Early-Onset Chronic Renal Failure as a Complication of Acute Intermittent Porphyria

In a retrospective survey of patients who have had a provenattack of acute intermittent porphyria (AIP) in the West ofScotland a highly significant association (p < 0·001)was observed between AIP and the development of early-onsetchronic renal failure. Six patients with AIP and chronic renalfailure arising in early middle-age are described. As no othercause could be attributed to the renal failure three possiblecausal links between these two conditions were considered, namely,enhanced susceptibility to analgesic nephropathy, porphyria-inducedhypertension, and nephrotoxic effects of porphyrins and theirprecursors. We suggest that porphyria-induced hypertension isthe most important factor in causing early-onset chronic renalfailure in acute intermittent porphyria     

Acupuncture for low back pain:a clinical practice guideline from the Hong Kong taskforce of standardized acupuncture practice

OBJECTIVE: To develop a clinical practice guideline to guide the treatment of low back pain by acupuncture. METHODS: An integrative approach of systematic review of literature, clinical evidence classification, expert opinion surveying, and consensus establishing via a Delphi program was utilized during the developing process. Both evidence-based practice standards and the personalized features of acupuncture were taken into considerations. RESULTS: Based on clinical evidence and expert opinions...     

Self‐care and mobility skills in children with cerebral palsy,related to their manual ability and gross motor function classifications

Aim The aim of this study was to investigate the acquisition of self‐care and mobility skills in children with cerebral palsy (CP) in relation to their manual ability and gross motor function. Method Data from the Pediatric Evaluation of Disability Inventory (PEDI) self‐care and mobility functional skill scales, the Manual Ability Classification System (MACS), and the Gross Motor Function Classification System (GMFCS) were collected from 195 children with CP (73 females, 122 males; mean age 8y 1mo; SD 3y 11mo; range 3–15y); 51% had spastic bilateral CP, 36% spastic unilateral CP, 8% dyskinetic CP, and 3% ataxic CP. The percentage of children classified as MACS levels I to V was 28%, 34%, 17%, 7%, and 14% respectively, and classified as GMFCS levels I to V was 46%, 16%, 15%, 11%, and 12% respectively. Results Children classified as MACS and GMFCS levels I or II scored higher than children in MACS and GMFCS levels III to V on both the self‐care and mobility domains of the PEDI, with significant differences between all classification levels (p<0.001). The stepwise multiple regression analysis verified that MACS was the strongest predictor of self‐care skills (66%) and that GMFCS was the strongest predictor of mobility skills (76%). A strong correlation between age and self‐care ability was found among children classified as MACS level I or II and between age and mobility among children classified as GMFCS level I. Many of these children achieved independence, but at a later age than typically developing children. Children at other MACS and GMFCS levels demonstrated minimal progress with age. Interpretation Knowledge of a child’s MACS and GMFCS level can be useful when discussing expectations of, and goals for, the development of functional skills.     

Depression and diffuse physical symptoms in southern Chinese with temporomandibular disorders

Summary  The study investigated the experience of depressive symptoms and the relationship with diffuse physical symptoms reporting in southern Chinese seeking professional care for temporomandibular disorders (TMD) in Hong Kong. Eighty-seven new patients [77 females/10 males; mean age 39·3 years (SD 12·7)] referred to the specialist TMD clinic at the Prince Philip Dental Hospital, Hong Kong participated in this study. The Research Diagnostic Criteria (RDC)/TMD history questionnaire was used to derive Axis II psychological data. Psychological status was assessed through depression and non-specific physical symptoms (NPS) scores (pain items included and excluded) measured with RDC/TMD Axis II instruments; 42·5% of patients experienced moderate/severe depression symptoms; 59·8% and 57·5% had moderate/severe NPS scores when pain items were included and excluded, respectively. Strong, positive and statistically significant correlations were noted between depression scores and the NPS scores that included pain items ( r  = 0·80) and those that did not ( r  = 0·80). The correlations remained consistent and were of similar magnitude when male patients were excluded from the computation and also when the possible effect of patient age was controlled. While taking into account the modest patient sample which was related to a low rate of treatment seeking, depressive symptoms were common and similar to other western and Chinese patient groups. NPS reports were higher than in Singapore Chinese patients. There appeared to be a clear association between depression and diffuse physical symptoms. The findings should be considered in the holistic care of Chinese people with TMD.     

DIALYSIS AND RENAL TRANSPLANTATION OF CHILDREN IN EUROPE, 19751

ABSTRACT. The number of new paediatric patients accepted for treatment by regular dialysis and transplantation increased more slowly than in previous years. Survival in children above 10 years appeared to be better with all modes of therapy than in younger children. The only improvement in survival noted among the different treatments was in patient and graft survival of living donor transplants. A quarter of all children transferred to home dialysis were less than 10 years of age. Nephronophthisis and Henoch-Schönlein nephritis emerged as major primary renal diseases. In 1975 the proportion of retransplants in children rose and living donor grafts from fathers were more common than from mothers. Evening dialysis was practised more frequently in both hospital and home dialysis and rehabilitation in these patients seemed to be better than for those dialysed at other times. Renal osteodystrophy was present in at least half of all children dialysed for more than 1 year. The degree of growth retardation was affected by sex, chronological age and the primary renal disease. Body height on dialysis and after transplantation progressively reduced in the majority of children. Growth seemed to be more impaired in boys than in girls on dialysis. Bone age advanced faster than height age especially in girls. The pubertal growth spurt was usually delayed and depressed on long-term dialysis and the development of genitalia and pubic hair as well as menarche was retarded.     

Hyperinsulinism in infancy

Abstract Five infants with persistent hypoglycaemia due to hyperinsulinism were reported. Provocative tests for insulin release were unhelpful. Diazoxide was useful in the treatment of three patients but many side-effects were observed. These included petechial rash, hypertrichosis, acute renal failure, fluid retention and cardiac failure. Two patients underwent spontaneous remission. Three patients had nesidioblastosis, two of whom were subjected to 95% pancreatectomy. Postoperatively, recurrence of hypoglycaemia was due to hyperinsulinism in one patient and to presumed glucagon deficiency in the other. Phenytoin effectively corrected the hypoglycaemia in the patient who had postoperative hyperinsulinism. It is recommended that medical therapy with diazoxide (10–15 mg/kg per day) together with a diuretic be commenced once hyperinsulinism is diagnosed. Subtotal pancreatectomy should be performed early in these patients if hypoglycaemia cannot be controlled with medical therapy or if side-effects of treatment are documented.     

14-3-3β在人脑星形细胞瘤中的表达及意义

目的探讨14-3-3β在人脑星形细胞瘤中表达与肿瘤病理分级的关系。方法采用免疫组化法检测14-3-3β亚型在80例人脑星形细胞瘤和10例正常脑组织标本中的表达水平。结果在正常脑组织中，14-3-3蛋白6亚型只表达于神经元胞体和突起，在胶质细胞中未见表达。母亚型在人脑星形细胞瘤的表达阳性率及免疫反应评分（IRS）分别为60％、1．86±1．83。Ⅰ～Ⅳ级脑星形细胞瘤中β亚型表达阳性率分别为40％（8／20）、50％（10／20）、70％（14／20）和80％（16／20）。Ⅰ～Ⅳ级脑星形细胞瘤中β亚型IRS分别为0．88．4±0．27、1．15±0．28、2．19±0．37和3．23±0．47。不同恶性级别的脑星形细胞瘤中，14．3-3蛋白β亚型的阳性表达率无显著差异，但其IRS有显著差异（P〈0．05）。结论14-3-3β在人脑星形细胞瘤中高表达，且随着星形细胞瘤病理级别的增高而表达增强，14-3-3蛋白β亚型在脑星形细胞瘤的发生过程中具有重要作用。