquate. The laboratory diagnosis is obtained by isolating the virus in cell cultures from the CSF or blood of acute phase patients. Serology is anyway the main laboratory tool to perform this diagnosis. Complement fixation and EIA IgM are the most used methods: the latter technique is particularly sensitive in early infection.

Animals↗