The patient is a 5-year-old male with Kawasaki disease, whose involution of giant aneurysms of the left coronary arteries was surprising after a prolonged period of treatment, which lasted 80 uninterrupted days and comprised anti-inflammatory drugs associated with anticoagulation agents. The distal diameters of the anterior interventricular, the diagonal, and the circumflex arteries normalized by the end of the treatment. A residual giant aneurysm localized at the beginning of the anterior interventricular artery did not cause ischemia. Continuation of the medication for a prolonged period was recommended.Kawasaki disease, known since 1976 1 , has some evolutional characteristics that deserve mention 2-6 , among which is the reduction in coronary aneurysms with adequate anti-inflammatory treatment, which causes radical change both in the size and extension of the aneurysm, except for the rare regression of giant aneurysms 2,3,6 . This involution is possible and may even be significant in some cases. Therefore, in the acute phase of the disease, surgical indication, which is limited to the presence of significant dysfunction or myocardial ischemia, or both, should be very carefully considered 4,5 .We report a case of significant involution of giant coronary aneurysms with treatment. Case ReportThe patient is a 5-year-old male with findings suggestive of Kawasaki disease in the preceding 2 months, characterized by fever, enlargement of cervical lymph nodes, and tonsillitis, followed by generalized cutaneous erythema, pruritus, and edema of the limbs after a few days, and desquamation on the 10th day. The laboratory tests indicated an active inflammatory phase with an elevated sedimentation rate of 100 mm during the first minute, C-reactive protein of 50 U, leukocytosis of 12000/mm 3 , and, approximately 10 days later, an increased number of platelets (540000/mm 3 ). Despite the treatment with acetylsalicylic acid (80mg/kg/ day), gamma globulin (2g/kg) on the forth and seventh days, in addition to general measures, the patient developed giant aneurysms in the anterior interventricular, circumflex, and right coronary arteries. These aneurysms were detected in the initial third of the cited arteries on echocardiography ( fig. 1) approximately 33 days after the beginning of the disease and were confirmed on coronary angiographic study on the 46th day of evolution, in which the right coronary artery was obstructed and filled retrogradely from the left coronary artery. The left arteries were significantly dilated halfway up their trajectories, and saccular aneurysms continued the anterior interventricular artery up to the left ventricular tip. The diagonal artery was slightly impaired ( fig. 2).Due to persistence of the elevated sedimentation rate and C-reactive protein, the treatment with high doses of acetylsalicylic acid was reinitiated on the 47th day after the beginning of the disease, interrupted on the 17th day due to gastric irritation. Laboratory tests became normal 20 days after the restart of medication, whic...

We compared the levels of lipoprotein (a) in 48 Caucasian patients with pulmonary arterial hypertension, comprising 32 females and 16 males, aged 28.0 +/- 12.0 years, with a range from 4 through 52 years, with 48 normal Caucasian subjects matched for age and sex. Pulmonary hypertension was secondary in 41 patients with Eisenmenger's syndrome, these comprising 27 females and 14 males aged 27.0 +/- 12.0 years, with a range from 4 through 51 years, and primary in the other 7 patients, 5 females and 2 males, whose age was 30.0 +/- 14.0 years, with a range from 9 through 52 years. Lipoprotein (a) was measured using an immunoprecipitation and turbidimetric assay after a 12 hour fast. Levels of the protein, expressed as the median (% 25; % 75), were higher in those with Eisenmenger's syndrome than in normal controls (p=0.003). In addition, there was a greater prevalence of levels of lipoprotein greater than 30.0 mg/dl in those with secondary pulmonary arterial hypertension patients than in our normal population (p = 0.03). We have found no differences, however, in the levels of lipoprotein(a) in those who had primary pulmonary arterial hypertension when compared with their matched controls, albeit that the number of patients studied was small. We conclude that increased levels of lipoprotein (a) may be secondary to pulmonary arterial hypertension as a marker of tissue damage or may be genetically determined. In either way, the increase in lipoprotein (a) could be an additional factor predisposing to the vascular alterations known to occur in this disease.

Background Parenteral nutrition (PN) for neonates has to be infused by a central line, due to the high osmolarity resulting from the recommended requirements. The central catheter frequently needs to be removed, and therefore PN may have to be administered by a peripheral line. This problem has been resolved by infusion of enriched glucose solutions, minus the protein input, which is very important in order to avoid catabolism. Purpose To develop a standard PN with glucose, electrolytes and amino acids, suitable for peripheral infusion and available for the Neonatology department at any time. The aim is to infuse 100 mL/Kg. Materials and MethodsWe performed a literature search about standard PN and we made microbiological and biochemical cheques to ensure the stability and integrity of the solution, after keeping it refrigerated for seven days. ResultsWe developed a standard PN solution with the following composition per 100 mL: Amino acids: 2 g Glucose: 9.5 g Sodium: 4 mEq Potassium: 2 mEq Magnesium: 0.2 mEq Calcium: 1.5 mEq Phosphate: 0.8 mmol Osmolarity: 792 mOsm/L Total calories: 46 Kcal Weekly, we prepare four 500 ml bags from a stock solution. We give the neonatology department two so they can hold a small stock and we keep the other two in order to cheque when we need to make another batch. From implementation, in February 2012, the microbiological controls have always been negative and the biochemical controls have demonstrated that degradation does not occur after refrigeration for seven days. Conclusions This formulation makes it possible for the physicians to continue with the nutritional support, by peripheral infusion, at any time. However this type of nutritional solutions is only suitable for meeting the nutritional requirements for short periods, until a new central catheter is placed or the neonate is able to have complete enteral feeding. No conflict of interest.

Background Glucarpidase (Voraxaze) is effective in the treatment of methotrexate (MTX)-induced renal dysfunction but many cases this can be handled with standard treatment. PurposeTo describe the progress of a patient with MTX-induced renal failure in whose management glucarpidase was not used. Materials and MethodsA 13 year-old girl with acute lymphoblastic leukaemia treated with high-dose MTX. Baseline laboratory tests were normal, except for elevated transaminases and GGT. Results The patient received her first consolidation cycle with 500 mg/m2 of MTX in 30 minutes, followed by 4500 mg/m2 in 23.5 hours, oral mercaptopurine 30 mg/m²/day and triple intrathecal therapy. Simultaneously, she received hyperhydration/alkalinisation (3000 ml/m²/day).There was no pharmacological interaction to MTX. 24 hours after the MTX infusion stared, the serum creatinine level (Cr) had tripled (see the table below).The following measures were taken: hyperhydration/alkalinisation (4500 ml/m²/day), colestyramine (3 g/6 h) and folinic acid rescue at 500 mg/m²/6 h 31 hours after the start of the MTX infusion. Although the protocol provides for the possibility of administering glucarpidase, it was decided not to do this because the methotrexate level was <250μM and glucarpidase administration can be delayed until 96 hours after the start of MTX infusion. Difficulty in the subsequent monitoring, the absence of effect in renal function improvement and high cost were the reasons for delaying the treatment until at least having levels at 36 and 48 hours. Although Cr values were still high, elimination kinetics of the drug were seen as adequate. Without the use of glucarpidase, methotrexate levels were undetectable at day nine. The patient recovered her baseline renal function and did not have mucositis or liver toxicity. Conclusions An early intervention with supportive treatment based on folinic acid, hyperhydration and urine alkalinisation was effective in the management of MTX-induced renal toxicity. Abstract GRP-111 Table 1 Time since MTX infusion started (h) Cr (mg/dL) MTX levels (µM) 0 0.35 0 24 1.12 190 36 1.41 24 48 1.32 5.9 No conflict of interest.

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