[Pneumothorax in a renal transplant recipient with legionnaires' disease].
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Biomedical subjects
Publications and source records attributed to C Loirat.
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A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.
Metaphyseal sclerotic bone changes associated with benign phaeochromocytoma are very rare in childhood. We report four cases, in each of which the radiographic changes returned to normal after removal of the tumour.
The Prune Belly syndrome (PBS) is unfrequent. Fourteen cases have been followed in our unit during the last 20 years. Four infants (29%) died during the first months of life, because of neonatal sepsis (2 cases) or end-stage renal failure (2 cases). Among the other 10 cases, 6 (43%) had normal glomerular filtration rate at a mean age of 10 years 6 months (6 months to 15 years), 4 had chronic renal failure, including 3 cases who developed end-stage renal failure at 8, 8 years 8 months and 17 years respectively. Resection of an urethral obstruction was performed in 2 cases. This surgical indication remains widely accepted, while the current tendency is to limit ureteral surgery in PBS. Orchidopexy was performed in 4 children, 3 of them less than 6 years 6 months of age. Fertility of these early operated children remains to be established, as all adults reported in the literature remain sterile when orchidopexy was not performed or was performed after age 6.
This study was undertaken to evaluate the reliability of ambulatory blood pressure monitoring (ABPM) in children and its diagnostic value in teenagers with borderline hypertension. The apparatus used was a Nippon Colin (NC) ABPM 630 which simultaneously obtains BP levels by the auscultatory and oscillometric methods. Among 61 children (mean age 12 +/- 3 years), proportion of measurement failures was 14% (470/3399) for oscillometry, 26.5% (893/3360) for auscultation, and 2.7% for both methods together. Comparison of conventional auscultatory blood pressure measurements (mercury manometer) and simultaneous NC-auscultatory BP values (50 pairs) showed slightly lower diastolic blood pressure values with the NC monitor (-3.8 +/- 4.9 mmHg, p less than 0.01). Comparison of blood pressure values provided by the two NC methods (1580 pairs) demonstrated slightly higher systolic blood pressure values (+2.5 +/- 4 mmHg; p less than 0.001) and slightly lower diastolic blood pressure values (-5 +/- 4 mmHg; p less than 0.001) with the oscillometric method. Thirty-four ambulatory blood pressure recordings were obtained in 28 children (mean age 13.3: range 8-17) with outpatient clinic blood pressure values suggestive of borderline hypertension. Numbers of ambulatory blood pressure readings were 44 +/- 5 during the day and 9 +/- 1 during the night. The mean of daytime blood pressure values was compared with reference blood pressure values obtained at rest in normal French children.(ABSTRACT TRUNCATED AT 250 WORDS)
The immune response after hepatitis B (HB) vaccine HEVAC B was studied in 33 children (mean age 10 +/- 4 years) with advanced renal failure. Responders and protected patients were defined by antibody titres to HB surface antigen (anti-HBs) of greater than 10 and 50 mIU/ml, respectively. All received the initial recommended three injections at monthly intervals, and 23 received a booster injection (IB) 11 +/- 1 months after the third injection (I3). Loss of protection after I3 led to additional injections in 8 patients (25%). Vaccine was well tolerated and no HB infection occurred during the follow-up period (19 +/- 10 months). The percentage of responders was 91% 2 +/- 1 months after I3, and 100% 1 month, 13 +/- 1 months and 26 +/- 2 months after IB. The percentages of protected patients at these dates were 91%, 95%, 100% and 100%. Anti-HBs titres 1-3 months after I3 were useful for indicating those patients likely to have a rapid decline in anti-HBs titres, thus requiring serial anti-HBs determinations and additional injections to prevent a loss of protection. We conclude that at the expense of a reinforced vaccination schedule in 25% of patients, HEV AC B vaccine can safely achieve a sustained protection in more than 90% of uraemic children.
Three hundred and eighty children underwent 434 angioaccesses. Of these angioaccesses, 113 were constructed in 74 children weighing under 10 kg. Most accesses (n = 340) were distal arteriovenous fistulae (AVF). After microsurgery there was a 96% immediate patency. Seventy percent of AVF, excluding distal ulnarbasilic AVF, were functional, sometimes after secondary superficialization of the vein. Eighty-five per cent of the distal radial-cephalic AVF are still patent after 2 years, 60% are still patent after 4 years. These radial-cephalic AVF required 65 repeat anastomoses, and 12 ligations of the proximal-radial artery in order to reduce to 50% the high blood flow (pre-reduction average index = 900 ml/min per m2. The patency rate of arteriovenous-bridge grafts was not encouraging. The severity of stenoses in the proximal-venous trunks, sometimes related to previous catheterization, is emphasized. Advantages of the radial-cephalic wrist AVF in children are highlighted.
Diffuse mesangial sclerosis (DMS) has been described as a distinct morphological pattern observed in patients presenting with a congenital or infantile nephrotic syndrome (NS) leading to end stage renal failure (ESRF) before the age of 3 years (Habib & Bois: Helv. Peadiat. Acta 28: 91-107, 1973). In recent years, we diagnosed this entity in 36 infants and we wish to report our findings which extend our previous observations. The nephropathy was discovered before 1 year of age in 26 patients. In one of these the onset was neonatal. Five additional children were between 1 and 2 years of age at onset of the disease and the remaining 5 were between 24 and 42 months old. Thirty-three patients presented with a NS often preceded by a proteinuria and associated with renal failure in 6 of them. The 3 remaining patients presented with renal failure. Thirty-one patients reached ESRF before the age of 4 years. Progression was slower in 5 patients who were respectively 5, 5, 6, 8 and 11 years old at ESRF. Thirteen patients were transplanted and none recurred their original disease. Seven patients in 4 families had similarly affected siblings. In 16 patients (6 boys and 10 girls) the nephropathy was isolated. In the remaining 20 several associated findings were noted. In 14 infants a diagnosis of Drash syndrome was made on the basis of the association of a male pseudo-hermaphroditism (PsH) and Wilms tumour (WT) in 5, of a male PsH in 5 additional infants and of a WT in 4 female patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Recent studies have suggested that patients with neonatal onset of severe renal failure may be at risk for mental retardation. We studied the intellectual development of 13 pediatric patients with neonatal onset of severe renal failure who immediately received active medical therapy. The verbal, performance, and overall intelligence quotient (IQ) was determined using the Weschler-PPSI and the WISC-R at a mean age of 7 years and 4 months (4 years 5 months to 15 years 8 months). Mean overall IQ was 89 (73 to 106), mean verbal IQ was 94.7 (79 to 124) and mean performance IQ was 85.6 (69 to 112). Overall IQ was greater than 100 (101 to 106) in three patients (23%), and under 100 in ten patients (77%), with values of 87 to 92 in six cases (46%) and 73 to 84 in four cases (31%). A significant negative correlation (p less than 0.05) was found between the IQ and the length of hospital or medical institution stays during the first year of life. We found no significant correlation either between IQ and severity of renal failure, quality of growth or nutritional status before the age of two, or between IQ and dose of aluminum ingested. Our results suggest that early severe renal failure does not induce severe mental retardation. However, in 77% of studied children the IQ was within the lower portion of the normal range or under this normal range. This proportion is greater than that seen in normal children from similar socioeconomic backgrounds. Reducing hospital stays and separations from home, as well as even more active nutritional management, may help to improve these children's mental abilities.
Seventy-nine children with haemolytic uraemic syndrome (mean age 28 months) were randomly assigned either to a group receiving plasma infusions (plasma group, n = 39) or to a group treated conservatively (control group, n = 40). The duration of haemolysis, thrombocytopenia and anuria was similar in the two groups. Serum creatinine levels were similar in the two groups at the 1-month follow-up but were higher in the control group at 3 months (plasma group 49 +/- 14, control group 66 +/- 28 mumol/l; P less than 0.02) and at 6 months (plasma group 48 +/- 13, control group 63 +/- 21 mumol/l; P less than 0.005). The prevalence of proteinuria was also higher in the control group at the 6-month follow-up (plasma group 17%, control group 46%; P less than 0.02). However, differences were no longer significant after 1 year. Renal tissue was examined in 54 cases (plasma group, n = 27; control group, n = 27). Diffuse cortical necrosis was present in 7 cases in the control group but was absent in the plasma group (P less than 0.02). Taking into consideration the higher serum creatinine levels, the higher prevalence of proteinuria during the first 6 months of follow-up in the control group and the presence of diffuse cortical necrosis in this group compared with the plasma group, we conclude that plasma infusions should be regarded as beneficial. Further study is needed to determine which plasma fraction is involved.
Twelve Hickman catheters were inserted in nine children in order to establish access for haemodialysis or plasmapheresis. Catheters were implanted either through the external or internal jugular vein and the tip located in the right atrium or superior vena cava. Mean blood flow was 25-55 ml/min with single lumen catheters and 83-100 ml/min with double lumen catheters. Three catheters had to be removed because of obstruction, whilst seven remained in situ until an arteriovenous fistula had matured or renal function was restored. Infection in two cases was successfully treated with antibiotics and transient obstruction by urokinase instillation. Following catheter removal, angiographic studies showed that with one exception all catheterized vessels were obstructed, but this did not prevent from ipsilateral arteriovenous fistulas to mature satisfactorily.
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We report the cases of two children presenting with tumor lysis syndrome responsible for major hyperphosphatemia, hypocalcemia and acute renal failure and treated by hemodialysis. Twenty similar cases have been reported in the literature. Hyperphosphatemia responsible for hypocalcemia and renal failure occurs within 24 to 48 hours after the onset of chemotherapy, is maximal on the 2nd or 3rd day and is, on the average, of 7 days duration. Short-term functional renal prognosis is good but long-term studies are lacking. The usual preventive measures are not always sufficient to prevent these accidents. A dialysis is appropriate when phosphatemia rises rapidly and exceeds 5 mmol/l, when the creatinine plasma level exceeds 200 mumol/l and kaliemia 6 mmol/l and when hyperphosphatemia is associated with severe clinical signs.
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We compared the effect of plasma from 19 children with hemolytic uremic syndrome (HUS) on prostacyclin (PGI2) production by fresh rat aortic rings to the effect of plasma from 17 age- and sex-matched normal children, taking into account the PGI2 baseline aortic production (PGI2 release in presence of buffer, 21 determinations). After 10, 20, 30, 40, and 60 minutes incubation of rat aortic tissue with either plasma or buffer, the presence of PGI2 was studied by measuring by radioimmunoassay (RIA) the concentration of 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha). 6-keto-PGF1 alpha production increased with time in the two groups of plasma samples and in the presence of buffer, but 6-keto-PGF1 alpha production (ng/mg dried tissue) after 30 minutes incubation and mean 6-keto-PGF1 alpha production (slope of regression line, ng/mg/min) were significantly (P less than 0.01) lower in the presence of normal plasma compared with buffer, and significantly (P less than 0.01) higher in the presence of HUS plasma compared with normal plasma. There was no significant difference between buffer and HUS plasma. We conclude that, under our experimental conditions, normal plasma had an inhibitory activity on 6-keto-PGF1 alpha production by rat aorta. This inhibitory activity was absent in HUS plasma.
This study was performed to assess myocardial involvement in 18 children with severe hypertension (HT), using two dimensional (2D) guided M mode echocardiography, prior and during therapy. All patients but 2 had renal or renovascular disease. Septal diastolic thickness (SDT) was utilized as a serial marker. Except for one case, all patients had increased SDT initially (1.03 +/- .26 cm/m2, p less than .01 vs normal). Evolution under therapy allowed subdivision of patients: Group I: 12 patients showed left ventricular (LV) hypertrophy regression, within a follow-up period of 20 +/- 9 months (final SDT: .78 +/- .12 cm/m2 vs initial 1.09 +/- .28, p less than .01). Blood pressure (BP) was normalized in 9 patients, and borderline in 3. Therapy consisted on acebutolol (n = 10), captopril (n = 1), and renal artery surgery (n = 1). Group II: LV hypertrophy was unchanged (n = 3) or increased (n = 3), within a follow-up period of 19 +/- 8 months, with persistent severe (n = 3) or mild (n = 3) HT, under acebutolol (n = 5). Treatment was changed to captopril with subsequent normal BP and echocardiogram improvement (n = 3). In the overall population, final SDT was significantly correlated to the final BP (r = .69, p less than .01). In conclusion, echocardiographic follow-up allowed serial non invasive assessment of LV hypertrophy in our severely hypertensive pediatric population. At first echocardiogram, LV hypertrophy was present in all patients but one. Antihypertensive therapy allowed simultaneous decrease of BP and LV hypertrophy in 12 patients, 10 under acebutolol.