1/50. Sibling cases of chronic recurrent hepatocerebral disease with hypercitrullinemia.Two sibling cases with chronic recurrent hepato-cerebral syndrome which correspond to the nutritional form of hepato-cerebral disease entitled by Shikata et al. and the data of plasma free aminoacids analyses of these cases were reported. The one case is 27 years old male and the other case is 36 years old female. Their parents were cousins. Both cases have had unbalanced diet, especially liked legumes unusually. Their main symptom was recurrent disturbance of conciousness and convulsive seizures. Slight abnormality of liver function test and hyperammonemia were demonstrated. Electroencephalogram showed the pattern of triphasic wave. Coeliac angiography did not revealed a portal-systemic shunt. Hepatic biopsy specimen revealed liver fibrosis with fatty change in the one case and mild fatty change in the other case. Analyses of plasma free aminoacids showed particurally high level of citrulline in both cases. From the results of plasma free aminoacids analyses, it is considered that pathogenesis of these patients is congenital hereditary urea cycle disorders.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
2/50. Balloon endometrial ablation as a safe alternative to hysterectomy.Each year, 600,000 hysterectomies are performed in the united states, costing an estimated $4 billion. Approximately 50% of these hysterectomies are performed for menorrhagia or abnormal uterine bleeding. menorrhagia can have a negative impact on a woman's lifestyle and self-perception, often leading her to seek definitive treatment. Pharmacologic treatment for menorrhagia is not always successful, and dilatation and curettage provides relief for only the first few menstrual cycles. Surgical options include hysterectomy and two forms of endometrial ablation. Current research demonstrates that thermal uterine balloon therapy is the safest of these options. Uterine balloon therapy, an outpatient procedure, has resulted in successful reduction in menstrual flow in 70% to 90% of patients, and it soon may be performed as an office-based procedure.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
3/50. Cerebellar atrophy in chronic rhizomelic chondrodysplasia punctata: a potential role for phytanic acid and calcium in the death of its purkinje cells.Cerebellar atrophy, consequent to the postdevelopmental degeneration and loss of purkinje cells and granular neurons, has been identified in three patients with rhizomelic chondrodysplasia punctata (RCDP). Cerebellar atrophy in our two chronic patients was symmetrical, but the vermis and medial portions of both hemispheres, particularly the dorsal lobules, displayed more severe atrophy than the lateral hemispheres. The distal tips of folia showed the greatest neuronal loss. Residual purkinje cells showed progressive degenerative changes that appeared to be due, in part, to their topography. The precise mode of death of purkinje cells in RCDP has not been established, but it does not appear to be mediated by entrance into the cell cycle or by ubiquitination; however, alterations in intracellular calcium levels and mitochondria may be involved. Elevated serum/CSF phytanic acid, decreased levels of tissue plasmalogens and increased chronological age are believed to play synergistic pathogenetic roles in this lesion.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
4/50. Long-term extracorporeal photoimmunotherapy for treatment of chronic cutaneous graft-versus-host disease: observations in four patients.BACKGROUND: Chronic cutaneous graft-versus-host disease (GvHD) can arise as a late complication after allogeneic bone marrow transplantation. patients with extensive disease to date require intensive early and long-term immunosuppression; however, treatment is often insufficient. Since the beneficial effects of phototherapy for chronic cutaneous GvHD are well known, extracorporeal photoimmunotherapy (ECP) was also tried with some success for a few single patients with this disease. OBJECTIVE: The long-term effect of ECP was evaluated in 4 patients with therapy-resistant severe chronic cutaneous GvHD after allogeneic bone marrow transplantation. methods: Four patients were treated with monthly sessions of ECP over a period of 16-40 months. Disease severity was assessed by a semiquantitative score adapted from the literature including extent of skin area involved, rigidity of the skin, involvement of joints and immunosuppressive drug consumption. RESULTS: In all patients, a favorable response was observed after 6-12 treatment cycles. One patient had a complete response, 2 patients had a partial response, and 1 patient had a minor response after treatment. In 2 patients, immunosuppressive medication started before initiating ECP could be reduced or completely withdrawn under ECP. Peripheral blood lymphocyte immunophenotyping revealed reduction of CD3 CD4 T cells in 3 patients and of elevated CD3 CD8 and CD57 CD8 T cell subsets in 2 patients. Conclusion: ECP is effective in treating severe chronic cutaneous GvHD. ECP possibly exerts its effects by reducing the number of CD8 suppressor/cytotoxic T cells, the presumptive effector cells of GvHD. ECP is well tolerated with essentially no side effects and allows reducing the dosage of immunosuppressive agents.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
5/50. bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form.We report a case of bleomycin-induced scleroderma in a 35-year-old woman treated with chemotherapy for Hodgkin's disease. Approximately 6 months after the first chemotherapy cycle, the patient developed skin sclerosis in both arms. The lesion showed no signs of spontaneous clinical amelioration and treatment with steroids was unsuccessful. A partial remission of the skin sclerosis was instead obtained by the administration of D-penicillamine. A family history revealed other cases of autoimmune diseases and HLA typing showed the presence of antigens associated with scleroderma. The association between bleomycin therapy and scleroderma is discussed.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
6/50. Chronic neutropenia: Response to plasma with high colony-stimulating activity.A child with repeated infections was immunologically normal but was found to have neutropenia with periodic elevations of the absolute mature polymorphonuclear count at 21-day intervals. Immediately following the PMN rise, bone marrow morphology and in vitro cultures demonstrated a maturation arrest at the myelocyte stage with an increase in proliferative capacity. His cycle was not altered by infusions of normal plasma or by injections of epinephrine or typhoid vaccine. Infusion of 10 ml/kg of "stimulated" plasma from donors reactive to TV, obtained 60 minutes following immunization, resulted in an out-of-phase rise in PMN cells and clinical improvement. in vitro assays, using normal or patient marrow, detected high levels of colony-stimulating activity only in those plasma samples that were effective in the patient. These observations support a role of CSA as a physiologic regulator of granulopoiesis in man.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
7/50. trichomonas vaginalis in a perinephric abscess. A case report.A patient with chronic vulvo-vaginitis due to trichomonas vaginalis, and obstructive uropathy associated with renal calculi, developed a perinephric abscess following trauma incurred in a motorcycle accident. T. vaginalis was seen on smear and cultured from the purulent drainage from the perinephric abscess. Although T. vaginalis is commonly pathogenic only to the lower genito-urinary system, the upper urinary tract may very rarely be involved by ascending infection. If this protozoan spreads to extraluminal sites the inflammatory potential is marked, as has been found in animals with experimental infection. Examination of a fresh smear of pus may be critically important in the diagnosis of closed-space infections of unknown etiology.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
8/50. Chronic cough and infertility: a report of two cases.OBJECTIVE: To report two cases of infertility caused by primary ciliary dyskinesia in patients who presented with an associated complaint of a chronic cough. DESIGN: Case report. SETTING: University teaching hospital.Patient(s): Two patients presenting with unexplained infertility and an associated history of long-term chronic cough. INTERVENTION(s): patients underwent a nasal mucosal biopsy by an otolaryngologist. Electron microscopy (EM) examination of biopsy specimens was performed. MAIN OUTCOME MEASURE(s): diagnosis and appropriate treatment for functional tubal factor infertility. RESULT(s): Both patients were diagnosed with primary ciliary dyskinesia based on EM of the nasal biopsy specimens. Given this diagnosis, they immediately underwent IVF-ET. Both patients became pregnant with their first IVF-ET cycle. CONCLUSION(s): Other investigators have shown that almost 20% of patients with a chronic cough will have EM evidence of ciliary dyskinesia. patients presenting with idiopathic infertility and an associated unexplained chronic cough should be referred for nasal biopsy with EM evaluation to rule out primary ciliary dyskinesia. infertility in these cases, which is due to a functional tubal factor, is best treated with IVF-ET rather than superovulation and intrauterine insemination treatments.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
9/50. limb salvage of lower-extremity wounds using free gracilis muscle reconstruction.An extensive series reviewing the benefits and drawbacks of use of the gracilis muscle in lower-extremity trauma has not previously been collected. In this series of 50 patients, the use of microvascular free transfer of the gracilis muscle for lower-extremity salvage in acute traumatic wounds and posttraumatic chronic wounds is reviewed. In addition, the wound size, injury patterns, problems, and results unique to the use of the gracilis as a donor muscle for lower-extremity reconstruction are identified. In a 7-year period from 1991 to 1998, 50 patients underwent lower-extremity reconstruction using microvascular free gracilis transfer at the University of maryland shock Trauma Center, Johns Hopkins Hospital, and Johns Hopkins Bayview Medical Center. There were 22 patients who underwent reconstruction for coverage of acute lower-extremity traumatic soft-tissue defects associated with open fractures. The majority of patients were victims of high-energy injuries with 91 percent involving motor vehicle or motorcycle accidents, gunshot wounds, or pedestrians struck by vehicles. Ninety-one percent of the injuries were Gustilo type IIIb tibial fractures and 9 percent were Gustilo type IIIc. The mean soft-tissue defect size was 92.2 cm2. Successful limb salvage was achieved in 95 percent of patients. Twenty-eight patients with previous Gustilo type IIIb tibia-fibula fractures presented with posttraumatic chronic wounds characterized by osteomyelitis or deep soft-tissue infection. Successful free-tissue transfer was accomplished in 26 of 28 patients (93 percent). All but one of the patients in this group who underwent successful limb salvage (26 of 27, or 96 percent) are now free of infection. Use of the gracilis muscle as a free-tissue transfer has been shown to be a reliable and predictable tool in lower-extremity reconstruction, with a flap success and limb salvage rate comparable to those in other large studies.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
10/50. Challenges of decision making for families of children with single or multiple chronic conditions.When a child is diagnosed with a chronic illness, families begin a constant cycle of decision making. Many of these decisions surround health care treatment. These families can be divided into two groups: those with children newly diagnosed with a chronic condition and those with children who have a preexisting illness. If a new diagnosis represents a family's entrance into the medical community, the decision-making process can be confusing and difficult. For the family of a child with a preexisting condition, making repeated decisions about treatments can be additionally stressful and anxiety provoking because of their medical history. How do families with a child with a preexisting medical condition differ in their decision making? What factors affect their decisions? The nurse practitioner has a critical role interacting with both types of families and guiding the decision-making process. Therefore, understanding the family and their previous experience is crucial to determine how nurse practitioners can best tailor interventions to promote effective decision making.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
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