Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Juvenile chronic arthritis is the most common connective tissue disease in children. It is of great social and clinical interest for its chronicity, for the often unpredictable response to pharmacological treatment; for the spontaneous evolution toward infirmity and often blindness. The English classification of the disease is here been adopted. There are 3 different types of onset: systemic, poliarticular and pauciarticular. Large joints such as the knees, wrists and ankles are involved more often than small joints. Also the cervical spine is frequently affected. Systemic disease is accompanied by high spiking fever, rash, lynphoadenopathy, pericarditis and hepatosplenomegaly. Chronic uveitis is a feature of JCA, more frequently observed in pauciarticular than in the other types of onset, and it is almost always associated with antinuclear antibody seropositivity. Rheumatoid factor (RF) and subcutaneous nodules are unusual in JCA. Diagnosis is often not easy and it is essentially clinical. The diagnostic criteria adopted have been proposed by ARA in 1977. In the majority of children treatment with ASA is successful. Sometimes other types of more toxic drugs such as gold salts or penicillamine are needed. Their use is best confined to reference centers. Orthopedical and physiotherapic treatments are complementary to the pharmacological one. Multidisciplinary centers are therefore necessary for the total management of these children also to stress the importance of furthering physical and psychological growth.
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PMID:[Juvenile chronic arthritis]. 409 7

Primary acquired pure red cell aplasia is a rare occurrence in childhood. An eleven-year old boy presented to us with pallor, which required multiple packed red cell transfusions. He did not have hepatosplenomegaly, jaundice or lymphadenopathy. Bone marrow examination revealed the diagnosis of pure red cell aplasia. All possible investigations were done to exclude secondary causes of pure red cell aplasia. No secondary cause was found on investigations. Rheumatoid factor and anti-nuclear antibodies were positive. He was started on oral steroids, to which he did not respond. He was then given cyclosporine A. Response to cyclosporine was dramatic and the child now does not require any transfusions.
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PMID:Acquired pure red cell aplasia in a child. 1208 27

Patient 1: A 65-year-old Caucasian man presented with a 2-month history of large erythematous patches of the right temporal scalp. The patient was otherwise in good health and taking no medications. He denied a family history oflymphomas or autoimmune diseases. No hepatosplenomegaly or lymphadenopathy was appreciated. A complete blood cell count, serum protein electrophoresis, peripheral blood flow cytometric analysis, bone marrow biopsy, Helicobacter pylori titers, and Borrelia burgdorferi titers were within normal range. The antinuclear antibody titer was positive at 1:640 and showed a homogenous pattern. Rheumatoid factor, SSA (Ro), and SSB (La) antibody titers were negative. Computed tomography scans of the chest, abdomen, and pelvis were unremarkable. Two punch biopsies from different time points demonstrated an atypical lymphocytic infiltrate forming clusters in the dermal and subcutaneous tissue. These cells had a (14;18) translocation and were mostly positive for CD20 and bcl-6, but not bcl-2. The patient was diagnosed with low-grade primary cutaneous follicle center lymphoma based on clinicopathological evidence, and achieved complete remission after local radiation and 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. The patient has had no evidence of recurrence for 6 years.
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PMID:Antinuclear antibody seropositivity in men with cutaneous B-cell lymphoma of the scalp. 2533 55