PubMed Health⌕ Search

Biomedical subjects

F Leclerc

Publications and source records attributed to F Leclerc.

At least 109 records · Page 6Linked to original sources

Protein C and S deficiency in severe infectious purpura of children: a collaborative study of 40 cases.

We studied, in 40 children (mean age: 52 months) with severe infectious purpura, the relationships between protein C (PC) and protein S (PS) levels, and shock, disseminated intravascular coagulation (DIC) and outcome. We determined, on admission, PC antigen (ELISA) and activity (chromogenic test), and total PS (ELISA). Results were expressed as % of normal adult values. Statistical analysis was performed with SAS. Thirty children were in shock, 20 had DIC. All children with DIC, and 10 without DIC were in shock. Of 20 children who were in shock and had DIC, 7 died and 3 had an amputation. PC antigen was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.05). PC activity was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.005). Total PS was not decreased in shock children, but was significantly decreased in children with DIC (p less than 0.005), and in non-survivors (p less than 0.005). We conclude that PC and PS levels were decreased in our children, and that PC levels were significantly decreased in the presence of shock, DIC, and fatal outcome. PC and antithrombin III (AT III) supplementation, should be evaluated in children with severe infectious purpura with shock and DIC.

Adolescent↗

[Is pericerebral hemorrhage a cause of severe malaise in infants?].

The authors report 7 cases of infants presenting with an apparent life-threatening event associated with acute pericerebral haemorrhage (subarachnoid haemorrhage and/or subdural hematoma) without evidence of traumatism, abuse, or shaking. Clinical characteristics were the same in all cases, including limpness, severe dysautonomic disorders, and pallor; all infants had retinal and pre-retinal haemorrhages. Two infants died; the five survivors have severe neurologic sequelae. The symptoms revealing an infant's pericerebral haemorrhage are usually axial hypotonia and pallor. Traumatism remains the most common aetiology and must be searched for. Non-traumatic aetiologies are unusual and were excluded in these reported cases. The 'shaken baby' syndrome is not the sole aetiology of an apparent spontaneous pericerebral haemorrhage: a slight bump associated with predisposing vascular factors particular to infancy could be involved. When confronted with an apparent life-threatening event associating limpness and pallor, one must consider the diagnosis of pericerebral haemorrhage.

Female↗

[Striatal involvement with abnormal movements in hemolytic-uremic syndrome].

Two children with hemolytic-uremic syndrome (HUS) presented with alteration of consciousness and severe abnormal movements related to striatal involvement. They required dialysis and controlled ventilation. Steady improvement in neurologic function was seen during the following weeks, and abnormal movements disappeared. It is suggested that the pathogenesis of neurological dysfunction in these two children was related to intracerebral microangiopathy, because neither systemic arterial hypertension, nor metabolic disturbances were present. This hypothesis is supported by the cerebral blood flow study in one patient. Neuropathologic studies of patients with HUS who presented with CNS symptoms showed vascular thromboses within the CNS: CT scan demonstrated multiple small areas of infarction, particularly in the basal ganglia. The prognosis of HUS is generally worsened in case of severe CNS involvement, but abnormal movements may disappear without sequelae.

Brain Diseases↗

[Long-term outcome of congenital diaphragmatic hernia. A study of 17 patients].

In order to better define the outcome of patients with neonatal congenital diaphragmatic hernia (CDH), 17 patients between 3 and 19 years of age, among 34 survivors from 100 CDH have been re-examined clinically. All had a lung radiography, lung function studies, and radionuclide (Technetium 99m, Xenon 133) lung scans. Three patients suffered from asthma, 2 had recurrent bronchitis, 4 poor tolerance to effort, 3 gastrooesophageal regurgitation leading to endobrachyoesophagus and oesophagitis in one, 3 had scoliosis. Lung scans demonstrated hypoperfusion of the herniated side (less than 40%) in 6 patients. Chest films showed hypovascularisation on the herniated side. Lung function studies, performed in 4 of these 6 patients, showed a restrictive syndrome in 1 patient. Our results confirm those in the literature: perfusion is more altered than ventilation. Chest films at one year of age, completed if necessary by radionuclide lung scans, allow identification of children who have important pulmonary hypoplasia. These children need a regular follow-up: respiratory, digestive and orthopedic complications must be treated in order to preserve the respiratory function in adulthood.

Adolescent↗

[Hemostasis anomalies and prognosis during severe infectious purpura in children. Retrospective study in 69 cases].

In 69 children with severe infectious purpura (SIP), anomalies of hemostasis on admission were studied retrospectively. Forty-four children presented with disseminated intravascular coagulation (DIC) and 18 with factor VII deficiency +/- thrombocytopenia +/- antithrombin III deficiency. Seven patients were free of hemostasis anomalies. In 5 children, purpura was necrotic on admission, whereas in 9 additional patients skin necrosis occurred subsequently (5 patients with and 4 without DIC). Among the 18 children (26%) who died, 16 were in the DIC group. Factors II and V, fibrinogen and platelet counts were lower in children who died and were correlated with the prognostic score. In the DIC group, however, factor VII + X level was not correlated with the prognostic score and was equally low in fatal and non-fatal cases. Antithrombin III level was markedly decreased in patients who subsequently developed necrosis. These results indicate that factor VII level decreases early in the course of SIP and that consumption of physiologic coagulation inhibitors probably plays a central part in the development of necrotic purpura.

Adolescent↗

[Delayed appearance of left Bochdalek hernia disclosed by intrathoracic gastric perforation].

Late onset congenital diaphragmatic hernia is a rare condition that is difficult to diagnose. Complications such as intestinal obstruction and digestive perforation may occur. A previously healthy three year-old girl was admitted to hospital with dehydration, shock, and ketoacidosis. A chest X-ray film showed a left hydropneumothorax with an indefinable left diaphragm, that was confirmed by ultrasonography. Hexabrix introduced through a nasogastric tube showed opacification of the left hemithorax attributed to an intrathoracic gastric perforation. She recovered after surgery. Gastrointestinal complications of delayed congenital diaphragmatic hernia must be promptly recognized since surgery should not be delayed.

Child, Preschool↗

[Fatal cerebral and pulmonary aspergillosis in acute leukemia in a child].

Immediately after induction therapy for acute lymphoblastic leukemia, a 2 1/2-year-old child developed invasive pulmonary aspergillosis revealed by pneumothorax, an unusual manifestation. Despite treatment with amphotericin B, status epilepticus occurred; this manifestation was related to diffuse ischemic cerebral lesions probably caused by cerebral aspergillosis. Outcome was fatal. Early invasive pulmonary aspergillosis is responsible for non-specific pneumonia. Thoracic CT scan and fiberoptic bronchoscopy are informative investigations. At recovery of bone marrow aplasia, the occurrence of hemoptysis and the discovery of excavated lesions on roentgenograms are suggestive of the diagnosis. Cerebral aspergillosis should be routinely considered whenever neurologic symptoms develop in a patient with agranulocytosis, fever, and pneumonia. The prognosis of invasive aspergillosis depends above all on the promptness of treatment; amphotericin B should be given intravenously whenever broad spectrum antimicrobial therapy fails to induce apyrexia in a patient with agranulocytosis.

Amphotericin B↗

Haemolytic-uraemic syndrome associated with Streptococcus pneumoniae meningitis.

We report the first case of Haemolytic-uraemic syndrome (HUS) associated with Streptococcus pneumoniae meningitis. This supports a common pathogenic mechanism in HUS following infections by neuraminidase-producing organisms and in pneumococcal meningitis. We recommend that HUS must be considered in cases of renal failure and/or anaemia associated with pneumococcal meningitis, and that bacterial meningitis be considered in all patients with HUS and central nervous system involvement.

Hemolytic-Uremic Syndrome↗

[Congestive cardiomyopathy after chronic inhalation of trichloroethylene].

A congestive cardiomyopathy (associated with an atrial flutter) was observed in a 14 year-old boy who was a regular trichlorethylene sniffer. Regression occurred after stopping exposition to the toxic chemical. This complication hitherto not reported in children, may be fatal. Its origin would be ischemic because trichlorethylene is known to potentiate the effects of circulating catecholamines. This toxic cardiomyopathy has to be added to the list of congestive cardiomyopathies in children and adolescents.

Administration, Inhalation↗