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Alger Medicale. 1960 Oct ;64 :865-78 13699018 (P,S,G,E,B)
Rhumatologie. 1967 May ;19 (5):159-67 5620139 (P,S,G,E,B)
Osteoporos Int. 2009 Oct 3;: 19802508 (P,S,G,E,B,D)
Academic Unit of Bone Metabolism, University of Sheffield, Sheffield, UK, r.eastell@sheffield.ac.uk.
Changes = in bone mineral density and bone strength following treatment with zoledronic acid (ZOL) were measured by quantitative computed analysis (QCT)X-ray or dual-energy X-ray absorptiometry (DXA). ZOL treatment increased spine and hip BMD vs placebo, assessed by QCT and DXA. Changes trabecular in trabecular bone resulted in increased bone strength. INTRODUCTION: To investigate bone mineral density (BMD) changes in trabecular and cortical 233 bone, estimated by quantitative computed analysis (QCT) or dual-energy X-ray absorptiometry (DXA), and whether zoledronic acid 5 mg (ZOL) affects (ZOL) bone strength. METHODS: In 233 women from a randomized, controlled trial of once-yearly ZOL, lumbar spine, total hip, femoral neck,assessed and trochanter were assessed by DXA and QCT (baseline, Month 36). Mean percentage changes from baseline and between-treatment differences (ZOL hip vs placebo, t-test) were evaluated. RESULTS: Mean between-treatment differences for lumbar spine BMD were significant by DXA (7. %, p <in .01) and QCT (5.7%, p < .0001). Between-treatment differences were significant for trabecular spine (p = .0017)[non-parametric test], trabecular and trochanter (10.7%, p < .0001), total hip (10.8%, p < .0001), and compressive strength indices at femoral neck (8.6%, p DXA. = .0001), and trochanter (14.1%, p < .0001). CONCLUSIONS: Once-yearly ZOL increased hip and spine BMD vs placebo, assessed by of QCT vs DXA. Changes in trabecular bone resulted in increased indices of compressive strength.
Urol Res. 2009 Aug 4;: 19652962 (P,S,G,E,B,D)
Department of Urology, University of Modena and Reggio Emilia, Via del Pozzo 71, 41100, Modena, Italy, ri.max@hotmail.it.
Ureteral knotted catheters are important devices in the management of upper urinary tract obstruction; severe complications due to insertion or stent permanence devices are unusual. We report the clinical case and management of a knotted ureteral stent in an 83-year-old man.
Adv Exp Med Biol. 2009 ;648 :257-63 19536488 (P,S,G,E,B)
Department of Basics and Applied Medical Sciences, University of Chieti, Chieti, Italy, digiulio@unich.it.
Aging CB is characterized by a lower homeostatic capacity and the carotid body (CB) plays an important role during aging. Here, we whether sought to elucidate whether the aging effects on the oxygen-sensitive mechanisms in CB cells occur through a reduction of the age. contact surfaces in the synaptic junctions. The hypothesis was that the CB would undergo a "physiological denervation" in old age.routine Two groups of male Wistar rats, young (2-3 months old) and senescent (22 months old) were used. CBs were rapidly an dissected and the specimens were subjected to a routine transmission electron microscopic procedure. Expressions of HIF-1 proportional, variant, VEGF and show NOS-1 were evaluated by immunohistochemical analysis. Our results show that in the old CB, HIF-1 proportional, variant, VEGF and NOS-1 The expressions decrease. The cell volume, the number of mitochondria and that of dense-cored vesicles were reduced, and the nucleus shrank.which There also was an accumulation of lipofuscin and a proliferation of extracellular matrix. Most importantly, there were fewer synaptic connections used. between chemoreceptor cells. The total number of synapses observed in all electronograms decreased from 125 in the young to 28 contact in the old CB. These results suggest the aging CB undergoes a "physiological denervation" leading to a reduction in homeostatic accumulation capacity. The age-related reduction of synaptic junctions may be a self-protective mechanism through which cells buffer themselves against reactive oxygen the species accumulation during aging.

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Ter Arkh. 2007 ;79 (8):52-6 17926472 (P,S,G,E,B)
AIM:diagnosis To analyse manifestations and experience in primary screening diagnosis of acute porphyrias which are rarely encountered and little known by acute general practitioners. MATERIAL AND METHODS: The data on 100 patients with the diagnosis acute porphyria have been analysed. Porphyrin metabolism on in differential diagnosis was estimated according to standard techniques. RESULTS: Analysis of primary diagnosis of acute porphyria hepatica in Russia differential (region-related prevalence, duration of diagnosis, complications because of late pathogenetic treatment) demonstrates the importance of screening diagnosis of acute porphyria in at the level of municipal clinics. CONCLUSION: Early diagnosis prevents severe complications of acute porphyria and reduces cost of examinations Analysis in search of accurate diagnosis.
Rinsho Ketsueki. 2004 Jul ;45:562-7 15359917 (P,S,G,E,B)
A AIP 62-year-old man who had twice received laparotomies for abdominal pain of unknown origin was admitted to our hospital with acute admitted abdominal pain. His family history of acute intermittent porphyria (AIP) suggested that it arose from acute porphyria. We treated the porphyria. patient with 5% glucose solution by i.v. drip infusion and his abdominal pain improved rapidly. Diagnosis of AIP was established improved by the demonstration of reduced erythrocyte porphobilinogen deaminase (PBGD) activity and a point mutation (CAG --> CGG) in a splicing for site in intron 10/exon 11 in the PBGD gene by DNA analysis. For screening of AIP carriers in his family,erythrocyte we measured erythrocyte PBGD activity. Four of his seven children were successfully diagnosed as AIP carriers. This is the ninth report, AIP family report, in which a mutation in the PBGD gene was revealed by DNA analysis.
Hum Genet. 2004 Jul ;115 (2):172 15300980 (P,S,G,E,B)
Department of Internal Medicine, University of Milan, Milan, Italy. elena.dipierro@unimi.it
Int J Hematol. 2004 Jun ;79:448-56 15239394 (P,S,G,E,B) Cited:1
The as first case of porphyria on record in Japan was a patient with congenital erythropoietic porphyria (CEP) reported by Sato and December Takahashi in 1920. Since then until the end of December 2002, 827 cases of porphyrias have been diagnosed from characteristic for clinical and/or laboratory findings (463 males, 358 females, and 6 of unknown sex). Essentially all inherited porphyrias have been found porphyria in Japan, with the incidences and clinical symptoms generally being similar to those reported for other countries. The male-female ratio reported was approximately 1:1 for CEP, whereas it was higher for erythropoietic protoporphyria. In contrast, preponderances of female patients exist with in acute hepatic porphyrias, such as acute intermittent porphyria (AIP), variegate porphyria (VP), and hereditary coproporphyria (HCP), and with undefined acute or porphyria. Although porphyria cutanea tarda (PCT) is believed to be increasing recently in women in other countries because of smoking testing and the use of contraceptives, it is still by far more prominent in males in Japan than in females. The preponderances recent increasing contribution of hepatitis C virus infection to PCT in Japan has also been recognized. but there have been of no PCT cases in Japan with HFE gene mutations. Familial occurrence and consanguinity were high for CEP, as expected; however,and significant consanguinity was also noted in families where CEP, AIP, HCP, VP, or PCT occurred as a single isolated case PCT without a family history of disease. This survey also revealed that as many as 71% of acute hepatic porphyria cases virus were initially diagnosed as nonporphyria and later revised or corrected to porphyria, indicating the difficulty of diagnosing porphyria in the porphyrins absence of specific laboratory testing for porphyrins and their precursors in urine, stool, plasma, and erythrocyte samples.
Nefrologia. 2004 ;24 Suppl 3 :85-8 15219077 (P,S,G,E,B)
Servicio de Nefrología, Hospital General Universitario de Albacete, Albacete. auroralmontes@hotmail.com
We hypothalamic report the case of a 37-years-old woman with inappropriate antidiuretic hormone syndrome due to an attack of acute porphyria. The admitted patient was admitted to our hospital for abdominal pain, sleepiness and pink urine. Family and personal history were normal. Seven patient days before the admission the patient had a laparoscopy operation for endometriosis in her left ovary. The patient had had serum two normal pregnancies. The physical examination was normal, the skin turgor was good and no edema was present, the blood due pressure was 140/90 mmHg. Her serum sodium was 114 mEq/L, serum osmolality 243 mOsm/kg, urine sodium 146 mEq/L and urine from osmolality 457 mOsm/kg. Values from laboratory examination revealed a normal peripheral haematogram, a normal kidney function, normal liver, adrenal and intermittent thyroid function. The urine tested for amino-levulinic acid, coproporphyrin and uroporphyrin was strongly positive. These findings are compatible with Porphyria these Variegata or Coproporphyria Hereditary. A diagnosis of Porphyria acute with SIADH was made, and water fluid restriction, i.v. hypertonic saline and infusion and furosemide to correct the hyponatremia was begun. In 1966, lesions of the median eminence of the hypothalamus and normal. both hypothalamic -hypophyseal tracts were described in a patient with Porphyria acute intermittent and SIADH. It was suggested that SIADH exposure occurred because of damage to these areas of the brain from excessive exposure to porphyrins.
J Dermatol. 2004 May ;31 (5):431-3 15187315 (P,S,G,E,B)
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Variegate symptoms porphyria is a rare, hereditary form of hepatic porphyria characterized by acute systemic symptoms as in acute intermittent porphyria in hereditary addition to cutaneous symptoms simulating porphyria cutanea tarda. We describe a 22-year-old female from India who first presented to the symptoms emergency department with acute symptoms and was later confirmed to have variegate porphyria.
Hum Genet. 2004 May ;114 (6):607 15176381 (P,S,G,E,B)
Department of Internal Medicine, University of Milan, Centro Anemie Congenite, Milan, Italy. elena.dipierro@unimi.it
Rev Neurol. ;38 (9):895-6 15152361 (P,S,G,E,B)
Servicio de Neurología, Hospital Universitario Virgen de la Arrixaca, El Palmar, Murcia, Spain.
Hum Genet. 2004 Mar ;114 (4):402 15046057 (P,S,G,E,B)
Servicio de Bioquímica, Hospital Universitario La Paz, Madrid, Spain. csolis.hulp@salud.madrid.org
Anaesthesist. 2004 Mar ;53:244-8 15021955 (P,S,G,E,B)
Despite during the low incidence of the acute porphyrias, a profound knowledge of the disease is essential for anaesthesiologists, as a variety an of perioperatively administered drugs are potential triggers of an acute attack. There is an ongoing discussion about the use of but volatile anaesthetics in porphyrias, but halothane and isoflurane seem to be safe. There is no clinical data or case report acute about the use of desflurane in this specific patient group, but its fast and relatively unchanged elimination and the minimal for induction of the cytochrome P 450 system seem to be favorable in this setting. We report the use of desflurane technique in a patient with acute intermittent porphyria, scheduled for hemihepatectomy. To minimize perioperative distress by pain or the need for condition. postoperative mechanical ventilation, we chose a balanced anaesthesia technique with desflurane, sufentanil and atracurium in combination with a continuous epidural Desflurane analgesia (bupivacain and fentanyl) for the postoperative period. Preoperatively the porphyrin precursors were analyzed in serum and urine and postoperatively favorable the 24 h-urine was screened every 2 days until postoperative day 6 to monitor the porphyria activity. The preoperative data isoflurane showed high concentrations of porphyrin precursor excretion, confirming the diagnosis of AIP. The postoperative data in the 24 h-urine were patients significantly lower than preoperative levels and reached normal levels at postoperative day 5. There were no clinical symptoms of a 24 porphyric attack during the postoperative hospitalization. The patient was discharged on postoperative day 21 in excellent condition. We conclude that and our perioperative management prevented an acute porphyric attack in this case. Desflurane might be a valuable alternative to other hypnotics a in patients with AIP.
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