Renal failure, acute clinical presentation

This patient illustrates some of the difficulties in the diagnosis of HGPRT deficiency when the patient presents in acute renal failure, the clinical syndrome falls short of the full Lesch-Nyhan syndrome, and especially highlights the problems attendant on the evaluation of HGPRT activity from erythrocyte lysates alone. 

Because the severity of symptoms and the absolute serum concentration are poorly correlated in some patients, institution of therapy should be dictated by the clinical scenario. All patients with hypercalcemia should be treated with aggressive rehydration normal saline at 200 to 300 mL/hour is a routine initial fluid prescription. For patients with mild hypocalcemia, hydration alone may provide adequate therapy. The moderate and severe forms of hypercalcemia are more likely to have significant manifestations and require prompt initiation of additional therapy. These patients may present with anorexia, confusion, and/or cardiac manifestations (bradycardia and arrhythmias with ECG changes). Total calcium concentrations greater than 13 mg/dL (3.25 mmol/L) are particularly worrisome, as these levels can unexpectedly precipitate acute renal failure, ventricular arrhythmias, and sudden death. 

Renal Effects. The patient described by Letz et al. (1984) (see Section 2.2.3.1) who lived for 64 hours after exposure to toxic levels of 1,2-dibromoethane had acute renal failure as evidenced by severe oliguria 24 hours after exposure and abnormal clinical chemistry values (blood urea nitrogen, creatinine, and serum uric acid). Severe metabolic acidosis was present despite two hemodialysis procedures. 

Complications included acute respiratory distress syndrome, renal failure, and multi-organ failure. Evidence that the clinical spectrum of human H5N1 infections is not restricted to pulmonary symptoms was provided by a reported case of possible central nervous system involvement in a Vietnamese boy who presented with diarrhea, followed by coma and death. Influenza H5N1 virus was isolated from throat, rectal, blood, and cerebrospinal fluid specimens, suggesting widely disseminated viral replication. 

Hootkins R, Fenves AZ, Stephens MK. Acute renal failure secondary to oral ciprofloxacin therapy a presentation of three cases and a review of the literature. Clinical nephrology. 1989 Aug 32(2) 75-8. 

Chronic salicylism presents clinically in a similar fashion to the acute situation, although it is often associated with a delay in diagnosis, and a higher morbidity and mortality. Chronic salicylism is more often associated with pronounced hyperventilation, dehydration, pulmonary edema, renal failure, coma, seizures, and acidosis. Chronic salicylism can occur at serum salicylate levels as low as 15mgdl. ... 

The classic clinical finding of increased copper deposition in the eye is the Kayser-Fleischer ring, caused by deposition of copper in Descemet membrane at limbus of the cornea. Although found in about 95% of patients with neurological or psychiatric manifestations, it is present in only about half of patients with hepatic forms of Wilson s disease" and is rarely present in children. As mentioned earlier, hemolytic anemia and renal failure commonly accompany acute forms of Wilson s disease hemolytic anemia may be episodic even in chronic forms of Wilson s disease. ... 

The development of acute renal failure (ARF) presents a difficult challenge to the clinician. It has widely varying causes, and unlike other cases of organ failure such as neurologic or cardiovascular failure, the onset of ARF is often silent. In the ambulatory setting, patients may not notice ARF symptoms for days or weeks. Clinical and laboratory markers of its presence can be subtle and are often overlooked. Despite its often insidious presentation, ARF can be one of the most serious consequences that can occur, especially in a hospitalized patient. 

The diagnostic approach to the patient with ARF differs depending on the clinical setting in which the kidneys fail. For patients who present to the outpatient clinic or hospital with an elevated serum creatinine, the first objective is to determine if the renal failure is acute or... 

Acute allergic interstitial nephritis is the underlying cause for up to 3% of all cases of acute renal failure. Clinical manifestations of AIN typically present about 14 days after initiation of therapy and include fever, maculopapular rash, eosinophilia, pyuria, hematuria, proteinuria, and oliguria. 

A number of reports in the mid to late 1980 s described patients who developed rhabdomyolysis while using cocaine [118-120]. Some of these patients experienced acute renal failure [121-125]. While the exact incidence of acute renal failure secondary to cocaine rhabdomyolysis is unknown, in one reported series it occurred in only three of 211 admissions for cocaine related complications [114]. On the other hand, in another series of nearly 40 patients the incidence of cocaine related acute rhabdomyolysis increased over the period of enrollment from 2 patients in 1985 to 22 patients in 1987 [126]. Several reports of patients with cocaine-induced rhabdomyolysis have clearly defined both the clinical syndrome and the risk factors for the development of acute renal failure and an adverse outcome [123, 126, 127]. Most patients have been previously healthy young males (mean age 30-35 years old and 80-85% male). The cocaine has been smoked, used intravenously, snorted, or taken orally implying that route of administration was not relevant [122,123,126, 127]. In contrast to narcotic related rhabdomyolysis, a history of prolonged coma or stupor is absent. On presentation, the majority of patients are combative and... 

There are four possible relevant clinical presentations for acute CsA nephrotoxicity asymptomatic increases in serum creatinine without overt renal dysfunction, acute renal failure, delayed graft function after renal transplantation and recurrent or ie novo hemolytic uremic syndrome (Table 2). 

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