Novel measles virus genotype,East Timor and Australia

Measles outbreaks in 1999 in Queensland and Victoria, Australia, were caused by a novel strain of clade G virus (proposed name g3). Epidemiologic and molecular evidence supports independent circulation of this virus in Queensland, northern Australia, in addition to importation of the virus by East Timor refugees seeking safe haven in Australia.     

Childhood medulloblastoma in northwest England 1954 to 1997: incidence and survival

The incidence, survival patterns, and presenting symptoms of children with medulloblastoma were studied. Data were ascertained from the Manchester Tumour Registry which is population-based and has collected data on all childhood malignancies in northwest England since 1954. Incidence rates standardized to the European standard population were calculated and Poisson regression models were used to examine temporal changes in the incidence rates during the period 1954 to 1997. Kaplan-Meier survival curves were derived and used to study changes in survival patterns. World-standardized incidence rates were 5.5 per million child years in males and 3.4 per million child years in females. Incidence rates increased from the 1950s to the 1980s but have declined recently. The 5-year survival rate has improved from 29 to 58% with similar rates for males and females. The 1-year survival rate has also improved, but females had worse survival at this point (58%) than males (75%). The type of symptom or sign at presentation is strongly affected by age, with 10 of the 22 recorded symptoms or signs showing significant age differences. The older the child is, the more likely is the presentation to show pressure features of headache, vomiting, and ophthalmic signs. Younger children present with non-specific features such as lethargy, behavioural disturbance, or increasing head size. Ataxia is seen in about 75% of children across the age range.     

Family Influences: Mothers' and Daughters' Use of Multivitamin Mineral Supplements

Girls' multivitamin mineral (MVM) use was evaluated among a white middle-class sample (n = 192 mother-daughter pairs). Daughters' MVM use was predicted by mothers' use by mothers' beliefs attitudes practices regarding her own eating child feeding practices. Mothers who gave daughters supplements reported greater monitoring of daughters' intake were more likely to pressure daughters to eat. MVM user diets did not differ from nonusers with the exception that nonusers consumed more fats sweets. Patterns of intake revealed food group servings below recommended levels for both groups whereas vitamin mineral intakes exceeded the RDA. This pattern reflects girls' high levels of consumption of fortified foods.     

Neurofibromatosis type 1 and sporadic optic gliomas.

AIMS: To compare the natural history of sporadic optic glioma with those associated with neurofibromatosis type 1 (NF1). METHODS: Optic glioma cases were identified using both the Manchester Children's Tumour Registry (CTR) and the North West Regional NF1 Database (NF1DB), with detailed information on natural history available from the former (in 34 of 36 cases identified). RESULTS: A total of 52 cases over a period of 41 years were identified. From the 34 whose natural history was known, almost all (n = 31) were symptomatic, with mean ages of presentation of 4.5 and 5.1 years for NF1 and sporadic cases respectively. The majority (n = 22) presented with visual impairment, seven of whom were blind in at least one eye. Sporadic cases were over twice as likely as NF1 to have visual impairment. Recurrence occurred in 12 patients. Fewer NF1 patients died as a direct result of their optic glioma, but overall mortality and 5 and 10 year survival rates between the two groups were similar. All five primary (non-metastatic) second central nervous system (CNS) tumours occurred in NF1 cases, two of these following radiotherapy. CONCLUSIONS: Symptomatic sporadic optic gliomas presented with impaired vision more frequently and were more aggressive than NF1 optic gliomas. Only optic glioma cases with NF1 were at risk of developing a second CNS tumour. Aggressive treatment of sporadic optic gliomas and early surveillance of NF1 optic gliomas may be required. The use of radiotherapy in these children requires further clarification.     

Multiple subpial transection in Landau-Kleffner syndrome

We have considered multiple subpial transection (MST) as a treatment option for Landau-Kleffner syndrome (LKS) for the past 6 years. The effect of this technique on language and cognitive ability, behaviour, seizures, and EEG abnormalities is analysed here. Five children (4 males, 1 female; aged 5.5 to 10 years) underwent MST with sufficiently detailed pre- and postoperative data for analysis. Behaviour and seizure frequency improved dramatically after surgery in all children. Improvement in language also occurred in all children, although none improved to an age-appropriate level. All five had electrical status epilepticus in sleep (ESES) before surgery, which was eliminated by the procedure. One child has had an extension of his MST due to the recurrence of ESES and accompanying clinical deterioration with good effect. An attempt is made to set the effect of MST against the natural history of the condition. MST is an important treatment modality in LKS, although the timing of this intervention and its effect on final language outcome remains to be defined.     

Octylcyanoacrylate tissue adhesive as an alternative to mechanical fixation of expanded polytetrafluoroethylene prosthesis

In minimally invasive incisional hernia repair positioning and fixation of the expanded polytetrafluoroethylene (ePTFE) mesh prosthesis on the deep surface of the abdominal wall may be facilitated using tissue adhesives. Octylcyanoacrylate (OCTYL), a new adhesive, forms a strong flexible bond with antimicrobial properties. In a rabbit model for incisional hernia we investigated characteristics of the bond created by OCTYL between ePTFE and abdominal wall musculature. We studied initial bond strength and the postoperative host response to the adhesive over a 6-week period. We compared sutured, stapled, and glued mesh prostheses and examined the tissue-prosthesis interface. The ePTFE mesh was fixed successfully to the abdominal wall with OCTYL and remained tightly attached at 6 weeks. Prostheses fixed with OCTYL and spiral tacks induced few intra-abdominal adhesions compared with sutured mesh. All prostheses were completely reperitonealized at 2 weeks. The force required to displace mesh fixed with sutures and staples was greater than mesh fixed with OCTYL. Analysis of the ePTFE/tissue interface by light and scanning electron microscopy showed host cellular migration into the interstices of the mesh with fixation by tacks and suture, whereas an inflammatory infiltrate was seen on the muscular surface with OCTYL fixation of the mesh.     

Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): “SMD-Corner Fracture Type” (DCV/SMD-CF) demonstrated in most reported cases

Background. This paper reports three children with short stature: developmental coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and “ovoid” vertebral bodies in the first patient, and normal to slightly tall vertebral bodies in the third; metaphyseal changes in some long tubular bones including bone fragments similar to the corner fractures seen in child abuse in all three patients.¶Materials and methods. The first and second patients were sisters; their mother, also quite short, had surgical procedures in early life for bilateral “coxa vara”; their brother, also of short stature, had bilateral coxa valga with otherwise normal femoral heads and necks, and mild metaphyseal changes associated with two minute “corner fractures” in the proximal metaphysis of the left tibia.¶Results. A review of reported cases of developmental coxa vara associated with spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances.     

Congenital anomalies and genetic syndromes in 173 cases of medulloblastoma

One hundred seventy-three consecutive cases of medulloblastoma recorded in the Manchester Children's Tumour Registry from 1954 to 1989 were studied. After review of case notes, X-rays, and health surveys the clinical outcome and incidence of congenital anomaly was determined. A previously unreported association with Rubinstein Taybi syndrome was found. Evidence of a genetic syndrome or congenital anomaly was found in 6.4%. These figures provide further evidence of the higher-than-expected incidence of congenital abnormalities. © 1993 Wiley-Liss, Inc.     

Multiple primary tumours in a population-based series of patients with histopathologically peer-reviewed sarcomas.

Multiple primary tumours occurring in a three-year population-based series of patients with histopathologically peer-reviewed sarcomas from North West England were ascertained in order to look at the patterns of neoplasms seen. A total of 30 out of the 310 patients entered in the study had additional primary tumours. Very few patients were aged under 60 years at diagnosis of both their malignancies. The youngest was a known case of neurofibromatosis and, although seven patients were diagnosed with a sarcoma and carcinoma of the breast--a combination of cancers characteristic of the Li-Fraumeni cancer family syndrome--no other patients could directly be identified as suffering from any other cancer predisposition syndrome.