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Cat-scratch disease occurring in three siblings simultaneously.

We report for the first time three siblings with cat-scratch disease occurring simultaneously. Case 1 was an 8-year-old boy with enlarged supraclavicular lymph nodes and fever. Case 2 was the 11-year-old sister whose presentation was a prolonged febrile illness. Case 3 was a 13-year-old male adolescent with adenitis of the neck and thigh. All three patients were treated with rifampin with improvement of their symptoms.

Adolescent↗

[Intestinal effects of Buerger's disease (thromboangiitis obliterans) case report].

A case of an adult patient with intra-abdominal mass syndrome and acute surgical abdomen due to chronic intestinal ischemia with localized perforation and ileal necrosis associated to hepatic granulomatosis and mesenteric adenitis produced by Buerger's disease (thromboangiitis obliterans) is presented herein. The clinical presentation, diagnosis, handling and prognosis of this rare entity were examined.

Abdomen, Acute↗

Pasteurella pseudotuberculosis infection in man.

Pasteurella pseudotuberculosis has been considered a widespread animal pathogen for many years, but only within the last decade has its capacity to cause human disease been recognized. Two forms of human disease have been established-acute septicemia and mesenteric lymphadenitis. Because mesenteric adenitis is frequently indistinguishable from acute appendicitis, blood serum was obtained from 66 consecutive patients who underwent operation for appendicitis and was examined for agglutinins to seven serotype strains of P. pseudotuberculosis. Agglutinins were obtained in 21.2% of this series. Titres of over 1/100 were found in three of three cases of mesenteric lymphadenitis, one of 11 with no apparent disease, and one of 46 with appendicitis. P. pseudotuberculosis was isolated from a lymph node in the latter case. Two to four follow-up samples of sera in each of these five cases had increasing and then decreasing titres, indicative of active disease. Titres of 1/15 or less were found in five of the cases of appendicitis, in one case of salpingitis, and in three with no apparent disease. The occurrence of these nine cases with low titres may be indicative of previous contact with the organism.Human infection with P. pseudotuberculosis is not unusual in the Edmonton region and is responsible for at least some cases of mesenteric lymphadenitis.

Acute Disease↗

[Kikuchi's disease associated with cutaneous manifestations evoking Well's syndrome].

BACKGROUND: Wells' syndrome is a dermatological disease with polymorphous lesions characterized histologically by an eosinophilic infiltrate of the dermis with edema and flame figures. Kikuchi's disease is a benign cause of lymphadenitis corresponding histologically to a necrotizing histiocyting adenitis without neutrophils. We describe the association of a Kikuchi's disease and cutaneous lesions similar to Wells'syndrome. OBSERVATION: A 62-year-old man presented over 3 years several simultaneous episodes of left axillary lymphadenitis and of cutaneous lesions compatible with a diagnosis of Wells'syndrome. No precipitating event or disease described with the Wells'syndrome was established. Concerning the axillary nodes, a cancer, a lymphoproliferative syndrome, a systemic lupus and several infectious diseases were excluded. Standard histology and immunochemistry of a lymph node showed signs of Kikuchi's disease. Bilateral anterior uveitis was incidentally detected. The three conditions improved with oral corticosteroids (1 mg/kg/d). DISCUSSION: The patient had Kikuchi's disease and a recurrent dermatosis for which the clinical and histological aspects, the evolution and the absence of arguments for another cause, suggest a Wells'syndrome. Cutaneous manifestations occur in 16 to 40 p. 100 of cases of Kikuchi's disease and often have characteristic histologic features not corresponding to Wells'syndrome. The association of the 2 diseases may be then incidental, despite 4 simultaneous episodes. Two viral agents, VIH and VZV have been associated with the two diseases but do not seem to be the cause in our patient. Concerning the anterior uveitis, a single case of uveitis has been described for each condition.

Edema↗

[Otomastoiditis caused by Mycobacterium avium: a case report].

Non tuberculous Mycobacterial (NTM) Infections mainly affect immunocompromised patients, appearing as disseminated or pulmonary disease. In immunocompetent children the most common form of infection with NTM is cervical adenitis. Ear infection seems to be a rare disease. We present a case of otomastoiditis caused by Mycobacterium avium in a 15 months old child, immunologically normal. Patient was referred for persistent right otitis unresponsive to routine medical therapy. TC scan of the ear and temporal bones revealed: soft tissue in external auditory canal, Eustachian canal, and middle ear overlying ossicles with erosion of tegmen tympani. Tuberculin skin test was positive with 5 units PPD and culture yielded M. avium. The patient undergo timpanomastoidectomy and medical therapy with antituberculous drugs and Steroids, subsequently he was given Clarithromycin and Rifabutin. M. avium is an ubiquitous low grade pathogen found in soil, water, dust and food. There is no evidence of direct transmission. Only a few cases of otomastoiditis due to M. avium have previously been reported. The case presented underlines the importance of microbiological investigations. When a NTM infection is suspected surgeons and infectious diseases specialists should cooperate to find an optimal treatment regimen of this unusual disease.

English Abstract↗

[Piriform sinus fistula, a new disease].

Pyriform sinus fistula has recently been described as a new, rare and little known pathology. The process develops as latero-cervical suppurative adenitis or acute suppurative thyroiditis, usually on the left side. Radical surgery is mandatory to avoid recurrence. This congenital malformation must be present in the differential diagnosis of recurrent infectious cervical processes, especially in acute thyroiditis. The only curative treatment is complete exeresis of the fistula after meticulous dissection of the fistulous tract. We present three cases of this pathology. The diagnostic approach and surgical treatment are discussed. The recent literature on this topic is reviewed.

Branchial Region↗

Adverse effects of BCG vaccination; mainly BCG infection.

(1) Local and regional adverse effects (ulceration, abscesses and adenitis are generally mild, although they can persist for more than three months. Systemic effects are rare but potentially life-threatening (osteitis, disseminated BCG infection, anaphylaxis, etc.). (2) Some risk factors are related to the vaccine itself or to its administration (intradermal route, certain strains of vaccine, high doses, etc.). There are very few data on the adverse effects of multipuncture BCG administration. (3) The frequency of adverse effects is inversely related to age at vaccination. The vaccine can cause severe disseminated BCG infection, especially in subjects with congenital or acquired immunodeficiency (notably HIV infection and immunosuppressive therapy). (4) The risk of adverse effects can be minimised by avoiding patients with contraindications and by careful administration.

BCG Vaccine↗

A rare entity among childhood malignant lymphomas (large anaplastic T-cell Ki-1 +).

A case of peripheral type T-cell lymphoma is presented to underline the difficulty in distinguishing the initial clinical findings of an inflammatory neoplastic disorder since the diagnosis could only be arrived at after several repeated lymph node biopsies. An 11 10/12-year-old boy admitted to the hospital with inguinal lymph node enlargement was diagnosed as having adenitis and periadenitis. The disease had progressed and the patient had remittent fever rising to 39 degrees C, and another biopsy was taken. Cervical lymphadenomegaly was present, A diagnosis of chronic lymphadenitis with lymphocyte loss and fibrosis was established. The diagnosis could only be made from biopsy material taken from the deep cervical (jugularis interna) and axillary lymph nodes one year later, which showed Ki-1 antigen positive large T-cell lymphoma. The disease showed continuous activity in spite of chemotherapy and the child survived only 17 months.

Antineoplastic Combined Chemotherapy Protocols↗

Terrorism and blast phenomena: lessons learned from the attack on the USS Cole (DDG67).

Blast phenomena and injuries to the musculoskeletal system have been well documented for the past 50 years. The USS Cole was attacked in Aden Harbor in Yemen on October 12, 2000. Seventeen sailors were killed and 39 were wounded. The bombing of the USS Cole and an analysis of the pattern of injury are unique compared with previous terrorist bombing attacks in which the predominant injury pattern is from Type II and Type III blast phenomena. Because the ship superstructure did not collapse, there were no confounding variables in examining the pattern of injury as there would have been with shrapnel-generating devices or detonations with subsequent building collapse. The morbidity and mortality sustained by the victims was almost exclusively from Type I and Type III blast effects. The musculoskeletal system was a clear marker for mortality and morbidity. Fractures of the cranium, spine, pelvis, and long bones denoted increasing severity of injury to critical organ systems. Shipboard firefighting was successful in containing fires and there was very little morbidity from inhalational injuries or burns. Blast phenomena that affect ships or buildings that have been specifically built to absorb a blast attack likely will manifest a different mode and pattern of injury than those seen in traditional terrorist blast events.

Blast Injuries↗

Cross-sectional study of heart failure therapy with angiotensin converting enzyme inhibitors and digoxin.

OBJECTIVE: The aim of the present study is to show a better short-term (2 weeks) clinical improvement in patients with heart failure (HF) who are receiving angiotensin converting enzyme inhibitors (ACEIs) (with or without digoxin) when compared to the standard therapy excluding ACEIs. METHODS: The study was conducted in Al-Gamhuria Teaching Hospital, Aden, Yemen, from January to July 2003. In this study, 78 patients with HF were enrolled into 3 therapeutic groups (ACEIs alone, ACEI and digoxin and digoxin alone) and their responses within 2 weeks were recorded. Exclusion criteria were as follows: thyroid disorders, gastrointestinal disturbances (diarrhea, malabsorption), electrolyte unbalanced (unless corrected) and insufficient data. Serum creatinine was measured at the beginning and after 10 days. In addition, the patients' body weight and age were recorded. Criteria for a complete improvement within 2 weeks were the occurrence of the following: 1) The relief of pulmonary congestion, 2) Decrement in heart rate to less than 74 +/- 5, 3) Disappearance of the lower limb edema, and 5) Recorded positive electroencephalogram change. Partial amelioration was recognized if only 2 or 3 of the preceding criteria were observed. RESULTS: Nine patients received digoxin alone, while 40 patients were treated with ACEIs and digoxin. Treatment with ACEIs without digoxin was observed in 29 patients. The discrepancy between the number of patients was necessitated by the need of patients with HF. This last category of treatment regimen produced better clinical improvement (complete with 10.1%, partial with 24.3%) compared to the digoxin group without ACEI (complete 2.5% or partial 5.1%). Nevertheless, the addition of digoxin to an ACEI increased this ratio (17.8% for complete and 28.2% for partial improvement). A 49.3% increase in serum creatinine was observed after 10 days in 25 HF patients, who were randomly selected and followed up (the baseline concentration was 99.75 +/- 9.9 umol/L, while the level after 10 days was 148.97 +/- 19.8 umol/L, p=0.005). CONCLUSION: We confirmed that short-term use of ACEI regimens has a superior effect on the therapy of HF (34.4% complete and partial response) as compared to the therapy of not using ACEI (7.6% had a complete and partial response). The combination of ACEI and digoxin has resulted in the best outcome (46% had a complete and partial response). However, we also noticed a significant rise in serum creatinine by 49% concomitant with the use of ACEI (the baseline concentration was 99.75 +/- 9.9 um/L, while the level after 10 days was 148.97 +/- 19.8 umol/L, p=0.005).

Administration, Oral↗

Kawasaki syndrome.

Kawasaki syndrome is an acute mucocutaneous syndrome of young children characterized by prolonged fever, rash, mucositis, cervical adenitis, and changes of the peripheral extremities. Unlike most other illnesses, the rash is not distinctive and is remarkable for its variable morphology, site of onset, and pattern of progression. The cause and pathogenesis of Kawasaki syndrome remain a mystery, but most investigators favor a microbial agent as the trigger, followed by an immune-mediated vasculitis that has a predilection for the coronary arteries. Serious complications include coronary artery aneurysms, aneurysmal thrombosis, and death. Aspirin has been the most widely used treatment for Kawasaki syndrome, but it has not been shown to alter the basic pathology. Treatment with aspirin plus intravenous gamma globulin can reduce the frequency of coronary artery abnormalities. Definitive therapy of Kawasaki syndrome awaits the discovery of its cause and pathogenesis.

Child↗

Impact of the human immunodeficiency virus on the epidemiology of tuberculosis in area 15 of the Valencian community in Spain.

SETTING: Area 15 in Valencia. OBJECTIVES: To describe the epidemiology (1987-2001) of tuberculosis (TB) in human immunodeficiency virus (HIV) patients. METHODS: Study of annual incidence, age distribution, excess cases attributed to HIV, etiological risk fraction (ERF), population attributable fraction (PAF) and f factor. RESULTS: Of 476 cases diagnosed, 459 were TB, 16 environmental and one mixed; 76% of environmental cases were HIV-positive (P < 0.001). There was a mean annual TB incidence of 24.6/100000, with an annual reduction of 4%. Seventy-three patients were HIV coinfected (16%) (mean incidence 3834/100 000 seropositives). The principal risk factor was drug use (59%) for HIV+ and contact with TB for HIV-. We found no difference in pulmonary or extra-pulmonary location between groups, contrary to mixed cases (P < 0.001). In HIV+ there was a lower frequency of infiltrates (P < 0.001) and cavitation (P < 0.01), and a higher frequency of adenitis (P < 0.001), miliary or nodular pattern and normal X-ray (P < 0.001). Seropositives had a 174 times higher probability of developing TB. The mean ERF attributed to HIV was 99%, the PAF was 16% and the f factor was 1.19. Highly active antiretroviral therapy (HAART) reduced the risk of TB in HIV+ by 80%. CONCLUSIONS: TB has continued its decline, although HIV generated an excess of cases in the 1990s. HAART has reduced the TB risk in HIV+ and possibly the overall rate of TB.

Adolescent↗

[Bronchorrhea in a case of pneumonic type of bronchioloalveolar carcinoma].

The authors describe a case of 80-years old male hospitalized because of radiological and clinical signs suggestive of right-sided pneumonia. The main complaints of the patient were of productive cough with increasing amounts of watery sputum irregular fever up to 39 degrees C, progressive dyspnea, generalized weakness and loss of weight. Despite extensive use of antimicrobial and antituberculosis agents significant deterioration of patients general condition and the progression of X-ray picture were observed, inflammatory infiltration started to encompass the contralateral lung. Bronchial washing revealed the presence of atypical and neoplasmatic cells of adenous origin type. Since this finding contrasted with the pattern of radiological abnormality that did not show any tumor-like changes, another diagnostic approach was undertaken. Transthoracic fine needle aspiration biopsy revealed cells of non-small cell lung carcinoma. The diagnosis of bronchioalveolar carcinoma established on the basis of clinicoradiologic pattern was confirmed at autopsy. Increasing bronchorrhea was the most prominent symptom.

Adenocarcinoma, Bronchiolo-Alveolar↗

[Adverse events following BCG vaccination].

Bacillus Calmette-Guérin (BCG) is one of the safest vaccines applied worldwide today. Risk of occurrence of adverse effects upon BCG immunization varies with age of children being vaccinated, whereas local reactivity differs among vaccines due to applied strain as well as the number of viable bacilli and type of vaccine. In 1984, Lotte and co-workers analyzed and classified complications associated with BCG vaccination in detail. This classification is based on clinical, bacteriological, histological and biological information. Category 1 involves extensive local lesions and regional suppuration, BCG-related lymphadenitis, which is usually but not always bacteriologically and/or hitologically confirmed. Categories 2 and 3 comprise more serious complications. Non-fatal cases (localized or multiple changes) are included in category 2, while fatal cases (generalized lesions usually associated with immunodeficiency) are in category 3. Category 4 includes complications which occur upon BCG administration, but not definitely confirmed either bacteriologically or histologically. Keloid formation belongs to this category. Bacillus Calmette-Guerin lymphadenitis is the most common complication of BCG vaccination. There are two forms of BCG adenitis in natural course of lymphadenitis. Simple or non-suppurative lymphadenitis, which usually resolves spontaneously within a few weeks, and suppurative lymphadenitis characterized by fluctuation development with edema and erythema above glands. More serious adverse effects rarely occur at 1-3/1 mil of administered doses. In our country, not all adverse effects related to BCG vaccination have been established However, each complication should be reported, because its monitoring enables quality control both of the vaccine and techniques of its application.

BCG Vaccine↗

Hepatobiliary phaeohyphomycosis with secondary biliary cirrhosis.

We report a case of systemic phaeohyphomycosis in a 13-year old girl. The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy. The patient was diagnosed initially as tuberculous adenitis and was on antituberculous therapy, but with worsening of symptoms. Lymph node and liver biopsy showed granuloma with fungal hyphae suggestive of phaeohyphomycosis. The patient responded to antifungal therapy. Later she developed secondary biliary cirrhosis with cholangitis due to long stricture of the common bile duct and common hepatic duct requiring biliary stenting. To the best of our knowledge, this is the first report on phaeohyphomycosis causing biliary cirrhosis in the English lilterature.

Child↗

Abnormal distribution of aquaporin-5 in salivary glands in the NOD mouse model for Sjögren's syndrome.

OBJECTIVE: To localize aquaporin-5 in healthy salivary gland acinar cells and to check if it is abnormally translocated in an experimental NOD mouse model for Sjögren's syndrome (SS). METHODS: Healthy BALB/c control mice and autoimmune focal adenitis NOD mice were studied. Aquaporin-5 was stained using avidin-biotin-peroxidase complex and indirect immunofluorescence staining methods, and visualized using light and laser scanning confocal microscopy. RESULTS: Aquaporin-5 was found in the apical domain of the acinar cell plasma membrane in healthy BALB/c mice. In contrast, aquaporin-5 was found in the apical and basolateral acinar plasma cell membrane in parotid, submandibular, and sublingual glands in NOD mice. This was confirmed using laser scanning confocal microscopy for optical sectioning and image reconstruction. CONCLUSION: Our findings reveal an abnormal translocation of aquaporin-5 in an experimental SS animal model and support observations that implied a similar loss of the ordered and polarized expression of aquaporin-5 in human SS labial and lacrimal glands.

Animals↗

Unilateral segmental Darier disease following Blaschko lines: a case report and review of the literature.

Darier disease (DD) is an autosomal-dominant skin disorder that is characterized by multiple keratotic papules, loss of epithelial adhesion, and abnormal keratinization. We describe an unusual case of late-onset unilateral segmental DD that follows the lines of Blaschko. Our patient did not exhibit other classic findings of DD. Our case and review of the literature suggest that lesions previously classified as acantholytic dyskeratotic epidermal nevi (ADEN) are actually unilateral segmental presentations of DD.

Darier Disease↗

Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response.

PFAPA syndrome is characterized by periodic episodes of high fever, aphthous stomatitis, pharyngitis, and/or cervical adenitis. It is of unknown etiology and manifests usually before 5 years of age. We determined serum and intracellular cytokine levels in six PFAPA patients (4 males, 2 females, mean age 8 years (+/- 1.2 SEM), range 4-13) during the symptom-free period as well as 6-12 hours and 18-24 hours after fever onset. Values were compared to age-matched, healthy controls. Febrile PFAPA attacks led to a significant increase in IL-6 and IFN-gamma serum concentrations compared to symptom-free periods and to controls, with IL-1beta, TNF-alpha and IL-12p70 levels being significantly higher than in controls. Lymphocytic IFN-gamma and CD8+ IL-2 production was consistently significantly elevated compared to healthy children. During the asymptomatic period, serum concentrations of IL-1beta, IL-6, TNF-alpha and IL-12p70 were significantly increased compared to controls. Intracellular TNF-alpha synthesis was not elevated at any time point. Soluble TNFRp55 levels were even lower in between febrile episodes, reaching values comparable to controls during attacks, whereas soluble TNFRp75 levels increased during attacks compared to healthy children. Anti-inflammatory IL-4 in serum was at all times lower in PFAPA patients compared to controls with no difference in levels of intracellular IL-4 and IL-10 or serum IL-10. The observed increase of pro-inflammatory mediators, even between febrile attacks, suggests a dysregulation of the immune response in PFAPA syndrome, with continuous pro-inflammatory cytokine activation and a reduced anti-inflammatory response.

Adolescent↗