Biomedical subjects
Publications and source records attributed to C Piussan.
Following the occurrence in two siblings of parvovirus B19-related acute transient erythroblastopenia with specific IgMs, hereditary spherocytosis (HS) was diagnosed for the first time in the two patients as well as in three other members of the family.
We report a case of respiratory arrest in a ten-year-old asthmatic girl under beta-2-agonists and theophylline. The possibility that hypokalemia plays a part in the sudden deaths seen in some asthmatic patients is discussed.
A retrospective study comparing the findings of ultrasonography (5 MHz transducer) versus intravenous pyelography and voiding cystourethrography was conducted in 92 patients during the course of an initial urinary tract infection (greater than or equal to 10(5) bacteriae/ml, WBC greater than 25/mm3) gathered over a period of 5 years. The average interval of time between ultrasonography and intravenous pyelography was 8 days (range 1-70). False positive results for ultrasonography were noted in 11% (5 of 45 cases), while false negatives were observed in 36% (17 of 47). Of the 47 cases of obstructive uropathy diagnosed by conventional means, ultrasonography missed the diagnosis in 1 case of ureteral duplication as well as in 16 children with vesicoureteral reflux. In comparison, intravenous pyelography missed 8 of these 21 cases of vesicoureteral reflux. Overall sensitivity of ultrasonography was mediocre at 64% but excellent at 96% after excluding cases with vesicoureteral reflux. Specificity was poor for the group of patients with vesicoureteral reflux (33%) and did not improve when they were excluded (40%). Sensitivity and specificity were comparable whatever the age group. In conclusion, we would recommend that when both ultrasonography and voiding cystourethrography are normal in children with a urinary tract infection, intravenous pyelography could safely be deferred.
We report seven cases of acute renal failure (ARF) treated by continuous ambulatory peritoneal dialysis (CAPD). ARF was caused by hemolytic uremic syndrome in six patients and acute fetal distress in one neonate. Mean age was 28 months (range 6 days-6 years). A flexible Tenckhoff catheter was inserted surgically under brief (10 minutes) general anesthesia in all patients. During the first 24 hours, in order to avoid clogging of the catheter, exchanges were done every 45 minutes with 20 ml/kg dialysate initially, gradually increased to 40-50 ml/kg. Thereafter, 5 to 8 exchanges were done every day (mean duration 9 days, range 4-21) until creatinine clearance reached 15 ml/mn/1.73 m2. No case of peritonitis or leakage was recorded. In two patients, migration of the straight catheter caused flow obstruction. None of the crook-shaped catheters migrated. Because dialysis was continuous and fluid balance could be controlled by using a hypertonic dialysate if called for, optimal protein (1-1.5 g/kg/d) and energy intake were possible. This intake was ensured mainly by continuous enteral nutrition. Improved patient comfort (no immobilization, no repeated punctures) and staff comfort (technical simplicity, independence from a cycler) are further non-negligeable advantages of CAPD.
We report two exceedingly unusual and severe cases of Marfan syndrome with neonatal manifestations. Clinical features were caricatural. The skin was of the cutis laxa type. Both infants died shortly after birth as a result of severe cardiovascular lesions. Histologic examination of the aortic media confirmed the diagnosis. Because this genetic connective tissue dysplasia is inherited on an autosomal dominant basis with strong penetrance, and because in both instances the two parents had a negative family history, a dominant mutation seems likely in our cases and prompted us to be optimistic when giving genetic counseling. We point out the characteristic features of these exceptional early forms and describe the results of a detailed histologic examination of the skin that completes data from the literature.
We report the case of a 15-year-old who sustained neonatal distress and subsequently presented temporal seizures. These were ascribed only several years later to a temporal astrocytoma. Tumor-related epilepsy is infrequent in children. Tumors may be responsible for a pattern of isolated partial epilepsy of several years duration. Advances in medical imaging techniques ensure earlier diagnosis, but some CT scan images may be difficult to read. CT scan is not indicated in functional epilepsy but should be performed in partial epilepsy with no known etiology.
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After a protein load, the glomerular filtration increases to a maximum called maximal filtration capacity (MFC). The difference of this value with that observed in the fasting state defines the functional renal reserve (FRR). Fifty oral protein loads have been performed in 16 controls (13 adults and 3 children) and 31 patients with kidney disease or hypertension (22 adults, 9 children, 3 tests being performed twice). The load consisted in the adults of 80 g of various proteins. In the children the load consisted in the 100% of the protein intake recommended of the whole day. The urinary samples were made hourly during the 5 hours following the beginning of the meal. The MFC was not always observed during the 2 hours following the end of the meal. However the mean of the clearances measured during these 2 hours allowed an acceptable evaluation of the FRR in 42 out of 50 cases and had the advantage to reduce the errors linked to incomplete bladder voiding. In the adult controls the mean of hourly determined MFC is 156 +/- 27 ml/min, the mean of the clearances measured on the 2 hours following the meal is 136 +/- 21 ml/min and the fasting clearance 96 +/- 22 ml/min. The FRR is null in all patients with a nephropathy with a fasting clearance below 40 ml/min. It is modest or null in patients with acquired or congenital solitary kidney. Three out of five patients with proteinuria have MFC and FRR higher than the mean of the patients with comparable fasting clearances but without proteinuria suggesting that proteinuria is associated with a hyperfiltration state.
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Seventy-four children with juvenile chronic arthritis were entered into a 6-month, multicenter, comparative double-blind study of the efficacy of D-penicillamine versus placebo. The results were evaluated in 70 patients, 55 of whom completed 6 months of the study. Improvement was observed in the total number of stiff joints, total number of painful joints, and total severity index measuring joint pain. There was also a significant reduction in the concurrent use of nonsteroidal anti-inflammatory drugs. D-penicillamine was well-tolerated in all but 2 patients. Some children in the placebo group exhibited definite improvement; however, relapses that were observed were mainly in that group. These results confirm the efficacy of D-penicillamine for the treatment of joint involvement in juvenile chronic arthritis.
The authors work in an area located north of Paris, which contains roughly a population of .75 million, of which 82,000 are children less than 3 years of age. The center and main city of the area is the town of Amiens. The recruitment of their cases under study was essentially hospital based, which explains the relative severity of the trauma observed. The children were followed by various observation systems. Seventy-four children, of which more than two-thirds were less than 3 years of age were followed for a period varying from two to ten years. Forty-three percent of these children have been maintained in their biological families; the others have been placed. With this system only nine recurrences of abuse have been observed (12%), and only two instances of death have been observed. Other studies have demonstrated higher proportions. However, in two-thirds of the children observed, there were minor or major psychological or even psychiatric sequelae. Protection services have tried to stay away from institutional placement as much as possible; when necessary, the foster home system or adoption has been used. Striking is the lack of means available to help abusing families. Efforts should be made to assist the abusing families stay together.
Seventy-four children with juvenile chronic arthritis were admitted to a 6-month multicentric double blind comparative study of the efficiency of D-Penicillamine or placebo. The results evaluated in 55 patients who completed the study, indicated an improvement in the total number of stiff joints and in the total index of severity measuring joint pain and inflammation. A significant reduction of the concurrent use of non steroidal antiinflammatory drugs was also observed. The tolerance was good except in two patients. Some children in the placebo group exhibited a definite improvement.
Adrenocortical tumors are very rare, especially in their non secretory form. A 15 years old boy was hospitalized for evolutive fever since 6 weeks associated with asthenia and abdominal pain. Infections hematological, neurological and system diseases were eliminated. Abdominal ultrasonography reveal a poly-lobular mass adherent to the spleen and the posterior wall of the stomach. This is confirmed by fibroscopy and barium swallow. Abdominal scanner and scintigraphy did not give any new etiological argument. X Ray thorax is normal. Laparotomy reveals a retro-gastric mass adherent by its superior extremity to the spleen. A complete excision is done. The pathological examination shows all the histologic features of adrenocortical carcinoma. Its extension to the spleen grade this tumour with a height malignancy. No chemotherapy was undertaken, only a clinical, ultrasound and radiography observation with a follow up of 13 months.
Three families presenting one or several cases of brain or ophthalmic abnormalities and an hypopituitarism at least by one of the members have been observed. In the first family, the mother and one of her sons present bilateral choroidoretineal coloboma with amblyopia; one of these two suffers as well from panhypopituitarism. In the second family two premature twins, a brother and his sister, present a syndrome with hypophyseal dwarfism and ophthalmic abnormalities, consisting in the boy's case in an peripapillary depigmentation with no visible sight trouble whereas girl's is showing an extreme microphthalmia with major mental retardation. In the third family two 2nd degree cousins present a panhypopituitarism but only one of the two reveals through neuroradiological investigations corpus callosum and septum lucidum agenesia. The karyotype is normal in all the cases. An hereditary mechanism appears clearly in the first family. It is possible in the second, probable in the third one.