[Curable form of renal thrombotic micro-angiopathy in children. Anatomo-clinical study of 4 cases].
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Biomedical subjects
Publications and source records attributed to R Dumas.
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Two cases of moyamoya disease observed in two children are reported. The two cases recall the frequency of ischemic strokes, transient ischemic attacks, and seizures revealing the disease. Diagnosis is made by cerebral arteriography showing carotid stenosis and the dense deep arterial collateral as seen in these two cases. Magnetic resonance imaging makes it possible to identify this vascular disease as it reveals vascular varicosities in the basal ganglia, as in case 1. Strokes can sometimes be prevented by constructing an anastomosis between the superficial temporal artery and the middle cerebral artery, as demonstrated in case 1. Thus, the diagnosis of moyamoya disease has been improved by magnetic resonance imaging and there is now a surgical treatment to prevent ischemic stroke.
Six cases of ischaemic stroke which occurred during sporting activities are reported. The authors insist on the unusual features of the ischaemic events which had the same characteristics as other ischaemic strokes in young subjects: frequent involvement of the main cerebral arteries, good vital and functional prognosis in short--and mid-term. They consider that three factors might have concurred in precipitating the ischaemic accident, viz.: fibromuscular dysplasia (observed in 3 patients), warm summer weather encouraging haemoconcentration and poor physical training in 5 out of 6 cases.
A clinical history typical of multisclerosis began in a 20-year old man with transient, then permanent manifestations involving the optic tract and the pyramidal, extrapyramidal and cerebellar systems. The patient died at the age of 62, at the end-stage of a complex clinical situation which included paraplegia, bilateral cerebellar syndrome, optic nerve atrophy, epileptic seizures and dementia. When the patient was 54 years old, laboratory findings suggestive of systemic lupus erythematosus (SLE) were discovered, namely: antinuclear, native anti-DNA, anti-Sm antibodies, circulating anticoagulant, cryoglobulinaemia and low complement level. These abnormalities persisted up to the patient's death, 8 years later, without any non-neurological sign of SLE. Post-mortem examination showed lesions of focal demyelination characteristic of multiple sclerosis, but no evidence of cerebral or extracerebral SLE. This case raises the problem of borderlines or associations between systemic lupus erythematosus and multiple sclerosis. In our case, as in other cases of "lupoid sclerosis" reported in the literature, there was a frank and isolated elevation of serum IgM levels.
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We evaluated prospectively the occurrence of seizures within 15 days of a first stroke or transient ischemic episode in 1,640 patients to study relation between seizures and type of stroke. Seizures occurred in 90 patients (5.4%), including 36 (4.4%) of 814 with infarct owing to atheroma, 21 (16.6%) of 126 with infarct owing to cardiogenic embolus, 3 (1%) of 273 owing to lacunar infarct, 5 (1.9%) of 259 owing to transient ischemic attack (TIA), 21 (16.2%) of 129 owing to supratentorial hematoma, and 4 (16.6%) of 24 owing to subarachnoid hemorrhage. Thirteen (14.6%) of 89 subcortical infarcts were associated with seizures. Seizures were the initial sign of stroke in 80 (89%) of 90 cases and were usually single and partial. Seizure symptoms were most often motor, sensory, or visual.
5-Hydroxyindoleacetic acid (5-HIAA) and homovanillic acid (HVA) were measured by high-performance liquid chromatography (HPLC) in lumbar cerebrospinal fluid (CSF) obtained from febrile children subdivided according to the presence or absence of convulsions. Lumbar puncture was made either early (mean time 2 h) or late (3-6 days) after the febrile convulsion. The level of 5-HIAA was significantly decreased in children early and late after the febrile convulsion as compared with the convulsion-free group, but the HVA level was reduced only early after the febrile convulsion. These results support the hypothesis that a decrease in CSF 5-HIAA may be a biologic marker of susceptibility to convulsions and indicate that the transient decrease in HVA is a secondary phenomenon related to occurrence of convulsions.
The purposes of this program were to determine whether participants gained more comprehensive knowledge about depression and if that knowledge was applied in practice following attendance at a 3-day educational workshop. Twelve programs were conducted in three southwestern states. The majority of the 363 participants were Anglo-White female registered nurses whose ages ranged from 23 to 74 years and who worked in general medical-surgical units, nursing homes, or community health settings. This study was a repeated-measures design to evaluate the effects of depression education on depression knowledge and its clinical application. Participants demonstrated increased depression knowledge and practice application following the program. Knowledge of depression remained stable across postprogram measures. Although application of interventions decreased between 6 weeks and 6 months, assessment skills remained stable. Mental health nurses can improve patient outcomes through programs such as the one described by educating nurses in the detection and management of depression.
Two clinical studies were conducted to evaluate the safety and immunogenicity of concomitant administration of a new inactivated hepatitis A (HA) vaccine and either a typhoid fever (Vi) vaccine or a combination of Vi and yellow fever (YF) vaccines. In study 1, 62 healthy adults received HA+Vi into the deltoid muscle on contralateral sides. In study 2, 59 healthy adults received HA and a combined Vi/YF vaccine into the contralateral deltoid muscles. Reactogenicity was evaluated for 14 days following vaccination. Blood samples (for antibody titration) were obtained prior to vaccination on days 0 and 28 in study 1 and prior to vaccination on day 0 and on days 14 and 28 in study 2. The overall rate of local reactions was 19% at the HA injection site and 75% at the Vi injection site in study 1; the rate of systemic reactions was 41%. In study 2, local reactions occurred at 27% and 78% of the HA and Vi/YF injection sites; the rate of systemic reactions was 42%. All reactions were transient. Twenty-eight days after vaccination, the seroconversion rate was 100% for HA antibodies and 90% for the Vi vaccine in study 1. Rates of seroconversion in study 2 were 100% for the HA vaccine, 92% for the Vi vaccine, and 100% for the YF vaccine. Although this study was not comparative, both tested combinations were safe and immunogenic, and their routine use for persons at risk, such as travelers, can be recommended.
BACKGROUND/AIMS: Self-expanding metal stents (SEMS) represent a major advancement in the palliative treatment of dysphagia caused by neoplasms of the esophagus. Malignant cervical stenoses are a challenge for palliative techniques, due to their close relationship with the cricopharynx. METHODOLOGY: Six patients with a malignant stricture of the upper esophagus, within 2 cm of the cricopharyngeal muscle, were treated with a self-expanding metal stent. All patients had a circumferential squamous cell carcinoma histologically proven. Patients were intubated under general anesthesia, and the procedure was carried out under simultaneous endoscopic and fluoroscopic control. RESULTS: All patients had a remarkable improvement of dysphagia and none of them reported a foreign body sensation. In 3 patients, an insufficient expansion of the stent, detected 24 hours later, required a balloon dilation in two of them, whereas, for the third patient, it was necessary to introduce a second stent. Neoplastic in- and overgrowth occurred in 4 (67%) patients after 1, 2, 4 and 8 months, respectively. These complications were managed by placing a second stent in 3 patients; in the fourth patient, a neoplastic involvement of the cricopharynx did not allow for an endoscopic examination. Improvement of dysphagia was observed only in the patient who received a covered Cook-Z stent. In the other 2 patients, a percutaneous endoscopic gastrostomy was performed. The only major complication occurred in the patient who received three metal stents, as he complained of severe cervical pain. CONCLUSIONS: Our experience shows that uncovered self-expanding metal stents provide a good palliation in this subgroup of patients. Tumour ingrowth, overgrowth, and the progressive impairment of the swallowing mechanism due to a proximal submucosal infiltration remain complications difficult to solve.
PURPOSE: To evaluate the efficacy of through-the-scope metal stents for palliation of malignant duodenal stenosis. MATERIAL AND METHODS: Fourty two patients with malignant primary or secondary duodenal stenoses who were treated with a through-the-scope metal stent were analysed. When obstructive jaundice occurred either before, during, or after the initial episode of gastrointestinal luminal obstruction, a biliary stent was inserted. RESULTS: Duodenal metal stents were deployed in 40 patients. Endoprosthesis insertion led to restoration of oral intake in 39 patients. The procedure was not associated with morbidity or mortality. During a mean follow-up of 9.7 weeks, adequate oral intake was maintained in 38/39 cases. Tumour in-growth led to stent occlusion in 4 cases and re-cannulation was obtained by placement of another stent within the original stent. Obstructive jaundice occurred during the course of the illness in 32 patients and was successfully treated with a biliary metal stent in all cases. CONCLUSIONS: Endoscopically placed metal stents offer an effective, well-tolerated alternative to surgical palliation in case of incurable malignant obstruction to gastric outflow.
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BACKGROUND: The effect of calcium restriction on the plasma concentration of 1,25(OH)2D in normo- and hypercalciuric children remains unknown. METHODS: We studied phosphate and calcium metabolism of 8 normocalciuric and 8 hypercalciuric children aged 4 to 16 years, under 3 conditions: on a normal dietary calcium intake after a 5-day calcium-restricted diet, and after oral calcium loading. The healthy, normocalciuric children had histories that included no renal failure of abnormalities of phosphate and calcium metabolism. Four of the 8 hypercalciuric children had urolithiasis, 1 had hematuria and the 3 others had idiopathic hypercalciuria. Blood samples were analyzed for calcium, creatinine, immunoreactive parathyroid hormone, cAMP, 25(OH)D and 1,25(OH)2D concentrations. Urine samples were analyzed for calcium, phosphorus, creatinine and cAMP. RESULTS: On the normal dietary calcium intake, the hypercalciuric children had higher urinary calcium excretion and plasma 1,25(OH)2D levels and lower TmP that did the controls. The 1,25(OH)2D levels of the normocalciuric children were significantly increased after 5 days of dietary calcium deprivation, but those of the hypercalciuric children were not. The other parameters (essentially PTH, cAMP and TmP) varied similarly in the two groups. CONCLUSION: The results suggest that: a) calcium restriction influences 1,25(OH)2D levels in normocalciuric subjects via a PTH- and phosphor-independent mechanism; b) dietary control of renal vitamin D metabolism is impaired in hypercalciuric patients.
The immediate infectious pancreato-biliary complications of endoscopic retrograde cholangiopancreatography (ERCP) warranting transfer to a surgical unit are analyzed, in order to evaluate their frequency and severity as well as means of treatment and prevention. Thirty complications of this type were observed in a series of 3226 ERCP performed with or without endoscopic sphincterotomy (ES) over a six year period (0.9%). ES had been performed in 12 of 30 cases, but the complication could not be attributed to the procedure. Post-ERCP complications included: acute cholangitis: 16 cases (53%); acute cholecystitis: 8 cases (26%); acute pancreatitis: 4 cases (13%); infected pancreatic pseudocyst: 2 cases (6%). The global mortality rate was 16.6% (five patients): Four of the deaths were due to septic complications. Twenty-seven of the 30 patients underwent surgery, and three of them died (11%). Acute cholangitis was responsible for most of the deaths (four of five) and the mortality appeared related to the long interval before surgery (three of four deaths). Strict adherence to good endoscopic procedures (aseptic conditions, injection without excessive pressure, antibiotic prophylaxis) and decompression of the biliary tract (nasobiliary drain or transhepatic catheter) should help reduce the frequency of post-ERCP complications, and especially cholangitis, which appears to benefit from early surgical treatment.
Two children, 13 and 14 years old, presented an intractable epilepsy of Lennox-Gastaut. In front of dangerous tonic and atonic epileptic crisis, a corpus callosotomy was performed. Corpus callosotomy is becoming a more widely used procedure in the treatment of intractable epilepsy as Lennox-Gastaut syndrome, or frontal epilepsy. However, there have been very few series that have reported results in children. It is not a complete but a partial, callosal section including approximately the anterior two thirds of the callosum. After this surgery the two children improved dramatically because the dangerous tonic and atonic crisis disappeared. Secondly we observed improvement of pre-surgical psychiatric troubles, that is a data not developed in the literature. Before surgery, the two children had a frontal syndrome with hyperkinesia, distractibility, aggressiveness, alexithymia, loss of the program of ideas. During the two months after the section of the anterior two thirds of the corpus callosum, we observed a progressive improvement of the frontal syndrome, with possibility to learn new praxies. The intellectual quotient was not altered and associative functions, depending of the posterior third of the corpus callosum were spared. Anti-epileptic medications were not stopped. We think that the improvement of the frontal syndrome is due to reduction of seizures. Therefore, we insist on the interest of the section of the anterior two thirds of the corpus callosum as treatment of tonic and atonic seizures but also as treatment of psychiatric symptoms depending of a frontal syndrome.