The KBG syndrome.

We report on two boys with a combination of short stature, a broad face, macrodontia and developmental delay. These features suggest that they have the KBG syndrome, a rare but distinctive phenotype. The pattern of inheritance remains uncertain and both autosomal dominant and X-linked recessive inheritance should be considered in genetic counselling.

A fluid filtration and clearing technique to assess microleakage associated with three dentine bonding systems.

OBJECTIVES: The aims of this in vitro study were to (a) measure fluid flow through teeth restored with one of three dentine bonding systems and a resin composite restoration; (b) measure the distribution of a silver tracer through the same teeth, and make a comparison with fluid flow; and (c) investigate the effect of thermocycling on both measurement types. METHODS: Coronal segments of 30 premolars, randomly allocated to three equal groups, were assessed by fluid filtration. Each group was restored with a resin composite restoration in conjunction with Fuji Bond LC (FBLC), Scotchbond Multi-Purpose Plus (SMP+) or Prime&Bond 2.1 (P&B2.1). Fluid filtration rates were measured in the intact crown and then after cavity preparation, conditioning, dentine bonding, restoration and at 2 and 24 h, 1 week and 1 month following restoration. Six specimens from each group were thermocycled at 1 week. After final filtration measurements the specimens were perfused with silver nitrate and cleared before scoring tracer penetration. RESULTS: No significant differences (P > 0.05) in fluid filtration rates were found amongst the different bonding systems or at any restoration stage. Thermocycling was not associated with any significant (P > 0.05) increase in fluid filtration. Final fluid filtration and tracer distribution showed a weak and not statistically significant correlation (P > 0.05). The penetration of silver stain indicated a failure of the restorations to seal the cavity and demonstrated a possible pathway by which in vivo post-operative sensitivity could occur. CONCLUSIONS: Although not statistically significant, conditioning of the cavity increased the dentinal permeability but this effect was variable. Thermocycling had no statistically significant effect on microleakage.

In vitro dentinal penetration by tracers used in microleakage studies.

Many microleakage studies, in their methodology, employ different tracers which may penetrate around restorative materials to varying extents due to their physical or chemical characteristics. The present series of in vitro studies were designed to compare the ability of freshly obtained microleakage tracers to enter cut dentinal tubules. Tracers selected were 5% eosin, 2% methylene blue, 50% silver nitrate and Indian ink all buffered, where necessary, to a range of pH 6.9-7.2. The solution of Indian ink was further investigated by particle size analysis to determine the range of particle sizes within the solution. Particles ranged from < 1 micron to 600 microns. Buffered and unbuffered solutions of the tracers were applied for 1 h to open cavities, devoid of a smear layer, prepared in 42 premolars. Specimens were sectioned longitudinally through the cavities either with or without water coolant. Penetration into dentinal tubules by the tracer resulted in an area of stained dentine and this area was subsequently measured using image analysis. The results demonstrated that there were few statistically significant differences between the areas of dentinal penetration associated with each tracer. Neither the tracer used, its pH nor sectioning technique had a predictably significant effect on dentinal penetration. Indian ink was observed to be capable of entering dentinal tubules.

Peripheral nerve entrapment syndromes: diagnosis and management.

Symptoms due to nerve entrapment syndromes are a frequent reason for attendance at GP, rheumatology and orthopaedic clinics. This article outlines the presenting symptoms and signs of some of the more common syndromes and gives guidelines on the clinical diagnosis and subsequent management.

The natural history of chronic demyelinating neuropathy associated with benign IgM paraproteinaemia. A clinical and neurophysiological study.

Eighteen patients are described, all of whom had chronic demyelinating peripheral neuropathy and benign IgM paraproteinaemia. All patients had serum antibodies against peripheral nerve myelin or myelin-associated glycoprotein. Seventeen were followed up clinically and electrophysiologically for between 1 and 14 years (mean 7.4 years). The presenting symptoms and signs were almost always those of a distal sensory disturbance in the limbs followed by distal weakness. All patients developed tremor or ataxia in the arms, and gait ataxia. The severity of the neuropathy varied greatly between patients at similar stages. Some had a predominantly sensory deficit and others a predominantly motor deficit. All patients eventually developed both motor and sensory signs. The neuropathy became slowly worse over the first 2-5 years and then appeared to stabilize, although long-term follow-up did reveal a very slow progression in the group as a whole. No patient developed evidence of haematological malignancy but two patients died of malignancy involving other systems. On reviewing 75 patients from the literature, non-haematological malignancy was found to be the commonest cause of death.

Excessive wastage of blood resources in elective colorectal surgery.

A prospective study of perioperative blood transfusion requirements for elective colorectal surgery over a 6-month period has been conducted. A total of 106 procedures was performed, 94 of which were for malignancy. A median of 2 units of packed red cells was cross-matched per patient. Twenty-three patients (21.7%) required a transfusion in the postoperative period. Fourteen of these patients required a second cross-match sample because their transfusion occurred more than 48 h after the original request. The greatest transfusion requirements were for patients undergoing abdominoperineal resection or subtotal colectomy. Routine perioperative cross-matching and reservation of blood for elective colorectal surgery may be an unnecessary expense, resulting in wastage of resources. A selective policy of serum being retained for grouping in these patients is justified.

Small bowel adenocarcinoma complicating Crohn's disease.

Crohn's disease of the small intestine is usually managed by medical therapy with surgery being reserved for obstruction or fistula formation. A patient is described who developed small bowel obstruction due to an adenocarcinoma of the ileum after over twenty years of medical therapy for Crohn's disease, originally diagnosed at a laparotomy for acute abdominal pain. The possibility of malignancy in such long-standing disease should be considered.

Relationship of cranial ultrasonography, visual and auditory evoked responses with neurodevelopmental outcome.

Sixty-two preterm infants were followed up with flash visual evoked responses (VERs), brainstem auditory evoked responses (BAERs) and neurodevelopmental assessments for 18 months after term. Cranial ultrasonography showed that 18 infants developed intraventricular haemorrhage (IVH) during the newborn period and four of six infants with grade III or IV haemorrhage had absent VERs at term. The mean latency of the VER in the infants with IVH was significantly shorter than that of infants without IVH. There was no correlation between the degree of ventricular dilatation at term and the latency of the VER. Six months after term the four infants with previously absent VERs had normal responses, though there was still a significant difference between the latencies of infants with and without IVH. At 12 and 18 months of age these differences had disappeared. BAERs were not significantly different in the infants with and without IVH, and there was no difference in the VERs or BAERs of infants with neurodevelopmental delay and those developing normally. Neither flash VERs nor BAERs provide a good prognostic indicator of future neurodevelopmental disability or outcome.

Maintenance cimetidine instead of surgery for duodenal ulcer: the first decade.

Fifty five patients with severe longstanding dyspepsia originally selected as candidates for elective duodenal ulcer surgery were instead entered into a maintenance cimetidine study. Forty six have been reviewed after 10 years. Twenty patients had required surgery, all in the first three years, but six of them had proven or suspected recurrent ulceration during follow up. Twenty six patients avoided surgery, either with continuous cimetidine 400 mg nocte or intermittent full dose cimetidine. Of the 17 on continuous maintenance cimetidine, six had proven or suspected recurrent ulceration responding to full dose cimetidine. All nine patients on intermittent cimetidine experienced symptomatic relapse within eight to 12 weeks of drug withdrawal. By using cimetidine, 64% of patients selected for surgery have avoided operation over this 10 year period, although none has had drug free remission.

The compatibility of temporary soft materials with immersion denture cleansers.

This laboratory study evaluated the effect of five denture cleansers on five temporary soft materials that can be used as interim soft linings or as tissue conditioners. The materials were assessed at 3, 7, 14, and 21 days for evidence of changes in surface quality and softness. It was apparent that the correct combination of lining material and cleanser is essential to ensure the optimum function of the lining material. Since the materials used varied in softness, the appropriate choice should be based on the clinical requirement.

Effect of cimetidine on prognosis after simple closure of perforated duodenal ulcer.

A prospective controlled study in 60 patients undergoing simple closure of perforated duodenal ulcer demonstrated a significant (P less than 0.05) benefit following postoperative cimetidine treatment, in terms of avoiding subsequent dyspeptic symptoms and complications of peptic ulcer disease. Thus cimetidine is recommended in the follow-up management of perforated duodenal ulcers.

Isoflurane: effect on the encephalogram during carotid endarterectomy.

The effect of isoflurane on the electroencephalogram was studied in patients monitored during carotid endarterectomy. Increasing concentrations of this agent cause changes, but did not affect the ability to detect the onset of cerebral ischaemia consequent upon temporary occlusion of the internal carotid artery. At 1.0% and 1.5% concentration isoflurane caused significant decreases in mean arterial pressure which is undesirable in patients undergoing vascular surgery.

Chronic demyelinating neuropathy associated with benign IgM paraproteinaemia.

Twelve cases of chronic sensorimotor neuropathy associated with benign IgM paraproteinaemia are described. The onset was in the sixth or seventh decades and 9 cases were male. Tremor and ataxia were common features. The ESR was raised in 6 cases and the CSF protein content elevated in 10. There was severe reduction of motor nerve conduction velocity. Sera from all cases contained monoclonal IgM antibodies strongly reactive with human peripheral myelin, made up by the paraprotein. Histological studies on nerve biopsies taken from 8 cases all demonstrated a demyelinating neuropathy. Eight biopsies were examined by immunofluorescence. Monoclonal IgM was present on the myelin sheaths of surviving myelinated nerve fibres. The findings suggested that the neuropathy was of autoimmune origin and caused directly by the antimyelin antibody.

Neurological involvement in hereditary transcobalamin II deficiency.

A case of hereditary transcobalamin II deficiency with neurological involvement is described. The patient presented in early infancy with megaloblastic anaemia and was treated with folinic acid from 6 weeks of age. The diagnosis of transcobalamin II deficiency was not made until he was 2 years old when he showed severely retarded intellectual development, ataxia and pyramidal deficit in the limbs. Following treatment with hydroxocobalamin, his condition has slowly improved but he has remained with a severe neurological deficit. The consequences of vitamin B12 deficiency on neurological development in infancy are discussed.

Spinal somatosensory evoked potentials in hereditary spastic paraplegia.

Cervical somatosensory evoked potentials elicited by median nerve stimulation were recorded from 18 cases of hereditary spastic paraplegia. Motor and sensory nerve conduction in the median nerve was normal in each. In one third of the patients no spinal evoked potential was detectable. In the remainder the amplitude of the evoked potential was reduced in comparison with a control series; the latency was not significantly different. These changes suggest that a selective degeneration of the centrally directed axons derived from the dorsal root ganglion cells occurs in this disorder. The findings are discussed in relation to previous reports on the pathological appearances.

Long term cimetidine in the management of severe duodenal ulcer dyspepsia.

Sixty patients who had been referred for elective ulcer surgery, and in whom a remission had been induced, entered a prospective double blind controlled trial of a single daily dose of 400 mg of cimetidine given at bedtime, or placebo. Eighty per cent of patients receiving placebo suffered symptomatic relapse and recurrence of duodenal ulceration at endoscopy within 6 months. The mean interval to relapse was 10 weeks. On the other hand, only 27 percent of patients had a recurrence during the 6-month period on low dose cimetidine therapy. No significant toxic or other side effects which could be attributed to the drug were observed.