Brain perfusion SPECT in patients with PHACES syndrome under propranolol treatment.

INTRODUCTION: Children with PHACES syndrome (PS) and visual impairment or stridor show a dramatic and immediate response to propranolol. However, this beta-blocking drug could be responsible for an eventually increased risk of ischemic stroke due to the underlying cerebral vascular disease. To more accurately understand the effects of propranolol on brain vascularization, we examined PS patients treated with this drug for airway or visual complications using brain perfusion SPECT (Single Photon Emission Computed Tomography). In the past, this examination has been shown to be useful in the management of patients with different neurovascular disorders. METHODS: Clinical records and imaging studies were reviewed in 7 patients with a diagnosis of PS. All patients underwent magnetic resonance angiography (MRA), echocardiography, chest X-ray and ophthalmologic, neurological, and cardiologic assessments. They received 2-3 mg/kg/day propranolol in an attempt to treat stridor or avoid ophthalmologic occlusion. We performed SPECT after 3-6 months of treatment. RESULTS: SPECT showed a normal uptake in the frontal and temporal regions despite vascular abnormalities found with MRA imaging. Significant improvements of symptoms and in the volume of the hemangioma were noted in all cases without signs of a reduction of brain blood perfusion. CONCLUSIONS: Propranolol treatment was safe in our patients who did not show signs of perfusion changes. The high sensitivity for detecting functional impairment makes brain perfusion SPECT useful in the diagnosis and follow-up of patients with PS considered at risk of neurovascular impairment. Accurate knowledge of its pathophysiological basis, together with the appropriate technique and careful interpretation of reporting, will enhance the clinical use of brain SPECT in those patients.

Diagnostic value of CT, PET and combined PET/CT performed with low-dose unenhanced CT and full-dose enhanced CT in the initial staging of lymphoma.

AIM: The aim of this paper was to compare the accuracy of contrast-enhanced computed tomography (CT), positron emission tomography (PET), unenhanced low-dose PET/CT (LD-PET/CT) and full-dose enhanced PET/CT (FD-PET/CT) for the initial staging of lymphoma. METHODS: One hundred and one lymphoma patients were examined by [18F]FDG-PET/CT including unenhanced low-dose CT and enhanced full-dose CT. Each modality of PET/CT was evaluated by a nuclear medicine physician and a radiologist unaware of the other modality, while the CT and PET images were interpreted separately by another independent radiologist and nuclear medicine physician respectively. The nodal and extranodal lesions detected by each technique were compared with a reference standard. RESULTS: For nodal assessment, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+), and negative LR (LR-) of LD-PET/CT were 97%, 96%, 98%, 95%, 26 and 0.02 respectively, and those of FD-PET/CT were 97%, 97%, 98%, 95%, 36 and 0.02. These results were significantly better than those of PET (sensitivity 82%, specificity 81%, PPV 88%, NPV 72%, LR+ 4.3, LR- 0.21). Likewise, both PET/CT displayed a higher sensitivity, NPV and LR- than CT (91%, 84%, 0.1 respectively). For organ evaluation, both modalities of PET/CT also had significantly better sensitivity and NPV than that of PET (LD-PET/CT: sensitivity 92%, NPV 90%; FD-PET/CT sensitivity 94%, NPV 92%; PET: sensitivity 70%, NPV 69%). The sensitivity, specificity, PPV and NPV for bone marrow involvement were 29%, 84%, 45% and 72% respectively for PET, and 29%, 90%, 56%, and 74% for both, LD-PET/CT, and FD-PET/CT. No significant differences were found between LD-PET/CT and FD-PET/CT, but FD-PET/CT detected important incidental findings in 5.9% of patients. CONCLUSION: PET/CT is an accurate technique for the initial staging of lymphomas without significant differences between LD-PET/CT and FD-PET/CT. FD-PET/CT detects relevant incidental findings that are missed on LD-PET/CT.

Radioactive synoviorthesis for the treatment of haemophilic synovitis.

Radioactive synoviorthesis, with (90)Y (knees) and (186)Rh (elbows and ankles) is a highly effective procedure that decreases both the frequency and the severity of recurrent intra-articular bleeds related to joint synovitis. The procedure should be performed as soon as possible to minimize the degree of articular cartilage damage, which based on many studies is irreversible. It can also be used in patients with inhibitors. On average, radioactive synoviorthesis has a 75-80% satisfactory outcome in the long-term. From the clinical standpoint, such efficacy can be measured by the decrease in the number of haemarthroses, with complete cessation for several years in some cases. One should bear in mind that in 20-25% of cases, radioactive synoviorthesis fails to control haemarthroses. In such cases, it can be repeated. Personal experience and the general recommendation among orthopaedic surgeons and haematologists is that when three early consecutive radioactive synoviortheses (repeated every three months) fail to halt synovitis, a surgical synovectomy (open or by arthroscopy) should be immediately considered.

Falso positivo de divertículo de Meckel debido a riñón ectópico / False positive of Meckel's diverticulum due to ectopic kidney

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Abnormalities in the bone mineral metabolism in HIV-infected patients.

OBJECTIVE: To evaluate bone mineral metabolism in HIV infected and asymptomatic patients receiving highly active antiretroviral therapy (HAART) containing protease inhibitors (PI) and naïve patients. METHODS: We studied 30 asymptomatic HIV infected male patients, 13 in the naive group and 17 in the IP group, both without differences in demographics characteristics. We excluded women and patients with any known factor associated to osteopenia. We did a nutritional questionnaire, a DEXA scan in lumbar spine and femur, a study of CD4 lymphocytes, viral load and an analysis of bone formation and resorption markers in all patients. We compared vitamin D and PTH levels with a control group of healthy male volunteers age-pareated. For the statistical analysis we used the SPSS program. RESULTS: Osteopenia was present in 17/30 (57%), 8/13 (61.5%) in the naïve group and 9/17 (53%) in the PI group (not significant differences). We found a vitamin D deficiency in 86% of patients, with mean serum levels that was found to be significantly lower than those from a healthy control group (p=0.04). Testosterone level was significantly related to bone mineral density in lumbar spine (p

Quiste del colédoco: diagnóstico mediante gammagrafía hepatobiliar / Choledochal cyst: diagnosis by hepatobiliary scintigraphy

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[High prevalence of vitamin D deficiency in postmenopausal women at a rheumatology office in Madrid. Evaluation of 2 vitamin D prescription regimens]. / Alta prevalencia de deficiencia de vitamina D en mujeres posmenopáusicas de una consulta reumatológica en Madrid. Evaluación de dos pautas de prescripción de vitamina D.

BACKGROUND: Vitamin D deficiency has been frequently observed in the elderly population in Europe. However few information is available about the vitamin D status in postmenopausal women in the Mediterranean countries. The aim of this study was to evaluate the vitamin D status assessed by serum 25(OH)D3 (calcidiol) in postmenopausal women who attended a Rheumatology practice in Madrid area, and to evaluate calcidiol serum levels through one year after two forms of vitamin D administration. PATIENTS AND METHODS: Calcidiol serum levels were measured in 171 postmenopausal women (111 with osteoporosis and 60 without osteoporosis). 82 women with calcidiol serum levels < 10 ng/ml were distributed in two groups: Group I received 800 U/day of vitamin D3 associated with calcium (1 g/day) and group II, one dose of 80,000 U vitamin D orally as calcidiol and latter a daily dose of 800 U vitamin D3 plus 1 g calcium. Calcidiol serum levels were measured by RIA in both groups at basal condition and after three, six and twelve months under treatment. RESULTS: Three cut-offs were considered: 10, 15 and 20 ng/ml of calcidiol. Percentages of postmenopausal women with vitamin D deficiency for such cut-offs were: 35.3%, 64.1% and 87.1%, respectively. After three months of treatment, women from group II showed calcidiol serum levels higher than group I. At six and twelve months calcidiol serum levels were similar in both groups. CONCLUSIONS: A high prevalence of vitamin D deficiency was observed in a group of postmenopausal women who attended a rheumatology practice in Madrid area. Both forms of vitamin D administration seem not sufficient to maintain the adequate calcidiol serum levels in postmenopausal deficient women. A dose of 80,000 U of calcidiol twice a year should be considered.

[Polymorphism of the gene of vitamin D receptor and bone mineral density in postmenopausal women]. / Polimorfismo del gen del receptor de la vitamina D y densidad mineral ósea en mujeres posmenopáusicas.

BACKGROUND: Conflicting results have been reported on the association between restriction fragment length polymorphism at the vitamin D receptor (VDR) gene locus and bone mineral density (BMD). Population differences in environmental factors, such as calcium intake and calcidiol levels which have strong influence in BMD, may alter this association. PATIENTS AND METHODS: We analyzed the Bsml RFLP at the eight introm of the VDR gene in a population sample (n = 204) of postmenopausal Spanish women aged 50-65 years being seen clinically and studied calcium intake (dietetic questionnaire) and biochemical parameters (PTH and calcidiol). In parallel bone densitometry were measured in lumbar spine and proximal femur. RESULTS: We identified low BMD of the proximal femur in the BB group. This effect was not observed at other body locations. The calcium intake was lees than 500 mg/day in 60% of the studied population as calcidiol levels were lower than 10 ng/l in 36% of it. The total group population with normal calcium intake (> 1,000 mg/day) showed higher BMD (proximal femur and spine) than the group with low calcium intake, this variation not being observed in group BB alleles. Interestingly, we observed significant differences in BMD proximal femur between genotype groups BB versus Bb + bb when calcidiol levels were < 10 ng/l. Moreover, within the BB subgroup, those subjects with normal calcidiol levels have higher proximal femur BMD compared with those with low calcidiol levels. CONCLUSIONS: Our results indicate an effect of the VDR genotype on BMD proximal femur which is clearly influenced by calcium intake and calcidiol serum levels.

Cerebral blood flow changes in pseudomigraine with pleocytosis analyzed by single photon emission computed tomography. A spreading depression mechanism?

Pseudomigraine with pleocytosis is a benign and autolimited syndrome. The etiology has been related to viral infection, but its pathophysiology is not yet well identified. To investigate this point, and to see if there were changes in cerebral blood flow (as in migraine), we performed single photon emission computed tomography (SPECT) studies in four patients who fulfilled the diagnostic criteria for this syndrome. This was done during the acute phase and we repeated SPECT after resolution of the syndrome in two of them. We found a reduction in brain blood flow on the side of origin of the neurological deficits during the acute phase. This normalized after recovery of the syndrome. The finding suggests that the neurological deficits in this syndrome could be produced by a spreading depression-like mechanism similar to that proposed for migraine with aura.

Migrainous syndrome with CSF pleocytosis. SPECT findings.

Brain single photon emission computed tomography (SPECT) findings are described in four adult patients with the transient syndrome of headache with neurological deficits and cerebrospinal fluid (CSF) pleocytosis. Focal deficits consisted of right-sided hemisensory changes with or without motor dysphasia or dysarthric speech (n = 4) and confusional episodes (n = 1). All patients had a CSF pleocytosis (with a mean of 309 cells/mm3 on the first spinal tap; range 75 to 590) and an elevated total protein (mean 130.5 mg/dL; range 70 to 193). The EEG showed excessive focal slowing (n = 2). A technetium Tc 99m hexamethyl propylenamine oxime (HMPAO) brain SPECT was performed during a symptom-free period, within 8 and 25 days after the onset of symptoms (n = 4). Three patients showed a decreased tracer uptake in the anterior left hemisphere, topographically consistent with the neurological deficits and EEG slowing. One patient showed no abnormalities. These findings indicate either focally impaired neuronal metabolism or hypoperfusion in regional cerebral blood flow, which could bear some relationship with the clinical features. The possibility that SPECT abnormalities may represent an epiphenomenon was also considered.

[value of bone densitometry in pediatric renal transplantation]. / Valor de la densitometría ósea en el trasplante renal pediátrico.

OBJECTIVE: Renal transplantation improves many bone abnormalities inherent to chronic renal insufficiency, but also introduces new deleterious factors, especially those related to immunosuppressive drugs. We have studied the bone mineral content of 40 pediatric renal transplant recipients; moreover, we have analyzed its possible relationships with phosphorous-calcium metabolism, graft function and duration, steroid treatment and growth. PATIENTS AND METHODS: Bone mineral content was measured by dual energy X-ray absorptiometry (DEXA). The results were expressed as a z score in relation to age and sex. Immunosuppression was achieved by using triple therapy: azathioprine, cyclosporine A and prednisolone. At the time of the DEXA, 59% of the patients were on daily steroid treatment and 41% on alternate-day treatment. RESULTS: All patients presented bone mass losses with a maximal decrease between 5 and 21 months post-transplantation and a posterior tendency to recovery. Fifty percent of the children had severe osteopenia (bone mineral density < -1 SD). There was no statistically significant association between the severity of bone loss and values of ionized calcium, phosphorus, intact PTH, or calcitriol received. However, a linear correlation was found between cumulative steroid dose and osteopenia (r = -0.35, p < 0.05). Height and growth velocity were more affected in the severe osteopenia group. In this group, the proportion of children on daily steroid treatment was statistically higher (72% vs 45%, p < 0.05). CONCLUSIONS: Bone densitometry is an accurate, rapid and noninvasive method to measure renal transplant impact on the growing skeleton and it must be done in a periodic and standardized way.

Sturge-Weber syndrome without facial nevus.

A patient with Sturge-Weber syndrome without the characteristic facial nevus presented with focal seizures which were difficult to control and borderline mental level. CT disclosed calcification in the right occipital zone. A marked decrease of the regional cerebral blood flow that extended beyond the abnormalities depicted on CT was seen by SPECT. Venous magnetic resonance (MR) angiography revealed reduction of the superficial cortical veins and prominent deep collateral venous system in the same side of the cerebral lesion. Cranial MR imaging with Gd-DTPA demonstrated the pial angioma.

Relations between calcidiol serum levels and bone mineral density in postmenopausal women with low bone density.

The relationship between vitamin D and bone density was studied in 150 selected, mature (45-74), postmenopausal women with a lumbar spine Z score below 0. Vitamin D status was evaluated using calcidiol serum levels. Serum calcitriol and parathyroid hormone (PTH) values were also evaluated in some subjects. Bone mass was evaluated by ascertaining bone density and Z and T scores in the lumbar spine and femur region. The reference group consisted of 25 premenopausal women. The postmenopausal group was divided into subgroups according to age, i.e., under or over 60 years old. Additionally, the whole group was also subdivided according to their lumbar spine Z scores into group I (Z > -1), group II (Z < -1; > -2), and group III (Z < -2). Group III of postmenopausal women had higher PTH and lower calcitriol levels than premenopausal women. Calcidiol serum levels were lower in postmenopausal women groups II or III than in the group I and premenopausal women. Calcidiol serum levels and the bone mass values for the lumbar spine were correlated positively in all the postmenopausal women; in the women over 60 years of age, calcidiol levels also correlated with the bone mass values expressed as the bone density in three femur regions: femoral neck, trocanter, and Ward's triangle. In conclusion, mature post-menopausal woman showed high PTH levels and low calcidiol and calcitriol values. Calcidiol status is significantly related to bone mineral density in the lumbar spine and in women over 60 years, calcidiol levels also correlated with bone density in the femur regions.

Scintimetric study of the bone response around noncemented knee prosthesis. Preliminary results.

The bone response to a noncemented knee prosthesis was observed by quantified isotopic gammagraphy. One hundred six sequential scintimetric explorations were performed in 36 patients with total knee arthroplasty. Fifteen areas of interest were taken into consideration and their gammagraphic analyses were compared with radiologic findings during a postsurgery period of 24 months. Starting with a very high radionuclide uptake level obtained in the first postsurgery level, a gradual decrease was correlated with osteoblastic activity. In this first evaluation, the gammagraphic response around the implant is different in biologic prostheses than in cemented ones. These data may be useful for evaluating implants in orthopedic surgery.