Benign childhood epilepsy with centrotemporal spikes: is it always benign?

Most children with benign childhood epilepsy with centrotemporal spikes have few seizures, and some have only one. We describe two children with interictal and ictal findings consistent with this epileptic syndrome but with severe intractable seizures and cognitive decline that resulted in consideration for epilepsy surgery. Spontaneous remission occured in one child; the other is still young. Despite the high seizure burden and cognitive decline, surgical consideration should be withheld, as these seizures are likely to remit.     

Cutaneous Rosai-Dorfman disease--a pathologic review of 2 cases

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare but distinct clinicopathologic entity characterised histologically by a benign s histiocytic proliferation. Isolated involvement of extranodal sites without concomitant nodal disease is rare. We describe the pathological features of 2 cases of Rosai-Dorfman disease that were clinically confined to the skin. In both male adult Chinese patients, proliferation of histiocytes was accompanied by S-100 protein expression demonstrated immunohistochemically within the histiocytes. The pathology of Rosai-Dorfman disease and its microscopic differential diagnoses are discussed.     

Randomised controlled trial of zinc supplementation in malnourished Bangladeshi children with acute diarrhoea

OBJECTIVE: To evaluate the impact of zinc supplementation on the clinical course, stool weight, duration of diarrhoea, changes in serum zinc, and body weight gain of children with acute diarrhoea. DESIGN: Randomised double blind controlled trial. Children were assigned to receive zinc (20 mg elemental zinc per day) containing multivitamins or control group (zinc-free multivitamins) daily in three divided doses for two weeks. SETTING: A diarrhoeal disease hospital in Dhaka, Bangladesh. PATIENTS: 111 children, 3 to 24 months old, below 76% median weight for age of the National Center for Health Statistics standard with acute diarrhoea. Children with severe infection and/or oedema were excluded. MAIN OUTCOME MEASURES: Total diarrhoeal stool output, duration of diarrhoea, rate of weight gain, and changes in serum zinc levels after supplementation. RESULTS: Stool output was 28% less and duration 14% shorter in the zinc supplemented group than placebo (p = 0.06). There were reductions in median total diarrhoeal stool output among zinc supplemented subjects who were shorter (less than 95% height for age), 239 v 326 g/kg (p < 0.04), and who had a lower initial serum zinc (< 14 mmol/l), 279 v 329 g/kg (p < 0.05); a shortening of mean time to recovery occurred (4.7 v 6.2 days, p < 0.04) in those with lower serum zinc. There was an increase in mean serum zinc in the zinc supplemented group (+2.4 v -0.3 mumol/l, p < 0.001) during two weeks of supplementation, and better mean weight gain (120 v 30 g, p < 0.03) at the time of discharge from hospital. CONCLUSIONS: Zinc supplementation is a simple, acceptable, and affordable strategy which should be considered in the management of acute diarrhoea and in prevention of growth faltering in children specially those who are malnourished.     

Central motor conduction time in malnourished children

The functional status of the descending motor pathways was assessed in malnourished children using transcranial electromagnetic stimulation of the cortex. Twenty children with different severities of malnutrition and 20 control subjects were studied electrophysiologically. The circular coil of a Dentac MAG 2 magnetic stimulator was applied tangentially over the vertex to stimulate the cortex. The muscle evoked potential in the children's arms was recorded from the abductor pollicis brevis muscle and in their legs from the extensor digitorum brevis muscle on both sides of the body using surface electrodes. The muscle evoked potential of the abductor pollicis brevis and extensor digitorum brevis muscles was further obtained using root stimulation by applying the coil at the cervical and lumbar spine, respectively. The indices of cortical threshold, cortical latency, and central motor conduction time (ms) were evaluated in both arm and leg muscles on both sides. The results showed an increased cortical threshold (mean (SD) 1232.5 (134.06) in the study group v 1147.5 (99.31) in the control group) for the abductor pollicis brevis muscle and for the extensor digitorum brevis muscle (1325.00 (115.70) in the study group v 1190.0 (125.24) in the control group). Similarly, significant prolongation of the central motor conduction time (ms) (study group 6.67 (0.91) v control group 5.71 (0.74)) in the abductor pollicis brevis muscle was seen in malnourished children.     

Focal nodular hyperplasia: an unusual liver mass in a paediatric patient

We report an unusual case of focal nodular hyperplasia (FNH) occurring in a 19-month-old female without a typical central fibrous scar. Ultrasound demonstrated a solid, hypoechoic, highly vascular mass situated in the left lobe of the liver. Computed tomography showed a solid mass with no evidence of a central fibrous scar. A hepatic angiogram demonstrated enlarged right and left hepatic arteries supplying the vascular tumour, with early venous drainage into the inferior vena cava; a feature which has not been previously described. Surgical resection was carried out and a solid, nodular tumour measuring 9 x 4 x 4 cm was removed. The diagnosis of FNH was made histologically. The characteristic imaging findings of FNH will be discussed and a review of the literature of FNH in children will be presented.     

Does a single plasma phenylalanine predict quality of control in phenylketonuria?

A 1993 MRC working group on phenylketonuria suggested standardising blood phenylalanine measurements by taking blood samples at the same time each day. Since it is not known how representative of a 24 hour period a single phenylalanine concentration is, the aim of this study was to investigate the 24 hour variability of plasma phenylalanine in well controlled children with phenylketonuria. Sixteen subjects, 12 girls and four boys aged 1 to 18 years, had hourly venous blood samples collected for 13 hours between 09.00 and 21.00 on one day. Serial skin puncture blood specimens were then collected at 24.00, 03.00, and 06.00 within the same 24 hour period. All food and drink was weighed. The median variation in plasma phenylalanine concentration was 155 mumol/l/day, with a minimum of 80 and a maximum of 280. The highest concentration occurred in the morning between 6.00 and 9.00 in 63% of subjects; the lowest occurred between midday and midnight in 94%. Concentrations < 100 mumol/l occurred in 46% of children below 11 years, three having concentrations < 30 mumol/l for two, six, and seven hours respectively. Three of five subjects had concentrations above the MRC guidelines for 24% of the period studied. Except in two subjects, the blood concentrations did not rise in response to phenylalanine consumption. However, the greater the quantity of protein substitute taken between waking and the 16.00 specimen, the larger the decrease in daytime phenylalanine concentration (r = -0.7030) (p < 0.005). There is therefore wide variability in phenylalanine concentrations in a 24 hour period in children with phenylketonuria which is not reflected in a single observation. Further study is needed to investigate the effects of timing of protein substitute on the stability of phenylalanine concentrations.     

VASCULAR EMBOLOTHERAPY-HOW MUCH HAS BEEN ACHIEVED?

Emergency and elective embolotherapy of various systemic arteries in 64 patients was carried out at a tertiary centre of Armed Forces. Specific indications were haemoptysis (n=43), preoperative (n=18), haematuria (n=1), epistaxis (n=1) and chemoembolization (n=1). The procedures were performed with gelfoam pellets (n=46), gelfoam pellets and absolute alcohol (n=1), polyvinyl alcohol particles (PVA) (n=14), steel coils (n=2) and Adriamycin-in-oil emulsion (n=1). Embolotherapy resulted in complete haemostasis in 37 (82.2%) out of 45 cases of haemorrhage. In eight cases (17.8%), it resulted in significant improvement. Complete haemostasis was achieved in both cases of haematuria and epistaxis. Pre-operative embolotherapy resulted in considerable reduction of peroperative blood loss in all the cases. Chemoembolization of Hepatocellular carcinoma resulted in partial regression of the tumour. The purpose of this study was to assess the efficacy, safety and reliability of vascular embolotherapy for control of life threatening haemorrhage and preoperative reduction of lesions.KEY WORDS: Embolization, Embolotherapy, Haemorrhage     

COSMETIC SCAR AND SCOPE OF REVERSAL AFTER PUERPERAL STERILISATION

Female sterilisation is an important component of National Family Welfare Programme. The target group is best motivated during the puerperium for such a procedure. However laparoscopic sterilisation which has got some distinct advantages, is not technically feasible at this time. The authors have used a technique where the advantages of cosmetic appearance, reduced post operative morbidity and reversibility can be conferred on the puerperal women.After trying out the method individually in some cases, a formal case control study design has been made and an evaluation study has been performed in 122 cases. The technique has been found to be cosmetically more acceptable, both at clientele and peer evaluation levels (p<0.001). Though all the four parameters of post operative morbidity have shown better results for the technique evaluated as against the conventional technique, statistical significance has been achieved in two of the parameters (p<0.05).KEY WORDS: Cosmetics, Female sterilisation, Post operative morbidity     

Delay times and management of acute myocardial infarction in indigenous and non-indigenous people in the Northern Territory

OBJECTIVES: To investigate differences in presentation and management of Indigenous and non-Indigenous patients hospitalised with acute myocardial infarction (AMI). DESIGN: Retrospective review of hospital medical records. PARTICIPANTS AND SETTING: 122 patients with definite or possible AMI admitted to hospitals in the Top End of the Northern Territory (NT) in 1996. MAIN OUTCOME MEASURES: Percentage receiving thrombolytic therapy; delays from symptom onset to primary and emergency department presentations, first and diagnostic electrocardiograms, thrombolytic therapy and aspirin; drugs prescribed during hospitalisation. RESULTS: Thrombolytic therapy was given to 12/41 Indigenous patients (29%) and 38/81 non-Indigenous patients (47%) (P = 0.06). Presentation delay over 12 hours was the reason for not giving thrombolytic therapy for 14/29 Indigenous patients (48%) and 8/43 non-Indigenous patients (19%) (P < 0.01). Median delay times were longer for Indigenous patients for all six categories of delay, although the difference was significant only for delay to emergency department presentation (10:00 versus 3:26 hours; P < 0.01) and to diagnostic electrocardiogram (8:10 versus 3:50 hours; P < 0.01). Delays were also longer for patients from rural compared with urban areas. Once diagnosed, Indigenous patients were as likely as non-Indigenous patients to receive aspirin (93% versus 96%) and beta-blockers (70% versus 69%) and more likely to receive angiotensin-converting enzyme inhibitors (60% versus 40%; P = 0.03). CONCLUSIONS: Delays in presentation affect Indigenous people living in rural and urban areas as well as non-Indigenous people living in rural areas. Concerted efforts are needed to improve health service access in rural areas and to encourage Indigenous people with persistent chest pain to present earlier.     

Towards a pain-free venepuncture

A randomised, prospective trial was conducted to assess the efficacy of various means of alleviating the pain of subcutaneous lidocaine infiltration. One hundred and twenty-two patients were randomly allocated to different groups to receive buffered lidocaine 1%, warmed lidocaine 1% or infiltration by the counter-irritation technique. A visual analogue pain score was recorded at different stages of cannulation and results showed that pain scores were significantly lower in the group receiving buffered lidocaine 1% (p < 0.02) and in the counter-irritation group (p < 0.05). Thus buffering lidocaine 1% and administration of lidocaine 1% by the counter-irritation technique is effective in relieving the pain of lidocaine infiltration.