[A boy with Churg-Strauss syndrome and thrombosis associated with eosinophilia]. / Een jongen met churg-strauss-syndroom en trombose samenhangend met eosinofilie.

A 13-year-old boy, recently diagnosed as having Churg-Strauss syndrome, presented with a thrombus at the level of the aortic bifurcation. Echocardiography revealed a left-ventricular thrombus as well. He was treated by means of surgical thrombectomy, corticosteroids, heparinisation and a coumarine derivative. Eosinophilia (an increase in the number of eosinophilic granulocytes in the circulation or tissues) occurs in various diseases, such as allergic diseases, parasitic infestations, haematologic diseases and autoimmune diseases like the rare Churg-Strauss syndrome. It appears likely that eosinophilia played an important role in the development of both thrombi in the described patient. Eosinophils produce cationic proteins that may inhibit the effect of natural anticoagulants. Infiltration of eosinophils may also cause endocardial damage with the subsequent formation of intracardial thrombi.

Hypovitaminosis D-related myopathy in immigrant teenagers.

INTRODUCTION: Vitamin D deficiency resulting in a limb-girdle muscle weakness was diagnosed in three veiled immigrant teenage girls. PATIENTS: Three girls had a progressive muscle weakness and pain during a period varying from 6 months to two years. On examination limb girdle muscle weakness, predominantly of the lower extremities, without other neurological abnormalities was found. Serum examination showed a decreased level of vitamin D and phosphate and an increased alkaline phosphatase, and in two girls decreased calcium and increased parathyroid hormone levels were found. After supplementation with vitamin D, the pain subsided and muscle strength increased within weeks. Serum examination of the female relatives revealed eight persons with hypovitaminosis D, without any complaints. CONCLUSIONS: Vitamin D deficiency can result in a limb-girdle myopathy in veiled immigrant teenagers in the Netherlands. Vitamin D supplementation leads to rapid recovery of the muscle strength. The female relatives of these patients should be examined too.

Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis.

This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of this adverse event, in particular none developed severe infections. All patients regained normal immunoglobulin levels after discontinuing SSZ treatment. One patient with a partial IgA deficiency at the start of SSZ treatment showed a slow increase in the IgA level during treatment. During follow-up (4-6 years), one patient spontaneously developed a dysimmunoglobulinemia and one patient developed diabetes mellitus. Based on these case reports and review of the literature we advocate monitoring of serum immunoglobulin levels while on SSZ treatment.

Sulfasalazine in the treatment of juvenile chronic arthritis: a randomized, double-blind, placebo-controlled, multicenter study. Dutch Juvenile Chronic Arthritis Study Group.

OBJECTIVE: To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juvenile chronic arthritis (JCA). METHODS: We conducted a 24-week randomized, placebo-controlled, double-blind, multicenter study of patients with active JCA of both oligoarticular and polyarticular onset. Patients were treated with a dosage of 50 mg/kg/day of SSZ (maximum 2,000 mg/day) or placebo. The efficacy variables were joint scores, physician's, parents', and patient's overall assessments, and laboratory parameters of inflammation. RESULTS: Of the 69 patients enrolled, 52 (75%) completed the trial. Six patients (18%) withdrew from the placebo group, and 11 (31%) withdrew from the SSZ group (P = 0.18). In the intention-to-treat analysis of end point efficacy, between-group differences were significant for the overall articular severity score (P = 0.02), all global assessments (P = 0.01), and the laboratory parameters (P < 0.001). Adverse events occurred more frequently in the SSZ group and were the main reason for withdrawal (P < 0.001), but in all instances, these events were transient or reversible upon cessation of treatment. CONCLUSION: The results of this first placebo-controlled study show that SSZ is effective and safe in the treatment of children with oligoarticular- and polyarticular-onset JCA, although it was not well tolerated in one-third of the patients.

[Chronic fatigue syndrome in young persons]. / Het chronische-vermoeidheidsyndroom bij jongeren.

The prevalence of chronic fatigue syndrome (CFS) in teenagers is 10-20 per 100,000 inhabitants in the Netherlands. The natural course of the disorder is not favourable according to the literature. Proposed criteria for the diagnosis 'CFS' in adolescence are: absence of a physical explanation for the complaints, a disabling fatigue for at least six months and prolonged school absenteeism or severe motor and social disabilities. Exclusion criterion should be a psychiatric disorder. Factors that attribute to the persistence of fatigue are somatic attributions, illness enhancing cognitions and behaviour of parents as well as physical inactivity. The role of the physician and the role of parents can enhance the problems. The treatment should focus on decreasing the somatic attributions, on reinforcement by the parents of healthy adolescent behaviour, on the gradual increase of physical activity and on decreasing attention (including medical attention) for the somatic complaints.

[D-penicillamine in treatment of scleroderma "en coup de sabre"]. / D-Penizillamin in der Behandlung von Sklerodermie "en coup de sabre".

A ten year old boy with linear erythema above the right eyebrow and a groove in the underlying skull bone is described. Histological examination of a biopsy revealed a spotty, periadnexal, perivascular, lymphocytic infiltrate. The papillary dermis was almost absent and the collagen fibres were slightly thickened. Based on the clinical appearance and the histology a diagnosis of linear scleroderma (en coup de sabre) was made. As the signs and symptoms were progressive, treatment with D-penicillamine was started. Subsequently the erythema disappeared. The shallow groove in the skull bone remained palpable. After thirteen months, treatment was stopped. The patient is currently free of signs and symptoms after a follow-up period of three and a half years. We feel that the strong clinical improvement is due to treatment with D-penicillamine rather than to the natural course of the disease.

Pseudoporphyria due to naproxen. A cluster of 3 cases.

Pseudoporphyria is a photo-induced blistering disorder with increased skin fragility, caused among others by nonsteroidal antiinflammatory drugs. Lesions heal with scarring and milia. Porphyrin screen studies are normal in this disease. Histology and immunofluorescence resembles porphyria cutanea tarda. In this report we describe a cluster of three cases of naproxen-induced pseudoporphyria, and review briefly previously reported cases induced by naproxen. The majority of reported cases involve children. Physicians should be aware of this reversible skin disorder.

Altered immunoglobulin concentrations and light chain ratios in juvenile onset mixed connective tissue disease.

Immunological disturbances may result in altered immunoglobulin concentrations and kappa/lambda light chain (kappa/lambda) ratios. We measured the kappa/lambda ratios of total serum immunoglobulins and of polyclonal IgG, A, and M separately as well as concentrations of these immunoglobulins in fourteen patients with juvenile onset mixed connective tissue disease. When comparing the patient group with a reference group the mean serum IgG and IgA concentrations were respectively 2.98 G/L (p = 0.0012) and 0.79 G/L (p = 0.0114) higher in the group of patients with juvenile onset mixed connective tissue disease. The mean IgM concentration was 0.39 G/L (p = 0.0002) lower. The mean kappa/lambda ratios of total serum immunoglobulins, serum IgG, and serum IgA were respectively 0.20 (p = 0.0226), 0.28 (p = 0.0016) and 0.10 (p = 0.0732), higher in the group of patients with mixed connective tissue disease as compared with the reference group. Mean serum IgM kappa/lambda ratio, however, was 0.21 (p = 0.0046) lower. The alterations of the serum immunoglobulin concentrations and of the kappa/lambda ratios reflect immunological disturbances in patients with juvenile onset mixed connective tissue disease. The increased concentration of serum IgG and raised IgG kappa/lambda ratio and decreased concentration of serum IgM with decreased IgM kappa/lambda ratio indicate that the synthesis of kappa-bearing immunoglobulins mainly is affected.

[Congenital complete atrioventricular block in neonatal lupus erythematosus]. / Congenitaal compleet atrioventriculair block bij neonatale lupus erythematosus.

Fetal bradycardia of 60 beats per minute was diagnosed in the 22nd week of a normal pregnancy. Echocardiography showed a complete fetal atrioventricular heart block. The fetal heart was without structural abnormalities. Auto-antibodies (Ro/SS-A and antinuclear antibodies) could be demonstrated in both mother and child. The mother, however, showed no signs or symptoms of a connective tissue disease. We describe neonatal lupus erythematosus as an important cause of congenital heart block.

Juvenile-onset mixed connective tissue disease: longitudinal follow-up.

To establish the symptoms and clinical course of juvenile-onset mixed connective tissue disease, we studied 14 patients, classified according the criteria of Kasukawa et al. The patient records were studied retrospectively and all patients were examined in a 1-day follow-up program. Systemic lupus erythematosus and polymyositis/dermatomyositis-like symptoms disappeared in time, whereas scleroderma-like symptoms (such as in the Raynaud phenomenon) and joint abnormalities persisted. Extensive limitation of joint function was found in four patients. At the time of follow-up, no active renal disease was found. Thrombocytopenia was still present in one of the three patients who had had this feature. All patients had abnormalities of esophageal motility. Long-term corticosteroid treatment was associated with aseptic bone necrosis in three patients and growth retardation in one. We conclude that the Kasukawa criteria are easy to apply to children, and that juvenile-onset mixed connective tissue disease has many similarities to the adult form of the disease.

Mononeuritis multiplex in a child with cutaneous polyarteritis.

A 14-year-old boy presented with a febrile illness associated with arthritis. Shortly later he developed mononeuritis multiplex. After certain typical skin lesions had developed after two months, the diagnosis cutaneous polyarteritis could be made. The diagnostic features of this benign disease, which may involve peripheral nerves, are discussed.

[Recognition of joint symptoms in childhood]. / (H)erkenning van gewrichtsklachten op de kinderleeftijd.

Joint complaints do occur frequently in childhood. Mostly the underlying cause is innocent and the complaints are mostly self-limiting. It is of the utmost importance to recognize diseases with joint involvement. The symptoms and differential diagnosis of arthritis in childhood are briefly discussed.

[Pauciarticular juvenile chronic arthritis]. / Pauciarticulaire juveniele chronische arthritis.

On basis of clinical and immunogenetic factors most children with pauciarticular juvenile chronic arthritis can be included in one of the subtypes: type 1 and type 2 pauciarticular JCA. Type 1 occurs in young children, mainly girls, with involvement of knees, ankles or elbows. In the majority of children antinuclear antibodies can be detected. The presence of these autoantibodies is associated with chronic anterior uveitis. Type 2 or the juvenile spondylarthropathies include morbus Bechterew, the reactive arthritides and arthritis associated with psoriasis and inflammatory bowel diseases. Large joints of the lower extremities are involved, back pain is unusual at onset, but enthesitis is frequently present. There is a strong association with HLA-B27. Treatment of both subsets consists of non-steroidal anti-inflammatory drugs, application of intra-articular steroids, physio- and hydrotherapy and splinting. In children with a polyarticular course of type 1, or a prolonged course of type 2 disease modifying drugs are often needed.

[A child with cutaneous polyarteritis]. / Een kind met cutane polyarteriitis.

A 14 year-old boy became seriously ill, following an upper respiratory tract infection, with high fever, arthralgia and arthritis, and a complete paralysis of the left median nerve. There was inadequate response to NSAIDS, but improvement with prednisone. During reduction of prednisone the boy developed painful erythematous swelling of the feet, subcutaneous nodules and afterwards livedo reticularis. Skinbiopsy confirmed the diagnosis of cutaneous polyarteritis. Different aspects of the disease are discussed.

Urinary excretion of prostaglandins during infancy and childhood: influence of age, sodium restriction and posture.

The influences of age, sodium restriction and posture on 24-hour urinary excretion of prostaglandin E2 (PGE2), prostaglandin F2 alpha (PGF 2 alpha), 6-keto-prostaglandin F1 alpha (6-keto-PGF 1 alpha) and thromboxane B2 (TXB2) were investigated in 111 healthy children and youngsters in the age between 1 day and 16 years. A considerable degree of variation was found in normal 24-hour urinary prostaglandin excretion in all age groups. There was no significant effect of age on the urinary excretion of prostaglandins when data were corrected for body surface area. In addition, sodium restriction and posture had no influence on the excretion of PGE2, PGF 2 alpha, 6-keto-PGF 1 alpha and TXB2. Our results indicate that in the first days of life the kidney already has the capacity to synthesize prostaglandins in amounts comparable to older children.

Acute transverse myelopathy as the initial manifestation of probable systemic lupus erythematosus in a child.

A 10-year-old girl was presented with acute transverse myelopathy. She had three mild relapses within one year. Systemic lupus erythematosus (SLE) was suspected on the basis of positive antinuclear antibodies (ANA), moderately decreased total hemolytic complement, antibodies to histone, immunological abnormalities of kidney and skin biopsy. Symptoms of SLE involving other organs were absent.