Hip Displacement in Cerebral Palsy: The Role of Surveillance

IntroductionHip displacement is common in cerebral palsy (CP) and is related to the severity of neurological and functional impairment. It is a silent, but progressive disease, and can result in significant morbidity and decreased quality of life, if left untreated. The pathophysiology of hip displacement in CP is a combination of hip flexor-adductor muscle spasticity, abductor muscle weakness, and delayed weight-bearing, resulting in proximal femoral deformities and progressive acetabular dysplasia. Due to a lack of symptoms in the early stages of hip displacement, the diagnosis is easily missed. Awareness of this condition and regular surveillance by clinical examination and serial radiographs of the hips are the key to early diagnosis and treatment.Hip surveillance programmesSeveral population-based studies from around the world have demonstrated that universal hip surveillance in children with CP allows early detection of hip displacement and appropriate early intervention, with a resultant decrease in painful dislocations. Global hip surveillance models are based upon the patients’ age, functional level determined by the Gross Motor Function Classification system (GMFCS), gait classification, standardized clinical exam, and radiographic indices such as the migration percentage (MP), as critical indicators of progressive hip displacement.ConclusionDespite 25 years of evidence showing the efficacy of established hip surveillance programmes, there is poor awareness among healthcare professionals in India about the importance of regular hip surveillance in children with CP. There is a need for professional organizations to develop evidence-based guidelines for hip surveillance which are relevant to the Indian context.     

Recognition and management of phaeochromocytoma

Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in preoperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before, during and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.     

Glomerular lesions in adolescents with gross hematuria or the nephrotic syndrome

We report clinical and pathological data in 56 adolescents presenting with gross hematuria (GH) and 65 presenting with idiopathic nephrotic syndrome (INS). IgA nephropathy (present in 52%) and other mesangial lesions were found in the majority of the 56 patients with GH. Many of these patients had complex urological procedures prior to consideration of a nephrological problem. This often led to significant delays in making the appropriate diagnosis. Pathological lesions in the 65 patients with INS included minimal change NS (MCNS) in 31%, membranous glomerulonephritis (MGN) and focal segmental glomerulosclerosis (FSGS) in 18.5% each, and membranoproliferative GN (MPGN) in 12%. In 47 of the patients with INS, in whom no specific treatment had been given prior to renal biopsy, MCNS and MGN were observed with a similar frequency (26% and 23%, respectively), with FSGS and MPGN being found in 21% and 11%. These results indicate that the pathological lesions in adolescents with INS who undergo a renal biopsy more closely resemble those in adults, and are usually more severe than those in young children. However, it should be noted that our study was retrospective. Hence, there were probably some adolescents with INS who had a successful response to therapy and therefore did not have a renal biopsy performed. Southwest Pediatric Nephrology Study Group (Central Office, Baylor University Medical Center at Dallas, Tex., USA). Director, Ronald J. Hogg; Associate Directors, Fred G. Silva and F. Bruder Stapleton; Statistician, Joan S. Reisch; Administrative Assistant, Kaye Green. Participating Centers—Baylor College of Medicine, Houston, Tex.: Phillip L. Berry, L. Leighton Hill, Sami A, Sanjad, Edith Hawkins; Baylor University Medical Center, Dallas, Tex.: Ronald J. Hogg, Kaye Green; Tulane University Medical Center, New Orleans, La.: Frank Boineau, John E. Lewy, Radhakrishna Baliga, Patrick Walker; University of Arkansas, Little Rock, Ark.: Watson Arnold, Eileen Ellis, Edward Uthman; University of Colorado Health Science Center, Denver, Colo.: Gary M. Lum, Wiliam Hammond; University of Oklahoma Medical Center, Oklahoma City, Okla.: James Wenzl, James Matson, Geoffrey Altshuler, Sarah Johnson; University of Tennessee, Memphis, Tenn.: F. Bruder Stapleton, Shane Roy, III, Robert J. Wyatt, Charles McKay, William Murphy; University of Texas Health Science Center at Dallas, Tex.: Billy S. Arant Jr, Michel Baum, Fred G. Silva, Arthur Weinberg, Craig Argyle, Joseph Rutledge, Ed Eigenbrodt; University of Texas Medical School, Houston, Tex.: Susan B. Conley, Jacques Lemine, Ron Portman, Ann Ince, Regina Verani; University of Texas Health Science Center at San Antonio, Tex.: Michael Foulds, Sudesh Makker, Kanwal Kher, Melanie Sweet, Victor Saldivar, Fermin Tio; University of Texas Medical Branch, Galveston, Tex.: Ben H. Brouhard, Alok Kalia, Luther B. Travis, Lisa Hollander, Tito Cavallo, Srinivasan Rajaraman; University of Utah Medical Center, Salt Lake City; Utah: Eileen Brewer, Richard Siegler, Elizabeth Hammond, Theodore Pysher. Note that this list reflects the investigators' addresses and positions during the period of this study and not necessarily their current situations.     

National intercomparisons of I radioactivity measurements in nuclear medicine centres in India

National intercomparisons of activity measurements of ¹³¹I, a radioisotope widely used for diagnosis and therapy of thyroid related ailments, were initiated in 1979 as a quality assurance program, towards improving radiation safety procedures and related dosimetry in Nuclear Medicine Centres (NMCs) in India. Oral administration of a known quantity of radioiodine to patients requires accurate radioactivity measurements to be performed on a well-calibrated isotope calibrators. Under or over estimation of the activity due to a faulty or uncalibrated isotope calibrator could provide misleading results. Calibration of isotope calibrators and the traceablity of subsequent measurements to the national standards laboratory is one of the essential basic radiation safety requirement of the IAEA. In view of the stringent quality assurance requirements for activity measurements imposed by Atomic Energy Regulatory Board, a National Intercomparison Program was initiated and to date ten such intercomparison programs have been conducted by the Radiation Safety Systems Division, of the Bhabha Atomic Research Centre. This program has benefited the participants by making their measurements traceable to the National Primary Standards. Over the years there has been a marked increase in the number of NMCs participating in the intercomparison programs. As a result, the number of institution showing large deviation from the correct value has decreased considerably over the years. This program thus, has enabled participating NMCs to check their isotope calibrators so as to ensure proper delivery of radiation dose to the patients and hence to optimise patient exposure.     

哈乐在良性前列腺增生伴急性尿潴留中的应用

目的 :探讨α1A肾上腺素能受体阻滞剂哈乐 (tamsulosin)对良性前列腺增生 (BPH)伴急性尿潴留病人的治疗作用。方法 :对 72例BPH伴急性尿潴留病人采用随机、对照研究 ,分为治疗组和对照组。病人均行保留导尿 ,口服抗生素治疗。治疗组加用哈乐 0 .4mg ,1次 /d ,连续服用 3次。 72h后拔除导尿管。　结果 :拔除导尿管后 4 4 % (32 / 72 )的病人能自行排尿。有效率治疗组为 6 1% (2 2 / 36 ) ,对照组为 2 8% (10 / 36 ) ,两组比较差异有显著性 (P <0 .0 1)。　结论 :对BPH伴急性尿潴留应用哈乐治疗 ,可提高早期拔除导尿管后病人自行排尿的成功率 ,且疗效与前列腺体积大小无关。     

A liquid-scintillation-based primary standardization of Pb

A new radioactivity solution standard of ²¹⁰Pb has been developed and will be disseminated by the National Institute of Standards and Technology (NIST) as standard reference material (SRM) 4337. This new ²¹⁰Pb solution standard is contained in a 5 mL flame-sealed borosilicate glass ampoule, consists of (5.133±0.002) g of a nominal 1 mol L⁻¹ nitric acid solution, has a density of (1.028±0.002) g mL⁻¹ at 20 °C, has carrier ion concentrations of about 11 μg Pb²⁺ and 21 μg Bi³⁺ per gram of solution, and is certified to contain a massic activity (9.037±0.22) kBq g⁻¹ as of the reference time 1200 EST, 15 June 2006. All of the uncertainties cited above correspond to standard uncertainties multiplied by a coverage factor k=2. The standardization for the ²¹⁰Pb content of the solution was based on 4πβ liquid scintillation (LS) measurements using CIEMAT/NIST ³H-standard efficiency tracing (CNET). Confirmatory determinations were also performed by high-resolution HPGe γ-ray spectrometry, by 2π spectrometry with a Si surface barrier detector of separated ²¹⁰Po, and by 4πβ(LS)–γ(NaI) anticoincidence counting.     

α核素非均匀微观分布对细胞微剂量的影响

目的探讨放射免疫治疗中α核素在细胞水平上非均匀微观分布对细胞S因子的影响。方法利用蒙特卡罗方法随机模拟α粒子的发射;基于连续慢化近似模型,根据α粒子剩余射程-能量的关系,采用插值法计算α粒子入射动能和出射动能,得到其在靶区内的能量沉积。以213Po为例,计算了不同细胞大小及核素不同微观分布（均匀分布、中心分布、随核素距细胞中心的距离线性递增、线性递减、指数递增、指数递减）下细胞对细胞的S因子。结果213Po均匀分布下的S（C←C）与Hamacher给出的结果符合得很好;不同微分布下的S（C←C）有显著差别。分析表明,核素不同微观分布所造成的α粒子在靶区内运动的平均弦长的显著变化,以及由此产生的α粒子平均碰撞阻止本领的变化,是造成细胞S因子有显著差别的主要原因。结论放射性核素非均匀微分布类型显著影响细胞S因子的大小,在估算细胞吸收剂量时应予以重视。     

Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ

Lung function, smoking, age and mortality data in 158 adult severe alpha₁-antitrypsin deficient, PiZZ individuals, followed from 1963 to 1982 were analyzed. Low initial FEV₁ value was significantly associated with increased mortality (p < 0.005). A 3 yr mortality rate of 40% was found in individuals whose initial FEV₁ values were less than 30% of that predicted. In contrast, the corresponding 3-yr mortality among those whose initial FEV₁ values were between 30 and 65% of that predicted was only 7%. Smokers were found to have significantly lower FEV₁ levels (p = 0.008) and higher mortality (p < 0.005) than non-smokers. The difference between current and ex-smokers in mortality and FEV₁ level were not statistically significant (p = 0.9 and p>0.25, respectively). Cross-sectional analysis of the initial FEV₁ values indicated a significant decline (p < 0.005) of FEV₁ with increasing age. This decline was greater among smokers than non-smokers. Longitudinal analysis of FEV₁ rates of decline in 80 cases with follow-up FEV₁ measurements failed to detect any significant differences between smokers and non-smokers, but was performed late in the disease process. The application of these results to the planning of studies on replacement therapy, smoking intervention strategy and longitudinal follow-up is discussed.