Prognostic aspects of scintigraphy and MRI during the first 6 months of reflex sympathetic dystrophy of the distal lower limb: a preliminary prospective study of 4 cases]

PURPOSE: The appearance of scintigraphic and magnetic resonance imaging during the evolution of reflex sympathetic dystrophy of the foot is not well known and subject to controversies. The purpose of this preliminary study is to compare these two types of investigation during the first 6 months of evolution. METHODS: 4 non-selected patients with a diagnosis of acute reflex sympathetic dystrophy of the foot (no more than 2 months of evolution and more than 3 out of 6 clinical criteria suggesting a "warm" phase) were studied prospectively. A clinical evaluation, scintigraphy and magnetic resonance imaging (MRI) were performed at diagnosis and after 3 and 6 months. All available examinations (n = 22) were analyzed independently by independent observers. RESULTS: Clinically 3 of 4 patients developed or previously had another site of sympathetic dystrophy confirmed by total body scintigraphy. After 6 months 2 of 4 patients still have a "warm" dystrophy. Bone scanning showed localized or diffuse tracer uptake and MRI showed bone and soft tissue edema when the sympathetic dystrophy was and/or remained clinically "warm". MRI bone edema moved from one location to another in 3 of 4 patients during the follow-up. There was a good correlation between bone scan and MRI images. In "warm" dystrophy, MRI was positive 5 out of 7 times and scintigraphy in all cases. Bone edema and tracer uptake faded simultaneously although the latter was more diffuse and more persistent than the former. CONCLUSION: MRI as well as bone scintigraphy are suitable for the diagnosis of warm dystrophy of the foot. Both exams remain positive when the dystrophy is still "warm" after 6 months. The radiographic evolution after 6 months still has to be evaluated.     

SAPHO syndrome masquerading as metastatic bone disease

A 46-year-old woman who had had a right mastectomy for breast carcinoma a month before underwent bone scintigraphy. The examination revealed multiple pelvic, vertebral and sternal hot spots suggestive of bone metastases. Standard X-rays and CT confirmed the presence of bony lesions but they were not typical of bone metastases. As the radiographic appearance was reminiscent of SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), bone biopsies were performed. Histology showed fibrosis and hyperostosis but no tumour cells. On further questioning, the patient revealed she had had palmar pustulosis and sacroiliitis some years earlier. The purpose of the case report is to show that accurate diagnosis of SAPHO syndrome requires careful clinical and radiological examinations.     

Febrile osseous pain in children with sickle cell disease: MRI findings

PURPOSE: The aim of this study was to report the MRI findings that can suggest a vaso-occlusive crisis in cases of febrile osseous pain in children suffering from sickle cell disease. MATERIALS AND METHODS: MRI (T1 and T2 weighted sequences and T1 weighted sequence with fat saturation before and after gadolinium injection) was performed in 10 children with sickle cell disease, presenting with febrile osseous pain. The diagnosis of vaso-occlusive crisis was made after fast improvement due to symptomatic treatment and negative bacteriological result. RESULTS: MRI was abnormal in all cases. A multifocal localisation was found in 2 cases. Bone marrow abnormalities were constant. In 10 cases, high T1 and T2 signal and metaphyso-diaphysial lesions were noted. Heterogeneous medullar enhancement with "ink stain" feature was constant. Early periosteal abnormalities were noted in 8 cases with inflammatory or stratified features. Cortical thinning was found in 1 case. Soft tissue abnormalities were observed in 5 cases with inflammatory features in 4. CONCLUSION: Multifocal synchronous localisation, medullar abnormalities resulting from hemoglobin degradation, heterogeneous enhancement, early periosteal abnormalities and associated soft tissues swelling are MRI findings suggesting acute vaso-occlusive disease.     

Pulmonary hypertension in sickle cell anemia. A case report

We report the case of a 33-years-girl with SCA and severe PH. She developed six month before admission, non productive cough and dyspnea. Physical examination at admission revealed shortness of breath and right heart ventricular failure. Electrocardiography showed sinus rhythm and an incomplete left bundle branch block. Chest roentgenography revealed cardiomegaly with cardiothoracic index at 0.66 and pulmonary infiltrates. Laboratory tests revealed an anemia with hemoglobin of 7.1 g/dl, white blood cell count of 12,500/mm, moderate renal failure (cretininemia = 178 mumol/l) and hypoxemia with oxygen pressure of 60 mmHg. Hemoglobin electrophoresis revealed on heterozygous SCA. Echocardiography revealed dilatation of right heart cavities and a systolic pulmonary artery pressure of 60 mmHg. A perfusion lung scintigraphy demonstrated multiple subsegmental perfusion defects. PH is a common complication of adult patients with SCA. Appropriate therapies and strategies for prevention of PH in SCA are unknown. Further research exploring therapies such as oxygen, nitric oxide, prostacyclin and hydroxyurea are indicated.     

Renal amyloidosis followed more than 5 years: report of 12 cases

Renal involvement with amyloidosis is common but causes patient survival to be poor, rarely reaching 5 years. In this study, we retrospectively reviewed clinical and biological characteristics as well as treatments and outcomes of patients with renal amyloidosis followed for more than 5 years. Between 1975 and 2003, 485 patients were diagnosed with renal amyloidosis including only 12 patients who were followed more than 5 years. The six men and six women of mean age 42.4 years (range 18 to 66 years) displayed renal signs of lower limb edema in all cases; hypertension in four cases, proteinuria on urinalysis in all cases with microscopic hematuria in five cases. Biological tests showed nephrotic syndrome in 11 patients, normal renal function in nine patients, and renal failure in three patients whose mean creatinine was 481.6 micromol/L (range 294 to 726). The amyloidosis was AA type in 11 cases and non-AA in one case. An etiologic survey revealed spondylarthropathy in one patient, pulmonary tuberculosis in two patients, chronic bronchitis in three patients, hepatic hydatic cyst in one patient, Mediterranean familial fever in two patients, Crohn's disease in one patient, Hodgkin's lymphoma in one patient, and multiple myeloma in one patient. Specific treatment was initiated with colchicine in seven patients. At a 110-month mean follow-up (range 53 to 153 months), remission of nephrotic syndrome was observed in four cases, progression to chronic renal failure in two patients, and to end-stage renal failure in five cases (range 53 to 196 months), with stabilization of renal function in seven patients. In conclusion, primary amyloid disease should be optimally suppressed in patients with renal involvement. The role of this treatment in remission of renal amyloidosis is not well established. This efficacy of the treatment has been demonstrated in some patients with improved survival.     

Gastric cancer with lymphoid stroma: a rare form associated with Epstein-Barr virus. Five cases

We report the cases of 5 patients with gastric cancer with lymphoid stroma, aged from 40 to 66 years. The tumor was located in the upper and the middle third of stomach in four patients. Using in situ hybridization, the tumor was Epstein-Barr virus-positive in all patients whereas immunohistochemical analysis was negative. Four patients had total gastrectomy associated in two cases with splenectomy and caudal pancreatic resection. The last patient had subtotal gastrectomy. One patient was lost to follow-up. After a mean follow-up of 31 months, 4 patients are alive and free of local recurrence.     

A programmed autologous transfusion. A study of 47 patients

From December 1994 to January 2002, 44 among the 47 patients proposed have been integrated in the protocol of programed autologous transfusion. 18 patients were belonged to the male sex and 26 others belonged to the female one. The average age was 53.2 years (range 15-82 years old). 39 among the patients admitted in the protocol had an orthopaedic pathology. The protocol has associated the teams of surgery, of anesthesia and of transfusion and has occurred in the respect of the regulation (circular 91/2000). The blood taking have led to a significant modification of the hemoglobin rate (average decrease of 2 g/100 ml), of hematocrit (average decrease of 6.1%) and of the rate of platelets (average increase of 29,324 platelets/mm3). On the other hand, a transfusional complement by the concentrated homologous red corpuscles was necessary for 2 patients (2/40).     

Haemophilus influenzae purulent meningitis in adults: looking for a predisposing factor

We bring back an adult case of purulent meningitis to Haemophilus influenzae. We insist on the particular aspects of the host of this meningitis type at the adult. These aspects must be searched every time that Haemophilus influenzae is isolated in cerebrospinal fluid in adult's meningitis.