Radiographic Exhibit
papillary necrosis in adults with either homozygous sickle-cell disease (S-S) or sickle-cell hemoglobin C disease (S-C) (2-4, 9). Our patient appears to be the first child with homozygous S-S sickle-cell anemia to have papillary necrosis. The diagnosis is usually established by excretory urography and histological examination of papillae voided in the urine. The histopathology has been documented by Mostofi et el., who examined kidneys removed from adults with sickle-cell disease and found congestion, sickled erythrocytes, peritubular hemorrhage, and ischemic necrosis of the medullary pyramids (7). Bernstein and Whitten (1) subjected the kidneys removed from children with S-S anemia to histological examination and demonstrated scarring and obliteration of the tubular elements in the medullary pyramids; however, no active necrosis or papillary inflammation was seen, although loss of substance of several pyramids was described in one case. The pathogenesis of papillary necrosis is usually related to vascular injury with resulting coagulation necrosis. The medulla tends to be the site of pathological change because of sluggish blood flow in the vasa recta and increased osmolality of sodium and chloride ions, leading to stasis and decreased oxygen tension (6).
Papillary Necrosis in a Child with Homozygous Sickle-Cell Anemia 1 Vivian J. Harris, M.D., Nihal S. Gooneratne, M.D., and Harvey White, M.D. A 4 %-year-old girl with S-S sickle-cell anemia was found to have papillary necrosis on excretory urography. This is believed to be the first reported case of papillary necrosis in a child with homozygous S-S sickle-cell anemia. INDEX TERMS:
Kidneys, necrosis, 81.216 • Sickle Cell Disease (SS, SC), 48.651;
81.651 Radiology 121:156, October 1976
evidence of renal abnormalities in children with sickle-cell anemia has rarely been described in the literature. We report a case in which radiographic evidence of renal papillary necrosis was seen in a 4%-year-old child. ADIOGRAPHIC
R
CASE REPORT A 4 Y2-year-old black girl presented with S-S sickle-cell anemia. Both parents had the sickle-cell trait, and an older brother also had S-S sickle-cell anemia. At 7 months of age the patient was found to be anemic, with a hemoglobin level of 5.0 g and a hematocrit of 19 mg/1 00 ml. Her sickle-cell preparation was strongly positive, and the peripheral smear showed sickle cells. Hemoglobin electrophoresis showed 77 % S hemoglobin. She had several episodes of dactylitis and hand-foot syndrome over a two-year period and also exhibited cardiomegaly with a blowing systolic murmur secondary to anemia and a stretched mitral valve. Two bouts of urinary tract infection, one of which gave a positive culture for Escherichia coli, were successfully treated with ampicillin. Excretory urography following the last infection showed medullary papillary necrosis (Fig. 1).
Department of Radiology Cook County Children's Hospital Chicago, Ill. 60612
REFERENCES 1. Bernstein J, Whitten CF: A histologic appraisal of the kidney in sickle cell anemia. Arch Pathol 70:407-418, Oct 1960 2. Harrow BR, Sloane JA, Liebman NC: Roentgenologic demonstration of renal papillary necrosis in sickle-cell trait. N Engl J Med 268:969-976,2 May 1963 3. Kay CJ, Rosenberg MA, Fleisher P, et al: Renal papillary necrosis in hemoglobin SC disease. Radiology 90:897-899, May 1968 4. Margulies SI, Minkin SO: Sickle cell disease. The roentgenologic manifestations of urinary tract abnormalities in adults. Am J RoentgenoI107:702-710, Dec 1969 5. Marquis JR, Khazem B: Sickle-cell disease. Renal roentgenographic changes in children. Radiology 98:47-52, Jan 1971 6. Mellins HZ: Chronic pyelonephritis and renal medullary necrosis. Semin Roentgenol 6:292-309, Jul 1971 7. Mostofi FK, Vorder Bruegge CF, Diggs LW: Lesions in kidneys removed for unilateral hematuria in sickle-cell disease. Arch Pathol 63:336-351, Apr 1957 8. Plunket DC, Leiken SL, LoPresti JM: Renal radiologic changes in sickle cell anemia. Pediatrics 35:955-959, Jun 1965 9. Vix VA: Urographicdemonstration of renal medullary necrosis in hemoglobin SA and SC disease. Radiology 85:320-324, Aug 1965
DISCUSSION Marquis and Khazem studied the excretory urograms of 18 children with homozygous sickle-cell disease (S-S) 4 to 16 years of age and observed faint visualization, calyceal blunting and irregularity, and bilateral renal enlargement (5). Plunket et al. studied 25 children 10 months to 16 years of age who had either S-S or heterozygous S-A sickle-cell anemia and found calyceal blunting in many of them (8). We know of no reported instance of renal papillary necrosis in children with sickle-cell anemia. Roentgenographic demonstration of renal papillary necrosis in adults with the sickle-cell trait was first described by Harrow et al. in 1963 (2). Since then there have been several reports of
Fig. 1. Numerous small, dense oval medullary cavities are present in both kidneys, more prominenton the right side (arrow). An additional ring shadow is seen on the inferior calyx on the right.
1 From the Departments of Pediatric Radiology, Cook County Children's Hospital and Children's Memorial Hospital, and Hektoen Institute of Research, Chicago, III. Accepted for publication in February 1976. sjh
156
papillary necrosis in adults with either homozygous sickle-cell disease (S-S) or sickle-cell hemoglobin C disease (S-C) (2-4, 9). Our patient appears to be the first child with homozygous S-S sickle-cell anemia to have papillary necrosis. The diagnosis is usually established by excretory urography and histological examination of papillae voided in the urine. The histopathology has been documented by Mostofi et el., who examined kidneys removed from adults with sickle-cell disease and found congestion, sickled erythrocytes, peritubular hemorrhage, and ischemic necrosis of the medullary pyramids (7). Bernstein and Whitten (1) subjected the kidneys removed from children with S-S anemia to histological examination and demonstrated scarring and obliteration of the tubular elements in the medullary pyramids; however, no active necrosis or papillary inflammation was seen, although loss of substance of several pyramids was described in one case. The pathogenesis of papillary necrosis is usually related to vascular injury with resulting coagulation necrosis. The medulla tends to be the site of pathological change because of sluggish blood flow in the vasa recta and increased osmolality of sodium and chloride ions, leading to stasis and decreased oxygen tension (6).
Papillary Necrosis in a Child with Homozygous Sickle-Cell Anemia 1 Vivian J. Harris, M.D., Nihal S. Gooneratne, M.D., and Harvey White, M.D. A 4 %-year-old girl with S-S sickle-cell anemia was found to have papillary necrosis on excretory urography. This is believed to be the first reported case of papillary necrosis in a child with homozygous S-S sickle-cell anemia. INDEX TERMS:
Kidneys, necrosis, 81.216 • Sickle Cell Disease (SS, SC), 48.651;
81.651 Radiology 121:156, October 1976
evidence of renal abnormalities in children with sickle-cell anemia has rarely been described in the literature. We report a case in which radiographic evidence of renal papillary necrosis was seen in a 4%-year-old child. ADIOGRAPHIC
R
CASE REPORT A 4 Y2-year-old black girl presented with S-S sickle-cell anemia. Both parents had the sickle-cell trait, and an older brother also had S-S sickle-cell anemia. At 7 months of age the patient was found to be anemic, with a hemoglobin level of 5.0 g and a hematocrit of 19 mg/1 00 ml. Her sickle-cell preparation was strongly positive, and the peripheral smear showed sickle cells. Hemoglobin electrophoresis showed 77 % S hemoglobin. She had several episodes of dactylitis and hand-foot syndrome over a two-year period and also exhibited cardiomegaly with a blowing systolic murmur secondary to anemia and a stretched mitral valve. Two bouts of urinary tract infection, one of which gave a positive culture for Escherichia coli, were successfully treated with ampicillin. Excretory urography following the last infection showed medullary papillary necrosis (Fig. 1).
Department of Radiology Cook County Children's Hospital Chicago, Ill. 60612
REFERENCES 1. Bernstein J, Whitten CF: A histologic appraisal of the kidney in sickle cell anemia. Arch Pathol 70:407-418, Oct 1960 2. Harrow BR, Sloane JA, Liebman NC: Roentgenologic demonstration of renal papillary necrosis in sickle-cell trait. N Engl J Med 268:969-976,2 May 1963 3. Kay CJ, Rosenberg MA, Fleisher P, et al: Renal papillary necrosis in hemoglobin SC disease. Radiology 90:897-899, May 1968 4. Margulies SI, Minkin SO: Sickle cell disease. The roentgenologic manifestations of urinary tract abnormalities in adults. Am J RoentgenoI107:702-710, Dec 1969 5. Marquis JR, Khazem B: Sickle-cell disease. Renal roentgenographic changes in children. Radiology 98:47-52, Jan 1971 6. Mellins HZ: Chronic pyelonephritis and renal medullary necrosis. Semin Roentgenol 6:292-309, Jul 1971 7. Mostofi FK, Vorder Bruegge CF, Diggs LW: Lesions in kidneys removed for unilateral hematuria in sickle-cell disease. Arch Pathol 63:336-351, Apr 1957 8. Plunket DC, Leiken SL, LoPresti JM: Renal radiologic changes in sickle cell anemia. Pediatrics 35:955-959, Jun 1965 9. Vix VA: Urographicdemonstration of renal medullary necrosis in hemoglobin SA and SC disease. Radiology 85:320-324, Aug 1965
DISCUSSION Marquis and Khazem studied the excretory urograms of 18 children with homozygous sickle-cell disease (S-S) 4 to 16 years of age and observed faint visualization, calyceal blunting and irregularity, and bilateral renal enlargement (5). Plunket et al. studied 25 children 10 months to 16 years of age who had either S-S or heterozygous S-A sickle-cell anemia and found calyceal blunting in many of them (8). We know of no reported instance of renal papillary necrosis in children with sickle-cell anemia. Roentgenographic demonstration of renal papillary necrosis in adults with the sickle-cell trait was first described by Harrow et al. in 1963 (2). Since then there have been several reports of
Fig. 1. Numerous small, dense oval medullary cavities are present in both kidneys, more prominenton the right side (arrow). An additional ring shadow is seen on the inferior calyx on the right.
1 From the Departments of Pediatric Radiology, Cook County Children's Hospital and Children's Memorial Hospital, and Hektoen Institute of Research, Chicago, III. Accepted for publication in February 1976. sjh
156
