Outcome of surgery in children with focal cortical dysplasia younger than 5 years explored by stereo-electroencephalography.

PURPOSE: Focal cortical dysplasia (FCD) is the most frequent etiology for drug-resistant epilepsy in young children. Complete removal of the lesion is mandatory to cure the epilepsy. Stereo-EEG (SEEG) is an excellent method to delimitate the zone to be resected in older children and adults. We studied its feasibility in younger children. METHODS: We retrospectively studied 19 children under 5 years of age who underwent SEEG between January 2009 and December 2012 and were subsequently operated on. FCD was diagnosed in all. We reviewed magnetic resonance imaging (MRI), electrophysiological and clinical data, as well as postoperative seizure outcome. We also included fluoro-deoxyglucose positron emission tomography (FDG-PET) studies, which had been systematically performed before invasive recording in 16 of the 19 children. RESULTS: The mean patient's age at the time of SEEG was 38.6 months, and the mean age at seizure onset was 8 months. Three patients had normal MRI. No SEEG-associated complications occurred. We were able to delineate the epileptogenic zone in all children, and electrode stimulation localized the motor area when necessary (12 patients). Hypometabolic areas on FDG-PET included the epileptogenic zone in 13 of the 16 children, with a lobar concordance in 9 (56 %) and the same anatomical extent in 6 (38 %). Twelve children subsequently underwent focal or sublobar resection, six had multilobar resection, and one had hemispherotomy. The etiology was FCD type 2 in 15 and FCD type 1 or type 3 in three children. Eighty-four percent of our population have remained seizure-free at a mean follow-up of 29 months (12-48 months). CONCLUSION: Although children with FCD can successfully undergo resective surgery without invasive EEG, poor seizure semiology at this age inclines to perform SEEG when the dysplastic lesion is ill-defined and/or the electroclinical correlation is unclear. In cases with normal imaging as well as with suspected huge malformations, as was the case in 52 % of our patients, we consider it to be indispensable.

Safety of shock wave lithotripsy for treatment of pediatric urolithiasis: 20-year experience.

PURPOSE: This retrospective study was designed to assess the impact of shock wave lithotripsy on the pediatric kidney using pretreatment and posttreatment (99m)technetium dimercapto-succinic acid renal scintigram. MATERIALS AND METHODS: A total of 182 patients 5 months to 19.8 years old (mean 5.3 years) were treated for renal calculi with shock wave lithotripsy during a 20-year period. Pretreatment evaluation included clinical assessment, urine culture, renal ultrasound and plain abdominal radiograph with or without excretory urogram. Dimercapto-succinic acid scintigram was performed before and 6 months after completion of treatment in 94 patients (52%). RESULTS: Patients underwent 1 to 4 sessions of shock wave lithotripsy per kidney with at least 1 month between treatments. Median number of shocks delivered per session was 3,000 (IQR 2,601 to 3,005). No new scars were observed on any posttreatment dimercapto-succinic acid scan. Regarding renal function, patients fell into 1 of 4 groups. Group 1 (66 patients, 70%) had normal function on dimercapto-succinic acid scan before and after treatment, group 2 (18, 19%) had decreased function in the affected kidney on pretreatment scan with no change after treatment, group 3 (2, 2%) had impaired function in the treated kidney that was transient (1) or permanent (1) and group 4 (7, 7%) had improved function in the treated kidney. CONCLUSIONS: Shock wave lithotripsy is an effective treatment for renal calculi in children. Renal parenchymal trauma associated with extracorporeal shock wave lithotripsy does not seem to cause long-term alterations in renal function or development of permanent renal scars in children.

[Noninfectious febrile inflammatory syndromes in children: diagnosis and usefulness of diagnostic procedures]. / Syndromes inflammatoires fébriles non infectieux de l'enfant: diagnostic, contribution des examens complémentaires.

OBJECTIVE: To determine the causes and to quantify the benefits obtained from further diagnostic investigations in children presenting with a non infectious inflammatory fever. METHODS: The records of 62 children aged from two-months to 15 years (median: four years) admitted to a paediatric department between 1990 and 2000 for the evaluation of a fever associated to an inflammatory syndrome, defined as temperature over 38 degrees C with an increase of the erythrocyte sedimentation rate (ESR) more than 20 mm/h and/or a serum C-reactive protein level (CRP) > 20 mg/L, and excluding overt infectious diseases, were retrospectively reviewed. RESULTS: Of these patients, 79% children (49 cases) had inflammatory systemic disease, 3.2% (two cases) had malignancy, and 17.8% (11 cases) had undiagnosed disorders. The most frequent disease was Kawasaki disease (22 children), especially in young children. Increase of ESR above 100 mm/h and of CRP above 100 mg/L was present in 59% of Kawasaki disease, 71% of idiopathic juvenile arthritis, 100% of malignancies and 7% of unknown diagnoses. Increase of ESR below 50 mm/h and of CRP below 50 mg/L was present in 75% of hemophagocytic syndromes and 46% of unknown diagnosis. The polymorphonuclear count, hepatic function evaluation, triglycerides levels, abdominal ultrasound, abdominal computed tomography, echocardiography, biopsies were useful diagnosis tools. Technetium scintigraphy was helpful only when abnormalities were found on physical examination. CONCLUSION: The diagnosis of Kawasaki disease must be quickly suspected in febrile young children with inflammatory syndrome without infection. ESR and CRP values, abdominal ultrasound and echocardiography are helpful tools for the diagnostic procedure.

Monotherapy extracorporeal shock wave lithotripsy for the treatment of staghorn calculi in children.

PURPOSE: We evaluate the efficacy and parenchymal consequences of extracorporeal shock wave lithotripsy (ESWL) for staghorn calculi in children. MATERIALS AND METHODS: From 1991 to 1999, 16 young patients 5.5 months to 2 years old and 7 older children 6 to 11 years old were treated for complete (6) or partial (17) staghorn calculi. Infection was the main factor of lithogenesis, particularly in younger children. In 21 patients the stone burden was more than 20 mm. All patients were treated under general anesthesia using a 14 kV. Sonolith 3000 (14) or a 12 to 20 kV. Nova (9) lithotriptor. In 5 of the 7 older children a Double J section sign stent was inserted before treatment. In the younger children group calculi were fragmented after 1 (11) or 2 (5) sessions. Of the 7 older children 3 required 3 (1) and 4 (2) treatment sessions. To assess the long-term effect of ESWL on the parenchyma, dimercapto-succinic acid renal scan was performed the day before and 6 months after therapy in 20 patients. RESULTS: Of the 16 younger children 14 became stone-free (87.5%) and 2 had small asymptomatic residual fragments. Of the 7 older children 5 were stone-free (71.4%) and 2 required additional surgery. No steinstrasse or pyelonephritis occurred after ESWL and no parenchymal or ureteral lesion related to ESWL was identified on conventional dimercapto-succinic acid scintigraphy. At a followup of 9 months to 9 years all patients have normal blood pressure. CONCLUSIONS: ESWL as a first option is safe and appropriate for the treatment of staghorn calculi in children particularly in younger children with infected calculi.

Scintigraphic screening for renal damage in siblings of children with symptomatic primary vesico-ureteric reflux.

OBJECTIVE: To define prospectively the incidence of renal parenchymal lesions in the siblings of patients treated at one institution for primary vesico-ureteric reflux (VUR). PATIENTS AND METHODS: From January 1997 to October 1998, a prospective study including renal scintigraphy (using dimercaptosuccinic acid, DMSA) and a radionuclide cystogram was proposed systematically to the asymptomatic siblings of children treated for primary VUR. The radionuclide cystograms were interpreted as showing the presence or absence of VUR and the DMSA scan as symmetrical or asymmetrical differential function, with or with no renal defect. RESULTS: Fifty-five families gave informed consent, of whom 46 completed the study (eight refused secondarily and one was omitted by exclusion criteria), representing 46 symptomatic patients and 65 siblings. There were 17 siblings with VUR (26%) including two of 13 infants and 15 of 52 children aged > 18 months. One radionuclide cystogram failed. Of the 17 refluxing siblings, four had a history of symptomatic urinary tract infection; 62 of the 65 siblings had a DMSA scan, of which 56 were normal and six (10%) showed abnormalities (five asymmetrical differential function and one parenchymal defect). Only one of these six patients had VUR at the time of the evaluation and only one had a small kidney detected by ultrasonography on one side (and no VUR). There were no adverse effects associated with screening. CONCLUSION: This study confirms a significant overall incidence of VUR (26%) in the asymptomatic siblings of patients treated for primary VUR. From the results of the DMSA scan (only one sibling had a parenchymal defect), the systematic screening of asymptomatic siblings does not appear to be beneficial.

Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis.

Clinical manifestations of hereditary spherocytosis (HS) can be abrogated by splenectomy. However, concerns exist regarding exposure of patients to a lifelong risk for overwhelming infections and, to a lesser extent, to vascular complications after total splenectomy. In the search for alternative treatment modalities, we assessed, in a previous pilot study, the potential usefulness of subtotal splenectomy in a small population of patients. During a mean follow-up period of 3.5 years, subtotal splenectomy was shown to be effective in decreasing the hemolytic rate, while maintaining the phagocytic function of the spleen. In the current study, we evaluated the clinical and biologic features of 40 patients with HS who underwent subtotal splenectomy and were monitored for periods ranging from 1 to 14 years. The beneficial effect of subtotal splenectomy included a sustained decrease in hemolytic rate and a continued maintenance of phagocytic function of the splenic remnant. However, mild-to-moderate hemolysis was persistent and accounted for secondary gallstone formation and aplastic crisis in a small subset of patients. Surprisingly, regrowth of the remnant spleen did not seem to have a major impact on the beneficial outcomes of these individuals. Our results suggest that subtotal splenectomy appears to be a reasonable treatment option for management of patients with HS, especially young children.

Evaluation of the treatment of thyrotropin-secreting pituitary adenomas with a slow release formulation of the somatostatin analog lanreotide.

Somatostatin analogs have been shown to be effective for the treatment of TSH-secreting pituitary adenomas. However, their use in this indication is limited by the fact that available analogs require several daily sc injections. The present study was performed to evaluate the effects of a slow release formulation of the somatostatin analog lanreotide (SR-L) on both hormone secretion and tumor size and to assess the tolerance in a series of thyrotropinomas treated for 6 months. Eighteen patients with hyperthyroidism related to a TSH-secreting pituitary adenoma, evidenced by pituitary magnetic resonance imaging, were studied. After a basal assessment, each patient received 30 mg SR-L, im, every 14 days for 1 month. Then, according to the free T3 (fT3) plasma level measured, 9 of 18 patients were injected twice monthly, and 7 of 18 patients received SR-L every 10 days for 5 additional months. One patient was dismissed from the study in month 1 of the study for side-effects and another in month 3 for noncompliance to the protocol. Clinical and biological evaluations (plasma TSH, free alpha-subunit, fT4, fT3, and lanreotide levels) were performed before and in months 1, 3, and 6 of treatment. Pituitary magnetic resonance imaging and gallbladder ultrasonography were performed both at entry and at the end of the study. Clinical signs of hyperthyroidism improved within 1 month in all 16 evaluable patients. Mean (+/- SEM) plasma lanreotide levels reached 1.11 +/- 0.43 and 1.69 +/- 0.65 ng/mL in month 3 using 2 and 3 injections/month, respectively, then remained stable until the end of the study. During therapy, the plasma TSH level decreased from 2.72 +/- 0.32 to 1.89 +/-0.27 mU/L (P < 0.01), with parallel significant changes in free alpha-subunit. During the same period, plasma fT4 and fT3 levels decreased from 37.9 +/- 2.9 to 19.7 +/- 2.3 pmol/L (P < 0.01) and from 14.6 +/- 1.1 to 8.3 +/- 0.8 pmol/L (P < 0.01), respectively. No statistically significant change in mean adenoma size was observed after 6 months of treatment. Side-effects, including pain at the injection point, abdominal cramps, and diarrhea, were mild and transient and did not lead to interruption of the treatment. No gallstones occurred during the study. SR-L appears to be able to suppress clinical signs of hyperthyroidism in our series of patients with TSH-secreting pituitary adenomas. The analog also reduces plasma TSH and thyroid hormone levels, which were normalized in 13 of 16 cases. The effect was maintained throughout the treatment using 2 or 3 SR-L injections monthly without any problem of tolerance. We conclude that SR-L is a safe and effective treatment of thyrotropinomas and avoids the drawbacks of the modes of administration of other somatostatin analogs, given three times daily.

The efficacy and parenchymal consequences of extracorporeal shock wave lithotripsy in infants.

OBJECTIVES: To determine the efficacy of extracorporeal shock-wave lithotripsy (ESWL) in young children and to evaluate, using renal scintigraphy, any possible adverse effects on renal parenchyma. PATIENTS AND METHODS: From January 1991 to October 1998, 19 infants (aged 5-24 months) underwent ESWL for kidney urolithiasis using a Sonolith 3000 (14 kV, Technomed Corp, Lyon, France) or a Nova (14-20 kV, Direx Medical Systems, Paris, France) lithotripter. The treatment and its effects were evaluated using a physical examination, conventional imaging (plain abdominal X-ray and ultrasonography) and renal scintigraphy 24 h before ESWL and again at least 6 months after the last session of treatment. RESULTS: Ten children were rendered stone-free by ESWL after one session and 18 after two sessions. At the follow-up (8 months to 8 years, mean 36 months) no hypertension was recorded and no acquired parenchymal damage was detected with conventional imaging. No scars or significant variation of differential function attributable to ESWL were identified on renal scintigraphy. CONCLUSION: ESWL is clearly effective for treating infant urolithiasis. There were no renal parenchymal lesions associated with ESWL, even in previously damaged kidneys or after the treatment of staghorn calculi. A long-term follow-up (assessing blood pressure) is mandatory and renal scintigraphy before and 6 months after ESWL in infants is recommended to confirm these results in a larger series.

[Extracorporeal lithotripsy in children. Study of its efficacy and evaluation of renal parenchymal damage by DMSA-Tc 99m scintigraphy: a series of 39 children]. / La lithotritie extracorporelle chez l'enfant. Etude de son efficacité et évaluation de ses conséquences parenchymateuses par la scintigraphie au DMSA-Tc 99m: une série de 39 enfants.

AIM: The objectives of the study were to confirm the efficacy of extracorporeal shock wave lithotripsy (ESWL) in infants and children and to evaluate potential long-term renal parenchymal damage by 99m Tc DMSA renal scan. MATERIALS AND METHODS: Between November 1989 and November 1997, 39 children between 10 months and 17-1/2 years of age (average: 7 years) were treated by extracorporeal shock wave lithotripsy for kidney or ureteral stones with a Sonolith 3000 lithotriptor (Technomed Corp). Forty-six stones were treated. Eight metabolic and 11 urological abnormalities were identified. The evaluation of the treatment and its consequences were based on a clinical examination, conventional imaging and a DMSA renal scan performed 24 h before extracorporeal shock wave lithotripsy and at least 6 months after treatment. RESULTS: Treatment was successful (stone fragmented and eliminated) in 84.6% at 3 months after one to four sessions. Sixty-one sessions were necessary and two patients underwent open surgery for failed extracorporeal shock wave lithotripsy. Three recurrences were also retreated. At long term follow-up (6 months to 8 years) no incidents of high blood pressure were observed, nor parenchymal lesions imputable to extracorporeal shock wave lithotripsy. CONCLUSIONS: The efficacy of the extracorporeal shock wave lithotripsy for children is proven. This study also confirms the innocuousness of extracorporeal shock wave lithotripsy for renal parenchyma even in infants. However, long term follow-up and further evaluation with the other categories of lithotriptors are necessary to make definitive conclusions.

[Long-term evaluation with DMSA-Tc 99m scintigraphy of renal parenchymal involvement in children after shockwave extracorporeal lithotripsy]. / Evaluation à long terme par la scintigraphie au DMSA-Tc 99 m des atteintes parenchymateuses rénales chez l'enfant après lithotritie extra-corporelle par ondes de choc.

OBJECTIVES: This study evaluated the long-term effects of extra-corporeal shock-wave lithotripsy (ESWL) on the renal parenchyma of children using DMSA-Tc 99m scintigraphy. MATERIAL AND METHODS: Between November 1989 and April 1997, twenty-three children wee treated for renal stones using a SONOLITH 3000 lithotriptor (Technomed-Corp. Evaluation of treatment and its consequences was based on clinical examination, conventional imaging and comparison with DMSA-TC 99m renal scintigraphy performed the day before and at least 6 months after treatment. RESULTS: The success rate (fragmented and eliminated stones) was 90% at 6 months. Long-term follow-up did not reveal any alteration of blood pressure or renal function. Scintigraphic examinations did not demonstrate any significant parenchymal lesions attributable to treatment. CONCLUSION: Extracorporeal shock wave lithotripsy is effective in adults and young children. Its safety on the renal parenchyma was demonstrated during this study. However, evaluation of larger series with the use of other lithotriptors is necessary before reaching any definitive conclusions.

[Extracorporeal shock-wave lithotripsy in infants. Study of its repercussions on the renal parenchyma]. / La lithotritie extra-corporelle par ondes de choc chez le nourrisson. Etude de son retentissement sur le parenchyme rénal.

PURPOSE: To evaluate the efficacy of extracorporeal shock-wave lithotripsy (ESWL) in infants (less than 2-years-old) and study the potential long-term renal parenchymal damage by 99 m Tc DMSA renal scan. MATERIALS AND METHODS: Between November 1993 and February 1998, 12 children underwent ESWL treatment for kidney and ureter urolithiasis with a Sonolith 3,000 lithotriptor (Technomed-Corp). All children were less than 2-years-old. Evaluation of treatment and its consequences was based on clinical examination, conventional imaging (plain abdominal radiography excretory urogram) and a DMSA renal scan performed 24 hours before ESWL and at least 6 months after treatment. RESULTS: Treatment was successful in 84.6% of cases, by achieving stone-free status after one session and 100% after 2 sessions. Long-term follow-up (one month to four years) did not reveal any blood pressure or renal function changes. No acquired parenchymal scar was identified. On DMSA renal scan. CONCLUSIONS: The efficacy of ESWL in treating infant urolithiasis is clearly established. Renal parenchymal damage associated with ESWL did not appear in this study, but long-term follow-up mandatory (blood pressure) and each infant should have a DMSA renal scan before and after ESWL to confirm these preliminary results on a larger series.

99mTechnetium-dimercapto-succinic acid renal scan in the evaluation of potential long-term renal parenchymal damage associated with extracorporeal shock wave lithotripsy in children.

PURPOSE: Extracorporeal shock wave lithotripsy (ESWL) has been reported as efficient and safe in children. Because of reports of renal parenchymal damage in adults, this study was designed to assess the effects of ESWL in pediatric kidneys evaluated before and after treatment with (99m)technetium dimercapto-succinic acid (DMSA) renal scan. MATERIALS AND METHODS: A total of 15 children, 9 months to 15 years old (mean age 6.5 years), underwent ESWL treatment for urolithiasis. Evaluation imaging included plain abdominal radiography, excretory urogram and/or renal sonography. DMSA renal scan was performed 24 hours before ESWL and at least 6 months after treatment. RESULTS: ESWL was performed in 1 session for 8 patients, 2 sessions for 6 and 3 sessions for 1, delivering a range of 600 to 3,000 shock waves per session. Treatment was successful in achieving stone-free status in 87% of the cases. Long-term followup (1 to 5 years) showed no blood pressure changes. On DMSA renal scan no acquired parenchymal scar was identified at least 6 months after ESWL treatment. CONCLUSIONS: The efficacy of ESWL in treating pediatric urolithiasis is confirmed. Renal parenchymal trauma associated with ESWL does not appear to cause long-term lesions identifiable by DMSA renal scan.

[Extracorporeal shockwave lithotripsy in children. Study of the effectiveness and renal consequences in a series of eighteen children]. / Lithotripsie extracorporelle par ondes de choc chez l'enfant. Etude de l'efficacité et des conséquences rénales sur une série de 18 enfants.

17 children from 18 months to 16 years old were treated by ESWL for calculi of the upper urinary tract using a Sonolith 3000 lithotriptor. 31 calculi (5 to 60 mm) have been treated on 16 renal units and 3 pelvic ureters. The renal parenchyma of all treated kidneys was considered normal on preoperative ultrasound and IVU; 8 patients had a preoperative DMSA. The treatment required 1 to 4 sessions delivering 400 to 3000 shocks per session and 400 to 6000 shocks per treatment at 14 kV. Within three months post treatment, 14 patients (88.23%) were stone free and 3 patients had residual fragments (2 needed further surgical extraction). With a follow up of three months to five years, all patients have a normal blood pressure, serum creatinine and sterile urine. In no instance, IVU or ultrasound revealed a lesion imputable to ESWL. Seven patients had a DMSA six months or more after the last session; 4 of these were normal but the three other patients had a loss of function and an heterogeneous fixation on the treated side. These preliminary results confirm that ESWL is efficient for upper urinary tract calculi destruction in children. However ESWL seems to be susceptible to induce parenchymal damage, detected by DMSA, whose incidence and long term effects particularly on blood pressure need further evaluation.

[Measurement of global and separate renal blood flow using cine-computed tomography]. / Mesure du débit sanguin rénal global et séparé par la ciné-tomodensitométrie.

EBCT flow study offer a promising new approach to measure the renal blood flow. In vitro and in vivo studies were performed to resolve methological problems such as checking the linear relation between contras concentration and Hounsfield units or determination of the Treshold used for the mapping. First measurements of renal volumes and flow performed in patients showed expected values.

Left ventricular function during weaning of patients with chronic obstructive pulmonary disease.

OBJECTIVE: Determine the evolution of left ventricular ejection fraction during weaning. DESIGN: Prospective study. SETTING: Intensive care unit of a university teaching hospital. PATIENTS AND PARTICIPANTS: 12 consecutive mechanically ventilated patients, without documented coronary artery disease, suffering from acute exacerbation of chronic obstructive pulmonary disease and able to be weaned. MEASUREMENTS AND RESULTS: Left ventricular ejection fraction was determined during mechanical ventilation, inspiratory pressure support (10 cmH2O) and spontaneous ventilation with constant inspiratory oxygen fraction using technetium 99m radionuclide angiography. Spontaneous ventilation induced a significant decrease in left ventricular ejection fraction from 54.5 +/- 12.4 to 47.0 +/- 13% (p < 0.01). Inspiratory pressure support induced a slight but non-significant decrease in left ventricular ejection fraction from 55.0 +/- 12.1 to 50.3 +/- 12.4%. Left ventricular ejection fraction was homogeneously reduced by spontaneous ventilation without patent regional wall motion abnormalities of the left ventricle. Myocardial 201thallium imaging performed 15 min after weaning showed a normal perfusion in the left ventricle anterior and posterior free wall. CONCLUSION: Weaning of patients suffering from chronic obstructive pulmonary disease without coronary artery disease induced a significant reduction in left ventricular ejection fraction. The non significant decrease in left ventricular ejection fraction observed with inspiratory pressure support suggested that our results might be explained by a weaning induced increase in afterload.

Diagnosis of malignancy in thyroid nodules by factor analysis of spectral and dynamic structures: a simultaneous dual-isotope dynamic study with thallium-201 and iodine-131.

The aim of this study was to identify malignant thyroid nodules using iodine-123 and thallium-201 simultaneous dynamic acquisition. The image sequences acquired were processed by factor analysis of spectral and dynamic structures (FASDS). Some 49 patients were investigated, and their diagnoses were confirmed by histological examination. Data processing enables the estimation of the spectra of the two isotopes and the evaluation of the kinetics and spatial structures related to each tracer. The superimposition of thallium and iodide sum images allowed us to delineate the nodule accurately. Two groups were defined: 21 patients who had 201Tl uptake in the nodule, and 28 who had none. In the first group, 5 nodules were carcinomas, whereas all nodules in the second group were benign. The results of the 201Tl dynamic study improved the diagnosis of carcinoma as the number of false-positive cases decreased. FASDS succeeds in extracting spectral and kinetic information, proving its usefulness in clinical diagnosis.

[Fortuitous discovery of the association of true and false aneurysm of the left ventricle after cardiogenic shock]. / Découverte fortuite de l'association d'un vrai et d'un faux anévrysme du ventricule gauche au décours d'un choc cardiogénique.

The authors report the fortuitous diagnosis of a true and a false left ventricular aneurysm in a 77 year old man with severe ischaemic heart disease and calcific aortic stenosis, admitted for cardiogenic shock. The association of these two forms of aneurysm is very rare. Clinical and paraclinical diagnosis is difficult. Conventional left ventriculography is the investigation of reference but the diagnosis has been facilitated by Technetium 99 cardiac scintigraphy and color Doppler echocardiography. Surgery may be considered in cases of true aneurysm especially in patients with left ventricular failure, but the indication is formal in cases of false aneurysm.

[Wilson's disease with liver copper in normal concentration]. / Maladie de Wilson avec concentration hépatique en cuivre normale.

The cases of 2 children with Wilson's disease revealed by persistent hypertransaminasemia are reported. Blood ceruloplasmin concentration was low but the liver content of copper was lower than usually seen in presymptomatic forms of the disease. The apparently low liver copper concentration could be explained by an unusually important steatosis.