Ovarian and uterine characteristics after total body irradiation in childhood and adolescence: response to sex steroid replacement.

OBJECTIVE: To study the effect of total body irradiation (14.4 Gray) in childhood and adolescence on ovarian and uterine characteristics, and to investigate the response to physiological sex steroid serum concentrations. DESIGN: All long term post-pubertal female survivors of total body irradiation who had been treated in paediatric centres in Scotland were identified. Their ovarian and uterine characteristics were studied. SETTING: Recruitment was from follow up oncology clinics. SAMPLE: Nine women were identified, eight of whom were assessed and five progressed to detailed investigation. A control population of 12 women treated for acute leukaemia, but not treated with total body irradiation, and five healthy women with no history of childhood malignancy were recruited as controls. METHODS: Ovarian function was determined by measurement of serum gonadotrophins and sex steroids. Uterine response to physiological sex steroid replacement was investigated in women with ovarian failure, and to endogenous sex steroid production in women with ovarian function by ultrasound scan. The physiological sex steroid replacement was achieved with transdermal oestradiol patches and self administered vaginal progesterone pessaries. MAIN OUTCOME MEASURES: Determination of ovarian function and uterine response to physiological sex steroid serum concentrations. RESULTS: Six of eight women treated with total body irradiation had ovarian failure; preservation of function was seen in two girls treated pre-pubertally who had biochemical evidence of incipient ovarian failure. Four women with ovarian failure had reduced uterine volume, undetectable blood supply and absent endometrium at baseline assessment; after three months of physiological sex steroid replacement treatment uterine blood supply and endometrial response were not significantly different from controls; uterine volume improved but remained significantly smaller than controls and correlated with age at total body irradiation. CONCLUSIONS: Ovarian failure after total body irradiation is common and risk relates to age at treatment. Physiological sex steroid replacement improved uterine measures and these women may benefit from assisted reproductive technology.

Clinical follow-up of high mid-trimester maternal serum intact human chorionic gonadotrophin concentrations in singleton pregnancies.

Mid-trimester biochemical screening of 38 143 pregnancies in south-east Scotland revealed 127 cases (0.34 per cent) in which the maternal serum (MS) intact human chorionic gonadotrophin (hCG) concentration was > or = 4 multiples of the median in singleton pregnancies (MOM). Three were lost to follow-up but in 72 (58 per cent) complications developed or there were associated fetal abnormalities. This percentage was greatest at very high hCG concentrations, 92 per cent with hCG > or = 10 MOM (n = 12) compared with 48 per cent with hCG concentrations of 4-4.99 MOM (n=69). 22 cases had an MS alpha-fetoprotein > or = 2 MOM in addition to an MS hCG > or = 4 MOM, and in only 3 of these was the pregnancy uneventful; 86 per cent were associated with abnormalities or pregnancy complications.

Audit of a screening service for fetal abnormalities using early ultrasound scanning and maternal serum alpha-fetoprotein estimation combined with selective detailed scanning.

The objective of the study was the evaluation of a screening service for fetal abnormality using early ultrasound scanning to date and establish viability of the pregnancy combined with maternal serum alpha-fetoprotein estimation at 16 weeks and thereafter selective detailed scanning at 18-20 weeks based on clinical indication. The study was designed to be retrospective, emphasizing the possibility of diagnosis. Early scans were performed by radiographers and radiologists, detailed ones by radiologists and obstetricians with subspecialty training in prenatal diagnosis. The study was carried out in the ultrasound department of a large University Hospital. The subjects studied were all cases of abnormality identified pre- or postnatally in women delivering in the hospital over 4 years and to whom the screening service applied. Information was obtained from ultrasound and maternity records, birth notifications, pathology and autopsy reports and a special needs register. Cases were classified as not detectable, potentially detectable and usually detectable. Major fetal anomalies numbered 255 (total deliveries 19,497), a prevalence of 1.3%; 130 (51%) were diagnosed by ultrasound with 64% before 24 weeks and 36% later. Eleven chromosomal anomalies (4%) were diagnosed by genetic methods and 114 (45%) anomalies were not diagnosed antenatally. The sensitivity of the program was 37% before 24 weeks. Chromosomal anomalies, lesions of the central nervous system and cardiac defects were most common, followed by lesions of the gastrointestinal and urinary tracts. A large proportion of the detected and detectable anomalies were lethal.(ABSTRACT TRUNCATED AT 250 WORDS)

Oral teratoma (epignathus) with intracranial extension: a report of two cases.

Epignathi are unusual congenital tumours presenting as oropharyngeal masses, often resulting in rapid asphyxia following birth. Occasionally, intracranial extension of the tumour is present, and two such cases are described. The presence of this complication, diagnosable by ultrasound examination, indicates that aggressive surgical treatment is inappropriate.

Ultrasound evaluation of ectopic pregnancy including correlation with human chorionic gonadotrophin levels.

A total of 267 patients referred for ultrasound examination because of clinical suspicion of ectopic pregnancy were studied prospectively. The outcome was correlated with the ultrasound findings and human chorionic gonadotrophin levels using a quantitative serum assay. The value of the following in the diagnosis of ectopic pregnancy are assessed: ultrasound criteria for early diagnosis of intrauterine pregnancy before visualization of a viable fetus, the discriminatory zone of hCG and extrauterine abnormalities.

A comparison of fetal abdominal circumference measurements and Doppler ultrasound in the prediction of small-for-dates babies and fetal compromise.

A total of 145 pregnancies clinically suspected of being small-for-dates was studied at presentation with a single measurement of the fetal abdominal circumference and Doppler studies of the umbilical and arcuate arteries. The abdominal circumference measurement gave the best prediction of the small-for-gestational-age (SGA) baby (sensitivity 73%, umbilical artery sensitivity 47%, arcuate artery sensitivity 29%). The umbilical artery measurement gave the best prediction of antenatal fetal compromise; the performance of the tests was compared for a fixed sensitivity of 100% (i.e. all cases of antenatal compromise would be detected), the specificity of the umbilical artery measurement was 77%, abdominal circumference measurement 12% and arcuate artery measurement 2%. In our data, umbilical artery studies were not a sensitive predictor of the SGA baby but they did give an accurate prediction of the potentially compromised SGA fetus.

Heartrate variation of umbilical artery Doppler waveforms.

Umbilical artery Doppler waveforms from 20 patients were used to investigate the dependence of resistance index and pulsatility index on beat to beat pulse length over short time periods for individual patients, and on the usefulness of a common normalisation formula. For individual patients the resistance index and pulsatility index were only partially correlated with pulse length. Changes in both indices occurred independently of pulse length. Use of a common normalisation formula resulted in no significant reduction of the coefficient of variation of the resistance index (p greater than 0.1), and a reduction in the coefficient of variation of the pulsatility index of 10% (p greater than 0.001). It is concluded that short term changes in resistance index and pulsatility index cannot be corrected by a common normalisation formula.

The choice of index for umbilical artery Doppler waveforms.

Umbilical artery Doppler waveforms acquired from 211 patients were used to investigate the power of different waveform indices in predicting antenatal fetal compromise. Waveform indices were calculated using a BBC microcomputer. The specificity at 100% sensitivity for detection of antenatal fetal compromise was not significantly different for resistance index, pulsatility index, normalised resistance index and normalised pulsatility index, and was approximately 80%. For the indices of resistance time and downslope the specificity was significantly lower. This similarity in the power of a number of indices in the detection of antenatal fetal compromise suggests that standardisation to one of the simpler indices such as resistance index or pulsatility index could be adopted.

The effects of placental site on the arcuate artery flow velocity waveform.

Eighty-three consecutive patients were studied to determine the effect of placental site on the arcuate artery flow velocity waveform. The resistance indices derived from signals obtained from the placental side of the uterus were significantly lower than those derived from signals obtained from the nonplacental side. Doppler studies of the uteroplacental circulation should be performed preferably with knowledge of the placental site.

Determination of the presence of fetal apnoea using umbilical artery and umbilical vein Doppler waveforms.

Fetal breathing movements were observed using real-time ultrasound imaging. Simultaneous Doppler recordings of the flow velocity waveform from the umbilical artery and umbilical vein were obtained. Twenty patients were studied. The Doppler traces obtained during fetal apnoea and fetal breathing for each patient were analysed using a BBC microcomputer. A combination of venous variation index (VVI) and the coefficient of variation of arterial pulsatility index (CVPI) gave complete separation between breathing and apnoeic groups (lower values of VVI and CVPI being associated with the apnoeic group). Hence, when stand alone continuous wave Doppler units are used to acquire umbilical artery Doppler waveforms during fetal apnoea, the presence of apnoea can be determined using the variability of the arterial and venous trace without the need for visualisation of the fetus using imaging equipment.

Umbilical artery Doppler waveform in pregnancies with uncomplicated intra-uterine growth retardation.

Seventy-four pregnancies with uncomplicated intra-uterine growth retardation were assessed at the time of diagnosis by Doppler ultrasound umbilical artery flow velocity waveform. The results were not disclosed to the clinicians. Only those pregnancies with a complete absence of pre-existing disease or pregnancy complications were included. Ten patients showed evidence of fetal compromise due to asphyxia either before or during labour. In all cases the umbilical artery flow velocity waveform had been abnormal, and this abnormality always preceded cardiotocogram abnormality by up to 5 weeks. Though the resistance index was significantly associated with birth weight (p less than 0.001), this relationship was clearly dependent on the compromised fetuses who tended to be very small, and the true association is probably between resistance index and compromise, with birth weight being an intervening variable.

"Bullet"-shaped head in fetuses with spina bifida: a pointer to the spinal lesion.

A "bullet"-shaped head, characterized by flattening in the parietal regions with pointing of the frontal bones, was noted in ultrasound examinations of fetuses with neural tube defects. In a series of 12 fetuses with spinal defects, this finding was present in nine. This abnormal head shape is a useful pointer to the spinal lesion.

Periosteal new bone in patients on intermittent haemodialysis: an early indicator of aluminium-induced osteomalacia?

Periosteal new bone forming along the distal shafts of the tibia, fibula and pelvic inlet was observed to be an unusual feature in patients on intermittent haemodialysis. Sequential skeletal surveys of 13 patients exhibiting this feature were reviewed and correlated with the biochemical, histological and clinical data. The radiological features comprised periosteal new bone, minimal or no evidence of secondary hyperparathyroidism, sclerosis (not in the classical 'rugger jersey' spine distribution but affecting, particularly, the femoral heads) and, in several patients, numerous fractures, particularly of the ribs. There were 10 patients with osteomalacia, in seven of whom the features were consistent with aluminium-induced bone disease. We suggest that the finding of periosteal new bone in the above distribution in a patient on intermittent haemodialysis should alert the clinician to the possibility of aluminium intoxication.

Real time ultrasound scanning of the head in neonates and infants, including a correlation between ultrasound and computed tomography.

The results of ultrasound (US) head scans performed in a children's hospital over a 6 month period have been reviewed and correlated with the results of computed tomography (CT). Two hundred and twenty-four scans were performed on 141 children ranging in age from a few days to 18 months. The clinical indications with which these children were referred were: abnormal neurological symptoms or signs, screening for hydrocephalus in neural tube defects (NTD), enlarging heads, suspected intra-cranial haemorrhage (ICH) and its sequelae, suspected complications of meningitis, investigations of suspected syndromes and following trauma. Twenty-nine of these children also had CT scans. There was good correlation between the two examinations and in only one case (a subdural collection) was a serious abnormality missed by US. US head scanning has been found to be a very reliable technique. It is the initial investigation of choice for imaging the brain of neonates and infants and in many instances it is the only investigation necessary.

A comparison of dilute barium and dilute water-soluble contrast in opacification of the bowel for abdominal computed tomography.

Dilute barium was compared with dilute water-soluble contrast medium as an oral preparation for abdominal computed tomography (CT). Patients generally preferred the barium to the water-soluble contrast medium. Both preparations opacified the bowel satisfactorily. Barium caused no streaking artefacts. Dilute barium is the preparation of choice for abdominal CT.

Subunit structure of higher plant glyceraldehyde-3-phosphate dehydrogenases (EC 1.2.1.12 and EC 1.2.1.13).

In a previous publication (Cerff, R. (1979) Eur. J. Biochem., 94, 243--247) we demonstrated that chloroplast NADP-linked glyceraldehyde-3-P dehydrogenase (EC 1.2.1.13) from higher plants consists of two separate isoenzymes with apparent subunit compositions A2B2 (isoenzyme 1) and A4 (isoenzyme 2), where Subunits A and B are distinguished by slightly different molecular weights (A smaller than or approximately to B). In the present study we compare isoenzymes 1 and 2 from Sinapis alba and Hordeum vulgare on the basis of antigenic cross-reactivity, tryptic peptides, and amino acid composition. Isoenzymes 1 and 2 show immunochemical identity. They also have very similar tryptic peptide maps and amino acid compositions. This strongly suggests that Subunits A and B of the NADP-linked enzyme are very similar in primary sequence. As opposed to this, cytoplasmic NAD-specific glyceraldehyde-3-P dehydrogenase (EC 1.2.1.12) does not cross-react with antisera raised against the NADP-linked enzyme. Furthermore, tryptic peptide maps of the NAD-specific enzyme show little or no similarity with those of the NADP-linked enzyme. This indicates that the subunits of the NADP-linked enzyme and the subunit of the NAD-specific enzyme are different proteins coded by separate genes. The differences in the amino acid compositions between the two species corresponds to a SdeltaQ value of 21, suggesting some sequence resemblance and a common phylogenetic origin.