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The case history is presented of two patients with attacks of cherry-red urine after strenuous exercise. Both had varicosity of the long saphenous veins. Haemoglobinuria disappeared after crossectomy of the long saphenous vein and resection of the convolutes. After one and two years, they have no recurrence.

During a 12-month period, 42 patients required admission to hospital after sjambok beatings. Eighteen showed no abnormality of serum electrolyte values or renal function but urinary pigment precipitation was present in 9 patients. A further 16 patients demonstrated abnormalities of serum urea and electrolyte values, the commonest of which was a subnormal venous carbon dioxide content, in conjunction with urinary pigment precipitation in 14 cases. The remaining 8 patients presented with, or developed, varying degrees of renal failure. In 4 of these patients, the sole initial biochemical abnormality was a subnormal venous carbon dioxide level. Based on the initial laboratory findings, four groups of patients may be identified with gradations of soft-tissue injury. Recognition of those patients at risk of developing crush syndrome is facilitated and appropriate treatment may be instituted at an early stage thereby reducing morbidity and mortality.

A case of hemolytic transfusion reaction, accompanied by hypotension and followed by transient renal failure, occurred after the transfusion of 1 unit of previously frozen autologous red cells. Subsequent investigation revealed the probable cause of the hemolysis to be inadequate deglycerolization of the unit. The cause of the associated symptoms is unknown. Possibilities include nephrotoxic effects of hemoglobin or stroma, toxic effects of glycerol, or release of vasoactive or thrombogenic substances from lysed red cells. This case of a hemolytic reaction adds to the known risks of autologous transfusion.

Acute anaemia was induced in calves aged 1 week, using the oxidant chemical phenylhydrazine-hydrochloride (PHH). The hematoclinical responses to graded doses were evaluated. Mild anaemia was observed with the 60 mg/kg body weight (B.W.) dose. The 100 mg/kg B.W. dose was too toxic. The sublethal 80 mg/kg B.W. dose produced the desired type of hemolytic anaemia, the peak of which being observed on d 4 post-injection (p.i.). Hemolytic anaemia was evidenced by hemoglobinaemia and hemoglobinuria. The acute toxicity symptoms lasted for about 30 min. Thereafter, the animals appeared to overcome the stress. In general, increases in pulse and respiration coincided with progress of the hemolytic syndrome. Peak response occurred 48 to 60 h p.i.

Hypovolemia, low cardiac output, and systemic vasoconstriction are major etiologic factors in acute renal failure occurring in the early postburn period, and elevated levels of stress-related hormones (catecholamines, angiotensin, aldosterone, and vasopressin) are implicated in the mechanism. By counteracting the effects of the hormones, atrial natriuretic polypeptide (ANP) regulates the renal response to burns. ANP was elevated after burns, protecting the kidneys by increasing renal blood flow and urine output. In pulmonary acid injury, increased ANP levels were associated with natriuresis which was reduced by administration of anti-ANP serum. Exogenous ANP given to dogs under constant norepinephrine infusion resulted in improvement of hemodynamic and renal parameters. To prevent tubular damage due to hemoglobinuria, a haptoglobin preparation is administered to patients with extensive third-degree burns. With sufficient fluid replacement, these new treatments will reduce the incidence of acute renal failure in the early postburn period.

A 2-year-old Suffolk gimmer presented with clinical signs of haemoglobinuria, jaundice, anaemia and acute mastitis. Clinical investigation and bacteriological examination of milk samples revealed Clostridium perfringens type A to be the causal agent.

The authors present two infants with isoimmune hemolytic disease due to ABO incompatibility complicated by massive hemoglobinuria and secondary acute renal failure. This represents an incidence of 0.36% of all neonates with ABO hemolytic disease in the author's newborn population. Only two patients have been reported previously to have similar complications. Analysis of data of these four infants revealed the clinical characteristics of this complication of ABO incompatibility: 1) very low frequency; 2) early onset of hemoglobinuria (first voided urine) and of acute renal failure (first 2 days of life); 3) lack of correlation between the clinical presentation of hemolytic disease and appearance and severity of renal failure; 4) complete recovery of renal functions following intravenous fluid administration; and 5) normal renal radiologic investigations.

Postparturient haemoglobinuria occurred in 11 Holstein-Friesian cows in 8 dairy herds in East Gippsland, Victoria. The affected cows had a wide range of ages, body condition and production level. Seven of 9 cows sampled were hypophosphataemic, 4 of 8 cows examined had Heinz bodies present in erythrocytes and strong ketonuria was present in 6 of 10 urine samples tested. Herdmates having similar calving dates had mostly normal serum phosphorus levels and none had Heinz bodies. There was no evidence for dietary phosphorus (P) deficiency, hypocuprosis or excessive P drain from high production being predisposing factors. There were no cruciferous plants in the diet. Haemolysis persisted in 4 cows despite treatment to raise serum P levels. An hyposthesis is proposed for the sporadic nature and clinical manifestations of the disease. This is based on mechanisms of hypophosphataemia occurring in man and mechanisms of Heinz body formation and haemolysis in other circumstances. We suggest that hypophosphataemia occurs following recovery from preparturient keto-acidosis caused by undernutrition in late pregnancy and then becoming nutritionally replete in the early postparturient period. Circulating oxidants may cause erythrocyte damage which may be predisposed to by hypophosphataemia.

A three-month-old baby with persistent intravascular hemolytic anemia following patch closure of subpulmonic ventricular septal defect was reported. Mechanical hemolysis was diagnosed by the presence of blood cell fragments and schistocytes in peripheral blood smear. It was thought that destruction of red cells developed by turbulent flows and direct contact of red blood cells with bared rough surfaces of the teflon patch at the site of dehiscence. When the cause of severe hemolysis was attributed to inadequate intracardiac repairs, immediate second surgical intervention should be performed.

A large-animal model is essential for the assessment of functional parameters in cardiovascular surgical research. To date the canine model has been used successfully because of its availability and tolerance to cardiopulmonary bypass. However, because of decreased availability and increased cost, an alternative animal model is now needed. The swine model has been used in experimental cardiac procedures, but complications during cardiopulmonary bypass have presented a formidable challenge. These complications include enormous fluid shifts from the vascular bed, increased metabolic acidosis, and marked hemoglobinuria. To eliminate these deleterious complications within the swine model, a number of technical alterations were achieved. The priming solution used for the extracorporeal circuit was altered to consist of 1000 mL lactated Ringer's solution. 500 mL 20% mannitol, 500 mL 6% dextran in 5% detrose solution. 50 mEq sodium bicarbonate, and 10,000 IU heparin. The extracorporeal circuit employed the use of membrane oxygenation. Three different blood flow rates (150, 175, and 200 mL/kg min-1) were studied. We conclude that the optimum blood flow rate for cardiopulmonary bypass in swine is in the range of 175-200 mL/kg min-1. Membrane oxygenation results in less damage to blood during cardiopulmonary bypass. The asanguinous hyperosmolar priming solution is beneficial for cardiopulmonary bypass in swine to greatly reduce fluid shifts, prevent metabolic acidosis, and eliminate hemoglobinuria.

Nephrotoxicity is a problem of hemoglobin solutions (HbS) that still awaits full elucidation and correction. Therefore, a study was conducted using five HbS with different characteristics to replace 1/3 of blood volume in five groups of rabbits. All HbS contained bovine Hb, 6.5 g/dl, dissolved into a balanced electrolyte solution. HbS-I was Hb incompletely purified of stromal phospholipids and environmental bacterial endotoxins, and uncrosslinked; HbS-II was pure Hb non crosslinked; HbS-III was completely purified and crosslinked; HbS-IV was like HbS-III, but with pH 8.4; and HbS-V was like HbS-III, with the addition of mannitol. The effects of blood replacement with these solutions were studied on: (a) PAH clearance (expression of renal plasma flow); (b) endogenous creatinine clearance (expression of glomerular filtration); (c) fractional excretion of sodium and (d) urine/plasma osmolarity (expressions of tubular function). Histological changes were assessed after 24 hours. Significant alterations were observed in decrescent order following the administration of HbS-I, -II and -III, while HbS-IV and -V were well tolerated. These results suggest that the nephrotoxicity of Hb solutions can be prevented by the following steps: (1) complete purification of Hb; (2) complete crosslinking; and (3) protection of the kidney by alkalinization of the urine and/or the addition of mannitol.

There is a definite need for the development of a specific and sensitive diagnostic test for the detection of hematuria in patients with bladder and kidney cancer. It is well established that hemoglobin in the urine may be indicative of bladder and/or kidney cancer as well as other types of lesions of the urinary tract. We have developed a specific and sensitive immunological assay for the detection of human hemoglobin in urine. This test has several important advantages over the currently used chemical tests. A mouse monoclonal antibody specific for human hemoglobin was produced and used in the development of a double-antibody biotinylated enzyme-linked immunosorbent assay (ELISA) that specifically detected hemoglobin in the urine of 195 urology patients. The results from this assay were compared with data from an evaluation of the same specimen using a polyclonal antibody biotinylated ELISA (PE) as well as a dipstick test in a blind screening study. The monoclonal antibody biotinylated ELISA (ME) possessed a much higher level of sensitivity and specificity over the dipstick test. In addition, we determined that monoclonal as well as polyclonal antibody can detect not only normal hemoglobin but also abnormal hemoglobins in urine. Of the total 195 urology patients in all diagnostic categories, the ME test was positive for detection of hemoglobin in 28 more patients (35% more) than were detected by the dipstick test. Importantly, four of these 28 patients detected by the ME but undetected by the dipstick were patients with bladder or kidney cancer.(ABSTRACT TRUNCATED AT 250 WORDS)

Four severe cases of imported malaria are reported here. Three of them are pernicious and the fourth is a black water fever (hemoglobinuria). The difficulties of initial diagnosis are exposed. There was no mortality in these four cases which represent the annual recruiting of a multipurpose intensive care unit. But the complications are severe in contradiction with easy effective chemoprophylaxis. The principles of treatment and prophylaxis are resumed in the light of these four observations.

Multiple placental chorioangiomas in a 31-year-old woman led to a severe, acute fetal-to-maternal transfusion and maternal hemoglobinuria from hemolysis of the fetal red blood cells. Fetal distress was manifest by a sine wave fetal monitor pattern. A severely asphyxiated infant was delivered by cesarean section. The infant did well after transfusion therapy.

Verdoglobinuria is considered to be an ominous sign of Pseudomonas aeruginosa septicaemia in burns. No report of the survival of a case with this phenomenon could be found in the literature. Stone et al. (1964) first reported verdoglobinuria as an important diagnostic basis for P. aeruginosa septicaemia in burns. A patient with high-tension electrical injury was admitted to our hospital burn department in 1977. Verdoglobinuria occurred. This patient is reported as follows. There was a large defect in the left chest wall with exposure of the heart and secondary P. aeruginosa pyothorax which happened during the early stage of injury. It led to septicopyraemic shock. The patient's urine was examined under ultraviolet rays, the fluorescence was ultrapositive. Verdoglobinuria was positive proof. After active measures were administered, the patient was out of danger from this septicopyraemic shock. The metabolism mechanism of verdoglobin, diagnostic effect of verdoglobinuria on P. aeruginosa septicaemia in severe burns and treatment are discussed.

Three patients with typhoid fever, initially misdiagnosed, developed intravascular haemolysis, disseminated intravascular coagulation, haemoglobinuria and acute renal failure. 2 of the patients were deficient in erythrocyte glucose-6-phosphate dehydrogenase; Plasmodium falciparum was present in the blood of the third. Among the indigenous population of endemic areas, typhoid fever is the likely diagnosis in any pyrexial illness associated with haemoglobinuric renal failure.

In 5 patients of phenotype ccDEe or ccDEE, the transfusion of 2--14 U of C-positive blood was folloued 5--9 days later by haemoglobinuria lasting 2--4 days. Anti-C was the only antibody present in all 5 cases but was always of low titre; with CC cells the maximum titre recorded with the indirect antiglobulin test was 8 and with the agglutination of enzyme-treated cells, 128. The discordance between the weakness of the antibody in vivo and the amount of haemoglobin released intravascularly is surprising but may be related to the type of IgG molecule of which anti-C is composed.

A 16 years old boy had a typical exertional hemoglobinuria after walking with his new leather shoes on. The attacks of hemoglobinuria were associated with the appearance of an unstable hemoglobin in red cells. A slightly decreased fragility curve by 24 hr incubated red cells, the increase of autohemolysis and the appearance of heat labile hemoglobin were observed after exertion. These findings support that exertional hemoglobinuria may be regarded as a transitory erythropathia.

Hemolysis does not necessarily result in acute renal failure in severely burned patients, but free serum hemoglobin may play some important role in the development of renal damage. This controlled study of the effects of haptoglobin administration in severely burned patients presenting with hemoglobinuria produced the following results: As long as free hemoglobin was present in the plasma, free serum haptoglobin remained undetectable. Free serum hemoglobin dropped rapidly after haptoglobin treatment, whereas the free serum hemoglobin levels in control patients remained unchanged for at least 12 hours. The time required for macroscopic hemoglobinuria to clear showed a statistically significant difference between the haptoglobin-treated patients and the control patients. Some patients among the haptoglobin-treated group had prolonged hemolysis and hemoglobinuria which might have cleared with additional doses of haptoglobin.

This report of a runner in whom progressive macrocytosis developed with increasing mileage deals with the hematologic adaptations to exercise, introduces the concept of "runner's macrocytosis" as a compensated hemolysis of older red cells, and makes a case for "runner's anemia" as a benefit versus "runner's hemolysis" as a detriment. It presents the characteristic hematologic profile of footstrike hemolysis and explores the influence of racing, different levels of training, and different shoes. It shows that runner's hemolysis can be reduced by reducing mileage but not necessarily by changing shoes, and it suggests that runner's hemolysis can impair race performance by preventing the attainment of an optimal red cell mass and, in time, by evolving into iron-deficiency anemia.

The clinical and hematological features of a rare autoimmune disorder, PCH, are reviewed. Based on the case reports of 24 patients suffering from this disease, the presence of cold hemolysins in the sera of these patients as the main cause for the red cell destruction following exposure to cold is further discussed. However, recent electron microscope and biochemical studies suggest, in addition, that alterations of the red cells, and especially of their membranes, may play a role in the hemolytic process. The role of other factors, such as thermal range, specificity, and the immunological properties of the Donath-Landsteiner antibodies, as well as the role of the complement in the cold and warm phase of the Donath-Landsteiner reaction, is discussed. The differential diagnosis between PCH and the closely related CHD is outlined. While most of the reports deal with the mechanism of red cell destruction, relatively little attention is given to the treatment of the disease. It appears that the oldest remedy, i.e., warming of the patient and prevention of his exposure to cold, remains the best.

Previous studies have reported a high incidence of hemoglobin E (HbE) in Northeast Indian populations. In the present study 10 endogamous populations of Assam belonging to two racial groups, Caucasoid and Mongoloid, were examined. The frequency of HbE gene (Hb beta E) in the Caucasoid caste populations is around 0.1, whereas the gene is highly prevalent in the Mongoloid populations, frequencies ranging between 0.2 and 0.6. Predominance of Hb beta E in the Tibeto-Burman speakers is contrary to observations made in Southeast Asia, where an association between Austro-Asiatic speakers and high prevalence of HbE exist. The highest occurrence of the gene in this area, which is on the far end of the proposed centre of distribution in Northern Kampuchea and Northeast Thailand, is also a deviation from the expected pattern of gene distribution. It is speculated that Hb beta E in the Tibeto-Burman populations of Assam arose by an independent mutation which contributed to the high frequencies of Hb beta E in the Northeast Indian populations.

The management of two patients with haemoglobin H disease, who underwent open-heart surgery, is described and the problems that relate to anaesthesia in the presence of this condition are discussed.

Ten months after aortic valve replacement (Björk-Shiley prosthesis) a 57-year-old man had thrombotic encapsulation of his prosthetic valve. The only clinical manifestation of this serious complication was gross hemoglobinuria. The management of the patient is described. The slightest change in the condition of a patient after prosthetic valve replacement should be viewed as a possible sign of thrombosis.

The erythrocyte membrane proteins and glycoproteins and urinary polypeptides have been examined in a patient exhibiting intermittent pigmenturia associated with conga drumming. Significant excretion of haemoglobin, albumin and probably erythrocyte carbonic anhydrase but not myoglobin occurred during the acute phase of the conga drumming-induced pigmenturia. This usually ceased within 24-48 h. We found no evidence of aberrant erythrocyte membrane components on electrophoresis with either protein staining or a range of 125I-labelled lectins used for detection.

Nine patients with intractable hemolysis caused by perivalvular leakage following mitral valve replacement with a St. Jude Medical prosthesis are presented. All patients had dark-colored hemoglobinuria, which appeared from 1 day to 44 days after the operation, with moderate or severe hepatorenal insufficiency. One patient died of multiorgan failure. The other 8 patients underwent reoperation, and all survived. Reoperation revealed that all leakages were tiny and had no adverse effect on hemodynamics. In all the patients having reoperation, hemoglobinuria disappeared immediately after the procedure. Surface-scanning electron microscopy of the sewing cuff of the St. Jude Medical prosthesis revealed the rough surface structure of the sewing ring. Because of this irregular, shaggy surface structure, greater shearing forces against erythrocytes can be generated when there is perivalvular leakage.