Hyperammonaemia due to Klebsiella infection in a neuropathic bladder

Cheang, Hon-Kit; Rangecroft, L.; Plant, Nick; Morris, Andrew A. M.

doi:10.1007/s004670050523

Cited by 35 publications

(32 citation statements)

References 9 publications

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“…Secondly, Corynebacterium spp. was only reported once in a similar condition [6], the other isolated pathogens being Proteus mirabilis [3,4,7,8], Klebsiella oxytoca and Klebsiella pneumoniae [1,2,3], Enterococcus faecalis [1], Moraxella catarrhalis [1] and Enterobacter [3]. Thirdly, elevated plasma glutamine points to prolonged hyperammonaemia, but normalisation of plasma ammonia within a few hours of therapy is inconsistent with a primary metabolic disorder.…”

Section: Discussionmentioning

confidence: 93%

“…Proteus mirabilis, Klebsiella oxytoca and Klebsiella pneumoniae and Corynebacterium group D-2, has been reported in a few children with known underlying obstructive uropathy [1,2,3,4,5,6,7,8,9,10]. Three concurrent pathogenetic mechanisms are known to cause hyperammonaemia secondary to UTI: (1) infection with urease producing organisms splitting urea into ammonia [1,2,3,4,5,6,7,8,9,10], (2) slow passage of large urinary volumes along a large mucosal surface area [6], and (3) an alkaline urinary pH favouring diusion of ammonia into the blood stream [3,5,6,7].…”

Section: Discussionmentioning

confidence: 99%

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Hyperammonaemic encephalopathy in a 13-year-old boy

et al. 2002

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A previously healthy 13-year-old boy presented with a 3-day history of nausea, vomiting and progressive weakness and lethargy. When admitted to a peripheral hospital, he was afebrile, mildly dehydrated and had a Glascow Coma Scale of 7. Within a few hours, he became comatose and required intubation. A cerebral CT scan was normal. Plasma ammonia concentration, however, was markedly elevated with 250 lmol/l (normal 12±48 lmol/l). Renal function was mildly impaired with a plasma creatinine of 85 lmol/l (normal 45± 80 lmol/l) and plasma urea of 11 mmol/l (normal <7 mmol/l). Blood cell count showed a leukocytosis of 13.3´10 9 /l with 47% neutrophils. Electrolytes, serum C-reactive protein, liver function and coagulation tests were normal. A putative diagnosis of a late-onset ornithine transcarbamylase (OCT) de®ciency was made and the patient transferred to the University Children's Hospital, Zurich.On admission, plasma ammonia was 179 lmol/l (normal <55 lmol/l) and glutamine 909 lmol/l (normal <600 lmol/l). The urine was turbid; urinalysis showed leucocyturia (445 cells/ll), a positive nitrite test, bacteriuria and microhaematuria (640 cells/ll); the pH was 8. Emergency analysis of organic acids, amino acids and orotic acid in plasma and urine ruled out a primary metabolic defect. Ammonia excretion was promoted with both a catheter draining the bladder continously and forced intravenous¯uids (100 ml/kg daily). Plasma ammonia decreased rapidly into the normal range (48 lmol/l) within 6 h after admission. Ultrasound revealed hyperechogenic urinary¯uid, a large bladder, a dilated proximal urethra suggestive of posterior urethral valves and markedly dilated ureters and pyelons. The structure of both kidneys appeared normal. A micturition cystogram revealed remnants of posterior urethral valves and gross bilateral vesicoureteric re¯ux with a left duplex system. DMSA scintigraphy showed bilateral patchy photon defects with a dierential function (right to left) of 32%:68%. Antibiotic treatment with ceftriaxone was initiated (60 mg per kg body weight daily i. v.). When the urinary culture grew Corynebacterium pseudodiphtheriticum (10 6 CFU/ll), therapy was changed to amoxicillin (100 mg/kg daily i. v.).The boy was extubated after 24 h and the level of consciousness returned to normal within 2 days. At discharge 3 weeks later, neurological examination showed mild dysarthria and a limp of the right leg. MRI of the brain was normal, while an EEG showed diuse slow waves on the left fronto-central side, but no speci®c features suggesting encephalitis or epilepsy. The glomerular ®ltration rate, assessed as a single Cr 51 injection clearance, was normal (99 ml/min per 1.73 m 2 body surface area). Six months later, the neurological examination was normal.

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Hyperammonaemic encephalopathy in a 13-year-old boy

et al. 2002

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“…Her high ammonia level is thought to have resulted from the production of excessive amounts of ammonia within the over distended bladder. [2][3][4][5][6][7][8][9] Ureasplitting bacteria produce urease in UTIs and the hydrolysis of urinary urea to ammonium and carbon dioxide results in highly alkalinized urine. Proteus mirabilis, Pseudomonas aeruginosa, Klebsiella species, Morganella morganii and Corynebacteria are the best known urea-splitting bacteria, but Enterococcus faecium also produce urease.…”

Section: Discussionmentioning

confidence: 99%

“…1 In addition, non-hepatic hyperammonaemic encephalopathy had been reported in some patients with urea-splitting urinary tract infections. [2][3][4][5][6][7][8][9] In particular, recurrent hyperammonaemic encephalopathy had been reported in patients with urinary diversion or vesico-colonic fistula. 2,3 However, simple urinary tract infection (UTI) and urinary retention without appropriate treatment might be associated with recurrent hyperammonaemic encephalopathy.…”

Section: Introductionmentioning

confidence: 99%

Recurrent Hyperammonaemic Encephalopathy in a Patient with Urinary Tract Infection and Urinary Retention

Wee²,

Choi³

2012

Hong Kong Journal of Emergency Medicine

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Recurrent hyperammonaemic encephalopathy in a patient with urinary tract infection and urinary retention 一位泌尿道感染和尿瀦留患者的重復性高氨血症性腦病 SH Oh, JH Wee, SP Choi Acute hyperammonaemia is a medical emergency. Most cases are resulted from severely impaired liver function but some cases of non-hepatic causes do occur. In patients with unexplained altered conscious state, immediate measurement of plasma ammonia level is a simple but important step in early diagnosis of hyperammonaemia and prompt reduction of ammonia level to minimise permanent brain damage. We reported a case of recurrent hyperammonaemia and cerebral dysfunction due to urinary tract infection with urinary retention in a patient without underlying liver disease. (Hong Kong j.emerg.med. 2012;19:417-419) 急性高氨血症是一種內科急症。大多數情況下都是由於嚴重肝功能衰退引起，但也有非肝源性的病例發生。在原因不明的意識障礙患者中，直接測量血氨水平是及早診斷高氨血症，迅速減低血氨含量以及盡量減少永久性的腦損害的一個簡單而重要的步驟。我們報導了一位因尿瀦留引發泌尿道感染的患者在沒有潛在肝臟疾病的情況下所出現的重復性高氨血症和腦功能障礙。

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“…To our knowledge approximately 20 children have been reported in the literature with this particular condition. All of these cases had predisposing conditions: prune belly [3][4][5][6][7], hydronephrosis [8,9], ureterocele [8], anti-reflux surgery or other urological intervention [10,11]. Murray et al reported infection with Providencia rettgeri, not previously described as a causative organism [6].…”

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confidence: 97%

A confused child: Answer

2007

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This boy had a urinary tract infection (UTI), dilated urinary system and associated hyperammonaemic encephalopathy. Differential diagnoses include infectious, metabolic, toxic or hypertensive encephalopathy. This boy responded over 24 h to rehydration, urinary tract drainage and antibiotics. His blood pressure settled to 100/60 without specific antihypertensive treatment and did not become a chronic problem. We did not culture an organism from this boy's urine on presentation; however, approximately 10 days later Corynebacterium urealyticum (mucoid colony) was cultured from a subsequent urine. As he remained apyrexial and had no other signs of sepsis with a rapid recovery after the measures above, it is likely he had a lower urinary tract infection. He had a grossly dilated urinary tract and, in the setting of urinary tract infection, urinary stasis and dehydration, an increased urea load. Urease-producing bacteria in the urine of the dilated urinary tract and bladder form ammonia from urea and this results in alkalinization of the urine. As the dissociation constant of the ammonia/ammonium ion pair is 9.8, an alkaline environment favours the formation of proportionately more ammonia than ammonium ions compared with that at a lower pH. Ammonia is lipid soluble and can readily diffuse across cell membranes of the dilated urinary tract and bladder. At a physiological pH of about 7.40 within the venous circulation the equilibrium is overwhelmingly in favour of ammonium formation which is significantly less lipid soluble [1,2]. This mechanism is known as diffusion trapping. Complete hydrolysis of the 30 g (0.5 mol) of urea usually excreted by an adult each day would lead to 17 g (1.0 mol) of ammonia potentially being absorbed. This is four times the amount produced each day by the gastrointestinal tract [3]. The venous drainage from the bladder bypasses the liver and thus is not detoxified. The serum ammonia concentration rises and encephalopathy may develop. Hyperammonaemia leads to glutamine-mediated toxicity with mitochondrial toxicity, astrocyte swelling and cerebral oedema. Urea-splitting bacteria include Ureaplasma urealyticum, Corynebacterium urealyticum, Proteus, Pseudomonas, Klebsiella and Staphylococcus.Increased absorption of ammonia at a renal parenchymal level is another potential mechanism. With infection of the parenchyma with Proteus for example, hydrolysis of urea occurs within the tubules leading to a high intraluminal ammonia concentration and favouring diffusion into the tubular cells and thus into the systemic circulation. Ammonia is also produced within the renal tubular cells. Generally 75% is excreted into the urine; however, with a high urinary pH the ammonia tends to be reabsorbed.

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Hyperammonaemia due to Klebsiella infection in a neuropathic bladder

Cited by 35 publications

References 9 publications

Hyperammonaemic encephalopathy in a 13-year-old boy

Hyperammonaemic encephalopathy in a 13-year-old boy

Recurrent Hyperammonaemic Encephalopathy in a Patient with Urinary Tract Infection and Urinary Retention

A confused child: Answer

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