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Biomedical subjects

A Gedalia

Publications and source records attributed to A Gedalia.

At least 37 records · Page 2Linked to original sources
PubMed1997

Low-dose methotrexate therapy for childhood sarcoidosis.

OBJECTIVE: To evaluate the effectiveness of low oral doses of methotrexate as a steroid-sparing agent in children with sarcoidosis. STUDY DESIGN: An open-label, noncontrolled trial. Methotrexate was administered orally at a single weekly dose of 10 to 15 mg/m2. Duration of therapy was open ended, but patients received treatment for a minimum of 6 months to be considered as having completed the study. RESULTS: Seven children with biopsy-proven sarcoidosis completed the study. The mean dose of prednisone was successfully tapered from 49 mg/day (1.3 mg/kg) to 18 mg/day (0.5 mg/kg) after 3 months of methotrexate therapy and to 9.9 (0.2 mg/kg) and 7.3 mg/day (0.1 mg/kg) after 6 months and at the end of the follow-up period, respectively. Other clinical and laboratory parameters improved significantly after methotrexate therapy was started. There was significant clinical improvement, as confirmed by the reduction of the clinical severity score from 8 +/- 1.1 to 0.8 +/- 0.5 point after 3 months of methotrexate therapy, and to 0.7 and 0.5 +/- 0.3 point after 6 months and at the end of the follow-up, respectively. Laboratory measurements revealed marked improvement, as reflected by a significant reduction in the erythrocyte sedimentation rate and an increase of hemoglobin values. The mean serum angiotensin-converting enzyme activity dropped significantly. No adverse side effects were noted with methotrexate therapy. CONCLUSION: Our study demonstrated that low-dose oral methotrexate therapy was effective and safe and had steroid-sparing properties in seven children with sarcoidosis.

Administration, Oral↗
PubMed1997

Anti-Ro/SS-A and anti-La/SS-B antibodies associated with cardiac involvement in childhood systemic lupus erythematosus.

OBJECTIVES: To determine the frequency and type of cardiac manifestations in children with systemic lupus erythematosus (SLE) and investigate whether cardiac involvement of SLE in children was associated with any autoantibody pattern. METHODS: Retrospective analysis of the medical records of all children with SLE (31 patients) seen between January 1984 and January 1994 by the paediatric rheumatology service at Children's Hospital in New Orleans. All patients satisfied the American College of Rheumatology criteria for the diagnosis of SLE. Paediatric SLE patients with cardiac manifestations based on echocardiogram were identified. Autoantibody tests at diagnosis were identified retrospectively by chart review, and the correlation between autoantibodies and cardiac involvement was analysed using the two tailed Fisher's exact test. RESULTS: Thirteen (42%) of 31 SLE patients had cardiac manifestations of SLE. Seven (22%) had pericarditis without myocarditis, five (16%) had pericarditis and myocarditis, and one (3%) had myocarditis without pericarditis. Two patients (6%) with pericarditis had cardiac tamponade. Cardiac manifestations of SLE usually occurred at the time of diagnosis or within six months. Anti-Ro/SS-A antibodies were present in serum samples of nine of 11 (82%) patients with cardiac involvement and in five of 15 (33%) without cardiac involvement (p = 0.02). Anti-La/SS-B antibodies were present in serum samples of six of 10 (60%) patients with cardiac involvement and two of 15 (13%) without cardiac involvement (p = 0.03). Anti-Sm and anti-RNP antibodies showed no correlation with the presence of cardiac disease. CONCLUSIONS: Cardiac involvement in our paediatric SLE population was frequently found and correlated significantly with the presence of anti-Ro/SS-A and anti-La/SS-B antibodies.

Adolescent↗
PubMed1996

Abdominal aortic aneurysm associated with childhood sarcoidosis.

Childhood sarcoidosis is a rare disease with multisystemic organ involvement. A wide spectrum of vasculitides, including large vessel involvement, have been reported in childhood sarcoidosis. We describe a 7-year-old boy with childhood sarcoidosis who presented with prolonged fever, iritis, and abdominal aortic aneurysm. He underwent successful surgical repair of the abdominal aortic aneurysm and followup for 12 months was uneventful. This is first report of abdominal aortic aneurysm complicating childhood sarcoidosis.

Aortic Aneurysm, Abdominal↗
PubMed1996

Protein losing enteropathy as the initial manifestation of childhood systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem organ disease, and involvement of the gastrointestinal system is relatively rare. We describe a 13-year-old girl who presented initially with abdominal pain, diarrhea, edema, and hypoalbuminemia. She was diagnosed with protein losing enteropathy (PLE) based on the significant increase of alpha 1-antitrypsin clearance in the stool. Two weeks after admission she developed clinical and serological findings that fulfilled the ACR criteria for SLE. Over 22 cases of lupus associated PLE have now been reported, but only 3 in children. Children with PLE should be evaluated for SLE. In addition, PLE should be suspected as a possible cause of unexplained edema and/or hypoalbuminemia in SLE.

Adolescent↗
PubMed1995

Case report: steroid sparing effect of intravenous gamma globulin in a child with necrotizing vasculitis.

This article reports the case of a boy aged 2 years 6 months, who had fever, arthritis, and necrotizing cutaneous vasculitis. Evaluation revealed no evidence of direct infectious causes. However, high anti-streptolysin and streptozyme titers during the acute phase support the possibility that streptococcal infection played an important role in the pathogenesis of this disease. The patient's condition improved significantly with the administration of prednisone. However, several attempts to diminish the prednisone dose resulted in relapses. Subsequently, the prednisone was successfully tapered and discontinued after intravenous gamma globulin administration. It is recommended that intravenous gamma globulin should be considered before immunosuppressive therapy in the treatment of necrotizing vasculitis.

Child, Preschool↗
PubMed1995

Fibromyalgia syndrome in children--an outcome study.

OBJECTIVE: To assess the outcome of fibromyalgia syndrome (FM) in a 30-month followup study of children with FM. METHODS: In the original study to assess the prevalence of FM in healthy schoolchildren, we found 21 children with FM and an additional 7 fulfilling the point count criterion only (11 of 18). Fifteen of the 21 and all 7 were recruited for a 2nd assessment in our present study. In all children, a count of 18 tender points (TP) was conducted by thumb palpation. Tenderness of 9 of the TP sites as well as 4 control point sites was further assessed using a Chatillon dolorimeter. All children were questioned concerning the presence of widespread pain or aching. Children were considered to have FM if they met the American College of Rheumatology criteria for diagnosis. RESULTS: After 30 months, 11 of the 15 children with FM (73%) were no longer fibromyalgic. The mean point count of the 15 children significantly decreased from 12.5 to 4.6 (p < 0.001). The mean tenderness threshold of the 9 tender sites increased from 2.4 to 3.4 kg (p < 0.01), and the mean tenderness threshold of the 4 control sites increased from 4.1 to 5.6 kg (p < 0.05). Of the 7 children initially fulfilling the point count criterion only, none had developed FM. Their mean point count decreased from 11.4 to 3.4 (p = 0.001), and their mean tenderness thresholds increased from 2.7 kg to 3.9 kg (p = 0.001) at tender sites and from 4.3 kg to 6.8 kg (p < 0.001) at control sites. CONCLUSION: We suggest that the outcome of FM in children is more favorable than in adults. More followup studies are needed to clarify the longterm outcomes of FM in children and adults.

Adolescent↗
PubMed1993

Sulphasalazine in the treatment of pauciarticular-onset juvenile chronic arthritis.

Sulphasalazine in a dose of 50 mg/kg/day was administered to ten patients with pauciarticular-onset juvenile chronic arthritis (JCA), with active disease not adequately controlled by nonsteroidal anti-inflammatory drugs (NSAID). The treatment was initiated with 1/4 of this dose and increased by weekly increments of 250-500 mg until the total dose was reached. In all patients sulphasalazine was the first disease-modifying agent tried. Among nine of the ten patients there was significant improvement in all clinical scores, including the number of active joints and the severity grading (tenderness and limitation of motion). Within 3 months of sulphasalazine therapy the laboratory measurements revealed marked improvement in the erythrocyte sedimentation rate (ESR) and haemoglobin values. One patient, in whom the ESR and haemoglobin were normal at onset, had no change in clinical scores. Transient skin rash and elevated liver enzyme levels developed in one patient. These preliminary data suggest that sulphasalazine is an effective and safe second-line agent in the management of pauciarticular-onset JCA. More trials with this drug are needed, including double blind, to study efficacy and safety of sulphasalazine in JCA.

Adolescent↗
PubMed1993

Joint hypermobility and fibromyalgia in schoolchildren.

OBJECTIVES: To test the hypothesis that joint hypermobility may play a part in the pathogenesis of pain in fibromyalgia, schoolchildren were examined for the coexistence of joint hypermobility and fibromyalgia. METHODS: The study group consisted of 338 children (179 boys, 159 girls; mean age 11.5 years, range 9-15 years) from one public school in Beer-Sheva, Israel. In the assessment of joint hypermobility, the criteria devised by Carter and Bird were used. Any child who met at least three of five criteria was considered to have joint hypermobility. Children were considered to have fibromyalgia if they fulfilled the 1990 American College of Rheumatology criteria for the diagnosis of fibromyalgia, namely, widespread pain in combination with tenderness of 11 or more of the 18 specific tender point sites. The blind assessments of joint hypermobility (by AG) and fibromyalgia (by DB) were carried out independently. RESULTS: Of the 338 children 43 (13%) were found to have joint hypermobility and 21 (6%) fibromyalgia; 17 (81%) of the 21 with fibromyalgia had joint hypermobility and 17 (40%) of the 43 with joint hypermobility had fibromyalgia. Using chi 2 statistical analysis, joint hypermobility and fibromyalgia were found to be highly associated. CONCLUSIONS: This study suggests that there is a strong association between joint hypermobility and fibromyalgia in schoolchildren. It is possible that joint hypermobility may play a part in the pathogenesis of pain in fibromyalgia. More studies are needed to establish the clinical significance of this observation.

Adolescent↗
PubMed1993

Assessment of nonarticular tenderness and prevalence of fibromyalgia in hyperprolactinemic women.

OBJECTIVE: To assess nonarticular tenderness and prevalence of fibromyalgia syndrome (FMS) in hyperprolactinemic subjects. METHODS: Twenty-one consecutive women with hyperprolactinemia (HPRL) and 44 consecutive women with normal prolactin levels (PRL) were examined in the fertility unit by one observer during a 24-month period. Mean age was 31 years, range 22-46 years. Hyperprolactinemia was defined as PRL levels > 24.5 ng/ml (immunoradiometric assay). In all women, a count of 18 tender points (TP) was conducted by thumb palpation. Tenderness of some of the TP sites (9 points) as well as control point sites (4 points) was further assessed using a Chatillon dolorimeter. All women were questioned about the presence of widespread pain or aching. Women were considered to have FMS if they met the American College of Rheumatology (ACR) criteria for diagnosis. RESULTS: Of the 21 women with HPRL, 15 (71%) had FMS vs only 2 of 44 (4.5%) normoprolactinemic women (p < 0.0001). Thresholds of tenderness of 9 fibrositic points were 2.7 (1.5) kg [mean (standard deviation)] for HPRL women vs 5.0 (1.4) (kg) for women with normal PRL (p < 0.0001). Thresholds of tenderness of 4 control points were 5.2 (1.5) (kg) for women with HPRL vs 6.8 (1.1) (kg) for normoprolactinemic women (p < 0.0001). The frequency of FMS was directly associated with the level of PRL. CONCLUSIONS: We suggest that FMS is very common in a subset of women with HPRL and that its frequency is directly associated with the degree of hyperprolactinemia. Women with HPRL have lower thresholds of tenderness than women with normal PRL. More studies are needed to clarify the relationships between PRL, FMS and tenderness.

Adult↗
PubMed1993

Assessment of nonarticular tenderness and prevalence of fibromyalgia in children.

Fibromyalgia syndrome (FS) is most common in midlife, but may be seen at any age. Its prevalence and assessment of tenderness in healthy children is not known. We assessed 338 healthy schoolchildren for tenderness thresholds and prevalence of FS. In all children a point count of 18 tender points (TP) was conducted by thumb palpation and tenderness of some of the TP sites as well as control point sites was further assessed using a Chatillon dolorimeter. All children and their parents were questioned about the presence of widespread pain or aching. Children were considered to have FS if they met the American College of Rheumatology (ACR) criteria for diagnosis of FS. Of the 338 children, 21 (6.2%) had FS. Thresholds of tenderness of 9 TP were 5.0 (1.2) (kg) [mean (standard deviation)] for boys vs 3.6 (0.8) (kg) for girls (p < 0.001). Thresholds of tenderness of the control point sites were 7.1 (1.4) (kg) for boys vs 5.5 (1.1) (kg) for girls (p < 0.001). Thresholds of tenderness of TP and control points in the children with FS were 2.5 (0.4) (kg) and 4.2 (0.5) (kg) vs 4.5 (1.2) (kg) and 6.6 (1.4) (kg) respectively in the children without FS (p < 0.001). We suggest that FS is common in the pediatric age group. Boys have lower tenderness than girls; children with FS have lower thresholds for tenderness both at control and TP compared to the subjects without FS.

Adolescent↗
PubMed1993

Joint hypermobility in pediatric practice--a review.

It has been well established that joint hypermobility may predispose children to the development of joint pain. Joint hypermobility represents an extreme variation of normal joint mobility with no underlying connective tissue diseases. Its prevalence among children varies from series to series. Evidence that there are differences between races and sexes exist. Joint hypermobility diminishes markedly throughout childhood and then more slowly during adult life. Although quantitative measurements in the assessment of joint hypermobility have been described, the criteria defined by Carter and Bird are preferred. Any subject who is able to perform 3 or more of the 5 maneuvers devised by these criteria was considered to have joint hypermobility. The mechanism in which joint symptoms develop is not well understood. Excessive motion or inappropriate physical activities that hyperextend the joint capsule, the ligaments and soft tissue constituent causing injury. These injuries are manifested by pain. The clinical features, prognosis and relevance to pediatric practice are discussed.

Child↗
PubMed1992

[Evans syndrome in a child].

A Bedouin girl, aged 2 3/12, was first seen at the age of 9 months for sequential onset of Coombs-positive hemolytic anemia and immune thrombocytopenia. Comprehensive laboratory workup revealed no underlying disease. Evans' syndrome, relatively rare in childhood, was diagnosed and corticosteroid therapy initiated. She responded well and normal hemoglobin and thrombocyte counts were achieved within a month. All attempts at tapering-off the dose of corticosteroids resulted in relapses, until high-dose intravenous gamma-globulin was given.

Adrenal Cortex Hormones↗