Gross hematuria in sickle cell trait and sickle cell hemoglobin-C disease

Chapman, A; Reeder, Paul S.; Friedman, Irving A.; Baker, Lyle A.

doi:10.1016/s0002-9343(55)80022-8

Cited by 49 publications

(9 citation statements)

References 18 publications

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“…J., 1967, 3, 283-284 Abel and Brown (1948) first drew attention to profuse haematuria in sickle-cell anaemia. Subsequent reports have confirmed that this association is more than fortuitous (Harrison and Harrison, 1952;Chapman et al, 1955 ;Sharpe et al, 1959). Haematuria is also known to occur in sickle-cell trait, sickle-cell haemoglobin C disease, sickle-cell thalassaemia, and a variety of combinations of haemoglobin S with other abnormal haemoglobins.…”

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confidence: 85%

Renal papillary necrosis in sickle-cell haemoglobinopathy.

Akinkugbe¹

1967

BMJ

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Abel and Brown (1948) first drew attention to profuse haematuria in sickle-cell anaemia. Subsequent reports have confirmed that this association is more than fortuitous (Harrison and Harrison, 1952;Chapman et al., 1955 ; Sharpe et al., 1959). Haematuria is also known to occur in sickle-cell trait, sickle-cell haemoglobin C disease, sickle-cell thalassaemia, and a variety of combinations of haemoglobin S with other abnormal haemoglobins. The present report of four cases of haematuria in sickle-cell haemoglobinopathies is prompted for two reasons. Firstly, detection of blood in the urine of young adults in an environment endemic for schistosomiasis need not always indicate the presence of the infection. Of more practical importance is the surgical diagnostic problem sometimes posed by gross haematuria simulating renal neoplasm. Awareness of the possibility that this symptom may exist in sickle-cell haemoglobinopathy makes careful appraisal necessary, to avoid the inadvertent sacrifice of an apparently normal-functioning kidney. (Fig. 1). Her haematuria cleared gradually within a fortnight and she has since remained well and active.Case 2 A tailor aged 47 was subject to recurrent episodes of painless haematuria for two months. Examination revealed severe anaemia (Hb 3.0 g./100 ml.). His urine contained a trace of albumin and its S.G. was 1006; red blood cells were detected at microscopy, but there were no casts. Blood group was O-positive, sickling was present, and his genotype AS. His blood urea was 16 mg./100 ml. Cystoscopy showed blood spurting from the left ureteric orifice. Left ureteric catheterization was followed by a retrograde pyelogram, after which a nephrectomy was performed. Naked-eye appearance of the kidney showed marked congestion of the vessels close to the papillae, with softening of the central and apical portions of the pyramid; there were blood clots in the renal pelvis. Microscopy did not reveal any abnormalities in either glomerular or tubular morphology. He remained well for five years, but has lately developed proteinuria and hypertension. The latter event has been complicated by congestive heart failure, but this has responded well to digoxin, thiazides, and reserpine. At the time of writing he remained practically symptom-free.

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confidence: 85%

Renal papillary necrosis in sickle-cell haemoglobinopathy.

Akinkugbe¹

1967

BMJ

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“…The fact that it is seen in sickle-cell trait suggests that the physiological milieu in the blood vessels of the kidney is particularly favourable to sickling. Haematuria in sickle-cell haemoglobin C disease was reviewed by Chapman, Reeder, Friedman, and Baker (1955). It is characteristically gross and painless, ceasing spontaneously but tending to recur.…”

Section: Haematuriamentioning

confidence: 99%

Sickle-cell haemoglobin C disease in London

Black¹,

Condon²,

Gompels³

et al. 1972

J Clin Pathol

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The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented in London, two of which were fatal. The special hazards of the condition in relation to pregnancy, air travel, and general anaesthesia are discussed.

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“…The distribution of the small wedge-shaped infarcts at the upper, middle, and lower zones of the kidney would tend to support a haematological abnormality such as sickling rather than thrombosis or organic narrowing of a major renal vessel. Infarcts of the renal papillae (Chapman et al, 1955 ;Akinkugbe, 1967), lungs (Moser and Shea, 1957), and spleen during high altitude flying (Rotter et al, 1956) are well-recognized complications of the sickle-cell trait.…”

Section: Commentmentioning

confidence: 99%

“…

The renal complications of sickle-cell trait are hyposthenuria (Zarafonetis et al, 1954) and massive haematuria from renal papillary necrosis (Chapman et al, 1955; Akinkugbe, 1967). These and several other reports have shaken the view that sicklecell trait is a benign condition.

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Renal cortical infarcts in sickle-cell trait.

Femi-Pearse¹,

Odunjo²

1968

BMJ

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The renal complications of sickle-cell trait are hyposthenuria (Zarafonetis et al., 1954) and massive haematuria from renal papillary necrosis (Chapman et al., 1955; Akinkugbe, 1967). These and several other reports have shaken the view that sicklecell trait is a benign condition. Also, Raper (1952Raper ( , 1953 claimed that cicatricial obliteration of glomeruli, which he observed from histological examination of necropsy kidneys, was often associated with sickle-cell trait.Though several cases of haematuria from renal papillary necrosis have been documented in patients with sickle-cell trait, nothing has been written about renal cortical infarcts in these patients. After a few days in hospital he gave a history of having had good health until mid-August 1967, when he felt a dull pain of gradual onset in the low back and left loin. He attended another hospital for treatment. The pain disappeared within a week. Three days before the present admission he felt dizzy on standing upright and was breathless and had palpitations on exertion.Investigations.-The following were found on his admission: Hb 9.8 g./100 ml.; P.C.V. 33% ; sickling positive; Hb genotype A.S.; E.S.R. 79 mm. in the first hour; blood urea 62 mg./100 ml.; serum HCO3-21 mEq/l.; Cl-96 mEq/i.; Na+ 132 mEq/l., K+ 4.2 mEq/l. Antistreptolysin 0 titre, 100 Todd units; blood W.R.and Kahn test were negative; serum cholesterol was 233 mg./100 ml. Chest x-ray examination showed an enlarged heart with pulmonary oedema. E.C.G. was of left ventricular hypertrophy pattern. Urine microscopy and culture were normal. With control of left ventricular failure the blood urea fell to 30 mg./100 ml. Intravenous pyelography (I.V.P.) showed a non-functioning contracted left kidney; the right kidney was normal. Cystoscopy showed a very small left ureteric orifice. Retrograde pyelography was therefore not performed. Facilities for aortography were not available at that time. The 24-hour urinary catecholamines were normal.Operative Findings (Professor A. 0. Adesola).-On 26 October the left kidney was explored; it appeared to be of normal size. Three branches of the renal artery were identified, but the superior branch, which appeared to be the largest, was not pulsating. Nephrectomy was performed.Pathological Findings.-The resected kidney measured 8 by 4 by 2 cm. (see Fig.). The capsule stripped easily, revealing a smooth brownish-red surface with three wedge-shaped yellowish infarcted areas depressed for 2 to 3 mm. below the renal surface. One was at the upper pole and the others in the middle and lower zones. On sectioning, the renal vessels were seen to be filled with clotted blood. The cortex and medulla were clearly demarcated. Sections from various parts of the kidney showed large, small, and mediumsized vessels full of red cells with sickle appearances. Many glomeruli also showed plugging of glomerular capillaries with sickle cells. Some glomeruli were of normal size, while others were small and showed partial or total obliteration of the tufts. In some fields the glome...

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Gross hematuria in sickle cell trait and sickle cell hemoglobin-C disease

Cited by 49 publications

References 18 publications

Renal papillary necrosis in sickle-cell haemoglobinopathy.

Renal papillary necrosis in sickle-cell haemoglobinopathy.

Sickle-cell haemoglobin C disease in London

Renal cortical infarcts in sickle-cell trait.

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