Total Parathyroidectomy Alone or with Autograft for Renal Hyperparathyroidism?

Seventy-six patients underwent parathyroidectomy for renal hyperparathyroidism.There were 10 subtotal parathyroidectomies, 49 total parathyroidectomieswith implantation of part of one gland as an autograft, ninetotal parathyroidectomies with no autograft, and eight patientsin whom only three parathyroid glands were found. In 34 dialysis patients who underwent total parathyroidectomywith an autograft there was a high rate of recurrent hyperparathyroidismafter 6 years in those remaining on dialysis. Fifty per centhad asymptomatic recurrent hyperparathyodism and 30 per centrequired partial autograft excision for symptomatic hyperparathyroidism.In contrast, recurrent hyperparathyroidism was rare in renaltransplant recipients with good renal function. This favourableoutcome did not depend upon whether parathyroid surgery wasperformed before or after transplantation, or on the type ofparathyroidectomy. Total parathyroidectomy without an autograftwas performed in nine dialysis patients without any short-termadverse effects, and with clinical and pathological improvementin bone disease. In summary, the results of surgery for renal hyperparathyroidismwere excellent in patients who received a successful renal transplant.However, there was a high incidence of recurrent hyperparathyroidismin patients who remained on long-term dialysis. Total parathyroidectomywithout an autograft may be the treatment of choice in patientsunlikely to receive a renal transplant.     

Benzodiazepine drugs in Australia: associated mortality and morbidity

The use of benzodiazepine drugs in Australia results not only in widespread therapeutic benefits but also in harmful outcomes at both an individual and societal level. Accurate measurement of the level of harm has been precluded by a lack of robust data relating to the use of these drugs and to the resulting adverse consequences. This paper examines existing mortality and morbidity data, comments upon the available data sources and recommends areas where research is needed in order to clarify the relationship between the use of benzodiazepines and associated adverse effects.     

Genomic screening for beta-sarcoglycan gene mutations: missense mutations may cause severe limb-girdle muscular dystrophy type 2E (LGMD 2E)

Autosomal recessive limb-girdle muscular dystrophies (LGMDs) are genetically heterogeneous. A subgroup of these disorders is caused by mutations in the dystrophin-associated sarcoglycan complex. Truncating mutations in the 43 kDa beta-sarcoglycan gene (LGMD 2E) were originally identified in a sporadic case of Duchenne-like muscular dystrophy, and a common missense mutation (T151R) was identified independently in Indiana Amish pedigrees with a milder form of LGMD. To facilitate mutational analysis of larger numbers of patients directly from genomic DNA, as opposed to reverse transcribed RNA from muscle biopsies, we have determined the genomic structure of the beta-sarcoglycan gene. The open reading frame of the beta-sarcoglycan coding region extends over six exons. Primers were designed for PCR amplification of single exons from genomic DNA and subsequent single strand conformation polymorphism (SSCP) analysis. We screened 15 patients from the Brazilian LGMD patient population, 13 of whom followed a severe course. Most of the patients had been assessed previously for deficiency of alpha- sarcoglycan immunofluorescence on muscle biopsy sections as a marker for disease of the sarcoglycan complex. Novel mutations in two familial and two sporadic cases of severe childhood-onset LGMD were identified. Only one of these patients carried a truncating mutation (homozygous 2 bp deletion, FS164TER), while the other three carried missense mutations (homozygous R91P, homozygous M100K, heterozygous recessive L108R; only one allele could be identified in this family). All three missense mutations occurred in exon 3, coding for the immediate extracellular domain. Complete absence for all three of the known sarcoglycans was noted by immunohistochemistry on muscle biopsy sections of the patients.      

内镜下扩大经鼻蝶入路至斜坡区的解剖学研究

目的探索内镜下经扩大鼻蝶入路显露斜坡区的可行性,为切除斜坡区病变提供解剖学参考。方法在10例成人头部固定标本上,内镜下模拟扩大经鼻蝶手术入路显露斜坡区,观察有关显微解剖标志。结果扩大经鼻蝶内镜入路可磨除从鞍后到斜坡、枕骨大孔前缘的骨性结构;可显露斜坡区腹侧硬膜下的椎基底动脉及其分支、后交通动脉及其与大脑后动脉汇合处、动眼神经、脑干腹侧等结构。此入路的手术标志主要包括:蝶筛隐窝、蝶窦开口、视神经隆突、颈内动脉隆突与颈内动脉视神经隐窝、咽结节、枕骨大孔前缘。结论内镜下扩大经鼻蝶手术入路可充分显露鞍后-斜坡区的腹侧硬膜下结构,适用于此区病变的手术治疗。     

Reduction of cerebral infarct volume by apocynin requires pretreatment and is absent in Nox2-deficient mice

Background and purpose:

Reactive oxygen species (ROS) derived from Nox2-containing reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase activity is reportedly detrimental in cerebrovascular disease. However, ROS generation by other Nox isoforms may have a physiological role. No Nox2-selective inhibitors have yet been identified, and thus it is unclear whether isoform non-selective Nox inhibitors would necessarily improve outcome after stroke. We assessed the effect of apocynin on cerebrovascular ROS production and also on outcome following cerebral ischaemia when administered either before ischaemia or after cerebral reperfusion. The involvement of Nox2-containing NADPH oxidase in the effects of apocynin was assessed using Nox2^−/− mice.

Experimental approach:

Transient cerebral ischaemia was induced by 0.5 h middle cerebral artery occlusion followed by 23.5 h reperfusion. Mice received apocynin (2.5 mg·kg⁻¹, i.p.) either 0.5 h before ischaemia or 1 h after reperfusion. In situ superoxide production after cerebral ischaemia-reperfusion was measured in brain sections of wild-type mice at 24 h using dihydroethidium fluorescence.

Key results:

Treatment with apocynin 0.5 h before ischaemia reduced total infarct volume, neurological impairment and mortality in wild-type but not Nox2^−/− mice. Conversely, treatment with apocynin 1 h after initiation of reperfusion had no protective effect. Cerebral ischaemia and reperfusion increased superoxide production in the brain at 24 h, and pretreatment but not posttreatment with apocynin reduced superoxide levels.

Conclusions and implications:

Apocynin improves outcome following stroke when administered before ischaemia in wild-type but not Nox2^−/− mice.     

NON-CONVULSIVE STATUS EPILEPTICUS AS A COMPLICATION OF Mycoplasma pneumoniae INFECTION

SUMMARY Described is a confirmed case of non-convulsive status epilepticus, an unusual presentation of M. pneumoniae infection. The postulated pathological mechanisms in this infection are reviewed.     

Infections and Wegener's granulomatosis--a cause and effect relationship?

The association of infections and autoimmune disease has been noted by various authors. Several mechanisms have been proposed to explain this, with no current consensus. Wegener's granulomatosis (WG) is an autoimmune disease involving predominantly the pulmonary and renal systems, and is associated with a distinct autoantibody-the anti neutrophil cytoplasmic antibody (ANCA). Although no solid evidence implicates infections in the emergence of WG, direct and circumstantial data suggest this relation. We review this evidence and discuss possible underlying mechanisms. We emphasize the relationship between infections and ANCA, and their role in the maintenance of the 'on- going' inflammatory response.