1/10. Acquired anti-FVIII inhibitors in children.Acquired inhibitors to FVIII (anti-FVIII) are uncommon in children. An acquired anti-FVIII developed in a previously healthy 4-year-old boy treated with penicillin for streptococcal pharyngitis. aspirin prophylaxis begun for suspected rheumatic fever led to compartment syndromes of all four extremities, which resolved with high-dose FVIII and surgical decompression. Anti-FVIII in this patient, and the five additional cases identified in a survey of 160 haemophilia treatment centres, occurred at a median age of 8 years, with median initial and peak titres of 4.6 and 6.9 Bethesda Units (BU), respectively. All six presented with bleeding, including haematomas (three intramuscular, one intracranial), and ecchymoses in three. The median baseline FVIII was 0.05 U mL(-1), and the median baseline activated partial thromboplastin time (APTT) was 79.8 s. The inhibitor resolved completely in five patients (83%) within a median 5 months, after treatment with FVIII concentrate, steroids, cytoxan, methotrexate, and no treatment. The inhibitor persisted in the patient with Goodpasture's disease, despite steroids, cytoxan, cyclosporin, and intravenous gamma globulin. aspirin therapy, in two, worsened ongoing bleeding. The association of penicillin-like drugs in this and three other cases in the literature suggest that to avoid potential catastrophic bleeding, it is prudent to obtain an APTT prior to initiating aspirin for suspected rheumatic fever. In conclusion, acquired anti-FVIII inhibitors in children may cause severe bleeding, and remit in the majority after FVIII and/or immunosuppressive therapy.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/10. Growing pains: fact or fiction?Growing pains are recurrent limb pains peculiar to children. Brief episodes of leg pains occurring intermittently at night are typical, but the clinical spectrum is variable. A diagnosis of growing pains can be made with certainty only after other possible conditions have been ruled out by appropriate studies and after the child has been observed carefully for a period of months. The two diseases most often confused with this syndrome are rheumatic fever and juvenile rheumatoid arthritis. aspirin and supportive measures are beneficial.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
3/10. A case of sjogren's syndrome with valvular diseases.The case of a 61 year old woman with sjogren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of sjogren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and sjogren's syndrome is rare and the etiological correlation is discussed.- - - - - - - - - - ranking = 7keywords = syndrome (Clic here for more details about this article) |
4/10. A syndrome of immunoglobulin a deficiency, diabetes mellitus, malabsorption, a common HLA haplotype. Immunologic and genetic studies of forty-three family members.Three persons in a kindred of 43 had variable expression of a syndrome consisting of immunoglobulin a deficiency, diabetes mellitus, malabsorption, and a common HLA haplotype. Findings from the proband included life-threatening malabsorption; idiopathic intestinal mucosal atrophy with infalmmation; iga deficiency and antibodies to multiple endocrine organs; insulin-dependent diabetes mellitus; and the major histocomptability antigens HLA-A2, B8, and DW3. In addition to the described syndrome other conditions present in the family include Graves' disease, vitiligo, hypocomplementemia, rheumatic fever, multiple sclerosis, and a high frequency of antibodies to endocrine tissue. Since Graves' disease, diabetes mellitus, and idiopathic Addison's disease have all been described in association with HLS-B8 and DW3, we believe that the occurrence of these diseases in this family suggests that a single immune response gene or gene complex is linked with HLA-B8 and DW3.- - - - - - - - - - ranking = 6keywords = syndrome (Clic here for more details about this article) |
5/10. Acute rheumatic fever with extracapillary glomerulonephritis and the nephrotic syndrome.A 34 year old woman is described in whom carditis, arthritis, fever, leukocytosis and a high sedimentation rate developed two weeks after a streptococcal infection. The patient also had the nephrotic syndrome and rapidly progressive renal insufficiency. The renal biopsy specimen showed acute extracapillary (crescentic) glomerulonephritis. The initial response to corticosteroid therapy was good, but later a slowly progressive renal function impairment was seen.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
6/10. mycoplasma pneumoniae infection presenting as acute rheumatic fever.We describe five children in whom symptoms and signs fully consistent with a diagnosis of acute rheumatic fever arose in association with infection by mycoplasma pneumoniae. With the decline of streptococcal disease as a causative agent of acute rheumatic fever, other etiologic possibilities capable of reproducing the clinical syndrome - especially mycoplasma pneumoniae - should be considered, as this may have therapeutic and prognostic implications.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
7/10. Coronary artery thromboembolism and unexpected death in childhood and adolescence.Two cases of unexpected death in childhood and adolescence associated with coronary artery thromboembolism are reported involving a 6-year-old girl with acute rheumatic fever and left ventricular vegetations, and an 18-year-old adolescent with down syndrome and congenital heart disease. Although coronary artery thromboembolism is rarely reported in childhood or adolescence, these cases demonstrate that careful examination of the coronary arteries during pediatric autopsy may be helpful in determining factors contributing to death, even at quite young ages. This is particularly so in the presence of predisposing cardiac pathology.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
8/10. Poststreptococcal reactive rheumatic syndrome.Poststreptococcal reactive arthritis remains a poorly recognized clinical entity that is rarely considered in adults. We describe a case of poststreptococcal rheumatic syndrome in a 63-year-old woman to highlight that prolonged bone pain, night sweats, arthralgia, and laboratory evidence of active inflammation may be the principal manifestations, and that it may occur even in elderly patients. Poststreptococcal rheumatic manifestations can be viewed as a continuum extending from acute rheumatic fever with or without carditis to poststreptococcal reactive rheumatic syndrome with either reactive arthritis or systemic symptoms predominating.- - - - - - - - - - ranking = 6keywords = syndrome (Clic here for more details about this article) |
9/10. The co-occurrence of acute rheumatic fever and AIDS.We describe a patient with advanced acquired immunodeficiency syndrome (AIDS) who presented with evidence of carditis, arthritis, and chorea in the setting of fever, and serologic evidence for recent streptococcal infection. Several features atypical for rheumatic fever were present and included persistently high titer of antistreptolysin O, the simultaneous occurrence of chorea and arthritis, and the presence of chorea in a sexually mature adult man. The differential diagnosis of arthritis in a host at risk for human immunodeficiency virus should be expanded to include acute rheumatic fever, which may manifest atypical features.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
10/10. The clinical spectrum of post-streptococcal syndromes with arthritis in children.Acute rheumatic fever (ARF) and post-streptococcal reactive arthritis (PSRA) are well known complications of streptococcal throat infections. We describe four children with arthritis following a streptococcal throat infection. In addition to arthritis, other clinical manifestations included erythema nodosum, livedo reticularis and cutaneous vasculitis. Because of the very diverse clinical manifestations that may appear after a streptococcal throat infection, we suggest a classification and treatment of post-streptococcal syndromes according to the severity of the disease.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
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