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Biomedical subjects

A Gedalia

Publications and source records attributed to A Gedalia.

68 records · Page 4Linked to original sources

Pneumococcal osteomyelitis. An unusual cluster of cases.

Between 1979 and 1981, 22 children with osteomyelitis and/or septic arthritis were hospitalized at Soroka University Hospital. Streptococcus pneumoniae was cultured in five of the children, four of whom were under 2 years of age. In four other children under 2 years of age, Haemophilus influenzae was cultured. Staphylococcus aureus was identified less frequently in the younger age group. In deciding on initial antibiotic therapy, the possibility of such a bacteriological spectrum should be considered.

Arthritis, Infectious↗

Severe rheumatic mitral valve disease in children. Evaluation by echocardiography.

Severe rheumatic mitral stenosis with or without mitral insufficiency is virtually unseen in children in the affluent countries of the western world, but is not uncommon in developing countries. Fifteen cases of rapidly progressive rheumatic mitral valve disease in children are presented to illustrate the value of echocardiography in the diagnosis and evaluation of this lesion. Cardiac catheterization was performed in nine and simply confirmed the diagnosis made noninvasively. Our experience demonstrates that thorough echocardiographic examination in these cases frequently enables the examiner to assess the need for surgical intervention without cardiac catheterization.

Adolescent↗

Systemic lupus erythematosus and sickle cell disease.

A case of 10-year-old girl with an unusual association of sickle cell disease (SCD) with systemic lupus erythematosus (SLE) is presented. The report discusses the clues to the diagnosis of this rare combination with review of relevant literature and highlights the diagnostic dilemma that may arise because of the similarity of symptoms.

Anemia, Sickle Cell↗

Neurologic manifestations in familial Mediterranean fever.

Neurologic involvement in children with familial Mediterranean fever is relatively uncommon and rarely described in the pediatric literature. Although headaches occur frequently, meningitis and convulsions are rare. Thirteen of 101 children with familial Mediterranean fever developed neurologic manifestations. Of these 13 patients, 10 had headaches during acute episodes of the fever. Two patients had convulsions with fever before the age of 5 years; the convulsions and acute episodes recurred at ages 9 and 10 years. Another patient had two episodes of aseptic meningitis followed by convulsive disorder before the diagnosis of familial Mediterranean fever was made; his convulsions were resistant to antiepileptic drugs alone and subsided only when colchicine was added. The possibility of neurologic involvement should be considered in patients with familial Mediterranean fever.

Anticonvulsants↗

Sickle cell anemia with systemic lupus erythematosus: response to hydroxyurea therapy.

PURPOSE: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily. RESULTS: A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9% and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life. CONCLUSIONS: HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.

Adolescent↗

Low-dose methotrexate in the treatment of severe juvenile rheumatoid arthritis and sarcoid iritis.

OBJECTIVE: To assess the efficacy of low-dose oral methotrexate (MTX) therapy for children with severe iritis. METHODS: MTX in a weekly dose of 7.25 to 12.5 mg/m2 was administered orally to four patients (two with juvenile rheumatoid arthritis [JRA] and two with sarcoidosis) with severe iritis not adequately controlled by topical and systemic corticosteroid therapy. The treatment was initiated with half of the total dose and increased every 2 weeks until the final dose was reached. Iritis was graded from 0 to +4 according to the density of cells in the anterior chamber of the eye. RESULTS: There were three girls and one boy with a mean age of 10.5 years. Two patients were African American and two were Caucasian. The mean age at onset of iritis was 6 years. The mean duration of MTX therapy was 28.8 months. Significant improvement was noted in two of the four patients in ocular inflammation, demonstrated by reduction of cell density from +4 to +1. Two patients had a mild improvement of the iritis. However, corticosteroids were significantly reduced in all patients. One patient was completely off steroids within 30 months of MTX therapy. In the remaining three cases, the steroid dose was successfully tapered from 0.82 mg/kg/d to 0.15 mg/kg/d (mean doses) within a mean duration of 20 months. No side effects were observed with MTX therapy. CONCLUSION: Low-dose MTX therapy was effective and safe, and displayed steroid-sparing properties in four children with severe iritis.

Administration, Oral↗

Fibromyalgia syndrome: experience in a pediatric rheumatology clinic.

OBJECTIVE: To report our experience of fibromyalgia syndrome (FMS) in pediatric rheumatology clinic settings. METHODS: Clinical and laboratory data were reviewed in all patients with FMS between March 1992 and March 1996. Patients with FMS and an underlying rheumatic disease were excluded from the study. At presentation and follow-up visits, all patients had a tender points (TP) count that was conducted by thumb palpation. Both the children and their parents were questioned concerning the presence of widespread pain or aching. All the patients fulfilled the ACR criteria for the diagnosis of primary FMS. All children were evaluated by a protocol that included relevant information on FMS. Telephone survey questionnaires were used for patients who missed some of their follow-up visits. RESULTS: There were 59 children (47 F and 12 M) diagnosed with primary FMS. The mean age at onset was 13.7 years, and the mean age at diagnosis was 15.5 years. The mean duration of follow-up was 18.3 months. Diffuse aching was reported in 57 patients (97%), headaches in 45 (76%), and sleep disturbances in 41 (69%). Less common were stiffness in 17 (29%), subjective joint swelling in 14 (24%), fatigue in 12 (20%), abdominal pain in 10 (17%), and joint hypermobility and depression in 8 (14%) and 4 (7%) patients, respectively. The mean ESR was 15 mm/h, RF was negative in all patients, and ANA was positive (mean titer 1:160) in 17 patients. The mean initial TP count was 14.6. Nine patients were not available for follow-up. There were 50 patients available for follow-up and survey analysis, and of these 30 (60%) had improved, while 18 (36%) remained unchanged, and 2 (4%) became worse when compared with initial presentation. At the end of study follow-up, 37 patients (74%) were still taking medication (20 of them daily). Out of 25 patients whose TP counts were available at the end of follow-up, the mean TP dropped from 14.12 to 12.04 (p = 0.09) for the total group, and 14.05 to 10.84 (p < 0.01) for the patients who had improved. 22 out of 30 patients in the improved group and 7 out of 20 in the unchanged or worse group had continued active exercise programs (p < 0.001). CONCLUSION: The clinical spectrum of FMS in children is similar to that of adults but with better outcomes. The TP count correlates with clinical status only in patients who had improved. Active exercise programs seem to correlate with better outcomes. Prospective and larger patient population studies, and a longer follow-up of children with FMS are needed to clarify these findings.

Adolescent↗

Role of MRI in diagnosis of childhood sarcoidosis with fever of unknown origin.

Childhood sarcoidosis is a disease with multisystem organ involvement, and initial presentation as fever of unknown origin (FUO) is relatively common. We describe herein three children (aged 9, 7, and 11 years) with sarcoidosis who were seen initially with FUO. Common clinical and laboratory features include fever of > 2 weeks' duration, weight loss, fatigue, leg pain, anemia, increased erythrocyte sedimentation rate, elevated immunoglobulin G level, negative antinuclear antibodies and rheumatoid factor, and negative purified protein derivative and Candida skin tests. Two patients had iridocyclitis, one had hilar adenopathy, and two had angiotensin-converting enzyme serum levels. All three had no evidence of pulmonary infiltrates on chest radiographs. Bone marrow biopsies for all three were normal, and with no evidence of malignancy. Plain radiographs of the lower extremities and bone scan were normal. Magnetic resonance imaging (MRI) of the lower extremities revealed intact bone architecture and multifocal nodular lesions within the marrow. Bone biopsy from the tibia performed on two patients showed normal bone trabeculae and a solitary noncaseating epithelioid cell granuloma. Noncaseating granuloma was found on reevaluation of the bone marrow biopsy in the third patient. All three patients had negative marrow stains and cultures for fungi and mycobacteria. We conclude that MRI was very useful in the diagnosis of sarcoidosis in children with FUO.

Child↗

Low-dose methotrexate as a steroid-sparing agent in a child with Takayasu's arteritis.

Takayasu's arteritis (TA) is a chronic inflammatory arteriopathy affecting the large vessels and has been described predominantly in young adult women. In children it presents as an aggressive disease usually requiring chronic corticosteroid therapy. At present, low dose oral methotrexate (MTX) appears to be an effective steroid-sparing agent in adult patients with active TA. We report a 4-year-old child with Takayasu's arteritis who was initially placed on oral prednisone (2 mg/kg/day) therapy. Three months later, low-dose oral MTX (10 mg/m2/week) was added. Prednisone was successfully tapered over the following year to 0.2 mg/kg every other day. A repeat angiography following 12 months of therapy revealed a dramatic improvement of the vascular lesions. No toxicity was observed with MTX therapy. In conclusion, low-dose oral MTX appeared to be an efficient, safe and steroid-sparing agent in the treatment of a young child with TA.

Adrenal Cortex Hormones↗