Bone strength in children with growing pains: long-term follow-up.

OBJECTIVES: To examine the changes in bone strength in a cohort of children with 'growing pains' (GP) after 5 years follow-up and the correlation with pain outcome. METHODS: Bone strength was measured by quantitative ultrasound. Subjects were 39 children with GP previously studied. Controls were normograms based on the measurement of bone speed of sound in 1085 healthy children. Current GP status was assessed by parental questionnaires. Bone strength was compared with pain outcome. RESULTS: We examined 30/39 (77%) patients after 5 years. Bone strength was significantly increased when compared to the first study (Z score 0.65±1.77 vs. -0.62±0.90, p<0.001). While overall there was no significant difference in the bone strength between the 16 (53%) patients whose GP resolved and the 14 (47%) who continued to have GP episodes (p=0.71), all 6 (20%) patients with a speed of sound Z-score <-1 continued to have GP (p=0.003). CONCLUSIONS: Our findings that pain improves in most patients parallel to the increase in bone strength may support the hypothesis of GP representing in some patients a local overuse syndrome.

Protracted febrile myalgia in children and young adults with familial Mediterranean fever: analysis of 15 patients and suggested criteria for working diagnosis.

OBJECTIVES: To present an analysis of patients with protracted febrile myalgia (PFM), a rarely reported manifestation of familial Mediterranean fever (FMF), and propose clinical criteria for working diagnosis. METHODS: A multicenter retrospective cohort study of children with PFM was performed. Clinical and laboratory data were obtained by medical record review. RESULTS: The study group included 15 patients with PFM. PFM occurred as the presenting sign of FMF in 33%. FMF was diagnosed clinically in all and by genetic analysis in 93%. M694V allelic involvement was noted in 93% of the patients. PFM occurred at a mean age of 9 +/- 3.4 years and was characterized by severe generalized muscle pain in all patients and fever in 71%. Mean duration up to diagnosis was 15.5 +/- 6 days. Mean erythrocyte sedimentation rate was 104 +/- 26 mm/h; mean C-reactive protein was 15.4 +/- 6.3 mg%. Creatine kinase was normal. Treatment included corticosteroids (4 patients) and nonsteroidal anti-inflammatory drugs (NSAIDs) (9 patients) with a symptomatic relief achieved at a mean of 7.7 +/- 4.3 days and 5 +/- 3.8 days, respectively (p = 0.14) (mean severity score 3 and 2.2, respectively, p = 0.075). Symptomatic relief in 2 untreated patients was achieved at a mean of 45.5 days. CONCLUSION: Based on our data, we propose criteria for working diagnosis including: severe disabling myalgia of at least 5 days in a young patient with FMF, associated with fever, elevated levels of inflammatory markers and presence of at least one M694V mutation.

Unintentional childhood poisoning in the Sharon area in Israel: a prospective 5-year study.

OBJECTIVES: To study the epidemiology and risk factors for unintentional exposure to poisoning among the Jewish and the Arab population in the Sharon area in Israel. METHODS: We prospectively evaluated visits to the pediatric emergency department because of unintentional poisoning exposure, at the Meir General Hospital. We collected demographic data, substance exposure data, and the clinical outcome of the poisoning. RESULTS: During the 5 years of the study, 502 children were evaluated for unintentional poisoning, 84% Jewish and 16% Arabs; 88.5% occurred in children younger than 5 years, with a peak incidence at the age of 2 years (39.5%). Medications including hormones, vitamins, and antibiotics were the most common cause of exposure. Most children (95%) had no symptoms or abnormal findings on physical examination (84%), and most (85%) were discharged after several hours of observation. However, children of Arab origin presented with severe clinical manifestations because of a high rate of pesticide poisoning. There was 1 death from organic phosphate poisoning. CONCLUSION: Exposure to poisoning is commonly encountered in children. Pesticides exposure is more common in the Arab community and is usually associated with more severe clinical manifestations. Educational preventive programs are mandatory.

Long-term outcome of mothers of children with complete congenital heart block.

OBJECTIVES: To determine the health of mothers of offspring with complete congenital heart block (CHB) both at the time of delivery of the affected child and in the long-term, and the percentage of mothers and children with CHB who had anti-SSA/Ro and/or SSB/La antibodies. PATIENTS AND METHODS: Sixty-four mothers of 64 children with CHB (seen between 1964 and 1993) were identified through the Cardiology database of The Hospital for Sick Children, Toronto, Canada. Medical information from these of children with CHB was evaluated. Data were obtained from the mothers by mailed questionnaire, telephone interview, and/or from the attending physicians. The presence of anti-Ro antibodies and anti-La antibodies were evaluated by ELISA assay. RESULTS: The mean age at the time of delivery of the first child with CHB was 28 +/- 6 years. At the time of delivery 42 (66%) mothers were healthy, 2 (3%) had systemic lupus erythematosus (SLE), 2 (3%) had linear scleroderma, 2 (3%) had rheumatoid arthritis; 3 (5%) had a history of rheumatic fever (but were otherwise well), 1 (2%) had Sjogren's syndrome (SS), and 12 (19%) had an undifferentiated autoimmune syndrome (UAS) (arthralgia, myalgia, photosensitivity, skin vasculitis, Raynaud's phenomenon). The mean time to follow-up from deliver to study was 121 +/- 88 months. The mean maternal age at study was 38 +/- 9 years. Three of 12 mothers who initially had a UAS progressed to SLE (average follow-up time of 80 months, median 96), and 2 developed SS (with average follow-up time 140 months, median 132) and 1 went into remission. The mean follow-up time for the other mothers who did not develop an autoimmune disease was 150 +/- 102 months. Thirty-six of the 42 initially healthy mothers remained well. One mother developed SLE; 1 developed hyperthyroidism; 1 developed anky-losing spondylitis; and 3 developed an UAS. The mean follow-up time of the 36 mothers who remained healthy was similar (123 +/- 97 months) to the 6 initial healthy mothers who developed an autoimmune disease (121 +/- 36 months). Anti-Ro and/or anti-La antibodies were positive in 32 of 53 (60%) mothers tested. Fourteen of the 53 mothers were symptomatic at the time of delivery and 39 were asymptomatic. Anti-Ro and/or anti-La antibodies were positive in 12 of 13 mothers tested at the time of delivery. CONCLUSIONS: The long-term maternal outcome in our cohort was very good as most of the initially healthy mothers remained well at follow-up. Twenty-five percent of the mothers with a UAS and only 2% of the initially healthy mothers developed SLE. The development of an autoimmune disease in an asymptomatic mother identified by the birth of a child with CHB was less common in our study than in previous studies. However, close follow-up of mothers with UAS is warranted.

Localized scleroderma in childhood: a report of 30 cases.

Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.

Childhood brucellosis in Israel.

BACKGROUND: Brucellosis has become a major medical problem in Israel particularly in the Muslim Arab population. METHODS: Eighty-eight children with acute brucellosis are described. Sixty-seven were studied retrospectively during 1987 through 1988, and 21 children were studied prospectively during 1989 through 1992. Epidemiologic, clinical and laboratory features were evaluated, and the outcome of 4 antimicrobial regimens are compared. RESULTS: Although the clinical manifestation varied, the classical triad of fever (91%), arthralgia or arthritis (83%) and hepato- and/or splenomegaly (63%) characterized most patients. Sixty-one percent of the children had elevated liver enzymes. Brucella melitensis was isolated from 61% of blood cultures. The relapse rate in patients who were treated with monotherapy (doxycycline) was 43% compared with 14% with regimens of combined therapy with rifampin and doxycycline, streptomycin and doxycycline or rifampin and trimethoprim-sulfamethoxazole (P < 0.049). Eleven children (33%) who were treated for 3 weeks had relapse compared with 1 patient (3.5%) treated for 4 weeks or longer. The total relapse or reinfection rate was 20%. All patients with relapse recovered after a second course of antibiotic therapy. During the 2 years of follow-up one child progressed to chronic osteomyelitis. CONCLUSIONS: Combination therapy and extending treatment for 4 weeks or longer gave significantly better results than monotherapy or shorter courses of therapy and resulted in fewer relapses.

Intravenous immunoglobulin therapy in a child with cutaneous polyarteritis nodosa.

Cutaneous polyarteritis nodosa (CPAN) may have a prolonged recurrent course which needs chronic corticosteroids treatment to achieve remission. In this report we describe a 9 year old boy who developed CPAN, which we treated with high dose intravenous immunoglobulin (IVIG), with an immediate favourable response. We discuss the advantages of IVIG over corticosteroids and speculate on its pathogenesis and mechanism of action.

Lymphocytic interstitial pneumonitis preceding polyarticular juvenile rheumatoid arthritis.

We describe the case of a 12-year-old girl who developed lymphocytic interstitial pneumonitis at 2 years of age which preceded polyarticular rheumatoid factor (RF) positive juvenile arthritis. Her disease had a chronic active course, but showed a good response to combination therapy. The pathogenesis of these two immune processes and of the lung involvement in juvenile arthritis is discussed.

Persistent pulmonary hemorrhage as the sole initial clinical manifestation of pediatric systemic lupus erythematosus.

Pulmonary hemorrhage (PH) is a rare but potentially life-threatening manifestation of systemic lupus erythematosus (SLE). In this report we describe a 13 year old girl with PH as the sole presenting clinical manifestation of her SLE. Her serology was diagnostic of SLE and one year after presentation she developed arthritis. She had a rapid serologic but delayed clinical response to combination therapy of intravenous pulse methylprednisolone, pulse cyclophosphamide and daily prednisone. Awareness of the possibility of pulmonary hemorrhage as a presentation of SLE may aid in the diagnosis and early, aggressive management of this condition.

Disease patterns of patients with Behçet's disease demonstrated by factor analysis.

OBJECTIVE: To explore the main patterns of Behçet's disease (BD) expression, applying factor analysis. METHODS: Sixty-eight BD patients were studied. The following disease manifestations were used for the factor analysis: genital ulcerations, typical skin lesions (erythema nodosum, folliculitis or papulo-pustular rash), uveitis, CNS involvement, joint disease, deep vein and superficial vein thrombosis, and gastrointestinal manifestations. The results were further analyzed according to sex, HLA typing, and childhood vs. adult-onset disease. RESULTS: Five factors were derived, which accounted for 69% of the variance of the matrix. Factor 1 represented the association between folliculitis and genital ulceration. Factor 2 represented the association between papulo-pustular rash and gastrointestinal symptoms. Factor 3 represented the inverse association between superficial vein thrombosis and erythema nodosum. Factor 4 represented the correlation between deep vein thrombosis and neuro-Behçet. Factor 5 represented joint disease. No difference was found between males and females in relation to factors 1, 2 or 5, but factors 3 and 4 had higher scores in male patients (p = 0.1 and p = 0.07, respectively). Factor 3 was significantly higher in patients with HLA-B5, compared to HLA-B5-negative BD patients (p < 0.001). Factors 1 and 3 were higher in patients with adult onset of the disease (p = 0.07, and p = 0.003, respectively), while factor 2 was higher in patients with childhood-onset BD (p = 0.07). CONCLUSIONS: The application of factor analysis revealed possible associations between distinct types of skin lesions, or venous thrombosis, and other disease manifestations of Behçet's syndrome, some of which were sex, age at onset, or HLA-related.

Juvenile Behçet's disease in Israel. The Pediatric Rheumatology Study Group of Israel.

OBJECTIVE: Behçet's disease (BD) is a vasculitis mainly observed in young adult males. Juvenile BD is rare and only small series of pediatric cases have been reported. The objective of this study was to define the epidemiology and clinical features of BD among Israeli children. METHODS: A questionnaire was sent to 8 pediatric rheumatology units in Israel and 30 cases of BD diagnosed before the age of 16 years were identified. RESULTS: Fifteen patients fulfilled the International Study Group Criteria for BD, while 15 had an incomplete form of BD. Among the patients with complete BD, stomatitis and skin involvement were the most common manifestations. Other symptoms included genital ulcers, uveitis, CNS involvement, arthritis, and gastrointestinal involvement. A positive family history was elicited in 3 patients. HLA B5 was found in 7 of 12 patients (58%). The 15 patients with incomplete BD all had recurrent stomatitis; other manifestations included uveitis, arthritis, and genital ulcers. HLA B5 was found in 94% of this group. CONCLUSION: Juvenile BD in Israel is not uncommon, and is frequently associated with HLA B5 positivity. This could indicate a genetic susceptibility in our region. Half of the patients in our series had an incomplete form of BD, which may represent a less severe variant of the disease. In any case, careful follow-up is required, since their condition could eventually evolve into complete BD.

The Hebrew version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Hebrew language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Hebrew CHAQ-CHQ were fully developed with 3 forward and 3 backward translations. A total of 144 subjects were enrolled: 80 patients with JIA (12% systemic onset, 34% polyarticular onset, 23% extended oligoarticular subtype, and 31% persistent oligoarticular subtype) and 64 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the JIA patients having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Hebrew version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Scleroderma in children.

Childhood scleroderma may present in a variety of clinical forms that differ in clinical presentation, extracutaneous features, clinical course, and outcome. All include hardening of the skin as a major feature. This article reviews these various entities, focusing on primarily the clinical features. In addition, current concepts regarding pathogenesis and treatment are discussed.

Assessment of quality of life of parents of children with juvenile chronic arthritis.

The aim of the study was to assess the quality of life (QOL) and the psychological status of parents of children with juvenile chronic arthritis (JCA). The QOL, anxiety and depression of the parents of 28 children with JCA were evaluated and compared to those of the parents of 28 healthy children. Mothers of JCA children and mothers of healthy children reported similar QOL. The reported anxiety and depression levels were similar for mothers and fathers in both groups. The parents of children with pauciarticular-type JCA reported lower QOL and higher levels of anxiety and depression than the parents of children with other types, namely polyarticular and systemic JCA. These findings may be explained by the fact that the pauciarticular patients had shorter disease duration and were less frequently seen in the outpatient clinic. The QOL of mothers of children with JCA was found to be slightly impaired in the group of children with pauciarticular JCA. Future larger studies are needed to confirm these results, as the number of subjects in the three groups was rather low.

[Hospitalization policy following minor head injuries].

Of 44 patients admitted with head injuries during 1980-84, 316 (72%) arrived fully conscious with normal neurological status and no X-ray evidence of skull fracture. These cases were admitted for observation, but in none was there any untoward development. On analysis of the cases that developed complications and in the light of the literature, we conclude that it is possible to define a group at low risk for developing complications following head injury. These patients could safely be discharged from the emergency room without admission for observation. This group included 176 (40%) of those admitted and fulfilled the following criteria. They arrived fully conscious, there was no recurrent vomiting, neurological examination was normal, there was no X-ray evidence of skull fracture, and they were between 16-70 years of age. However, these cases should only be discharged from the emergency room after a physician gives oral and written instructions to a responsible adult who accompanies the patient. Such a policy would result in considerable saving of resources by reducing admissions for observation of those at low risk for developing complications.

[Systemic lupus erythematosus in children in Israel].

Systemic lupus erythematosus (SLE) is a rare disease in children that might possibly be modulated by genetic and environmental factors. In order to delineate the characteristic features of SLE among Israeli children, we reviewed the medical records of 38 cases from 8 pediatric rheumatology clinics. All fulfilled the 1982 American Rheumatism Association revised criteria for SLE. The illness became apparent at the age of 16 years or younger and the mean age of onset was 11.9 +/- 2.4 y (range 7-16) and the mean duration of follow-up 4.0 +/- 4.8 y (range 0.5-15). The female to male ratio was 2.8:1; 28 were Jewish and 10 Arabs. Systemic complaints, such as fever, malaise and weight loss, were noted in 90%, malar rash in 65%, and other skin manifestations in 40%. Arthritis was noted in 57% and additional musculoskeletal complaints in 70%; 90% had hematological abnormalities. Major organ system involvement included: renal disease in 50% pulmonary involvement 28% and CNS involvement 28%. 2 patients are currently on renal dialysis and 1 died from hypertensive crisis. We conclude that the features of SLE in children in Israel are not influenced by ethnic or geographic factors, and are similar to those reported worldwide.