Diminished concentrations of insulin-like growth factor I in cystic fibrosis

Cystic fibrosis is frequently accompanied by a catabolic condition with low body mass index caused by a number of disease complications. Insulin-like growth factor-I (IGF-I) is an anabolic hormone and an important marker of nutritional status, liver function, and linear growth. Available data on IGF-I in cystic fibrosis are sparse and conflicting. From 1990-3, 235 of our 240 patients (114 males, 121 females, median age 16.2 years, ranged 0.1-44.0 years) had IGF-I measured once by radioimmunoassay. IGF-I was significantly reduced compared with a healthy Scandinavian control population: mean (-2 SD to +2 SD) IGF-I SD score was -0.97 (-3.7 to 1.7) in males and -0.67 (-3.2 to 1.9) in females. Height SD score was -0.95 (-3.3 to 1.4) in males and -0.81 (-3.2 to 1.6) in females. In patients who were still in the growth period a significant correlation of IGF-I SD score to height SD score (r = 0.28, p < 0.001) was found. The low IGF-I concentrations may reflect the catabolic state of many patients with cystic fibrosis and play a part in their abnormal growth pattern.     

Less severe retinopathy of prematurity induced by surfactant replacement therapy

To assess the effect of surfactant replacement therapy (SRT) on the prevalence and severity of retinopathy of prematurity (ROP), we compared data from 160 SRT-treated preterm infants with data from 230 historic controls. The prevalence of ROP was 30.6% in the treatment group and 23.4% in the control group. Severe ROP (stages 3-4) was seen in 6.1% of the infants with ROP in the treatment group and 20.3% of the ROP patients in the control group. Surfactant therapy had no influence on the prevalence of ROP (odds ratio 1.4, 95% confidence interval 0.797-2.459, p = 0.242). However, SRT was associated with a decreased risk for severe ROP, compared to mild ROP (odds ratio 0.226, 95% confidence interval 0.056-0.905, p = 0.036). These data suggest that SRT is associated with a decreased risk for severe ROP.     

The multidisciplinary management of a family with epithelial ovarian cancer.

OBJECTIVE: To describe the management of a family with an inherited predisposition to ovarian and breast cancer. Particular attention is paid to the problems of contraception, screening, prophylactic surgery and hormone replacement therapy. SETTING: The multidisciplinary Grampian Familial Epithelial Ovarian Cancer Study Group. SUBJECTS: 162 members of a family extending over five generations. In the third generation, five of the 10 women died with epithelial ovarian cancer. Three women in generation IV have developed pre-menopausal breast cancer. There are now 78 family members in the fifth generation aged between 2 and 22 years. INTERVENTIONS: Counselling of female family members is started at the age of 18 years. The combined oral contraceptive pill is advocated to suppress ovulation. Gynaecological follow-up after the age of 28 includes yearly pelvic examination, transvaginal ultrasonography and serum CA125 estimation. Laparoscopy with peritoneal cytology is indicated if any part of this yearly assessment is abnormal. Prophylactic oophorectomy is advised between the ages of 35 and 40 years after the family is complete. In generation IV, 20 of the 29 women have undergone prophylactic oophorectomy. Oestrogen hormone replacement therapy with a cyclical progestogen is recommended after prophylactic oophorectomy. Breast cancer screening starts at the age of 25 and involves annual clinical breast examination augmented by mammography and breast ultrasound. CONCLUSIONS: Only by the careful questioning and recording of family history, including at least third degree relatives (cousins), will similar groups with familial ovarian/breast cancer be identified. When predisposing genes are characterized it will be possible to identify carriers within the family and concentrate clinical effort on them while offering appropriate reassurance to those with decreased risk.     

Characterization of a Mycobacterium smegmatis mutant lacking penicillin binding protein 1

The ponA gene of Mycobacterium smegmatis encodes a 95-kDa penicillin binding protein, PBP1, that is similar to PBP1s of Mycobacterium tuberculosis and Mycobacterium leprae. Transposon disruption of ponA in M. smegmatis resulted in a PBP1-deficient mutant that was sensitive to beta-lactam antibiotics, was more permeable to glycine, and grew slowly in liquid culture.     

Volumetric rendering of MR images

The authors developed new techniques for three-dimensional display of magnetic resonance (MR) images that preserve soft-tissue definition, are fully automatic, and work with routinely used section thicknesses. MR images are segmented, selectively enhanced, and displayed by means of a volumetric rendering algorithm. These techniques were used to illustrate normal anatomy of the brain, knee, and liver. Three-dimensional rendering of balanced spin-echo images shows the ventricles and extracerebral veins and of T1-weighted images, the sulci and gyri. The large hepatic and portal vessels can be seen with these enhancement techniques. Three-dimensional views of the knee reveal articular surfaces of the tibia and clearly depict menisci and posterior and anterior cruciate ligaments. These techniques make it possible to image multiple soft tissues simultaneously while preserving the detail contained in the original images. Three-dimensional presentation of complex, overlapping anatomic regions is helpful in surgical planning and should lead to improved diagnosis.     

Reproducibility of linear cardiac output measurement by Doppler ultrasound alone.

Inclusion of a pig aorta in an artificial circulation with pulsed blood flow allowed correlation of minute distance, measured in the aorta by Doppler ultrasound, and absolute blood flow, measured by timed blood-volume collection. The correlation coefficient was 0.99 with a standard error of prediction that was 5.4% of the minute distance predicted at a standard flow rate of 5 litres per minute. The horizontal distance between 95% confidence limits for a single prediction expressed as a percentage of 5 litres per minute was 33%, and this corresponded to the range of flow rates of 1.65 litres per minute that could give rise to the same measurement. In 142 patients duplicate measurements of minute distance were made with repositioning of the ultrasound transducer between recordings. The mean difference between paired readings, expressed as a percentage of the average (SD) of each pair was 5.4 (4.7)%. Thus, the non-invasive measurement of linear cardiac output by Doppler ultrasound is similarly reproducible in vitro and in vivo and compares favourably with the measurement of volumetric cardiac output by thermodilution.