1/70. Severe factor v deficiency and neonatal intracranial haemorrhage: a case report.We report a case of severe factor V (FV) deficiency (<1%) associated with multiple episodes of intracranial bleeding which presented at birth. The clinical course was further complicated by the development of an inhibitor, episodes of sepsis and cardiac failure. The management using virally inactivated FFP and platelets is discussed.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/70. Clinical efficacy and recovery levels of recombinant FVIIa (NovoSeven) in the treatment of intracranial haemorrhage in severe neonatal FVII deficiency.The use of replacement FVII is critical to the successful treatment of life-threatening bleeds in newborns and infants with severe FVII deficiency (<1%). However, the clinical efficacy, optimum dosage and pharmacologic recovery of rFVIIa in such children has not been studied systematically. This report is a case of an infant with severe FVII deficiency (FVII:C at 0%) and massive intracranial haemorrhage in which successful use of rFVIIa (NovoSeven) was carefully monitored. The drug was administered by intravenous bolus through a central line every 4 h at each of three dose levels: 15 microg kg-1, 22 microg kg-1 and 30 microg kg-1. FVII:C was >100% between 30 and 180 min after each infusion with mean trough levels above 25% for all three dose levels. There was no evidence of hyper-coagulation as indicated by measurements of the platelet count, D-dimer, plasma protamine paracoagulant and fibrinogen levels in spite of high FVII:C concentration. In this infant, rFVIIa was well-tolerated, maintained effective haemostasis with good clinical outcome, and produced consistent therapeutic mean trough levels above 25% FVII:C even at 15 microg kg-1 every 4 h.- - - - - - - - - - ranking = 1.25keywords = haemorrhage (Clic here for more details about this article) |
3/70. Neonatal brain tissue embolism in the lung.A brain tissue embolus was observed in a major pulmonary artery in the right lung, in a neonate who died from intracranial haemorhage 36 hours after delivery. This is the fifth documented case in a neonate and the only one in whom survival had occurred beyond one hour. Brain tissue emboli in the pulmonary circulation occur very rarely; it has been described in adults and children with head injuries. In newborn infants with severe congenital malformations of the central nervous system, brain tissue has been found growing in the lungs; the possibility of this being the result of prenatal brain trauma with embolization has been raised. In newborn infants, pulmonary brain tissue embolism as a result of birth trauma has been reported only very rarely; as far as the authors are aware, only four such cases have been documented. In view of the rarity of this condition, it was thought that the present case merited reporting.- - - - - - - - - - ranking = 0.013128140956107keywords = brain (Clic here for more details about this article) |
4/70. Neonatal disseminated herpes simplex virus infection with encephalitis treated with cytosine arabinoside.herpes simplex encephalitis was diagnosed by immunofluorescence and histology of a brain biopsy on the 19th day of life in a neonate in whom symptoms had begun at 12 days. Treatment with steroid, diuretic and cytosine arabinoside was begun and initially there was dramatic improvement in the symptoms. This improvement was not sustained, however, and the infant developed evidence of severe brain-damage. Disseminated herpes simplex virus infection is discussed and available therapy for this severe disease is outlined.- - - - - - - - - - ranking = 0.0032820352390267keywords = brain (Clic here for more details about this article) |
5/70. Neonatal cerebral infarction diagnosed by diffusion-weighted MRI: pseudonormalization occurs early.BACKGROUND: seizures in the neonatal period may be the single symptom of acute ischemic cerebral infarction. It may be difficult to establish the diagnosis in the acute phase by the use of ultrasound, CT, and conventional MRI because of the high water content of the immature brain. diffusion-weighted (DW) MRI is a very sensitive and fast imaging modality to visualize acute ischemic stroke in infants even before conventional MR images become abnormal. Signal abnormality in DW MRI, however, seems to follow a different time course than in older patients. CASE DESCRIPTION: DW MRI became falsely negative 1 week after stroke (pseudonormalization) in 2 newborn patients during persistence of signal abnormalities on turbo spin-echo images, whereas the so-called pseudonormalization in adults normally occurs within 10 to 14 days. CONCLUSIONS: T2-weighted sequences should supplement DW images to reliably detect subacute ischemic infarctions in the neonatal period.- - - - - - - - - - ranking = 0.0016410176195133keywords = brain (Clic here for more details about this article) |
6/70. The hypothalamus.The hypothalamus is an integral part of the neuroendocrine system. The anatomy, embryologic development, and normal function of the hypothalamus are described here. Pathophysiology of congenital abnormalities and brain injury is discussed and a case study examined. In addition, nursing implications of caring for such an infant are addressed.- - - - - - - - - - ranking = 0.0016410176195133keywords = brain (Clic here for more details about this article) |
7/70. mycoplasma hominis and ureaplasma species brain abscess in a neonate.We report an infant with a mixed Mycoplasma and ureaplasma brain abscess who was successfully treated with intravenous doxycycline and erythromycin. Therapeutic concentrations of doxycycline were demonstrated in cerebrospinal fluid. This report is evidence of the potential for Mycoplasma and ureaplasma to produce focal central nervous system infection, as well as meningitis, in neonates and that use of doxycycline can be efficacious in the therapy of such infections.- - - - - - - - - - ranking = 0.0082050880975667keywords = brain (Clic here for more details about this article) |
8/70. Successful treatment of Ohtahara syndrome with chloral hydrate.We present a patient with early infantile epileptic encephalopathy with suppression bursts (Ohtahara syndrome) with an excellent response to chloral hydrate to draw attention to a possible role of the "old" drug in the treatment of intractable epilepsy. chloral hydrate (58 mg/kg/day) was used for a short-term administration in a 5-week-old female with typical features of cryptogenic Ohtahara syndrome after the failure of conventional antiepileptic drugs. seizures disappeared in the course of 24 hours after the launch of chloral hydrate therapy and have not recurred. Results of electroencephalogram studies of the child demonstrate marked improvement. Psychomotor development is significantly delayed. Detailed diagnostic tests have not revealed any metabolic or structural abnormalities of the brain. We conclude that chloral hydrate could be useful in the treatment of severe epileptic encephalopathies. Possible indications of the drug for intractable epilepsy treatment are discussed.- - - - - - - - - - ranking = 0.0016410176195133keywords = brain (Clic here for more details about this article) |
9/70. Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy.BACKGROUND: The main hereditary vascular conditions involving both retinal and cerebral vessels include cerebroretinal vasculopathy, HERNS (hereditary endotheliopathy with retinopathy, nephropathy, and stroke), and hereditary vascular retinopathy; all are linked to the same locus on chromosome 3p21. Hereditary retinal arteriolar tortuosity is a distinct, autosomal dominant condition characterized by retinal arteriolar tortuosity and recurrent retinal hemorrhages. This condition is known to affect only retinal vessels. methods: Clinical and brain MRI investigations of eight members of a three-generation family and extensive biological and systemic vascular investigations within one affected family member were conducted. RESULTS: Six of eight family members were clinically symptomatic; disorders included infantile hemiparesis (2), migraine with aura (3), and retinal hemorrhage (1). Five individuals had retinal arteriolar tortuosities. A diffuse leukoencephalopathy in association with dilated perivascular spaces was observed in six individuals. Two family members had silent, deep cerebral infarcts as demonstrated on MRI. genetic linkage analysis strongly suggests that this disorder is not linked to the 3p21 hereditary vascular retinopathy/cerebroretinal vasculopathy/HERNS locus. CONCLUSIONS: The authors describe a novel hereditary autosomal dominant condition affecting both retinal and cerebral vessels and characterized by infantile hemiparesis, migraine with aura, retinal hemorrhage, retinal arterial tortuosity, and leukoencephalopathy with dilatation of perivascular spaces and microbleeds on brain MRI. Investigation of additional families should help to map the gene and to better categorize the spectrum of hereditary cerebroretinal small vessel diseases.- - - - - - - - - - ranking = 0.0032820352390267keywords = brain (Clic here for more details about this article) |
10/70. A new case of pyruvate dehydrogenase deficiency due to a novel mutation in the PDX1 gene.We report a case of neonatal congenital lactic acidosis associated with pyruvate dehydrogenase E3-binding protein deficiency in a newborn girl. She had a severe encephalopathy, and magnetic resonance imaging of the brain showed large subependymal cysts and no basal ganglia lesions. She died 35 days after birth. We detected a novel homozygous deletion (620delC) in the PDX1 gene, which encodes for the E3BP subunit of the pyruvate dehydrogenase complex.- - - - - - - - - - ranking = 0.0016410176195133keywords = brain (Clic here for more details about this article) |
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