Proposal for a new therapeutic high dosage of Pidotimod in children with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome: a randomized controlled study.

BACKGROUND: Despite to PFAPA syndrome is considered a benign and self-limited condition in childhood its impact on patients and families can be remarkable in many cases. Currently, the therapeutic options for managing are non-specific and no consensus exists about the best treatment to use. Pidotimod has been suggested as a new potential treatment in PFAPA syndrome for its immunodulatory effects. We conducted a preliminary, prospective, controlled, open, cross-over trial to assess the efficacy and the safety of Pidotimod in the treatment of children with PFAPA syndrome. METHODS: 22 children with PFAPA syndrome were randomly allocated to treatment with pidotimod (with 2 vials of 400 mg daily) in combination with betamethasone 0.5-1 mg on need, based on parents/caregivers' decision (group A) or betamethasone 0.5-1 mg on need, based on parents/caregivers' decision (group B). Each treatment period was for 3 months (Phase 1), after that patients were switched to the other arm for other 3 months (Phase 2). Efficacy was expressed in terms of number of episodes of fever, pharyngitis, or aphthous stomatitis, as well as the additional use of betamethasone on need. Safety and tolerability of the Pidotimod were evaluated on the basis of the number and type of adverse events (AEs) recorded during the treatment. RESULTS: Patients receiving Pidotimod and use betametasone showed a significant decrease in frequency of fevers (p = 0.002); number of episodes of pharyngitis (p = 0.049); aphthous stomatitis (p = 0.036) as well as the betamethasone use on need (p = 0.007). Overall, 19/22 (86.4%) showed benefits from Pidotimod administration. The safety profile of Pidotimod was excellent as no serious adverse events have been reported in the treated groups. CONCLUSIONS: We firstly showed that high dosage of Pidotimod could be an effective and safe to reduce the PFAPA attacks in children.

New sequence type ST3756 of Staphylococcus aureus subspecies anaerobius as the causative agent of abscessing lymphadenitis in sheep.

Morel's disease is a form of abscessing lymphadenitis of sheep and goats caused by Staphylococcus aureus subspecies anaerobius. In Europe and Africa, the disease is linked to S. aureus of multilocus sequence type 1464. In an outbreak recorded in 2015 in a flock of 530 animals in the district of Nymburk, Czech Republic, Europe, the causative agent was cultured and subsequently confirmed by Maldi-TOF. Neither antibiotic therapy nor surgical interventions met any success, although the strain isolated was found to be sensitive to antibiotics used. Vaccination and revaccination with inactivated autogenous vaccine administered subcutaneously was relatively successful. Subsequent multilocus sequence typing revealed the presence of new S. aureus sequence type 3756, different from 1464 in three out of seven genes typed. The isolate thus represents a new sequence type of Staphylococcus aureus ssp. anaerobius which should be considered as a causative agent of Morel's disease.

Linfadenitis por Mycobacterium bovis, una entidad a tener en cuenta / Mycobacterium bovis lymphadenitis, an entity to consider

Las medidas de control del ganado y la pasteurización de los productos lácteos han hecho que la enfermedad tuberculosa por Mycobacterium bovis sea infrecuente en nuestro medio. El diagnóstico de esta entidad requiere un alto índice de sospecha, fundado en una historia clínica detallada y pruebas complementarias oportunas. La presentación más frecuente es extrapulmonar, principalmente como linfadenitis, por lo que ante una adenopatía de mala evolución se recomienda remitir al paciente para estudio y valorar la realización de punción aspiración con aguja fina para examen citológico y cultivo. Presentamos un caso de linfadenitis por M. bovis en una niña de siete años que respondió adecuadamente al tratamiento médico (AU9

Cattle control measures and milk products pasteurization have made Mycobacterium bovis disease uncommon in our environment. The diagnosis of this disease requires a high index of suspicion based on a detailed medical history and appropriate laboratory tests. Extrapulmonary cases are the main presentation, especially lymphadenitis, for this reason, is recommended to refer to study all the adenopathies with poor evolution, for evaluating de need of a fine-needle aspiration for citologý examination and culture. We report the case of M. bovis lymphadenitis in a 7-year-old girl with adequate response to medical treatment (AU)

Sebaceous adenitis and mural folliculitis in a cat responsive to topical fatty acid supplementation.

OBJECTIVE: To describe a case of feline sebaceous adenitis and mural folliculitis, and its successful treatment with topical fatty acids. ANIMAL: A 5-year-old, male castrated Norwegian Forest cat was presented with a progressive seborrhoeic dermatitis. METHODS: Clinical examination and histopathological examination of skin biopsies. RESULTS: There was severe, multifocal, lymphocytic mural folliculitis and perifollicular dermatitis, moderate hyperkeratosis and sebaceous adenitis on histopathology. Sebaceous glands were either absent or almost completely effaced by a dense lymphocytic infiltrate. Clinical signs began in spring on the face and neck and progressed over an 18 month period to involve the legs. Initially, topical and systemic antibacterial therapy for a mild bacterial overgrowth resulted in partial clinical response. There was no improvement with oral omega 6 fatty acids and surface cleaning. Treatment with a spot-on product containing essential oils, smoothing agents and vitamin E as the sole therapy was associated with a good--but incomplete--clinical response over a 6 month period, with hair regrowth and a marked decrease in seborrhoeic dermatitis. This improvement was sustained until 12 months later when a severe deep pyoderma with associated anorexia and depression occurred. This was symptomatically treated and the cat remained clinically stable for a further 18 months. Periocular and perinasal seborrhoea was a persistent feature. CONCLUSION AND CLINICAL IMPORTANCE: Topical essential fatty acid therapy may offer a viable alternative to ciclosporin, which has been reported for the successful treatment of this rare disease in cats.

[Effectiveness of pidotimod in combination with bacterial lysates in the treatment of the pfapa (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome]. / Efficacia del Pidotimod in associazione a lisato batterico nel trattamento della syndrome PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis).

AIM: PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is the most common autoinflammatory syndrome in pediatrics, accepted as an hyperimmune condition. Pidotimod is a molecule with immunomodulatory activity on both innate and adaptive immune responses; it also has the capacity to modulate the function of the respiratory epithelial cells through the activation of a NK-KB pathway which would involve the host-virus interaction. Moreover, the proven beneficial effect of Pidotimod in enhancing the immune response during vaccination, and its benefits in the prevention of respiratory tract infections, should be noted. METHODS: A joint combination of Pidotimod and bacterial lysates was used to treat 37 children with a clinical diagnosis of PFAPA; within the end of the first year of therapy, the healing rate of PFAPA symptoms was 67.5% (25 children), with a 10.8% (4 cases) still in complete remission within the end of the second year of follow-up. RESULTS: It is important to highlight that 29 children (78.3%) had benefitted from this therapy, in terms of healing, with a marked decrease in the incidence of fever from a total of 360 to 106 episodes, and episodes of periodic fever occurring almost 4 times less frequently. The use of Pidotimod determined a significant reduction of surgical tonsillectomy's treatment. CONCLUSION: This approach had a strong impact on the children's quality of life; a significant decrement in the use of antipyretic drugs, as well as a lower rate of antibiotic prescription, were also noted. It also had a dramatic impact on families' lives, because the treatment lowers the number of absences of family members from work or school/kindergarten.

Microbiology and antimicrobial treatment of pediatric cervical lymphadenitis requiring surgical intervention.

BACKGROUND: Acute cervical lymphadenitis is a common condition often times requiring antibiotic therapy and possible surgical drainage. The objective of this study was to describe the clinical characteristics, diagnostic and therapeutic management of children requiring surgical drainage for acute cervical lymphadenitis. METHODS: A retrospective, descriptive study was performed at a Midwestern US tertiary-care children's hospital on all immunocompetent children who underwent an incision and drainage procedure of cervical lymphadenitis from January 1999 to July 2009. RESULTS: A total of 277 patients were identified. Males represented 51% and the median age was 28 months (IQR: 13-59). Lymphadenitis was unilateral in 243 (87.7%) cases and bilateral in 19 (6.9%). Median length of hospital stay was 4 days (IQR: 3-5). Aerobic, anaerobic, acid fast bacillus (AFB), and fungal cultures were obtained intraoperatively in 99%, 98%, 82%, and 78% of cases, respectively. However no fungal cultures were positive and only 1% of anaerobic and 2% of AFB cultures were positive. The most common bacterial etiology was Staphylococcus aureus (35.7%) and Streptococcus pyogenes (18.8%). Of all cultures, 32% were negative. Overall, 22% were positive for methicillin susceptible S. aureus (MSSA) and 13.7% for methicillin resistant S. aureus (MRSA), with 96% MSSA and 100% MRSA susceptible to clindamycin. Median duration of discharge antibiotics prescribed was 10 days (IQR: 7-11). Only 12 (4.5%) patients required a repeat incision and drainage within 3 months. CONCLUSIONS: A single antibiotic that treats S. pyogenes and S. aureus should be the empiric antibiotic for cervical lymphadenitis requiring incision and drain. We recommend sending only aerobic cultures intraoperatively as a routine practice as other pathogens are rare.

Oral vitamin A as an adjunct treatment for canine sebaceous adenitis.

Medical records of dogs with sebaceous adenitis diagnosed by histopathology over an 18-year period were reviewed. From a total of 40 cases, 24 were treated with oral vitamin A. Dogs ranged from 9 months to 12 years of age at the time of disease onset. Purebred as well as mixed-breed dogs were affected. Akitas represented approximately one-third of the affected population. No sex predilections were observed. Vitamin A was administered for a minimum of 1 month. Doses varied from 380 to 2667 IU/kg/day, with a mean of 1037 IU/kg/day. Two dogs received oral vitamin A exclusively. Concurrent treatments included systemic antibiotics, systemic antifungal medications, fatty acid supplementation and various topical treatments. Of 24 dogs treated with vitamin A, three were lost to follow-up. Twelve owners were satisfied with the overall appearance of their dogs, reporting ≥25% improvement in clinical signs, including level of pruritus, amount of scale, alopecia and overall coat quality, compared with pretreatment appearance. Three owners observed adequate initial improvement, with regression to pretreatment state within 6 months of starting treatment. Two owners reported 25-50% improvement in clinical signs while on oral vitamin A supplementation; however, changes were attributed to concurrent topical treatment. Six owners reported no improvement and discontinued oral administration of vitamin A within 7 months. No correlations could be made between vitamin A dosage and response to treatment; prognoses could not be made based on clinical and histopathological findings.

Long-term clinical profile of children with the low-penetrance R92Q mutation of the TNFRSF1A gene.

OBJECTIVE: To analyze the long-term impact of the R92Q mutation of TNFRSF1A in children with periodic fever, in comparison with children with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) with TNFRSF1A structural mutations and children with periodic fever of unknown origin fulfilling the criteria for periodic fever, aphthosis, pharyngitis, and adenitis syndrome (PFAPA). METHODS: The extracellular region of TNFRSF1A was analyzed in 720 consecutive children with periodic fever, using denaturing high-performance liquid chromatography and DNA sequencing. Followup data on 11 pediatric patients with TNFRSF1A structural mutations (cysteine or T50M), 23 pediatric patients with an R92Q substitution, and 64 pediatric patients with PFAPA were collected during routine clinic visits. The 50-item Child Health Questionnaire was used to assess health-related quality of life (HRQOL). RESULTS: The frequency of typical TRAPS-related clinical manifestations was significantly lower and the impact of the disease on HRQOL was significantly reduced in patients with the R92Q mutation compared with TRAPS patients carrying structural mutations of TNFRSF1A. Followup data on 11 TRAPS patients with TNFRSF1A structural mutations (mean followup 7.9 years), 16 patients with theR92Q substitution (mean followup 7.3 years), and 64 patients with PFAPA (mean followup 5.2 years) were available. Patients with R92Q mutations and patients with PFAPA displayed a higher rate of self-resolution or amelioration of the fever episodes than did TRAPS patients with structural mutations. CONCLUSION: Although some cases may progress to a more chronic disease course, the majority of children with an R92Q mutation of the TNFRSFA1 gene show a milder disease course than that in children with TNFRSFA1 structural mutations and have a high rate of spontaneous resolution and amelioration of the recurrent fever episodes.

Sebaceous adenitis in Havanese dogs: a retrospective study of the clinical presentation and incidence.

Sebaceous adenitis is a suspected immune-mediated disease that targets and destroys sebaceous glands. This retrospective study evaluated the clinical presentation and incidence of sebaceous adenitis in Havanese dogs. Sebaceous adenitis was diagnosed in 35% (12 of 34) of Havanese dogs presented over a 5-year period. Onset of clinical signs occurred during young adulthood. Follicular casts were present in 92% (11 of 12) of affected dogs. Other common clinical signs included alopecia and hypotrichosis. The trunk, head and ears were commonly affected, with 67% (8 of 12) of cases having pinnal and/or external ear canal involvement. Secondary pyoderma was seen in 42% (5 of 12) of dogs. Histopathology revealed absent sebaceous glands in 83% (10 of 12) and a lymphoplasmacytic periadnexal infiltrate in 92% (11 of 12) of samples. Treatment included multiple modalities. Cyclosporin was prescribed in 83% (10 of 12) of cases. Other systemic therapies included vitamin A and fatty acid supplementation. Topical therapies included antiseborrhoeic shampoos and sprays, and oil soaks. Follow-up ranging from 2 months to 3 years was obtained in 67% (8 of 12) of dogs. Improvement ranged from minimal to marked, with better clinical response associated with longer duration of treatment. Owners with follow-up of more than 1 year commonly reported occasional flares of the clinical signs. This study found that sebaceous adenitis was a common diagnosis in Havanese dogs, that the ears were commonly affected and that a lymphoplasmacytic periadnexal infiltrate associated with absent sebaceous glands was frequently seen on dermatohistopathological examination.

Management of suppurative cervical lymphadenitis in a healthy 24-year-old man.

Unilateral suppurative cervical lymphadenitis is characterized by acute onset of 1 or more tender cervical lymph nodes and can lead to fever, cellulitis, abscess formation, and bacteremia. This form of lymphadenitis is usually caused by gram-positive bacteria. The present case details the treatment of a previously healthy 24-year-old man who presented with an acutely inflamed cervical lymph node. The patient did not respond to antibiotic monotherapy or combination antibiotics but recovered rapidly after methylprednisolone and osteopathic manipulative treatment were added to his care.

WITHDRAWN: Antibiotics / anti-inflammatories for reducing acute inflammatory episodes in lymphoedema of the limbs.

BACKGROUND: Lymphoedema is a chronic, progressive condition and one area of debate is the optimum management for infective/inflammatory episodes (AIE's). OBJECTIVES: To determine whether antibiotic/anti-inflammatory drugs given prophylactically reduce the number and severity of AIE's in patients with lymphoedema. SEARCH STRATEGY: We searched the Cochrane Breast Cancer Group register in September 2003, the Cochrane Central Register of Controlled Trials (The Cochrane Library, Issue 4, 2003), CINAHL, MEDLINE, PASCAL, SIGLE, UnCover, reference lists produced by The British Lymphology Society, the National Research Register (NRR) and the International Society of Lymphology congress proceedings. SELECTION CRITERIA: Randomised controlled trials testing an antibiotic or anti-inflammatory drug against placebo (with or without physical therapies) were included. DATA COLLECTION AND ANALYSIS: Eligibility for inclusion was confirmed by two blinded reviewers. The papers were screened independently using a checklist of criteria relating to quality. Both reviewers extracted data from the eligible studies using a data extraction form. MAIN RESULTS: Four studies (364 randomised patients) were included. Two studied the effects of intensive physical treatment plus selenium or placebo in preventing AIE's, and two studied the effects of Ivermectin, Diethylcarbamazine (DEC) (anti-filarial agents) and penicillin as prophylactic treatment for adeno lymphangitis(ADL) versus placebo.Both selenium trials reported no inflammatory episodes during the trial period in the treated group but one case of infection in the two placebo groups respectively. Seven additional cases of infection in trial one and 14 cases in trial two required treatment in the three month follow up period.One anti-filarial trial reported 127 ADL episodes for all groups during the treatment year (compared with 684 episodes during the pre-treatment year). There were 228 ADL episodes during the trial follow-up year but no findings were significant. No link was found between the grade of oedema and the frequency of ADL episodes. There was a significant link between increased episodes and the rainy season. Penicillin reduced the mean number of inflammatory episodes from 4.6 to 0.5 after treatment, which increased to 1.9 at the end of follow-up. AUTHORS' CONCLUSIONS: The effectiveness of selenium in preventing AIE in lymphoedema remains inconclusive in the absence of properly conducted randomised-controlled trials. Anti-filarials do not appear to reduce ADL episodes in filarial lymphoedema. Penicillin appears to contribute to a significant reduction in ADL when combined with foot-care. The importance of foot-care should be recommended, which may also apply to care of the arm following breast cancer treatment. Properly conducted trials are needed to demonstrate any efficacy of these interventions.

[Diagnostics and comprehensive treatment of patients with odontogenic lymphadenitis complicated by phlegmons].

The duthor detected special changes in oral liquid of macro and trace substances concentrations and their ratio in dynamics of convalescence for 24 patients with odontogenic lymphadenitis complicated by phlegmon. It was established that termination of inflammatory process on earlier terms at addition in complex treatment of this pathology of polyvitaminic complex considerably lowering strontium concentration in oral liquid (Patent RU 2210378 from 20.08.2003).

Antibiotics / anti-inflammatories for reducing acute inflammatory episodes in lymphoedema of the limbs.

BACKGROUND: Lymphoedema is a chronic and progressive condition and current debate revolves around the best course of management for infective/inflammatory episodes. OBJECTIVES: To determine whether antibiotic/anti-inflammatory drugs given prophylactically reduce the number and severity of infective/inflammatory episodes in patients with lymphoedema. SEARCH STRATEGY: We searched the Cochrane Breast Cancer Group register in September 2003, the Cochrane Central Register of Controlled Trials (The Cochrane Library, Issue 4, 2003), CINAHL, MEDLINE, PASCAL, SIGLE, UnCover, reference lists produced by The British Lymphology Society, the National Research Register (NRR) and the International Society of Lymphology congress proceedings. SELECTION CRITERIA: Types of studies considered for review were randomised controlled trials testing an antibiotic or anti-inflammatory drug against placebo (with or without physical therapies). DATA COLLECTION AND ANALYSIS: Eligibility for inclusion was confirmed by two blinded reviewers who screened the papers independently using a checklist of criteria relating to the randomisation and blinding of a trial. Both reviewers extracted data from the eligible studies using a data extraction form. MAIN RESULTS: Overall, four studies (364 randomised patients) were included. Two of these studied the effects of intensive physical treatment plus selenium or placebo in preventing AIE, and two studied the effects of Ivermectin, Diethylcarbamazine (DEC) (anti-filarial agents) and penicillin as prophylactic treatment for adeno lymphangitis(ADL) versus placebo. Both selenium trials reported no inflammatory episodes during the trial period in the treated group but one case of infection in the two placebo groups respectively during the first three weeks of each trial. Seven additional cases of infection in trial one and 14 cases in trial two required treatment in the three month follow up period. One anti filarial trial reported a total of 127 ADL episodes for all groups during the treatment year (compared with 684 episodes reported for the same participants during the pre-treatment year). Another 228 ADL episodes were reported during the trial follow-up year but no significant differences were found between the three groups. No apparent link was found between the grade of oedema and the frequency of ADL episodes. However, there was a significant link between increased episodes and the rainy season. In the penicillin group the mean number of inflammatory episodes was reduced from 4.6 to 0.5 after treatment and increased to 1.9 at the end of the follow-up year. REVIEWERS' CONCLUSIONS: The effectiveness of selenium in preventing AIE in lymphoedema remains inconclusive in the absence of properly conducted randomised controlled trials. Anti-filarials (DEC and Ivermectin) do not appear to reduce ADL episodes in filarial lymphoedema. Foot care may be important in reducing ADL episodes, and penicillin appears to contribute to a significant reduction in ADL, when combined with foot-care. It seems reasonable to emphasise the importance of foot-care to patients and practitioners in preventing infection and this may also apply to care of the arm in women who develop lymphoedema following breast cancer treatment. However, properly conducted trials are needed to demonstrate any efficacy of these interventions.

Infection with Corynebacterium pseudotuberculosis in five alpacas.

Among the population of an alpaca breeding farm, 5 alpacas (22 days to 14 months old) developed focal swellings in the subcutaneous tissues of the head or neck. Infection with Corynebacterium pseudotuberculosis was confirmed on the basis of results of microbial culture of abscess material and a serum hemolysis inhibition assay to detect C. pseudotuberculosis toxin. The dams of the affected alpacas were seronegative for C. pseudotuberculosis toxin. The affected alpacas underwent surgical excision of the abscesses and were isolated from herdmates for 90 days; treatment was successful, and no other alpacas in the herd became infected. Common risk factors for sources of infection in the affected alpacas included housing in a maternity barn and a pasture. Also, the infection potentially originated from new alpacas introduced into the herd during the preceding 3 months. Infection with C. pseudotuberculosis should be considered as a differential diagnosis for camelids with peripheral lymphadenopathy or abscesses in subcutaneous tissues.

Cervical adenitis due to Mycobacterium fortuitum in patients with acquired immunodeficiency syndrome.

Rapidly growing mycobacteria, an infrequent cause of human disease, are increasingly being recognized as human pathogens rather than mere colonizers. Rapidly growing mycobacteria infrequently cause disease in patients with acquired immunodeficiency syndrome (AIDS). It is unclear whether patients with AIDS are more predisposed than others to infection by these organisms. The optimal regimen and duration of treatment is similarly uncertain. Mycobacterium fortuitum is a rare cause of lymphadenitis or neck abscess. We report two cases of M fortuitum neck abscesses in patients with AIDS which were successfully treated with antibiotics after initial drainage. In one of these patients, M fortuitum neck abscess was the AIDS-defining illness. We also present a review of the cases reported in literature. It appears that such infections may be treated with a combination of ciprofloxacin and clarithromycin after incision and drainage of the abscess.

Diagnosis and treatment of disease caused by nontuberculous mycobacteria. This official statement of the American Thoracic Society was approved by the Board of Directors, March 1997. Medical Section of the American Lung Association.

UNLABELLED: Diagnostic criteria of nontuberculous mycobacterial lung disease in HIV-seropositive and -seronegative hosts. The following criteria apply to symptomatic patients with infiltrate, nodular or cavitary disease, or a high resolution computed tomography scan that shows multifocal bronchiectasis and/or multiple small nodules. A. If three sputum/bronchial wash results are available from the previous 12 mo: 1. three positive cultures with negative AFB smear results or 2. two positive cultures and one positive AFB smear B. If only one bronchial wash is available: 1. positive culture with a 2+, 3+, or 4+ AFB smear or 2+, 3+, or 4+ growth on solid media C. If sputum/bronchial wash evaluations are nondiagnostic or another disease cannot be excluded: 1. transbronchial or lung biopsy yielding a NTM or 2. biopsy showing mycobacterial histopathologic features (granulomatous inflammation and/or AFB) and one or more sputums or bronchial washings are positive for an NTM even in low numbers. COMMENTS: these criteria fit best with M. avium complex, M. abscessus, and M. kansasii. Too little is known of other NTM to be certain how applicable these criteria will be. At least three respiratory samples should be evaluated from each patient. Other reasonable causes for the disease should be excluded. Expert consultation should be sought when diagnostic difficulties are encountered.