The metacarpal index in homozygous sickle-cell disease

The metacarpal index (MCI) was measured in 240 patients with homozygous sickle-cell (SS) disease and in 1082 normal persons constituting a comparison group. The MCI was greater in females than in males, but there appeared to be no significant difference between genotypes. Arachnodactyly may occur in some patients with SS disease, but there is no evidence of a genotype-related increase in metacarpal index.     

MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis

We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim-sulfomethoxazole, resulting in cerebral anoxia leading to permanent damage. Magnetic Resonance imaging revealed cortical laminar necrosis in arterial border zones in both cerebral hemispheres, ischemic changes in subcortical white matter of left cerebral hemisphere, and in the left putamen. Although cortical laminar necrosis is a classic entity in adulthood related to conditions of energy depletions, there are few reports available in children. A wide review of the literature is also presented.     

MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis

European Radiology - We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim–sulfomethoxazole,...     

MRI of the spleen: normal appearance and findings in sickle-cell anemia

The MRI appearance and characteristics of the normal and diseased spleen were prospectively evaluated using spin-echo imaging with a superconducting magnet operating at 0.35 T. Sixty normal patients and 13 patients with sickle-cell anemia involving the spleen were studied. The normal spleen was isointense or slightly more intense than liver at a repetition time of 0.5 sec. At a longer repetition time (TR 2.0 sec), the normal spleen was usually more intense than liver, becoming markedly hyperintense compared to liver on relatively T2-weighted images (TR 2.0 sec, TE 56 msec). All patients with sickle-cell anemia showed abnormally diminished signal intensity of the spleen.     

Papillary necrosis causing hydronephrosis in renal allograft treated by percutaneous retrieval of sloughed papilla

Obstructive uropathy is the second most common urological complication in a transplanted kidney. The usual causes of obstruction are ureteral stenosis and calculi. Papillary necrosis as a cause of obstruction in a transplant kidney is extremely rare with only one prior report published. Moreover, percutaneous removal of sloughed papilla in a transplant kidney has not previously been reported. We report an unusual case of a sloughed papilla causing hydronephrosis of a transplant kidney and its successful percutaneous removal. The recognition of renal papillary necrosis is important, not only because it can be a sign of acute rejection but also it because it can lead to obstruction, infection and potentially the loss of the transplant as exemplified by our case. Rapid diagnosis and meticulous retrieval technique are the crucial factors in minimizing the complications due to obstruction of a transplanted kidney by sloughed papilla.     

Papillary muscle head rupture in a patient with normal coronarography findings

BACKGROUND: Rupture of papillary muscle generally happens during acute myocardial infarction and is the cause of acute mitral regurgitation, pulmonary oedema, so it should be promptly recognized and managed. CASE REPORT. A patient, 52 year-old, was admitted to the Thoracic Department with fever, general weakness, dyspnea and cough as a case of suspected pneumonia. Two days before the admission he was treated with antibiotics. At thoracic ward, his clinical status got serious and he transferred to Intensive Care Unit (ICU) as pulmonary oedema. At the time of admission to ICU the patient was seriously ill with tachycardia, tachydyspnea, orthopnea and cyanosis image. Auscultatory, he showed pulmonal stasis at both sides and a tachyarrhythmic action, with a systolic murmur 5/6 grade above the mitral valve. Echocardiography showed grave mitral regurgitation with prolapsus of posterior leaflet with suspected chordal rupture. At coronarography no significant lesions of coronary arteries were found. After hemodynamic stabilization the patient was operated. During the operation, Transesophageal echocardiography (TEE) examination showed a rupture of the head of the posteromedial papillary muscle. He was surgically treated with atypical quadrantectomy of posterior leaflet with homologous pericardial patch anuloplasty. CONCLUSION: The recognition of acute mitral regurgitation caused by the papillary muscle rupture and prompt surgical treatment is of vital interest for the survival of patients.     

Thoracic extramedullary hematopoiesis in sickle-cell disease

The radiographic manifestations of thoracic extramedullary hematopoiesis are unilateral or bilateral, smooth, sharply delineated, often lobulated paraspinal masses without erosion of the vertebral bodies or ribs, sometimes associated with subpleural, paracostal masses. These radiographic findings were encountered in eight adult patients with homozygous sickle-cell disease. In one patient, the masses encompassed essentially the entire thoracic paravertebral area bilaterally. In the other seven patients, the masses were unilateral and limited to the region of the eighth to the 12th thoracic vertebrae. Two of the eight patients had lateral subpleural masses that were not contiguous with the paraspinal masses and that were located medial to the lateral portions of the ribs. Follow-up in seven of the cases ranged from 2 to 15 years and demonstrated, after relatively rapid growth initially, either no change in size or slow growth. Each patient was asymptomatic with regard to the thoracic masses. Histologic verification was not available in any case because of the lack of clinical indication for invasive diagnostic or therapeutic procedures. The presence of well-defined unilateral or bilateral paraspinal masses and/or paracostal masses in patients with homozygous sickle-cell disease and without related symptoms should alert one to the possibility of the presence of extramedullary hematopoiesis. These masses tend to be slow-growing and should not be subjected to aggressive diagnostic and therapeutic measures.