Dermatomyositis in a patient with high grade papillary urothelial carcinoma of urinary bladder

Inflammatory myopathies like dermatomyositis are associated with increased incidence of malignancies. This association has been commonly reported with malignancies of ovaries, gastro‐intestinal tract, breast and non‐Hodgkin's lymphomas but occurrence of dermatomyositis with bladder cancer has been rarely reported. We report a patient with carcinoma bladder who developed dermatomyositis while being treated for the bladder cancer.     

Disturbed myelinogenesis and recovery in hyperphenylalaninemia in rats: An immunohistochemical study

Chronic hyperphenylalaninemia (HPA) in rats has been used as an experimental model of the human inborn error of metabolism phenylketonuria (PKU). Impaired brain development in PKU and HPA is reflected in reduced myelin formation. We have used immunohistochemistry, with antibodies to cell-specific antigenic markers, to investigate the cellular basis of the hypomyelination in the corpus callosum and cerebral cortex of rats made hyperphenylalaninemic from Postnatal Days 3-17. The rats were then allowed to recover until Day 59. No effects were seen on the number and differentiation pattern of ganglioside GD3-expressing glial progenitors. Myelin basic protein and 2'3'-cyclic nucleotide 3'-phosphohydrolase (CNP) immunostaining demonstrated a reduction in myelin formation in the corpus callosum and subcortical white matter at 12 and 17 days postnatal. However, numbers of CNP+ oligodendrocytes appeared normal throughout development. No reactive astrogliosis was seen at any stage. The intensity of axonal neurofilament immunostaining was reduced in the corpus callosum at 17 days. In layers II and III of the cortical gray matter there was an increase in the cell packing density and a concomitant decrease in cell body size. Myelination in the corpus callosum was rapid during the recovery period with no difference noted at Day 59. Axonal neurofilament staining also returned to normal in the corpus callosum. However, recovery became increasingly incomplete away from the corpus callosum into the cortical gray matter. Our data suggest a primary effect of HPA on axonal maturation with hypomyelination consequential upon this.     

Heterogeneity in maple syrup urine disease: Aspects of cofactor requirement and complementation in cultured fibroblasts

Fibroblast strains derived from six patients with maple syrup urine disease have been investigated for their requirements of the cofactors NAD, CoASH, Mg⁺⁺ and TPP in comparison with 10 normal control strains. The reconstitution of the decarboxylase function of branched chain α-keto acid (BCKA) dehydrogenase complex in lysed cells was studied with respect to the substrates u-keto-isocaproic acid, α-keto-isovaleric acid, and α-keto-β-methylvaleric acid (KIC, KIVA, MEVA). The enzyme activity of all normal control strains for the substrates KIC and KIVA was not reconstituted by TPP + Mg⁺⁺ alone, but CoASH + NAD could reconstitute the enzyme activity with KIC and KIVA in different degrees. Only two control strains were tested with MEVA as substrate, and these showed in contrast that TPP + Mg⁺⁺ could partly reconstitute the enzyme activity. In contrast to the relative homogeneiy in the reconstitution profiles of normal strains, the five classical and one intermittent MSUD strains showed heterogeneity in cofactor requirements.
Complementation analysis using heterokaryons prepared from fibroblasts of four patients with classical MSUD and one patient with intermittent MSUD showed, in contrast to experiments with normal controls, a partial amelioration of the defect in two combinations; it is suggested that the defect in these strains is located at different functional subunits of the multienzyme complex.     

Comparing 3 Suture Techniques After Muscle Laceration Repair

Background: Skeletal muscle lacerations are a relatively common injury. Compared with nonrepaired lacerations, surgically repaired muscle lacerations regenerate faster, develop less scar tissue, have a higher return to baseline strength, and have lower incidence of hematomas. Despite the benefits of repair, the optimal repair technique is still unknown. The purpose of this study was to examine the biomechanical properties of common muscle repair techniques to determine the optimal repair. Methods: Forty-two fusiform porcine muscle specimens were dissected and used for this study. Three suture techniques were used for comparative analysis: Figure-eight, Mason Allen, and Perimeter. Each muscle was transected and then repaired using one of the 3 techniques. Fourteen muscle-tendon specimens were prepared for each group and tested for tensile failure using a material testing system. Biomechanical properties, including peak failure point and stiffness, were compared for differences between the suture groups by 1-way analysis of variance. The average time per repair technique was also recorded. Results: The Perimeter technique showed a statistically significant higher peak failure point than the Mason Allen technique (P = .03). Both the Figure-eight (P = .047) and Perimeter techniques (P < .001) were significantly stiffer than the Mason Allen technique. The repair time was comparable across all 3 techniques. Conclusions: The Figure-eight and Perimeter repairs were found to be similar in peak failure point and stiffness, whereas the Mason Allen technique showed significantly lower stiffness and peak failure point. The Figure-eight was the quickest repair to perform. The Figure-eight technique may be strongly considered for muscle laceration repairs due to its simplicity and efficiency.     

Juvenile dermatomyositis associated with autoantibodies to small ubiquitin-like modifier activating enzyme: a report of 4 cases from North India and a review of literature

Immunologic Research - Juvenile dermatomyositis (JDM) is the commonest inflammatory myositis in children. The clinical phenotype is often characterized by the presence of myositis-specific...     

Levels of Interleukin‐18 and Endothelin‐1 in Children with Henoch‐Schönlein Purpura: A Study from Northern India

Henoch‐Schönlein purpura (HSP) is an acute systemic vasculitis with unknown etiology, although several studies have found HSP to be related to cytokines such as tumor necrosis factor α, interleukin (IL)‐1, and adhesion molecules. In the present study we determined the levels of cytokines such as IL‐18 and endothelin‐1 (ET‐1) in children with HSP. Subjects were divided into three groups (group 1, 20 subjects with HSP; group 2, 10 subjects belonging to group 1 during their follow‐up 4 to 6 months later; and group 3, 16 controls who were healthy siblings of the subjects). IL‐18 and ET‐1 levels were determined using enzyme immunoassay and expressed as mean ± standard deviation. We observed higher IL‐18 levels in children with HSP (767.6 ± 145.1 pg/mL) than in controls (614.6 ± 66.54 pg/mL, p > 0.05), but IL‐18 levels were found to be significantly lower in subjects with HSP in remission (502.7 ± 60.81 pg/mL) than in those who were in an active phase (1,050 ± 244.5 pg/mL, p < 0.05, n = 10). ET‐1 levels were found to be significantly higher in subjects with HSP (1.93 ± 0.19 pg/mL) than in controls (1.10 ± 0.13 pg/mL, p < 0.05), although no significant difference was observed in ET‐1 levels between subjects in group 1 (1.88 ± 0.30 pg/mL) and group 2 (1.91 ± 0.120, p > 0.05, n = 10). A positive correlation was observed between IL‐18 and ET‐1 levels in subjects with HSP (correlation coefficient [r] = 0.5254, p < 0.01). These results suggest that levels of IL‐18 and ET‐1 are worth monitoring during the clinical course of the disease, but caution must be exercised in extrapolating data based on small study samples.     

Fifty years of Kawasaki disease–a tribute to Dr Tomisaku Kawasaki

It has been 50 years since the legendary Japanese pediatrician, Dr Tomisaku Kawasaki, published his classic paper in 1967. Little was he to know at that time that this condition would not only be known after his name but would also become the commonest cause of acquired heart disease in children in most of the developed world. The etiology of this condition continues to remain an enigma, and the diagnosis is still based on a set of criteria that are entirely clinical. All pediatricians must be familiar with the various clinical presentations of this disease because delays in diagnosis and treatment can have disastrous consequences.