Transient splenial lesion due to non-cirrhotic hyperammonaemia in dengue fever.

Transient splenial lesion(TSL) is seen in a variety of conditions and is detectable only on MRI of the brain. Dengue fever (DF) is a common viral infection encountered in the tropics. The affected patients may face neurological complications like encephalopathy and intracranial haemorrhage, or even ischaemic stroke. Non-cirrhotic hyperammonaemia is a rare scenario; and its occurrence in DF is unknown. The patient being described had DF and developed dysarthria. His MRI brain showed splenial hyperintensity. Further evaluation revealed non-cirrhotic hyperammonaemia. To the best of our knowledge, TSL due to non-cirrhotic hyperammonaemia in DF is an unreported scenario.

Fetus in fetu - a rare developmental anomaly.

Fetus in fetu is a rare condition which most often presents as a fetiform calcified mass in the abdomen of its host, fetus or newborn. We report a case of 8-month-old girl with history of abdominal distension. Ultrasonography and computed tomography scan revealed a mass in which the contents favor a fetus in fetu rather than a teratoma. She underwent surgery and the mass was resected in toto. Radiograph of the resected specimen showed the presence of rudimentary vertebral column which was later confirmed by pathologist.

Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major.

BACKGROUND: Combination therapy with deferoxamine and oral deferiprone is superior to deferoxamine alone in removing cardiac iron and improving left ventricular ejection fraction (LVEF). The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation. We assessed the effects of combination therapy on the RV in thalassaemia major (TM) using cardiovascular magnetic resonance (CMR). METHODS: We retrieved imaging data from 2 treatment trials and re-analyzed the data for the RV responses: Trial 1 was a randomized controlled trial (RCT) of 65 TM patients with mild-moderate cardiac siderosis receiving combination therapy or deferoxamine with placebo; Trial 2 was an open label longitudinal trial assessing combination therapy in 15 TM patients with severe iron loading. RESULTS: In the RCT, combination therapy with deferoxamine and deferiprone was superior to deferoxamine alone for improving RVEF (3.6 vs 0.7%, p = 0.02). The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47). In patients with severe cardiac siderosis, substantial improvement in RVEF was seen with open-label combination therapy (10.5% ± 5.6%, p < 0.01). CONCLUSIONS: In the RCT of mild to moderate cardiac iron loading, combination treatment improved RV function significantly more than deferoxamine alone. Combination treatment also improved RV function in severe cardiac siderosis. Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.

Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction.

BACKGROUND: In thalassemia major (TM), severe cardiac siderosis can be treated by continuous parenteral deferoxamine, but poor compliance, complications and deaths occur. Combined chelation therapy with deferiprone and deferoxamine is effective for moderate myocardial siderosis, but has not been prospectively examined in severe myocardial siderosis. METHODS: T2* cardiovascular magnetic resonance (CMR) was performed in 167 TM patients receiving standard subcutaneous deferoxamine monotherapy, and 22 had severe myocardial siderosis (T2* < 8 ms) with impaired left ventricular (LV) function. Fifteen of these patients received combination therapy with subcutaneous deferoxamine and oral deferiprone with CMR follow-up. RESULTS: At baseline, deferoxamine was prescribed at 38 +/- 10.2 mg/kg for 5.3 days/week, and deferiprone at 73.9 +/- 4.0 mg/kg/day. All patients continued both deferiprone and deferoxamine for 12 months. There were no deaths or new cardiovascular complications. The myocardial T2* improved (5.7 +/- 0.98 ms to 7.9 +/- 2.47 ms; p = 0.010), with concomitant improvement in LV ejection fraction (51.2 +/- 10.9% to 65.6 +/- 6.7%; p < 0.001). Serum ferritin improved from 2057 (CV 7.6%) to 666 (CV 13.2%) microg/L (p < 0.001), and liver iron improved (liver T2*: 3.7 +/- 2.9 ms to 10.8 +/- 7.3 ms; p = 0.006). CONCLUSION: In patients with severe myocardial siderosis and impaired LV function, combined chelation therapy with subcutaneous deferoxamine and oral deferiprone reduces myocardial iron and improves cardiac function. This treatment is considerably less onerous for the patient than conventional high dose continuous subcutaneous or intravenous deferoxamine monotherapy, and may be considered as an alternative. Very prolonged tailored treatment with iron chelation is necessary to clear myocardial iron, and alterations in chelation must be guided by repeated myocardial T2* scans. TRIAL REGISTRATION: This trial is registered as NCT00103753.

Angina pectoris: interventional therapies and treatment of restenosis.

Angina pectoris is a clinical syndrome of symptoms caused by myocardial ischaemia due to oxygen demand exceeding supply. The most common cause is coronary artery stenosis due to progressive atherosclerotic disease. Angina has a prevalence of approximately 5% and increases with age. Despite improvements in treatment there remains a yearly mortality of 2-3%. A major advance in the treatment of symptomatic angina was the introduction of percutaneous transluminal coronary angioplasty (PTCA). This initial enthusiasm was dampened by significant numbers developing symptomatic restenosis from vascular elastic recoil and neointimal hyperplasia (NI). The widespread introduction of stent deployment following the initial angioplasty reduced the rates of elastic recoil but failed to prevent NI and may actually stimulate it. Currently, there is much interest in mechanisms that alter cell proliferation thereby decreasing NI. Techniques include brachytherapy, photodynamic therapy and drug-eluting stents. Provisional data for these new stents, which slowly release medication that inhibits cell turnover, are very good with few occurrences of restenosis. Results from larger randomised trials are awaited.