Delayed adolescent growth in homozygous sickle cell disease

Analysis of the growth abnormalities in sickle cell disease has been limited by the lack of longitudinal observations in individuals, and by an inability to quantitate the observed patterns. To investigate the timing and pattern of the adolescent growth spurt, longitudinal observations of height from the Jamaican cohort study were fitted to a mathematical model of growth (Preece-Baines model 1). The study included 44 children with homozygous sickle cell (SS) disease, 44 age and sex matched subjects with sickle cell haemoglobin C (SC) disease, and 44 age and sex matched controls with normal (AA) haemoglobin. Compared with AA controls, the onset of the adolescent growth spurt was delayed in SS disease by 1.4 years (95% confidence interval 0.8 to 2.0) with no significant sex difference. The age at peak height velocity was delayed by 1.6 years (0.9 to 2.3) in SS compared with AA subjects but the adolescent growth of SS children was otherwise normal and there was no difference in the attained height by age 17.9 years. The growth spurt was not delayed in SC disease. The age at menarche in girls with SS disease (mean (SD) 15.4 (1.3) years) was significantly later than girls with SC disease (13.7 (1.7) years) and those with AA haemoglobin (13.1 (1.3) years) but these genotype differences were no longer significant after controlling for the delay in the adolescent growth spurt. The normally coordinated but slightly delayed pattern of growth and normal adult heights suggests a good prognosis for adolescent growth delay in SS disease. Most children with SS disease can therefore be reassured on the outcome of retarded adolescent growth.     

Delayed adolescent growth in homozygous sickle cell disease.

Analysis of the growth abnormalities in sickle cell disease has been limited by the lack of longitudinal observations in individuals, and by an inability to quantitate the observed patterns. To investigate the timing and pattern of the adolescent growth spurt, longitudinal observations of height from the Jamaican cohort study were fitted to a mathematical model of growth (Preece-Baines model 1). The study included 44 children with homozygous sickle cell (SS) disease, 44 age and sex matched subjects with sickle cell haemoglobin C (SC) disease, and 44 age and sex matched controls with normal (AA) haemoglobin. Compared with AA controls, the onset of the adolescent growth spurt was delayed in SS disease by 1.4 years (95% confidence interval 0.8 to 2.0) with no significant sex difference. The age at peak height velocity was delayed by 1.6 years (0.9 to 2.3) in SS compared with AA subjects but the adolescent growth of SS children was otherwise normal and there was no difference in the attained height by age 17.9 years. The growth spurt was not delayed in SC disease. The age at menarche in girls with SS disease (mean (SD) 15.4 (1.3) years) was significantly later than girls with SC disease (13.7 (1.7) years) and those with AA haemoglobin (13.1 (1.3) years) but these genotype differences were no longer significant after controlling for the delay in the adolescent growth spurt. The normally coordinated but slightly delayed pattern of growth and normal adult heights suggests a good prognosis for adolescent growth delay in SS disease. Most children with SS disease can therefore be reassured on the outcome of retarded adolescent growth.     

The utility of tumour markers in assessing the response to chemotherapy in advanced bladder cancer

In patients with advanced bladder cancer receiving chemotherapy, early assessment of response can avoid unnecessary toxicity. The aim of this study was to assess the role of tumour markers in monitoring response. Serum levels of one or more of markers beta human chorionic gonadotrophin (betahCG), carcinoembryomic antigen (CEA), CA125 and CA19.9 were measured in 74 patients with advanced bladder cancer receiving chemotherapy from 1992 to 1997. Forty-three of 74 (58%) of patients had at least one raised marker (1.5 times upper limit of normal range). This was more common in patients with extra-pelvic disease than in those with disease confined to the pelvis (P = 0.002). Thirty-eight of 78 (49%) assessable patients had a radiological response. Neither clinical response (P = 0.81) nor survival (P = 0.16) differed between marker-negative and marker-positive patients. Clinical response was strongly related to marker response in the 35 comparable patients (P = 0.0001). No patient had a clinical response without response of at least one marker. Ninety per cent of patients who achieved a marker response had done so by 8 weeks. Monitoring of tumour markers in patients with advanced bladder cancer can help predict the response to chemotherapy.     

Evaluation of an instrument to assess the needs of patients with cancer. Supportive Care Review Group

BACKGROUND: This study aimed to assess the face, content, and construct validity and the internal reliability of a tool for assessing the generic needs of patients with cancer (the Supportive Care Needs Survey). METHODS: A total of 1,492 consecutive patients attending the surgical, radiation, or medical oncology departments of 9 cancer treatment centers in New South Wales, Australia, were asked to participate. Of the 1,370 eligible patients, 1,354 (99%) consented to participate and 888 (65%) completed the survey. Eligible consenting patients were given a Supportive Care Needs Survey to complete at home and return by mail within 7 days. RESULTS: In the assessment of construct validity, the principal components method of factor analysis identified 5 factors with eigenvalues greater than 1, which together accounted for 64% of the total variance (patients' needs in the domains of psychologic, health system and information, physical and daily living, patient care and support, and sexuality). Face and content validity were found to be high following pilot tests and tests of reading ease. Internal reliability coefficients (Cronbach alpha) of all 5 factor-based scales were found to be substantial, ranging from 0.87 to 0.97. CONCLUSIONS: These findings suggest that the Supportive Care Needs Survey provides a reliable and valid index of the global needs of oncology patients. The standardized and widespread application of this instrument is recommended following further refinement and evaluation.     

Familial incidence of bifid and double ureters

The incidence of a duplex urinary tract was determined in the parents and sibs of 30 index patients with either unilateral or bilateral bifid or double ureters. Our results support the hypothesis that a duplex urinary tract is inherited by an autosomal dominant gene of variable penetrance. Routine screening by limited intravenous pyelography of sibs of index patients is recommended in order to detect a duplex urinary tract before the onset of complications.     

Office-based insertion of pressure equalization tubes: the role of laser-assisted tympanic membrane fenestration

OBJECTIVE: To describe the role of the hand-held otoscope combined with a flashscanner CO2 laser, OtoLAM (ESC/Sharplan, Yokneam, Israel), for pressure equalization tube (PET) insertion in an office setting. STUDY DESIGN: Prospective, multisite, clinical cohort trial (Institutional Review Board approved; informed consent) in the setting of pediatric otolaryngology outpatient departments at four tertiary care children's hospitals. METHODS: Selected for the study were 54 patients (96 ears), ages 6 months to 23 years, who met standard indications for PET insertion using cold-knife myringotomy and tube insertion under general anesthesia. PETs were indicated for recurrent otitis media, chronic otitis media with effusion, and eustachian tube dysfunction-all unresponsive to medical therapy. Topical anesthesia was achieved with iontophoresis (n = 1) or topical anesthesia: 8% tetracaine on an Otowick (Xomed Surgical Products, Jacksonville, FL, catalogue No. 400141) against the tympanic membrane for 45 to 180 minutes (n = 53). Laser-assisted tympanic membrane fenestration was performed with the OtoLAM set at single pulse, 2.0- to 2.6-mm spot size, and between 3 and 18 W. Insertion of grommets was accomplished using the otomicroscope and an "alligator" microforceps. Restraints with papoose were used in 79% of children with a mean age of 34.4 months (SD = 60.9 mo). Clinical, parent/patient, and physician satisfaction and comparative cost impact outcomes are described. RESULTS: All ears but three (3%) underwent successful placement of a PET. Pain was described as "absent" in 39%, "present but tolerable" in 30%, and "severe" in 30% of children at the time of procedure; 5 minutes after the procedure pain was described as "absent" in 75%, "present but tolerable" in 22%, and "severe" in 3%. Tube plugging (3 of 74 available ears; 4%) or persistent otorrhea (1 of 74 ears; 1.4%) occurred infrequently at the 1-month follow-up. Before PET insertion, hearing loss was noted in 66% of cases (mild, 38%; moderate, 22%; and severe, 6%). Mild hearing loss was noted in only 8% and moderate hearing loss in 2% of 47 (50%) of the ears at the 3-month follow-up. Ninety-two percent of parents were highly satisfied with the procedure in preference to PETs in the operating room under general anesthesia, and 97% preferred OtoLAM with PET insertion, rather than further courses of antibiotics; only one parent would rather have had the PET insertion under general anesthesia. Cost savings to health care organizations, particularly payers, and to parents are substantial (32%-48%) and warrant attention. Cost to the physician is manageable only if an appropriate approach to the third party payers results in a substantial increase in reimbursements. CONCLUSIONS: The data indicate excellent clinical effectiveness, reduced risk, and high parent and physician satisfaction. Strong incentives for physicians to use this technique are in all stakeholders' best interests. These incentives need to evolve as soon as possible for the more widespread acceptance of OtoLAM with PET insertion in an office setting for appropriately selected patients.     

Observer variability in interpretation of abdominal radiographs of infants with suspected necrotizing enterocolitis

We examined (1) the observer variability (both interobserver and intraobserver) in interpretation of abdominal radiographs of infants with suspected necrotizing enterocolitis (NEC), (2) the interobserver variability for individual radiologic signs used to diagnose NEC, and (3) the influence of experience in determining the extent of observer variability. Our hypotheses were (1) there would be considerable observer variability in interpretation of abdominal radiographs of infants with suspected NEC; (2) the extent of observer variability would differ for individual radiologic signs of NEC; and (3) the extent of observer variability would be determined by the observer's experience. The participants included 12 observers: two pediatric radiologists, four attending neonatologists, three neonatal fellows, and three pediatric residents. The participating observers under similar interpretation conditions, twice independently, interpreted the same 40 pairs of abdominal radiographs from infants with suspected NEC. The interval between the two interpretations was 3 to 6 months. Intraobserver and interobserver variability was assessed by applying the Kappa statistic to the radiologic signs of NEC for the two separate interpretations. The observers were blinded to patient's identity and the clinical course. Each observer recorded the absence, suspicion, or presence of (1) intestinal distention, (2) air fluid levels, (3) bowel wall thickening, (4) pneumatosis intestinalis, (5) portal venous gas, (6) pneumoperitoneum, and (7) NEC. We found low intraobserver and interobserver agreements. There was considerable variation in observer variability for individual radiologic signs. Trained observers performed better than intraining observers. We conclude that the radiologic signs in isolation should not be considered reliable. We recommend studies to formulate more objective criteria for many of the radiographic features of NEC. Standardization and periodic enforcement of these criteria among observers could reduce observer variability. We suggest that, to decrease both false-negative and false-positive interpretation, an experienced observer should always review the radiographs of infants with suspected NEC.     

New areas for psychological assessment in general health care settings: what to do today to prepare for tomorrow

Unique roles of the professional psychologist are outlined with respect to increasingly restrictive utilization practices of managed care. Suggestions of how to develop less traditional venues of practice, the types of instruments to use, and report formats, along with ways to persuade primary care physicians (PCPs), managed care organizations, and facilities to utilize psychological assessment services are provided. Medical cost offsets, cost-efficient quality of care, and models of practice are also discussed.     

Reduced N-acetylaspartate in the temporal cortex of rats reared in isolation.

BACKGROUND: Isolation rearing of rats is a nonpharmacologic, nonlesion manipulation that leads to deficits in prepulse inhibition (PPI) and other behavioral and neurochemical alterations reminiscent of schizophrenia. N-acetylaspartate (NAA) is present in high concentrations in the central nervous system and is found primarily in neurons. N-acetylaspartate is considered to be a marker of both neuronal loss and cellular dysfunction. Magnetic resonance spectroscopy studies have shown reductions of cortical and hippocampal NAA in schizophrenia, and a recent postmortem study has demonstrated a regionally selective temporal cortex deficit. METHODS: The aim of the present study was to determine whether rats reared in isolation exhibit deficits in PPI and reductions in NAA in discrete brain regions, namely the temporal cortex, frontal cortex, hippocampus, and striatum. RESULTS: Compared with socially housed rats, isolation rearing resulted in PPI deficits (p <.05) and reductions in NAA in the temporal cortex (p <.001), with no significant change in the other regions investigated. CONCLUSION: These results suggest a disturbance of neuronal function, reflected by NAA reductions in the temporal cortex in isolation-reared rats, providing further evidence that isolation rearing can mimic aspects of the neuronal pathology of schizophrenia.