1/18. Hereditary renal amyloidosis associated with variant lysozyme in a large English family.BACKGROUND: Two kindreds with hereditary systemic amyloidosis caused by the first two mutations to be described in the human lysozyme gene were discovered recently and study of the variant lysozyme has been powerfully informative about mechanisms of amyloid fibrillogenesis. However, the clinical manifestations in these families, additional members of which have lately been identified, have not previously been reported in detail. methods: The proband presented with proteinuria aged 50 and a family history of amyloidosis, and underwent renal biopsy, whole-body serum amyloid P component (SAP) scintigraphy, and sequencing of the lysozyme gene. Her family history and the phenotype of hereditary lysozyme amyloidosis were thoroughly documented and compared with the presentation and natural history of all other known patients with this condition. RESULTS: The proband belonged to an extended English family other members of which were known to have hereditary lysozyme amyloidosis. Those with amyloid in previous generations presented with renal involvement, frequently developed complications due to gastrointestinal amyloid, and died before age 60. All amyloid deposits were composed of lysozyme and complete concordance was established between amyloid and heterozygosity for a point mutation in the lysozyme gene, encoding the previously reported Asp67His substitution in the mature protein. CONCLUSION: The phenotype, reported for the first time in this extended kindred, contrasts with that of an apparently unrelated family carrying the same mutation who presented with spontaneous hepatic haemorrhage and rupture, and with the manifestations in a family with the lysozyme Ile56Thr variant who presented with dermal petechiae before proceeding to fatal visceral amyloidosis. A remarkably wide spectrum of disease can be caused by the same amyloid fibril protein, although renal involvement predominates in all cases except those dying of hepatic rupture.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/18. Spontaneous subcapsular renal haemorrhage presenting with pleuritic chest pain.We present an unusual case of spontaneous renal subcapsular haematoma in a normal kidney presenting with pleuritic chest pain and mimicking pulmonary embolism. The literature suggests that the majority of these cases occur in association with renal tumours and that the diagnosis can best be made by computed tomographic scanning. Treatment is expectant but because of the high incidence of tumours, nephrectomy is usually necessary.- - - - - - - - - - ranking = 4keywords = haemorrhage (Clic here for more details about this article) |
3/18. systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation. To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.- - - - - - - - - - ranking = 5keywords = haemorrhage (Clic here for more details about this article) |
4/18. Massive peri-renal haemorrhage from ruptured renal artery aneurysm in the presence of normal renal function.A 65-year-old man presented acutely with a large right peri-nephric haematoma as seen on a CT scan. Intravenous urography demonstrated normal function. An arteriogram later revealed a 1 cm renal artery tributary aneurysm, which was successfully embolized.- - - - - - - - - - ranking = 4keywords = haemorrhage (Clic here for more details about this article) |
5/18. Carboxyatractyloside poisoning in humans.OBJECTIVE: Cocklebur (xanthium strumarium) is an herbaceous annual plant with worldwide distribution. The seeds contain the glycoside carboxyatractyloside, which is highly toxic to animals. We describe nine cases of carboxyatractyloside poisoning in humans which, to our knowledge, has not previously been reported. The clinical, laboratory and histopathological findings and our therapeutic approach are also discussed. SUBJECTS AND methods: The patients presented with acute onset abdominal pain, nausea and vomiting, drowsiness, palpitations, sweating and dyspnoea. Three of them developed convulsions followed by loss of consciousness and death. RESULTS: Laboratory findings showed raised liver enzymes, indicating severe hepatocellular damage. BUN and creatinine levels were raised, especially in the fatal cases who also displayed findings of consumption coagulopathy. CPK-MB values indicative of myocardial injury were also raised, especially in the fatal cases. Three of the patients died within 48 hours of ingesting carboxyatractyloside. Post-mortem histopathology of the liver confirmed centrilobular hepatic necrosis and renal proximal tubular necrosis, secondary changes owing to increased permeability and microvascular haemorrhage in the cerebrum and cerebellum, and leucocytic infiltrates in the muscles and various organs including pancreas, lungs and myocardium. CONCLUSIONS: Carboxyatractyloside poisoning causes multiple organ dysfunction and can be fatal. Coagulation abnormalities, hyponatraemia, marked hypoglycaemia, icterus and hepatic and renal failure are signs of a poor prognosis. No antidote is available and supportive therapy is the mainstay of treatment.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
6/18. Spontaneous bilateral rupture of kidneys in a patient with polyarteritis nodosa. A case report.A previously well 42-year-old man presented with a three-hour history of pain in the right flank of acute onset. At laparotomy he was found to have a ruptured right kidney which was treated by nephrectomy. Eight days later he developed similar symptoms on the left: at operation 21 of blood were drained and nephrostomy and catheterisation carried out. He recovered after a complicated postoperative course, and histological examination of the removed kidney, and biopsy specimens, showed classic polyarteritis nodosa. This is a rare cause of spontaneous rupture of the kidney but must be considered whenever a patient presents with renal haemorrhage of unknown cause.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
7/18. Perirenal and renal subcapsular haematoma as presenting symptoms of polyarteritis nodosa.Two young men, were hospitalized due to acute massive blood loss with left abdominal flank pain. In both cases renal angiography showed signs of a haemorrhagic event in the left kidney, perirenal in one and subcapsular in the other. Microaneurysms indicated a diagnosis of polyarteritis nodosa, supported by renal biopsy in one case. Renal haemorrhage is an infrequent presentation of polyarteritis nodosa. Furthermore, one patient suffered also from familial mediterranean fever, and is the fifth reported case with this combination of diseases.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
8/18. Haemorrhage due to rupture of the kidney with intraparenchymal calculi, chronic pyelonephritis and acute obstructive uropathy. A case report.Massive left perirenal haemorrhage due to rupture of the kidney with intrarenal calculi has not been previously described and in this case is thought to have been due to erosion of small vessels by intraparenchymal calculi aggravated by oedema and ischaemia caused by associated acute obstructive uropathy.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
9/18. The pathology and pathogenesis of chronic lead nephropathy occurring in queensland.Many children who suffered acute lead poisoning in queensland eventually died with contracted kidneys. In most cases the kidneys were granular and showed microscopically fibrosis, hypertensive vascular changes and "alterative glomerulitis". Clinically in these patients, hypertension and chronic renal insufficiency had always preceded death which was usually due to uraemia. In a minority of cases the kidneys showed the changes of benign hypertension but were unusually small; fibrosis and "alterative glomerulitis" were not present. Clinically these patients had had hypertension but minimal renal insufficiency and death was usually due to cerebral haemorrhage. The evidence indicates that lead caused severe damage to the kidney at the time of the lead intoxication by some mechanism other than hypertension. The sequence of events postulated comprises severe renal damage with destruction of glomeruli during childhood lead poisoning, disappearance of the destroyed tissue during childhood and adolescence, onset of hypertension in adolescence or early adult life, gradual onset and progress of chronic uraemia during which fibrosis and granularity developed. In milder cases the sequence is not complete because renal function has remained adequate.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
10/18. Control of bleeding after renal biopsy with epsilon-amino-caproic acid.EACA proved useful in controlling severe bleeding after renal biopsy. In 6 patients who required transfusion for prolonged or severe bleeding, haemorrhage ceased and no further transfusions were necessary after EACA therapy. No serious side-effects were encountered.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
| | Next -> |