1/319. hypersensitivity reaction in a child due to lamotrigine.Lamotrigine is an anticonvulsant with a broad spectrum of activity that has been approved in the united states for use in adults with either partial or generalized seizures. This drug is being widely prescribed by pediatricians and neurologists because it is effective in children with idiopathic, resistant, generalized seizures and does not impair cognition. As with other anticonvulsants, a hypersensitivity syndrome has been described. Anticonvulsant hypersensitivity syndrome consists of the hallmark features of fever, rash, and lymphadenopathy. We report the first case of hypersensitivity syndrome in a child due to lamotrigine in which we believe the coadministration of valproic acid increased the duration of the reaction. Our patient had a high spiking fever, generalized morbilliform eruption, facial edema, lymphadenopathy, eosinophilia, atypical lymphocytosis, and an elevation in his liver function tests. The syndrome resolved with the discontinuation of the medication. Anticonvulsant hypersensitivity syndrome may occur with the administration of lamotrigine. Variable presentations may be seen, as hypersensitivity syndromes may be multisystem in nature. The prompt recognition of the signs and symptoms of this condition allows an accurate diagnosis so that the drug may be discontinued and other anticonvulsant treatment options instituted.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
2/319. Riedel's thyroiditis.Riedel's thyroiditis is a rare chronic inflammatory disease of unknown aetiology and may be one manifestation of multifocal fibrosis. Four patients were seen over a period of 12 years and unusual presentations in 3 of them caused difficulty in diagnosis. The aetiology and methods of treatment are discussed.- - - - - - - - - - ranking = 0.76261715351262keywords = inflammatory disease (Clic here for more details about this article) |
3/319. Anticonvulsant hypersensitivity syndrome occurring as sepsis with multiorgan dysfunction.phenytoin is a highly effective and widely prescribed anticonvulsant agent. However, it is associated with both dose-related side effects and hypersensitivity reactions. life-threatening anticonvulsant hypersensitivity syndrome in one patient was characterized by a skin eruption and multisystem organ dysfunction.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
4/319. Crescentic glomerulonephritis in hyper IgD syndrome.The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS.- - - - - - - - - - ranking = 0.76261715351262keywords = inflammatory disease (Clic here for more details about this article) |
5/319. Periventricular brain heterotopias in a child with adrenocortical insufficiency, achalasia, alacrima, and neurologic abnormalities (Allgrove syndrome).We describe a previously unreported finding of periventricular heterotopias in a brain magnetic resonance imaging (MRI) study, in a girl with adrenocortical insufficiency, alacrima, achalasia, and neurologic deterioration (Allgrove syndrome). This finding could indicate that the underlying mechanism in this syndrome can be traced to the first half of fetal life and also might cause abnormal neuronal migration. This disorder recently has been linked to chromosome 12q13. There could be several explanations for the clinical heterogeneity in this syndrome: a contiguous gene syndrome involving multiple genes, including one whose deletion causes heterotopias, or a deficiency of a gene for a neurotrophic factor active during pre- and postnatal life and responsible for both migration and survival of neurons could be the cause. The identification of the responsible gene(s) will lead to further understanding of this multisystem disorder.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
6/319. Cogan's syndrome with refractory abdominal aortitis and mesenteric vasculitis.Cogan's syndrome is a rare multisystem disease characterized by ocular inflammation, vestibuloauditory dysfunction, and vasculitis. We report a 26-year-old Caucasian woman who died from Cogan's syndrome. Her case illustrates that patients with Cogan's syndrome can have abdominal aortitis and mesenteric vasculitis, and that the vasculitis can be refractory to methotrexate, cyclophosphamide, cyclosporine, and chlorambucil.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
7/319. Fatal group A Streptococcal toxic shock-like syndrome in a child with varicella: report of the first well documented case with detection of the genetic sequences that code for exotoxins spe A and B, in Sao Paulo, brazil.A previously healthy seven-year-old boy was admitted to the intensive care unit because of toxaemia associated with varicella. He rapidly developed shock and multisystem organ failure associated with the appearance of a deep-seated soft tissue infection and, despite aggressive treatment, died on hospital day 4. An M-non-typable, spe A and spe B positive Group A streptococcus was cultured from a deep soft tissue aspirate. The criteria for defining Streptococcal toxic shock-like syndrome were fulfilled. The authors discuss the clinical and pathophysiological aspects of this disease as well as some unusual clinical findings related to this case.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
8/319. pregnancy in bone marrow failure syndromes: diamond-Blackfan anaemia and Shwachman-diamond syndrome.pregnancy in bone marrow failure syndromes has risk to mother and fetus. There are fewer than 30 reports of cases with diamond-Blackfan anaemia (DBA), and none with Shwachman-diamond syndrome (SD). We report two DBA and one SD cases. One DBA mother received transfusions intra-partum, and the other only post-partum. Both required caesarean sections (C-sections) for failure of labour to progress and severe pre-eclampsia respectively. Both subsequently resumed pre-pregnancy steroid-induced control of anaemia. approximately 40% of DBA pregnancies required maternal transfusions; 25% delivered by C-section. The SD patient also had Ehlers-Danlos (ED) syndrome and urticaria pigmentosa (UP). Her blood counts were adequate until week 38, when the platelet count dropped and a C-section was performed. pregnancy management in marrow failure disorders requires obstetricians with expertise in high-risk pregnancies, and haematologists with experience with marrow failure syndromes.- - - - - - - - - - ranking = 0.044667700747006keywords = urticaria (Clic here for more details about this article) |
9/319. Angiographic manifestions of regional enteritis.Regional enteritis may be a protean disease, easily diagnosed when the common clinical presentation is noted. Occasionally, however, "atypical" features lead to the clinical suspicion of neoplastic disease. Four patients underwent angiographic evaluation in this clinical setting, all demonstrating similar changes in the mesenteric arteries. A serrated, "corkscrew" pattern was present and corresponded well to the perivascular fibrosis noted histologically. Recognition of this angiographic pattern may be important in differentiating neoplastic from inflammatory disease processes.- - - - - - - - - - ranking = 0.76261715351262keywords = inflammatory disease (Clic here for more details about this article) |
10/319. Surgical treatment of choanal atresia in CHARGE association: case report with long-term follow-up.A patient affected by a multisystem malformation, the CHARGE association, is described. The choanal atresia is one of the stigmata characterizing this syndrome. The main anomalies of the association, the surgical correction (choanoplasty with endoscopic transnasal approach followed by the application of nasal stenting) and a comparison with different techniques are reported.- - - - - - - - - - ranking = 1keywords = multisystem (Clic here for more details about this article) |
| | Next -> |