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The distribution of the free and bound hopanoic acids in both unheated and heated (350 degrees C for 50 h) kerogens, isolated from the Messel oil shale, were analyzed by GC-MS. The bound acids were released by subjecting the kerogen to three different treatments, namely, thermochemolysis in the presence of tetramethylammonium hydroxide (TMAH), as well as basic and acidic hydrolyses. All of these methods gave a series of hopanoic acids ranging from C(30) to C(34), in which the biological 17beta, 21beta(H) configuration is prominent. Both 22R and 22S epimers are present for the C(30) acid, whereas the others are dominated by the sidechain 22R-configuration. Thermochemolysis in the presence of TMAH was the most efficient in releasing kerogen-bound hopanoids. Following pyrolysis, the acids are generated and released into the free fraction with apparent epimerization occurring at C-17, C-21, and C-22. The bound hopanoic acids may be both chemically bonded as well as possibly being physically encapsulated within the macromolecular fraction of sedimentary organic matter. They are therefore either generated by breaking the bonds which bind them to the kerogen or they are released as a result of the macromolecular cage being broken apart.

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The oyster mushroom (Pleurotus ostreatus) is widely cultivated on wheat straw (Triticum aestivum); however, there is a need to better understand the relationship between the chemical composition of the compost and mushroom growth. Wheat straw was degraded over a period of 63 days by P. ostreatus during which time it was sampled at weekly intervals. Off-line thermochemolysis with tetramethylammonium hydroxide and solid-state (13)C NMR were then used in the molecular characterization of the undegraded wheat straw and the degraded samples. The degraded wheat straw samples had a lower proportion of syringyl- to guaiacyl-derived moieties and cinnamyl- to guaiacyl-derived moieties than the undegraded control. There were increases in both guaiacyl and syringyl acid to aldehyde ratios with composting time, which showed that side-chain oxidation has been mediated by P. ostreatus. The (13)C NMR spectra confirmed the increase in carboxyl content but indicated that the overall lignin and methoxyl contents remained relatively constant, although some nonsystematic variations were observed. The spectra also showed a decrease in amorphous noncellulosic polysaccharides in relation to the crystalline cellulose upon degradation.

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AIMS: To evaluate the short and long term morbidity of gastrostomy insertion, and to identify ongoing management requirements. METHODS: A retrospective review was undertaken of the hospital casenotes of children aged up to fifteen years who had a gastrostomy placed in Christchurch over a six year period to March 1998. RESULTS: 42 children had a gastrostomy fashioned, 35 in the last three years of the period reviewed. The most common underlying diagnosis was neurological disease (48%), and the most common indication for tube placement was failure to feed orally. Complications were frequent but minor. Morbidity was related to local erythema and infection around the stoma (85 episodes in 23 children), persistent and major gastric fluid leakage (three episodes), and mechanical failure of the tube (21 episodes). Gastro-oesophageal reflux was seen in fourteen children, nine of whom had primary neurological disease. Complications were seen more after open gastrostomy than after percutaneous endoscopic placement (6.6:4.7). There was no mortality related directly to the gastrostomy tube or tube placement. CONCLUSIONS: An increase in the frequency of gastrostomy placements has been seen over this period. As the number of children with a gastrostomy increases, so too have the demands on medical and nursing staff to care for and manage the devices. The frequency of minor ongoing problems necessitates ongoing support of the child and care of the gastrostomy. A close working relationship between outreach nursing staff, stoma therapists and medical staff is required if morbidity is to be minimised. Education, audit and review remain important additional aspects of care.

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AIMS: To determine the prevalence of coeliac disease in selected groups of children presenting to a paediatric department. METHODS: Children presenting to the Paediatric Department at Christchurch Hospital were enrolled upon identification of one or more factors associated with increased risk of coeliac disease. All subjects were screened with anti-endomysial antibody and antigliadin antibody tests. Those children with positive tests underwent small bowel biopsy. RESULTS: 36 of 153 children had abnormal antibody tests. Seven (4.5%) of 34 children who underwent small bowel biopsies were found to have histological findings consistent with coeliac disease. Five of these children had presented with symptoms not classically ascribed to coeliac disease (failure to gain weight or non-specific abdominal pain). CONCLUSIONS: The possibility of coeliac disease should be considered in children with atypical symptoms and the diagnosis excluded by appropriate testing. Recognition of the variable presentations associated with coeliac disease in children is clinically relevant.

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OBJECTIVE: Postnatal investigation of mild degrees of fetal hydronephrosis has allowed subsequent detection of infants with vesicoureteric reflux (VUR). This study was designed to provide short to medium term information on such infants who had primary VUR, the rates of renal damage and progression over time, the risk factors for such damage and to compare the characteristics of those who had mild dilatation of the fetal renal pelvis (4-9 mm) with those who had moderate-severe dilatation (> or = 10 mm). METHODOLOGY: Since June 1989, infants whose antenatal sonography had identified a fetal renal pelvis with an anteroposterior diameter of > 4 mm were investigated postnatally with renal ultrasonography and micturating cystourethrogram (MCU), and placed on antimicrobial prophylaxis. Those with VUR received 99mTc-dimercaptosuccinic acid (DMSA) scintigraphy. Infants were followed until discharge based on resolution of VUR, surgery, or low grade VUR. A 5.5 year cohort between June 1989 and December 1994 formed the study population. A review of notes and clinical review (if still under follow up) was undertaken. Vesicoureteric reflux on MCU was regraded according to the International Classification, and reflux nephropathy on DMSA scans was regraded according to criteria proposed by Goldraich. Regression analysis was used to assess risk factors for renal damage. RESULTS: There were 69 infants (37 girls, 32 boys) who were identified with primary VUR, with 37/69 having bilateral reflux. Eight had a urinary tract infection during the follow-up period. There was a broad distribution of grades of reflux detected (Grades I-3, Grades II-23, Grades III-19, Grades IV - 17, Grades V-7). 99m-Tc-dimercaptosuccinic acid scans on 57/69 (83%) demonstrated renal damage in eight infants (14%). This was predominantly global contraction of function. No progression of renal damage was seen over 2-7 years. Regression analysis showed a strong association between Grades IV, V reflux and the presence of renal damage (P < 0.001). Review of the degrees of fetal renal pelvic dilatation showed that 60/69 infants were detected because of mild (4-9 mm) dilatation. The majority (43/60) had lower grades of reflux (Grades I, II, 3), but there was no obvious cut-off between 4 and 9 mm that could predict high grade VUR (Grades IV, V). CONCLUSIONS: The use of 4 mm to define an abnormal fetal renal pelvis allows a much larger group of infants with high grade primary VUR to be detected than if a higher cut-off measurement is used. Although it also detects many more infants with low grade primary VUR, there is no obvious cut-off point at which this effect predominates. Progressive renal damage was not seen in follow up of up to 7 years of age. Renal damage on DMSA scanning in this group is almost exclusively a pattern of global contraction of function. The presence of high-grade VUR appears to be the only important factor in predicting the presence of renal damage.

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OBJECTIVE: During a study of infants who were being examined for antenatal renal dilatation, we noted that many had growth lines in their proximal femur on postnatal radiographs. We decided to determine the prevalence of growth lines in healthy infants. MATERIALS AND METHODS: Voiding cystourethrograms of 791 neonates and infants, 0-6 months old, were reviewed. All who had documented severe illness or no reasonable view of the proximal femur were excluded, resulting in a cohort of 633 healthy infants. Each study was coded for presence or absence of a growth line in the proximal femur, and the distance of the growth line from the metaphyseal edge was measured to the nearest 0.5 mm. Radiographs were obtained on a unit with fixed tube-film distance. Data on the mode of delivery were collected for 136 infants. RESULTS: Of 633 eligible infants, 247 (39%) of 633 had a discernible growth line. The distance of the growth line from the metaphysis, in millimeters, correlated significantly with age in days (r = .81, p < .01). Infants delivered vaginally were more likely to have a growth line than were those born by cesarean delivery (p = .049). CONCLUSION: A growth line in the proximal femur is common in healthy infants. The rate of longitudinal growth of the proximal femoral metaphysis, on radiographs, is approximately 1 mm per 11 days (1 mm per 13 days when corrected for magnification). Approximation of the timing of prenatal and postnatal stressful events that result in a growth disturbance line may be possible.

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Short bowel syndrome can present many complex management issues and may be complicated by various metabolic problems. D-lactic acidosis in the setting of short bowel syndrome has been described only rarely in children. A further case is presented with a review of typical clinical manifestations of D-lactic acidosis and reported management options. Early recognition and appropriate management is essential to avoid morbidity secondary to this complication of short bowel syndrome. Probiotic therapies may have an increasing role in prevention and management of this complication.

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Coeliac disease and inflammatory bowel disease (IBD) individually are not uncommon in children, but the occurrence of both conditions together is rare. The combined presentation of coeliac disease and IBD in a girl of 7 years is presented with a review of the related literature. The occurrence of coeliac disease with IBD should be considered at the time of diagnosis and at relapse, or where there is difficulty maintaining remission in established IBD. Screening with serum antibody tests may be helpful.

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There has been a low yield of primary vesicoureteric reflux (VUR) from screening the fetal urinary tract during obstetric sonography. We sought to determine whether changing the cut-off level of fetal renal pelvic diameter from 10 mm to 4 mm would improve the yield of VUR. In a prospective community-based study, a fetal renal pelvic diameter of 4 mm or more on a transverse view of the fetal renal hilum at obstetric sonography after 16 weeks' gestation was found in 426 fetuses from 9,800 consecutive pregnancies. After birth, renal sonography was performed on 386 of the 426 babies. Of the 386 babies, 264 (187 boys) had a voiding cystourethrogram (VCUG) at a mean age of 9 weeks. Primary VUR was detected in 33 (16 boys) of the 264 infants (13%), and secondary VUR in another 5 (2%). Only 5 of the 33 (15%) babies with primary VUR would have been detected if a cut-off point of 10 mm for fetal renal pelvic diameter had been used. The prevalence of reflux was similar at each cut-off level of antenatal renal pelvic diameter from 4 to 10 mm. Neither calyceal nor ureteric dilatation was helpful in differentiating those with from those without VUR. The postnatal renal sonogram did not distinguish whether reflux was present or not. More infants with primary VUR, particularly girls, were found by changing the cut-off point for fetal renal pelvic diameter from 10 mm to 4 mm, and performing a VCUG on all such infants even if the postnatal renal sonogram was normal. Of the 33 infants with primary VUR, 9 (27%, 5 boys) had an abnormal dimercaptosuccinic acid scan. Our findings support the screening of the obstetric population for a fetal renal pelvic diameter of 4 mm or more, and then investigating the infants for VUR after birth.

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AIM: To review the children who presented to Christchurch Hospital emergency department with aerodigestive tract foreign bodies during 1994. METHOD: The emergency department, and impatient notes were retrospectively reviewed for all children presenting with a complaint of an aspirated or ingested foreign body, for the period 1 January 1994 to 31 December 1994. One hundred and thirty-nine children were identified as being eligible for study. A telephone followup interview to determine outcome was also conducted. RESULTS: The 139 children had a median age of 3 years, 2 months and an even gender-distribution. The foreign bodies implicated were 47 coins, 23 sharp objects, 4 button batteries and a wide variety of blunt, noncorrosive foreign bodies. Twelve of the foreign bodies required removal (5 oesophagoscopies, 4 bronchoscopies, 2 indirect laryngoscopies and 1 laparotomy). Forty-five of 139 children had no significant symptoms at any time and these children had a total of 43 x-rays and 5 followup visits, none of which changed management. No child assessed by followup interviews had suffered complications requiring further evaluation or treatment. CONCLUSION: Children who have ingested blunt, noncorrosive foreign bodies, and who have had no significant symptoms at anytime require no investigations after exclusion of oesophageal impaction but should be reviewed if symptoms develop.

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AIM: To assess the value of plasma assays of insulin like growth factor (IGF-1) and insulin like growth factor binding protein 3 (IGFBP-3) in the diagnosis of growth hormone disorders in children and adults. METHODS: Plasma IGF-1 and IGFBP-3 were measured in 47 children referred for the assessment of short stature, 26 adult subjects with hypopituitarism and in 10 adult subjects with acromegaly. Findings were compared with results obtained in 148 normal children and 124 normal adult subjects who comprised the reference range. RESULTS: Levels of both growth factors and especially IGF-1 are highly age dependent in normal children and adults. Six of 47 short children had growth hormone deficiency and in these cases both IGF-1 and IGFBP-3 were close to the lower limit or below the normal reference range. In young children ( < 10 yr) IGFBP-3 was more informative than IGF-1, distinguishing normal short children from those with growth hormone deficiency. IGF-1 levels were raised in all 10 acromegalic adults, eight of whom had normal levels of IGFBP-3. Similarly growth hormone deficient adults were better identified (23 of 26 patients) by IGF-1 whereas IGFBP-3 was subnormal in only eight cases. CONCLUSIONS: Provided results are reviewed in relation to an age related normal reference range, both IGF-1 and IGFBP-3 are simple and convenient screening tests for assessing growth hormone deficiency in children. In adults plasma IGF-1 is the diagnostic test for a disorder of growth hormone excess. Low IGF-1 in an adult with a history of pituitary disease strongly suggests the presence of growth hormone deficiency.

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Primary vesicoureteric reflux (VUR) is one of the more common genetic disorders. Little is yet known about the genetics of this potentially manageable childhood condition, which is characterised by regurgitation of urine from the bladder to the kidney. The VUR phenotype is associated with shortness of the submucosal segment of the ureter due to congenital lateral ectopia of the ureteric orifice. VUR is found in 30-50% of infants and young children with a urinary tract infection. A serious concern in families with an affected patient is that approximately one half of siblings or offspring will be affected, but up to a half of these affected siblings and offspring may be asymptomatic in childhood. If left untreated, these patients may present later in life with proteinuria, hypertension or renal failure. VUR is the commonest cause of end-stage renal failure in children, and an important cause in adults. As the kidney damage resulting from severe VUR is preventable, early detection is desirable. The techniques for clinical diagnosis are invasive and costly, reinforcing the importance of identification of a gene for VUR to facilitate genetic screening. Although family studies suggest a major dominant gene, the inheritance pattern is still a matter of debate. In rare instances, VUR occurs in association with other diseases, such as the coloboma-ureteric-renal syndrome, which is caused by a PAX2 gene mutation. In this review, we present evidence that this common disorder may be caused by mutations in the developmental pathway of which the PAX2 gene forms a part.

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AIM: To assess outcome (final height and sexual maturation), growth patterns and blood pressure in 16 children with congenital adrenal hyperplasia treated at one institution over a 30 year period. METHODS: Growth patterns and maturation were determined by retrospective review (median follow up period 14 years). Dose adjustment of corticosteroid replacement treatment, sufficient to maintain normal levels of adrenal precursor secretion, was determined using assays of urinary pregnanetriol excretion (up to 1975) or early morning measurements of plasma 17 hydroxy progesterone and plasma renin activity at intervals of 4-6 months. RESULTS: In 7 of 15 patients the growth pattern during infancy was retarded--13 not exceeding the population mean. Catchup growth as steroid dose fell with age was not usually observed. In boys, height potential was further compromised by a significant reduction in growth velocity during puberty. None of nine patients evaluated at final height had attained the target height. During the first year of life, plasma renin activity was suppressed below the reference range in six of nine infants. Despite this, and lower than normal levels of plasma renin activity in childhood, most children were normotensive. CONCLUSIONS: Avoiding salt depletion in infancy and excessive androgen secretion during childhood do not ensure normal growth patterns or normal final height. Impaired final height in congenital adrenal hyperplasia is more likely to be due to over treatment, particularly in infancy when lower doses of corticosteroids may improve height prognosis.

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AIM: Assess the pregnancies of our female renal transplant recipients and to document long term maternal and fetal outcome. METHODS: Between 7 June 1972 and 31 December 1992 112 females had at least one renal transplant. Sixty-four of these 112 women were in the reproductive age and had a functioning graft. RESULTS: Nine women had 16 pregnancies which resulted in 11 live births and three first trimester abortions. Two unplanned pregnancies were terminated. Mean age at transplantation was 17.2 yr [range 16-22.5 yr] and mean interval from transplant to pregnancy was 6.8 yr [range 1.8-9.0 yr]. Prednisone and azathioprine were used in all patients and cyclosporin in five. For seven of the successful pregnancies plasma creatinine remained < or = 0.10 mmol/L. One of these women developed allograft nephropathy 5 years after delivery and returned to dialysis 9 years later. For the other four successful pregnancies the preconception plasma creatinine was 0.12-0.14 mmol/L. The woman with two successful pregnancies had a halving of glomerular filtration rate during the second pregnancy, but it has remained stable for 15 years; one was poorly compliant with her immunosuppressive regimen and reached endstage renal failure two years after delivery; one developed cyclosporin nephrotoxicity, but 18 months later renal function was stable after a dosage reduction. Ten infants were delivered by caesarean section, four of them urgently. Three babies were preterm and five growth retarded. One died of sudden infant death syndrome at four months. All other infants developed normally. CONCLUSION: There is no contraindication to pregnancy in female transplant recipients who have stable graft function and controlled blood pressure. Management of such pregnancies should be by shared obstetrical/nephrological/paediatric care.

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OBJECTIVE: The purpose of this study was to compare the usefulness of renal sonograms obtained 6 days and 6 weeks after birth in differentiating obstruction from nonobstruction in patients with antenatal pyelocaliceal dilatation shown by sonography and to establish sonographic criteria to determine the degree of postnatal pyelocaliceal dilatation that warrants further investigation. MATERIALS AND METHODS: Criteria for an infant to enter the study were fetal pyelectasis of 4 mm or greater, two postnatal sonograms with the second showing persisting pyelectasis extending at least into the infundibula, and a voiding cystourethrogram showing normal findings. One hundred thirty kidneys in 100 infants met the study criteria. The first postnatal sonogram was obtained at a mean age of 6 days (range, 1-14 days) and the second at a mean age of 6.6 weeks (range, 3-16 weeks). The degree of pyelectasis was measured in the anteroposterior direction on the transverse postnatal sonograms. The diagnosis of obstruction was made by excretory urography in 99 infants and nephrostography in one infant. Kidneys were categorized as definitely obstructed, possibly obstructed (anatomic features of obstruction on excretory urogram but functionally not obstructed), or not obstructed. Receiver-operating-characteristic (ROC) curves based on renal pelvic diameters were plotted for both sonograms; the ability to detect definite obstruction or possible obstruction was compared for the two time periods; and optimal cutoff points were determined. RESULTS: The mean diameter of the renal pelvis was not significantly different between the sonogram obtained at 6 days and the sonogram obtained at 6 weeks for the 86 nonobstructed kidneys. For the 27 kidneys that were obstructed, the mean pelvic diameter increased from 18 mm (range, 5-54 mm) on the sonogram obtained at 6 days to 22 mm (range, 11-60 mm) on the sonogram obtained at 6 weeks. The mean pelvic diameter of 17 kidneys categorized as possibly obstructed increased from 6 mm (range, 0-11 mm) to 10 mm (range, 6-20 mm) between the first and second sonograms. The ROC curves for all sonograms obtained at 6 weeks provided cutoff points with greater sensitivity and specificity than did the curves for the sonograms obtained at 6 days. The optimal cutoff points were 6 mm for possible obstruction (sensitivity, 100%; specificity, 57%) and 11 mm for definite obstruction (sensitivity, 100%; specificity, 57%) and 11 mm for definite obstruction (sensitivity, 100%; specificity, 96%). CONCLUSION: Renal obstruction may be underestimated or missed on a renal sonogram obtained 6 days after birth. A sonogram obtained 6 weeks after birth is more specific for detecting obstruction.

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OBJECT: Evaluation of the performance of a general paediatric unit in diagnosis and treatment of infantile hypertrophic pyloric stenosis, including morbidity and measurable outcomes in relationship to changing management. METHODS: Retrospective case note audit. RESULTS: In the 11 year period 1980-91, 103 infants underwent surgical correction of pyloric stenosis, after correction of metabolic disturbance. There was a predominance of males (82.5%), but the incidence of first born infants affected was no greater than the population birth order. While the clinical diagnosis was firm in the majority of infants, over 75% had at least one radiologic imaging procedure. On objective criteria, 80% of patients were less than 5% dehydrated, despite having electrolyte and acid-base derangement. The relatively high incidence of mucosal perforation (31.1%) did not lead to further morbidity. The incidence of postoperative vomiting (19.4%) was significantly less than in previously reported series. There were no deaths, one patient suffered longterm neurologic deficit following profound hypoglycaemia. A change in preoperative management to aggressive rehydration did not reduce morbidity, and was associated with an increased median postoperative stay. CONCLUSIONS: Pyloric stenosis remains a relatively common surgical problem of infancy. The cornerstone of diagnosis remains a clinical one; however there is a place for radiology. Operative repair is elective, following correction of metabolic derangement. In a general paediatric unit, long term sequelae of initial morbidity are rare.

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AIM: The purpose of this prospective study was to document the efficacy of ceftriaxone in the treatment of childhood bacterial meningitis in a general paediatric unit. METHODS: All children presenting with bacterial meningitis to Christchurch Hospital between January 1987 and June 1991 were enrolled in this prospective study and received ceftriaxone 100 mg/kg/d for seven days. Outcome was defined by parameters including mean time to fever defervescence, prolonged fever, days in hospital, seizures, and other acute neurological sequelae, requirement for ventilation, mortality and morbidity. Audiology was performed at six weeks and again at three months if abnormal. Neurodevelopmental assessment was performed at three months. Side effects were recorded. RESULTS: There were 62 evaluable children. The mortality rate was 4.8% (3 children). Two children (3.4%) had clinically detectable neurological sequelae at the three month assessment. The mean duration of stay was 8.7 nights. Five children (8%) required ventilation. Mild self limiting diarrhoea occurred in 29%. CONCLUSIONS: Ceftriaxone is an effective, safe and well tolerated antimicrobial for the treatment of childhood meningitis. It compares favourably with other equipotent antimicrobials. With a relatively long half life once daily administration is possible with a cost advantage.

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7B2 is a neuroendocrine polypeptide of unknown function, the gene for which is sited near or within the chromosomal region deleted in Prader-Willi syndrome and Angelman's syndrome. Plasma immunoreactive 7B2 levels were measured in 26 individuals with Prader-Willi syndrome, and appropriate controls. Plasma 7B2 levels were within normal limits compared to the control groups, in adults with Prader-Willi syndrome. 7B2 levels in children with Prader-Willi syndrome were higher, this age-dependent variation having been previously reported in normal children.

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AIM: To evaluate ondansetron as the sole antiemetic in children treated with emetogenic chemotherapy and irradiation. METHODS: Fifteen children aged 3-11 years were studied. Seven had acute lymphoblastic leukaemia, two acute myeloid leukaemia, two lymphoma and four had other tumours. Ondansetron 5 mg/m2 IV or 4 mg by mouth was given immediately before chemotherapy or radiation treatment and continued eight hourly for 24 hours. Nausea and vomiting was assessed during treatment and for the next 48 hours, and graded using WHO criteria. RESULTS: Thirty-eight courses of chemotherapy were assessed, 27 severely emetogenic and 11 moderately emetogenic. Two included total body irradiation. The most severe nausea and vomiting was grade 2 (transient vomiting) reported in six children. Nausea and vomiting was abolished on subsequent courses in four of these children by increasing the ondansetron dose frequency to six hourly. The remaining children experienced no nausea or vomiting (n = 7) or only nausea (n = 2). Nausea and vomiting were each completely controlled in 27 courses. CONCLUSIONS: Ondansetron is a cost effective and safe antiemetic in children receiving chemotherapy and total body irradiation, minimises weight loss on treatment and enables outpatient chemotherapy in some cases.

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