1/448. Acute renal failure in an adult patient with Henoch-Schoenlein purpura after episode of macroscopic hematuria. A case of severe prolonged acute renal failure with a histological picture of acute tubulointerstitial lesions in an adult patient with Henoch-Schoenlein purpura after an episode of macroscopic hematuria is described. The macroscopic hematuria lasted only for 5 days and the renal biopsy was performed 50 days after the end of the macroscopic hematuria. Restoration of renal function was not complete six months after the beginning of improvement. Fewer than 65 cases of acute renal failure due to tubulointerstitial nephritis in patients with glomerulonephritis and after episode of macroscopic hematuria have been described in the international literature. Only one of these patients was suffering from Henoch-Schoenlein purpura. ( info) |
2/448. Compound heterozygosity for one novel and one recurrent mutation in a Thai patient with severe protein s deficiency. Homozygous or compound heterozygous protein S (PS) deficiency is a very rare disorder in the anticoagulant system, that can lead to life-threatening thrombotic complications shortly after birth. This report describes the results of the genetic analysis of the PROS 1 genes in a Thai girl patient. She was reported in 1990 as the first case with homozygous PS deficiency and neonatal purpura fulminans. In the present report, we identified the mutations in this patient by direct sequencing of PCR products representing all 15 exons of the PROS 1 gene and their flanking intronic regions. The patient turned out to be compound heterozygous for two null mutations. One allele contained a novel sequence variation, an A-insertion in an A5-tract covering codon 146 and 147, that results in a frameshift and a stop codon (TAA) at position 155. The other allele contained a nonsense mutation in exon 12 by a transition at codon 410 CGA (Arg) to TGA (stop). Cosegregation of PS deficiency with these two genetic defects was observed in her family. ( info) |
3/448. Henoch-Schonlein purpura: a case report. A case of Henoch-Schonlein purpura with the rare complications of facial and scalp oedema, followed by neurological complications manifesting as focal convulsions with transient conjugate eye deviation and cortical blindness, is described. ( info) |
4/448. Henoch-Schonlein syndrome associated with human parvovirus B19 primary infection. We report a case of Henoch-Schonlein syndrome associated with human parvovirus B19 primary infection. The patient, a 27-year-old Filipino woman, presented with an erythemato-papular-purpuric eruption localized to the lower limbs. General symptoms and signs included fever, hypotension, nausea, vomiting, abdominal pain, inguinal lymphadenopathy and polyarthralgia. Laboratory examinations showed leukocytosis, increase in total serum IgA, proteinuria and haematuria. Circulating IgA immune complexes were also present. The ELISA test for anti-human parvovirus B19 IgM was positive. Histopathological examination revealed a leukocytoclastic vasculitis. This case confirms that also in adult patients, Henoch-Schonlein syndrome may be associated with human parvovirus B19 infection. ( info) |
| Topical nitroglycerin has been previously described as an adjunctive therapy to increase perfusion to areas of purpura affected in purpura fulminans. We report a case of purpura fulminans in which topical nitroglycerin was found to provide analgesia after its application to purpuric lesions. The broader role for the use of topical nitroglycerin in pain management deserves further study and evaluation. ( info) |
6/448. Reconstructive surgery in children after meningococcal purpura fulminans. BACKGROUND/PURPOSE: purpura fulminans (PF) is a serious, often life-threatening disease. As more children are surviving their disease, surgeons are presented with increasing numbers of multiple and complicated wounds as sequelae of PF. The purpose of this paper is to review the management of nine cases of PF, and present the reconstruction method in treating bilateral exposed elbow and knee joints. methods: All cases of pediatric patients with PF and treated by the division of plastic and reconstructive surgery between 1986 and 1998 were reviewed. RESULTS: Seven children (78%) had meningococcal PF, and one (11%) had PF after haemophilus influenza septicemia. PF developed in one (11%) but with no growth in either blood or cerebrospinal fluid cultures. Five children (56%) required amputation procedures. Two children (22%) required knee disarticulation. Two patients (22%) had free myocutaneous flap transfers for bone coverage. One (11%) had PF involving the face. CONCLUSIONS: Meningococcal PF is a rare, often life-threatening disease generally of childhood. More children are surviving their diseases but with devastating sequelae. Successful reconstructive treatment outcome of these children requires a multidisciplinary team approach involving multiple specialties. The goal is to preserve function, maintain maximal length, and salvage limbs when possible. Flexibility and innovation are necessary in treating these multiple and complicated wounds. ( info) |
7/448. Successful treatment of adult-onset Henoch-Schonlein purpura nephritis with high-dose immunoglobulins. A 26-year-old woman was admitted for the evaluation of edema and massive proteinuria. She had a history of purpura of the lower extremities, abdominal pain and melena. Laboratory investigations showed hypoalbuminemia, hypercholesterolemia and proteinuria of over 10 g/day. Renal biopsy showed moderate proliferative glomerulonephritis with mesangial immunoglobulin a (IgA) deposition. She was diagnosed as having Henoch-Schonlein purpura nephritis. Oral prednisolone, dipyridamole and intravenous heparin treatment were not effective. Steroid pulse therapy induced a partial improvement of proteinuria to 2-3 g/day. High-dose intravenous immunoglobulin (i.v.-IG) treatment was introduced and a dramatic improvement of proteinuria was noted. I.v.-IG should be fully considered in patients with steroid-resistant Henoch-Schonlein purpura nephritis. ( info) |
8/448. Ischemic necrosis of bile ducts complicating Schonlein-Henoch purpura. Gastrointestinal complications of Schonlein-Henoch purpura are frequent and sometimes severe. However, there seem to be no reports of liver involvement. A child is described in whom Schonlein-Henoch purpura was complicated by bile duct lesions, resulting in biliary cirrhosis and requiring liver transplantation. At surgical removal, the liver had lesions of bile ducts and of adjacent small blood vessels in the hilum, very similar to those complicating hepatic artery thrombosis after liver transplantation. These findings suggest that Schonlein-Henoch purpura can be complicated by vasculitis of the peribiliary vessels resulting in ischemic necrosis of the bile ducts. Schonlein-Henoch purpura can be added to the list of causes of ischemic cholangiopathies. ( info) |
| BACKGROUND: neisseria meningitidis is the most frequent isolate associated with purpura fulminans in children. Although streptococcus pneumoniae infection has been associated with purpura fulminans, with the exception of one adult, it has only been reported in immunocompromised hosts. PURPOSE: We report an apparently previously healthy child who presented with purpura fulminans associated with pneumococcal meningitis. methods: Case report and review of the medical literature from September 1966 to June 1997, using a medline search. CONCLUSION: While systemic pneumococcal infection is common in childhood, progression to purpura fulminans does not typically occur in overtly healthy children. Our patient illustrates that invasive pneumococcal infection should be considered and empirically treated in a child who presents with purpura fulminans, even in the absence of preexisting functional or anatomic asplenia. ( info) |
10/448. Recurrent gastrointestinal Henoch-Schonlein purpura. A 7-year-old boy was seen for severe abdominal pain, vomiting, and a 2.0-kg weight loss of 2 weeks duration. Stools were Hemoccult positive. Upper gastrointestinal (UGI) endoscopy showed multiple, raised red lesions in the duodenal bulb and descending duodenum. Although the patient did not have the typical cutaneous eruption, other findings such as acute onset of abdominal pain in a previously healthy boy, absence of infectious or surgical lesions, and more importantly endoscopic changes seen typically in the descending duodenum, led to the likely diagnosis of Henoch-Schonlein purpura (HSP). The patient was treated with prednisone and the duodenal lesions resolved. The diagnosis of HSP was confirmed 24 weeks after the initial symptom when he developed a palpable purpuric rash over both legs. Thirteen months following the initial symptoms and 6 months after the onset of rash, severe abdominal pain with epigastric tenderness recurred and stools were Hemoccult positive. UGI endoscopy showed multiple, raised red lesions in the descending duodenum as seen earlier. The patient was diagnosed with recurrent HSP. This presentation is atypical because of the abnormally long interval between the onset of abdominal pain and the appearance of the skin rash, and unique because of the endoscopically demonstrated recurrent gastrointestinal lesions. ( info) |