Hypermobility of the joints in juvenile episodic arthritis/arthralgia

It has been suggested that hypermobility of the joints may predispose children to the development of arthritis or arthralgia. To determine the normal frequency of hypermobility, 260 normal schoolchildren (5 to 17 years of age) were examined. In addition, 34 patients with juvenile rheumatoid arthritis (JRA) and 32 children with juvenile episodic arthritis/arthralgia (JEA) were tested. Any child who met at least three of the following criteria was considered to have joint hypermobility: (1) passive apposition of the thumbs to the flexor aspect of the forearms; (2) passive hyperextension of the fingers so that they lie parallel with the extensor aspect of the forearms; (3) hyperextension of the elbows greater than 10 degrees; (4) hyperextension of the knees greater than 10 degrees; (5) flexion of the trunk with knees extended so the palms rest on the floor. Thirty-two (12%) of 260 normal schoolchildren and 21 (66%) of 32 with JEA had hypermobility. Further, a significantly higher proportion (23 of 126) of normal girls than normal boys (nine of 134) had hypermobility (chi 2 = 8.0, P less than 0.005). Hypermobility was not common in children with JRA. These findings support the hypothesis that hypermobility may be an important factor in the cause of JEA.     

Prognosis of children with poststreptococcal reactive arthritis

Patients with Group A beta-hemolytic streptococcal infection and articular disease who do not fulfill the modified Jones criteria for a diagnosis of acute rheumatic fever (ARF) have been classified as poststreptococcal reactive arthritis/arthralgia. We reviewed the initial clinical characteristics and outcome of 12 poststreptococcal reactive arthritis/arthralgia patients. During the initial episode all had arthritis or arthralgia and a documented streptococcal infection. None had carditis and none received prophylactic antibiotic therapy during an average follow-up of 17 months (range, 6 to 42 months). One patient developed classic ARF with valvulitis 18 months after the initial episode. Two children had later episodes of arthritis and two had at least one additional episode of arthralgia. Poststreptococcal reactive arthritis/arthralgias seems to be part of the disease spectrum of ARF and therefore the use of prophylactic antibiotic therapy to prevent subsequent development of ARF and carditis in these patients should, perhaps, be reconsidered.     

Bone alterations in patients with idiopathic hypercalciuria and calcium nephrolithiasis

Objectives. To verify whether alterations in bone density and turnover in patients with calcium nephrolithiasis and hypercalciuria are observable in various subgroups of patients divided according to the pathogenesis of the hypercalciuria.Methods. Seventy patients with calcium nephrolithiasis and idiopathic hypercalciuria, 19 to 64 years old, were assessed for spine and femur mineral metabolism and bone density using a Dexa evaluation system. After a low calcium diet, the subjects were classified into two groups: fasting hypercalciuria (FH, 39 patients) and absorptive hypercalciuria (AH, 31 patients).Results. Only in the patients with FH was the lumbar spine bone density lower than in the controls (P <0.001). Also, only the patients with FH had higher bone alkaline phosphatase and urinary hydroxyproline levels than the control group (P <0.005 and <0.015, respectively). The blood pH levels were lower, even though within the normal range, in the hypercalciuric patients than in the controls (P <0.01). There was a negative correlation between the urinary hydroxyproline level and lumbar spine and femoral neck density in patients with FH (P <0.001 and <0.005, respectively), and the blood pH correlated positively with the lumbar spine bone density.Conclusions. Altered bone metabolism and overall bone loss were found only in the patients with FH. Overloading of acid valences, perhaps of dietary origin, could be the pathogenic factor responsible.     

Immunophenotype of pleomorphic xanthoastrocytoma

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor, often seizure-associated and occurring in the temporal lobe of young adults. Although its cells are considered astrocytic in nature, recent studies suggest the presence of neuronal differentiation and a possible relationship to glioneuronal neoplasms. We immunostained 40 cases of PXA, including two composite PXA-gangliogliomas (PXA-GG), with a panel of glial (glial fibrillary acidic protein, S-100 protein) and neuronal markers (class III beta-tubulin, synaptophysin, neurofilament proteins, MAP2, and chromogranin A). Conventional PXAs demonstrated immunoreactivity for glial fibrillary acidic protein (100% of cases), S-100 protein (100%), class III beta-tubulin (73%), synaptophysin (38%), NF proteins (18 and 8%), and MAP2 (8%). Chromogranin A stain was absent in all conventional PXA cases. Neoplastic ganglion cells in both PXA-GGs stained with class III beta-tubulin, synaptophysin, and chromogranin A. Ultrastructural studies, performed in nine cases, demonstrated neuronal features including microtubules, dense core granules, and/or clear vesicles largely limited to cell processes (two PXAs) and in the cytoplasm (PXA component of one PXA-GG). Although the essential nature of PXA is clearly and uniformly glial, the significance of the limited neuronal differentiation is unclear, as it is the relationship between conventional PXA and PXA-GG. We found no evidence that the former is a precursor of the latter.     

Nociceptive sensitization by the secretory protein Bv8

1 The small protein Bv8, isolated from amphibian skin, belongs to a novel family of secretory proteins (Bv8-Prokineticin family, SWISS-PROT: Q9PW66) whose orthologues have been conserved throughout evolution, from invertebrates to humans. 2 When injected intravenously or subcutaneously (from 0.06 to 500 pmol kg(-1)) or intrathecally (from 6 fmol to 250 pmol) in rats, Bv8 produced an intense systemic nociceptive sensitization to mechanical and thermal stimuli applied to the tail and paws. 3 Topically delivered into one rat paw, 50 fmol of Bv8 decreased by 50% the nociceptive threshold to pressure in the injected paw without affecting the threshold in the contralateral paw. 4 The two G-protein coupled prokineticin receptors, PK-R1 and PK-R2, were expressed in rat dorsal root ganglia (DRG) and in dorsal quadrants of spinal cord (DSC) and bound Bv8 and the mammalian orthologue, EG-VEGF, with high affinity. In DSC, PK-R1 was more abundant than PK-R2, whereas both receptors were equally expressed in DRG. IC(50) of Bv8 and EG-VEGF to inhibit [(125)I]-Bv8 binding to rat DRG and DSC were 4.1+/-0.4 nM Bv8 and 76.4+/-7.6 nM EG-VEGF, in DRG; 7.3+/-0.9 nM Bv8 and 330+/-41 nM EG-VEGF, in DSC. 5 In the small diameter neurons (<30 microm) of rat DRG cultures, Bv8 concentrations, ranging from 0.2 to 10 nM, raised [Ca(2+)](i) in a dose-dependent manner. 6 These data suggest that Bv8, through binding to PK receptors of DSC and primary sensitive neurons, results in intense sensitization of peripheral nociceptors to thermal and mechanical stimuli.     

Inter-observer reproducibility and responsiveness of a clinical severity scale in surgically treated carpal tunnel syndrome

OBJECTIVES: To test a recently proposed carpal tunnel syndrome (CTS) clinical severity scale for reproducibility between two observers (neurosurgeon and neurophysiologist) before surgery, for responsiveness to changes in clinical status 6 months after surgery, and for correlations with the electrophysiological findings and 'Boston Carpal Tunnel Syndrome Questionnaire' (BQ). MATERIAL AND METHODS: The tests were applied prospectively to a consecutive series of 254 hands with idiopathic CTS, referred for surgical decompression. The hands belonged to 219 subjects (177 women and 42 men, mean age 55.6). RESULTS: Percentage agreement between the two observers in assigning severity to the same class was 78% and Cohen coefficient kappa was 0.69 (P < 0.001). The scale was found to be responsive to changes in clinical status after surgery. Direct correlations were also found between the scale and patient age, duration of symptoms, BQ scores and the neurophysiological severity scale. The significance of these associations was maintained for 6 months after the operation. CONCLUSION: This clinical severity scale is simple, reproducible and sensitive for evaluating severity of CTS in patients undergoing surgery.     

Magnesium oxide augmentation of verapamil maintenance therapy in mania

The authors compared the antimanic effects of a verapamil-magnesium oxide (V-M) combination with a verapamil-placebo combination (V-P) in patients pretreated with verapamil. BPRS scores and serum magnesium levels were compared. The V-M combination was found to be significantly more effective than V-P in reducing manic symptoms (P=0.015). Serum magnesium levels were significantly higher in the V-M group (P<0.04). These data suggest that magnesium may increase antimanic efficacy of verapamil by mechanisms which may operate at the intracellular level. The magnesium-verapamil combination may have clinical application as an adjunct to verapamil in the maintenance therapy of mania.     

Variable expression of familial Alzheimer disease associated with presenilin 2 mutation M239I

In a family with autopsy-confirmed Alzheimer disease, the authors found a mutation in the presenilin 2 (PS2) gene (PSEN2) that predicts a methionine-to-isoleucine change at PS2 residue 239 (M239I), at which a change to valine was known in another family. Phenotypic expression of M239I was highly variable, with disease onset between age 44 and 58 years, and two nonaffected mutation carriers at age 58 and 68 years. The data showed no influence of APOE but were compatible with other possible genetic modifiers of the phenotype or penetrance of M239I.