Effect of growth hormone on height, weight, and body composition in Prader-Willi syndrome

AIMS: To evaluate the effect of the administration of growth hormone on stature, body weight, and body composition in children aged between 4 and 10 years with Prader-Willi syndrome. METHODS: Height, weight, and skinfold thickness were recorded in 25 children using standard anthropometric techniques at recruitment, and six months later, shortly before the start of daily subcutaneous injections of growth hormone. Body composition was assessed via a measurement of total body water using stable isotopes. Measurements were repeated at the end of the six months of growth hormone administration. Measurements of height, weight, and skinfold thickness were expressed as standard deviation scores (SDSs). RESULTS: There was a significant reduction in the percentage of body fat after growth hormone treatment; height velocity doubled during treatment; body weight did not change significantly when expressed as an SDS. Skinfold thickness at both the triceps and subscapular site decreased in absolute terms and when expressed as an SDS. CONCLUSIONS: These results indicate sufficient potential benefit to justify a more prolonged trial of growth hormone treatment and an exploration of different dosage regimens in children with Prader-Willi syndrome.     

Vitamin A intake and xerophthalmia among Indian children

OBJECTIVE: To estimate prevalence of xerophthalmia and to assess dietary intake of vitamin A in Indian children aged under 6 y. DESIGN: Cross sectional study. STUDY SETTING: Urban slums under Urban Health Centre affiliated to Department of Preventive and Social Medicine, Government Medical College, Nagpur, India. PARTICIPANTS: The study included 1094 all children under 6 y of age, from two randomly selected urban slums. METHODS: Xerophthalmia was diagnosed on the basis of ocular signs and symptoms (WHO recommendations). Dietary intake of vitamin A was assessed by using one year recall method recommended by International Vitamin A Consultative Group. RESULTS: Prevalence of xerophthalmia was estimated to be 8.7%. Nine hundred and ninety-five (90.9%) study subjects were identified as inhabitants consuming dietary vitamin A at below recommended levels. (UPF score < 210). Five hundred and ninety-three (54.2%) study subjects were consuming dietary vitamin A at approximately less than 200 RE/d (UPF score < 120) while 402 (36.2%) were consuming approximately 200-300 RE/d (UPF score 120-210). The prevalence of xerophthalmia was found to be decreasing as the score of usual pattern of food consumption (UPF) increased. CONCLUSIONS: Children with a dietary intake represented by a UPF score of less than 120 were at high risk of developing xerophthalmia, whereas, those consuming vitamin A equal to a UPF score greater than 120 were at comparatively less risk despite being below the recommended levels.     

Exploring NANDA's Definition of Nursing Diagnosis: Linking Diagnostic Judgments with the Selection of Outcomes and Interventions

Nursing diagnoses are described by definition as the basis for selection of interventions to achieve health outcomes. Processes used to link diagnostic judgments with desired outcomes and interventions can be holistic or reductionistic. A model of clinical inference is presented to illustrate the interrelated nature of judgments about diagnoses, desired outcomes, and interventions. Implications for development of diagnostic and taxonomic tools to guide holistic judgments are explored.     

Surgical treatment of accessory atrioventricular pathways and symptomatic tachycardia in children and young adults

Twenty-seven patients aged 21 years or younger (mean 15) with symptomatic tachycardia underwent operation for ablation of an accessory atrioventricular pathway. Six patients had associated Ebstein's malformation of the tricuspid valve. Supraventricular tachycardia had been present for a mean of 5 years. At electrophysiologic study, 4 patients were found to have 2 accessory pathways. Left ventricular free wall pathways were found In 14 patients, right ventricular free wall pathways in 10 and septal pathways in 6. Successful initial ablation of all the pathways was achieved in 26 of the 27 patients. No patient died perioperatively and none had persistent complete heart block. During a mean follow-up of 11 months, no patient had recurrence of an arrhythmia related to the accessory pathway. Thus, the surgical treatment of children and young adults with accessory atrioventricular pathways and symptomatic supraventricular tachycardia is safe and effective. For these patients, unless the tachycardia can be easily controlled with a minimal number of drugs and adverse effects, surgical ablation should be considered early in the clinical course.     

Evolution of Sezary syndrome in the course of hairy cell leukemia

A patient with a history of "leukemia" for 19 yr and documented hairy cell (HC) leukemia for 10 yr developed mycosis fungoides and the Sezary syndrome. The manifestations of both diseases were diagnostic on clinical and pathologic grounds. Ultrastructural, immunohistochemical, and surface marker techniques proved the HC to have phenotypic characteristics of the T-helper subset of lymphocytes to which the Sezary cells (SC) also belonged. Both types of cells contained tartrate- resistant acid phosphatase. HC did not infiltrate the skin. SC did not contain ribosome lamellar complexes. Because of otherwise overlapping morphology and the apparent replacement of HC by SC, it is likely that the Sezary cells constituted a genetic variant of the original neoplastic clone represented by the hairy cells. Since the biologic and therapeutic implications of such clonal evolution may be important, subtle phenotypic changes should be looked for repeatedly in patients with these diseases.     

Expression of the cutaneous lymphocyte antigen and its counter-receptor E-selectin in the skin and joints of patients with psoriatic arthritis

We have investigated whether the skin-homing T lymphocytes identified by the cutaneous lymphocyte antigen (CLA) are increased in the synovial membrane of patients with psoriatic arthritis. Twenty-six synovial samples (13 psoriatic arthritis, seven rheumatoid arthritis, six osteoarthritis) were obtained from involved knees. Lesional skin biopsies were taken from nine of the patients with psoriatic arthritis and six patients with psoriasis alone. All samples were single- and dual-stained for CLA and CD3 (to identify T lymphocytes) using HECA-452 (anti-CLA) and anti-CD3 monoclonal antibodies. E-selectin expression was also determined. The percentage of dual-stained lymphocytes was significantly greater in psoriatic skin than in synovium (P < 0.001) and similar between psoriatic and rheumatoid synovium. There was no significant difference in the percentages of CLA-positive cells in psoriatic skin in patients with psoriatic arthritis compared with psoriasis alone. The intensity of endothelial E-selectin expression was significantly greater in skin psoriasis than in synovium (P < 2 x 10(- 5)), and rheumatoid synovium had significantly greater expression than psoriatic synovium (P < 0.05). However, there was no significant correlation between E-selectin expression and the percentages of CLA- positive lymphocytes. This study provides further evidence that the CLA antigen is enriched on skin-homing lymphocytes. Conversely, the link between skin and joint inflammation in psoriatic arthritis does not seem to be explained by increased trafficking of CLA T cells to psoriatic synovium.