Calciphylaxis in a patient with Crohn's disease in the absence of end-stage renal disease.

Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification previously described in patients with end-stage renal disease and hyperparathyroidism. Early diagnosis may be important in improving the poor outcome in these patients since early intervention may forestall the development of life-threatening complications. We describe a patient with Crohn's disease complicated by short-bowel syndrome and modest renal insufficiency (not requiring renal replacement therapy) who developed calciphylaxis. It appears that longstanding Crohn's disease and the short-bowel syndrome accelerated the development of calciphylaxis as the chronic renal disease was not end stage. Considering the possibility of calciphylaxis in this setting may avoid delaying the diagnosis and its consequences.

Hypercalcemia and electrolyte disturbances in malignancy.

Hypercalcemia and electrolyte abnormalities are common problems in patients with malignancy. In this article we discuss the pathophysiology, clinical features, and management of hypercalcemia, which is the most common metabolic abnormality. We also analyze the electrolyte disturbances that occur in association with malignancy, including hyponatremia, hypokalemia, hypomagnesemia, hypophosphatemia, and hyperkalemia. Recognition and treatment of these disturbances are important parts of the management of patients with malignant disease.

Timing of plasma exchange therapy for thrombotic thrombocytopenia purpura: a brief clinical observation.

Plasma exchange is the mainstay of therapy for thrombotic thrombocytopenia purpura. In this study, the authors retrospectively examined variables that may predict outcome in 18 patients with thrombotic thrombocytopenia purpura. They found that the time to first plasma exchange from admission and time to first dose of intravenous steroid are important in influencing outcome. A rise in platelets and a fall in lactic dehydrogenase 3 days after plasma exchange were also found useful in predicting survival. Therefore, early recognition and intervention in this disease is warranted.

Beer potomania: two cases and review of the literature.

The association of severe hyponatremia and the ingestion of large quantities of beer, termed beer potomania, has been known for several years. We report two new cases, and review 20 others from the medical literature. These patients usually have a history of binge beer drinking, poor dietary intake, and then present with severe hyponatremia and various mental status changes or seizures. Typical laboratory findings include hyponatremia, hypokalemia, and a very dilute urine. The patients respond quickly to the administration of sodium chloride containing i.v. fluids. We propose that the pivotal pathophysiologic mechanism in beer potomania syndrome is the minimal intake of solute and the hypoosmolality of the beer ingested. This will lead to the inability to excrete sufficient amounts of free water to keep up with the ingestion of large quantities of the hyposmolar beer. Treatment with isotonic sodium chloride results in the rapid clearance of the accumulated excess free water.

Exercise-associated collapse in cyclists is unrelated to endotoxemia.

Endotoxemia occurs when intestinal ischemia allows bacterial lipopolysaccharide to translocate from colonic flora into the bloodstream, which triggers release of cytokines that can cause hypotension, rigors, fever, shock, and even death. Recently, blood endotoxin levels were shown to be higher in athletes needing medical attention (330 pg.ml-1) than in their competitors with similar performances (81 pg.ml-1). Though there were no data showing that these athletes had elevated core temperatures or severe illness, speculation followed that endotoxin may play a causal role in heat stroke. We examined the relationship between endotoxemia and mild post-exertional illness in 39 cyclists after a 100-mile ride. Thirteen cyclists had at least one of the following: orthostatic hypotension, rigors, nausea, vomiting, diarrhea, or syncope. Only 2/26 case-controls had any of these symptoms. Data were collected on vital signs, hemoglobin, sodium, creatine kinase, creatinine, and uric acid. Endotoxin titer was determined by chromogenic assay; tumor necrosis factor alpha (TNF-alpha) titer was determined by ELISA. One ill cyclist had an endotoxin level of 330 pg.ml-1, one control had an endotoxin level of 150 pg.ml-1, but endotoxin level was < or = 64 pg.ml-1 in all others. Comparison of pre- and post-ride data showed that controls increased creatine kinase activity (154 +/- 34 vs 561 +/- 191 IU.dl, P < 0.05), creatinine concentration (1.5 +/- 0.0 vs 1.6 +/- 0.0 mg.dl-1, P < 0.05), and uric acid concentration (5.4 +/- 0.3 vs 6.3 +/- 0.3 mg.dl-1, P < 0.05). Ill cyclists had lower serum sodium than post-ride controls (138 +/- 2 vs 142 +/- 0.6 mEq.l-1, P < 0.05), but there were no differences between groups in CK, creatinine, or uric acid. These findings suggest that endotoxemia may complicate, but does not cause mild post-exertional illness in cyclists.

Mechanisms of rhabdomyolysis.

Rhabdomyolysis is a common disorder that occurs as a primary disease or as a complication of a broad spectrum of other diseases. Although some cases are caused by hereditary metabolic or structural abnormalities of the skeletal muscle cell, the majority of cases occur in healthy persons as a result of exhaustive exercise, infections, intoxications, deficiency states, or trauma. Although the causes of rhabdomyolysis are diverse, current evidence suggests that there may be a common final pathway that mediates cellular injury. Thus some noxious factor, perhaps a drug that injures the plasma membrane of the cell, a toxin that activates a cytolytic enzyme, a factor that interferes with metabolism and disrupts the integrity of the skeletal muscle cell, a cytokine such as tumor necrosis factor, or simple hypoxia that reduces energy production by the cell, serves to increase cellular permeability to sodium ions. When sodium ions accumulate in the cytoplasm of the cell, an increase of cytosolic or mitochondrial calcium follows. Calcium activates a variety of proteolytic enzymes that injure the cell membrane, allowing efflux of cellular components into the circulation. The ability to identify some of these components, such as myoglobin or creatine kinase, facilitates clinical recognition of rhabdomyolysis. The cytosolic components released into the circulation, under appropriate conditions, may be life threatening, eg, release of potassium causes hyperkalemic cardiotoxicity. In this review, I attempt to describe a variety of factors that are known to be injurious to skeletal muscle cells and, when possible, describe the apparent mechanism whereby these factors result in injury and disruption of the muscle cell.

Heat stroke and related heat stress disorders.

Medical disorders related to environmental heat exposure are exceptionally common in persons who perform hard work in hot climates. They are also common in competitive athletes as well as in persons who participate in casual exercise to maintain health. The important issue of salt and water disturbances consequent to heavy sweating in hot climates is discussed in detail as are mechanisms of potassium deficiency and its implications. The major forms of environmental heat illness including heat syncope, heat cramp, heat exhaustion, and heat stroke are presented in detail with relevant clinical examples. A discussion of the differential diagnosis of hyperthermia and rhabdomyolysis follows. Because of the difference in treatment and complications, heat stroke is subdivided into the classic variety that affects the elderly and very young and that form that follows heavy physical work and is always associated with rhabdomyolysis. Because severe heat exhaustion and heat stroke are life-threatening disorders, the chapter includes a detailed discussion of complications and plans for treatment.

Cocaine-associated rhabdomyolysis.

Three male patients developed a total of four episodes of acute rhabdomyolysis associated with documented cocaine intoxication (two caused "crack" and two caused by intravenous cocaine). Included is one patient who developed rhabdomyolysis after injecting cocaine and then redeveloped it 6 months later on "rechallenge." One of the four cases resulted in death related to severe hyperkalemia and ischemic bowel. The remaining three episodes followed a course of nonoliguric renal failure.