Patch testing in systemic drug eruptions

CrossLaps peptide [Glu-Lys-Ala-His-Asp-Gly-Gly-Arg], a part of the C-telopeptide of the alpha 1-chain of type I collagen of bone, is a recently developed biochemical marker of bone turnover. In this study, the clinical utility of measurement of urinary CrossLaps was investigated in eleven premenopausal women who received a gonadotropin-releasing hormone (GnRH) agonist for 6 months for treatment of adenomyosis (n = 1) or leiomyomas (n = 10). Along with urinary CrossLaps, the levels of various biochemical markers, and serum estradiol, calcitonin and intact parathyroid hormone (i-PTH) were measured, and lumbar spine bone mineral density (BMD) was also monitored before, during, and at the end of the course of GnRH agonist therapy. Apart from CrossLaps, markers of bone resorption tested were urinary pyridinoline, deoxypyridinoline and hydroxyproline. Markers of bone formation tested were serum osteocalcin and bone-specific alkaline phosphatase (B-ALP). Serum estradiol levels decreased to undetectable levels at 2 months of GnRH agonist therapy. The values for all biochemical markers increased significantly throughout the therapy. The degree of an increase in CrossLaps levels was greater than that in all other markers. Mean lumbar spine (L2-L4) BMD was decreased by 7.2% at 6 months of treatment. The percent change in BMD at 6 months of treatment correlated inversely with the percent change in CrossLaps levels from the baseline to 1, 2, and 5 months of treatment. These results indicate that measurement of urinary CrossLaps might be a useful tool to predict the risk of bone loss caused by hypoestrogenism including GnRH agonist therapy.     

Salivary gland involvement in systemic diseases

The salivary glands participate in systemic illnesses because they are secretory structures allied with the gastrointestinal tract. Thus they are involved in systemic inflammatory, allergic, and neoplastic conditions. Perhaps the most common condition is that of mumps in the acute phase. Of the chronic diseases, Mikulicz's disease or Sj?gren's syndrome is most frequently noted. In most instances of systemic involvement, the salivary gland enlargement is the rule, with the gland being tense, soft, and enlarged, and with progressive growth over a period of time. Usually all glands are involved. The diagnosis should be suspected from the history and the physical examination, noting that all the glands are equally involved by the enlargement. If there is any question, a biopsy should be performed. In the case of Sj?gren's syndrome, the biopsy specimen may be taken either from a major salivary gland or from one of the minor ones, or the nasal mucous membrane or oral cavity. In general, definitive therapy is unsuccessful for the systemic illness involving the salivary glands, and in most instances has to be supportive.     

Oral manifestations of systemic diseases

Many systemic diseases have oral manifestations. The oral cavity might well be thought of as the window to the body because oral manifestations accompany many systemic diseases. These oral manifestations must be properly recognized if the patient is to receive appropriate diagnosis and referral for treatment. We have reviewed a series of recent articles and summarized known and newly described oral manifestations of several systemic diseases. The lesions of the oral mucosa, tongue, gingiva, dentition, periodontium, salivary glands, facial skeleton, extraoral skin and other related structures caused by some of the more common systemic diseases are highlighted.     

Cutaneous manifestations of systemic diseases

Many aspects of dermatologic diagnosis are either of importance or interest to the nondermatologist, and there are many excellent textbooks available for guidance. This article focuses on four categories of conditions that are the source of frequent queries from the primary care setting: (1) common skin diseases that frequently mimic systemic illness, (2) common skin diseases that have important systemic associations, (3) common systemic diseases that have prominent cutaneous findings, and (4) the seldom seen but frequently raised concerns regarding cutaneous signs of internal malignancy.     

Febrile episodes in children with cancer in the United Arab Emirates

We report the first patient with a partial trisomy and a partial monosomy of the long arm of chromosome 4: 46,XY, inv dup(4)(pter-->q32::q32-->q26), del(4)(q32-->qter). The boy died from a complex cardiac defect (monoventricle, monoatrium and truncus arteriosus) in combination with a diaphragmatic hernia. In addition he had preaxial polydactyly of the right hand. We compare the clinical features with data from the literature. The phenotype of the patient mainly resembles that in patients with a terminal deletion 4q32.     

Relationships between chronic oral infectious diseases and systemic diseases

Contact-inhibited catalase-deficient fibroblast cell strain has been established from the homozygous hypocatalasemic C3H/Csb mutant mouse. This cell strain has low level of catalase enzyme activity and has normal level of enzyme activities of both glutathione peroxidase and superoxide dismutase. Catalase-deficient C3H/Csb mutant cell strain is markedly more sensitive to the toxicity of hydrogen peroxide compared to wild-type C3H/Csa cell strain. In addition, mutant cell strain is sensitive to X-rays and near-UV compared to wild-type cell strain, but shows the same sensitivities to topoisomerase II inhibitors, adriamycin and 4'-(9-acridinylamino) methanesulfon-m-anisidide (m-AMSA), and the DNA cross-linking agents, cisdiamminedichloroplatinum (II) (cis-Pt) and trans-diamminedichloroplatinum (II) (trans-Pt). These cell strains will be of use in the study of the roles which catalase plays in the intracellular prevention of DNA damage induced by oxidative stress.     

Cardiovascular manifestations of systemic autoimmune diseases

The systemic autoimmune diseases are a protean group of illnesses that primarily affect the joints, muscles, and connective tissue. All aspects of the cardiovascular system can be involved with clinical consequences ranging from asymptomatic abnormalities to serious life-threatening conditions. This article discusses the cardiovascular manifestations of the systemic autoimmune diseases with particular focus on clinical pathophysiology and management.     

Sports in patients with systemic inflammatory musculoskeletal diseases

A 73-year old woman consulted our hospital for consciousness disorder and was hospitalized under a diagnosis of cerebral infarction. On admission, retention of a large volume of ascites was noted. There were also marked increases in tumor markers. Serum CEA, CA19-9 and CA125 levels were 871.9 ng/ml, 1048.2U/ml, and 444.7U/ml, respectively. Cytological examination of the ascites showed class V. Abdominal CT showed a mass measuring 4 cm in diameter and dilatation of the small intestinal tract. During detailed examination, she developed ileus. Fluoroscopy through the lung tube suggested a small intestinal tumor. Laparotomy was performed to relieve ileus. In the abdominal cavity, the greater omentum formed a mass due to dissemination. Tumor was found in the jejunum approximately 60 cm from Treitz's ligament toward the anal side. Omentectomy and partial jejunectomy were performed. Macroscopic findings showed tumor protruding from the mucosal surface. Histological diagnosis was mucinous adenocarcinoma. Primary small intestinal cancer is very rare. In particular, only one case of mucinous adenocarcinoma of the small intestine was reported in Japan. We reported the second case in Japan with a review of the literature.     

Lung diseases in HIV-infected children

Pulmonary diseases, including opportunistic and bacterial infections, lymphoproliferative disorders, and neoplasia, substantially contribute to morbidity and mortality in HIV-infected children. Current treatment modalities are largely successful and thus justify invasive aetiological diagnostic procedures.     

Immunological liver diseases in children

Pristane induces a lupus-like syndrome characterized by autoantibody production and glomerulonephritis in nonautoimmune strains of mice. Although it has been suggested that this syndrome results from nonspecific immune activation, there is little evidence so far that B cells are activated nonspecifically by pristane or that this promotes autoimmunity. In this study, we examined whether polyclonal hypergammaglobulinemia occurs in pristane-induced lupus, and its relationship to the production of anti-DNA, nRNP/Sm, and Su autoantibodies. In conventionally housed mice, there was a marked increase in total IgM and IgG3 2 weeks after i.p. pristane injection, followed by increased IgG1, IgG2a, and IgG2b levels. IgM levels were higher in pristane-treated specific pathogen-free (SPF) mice than in conventionally housed mice, whereas IgG and IgA levels were reduced. Pristane induced anti-nRNP/Sm and Su autoantibodies in SPF mice, but their onset was delayed and levels were lower than those in conventionally housed mice. There was no consistent relationship between total IgG1, 2a, and 2b hypergammaglobulinemia and production of anti-nRNP/Sm and Su autoantibodies. Moreover, the total Ig levels were similar in the anti-nRNP/Sm-positive and -negative groups. In contrast, production of IgM anti-ssDNA antibodies paralleled IgM hypergammaglobulinemia in some, but not all, mice. These studies indicate that pristane-induced lupus is associated with marked hypergammaglobulinemia, the magnitude of which is influenced by the microbial environment. However, anti-nRNP/Sm and Su autoantibody production is at least partly independent of polyclonal B cell activation. The data strongly suggest that pristane-induced lupus is not exclusively the consequence of nonspecific immune stimulation. They also point to the importance of microbial stimulation in the development of hypergammaglobulinemia in this inducible lupus model.     

Nephritis in systemic lupus erythematosus in children

Twenty-five patients, aged six to 18 years, with lupus nephritis, followed for two to 103 months (median 22 months), have been classified according to renal histology into groups with diffuse proliferative glomerulonephritis (18), focal proliferative nephritis (2), and membranous nephropathy (5). Correlations have been made among specific histologic groups, clinical findings, renal function studies, urinary findings, and response to therapy. In sequential renal biopsies, lesions progressed in most patients with diffuse proliferative and membranous changes; however, chronic renal failure occurred in only one patient and the five-year survival rate (60.9%) is better than previously reported in pediatric patients.     

Perimenstrual eruptions

PURPOSE: To study the safety and feasibility of dobutamine-atropine stress echocardiography (DASE) in patients with known or suspected coronary artery disease. METHODS: There were 3,000 DASE that were studied in a prospective fashion. All symptoms and side effects were stored in a data base format. RESULTS: Major test-related complications observed included one case of myocardial infarction, four cases of sustained ventricular tachycardia and five cases of atropine intoxication. There was no death or ventricular fibrillation as a direct or indirect consequence of the test. The infusion protocol allowed to us to examine patients using beta blockers, and led to 95% feasibility. CONCLUSION: DASE is a safe and feasible method for the study of patients with suspected or known coronary artery disease.     

Angiitis of the central nervous system in systemic diseases

PURPOSE: This review is aimed at presenting classification and diagnosis criteria of isolated central nervous system (CNS) angiitis, and at proposing guidelines for diagnosis and management of this disease. CURRENT KNOWLEDGE AND KEY POINTS: Isolated CNS angiitis are rare and most information has been provided by studies of very small series. Angiitis can be primitive or secondary to infectious, neoplastic diseases, or toxics. Clinical manifestations and radiologic abnormalities are not specific. A brain biopsy is therefore often required to confirm the diagnosis, as numerous non-inflammatory vascular diseases can mimic both clinically and radiologically isolated CNS angiitis. PERSPECTIVES AND PROJECTS: To help guide the diagnosis and therapeutical management of patients with CNS angiitis, strict classification criteria should be used: 1) rule out the various diseases that can mimic clinical and radiological CNS aspects related to isolated angiitis and differentiate "isolated CNS angiitis" from "CNS angiitis associated with systemic diseases"; 2) search for factors associated with the development of a "secondary CNS angiitis"; 3) check presumed mechanism at the origin of the cerebral vascular disease: "angiitis" versus "angiopathy"; 4) if the diagnosis of "primary CNS angiitis" is still suspected, it seems reasonable to perform cerebral and leptomeningeal biopsies. Treatment is still unknown and has to be discussed on a case by case basis according to the severity and progression of symptoms.     

Radiologic manifestations of the systemic autoimmune diseases

Advances in thoracic imaging during the past two decades, such as CT scans and MR imaging, have enhanced our understanding of the pleuropulmonary abnormalities that develop in the systemic autoimmune diseases. In this article, the thoracic radiologic manifestations of several connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis, Sj?gren's syndrome, polymyositis/dermatomyositis, progressive systemic sclerosis, and anklyosing spondylitis), two granulomatous vasculitides, (Wegener's Granulomatosis and Churg-Strauss syndrome), and antiglomerular basement membrane disease are reviewed.     

The mechanism of autoantibody production in systemic autoimmune diseases

The appearance of autoantibodies to nuclear autoantigens is the hallmark of systemic autoimmune diseases. The mechanism of the autoantibody production has remained elusive, though it is generally accepted that autoantigen-specific T cells drive the production. These autoantigen-specific T cells as well as autoreactive B cells can be found in peripheral blood from healthy people, suggesting that these autoreactive cells are regulated peripherally. In this review dealing with autoantibody production mechanism, the brief introduction of our approach employing mouse genetics is also included.