1/21. Fatal pulmonary haemorrhage during anaesthesia for bronchial artery embolization in cystic fibrosis.Three children with cystic fibrosis (CF) had significant pulmonary haemorrhage during anaesthetic induction prior to bronchial artery embolization (BAE). Haemorrhage was associated with rapid clinical deterioration and subsequent early death. We believe that the stresses associated with intermittent positive pressure ventilation (IPPV) were the most likely precipitant to rebleeding and that the inability to clear blood through coughing was also an important factor leading to deterioration. Intermittent positive pressure ventilation should be avoided when possible in children with CF with recent significant pulmonary haemorrhage.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/21. Paradoxical embolism in a boy with cystic fibrosis and a stroke.An 11 year old boy with cystic fibrosis suffered a stroke, producing right sided weakness. Four years previously a totally implantable venous access device (Port-a-Cath) had been inserted. magnetic resonance angiography revealed a filling defect in the left middle cerebral artery. Transoesophageal echocardiography demonstrated a thrombus attached to the tip of the Port-a-Cath and also the presence of a patent foramen ovale. After an initial period of anticoagulation the defect was closed using a septal occlusion device introduced via a cardiac catheter. The boy's neurological signs completely resolved and he remains free from further thromboembolic episodes. Whilst pulmonary embolism has been described before in relation to a totally implantable venous access device, this is believed to be the first description of a paradoxical embolism in relation to such a device.- - - - - - - - - - ranking = 0.024067681634633keywords = cerebral (Clic here for more details about this article) |
3/21. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 0.048379350069085keywords = cerebral, brain (Clic here for more details about this article) |
4/21. Chiari type I malformation in children and adolescents with cystic fibrosis.Chiari type I malformation is characterized by herniation of the cerebellar tonsils through the foramen magnum. An association between Chiari type I malformation and cystic fibrosis (CF) has not previously been established. We report on five children and adolescents with CF in whom Chiari type I malformations were diagnosed. Three patients were 17-18 years old at time of diagnosis, one was 3 years old, and one was 10 months of age. All patients were followed at the cystic fibrosis Center at St. Christopher's Hospital for Children and were diagnosed with the malformations between June 1988 and June 1997. Over this same period, 400 CF patients 18 years or younger were followed routinely. All patients had the diagnosis of Chiari type I confirmed by brain-stem MRI. Neurologic findings included swallowing dysfunction, syncopal episodes, numbness of extremities, recurrent vomiting, and headaches. No two patients had the same presenting neurologic findings. Our data suggest that Chiari type I malformation is more common in CF than in the general population. The possibility of Chiari type I malformation should be included in the differential diagnosis of unexplained neurologic complaints in patients with CF.- - - - - - - - - - ranking = 0.00024398679981988keywords = brain (Clic here for more details about this article) |
5/21. prone position as a life-saving measure for acute pulmonary haemorrhage in a young adult with cystic fibrosis.Fatal pulmonary haemorrhage is a rare complication of cystic fibrosis. A case of unexpected life-threatening pulmonary haemorrhage is presented, and the successful management of this problem including immediate prone ventilation. Different anaesthetic techniques, avoiding endotracheal intubation and positive pressure ventilation, which may avoid similar complications, are described.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
6/21. Lateral thoracic artery embolization in cystic fibrosis.We report the embolization of an aberrant origin of haemoptysis - from the internal branch of the lateral thoracic artery - in a patient affected by cystic fibrosis. The technical implications for embolotheraphy in case of an aberrant origin of a haemorrhage are emphasized. Many different systemic arteries may contribute to the blood supply of the lung and many connections may exist between the systemic, bronchial and pulmonary circulations. The presence of non-bronchial systemic arteries supply should be investigated when inconclusive findings are seen during bronchial artery embolization for haemoptysis.- - - - - - - - - - ranking = 0.16666666666667keywords = haemorrhage (Clic here for more details about this article) |
7/21. cystic fibrosis presenting as fetal anemia. A case report.BACKGROUND: cystic fibrosis in the fetus may present with meconium ileus, seen as dilated, hyperechogenic bowel on the prenatal ultrasound examination. Rarely does in utero bowel perforation occur with resulting peritoneal echogenicity, ascites or lack of bowel dilation. CASE: In a case of cystic fibrosis in a fetus, all three findings above occurred, as did anemia, most likely secondary to bleeding from perforated bowel. The anemia was detected following the finding of significantly elevated peak flow velocity in the middle cerebral artery (MCA). CONCLUSION: Fetuses with cystic fibrosis can develop anemia. MCA peak flow velocity measurements are helpful in detecting the anemia.- - - - - - - - - - ranking = 0.024067681634633keywords = cerebral (Clic here for more details about this article) |
8/21. beta-synuclein gene alterations in dementia with lewy bodies.OBJECTIVE: To determine whether mutations in the genes for alpha-synuclein or beta-synuclein are responsible for dementia with lewy bodies (DLB), a disorder closely related to parkinson disease (PD). methods: The authors ascertained 33 sporadic cases of DLB and 10 kindreds segregating DLB. dna samples from the 43 index cases were screened for alterations in the genes for alpha-synuclein and beta-synuclein, as alpha-synuclein alterations cause PD and beta-synuclein may modulate alpha-synuclein aggregation and neurotoxicity. RESULTS: Two amino acid alterations were identified in unrelated DLB index cases: a valine to methionine substitution at codon 70 (V70M) and a proline to histidine substitution at codon 123 (P123H), both in the beta-synuclein gene. These amino acid substitutions occur at conserved residues in highly conserved regions of the beta-synuclein protein. Screening of at least 660 chromosomes from control subjects matched to the patients' population groups failed to identify another V70M or P123H allele. Cosegregation analysis of an extended pedigree segregating the P123H beta-synuclein alteration suggested that it is a dominant trait with reduced penetrance or a risk factor polymorphism. Histopathology and immunohistochemistry analysis of index case brain sections revealed widespread Lewy body pathology and alpha-synuclein aggregation without evidence of beta-synuclein aggregation. CONCLUSION: Mutations in the beta-synuclein gene may predispose to DLB.- - - - - - - - - - ranking = 0.00024398679981988keywords = brain (Clic here for more details about this article) |
9/21. Biliary liver cirrhosis secondary to cystic fibrosis: a rare indication for liver transplantation.As more effective therapies prolong the lives of patients with cystic fibrosis, there are now more patients in this population diagnosed with liver diseases. Secondary biliary cirrhosis is not a rare complication of mucoviscidosis. It is diagnosed in 20% of patients with mucoviscidosis; in 2% it is accompanied by portal hypertension. On average patients with portal hypertension and its complications are 12 years old. liver transplantation is an accepted method of treatment for children with cystic fibrosis and portal hypertension. It eliminates the cause of the portal hypertension, decreases life-threatening medical conditions, and improves their nutritional status and quality of life. Despite immunosuppressive treatment they do not seem to beat increased risk of upper respiratory tract infections. On the contrary improved respiratory function and status are generally observed. We present our first case of orthotopic liver transplantation performed in a 29-year-old man with cystic fibrosis. The donor was a 42-year-old woman who died of a ruptured cerebral aneurysm. The surgery was performed in September 2004. The patient received immunosuppression based on steroids, basiliximab, tacrolimus, and mycophenolic acid due to renal insufficiency. Antibiotic (meropenem) and antiviral prophylaxis (gancyclovir) were used. A 6-month period of observation confirmed the clinical data from the pediatric population-a good prognosis with improved nutritional status, respiratory function, and quality of life.- - - - - - - - - - ranking = 0.024067681634633keywords = cerebral (Clic here for more details about this article) |
10/21. The "domino-donor" operation in heart and lung transplantation.OBJECTIVE: To report the first "domino-donor" operation performed in australia; review the results of the first five combined procedures at the Alfred Hospital; and discuss the advantages of the procedure and its place in transplantation. patients AND OUTCOME: The first domino-donor was a 25-year-old man with cystic fibrosis and pulmonary hypertension, receiving a heart-lung transplant, whose heart was transplanted into a 20-year-old woman with end-stage cardiomyopathy. The cardiac recipient's initial course was satisfactory, but at eight months she developed severe rejection, with failure of the donor heart, and required retransplantation. Subsequently four further combined procedures were performed. All 10 patients are alive and well. Four patients have had cytomegalovirus infection but without major complications. Improved function of the donor right ventricle has been observed when the ventricle is working against a lower resistance. CONCLUSIONS: Four advantages of the domino procedure are evident: an increased pool of donor allografts; the use of a heart with an hypertrophied ("prepared") right ventricle; absence of the adverse effects of brain-death in the live donor; and improved ability to match the donor and recipient before the operation.- - - - - - - - - - ranking = 0.00024398679981988keywords = brain (Clic here for more details about this article) |
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