Assessment of the flexibility of the lumbar spine in childhood and adolescence.

Two different tape measurement techniques were tested for anterior spinal flexion in 393 children varying in age from 7 to 14 years. With one technique the skin distraction was found to be more than 6 cm and with the other one more than 4.7 cm. Both were age-dependent but were independent of height and weight.

Chorea as a manifestation of the antiphospholipid syndrome in childhood.

Chorea has been related to the presence of antiphospholipid antibodies (a-PL) in the context of systemic lupus erythematosus (SLE). Here we report the case of a 13-year-old girl with a-PL antibodies, who had developed thrombophlebitis at the age of 11 years and chorea two years later, in the absence of clinically evident SLE. Serological tests revealed a false positive test for syphilis, a prolonged activated partial thromboplastin time, hypocomplementaemia and positive anti-DNA antibodies.

Autoantibodies in Greek juvenile chronic arthritis patients.

The aim of this study was to investigate sera of Greek patients with juvenile chronic arthritis (JCA) for the presence of autoantibodies and correlate these antibodies with the clinical picture and disease activity. Sera from 69 JCA patients and sera from 66 healthy children matched for sex and age, were tested for antinuclear antibodies (ANAs), antibodies to extractable cellular antigens (ENAs), rheumatoid factor (RF), immunoglobulins (IgG, IgM), antibodies to double stranded (ds) DNA and anticardiolipin (CL). Our results indicate that: (a) autoantibodies to dsDNA are a not uncommon finding in JCA sera; (b) these autoantibodies have a low affinity for the antigen since they are found in low titers only by ELISA, while the Farr assay and Crithidia lucilliae immunofluorescence assay (IF) are negative; and (c) active JCA patients express many autoantibodies.

The Greek 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 Greek language of the parent's version of 2 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 Greek CHAQ CHQ were fully validated with 3 forward and 3 backward translations. A total of 143 subjects were enrolled: 82 patients with JIA (28% systemic onset, 24% polyarticular onset, 10% extended oligoarticular subtype, and 38% persistent oligoarticular subtype) and 61 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 systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Greek version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Subacute cutaneous lupus-erythematosus in childhood.

Subacute cutaneous lupus erythematosus (SCLE) has been described in adults in the last decade and represents a particular homogenous subgroup in which the skin lesions can be distinguished from scarring discoid lupus lesions both on a morphologic and histopathologic basis. This entity, however, seems to be very uncommon in childhood. In the present study we describe the clinical, immunological and histopathologic characteristics of a young girl who, at the age of 3, developed SCLE.

Clinical and serological spectrum of systemic lupus erythematosus in Greek children.

In the present study 19 Greek Caucasian children with systemic lupus erythematosus (SLE), onset before the age of 16, were followed up for 1-12 years (mean 5.6 yrs.). Diagnosis was determined early in 14 patients and delayed by 2 to 6 years in 5. The clinical manifestations and laboratory findings did not differ significantly from those reported in adults with lupus. The major organ system involvement at onset and early course were skin and joints (80%) followed by kidneys (42%). During the course of the disease 26% of the children developed central nervous system (CNS) involvement. All the patients were treated with steroids and/or cytotoxic drugs in severe uncontrolled progressive disease. At the mean 5.6 years follow-up most patients were in remission on small doses of steroids; one patient still presents signs of active lupus nephritis and one patient died from sepsis. All the patients with CNS involvement recovered without permanent CNS residue.

Changes of mineral metabolism in juvenile chronic arthritis.

Thirty five children with ambulant JCA were studied to assess the biochemical parameters of bone metabolism. The mean age of the study group was 8.8 +/- 4.1 years and the mean duration of active disease 3.8 +/- 1.3 years. According to the onset of the disease the children belonged to the systemic (7), polyarticular (12) and pauciarticular type (16). All the patients were treated with NSAIDs. In addition the polyarticular group received either gold injections or D-penicillamine and the systemic group, steroids for at least 3 months. Two groups of controls were studied. The first one included fifteen children without chronic arthritis or bone disease and the second, four children who were treated with corticosteroids for a variety of reasons. In the group with systemic JCA Se Pi (1.28 +/- 0.29 mmol/l) and renal phosphate reabsorption (TmP/GFR = 1.07 +/- 0.18) were significantly lower than in the control groups (1.50 +/- 0.19; 1.54 +/- 0.25 mmol/l, p < 0.01 and 1.35 +/- 0.18; 1.29 +/- 0.23 mmol/l GF, p < 0.05). Also lower were serum alkaline phosphatase (58 +/- 16.4 versus 83 /- 24.2 and 80 +/- 15.6 IU/l, p < 0.05), osteocalcin (5.5 +/- 4.7 versus 11.0 +/- 4.5 and 10.0 +/- 5.7 ng/ml, p < 0.05), 25OHD (15.6 +/- 4.9 versus 27.3 +/- 6.2 and 20.6 +/- 9.8 ng/ml, p < 0.001) and 1,25(OH)2D (12.1 +/- 6.0 versus 20.9 +/- 11.0 and 27.6 +/- 3.2 pg/ml, p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

The impact of childhood chronic neurological diseases on Greek families.

BACKGROUND: Although the impact of childhood chronic neurological diseases (CND) on patients' psychological well-being has been increasingly addressed, little attention has been given to the influence of these conditions on family members and family functioning. The purpose of the present study was to investigate the family characteristics of Greek children suffering from CND. METHODS: A total of 52 parents of children with CND were studied by using the Family Environmental Scale (FES), the Family Burden Scale, the General Health Questionnaire (GHQ-28) and a questionnaire on the knowledge of their children's illness, their coping strategies and their satisfaction with our services. During the same period, 30 parents of hospitalized children for common paediatric illnesses completed the FES. In both groups social and demographic features were registered. Appropriate statistical processes were applied to compare the above-mentioned family groups and to study the differences between the families of children with epilepsy (n=37) and the families of children with other CND (n=15). RESULTS: Parents of children with CND discuss their problems less freely, talk less openly around home, score highly on FES subscale of Conflict and, pay more attention to ethical and religious issues and values. Furthermore, the families of children with other CND were more burdened regarding the financial state and the health status of other family members in comparison with families of children with epilepsy. In addition, families of children with epilepsy were more involved in social and recreational activities, appeared to be more knowledgeable on the availability of help in critical conditions and were more satisfied with rendered medical services, in comparison with families of children with other CND. CONCLUSION: These preliminary findings provide important information concerning the special characteristics of Greek families of children suffering from CND, which may prove especially helpful in organizing specific support services.

Sjogren syndrome in childhood: report of two cases.

A 12-year-old boy who developed primary Sjogren syndrome and a girl, whose diagnosis of secondary Sjogren syndrome was established at the age of 3, are reported. The importance of some unusual manifestations at disease onset and possible differences in expression between children and adults are briefly discussed.

Outcome of pregnancy in patients with autoimmune rheumatic disease before the disease onset.

The outcomes of 419 pregnancies of 154 unselected patients with various auto-immune diseases, including 390 pregnancies before the disease onset, were studied retrospectively. The patients comprised 40 with systemic lupus erythematosus (SLE), 72 with rheumatoid arthritis, 21 with primary Sjögren's syndrome (1 degree SS), 14 with progressive systemic sclerosis (PSS), and seven with mixed connective tissue disease. The histories of 267 pregnancies of 98 healthy, age matched women served as controls. Our data indicate that compared with healthy controls autoimmune patients do not experience a higher incidence of fetal loss. The incidence of fetal loss before disease onset in the various groups of autoimmune patients (as well as after disease onset in patients with SLE and RA) was not significantly different from that of controls. Spontaneous abortions in patients with 1 degree SS and PSS before disease onset occurred significantly more frequently (p less than 0.05) than in controls. Nevertheless, it should be noted that this was not the case when the incidence per woman was considered. On the other hand, patients with SLE, both before and after disease onset, experienced a higher incidence of premature deliveries (p less than 0.05). Finally, the analysis of autoantibody profiles, including antibodies to nuclear antigens, to Ro(SSA) cellular antigen, to double stranded DNA, and to cardiolipin, could not demonstrate any association of autoantibodies with any particular pregnancy outcome.

Free urinary cortisol immunoreactive levels in premature and full term infants.

Urinary free immunoreactive cortisol excretion was measured in 20 full term, 20 premature and 20 premature newborns of mothers who had been treated with 12 mg betamethasone 48 hours before delivery. In 10 full term newborns delivered normally, values were 40 +/- 20 nmol/mmol creatinine on the first, 23 +/- 8 on the second and 21 +/- 6 on the third day of life. In 10 full term newborns with stressful delivery, the corresponding values were 63 +/- 39, 44 +/- 33 and 32 +/- 17 nmol/mmol creatinine in the first three days of life. The levels of urinary free immunoreactive cortisol of 10 premature newborns delivered without stress were 170 +/- 116, 91 +/- 75 and 70 +/- 61 nmol/mmol creatinine respectively, on days one, two and three of life. Ten premature infants with respiratory distress syndrome had values of 471 +/- 187, 526 +/- 465 and 636 +/- 906 nmol/mmol creatinine, respectively. The 10 premature newborns whose mothers had received betamethasone, had urinary free immunoreactive cortisol levels of 109 +/- 120, 55 +/- 42 and 66 +/- 84 nmol/mmol creatinine, lower than the other premature infants. This difference, however, was not statistically significant. We conclude that premature infants regardless of stress or normal labor have high urinary free immunoreactive cortisol excretion, suggesting that prematurity per se is a potent stress.

Subacute cutaneous lupus erythematosus in Greece. A clinical, serological and genetic study.

We present herein clinical, serological and genetic findings on 27 Greek, subacute cutaneous lupus erythematosus (SCLE) patients. Twenty-three (85 p. 100) presented with a psoriasifom rash in a photosensitive distribution. Two patients (7.5 p. 100) had an annular rash in a polycyclic pattern. Two other patients exhibited both types of lesions. The majority of our patients also had other skin manifestations, including butterfly rash, discoid lesions and maculopapular rash. Systemic manifestations were: arthralgias or arthritis in 50 p. 100, low grade fever in 35 p. 100, central nervous system involvement in 15 p. 100, Sjögren's syndrome in 18 p. 100 and serositis in 8 p. 100. Anti-nuclear antibodies were found in 93 p. 100; antibodies to Ro (SSA) and/or La (SSB) in 70 p. 100. Twenty-one (78 p. 100) satisfied the ARA criteria for systemic lupus erythematosus (SLE). No HLA-DR3 antigen association was found in the 16 patients tested. These findings are somewhat different from those reported previously: a) the frequency of the psoriasiform rash rather than the annular one is the highest in Greek SCLE patients; b) most patients also have other skin manifestations mimicking the full spectrum of SLE with prominent cutaneous lesions; and c) no HLA-DR3 antigen association was found in Greek patients. Environmental, ethnic or genetic factors may be responsible for these differences.

Middle ear function in patients with juvenile chronic arthritis.

The conductive hearing of 18 patients with juvenile chronic arthritis was studied. Tympanometry was therefore undertaken for the assessment of functional state of the tympanic membrane, the ossicular chain with its ligaments and muscles, and the air cushion of the tympanic cavity. Acoustic reflex was present in all patients but an abnormal tympanometric pattern (type As) was found bilaterally or unilaterally in 10 (mostly severe cases) patients. These findings indicate that juvenile chronic arthritis changes middle ear function, probably as a result of inflammation of the synovial ossicular joints, which is followed by stiffness of the tympanic membrane and ossicular chain.

Flow rate and chemistry of parotid saliva related to dental caries and gingivitis in patients with thalassaemia major.

Twenty-one child patients with thalassaemic major (TM) and 83 healthy control children were examined for dental caries and gingivitis. Stimulated parotid gland secretions were collected from each child. Parotid saliva flow rate was measured and the saliva samples were tested for calcium, phosphorus, potassium, sodium, urea, lysozyme and immunoglobulin levels (IgA, IgG, IgM). The results showed that dental caries experience was significantly higher in the TM group. Parotid saliva flow rates in TM patients were not significantly different from those in the healthy controls. However, the median saliva concentrations of phosphorus and IgA were significantly lower in the patients than in the controls. The concentration of lysozyme was also lower in the TM group, but the difference was not statistically significant. The findings could provide an explanation for the higher dental caries experience and gingivitis observed in the TM group.

Subgroups of primary Sjögren's syndrome. Sjögren's syndrome in male and paediatric Greek patients.

OBJECTIVES: To describe the clinical and serological findings in male and paediatric Sjögren's syndrome (SS) patients. PATIENTS AND METHODS: Using the European criteria for the diagnosis of SS 12 male and 13 paediatric patients were identified and compared with those of 30 consecutive unselected adult female SS patients. RESULTS: The mean (SD) age of paediatric patients was 9.4 (2.2) years, ranging from 6 to 14 years. Recurrent parotid gland enlargement was the initial clinical manifestation in the majority of the children with a statistical significance compared with male (p < 0.01) and with female patients (p < 0.0001). Sicca manifestations were the most common clinical symptoms in male and female patients at disease onset. The systemic manifestations were similar among the three groups except that men showed lower frequency of arthritis (p < 0.05) and Raynaud's phenomenon (p < 0.05) compared with women. No differences were found among the immunological profile of children and female patients, while male patients had a lower frequency of antinuclear antibodies (p < 0.025) and antibodies to Ro(SSA) nuclear antigens (p < 0.025) compared with women. CONCLUSION: Primary SS is rare in children and men in Greece. Recurrent parotid gland enlargement is the most common clinical finding at disease onset in children. Male patients seem to have less systemic manifestations and lower frequency of autoantibodies.