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8.1 Aseptic femoral necrosis in a patient with systemic JIA
A 15 year old girl with a systemic onset of JIA and a persistent systemic course, who had been followed up for 14 months in our clinic, presented with a continuous pain, unresponsive to analgesics, located in the soft tissues of the lower part of her right thigh. Up to that time the patient had received prednisone (PS) 0.8→0.3 mg/kg/d for 4 months and then on alternate days, methotrexate 15 mg/m2 and indometacin 2 mg/kg/d. At the time the pain occurred she was receiving indometacin, PS (20 mg/kg/d), and cyclosporin A (3 mg/kg/d). There was no previous history of trauma in the affected limb. Her imaging work up showed the following:x rays of knees and femur—skip lesions in the right femur, one sclerotic in the middle shaft with heavy periosteal reaction and another one mainly lytic with destruction of the cortical bone and periosteal reaction. Magnetic resonance imaging showed skip lesions localised in the middle third of diaphysis and in the distal metaphysis of the femur. The lesions destroyed the cortical bone, infiltrated the bone marrow, and produced a periosteal reaction without extension to the soft tissues or in the epiphysis. These lesions were indicative of a possible bone solid tumour—that is, osteosarcoma or Ewing's sarcoma. However, a thallium scan was not indicative of malignancy. Her haematological and biochemical findings, including alkaline phosphate and lactate dehydrogenase, were also within normal limits. An open bone biopsy was then performed and ruled out the chance of malignancy; it showed aseptic necrotic lesions in the femur. The case is presented because of the unusual location and imaging appearance of the aseptic necrosis.
8.2 Disablement by untreated systemic juvenile idiopathic arthritis
Background—We commonly only know the final state of systemic juvenile idiopathic arthritis (sJIA) from pictures in medical textbooks. Nowadays effective drugs exist which can stop the inflammation preventing disability.
We present an 8 years 3 months old girl who had had sJIA since the age of three.
Case report—Personal history: 1994 diagnosis of sJIA. 1995 begin treatment with non-steroidal anti-inflammatory drugs, 1995 additional treatment with steroids. 1996/97/98 tapering of steroids and discontinuation in November 1998. January 1998–99 treatment with growth hormone because of impressive growth retardation. January 1999 exacerbation of sJIA. A trial of treatment with methotrexate was discontinued after two weeks. Since then only homeopathic treatment has been given by the parents. Clinical examination December 1999: bad health, all joints are extremely swollen, very painful on movement and palpation, severe limitation of motion, and functional loss in every joint. The girl can nearly walk; she cannot stand up or sit down or lie down when in sitting position. She is not able to lie flat on her back or to dress or undress, her neck is fixed in a flexion contracture and she can neither look up nor turn her head at all. Growth retardation, retrognathia. x Ray: very severe destruction with loss of joint space, erosions, and signs of aseptic necroses. Possible instability of the atlanto-occipital joint.
Conclusions—Systemic JIA is a severe chronic illness, which untreated can lead to most severe destruction despite its mostly favourable prognosis. In most cases sJIA can nowadays be treated effectively with drugs to prevent disability. Children with sJIA therefore must be treated effectively and in time.
8.3 Pain in arms and legs in childhood
Objective—To describe the different causes and characteristics of a group of children with referred pain in the arms and legs.
Materials and methods—We studied, from 1995 to 1999, 208 school age children from Tenerife island, with referred pain in arms and legs. The diagnosis was made from a clinical history, physical examination, blood tests, and radiological study in all of them; additional tests were need for some of them (bone gammagraphy, computed tomography, or magnetic resonance).
Results—Of the 208 children, 102 presented organic causes and 106 were diagnosed as growth pains. In the group with organic pains, 38 were diagnosed as arthritis (17 were septic arthritis and 21 juvenile chronic arthritis, 12 oligoarticular, and 9 polyarticular), and 64 as myositis. In the group with arthritis the joints most commonly affected were the knees (left knee in 14 children, and right knee in 6). In the group with myositis 80% of the children had had an enteric or upper respiratory infection previously. In the group labelled as growth pains, additional tests (bone gammagraphy, muscle enzymes) were carried out in 8 children to rule out organic causes. The course was favourable in all the patients with growth pains and myositis. In the arthritis group, treatment was started in all of them and the outcome was favourable in only 29.
Conclusions—Pain of arms and legs, particularly growth pain, in childhood is common, though few reports exist in the current literature. Moreover, they are an important cause of absence from school, causing concern to parents. The results of our study confirm this assumption.
8.4 Cementless total hip arthroplasty in juvenile idopathic arthritis
From 1975 to 1997 40 patients with juvenile idiopathic arthritis (JA) had 68 cementless hip replacements. Most prostheses used were Zweymuller protheses. Autotopic bone grafts were used for important acetabular defects. The average age of the patients at the time of surgery was 17.5 years (range 11–29). JA was systemic in 12 cases, polyarticular in 28 cases. Sixteen hips had had a surgical synovectomy with triamcinolone hexacetonide previously and JA was active in 16 cases. Functional results were evaluated according to the Postel Merle d'Aubigné scale for the hip, and the Steinbrocker classification was used for global function. Mean follow up was 7 years (range 3–15). 6 patients required revision: one for infection, three for acetabular loosening with Endler component, one femoral shaft fracture and a loosening of the Zweymuller acetabular cup owing to a bad primary fixation.
Results for the hips were rated excellent and good for motion and pain. However, functional results observed were quite different because of the location of arthritis, and 8 patients needed another reconstructive procedure to recover good function. A radiographic review showed good bone integration of the components despite early migration in cases of bad primary fixation. It showed the thickness of the femoral shaft bone around the femoral piece. Use of cementless prostheses appears to be a good solution in this disease, preserving bone stock and showing good radiological and functional results.
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