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Retroperitoneal fibrosis (RPF) is an uncommon disease of unclear aetiology. The review of the literature over the past 20 years revealed 160 published cases. Till now, no accepted diagnostic or therapeutic strategy exist. Most of patients diseases progress to end-stage renal failure without pharmacological treatment. In the paper we report a case of a 58-year old man admitted to the department of urology due to body mass loss, lower-back pain, vomiting, development of oliguria and anuria and intermittent claudication. On physical examination arterial hypertension (180/100 mm Hg), peripheral oedema, tenderness of the enlarged kidneys and lower limbs ischemia were found. Creatinine serum level (Pcr) was 232 micromol/l (2.69 mg/dl). On ultrasonography, symmetrical hydronephrosis and the existence of a hypoechogenic mass along of the aorta and below of renal arteries was found. The diagnosis of RPF was confirmed with MRI. Ureteral catheters were inserted with subsequent decompression of both kidneys and the patient was discharged from the hospital. Seven months later he still presented symptoms of lower limbs ischemia, arterial pressure was high and Per decreased to 138 micromol/l (1.55 mg/dl). The patient was admitted to the department of internal diseases. The treatment with prednisone at the dose of 40 mg/d during 6 weeks was introduced, and the dose was decreased gradually to 10 mg/d within 6 months. Simultaneously, the patient received intravenous therapy with cyclophosphamide 600 mg/infusion once monthly during 6 months. Two month after starting immunosuppressive treatment the intermittent claudication disappeared and after six months MRI examination demonstrated the regression of RPF. The ureteral catheters were removed. After 18 months of follow up, no recurrence of RPF is observed and the kidney function is normal.
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PMID:[Outcome of immunosuppressive treatment of a patient with renal failure due to retroperitoneal fibrosis]. 1552 46

This was a retrospective study to assess the clinical profile of children admitted with acute renal failure and to identify factors associated with poor outcome. Fifty-four children (age one month to 12 years) with acute renal failure were studied. Males outnumbered females (38/54; 70%). The leading precipitating causes for renal failure were acute gastro-enteritis (85%), underlying renal pathology (43%), proven sepsis (22%) and suspected sepsis (22%). The main presenting complaints were diarrhoea (86%),oliguria (72%), rapid respiration (37%), oedema (37%), vomiting (19%) and seizures (13%). All patients underwent standard investigations and treatment. Forty-eight per cent of patients required peritoneal dialysis and 15% required ventilation. The overall mortality was 52%. Underlying renal pathology and sepsis both contributed to the high morbidity and mortality. Mortality due to sepsis was 83%; it was 65% in dialysed patients and 100% in those requiring ventilatory support. Biochemical profile of the above patients showed that hyperkalaemia was significantly associated with high mortality (83%) as against 75% in those with hypokalaemia and 33% with normal levels (p<0.001). Patients with hyponatraemia and hypernatraemia similarly had an adverse outcome. Acidosis, seen in 20 patients, had a mortality of 45%. The outcome was poorer in those with high creatinine levels (63%).
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PMID:Clinical profile and outcome of acute renal failure in South Indian children. 1571 79

Reversible posterior leukoencephalopathy syndrome is a recently described disorder with typical radiologic findings in the posterior regions of the cerebral hemisphere and cerebellum. The symptoms include headache, nausea, vomiting, visual disturbances, focal neurologic deficits, and seizures. A 10-year-old male was hit on his back, resulting in backache. He was medicated with sodium diclofenate and mefenamic acid. The next day, he had edema and oliguria. By the third day, his blood pressure increased and he began to experience restlessness and worsening mental status. He then complained of headache and visual disturbances and had a seizure. A magnetic resonance imaging scan revealed abnormalities in the posterior regions of the cerebral hemisphere and cerebellum. The patient was treated with antiepileptics and calcium antagonists. His hypertension and seizures were well controlled. On the 22nd day, he was discharged without any neurologic or renal deficits. Reversible posterior leukoencephalopathy syndrome does not occur frequently in childhood, and this is the first case report of reversible posterior leukoencephalopathy syndrome related to nonsteroidal anti-inflammatory drugs. One should consider reversible posterior leukoencephalopathy syndrome as a side effect of nonsteroidal anti-inflammatory drug use in daily medical treatment.
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PMID:Pediatric posterior reversible leukoencephalopathy syndrome and NSAID-induced acute tubular interstitial nephritis. 1650 99

A retrospective study was conducted in nine patients with rabies admitted to a hospital of Fortaleza, Brazil. Autopsy was performed in all cases. The ages ranged from three to 81 years and six were males. They all were bitten by dogs. The time between the accident and the hospital admission ranged from 20 to 120 days (mean 45 +/- 34 days). The time until death ranged from one to nine days (mean 3.3 +/- 5.5 days). The signs and symptoms presented were fever, hydrophobia, aerophobia, agitation, disorientation, dyspnea, sialorrhea, vomiting, oliguria, sore throat, pain and hypoesthesia in the site of the bite, headache, syncope, cough, hematemesis, mydriasis, hematuria, constipation, cervical pain and priapism. In three out of six patients, there was evidence of acute renal failure, defined as serum creatinine > or = 1.4 mg/dL. The post-mortem findings in the kidneys were mild to moderate glomerular congestion and mild to intense peritubular capillary congestion. Acute tubular necrosis was seen in only two cases. This study shows some evidence of renal involvement in rabies. Histopathologic findings are nonspecific, so hemodynamic instability, caused by autonomic dysfunction, hydrophobia and dehydration must be responsible for acute renal failure in rabies.
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PMID:Renal involvement in human rabies: clinical manifestations and autopsy findings of nine cases from northeast of Brazil. 1655 20

Cogan's syndrome is an unusual multisystemic disease characterized by interstitial keratitis in association with vestibuloauditory dysfunction and possible irreversible deafness, classified into 2 clinical types: typical and atypical. There is disagreement in the literature about corneal disease in the atypical variety. A 32-year-old woman complaining of ocular hyperemia and ocular pain, photophobia and visual acuity loss in the right eye associated with sudden left hearing loss, vomiting, diarrhea, oliguria, oropharynx pain and fever. Previous history of similar disease in left eye and right hearing. There was intense conjunctival hyperemia, nodular scleritis, episcleritis, and circular infiltrates in the corneal stroma. The patient received pulse-therapy with methylprednisolone and cyclophosphamide. She exhibited significant ocular improvement but poor hearing results. The reported case may be a typical Cogan's syndrome (according to authors that assert the non-existence of corneal disease in the atypical type) with some findings characteristic of the atypical type or an atypical Cogan's syndrome (for those asserting that it is a corneal disease). Differential diagnosis is also discussed.
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PMID:[Cogan's syndrome: ocular findings in an atypical case]. 1727 93

A 14-year-old male patient was admitted with fever, vomiting, muscular pain, mainly in the lower limbs, oliguria and oedema. The presence of rats in the vicinity of the home was reported. Laboratory tests on admission revealed severely compromised renal function and increased phosphokinase creatine and thrombocytopenia. Although the patient presented an atypical course without jaundice or meningeal involvement, early dialytic treatment was administered concomitant with empiric antibiotic therapy for leptospirosis. The probable infected serogroup by serology was Autumnalis. This atypical case illustrates that, in the presence of fever, renal failure, rhabdomyolysis and thrombocytopenia, leptospirosis should be considered, even in the absence of jaundice or meningitis, especially if there is a history of contact with carrier animals.
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PMID:Severe acute renal failure in a child: a rare complication of anicteric leptospirosis. 1763 21

A 59-year-old-woman received related non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) subsequent to autologous PBSCT in our hospital five years after she was diagnosed as oligo-secretory myeloma. She was admitted to our hospital because of vomiting and grayish diarrhea 4 months after non-myeloablative allogeneic PBSCT (mini-alloPBSCT). Although her initial symptoms improved after admission, she gradually showed thrombocytopenia, anemia, and oliguria during the 2 weeks after admission. Our diagnosis was thrombotic thrombocytopenic purpura (TTP) and acute renal failure (ARF) secondary to mini-alloPBSCT. After cessation of cyclosporine administration, we began to treat her with plasma exchange (PE) and hemodialysis. During the three and a half months after we started PE, the TTP gradually improved. Although PE had been reported to be ineffective for TTP post bone marrow transplantation, we could finally discontinue PE. In contrast, since her anuria continued, she was managed with hemodialysis. One month after PE was started, her activity of von Willebrand factor-cleaving protease was 41% (normal range, >50%) and the ultrasonographic investigation of both kidneys was normal. She could be discharged after four and a half months hospitalization and lived well as an outpatient for a further two months. She died shortly after readmission from multiple organ failure without the relapse of TTP. The patient's clinical course would suggest that TTP post mini-alloPBSCT could be treated with PE in some cases, despite the development of dialysis-requiring severe ARF being a poor prognostic factor.
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PMID:A case report of thrombotic thrombocytopenic purpura and severe acute renal failure post non-myeloablative allogeneic peripheral blood stem cell transplantation treated with plasma exchange and hemodialysis. 1784 2

We report on a 12-year-old female patient with steroid-dependent nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) since her 3rd year of life. She was twice treated with oral cyclophosphamide and received antihypertensive treatment with atenolol and enalapril. After 3 years without any control or therapy, she presented in a reduced general condition with hypertensive crisis and a blood pressure of 220/130 mmHg, headache, vomiting and loss of vision. Additionally, renal insufficiency (creatinine 11.4 mg/dl, urea 157 mg/dl), with oliguria, anaemia and a severe relapse of nephrotic syndrome, was present. Initial treatment with steroids, albumin-furosemide infusions and antihypertensive drugs was unsuccessful, and dialysis treatment was necessary. Renal biopsy showed an advanced stage of the known FSGS and, surprisingly, a thrombotic microangiopathy. Further diagnostic investigations revealed no signs of haemolytic-uraemic syndrome, but echocardiography showed left ventricular hypertrophy, and hypertensive retinopathy grade 3 was diagnosed, making severe hypertension the most likely reason for the thrombotic microangiopathy. While adequate antihypertensive treatment led to regress of left ventricular hypertrophy and hypertensive retinopathy, renal function did not recover, and the patient remained dialysis-dependent. In conclusion, severe hypertension in chronic kidney disease can lead to target organ damage and thrombotic microangiopathy, which may further worsen renal function.
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PMID:Thrombotic microangiopathy as a complication in a patient with focal segmental glomerulosclerosis. 1788 57

The major syndromes of mushroom poisoning can be divided by presentation timing: Early syndromes (symptom onset <6 hrs after ingestion) have little probability to cause organ damage. Epigastric pain, nausea, vomiting and diarrhea occur in most cases and treatment includes initial gastrointestinal decontamination with oral activated charcoal and fluid rehydration. In addition, an acute gastrointestinal syndrome can be combined with cholinergic toxicity, epileptiformic response or immuno-hemolytic anemia. Neurotoxic Syndromes may present as dysphoria, delirium, hallucinations or disulfiram-like reactions. Treatment is entirely supportive and if performed in hospital, the prognosis is good. Late syndromes (symptom onset >6 hrs after ingestion) are life-threatening due to liver- and renal failure. Patients who are jaundiced after an acute gastrointestinal episode, are suspected to be poisoned with Amatoxins. Patients with flank pain, hematuria, polyuria or oliguria in the absence of jaundice are suspected to have an intoxication with Cortinarius mushrooms. In both cases an intensive care management is indicated.
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PMID:[Mushroom poisonings: syndromic diagnosis and treatment]. 1803 May 54

The advantages of minimally invasive therapy can be utilised in the surgical disorders of pregnant patients. To our knowledge, there has not been a previous report describing laparoscopic management of diaphragmatic hernia (with mesh) in pregnancy. A 23-year-old pregnant (second trimester) woman was admitted with vomiting, epigastric pain, oliguria and dyspnoea of one month duration. Investigations revealed posterolateral diaphragmatic hernia of Bochdalek with gastric volvulus. Successful laparoscopic mesh-plasty of the diaphragmatic hernia was performed without mortality or morbidity to both mother and child. Principles of laparoscopic surgery for diaphragmatic hernias remain the same. Pregnancy poses challenges to both surgeon and anaesthetist due to changes in the physiology. Acute diseases that threaten the life of mother and child have to be dealt with urgently. We conclude that even complex laparoscopic surgery during pregnancy is feasible.
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PMID:Laparoscopic mesh repair of a Bochdalek diaphragmatic hernia with acute gastric volvulus in a pregnant patient. 1820 56


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