Growth, puberty and obesity after treatment for leukaemia

Final height, body proportions, pubertal growth and body mass index were studied retrospectively in 142 survivors of acute lymphoblastic leukaemia (ALL). Treatment consisted of combination chemotherapy and cranial irradiation (18 or 24 Gy). Significant standing height loss and disproportion, with a relatively short back, was seen in both radiation dose groups. Girls were more severely affected than boys. Pubertal growth was adversely affected, with a reduction in peak height velocity in both sexes. Puberty occurred early in girls but at the normal time in boys. Nearly half the group were obese at final height, with no significant difference in incidence between the sexes. The relative roles of cranial irradiation and chemotherapy in the disturbance of growth, puberty and body composition observed in survivors of childhood ALL remain unclear. The aetiology is almost certainly multifactorial, with radiation-induced growth hormone insufficiency, early puberty, steroids and chemotherapy all having a role.     

Renal transplantation and osteoporosis

A cross sectional study assessed the bone mineral density (BMD) of 20 young adult patients who received a renal transplantation in childhood. The BMD of the lumbar spine, mainly trabecular bone, and of the total body, mainly cortical bone, were measured and expressed as an SD score. Fourteen patients (70%) had a BMD SD score of the lumbar spine below -1, of whom six patients were below -2. Fifteen patients (75%) had a BMD SD score of the total body below -1, of whom seven patients were below -2, Both trabecular and cortical bone appeared to be involved in the osteopenic process. The cumulative dose of prednisone was inversely correlated to both lumbar spine and total body BMD SD score. In a multiple regression analysis the cumulative dose of prednisone appeared to be the only factor with a significant effect on BMD SD score. Most young adult patients who had received a renal transplantation in childhood had moderate to severe osteopenia. Corticosteroid treatment played a major part in the development of osteopenia in these patients.     

Darier's disease: epidemiology,pathophysiology, and management

Darier's disease is a rare cutaneous disease with an autosomal dominant mode of inheritance. Greasy papules and plaques arise on the seborrheic areas and in the flexures and almost all patients have nail abnormalities. Acantholysis and dyskeratosis are the typical histological findings. The underlying defect is a result of mutations in the ATP2A2 gene on chromosome 12q23-24 that encodes for a sarco/endoplasmic reticulum calcium ATPase (SERCA 2). Acantholysis is thought to result from desmosome breakdown. Darier's disease is an example of a dominantly inherited disease caused by haplo-insufficiency. Oral retinoids are the most effective treatment but their adverse effects are troublesome. Topical retinoids, topical corticosteroids, surgery, and laser surgery have their advocates but evidence for efficacy is sparse.     

窒息新生儿血清肝功能和心功能的变化及其临床意义

目的了解窒息新生儿血清肝功能和心功能的变化,有助于指导临床对窒息新生儿重要脏器损害的严重程度的判断,指导临床积极治疗。方法检测60例窒息新生儿(轻度窒息48例、重度窒息12例)的血清总胆红素(Tbil)、直接胆红素(Dbil)、谷丙转氨酶(ALT)、总胆汁酸(TBA)、肌酸激酶同工酶(CK-MB),并设40例正常新生儿作为对照。结果窒息后新生儿Tbil出现下降(P<0.05),并随着窒息程度加重下降愈明显(P<0.05);ALT、TBA、CK-MB出现升高(P<0.05),并随着窒息程度加重升高愈明显(P<0.05)。结论Tbil、ALT、TBA和CK-MB的变化反映肝功能和心功能损害情况,窒息时新生儿肝功能和心功能应予以保护,以减轻窒息对于新生儿重要脏器的损害。     

Effect of Modified Sinisan (四逆散) on Anorectal Manometry of the Constipation Predominant Type of Irritable Bowel Syndrome

Irritable bowel syndrome (IBS) of theconstipation predominant type is a functionalintestinal disease characterized by abdominaldistress or pain, changed habit of defecationand abnormal defecation. Its pathogenesishas not been totally clarified yet. This re search is to explore the mechanism of pa tients with IBS of the constipation predomi nant type through the determination of ano rectal pressure via anus and better efficacyhas been obtained in the study by modify…     

年轻妇女宫颈癌临床特征分析

目的分析35岁以下年轻妇女宫颈癌的发病趋势、临床特征变化等.方法对1998年-2003年我院收治的67例年轻宫颈癌患者（研究组）的临床资料进行回顾性分析,并随机选择同期有完整随访资料的82例年龄大于35岁的宫颈癌患者作对照组,分析两组资料间临床特点、病理及预后之间的差异.结果 1998年-2003年6年间年轻宫颈癌的构成比呈现逐渐上升趋势,分别为11.11%、13.63%、15.21%、17.28%、20.89%、23.58%;研究组以接触性出血为主要临床表现,占79.10%（53/67）,与对照组的40.24%（33/82）比较有显著性差异（P＜0.01）;初诊时67例患者中30例（44.77%）曾在基层医院以宫颈炎、宫颈糜烂行对症治疗,12例（17.91%）以月经紊乱行调经治疗,1例术前诊断为子宫肌瘤,术后病检证实为宫颈癌;病理特点研究组内生型比率较对照组明显增高,二者比较有显著性差异;病理类型及组织类型上二者无明显差异,均以鳞癌为主（97.01%、95.21%）,但术后淋巴病理检查显示对照组淋巴转移率高于研究组,有显著性差异.结论年轻宫颈癌患者的构成比呈现明显上升趋势,宫颈癌年轻化趋势明显;年轻宫颈癌临床症状较为隐匿,容易漏诊和误诊.     

A pilot randomised controlled trial of medical versus nurse clerking for minor surgery.

BACKGROUND: Nurse led clerking is currently practiced in a growing number of UK centres, but there is a paucity of evidence to underpin the safety of this innovation. AIM: To assess the safety of nurse led clerking in paediatric day case and minor surgery. METHODS: Children aged 3 months to 15 years were randomly assigned to clerking by either a nurse or a senior house officer (SHO) (resident). All children were then independently reassessed by a specialist registrar anaesthetist to provide a "gold standard" against which practitioner performance could be judged. RESULTS: In 60 children studied, nurses identified a significantly greater proportion of the detectable abnormalities present in the sample (p = 0.16). This difference is attributable to nurses' greater accuracy in history taking (p = 0.04); no conclusions regarding the comparability of nurses' and SHOs' skills in physical examination can be derived from the current study. CONCLUSION: Evidence attests to the likelihood of nursing having superior skills in history taking to SHOs. Exploration of nursing safety in undertaking physical examination, however, requires the conduct of a large scale equivalence study. Only then can conclusions be drawn as to whether nurse led physical assessment offers children a standard of care equivalent to that which they currently receive from SHOs.     

Increased risk of leukaemia in patients with juvenile chronic arthritis treated with chlorambucil

Two cases of acute leukaemia have developed in a group of 77 patients treated with chlorambucil (Chl) because of severe juvenile chronic arthritis. The total follow-up from the beginning of Chl treatment in these patients was 560 years, indicating a highly increased risk of leukaemia. Despite favourable results, especially in patients with secondary amyloidosis, Chl should only be used in selected cases.     

Prevalence and aetiology of neurological impairment in extremely low birthweight infants

Objective : To determine the prevalence and perinatal predictors of cerebral palsy, intellectual impairment, visual impairment and deafness in a cohort of extremely low birthweight (ELBW) infants at two years of age.
Methodology : The study population comprised 199 of the 224 (89%) ELBW infants managed at the Mater's Mothers Hospital, Brisbane, between July 1977 and February 1990 and who survived to two years. The prevalence of cerebral palsy, intellectual impairment, blindness and deafness was measured by clinical, psychometric and audiological assessment and the association with 24 risk factors examined.
Results : Cerebral palsy occurred in 20 children (10%). Risk of cerebral palsy was associated with ventricular dilatation, intraventricular haemorrhage, necrotizing enterocolitis and multiple birth, though only ventricular dilatation (OR 4.41; 95% Cl 1.32-14.8) remained significant in the adjusted analysis. Intellectual impairment occurred in 20 children (10%) and was independently associated with ventricular dilatation (OR 15.0; 95% Cl 2.2-102.8), ventilation F _iO₂ >80% (OR 3.4; 95% Cl 1.01-11.5), vaginal delivery (OR 3.5; 95% Cl 1.09-11.4) and male sex (OR 6.1; 95% Cl 1.67-22.3). No perinatal predictor was statistically associated with risk of deafness. Retinopathy of prematurity (OR 36.9; 95% Cl 2.8-495.5) was associated with risk of later visual impairment.
Conclusions : Intellectual impairment was associated with a broad range of perinatal variables. Cerebral palsy was associated with fewer variables, all of which were also associated with intellectual impairment. Neurologic injury was associated with male sex and multiple birth, which are not biological insults themselves, but may be markers of susceptibility to injury.     

Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations

OBJECTIVE: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI). METHODS: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino's triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21. RESULTS: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%). CONCLUSIONS: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.