1/26. Henoch-Schonlein purpura and stroke: antiphosphatidylethanolamine antibody in CSF and serum.A 15-year-old girl with features of Henoch-Schonlein purpura and brain infarct had a transient IgA antiphosphatidylethanolamine antibody (aPE) in her serum and CSF that disappeared 5 months after presentation. serum aPE is known to be associated with thrombotic events. The authors found no aPE in the CSF of two control individuals or in the serum of two patients with active Henoch-Schonlein purpura without neurologic involvement. The patient may represent a variant of antiphospholipid antibody syndrome.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/26. Cerebral vasculitis in Henoch-Schonlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone.Neurological complications are rare during the course of Henoch-Schonlein purpura (HSP). We report a 7-year-old girl with HSP who presented with seizures, loss of vision and disturbance of consciousness. magnetic resonance imaging (MRI) showed high signal intensity in the gray and white matter over the left parietal and both occipital lobes, compatible with MRI findings of cerebral vasculitis. The eye fundi revealed multiple branches of retinal artery occlusion. Intravenous pulse methylprednisolone (MTP) followed by oral steroid therapy was initially administered for HSP nephritis. Cerebral vasculitis developed 10 days post-MTP treatment, with progressive worsening of consciousness. Oral steroid was discontinued and plasmapheresis was performed alone. Her level of consciousness dramatically improved after plasmapheresis. The brain MRI and eye fundi findings were consistent with her clinical improvement. To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP.- - - - - - - - - - ranking = 1235.7688121599keywords = cerebral, brain (Clic here for more details about this article) |
3/26. Pulmonary haemorrhage in a 6-year-old boy with Henoch-Schonlein purpura.Henoch-Schonlein purpura (HSP) is the most common vasculitis in children. It is a multisystemic disease but pulmonary haemorrhage is extremely rare. We present the case of a 6-year-old boy with Henoch-Schonlein purpura, pulmonary haemorrhage and severe renal involvement. The patient responded to a combination of intravenous methylprednisolone and cyclophosphamide. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment. Renal biopsy is helpful in the differential diagnosis of HSP-mimicking pulmonary vasculitic syndromes. Combining cyclophosphamide with glucocorticoids may improve the outcome in severe HSP cases with pulmonary haemorrhage.- - - - - - - - - - ranking = 112529.84764705keywords = haemorrhage (Clic here for more details about this article) |
4/26. Henoch-Schonlein purpura with intracerebral hemorrhage.We describe a case of Henoch-Schonlein purpura (HSP) with massive intracerebral hemorrhage (ICH) in a 7-year-old-girl. A cranial CT scan revealed extensive ICH in the left parietal region and right parieto-temporal through occipital regions. At the time of ICH onset, hypertension and coagulation abnormality were not observed, but factor xiii activity was markedly reduced 9%. ICH was thought to have resulted from a marked decrease in factor xiii. factor xiii preparation was administered immediately after the onset of ICH, and enlargement of the hemorrhagic region was not seen. The present case is the only reported case of HSP complicated by ICH in which factor xiii level was measured.- - - - - - - - - - ranking = 5746.2264131273keywords = intracerebral, cerebral (Clic here for more details about this article) |
5/26. cerebral hemorrhage in Henoch-Schoenlein syndrome.BACKGROUND: Henoch-Schoenlein syndrome (HSS) is the most common form of vasculitis seen in childhood. The clinical diagnosis is based on the association of nonthrombocytopenic purpura, arthritis and abdominal pain. Nephropathy is the most common complication. Hemorrhages can occur in the respiratory, gastrointestinal and urinary tracts. Neurological complications are rare, though they may be particularly severe. CASE REPORT AND DISCUSSION: Intracranial hemorrhage is an extremely rare complication of the disease; we report the case of a child with cerebral hemorrhage in HSS and review the literature.- - - - - - - - - - ranking = 411.58960405329keywords = cerebral (Clic here for more details about this article) |
6/26. Homozygous protein c deficiency with purpura fulminans: report of a new case and a description of a novel mutation.We report here a quite rare case of severe homozygous protein c deficiency. The index case is a 9-month-old Saudi boy who was born after an uneventful pregnancy at 39 weeks. The diagnosis of epidermoloysis bullosa and the appearance of scrotal haematoma raised the diagnosis of thrombosis due to protein c deficiency. The clinical presentation and extremely low level of protein C activity (< 0.01 U/ml) in the index case suggested a severe case of protein c deficiency. The father is 28 years old and the mother is 25 years old and are consanguineous. Neither had a personal or family history of thrombosis. Genomic dna was extracted from peripheral blood of the patient and both parents, exons of protein C were amplified by polymerase chain reaction and sequenced. Sequencing revealed the presence of a novel CCTG duplicate nucleotide (effective insertion) after nucleotide 8826 in exon 9 of the protein C gene. This insertion is found in the homozygous state in the patient and in the heterozygous state in both parents. It results in a frame-shift mutation, which introduces a stop-codon, thereby generating a prematurely truncated protein. These molecular findings agree with the presence of quantitative protein c deficiency in the index case.- - - - - - - - - - ranking = 30.830383019537keywords = haematoma (Clic here for more details about this article) |
7/26. Successful treatment of mesenteric vasculitis caused by Henoch-Schonlein purpura with methylprednisolone pulse therapy.Although mesenteric vasculitis due to Henoch-Schonlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.- - - - - - - - - - ranking = 16075.692521007keywords = haemorrhage (Clic here for more details about this article) |
8/26. adult onset anaphylactoid purpura with severe gastrointestinal involvement.We treated a case of adult-onset severe-form anaphylactoid purpura. This case had been diagnosed as anaphylactoid purpura pathologically by skin biopsy. However, his clinical manifestations were atypical in view of complicated massive gastrointestinal hemorrhage. Intensive therapy including corticosteroid and cyclophosphamide transiently improved his condition. Nevertheless, the beta-D-glucan value in peripheral blood was gradually elevated. Prophylactic use of fluconazole failed to prevent contraction of invasive mycosis. Finally, he suddenly suffered from diffuse cerebral hemorrhage. Postmortem examination revealed systemic invasive aspergillosis as the cause of death.- - - - - - - - - - ranking = 411.58960405329keywords = cerebral (Clic here for more details about this article) |
9/26. Henoch-Schonlein syndrome and cerebellar hemorrhage: report of an adolescent case and literature review.BACKGROUND: Henoch-Schonlein syndrome (HSS) is a systemic necrotizing vasculitis predominantly affecting children. Symptoms are usually self-limited and only rarely do they involve the central nervous system. Only five published reports describe cases of radiologically proven intracranial hemorrhages complicating HSS. CASE DESCRIPTION: In this 17-year-old boy, a cerebellar hemorrhage developed after aspecific symptoms of upper respiratory tract infection. His past medical history and emerging evidence of systemic bleeding yielded a diagnosis of recurrent HSS. This was the fourth time the disease had recurred since the age of 4. The patient underwent surgical treatment and returned to his normal activities. CONCLUSIONS: Intracerebral hemorrhages during HSS share a favorable prognosis and a posterior lobar localization, typically involving the parieto-occipital region. The case described here is unusual because the patient did not have the typical purpuric rash and unlike published cases, the intracranial hemorrhage marked the onset of HSS rather than complicating a typical HSS presentation.- - - - - - - - - - ranking = 411.58960405329keywords = cerebral (Clic here for more details about this article) |
10/26. hypertension induced reversible posterior leukoencephalopathy syndrome: a report of two cases.Reversible posterior leukoencephalopathy syndrome (RPLS) is a recently described disorder with typical radiological findings of bilateral grey and white matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with RPLS are adults and it is rare in children. In this report, two patients with RPLS are presented. In the first patient the primary diagnosis was acute post-streptococcal glomerulonephritis, a known cause of RPLS both in adults and in children. The second patient had Henoch Schonlein purpura. Conclusion: These patients are presented to highlight the importance of reversible posterior leukoencephalopathy syndrome. As the spectrum of associated diseases is diverse, paediatricians must be aware of this syndrome in order to initiate appropriate management.- - - - - - - - - - ranking = 411.58960405329keywords = cerebral (Clic here for more details about this article) |
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