1/9. Prospective identification and treatment of children with pediatric autoimmune neuropsychiatric disorder associated with group A streptococcal infection (PANDAS).BACKGROUND: The current diagnostic criteria for pediatric autoimmune neuropsychiatric disorder associated with group A streptococcal infection (PANDAS) are pediatric onset, neuropsychiatric disorder (obsessive-compulsive disorder [OCD]) and/or tic disorder; abrupt onset and/or episodic course of symptoms; association with group A beta-hemolytic streptococcal (GABHS) infection; and association with neurological abnormalities (motoric hyperactivity or adventitious movements, including choreiform movements or tics). OBJECTIVE: To assess new-onset PANDAS cases in relation to acute GABHS tonsillopharyngitis. DESIGN: Prospective PANDAS case identification and follow-up. RESULTS: Over a 3-year period (1998-2000), we identified 12 school-aged children with new-onset PANDAS. Each patient had the abrupt appearance of severe OCD behaviors, accompanied by mild symptoms and signs of acute GABHS tonsillopharyngitis. Throat swabs tested positive for GABHS by rapid antigen detection and/or were culture positive. The GABHS serologic tests, when performed (n = 3), showed very high antideoxyribonuclease antibody titers. Mean age at presentation was 7 years (age range, 5-11 years). In children treated with antibiotics effective in eradicating GABHS infection at the sentinel episode, OCD symptoms promptly disappeared. Follow-up throat cultures negative for GABHS were obtained prospectively after the first PANDAS episode. recurrence of OCD symptoms was seen in 6 patients; each recurrence was associated with evidence of acute GABHS infection and responded to antibiotic therapy, supporting the premise that these patients were not GABHS carriers. The OCD behaviors exhibited included hand washing and preoccupation with germs, but daytime urinary urgency and frequency without dysuria, fever, or incontinence were the most notable symptoms in our series (58% of patients). Symptoms disappeared at night, and urinalysis and urine cultures were negative. CONCLUSION: To our knowledge, this is the first prospective study to confirm that PANDAS is associated with acute GABHS tonsillopharyngitis and responds to appropriate antibiotic therapy at the sentinel episode.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/9. Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis.Paroxysmal dystonic choreoathetosis (PDC) is an episodic, non-kinesogenic, extrapyramidal movement disorder. It is postulated that PDC is an ion channel disorder. We describe a sporadic case of paroxysmal dystonic choreoathetosis occurring after streptococcal pharyngitis. The episodes were characterized by abrupt-onset dystonic posturing, choreoathetosis, visual hallucinations and behavioral disturbance. Each episode lasted between 10 minutes and 4 hours, and occurred up to 4 times per day. In between attacks, examination was normal. The episodes waxed and waned in frequency during a 6-month illness. magnetic resonance imaging of the brain was normal. Post-streptococcal neuropsychiatric disease has a proposed autoimmune etiology, which is supported by the presence of serum antibasal ganglia antibodies. Western immunoblotting of this case's serum demonstrated antibody binding to a basal ganglia antigens of molecular weight 80 kDa and 95 kDa. immunohistochemistry examination demonstrated specific antibody binding to large striatal neurones. We propose that autoantibodies produced in post-streptococcal neuropsychiatric disease cause alteration in neurotransmission, possibly secondary to ion channel binding.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/9. Acute human immunodeficiency virus syndrome in an adolescent.Acute human immunodeficiency virus (hiv) seroconversion illness is a difficult diagnosis to make because of its nonspecific and protean manifestations. We present such a case in an adolescent. A 15-year-old boy presented with a 5-day history of fever, sore throat, vomiting, and diarrhea. The patient also reported a nonproductive cough, coryza, and fatigue. The patient's only risk factor for hiv infection was a history of unprotected intercourse with 5 girls. physical examination was significant for fever, exudative tonsillopharyngitis, shotty cervical lymphadenopathy, and palpable purpura on both feet. Laboratory studies demonstrated lymphopenia and mild thrombocytopenia. Hemoglobin, serum creatinine, and urinalysis were normal. The following day, the patient remained febrile. physical examination revealed oral ulcerations, conjunctivitis, and erythematous papules on the thorax; the purpura was unchanged. Serologies for hepatitis b, syphilis, hiv, and Epstein-Barr virus were negative. Bacterial cultures of blood and stool and viral cultures of throat and conjunctiva showed no pathogens. Coagulation profile and liver enzymes were normal. Within 1 week, all symptoms had resolved. The platelet count normalized. Repeat hiv serology was positive, as was hiv dna polymerase chain reaction. Subsequent hiv viral load was 350 000, and the cd4 lymphocyte count was 351/mm3. hiv is the seventh leading cause of death among people aged 15 to 24 in the united states, and up to half of all new infections occur in adolescents. Our patient presented with many of the typical signs and symptoms of acute hiv infection: fever, fatigue, rash, pharyngitis, lymphadenopathy, oral ulcers, emesis, and diarrhea. Other symptoms commonly reported include headache, myalgias, arthralgias, aseptic meningitis, peripheral neuropathy, thrush, weight loss, night sweats, and genital ulcers. Common seroconversion laboratory findings include leukopenia, thrombocytopenia, and elevated transaminases. The suspicion of acute hiv illness should prompt virologic and serologic analysis. Initial serology is usually negative. Diagnosis therefore depends on direct detection of the virus, by assay of viral load (hiv rna), dna polymerase chain reaction, or p24 antigen. Both false-positive and false-negative results for these tests have been reported, further complicating early diagnosis. Pediatricians should play an active role in identifying hiv-infected patients. Our case, the first report of acute hiv illness in an adolescent, emphasizes that clinicians should consider acute hiv seroconversion in the appropriate setting. Recognition of acute hiv syndrome is especially important for improving prognosis and limiting transmission. It is imperative that we maintain a high index of suspicion as primary care physicians for adolescents who present with a viral syndrome and appropriate risk factors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/9. adult acute disseminated encephalomyelitis associated with poststreptococcal infection.Acute disseminated encephalomyelitis (ADEM) is a monophasic illness that is thought to develop from antigenic mimicry with antibodies having cross-reactivity to host epitopes in the nervous system. The disorder typically follows an exanthematous or recent viral infection. In contrast, complications from bacterial poststreptococcal infections more commonly give rise to disorders in the pediatric population including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders, and ADEM. We present the novel case of documented streptococcal pharyngitis and elevated antideoxyribonuclease B (ADNB) titers in an adult giving rise to ADEM. Furthermore, the absence of basal ganglia abnormalities on MRI and the degree of leukocytosis in the CSF distinguish the adult form of ADEM from childhood ADEM and adult viral demyelinating diseases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/9. Apparent increase in the incidence of invasive group A beta-hemolytic streptococcal disease in children.Recently, among adults, an increase in the incidence of invasive disease caused by group A beta-hemolytic streptococci (GABS) has been noted, as has the appearance of a severe illness called "toxic shock-like syndrome," also caused by GABS. We now report an apparent increase beginning in 1987 in the incidence of invasive disease caused by GABS in children. Among these patients the manifestations were varied. One child had signs and symptoms compatible with the streptococcal toxic shock-like syndrome. Among the GABS isolates from our patients, 8 (80%) of 10 evaluated for M-protein antigens were nontypeable. Further studies will be necessary to determine the relationship between serotypes and virulence of GABS. physicians should be aware of the possibility of an increasing incidence of invasive GABS disease in children, as well as its manifestations, which may include toxic shock-like syndrome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/9. Choanal bleeding due to nasopharyngitis.Twenty-two patients with bleeding from the nasopharynx which due to secondary changes in acute nasopharyngitis were treated at the Fujisaki Hospital. The titers of IgG, IgM and IgA of the viral capsid antigen (VCA), IgG and IgA of the early antigen (EA), and EB nuclear antigen (EBNA) were measured. There was no correlation between season of coryza or changes in season and the onset of nasopharyngitis, except for a slightly greater occurrence during mid summer and mid winter. One of three patients with severe bleeding, underwent posterior nasal packing using a rolled 10 X 10 cm gauze sponge. The majority of cases of bleeding from the nasopharynx were most satisfactorily treated by administration of the ranitidine hydrochloride (Zantac) of a selective antagonist for the H2-receptor of histamine.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
7/9. Severe autoimmune protein s deficiency in a boy with idiopathic purpura fulminans.Idiopathic purpura fulminans usually occurs in young children and is frequently preceded by a preparatory viral or bacterial infection. Following a severe streptococcal pharyngitis, an 8-year-old boy developed purpura fulminans with disseminated intravascular coagulation and severe protein s deficiency (total antigen < 0.05 u/ml). Despite generous plasma infusions, skin necrosis progressed rapidly into compartment syndrome which required fasciotomy and skin grafting and resulted in the loss of three digits of the right foot. Total protein S remained low for over a month despite plasma supplementation and complete normalization of protein c levels. A polyclonal anti-protein S IgG was demonstrated in the patient's plasma, which decreased to 25% of baseline titre after 1 month and was undetectable 6 months after purpura fulminans, when plasma protein S had returned to normal. Transient, isolated and severe deficiencies of protein S have been reported in patients with idiopathic purpura fulminans and a previous preparatory infection. Autoimmune protein s deficiency may play a key role in the aetiopathogenesis of idiopathic purpura fulminans.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/9. Two family outbreaks of chlamydia pneumoniae infection.During autumn 1992, we observed two unrelated family outbreaks of chlamydia pneumoniae infection. family A consisted of grandmother (aged 77 yrs), father (aged 41 yrs), mother (aged 38 yrs), daughter (aged 10 yrs), and two sons (aged 6 yrs and 3 months, respectively). The grandmother and daughter suffered from pneumonia, father from pharyngitis and bronchitis and the older son from mild bronchitis. No symptoms were recorded in the mother and younger son. Symptomatic subjects showed a fourfold increase in immunoglobulin g (IgG) titre for chlamydia pneumoniae, determined by a microimmunofluorescence test with specific antigen (TW-183). Other serological studies against mycoplasma pneumonia, legionella pneumophila, influenza virus type A and B, adenovirus and respiratory syncytial virus (RSV) were negative. sputum culture gave a positive result for haemophilus influenzae, colony forming units (cfu) = 10(4).ml-1 in the grandmother. No serum positivity was recorded in the mother and younger son, who remained asymptomatic. All symptomatic patients were successfully treated with macrolides. family B consisted of mother (aged 63 yrs) and daughter (aged 36 yrs). Both suffered from chlamydia pneumoniae pneumonia. Diagnosis was made by means of serological microimmunofluorescence test, and direct identification using an indirect immunofluorescence test on pharyngeal swab. sputum culture and other serological tests remained negative. Both patients were successfully treated with macrolides. These observations emphasize the relevance of chlamydia pneumoniae in family cluster respiratory infections.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/9. Streptococcal toxic shock syndrome associated with marked gamma delta T cell expansion: case report.Streptococcal toxic shock syndrome (strepTSS) has been associated with various streptococcal soft-tissue infections including cellulitis, necrotizing fasciitis, and peritonitis in adults. We describe a 40-year-old patient with pharyngitis and strepTSS. Throat swab cultures yielded a strain of streptococcus pyogenes that produced large amounts of erythrogenic toxin A. fluorescence-activated cell sorter analysis of the patient's peripheral blood lymphocytes revealed generally enhanced expression of the T cell activity markers CD25 and human leukocyte antigen-DR and a marked increase in the number of gamma delta T cells, largely of the V delta 1-bearing subpopulation. Two more analyses, which were performed 2 weeks and 9 months later, respectively, documented the course of normalization after the acute episode of strepTSS. The T cells of this patient were stimulated in vitro with supernatants of his streptococcal isolate, and they proliferated in a dose-dependent manner. These proliferating T cells were mainly alpha beta T cells.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |