Protein-losing gastropathy associated with cytomegalovirus in two sisters - Case reports and review of the literature.

Ménétrier's disease is a protein-losing gastropathy that is uncommon in childhood. Its symptoms are unspecific, with abdominal pain, vomiting, and edema. Blood tests show hypoproteinemia and hypoalbuminemia, and upper digestive endoscopy reveals giant gastric folds. In children, cytomegalovirus has been identified as a possible cause. Here we describe two sisters presenting with Ménétrier's disease, 2 years apart. This diagnosis should be considered in the presence of hypoalbuminemia in children when a nephrotic syndrome is excluded.

Liver iron overload assessment by MRI R2* relaxometry in highly transfused pediatric patients: an agreement and reproducibility study.

AIMS: Perform an agreement and reproducibility study of the estimation of iron overload in highly transfused pediatric patients comparing R2* relaxometry (R2*=1000/T2*) to the reference technique liver/muscle signal intensity ratio (SIR). PATIENTS AND METHODS: Ninety-two MRI were performed in 68 children who were mainly transfused for sickle cell disease, mean age 9.9 years old. The examination included six sequences for the SIR protocol and a single multiecho T2* sequence. R2* relaxometry was measured by two radiologists independently, either by a region of interest (ROI) in the right liver, or an outline of the whole liver. Hepatic iron load was determined by the Wood formula (Fe mg/g=R2*×0.0254+0.202). The validity of R2* relaxometry compared to SIR was evaluated by the coefficient of variation and the quadratic weighted Kappa value. RESULTS: The correlation between R2* relaxometry and SIR was very good with a Pearson coefficient of 0.89 and a coefficient of variation of 17.3%. The inter- and intraobserver reproducibility of the measurement of R2* relaxometry by ROI and whole liver mapping was excellent. However, we observed a common positive variation of one class between SIR and R2* relaxometry, with higher hepatic iron content values with SIR than with R2* relaxometry. CONCLUSION: Hepatic iron content can be rapidly and precisely estimated on MRI by multiecho gradient-echo sequences.

Iron overload of hematological origin: validation of a screening procedure for cardiac overload by MRI in routine clinical practice.

PURPOSE: Screening for cardiac iron overload is generally done by magnetic resonance imaging (MRI) and demonstrated by a shortening of the myocardial T2* below 20 ms at 1.5 Tesla. This measurement was validated with a specific sequence and the CMRTools(®) calculation software (reference technique). The objective of this study was to validate the use of sequences and software programs that are available in routine clinical practice to screen for iron overload. MATERIAL AND METHODS: First, a phantom of 11 tubes with a T2* between 4 and 33 ms was tested at three sites that had MRI machines of different brands. Second, the myocardial T2* values of 75 patients were measured in routine clinical practice using two methods. The first method used the reference sequence specially installed in the machines associated with the CMRTool software. The second method used the standard acquisition sequences available in the machines followed by calculation on a computer spreadsheet. RESULTS: In the phantom, the mean of the differences in T2* between each machine was 0.6 ms. Thirteen patients had a lowered T2* value with the reference technique. Three cases were poorly classified using the routine technique and corresponded with false positives of low overload (T2* between 18 and 20 ms). CONCLUSION: Screening for myocardial iron overload can be done by MRI by using sequences and calculation software available in routine clinical practice during the same examination as the one for the evaluation of hepatic iron overload.

[Transcranial color Doppler in children with sickle cell disease]. / Doppler transcrânien chez l'enfant drépanocytaire.

Cerebral vascular disease in children with sickle cell disease is characterized by progressive stenosis of large arteries of the skull base, which can be detected sensitively, specifically and unexpensively by transcranial Doppler. Because sickle cell disease is responsible for basal high velocities, criteria used for diagnosis of stenosis are different than those used in non sickled children and moreover in adults. Mean velocity higher than 2 meters per second is predictive of a 40% risk for stroke in the three following years, but transfusion program maintaining hemoglobin S under 30%, reduces the risk to 2%. It is important to test each child with sickle cell disease by the age of one or two years, in order to detect cerebral vasculopathy before overt stroke and its residual deficits and to initiate appropriate treatment

Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation.

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.

[Imaging of cerebrovascular disease in sickle cell anemia]. / Exploration de la vasculopathie cérébrale drépanocytaire.

Cerebral vascular disease is a common and serious complication of sickle cell disease that mainly involves the large blood vessels of the skull base. Because recurrences are common and residual deficits severe, attention has turned to detection of preclinical cerebral involvement with the goal of preventing clinical damage. Magnetic resonance imaging (MRI), an extremely sensitive tool for detecting cerebral infarction/ischemia, has shown that 10% of asymptomatic patients exhibit white matter lesions that seem to be associated with impaired cognitive function and may be predictive of stroke; magnetic resonance angiography demonstrates occlusions of skull base arteries but is not reliable for the diagnosis of stenosis because of artifacts generated by rapid turbulent flow. Transcranial Doppler is sensitive and specific for the detection of arterial stenosis and occlusion, even in asymptomatic patients. Digitized cerebral angiography remains the gold standard investigation for pretreatment confirmation of lesions detected by Doppler and/or MRI.

Detection of cerebrovascular disease in patients with sickle cell disease using transcranial Doppler sonography: correlation with MRI, MRA and conventional angiography.

A prospective study of 58 patients with sickle cell disease (SCD) by transcranial Doppler sonography (TCD) included both MRI and MRA in patients over 7 years of age and those with abnormal TCD. Arteriography was performed in cases where a stenosis was suspected on TCD. Middle cerebral artery (MCA) and basilar artery (BA) velocities were significantly higher in the sickle cell hemoglobin SS group than in the hemoglobin SC group. Patients with a MCA mean velocity of over 1.90 m/s had stenoses found by arteriography. Patients with unilaterally undetectable MCA flow had experienced a stroke and MCA thrombosis was confirmed at MRA and arteriography. We concluded that TCD is valuable in detecting arterial stenosis in SCD and will lead to consideration of these patients for intensive therapy, such as bone marrow transplantation (BMT) or transfusion regimes.

Variability of clinical presentation of hepatic artery thrombosis in pediatric liver transplantation: role of imaging modalities.

Among a series of 90 pediatric liver transplantations, 9 cases of hepatic artery thrombosis (HAT) in 8 patients are reported. All cases were diagnosed in the first two weeks and confirmed angiographically and surgically. Clinical presentation was often unreliable with only 2 cases showing the typical pattern of massive hepatic necrosis. Five cases presented with biliary complications and in 2 cases, HAT was discovered fortuitously on duplex sonography. The role of imaging modalities is emphasized. Duplex sonography is the best non-invasive screening method and we recommend a routine daily examination in the first 2 weeks. CT is the most useful method to assess the extension of liver infarction. PTC remains necessary to evaluate biliary complications and to plan the best therapeutic approach in this devastating event. Two patients died, 2 had to be retransplanted (one patient twice) and are doing well, 2 are on a waiting list for retransplantation and one underwent an emergency surgical arterial desobstruction and is completely asymptomatic.

[Hemodynamic effects of dobutamine in asphyxiated newborn infants]. / Effets hémodynamiques de la dobutamine chez le nouveau-né asphyxié.

The hemodynamic effects of dobutamine were studied by non invasive techniques in 6 full term neonates with severe perinatal asphyxia. At 10 micrograms/kg/min, dobutamine significantly increased the cardiac output, the heart rate and the aortic blood flow velocity. The mean arterial pressure increased but not significantly and the stroke volume remained unchanged. These changes seem to be dose dependent. The increase of cardiac output may be due to a chronotropic and inotropic effect of dobutamine. Dobutamine seems to be an effective agent for the treatment of low cardiac output in asphyxiated neonates.

[Dihydralazine treatment of cardiac insufficiency in children]. / Traitement par la dihydralazine de l'insuffisance cardiaque de l'enfant.

The action, efficient dosage and tolerance of a pure vasodilator, dihydralazine, used for the treatment of severe heart failure were studied in 30 children aged 1 month to 14 years. All of them presented with heart failure from various causes, not controlled by the usual medical treatment. Dihydralazine was administered orally, without interrupting the digoxin-diuretic treatment, with a dose of 34 to 140 mg/m2/day given in 4 equal doses. Clinical efficacy was considered null in 12 cases, low in 12 cases and good in 6 cases, without relationship with the original heart defect. Five of the 6 good results were obtained with doses greater than or equal to 100 mg/m2/day. In the group of 16 children who were given doses greater than or equal to 100 mg/m2/day, a significant improvement of the ECG indexes of left ventricular performance was obtained: decrease in systolic left ventricular internal dimension (p less than 0.05 at day 5), increase of the shortening fraction (p less than 0.05 since day 1) and of velocity of shortening (p less than 0.01 since day 1), while the diastolic left ventricular internal dimension remained unchanged. The only transitory undesirable effects observed were headache, vomiting and/or rash in 9 cases.