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Hospital Italiano de Buenos Aires-Argentina carlos. ABSTRACT Hematuria is the presence of an excessive number of red blood cells in the urine at least three or more erythrocytes in a high-power field in centrifuged urine.
It is categorized as microscopic when it is visible only with the aid of a microscope and macroscopic when the urine is tea-colored, pink or even red. Hematuria can result from injury to the kidney or injury to another site in the urinary tract, and renal hematuria can be caused by glomerular or non-glomerular disease.
In the present report we presented a clinical case of a patient suffering from persistent and anemizing gross hematuria secondary to a mesangial proliferative glomerulonephritis associated with thick glomerular basement membranes which was solved using enalapril.
Only 5 ml of blood are needed to give pink macroscopic hematuria, while twice this blood volume will give easily visible hematuria 2. Even though the significance and seriousness of the finding of microscopic hematuria is independent of the number of the red blood cells, there is an increase in significant and life-threatening findings in relation to the quantity of blood in the urine if macroscopic hematuria is included in the analysis 2. The first step in hematuria interpretation is to determine if it is of glomerular or non-glomerular origin.
In this report we presented a curious case of a persistent anemizing macroscopic hematuria of glomerular etiology which was solved using enalapril. It had begun one year before suddenly with an episode of macroscopic hematuria that lasted three days. It disappeared spontaneously the first two times and the third time that it reappeared was two months before referral. That time the gross hematuria remained. This gross hematuria was never related to the presence of fever, sore throat nor to any infectious event.
The patient had neither hypertension nor familiar antecedents of hematuria. At the time of the nephrological consult we noticed that his urine had no clots and its red cells seemed to be of glomerular origin since it had a high proportion of dysmorphic red blood cells and acanthocytes TABLE I.
Departament of Nephrology. Universidad de Navarra. Cuba ABSTRACT Hematuria is the presence of an excessive number of red blood cells in the urine at least three or more erythrocytes in a high-power field in centrifuged urine. Renal imaging: ultrasound and TC scan were normal. Despite the glomerular pattern of his hematuria, it was so gross that it appeared as a consequence of an active bleeding state.
Because of that clotting studies and renal angiography, cystoscopy and intravenous pyelography were performed being all these studies normal. Urinary infections included urinary tuberculosis were ruled out. Besides, he was neither on any drug that could generate papillary necrosis non-steroidal anti-immflamatories, etc. A factitious hematuria was suspected but it did not explain the finding of dysmorphic blood red cells in his urine. A renal biopsy was performed being the result as follows: optic microscopy was normal and immunological deposits were absent an 8 glomeruli sample.
Electronic microscopy showed irregular basament membranes, some of them thickened. However, there were no membrane lamination, lamina densa fragmentation or concomitant areas of glomerular membrane thinness. There was a mild increase of the mesangial matrix without deposits, tubules with occasional atrophy, interstitium with mild and focal fibrosis and normal vessels.
From this renal histology his hematuria was interpreted as secondary to a mesangial proliferative glomerulonephritis. Regarding the documented increased in the basament membrane thickness, it was not interpreted as secondary to Alport syndrome since its typical histologic pattern was absent: membrane lamination, lamina densa fragmentation and concomitant areas of glomerular basement membrane thinness.
Moreover, hematologic, neurologic, oftalmologic and otorhinolaryngologic evaluation looking for alterations compatible with Alport syndrome were all normal. A month later the hematuria has disappeared.
This result persisted until the time of the present report one year later. The former because an old age can evoke a neoplastic etiology, and the latter because the presence of a urine with clots and isomorfic red blood cells suggests a non-glomerular disease 3.
This initial approach enable the physician to organize the sequence of the following diagnostic tests. For instance, if a glomerular origin of the hematuria is suspected the logic procedure would be a renal biopsy, whereas if an urological cause is suspected the adequate study would be an urological endoscopy 2.
In the present clinical case the patient suffered from a persistent macroscopic hematuria without clots, with proteinuria and dismorfic urinary red blood cells. Even though most of the features of his hematuria were compatible with a glomerular origin, because of its persistence and the fact that it had led him to anemia both features strongly related to non-glomerular etiology , we started his evaluation performing coagulation studies, renal angiography, cystoscopy and intravenous pyelography in order to rule out a bleeding disorder, a renal vascular malformation or an urological lesion respectively.
Studies for diagnosing paroxysmal nocturnal hemoglobinuria such as Ham test and sucrose test were also performed being all of them normal. Since all these studies were normal and there were findings supporting a glomerular origin of the hematuria ie: urinary dysmorphic red blood cells afterwards, we decided to perform a renal biopsy. Its result was compatible with a mesangial proliferative glomerulonephritis with concomitant presence of thick glomerular basement membranes 4.
Despite of the aforementioned concepts, a borderline sort of Alport syndrome can not be ruled out. Angiotensin converting enzyme inhibitors such as enalapril are used for treating hyperfiltration.
Besides, in the thin glomerular basement membrane disease, it has already been proposed to use angiotensin converting enzyme inhibitor as a means to reduce its associated hematuria. This strategy would be based on the reduction of the intra-glomerular pressure with the objective of minimizing the red blood cells passage through the altered glomerular barrier 6. Renal Diseases. Cecil Textbook of Medicine.
In Cameron S Ed. Oxford Textbook of Nephrology. Oxford University Press. Primary glomerular disease. Alport Syndrome: an inherited disorder of renal, ocular, and cochelear basement membranes. Hematuria and pigmenturia.
In Massry S, Glassock R. Textbook of nephrology. Hereditary nephritis: Alport syndrome and thin glomerular basement membrane disease. Renal Pathology. JB Lippincott Company. Ahora bien, en la biopsia renal realizada en ese este caso se advierte un resultado un tanto sorprendente. Referencias: 1.
J Pharmacol Exp Ther. Epub Sep 3. Glomerular abundance of nephrin and podocin in experimental nephrotic syndrome: different effects of antiproteinuric therapies. Am J Physiol Renal Physiol. Epub Jun 7. J Am Soc Nephrol. Renoprotective effect of early inhibition of the renin-angiotensin system in renal transplant recipients. Transplant Proc. Enalapril in paediatric patients with Alport syndrome: 2 years' experience. Eur J Pediatr. Proesmans W, Van Dyck M.
Enalapril in children with Alport syndrome. Pediatr Nephrol. Epub Jan ACE inhibitorenalapril diminishes albuminuria in patients with cystinosis.
Clin Nephrol. Loin pain-hematuria syndrome associated with thin glomerular basement membrane disease and hemorrhage into renal tubules. Kidney Int. Department of Nephrology. Cuba Se presenta un caso interesante, un adulto joven con macrohematuria continua que anemiza, que recuerda a la hematuria del tracto urinario excretor o de causas renales no glomerulares, con un sedimento urinario con elementos que se ven asociados a enfermedad glomerular.
En: Tratado de enfermedades renales. Greenberg A.
However, some uncertainties still exist in the literature about their morphological definition. The aim of this paper is to discuss this topic and to suggest a univocal definition of acanthocytes as erythrocytes with the shape of a ring with one or more protrusions.. In , Fairley and Birch described dysmorphic red blood cells and proposed their use to distinguish glomerular from non-glomerular haematuria. However, in spite of this, a univocal definition of acanthocyte is still missing. The aims of this paper are 1.