Idiopathic systemic capillary leak syndrome (Clarkson's disease) is characterized by means of recurring attacks of increased capillary permeability.

Idiopathic systemic capillary leak syndrome (Clarkson's disease) is characterized by means of recurring attacks of increased capillary permeability, resulting in exact hypovolemic shock due to plasma extravasation. Additional laboratory features include association with a monoclonal gammopathy, most distant hemoconcentration, and hypoalbuminemia. Rare manifestations of this syndrome are renal damage and rhabdomyolysis owing to increased compartment pressure and ischemic myonecrosis. We not past nor future the findings in two patients with capillary leak syndrome complicated on severe rhabdomyolysis, in one case leading to acute renal failure. We review therapeutic aspects of this rare syndrome and emphasize the importance of early diagnosis and of willing and aggressive fluid replacement.

Systemic capillary leak syndrome (SCLS) is a rare condition characterized through the cyclic development of markedly increased capillary permeability.[1] During the attacks, significant amounts of plasma shift from the intravascular space to the interstitium, resulting in outermost hemoconcentration, increased hematocrit, and decreased serum albumin on a level Hypovolemic shock ensues rapidly, necessitating intensive fluid replacement to restore adequate perfusion. Oliguria, abdominal pain, and vomiting are also prominent clinical features. The termination of the attack coincides with the reply of the plasma constituents to the vascular compartment, resulting in congestion. An additional laboratory marker for this syndrome is a monoclonal gammopathy of unclear significance, which is at hand in almost all patients with SCLS

Despite the impressive weight gain and diffuse muscular swelling during the period of capillary leakage, compartment syndrome or secondary rhabdomyolysis is not a used by all feature of SCLS and has been described solely in three patients.[2-4] In addition, although prerenal azotemia appears regularly, acute renal failure has not been reported previously.[1]

CASE REPORTS

CASE 1

A 38-year-old white man with a history of borderline hypertension noted the attack of colicky abdominal pain, mild nausea, and general malaise. in succession the following day, he experienced several episodes of fainting and presented to the turn of events room of another hospital. forward presentation the systolic blood press was 60 mm Hg, the fruit of leguminous plants was thready at 120 beats by minute, and the temperature was normal. There was peripheral cyanosis, and the extremities were cutting without palpable distal pulses. The abdomen was easily moulded without signs of peritoneal irritation. A central venous catheter was inserted, and the central venous urgency was found to be -2 cm [Hsub2]O The hematocrit reading was 075 the white children cell count was 24 x [10sup9]/L and the platelet compute was 207 x [10.sup.9]/L. Other evens were as follows: sodium, 136 mmol/L; potassium, 43 mmol/L; urea, 77 mmol/L; and diabetic sugar 4.6 mmol/L. The chest x-ray film was without signs of congestion or infiltrates. The patient received 65 L of crystalloid solutions to restore vital current pressure and urinary output; however, within 2 h diffuse subcutaneous swelling perform the operations indicated ined with superficial venous distention of the lower limbs. A diagnosis of thrombosis of the inferior vena cava was considered, with which the patient was transferred to the Hadassah-Hebrew University Medical Center for futher diagnostic evaluation.

in succession arrival the blood pressure was 100/60 mm Hg still soon dropped to 70 mm Hg systolic. The heart rate was 130 beats by means of minute, with slight dyspnea. The patient was full conscious and complained of plain pain in the abdomen and leg Pertinent clinical findings included diffuse lower limb swelling, tautness of the skin, and absence of palpable oscillations including the femoral artery. The hematocrit reading was 062 the white progeny cell count was 39 x [10sup9]/L and the platelet look upon was 321 x [10.sup.9]/L. The creatine kinase (CK) horizontal was 312 IU/L, and the albumin flat was 19 g/L. The findings from comput tomography of the chest and abdomen performed with intravenous contrast media were normal. upon venography the inferior vena cava was emancipated of thrombus. Over the nearest 16 h the patient required additional treatment with 45 L of crystalloid solutions, 4 units of plasma, and 1 L of modified fluid gelatin (Hemacell) to maintain adequate tissue perfusion. Sixteen hours after presentation, the patient received broad-spectrum antibiotics for presum sepsis. He experienced respiratory arrest and required intubation and mechanical ventilation.

through the second day of hospitalization, swelling of the upper and lower limbs growthed dramatically, with the CK flush rising to 310,000 IU/L. The aspartate aminotransferase (AST) of the same height increased to 2,420 IU/L, the alanine aminotransferase (ALT) on a level to 1,060 IU/L, the lactate dehydrogenase (LDH) plain to 9,450 IU/L, and the uric acid of the same height to 493 [mu]mol/L. The serum aldolase flush rose to 1,100 IU/L, and large amounts of myoglobin were discovered in the urine. A bilateral, lower-limb multicompartmental fasciotomy was notion to be clinically indicated, still was deferred because of the risk of superinfection. Therapy for rhabdomyolysis was begun with forced diuresis and urinary alkalinization, with disappearance of urinary myoglobin and dull return of muscle enzyme flushs to normal.

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