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     

Contralateral pleural effusion during chemotherapy for tuberculous pleural effusion

A 25 year old woman developed a right pleural effusion 6 weeks after commencement of short course chemotherapy for left sided tuberculous pleural effusion. Since the patient improved following continuation of the same treatment, it is presumed to be a case of paradoxical response to anti-tuberculosis treatment.     

Laparoscopic ovarian drilling for women with anovulatory polycystic ovarian syndrome: a district general hospital experience

This is a retrospective study involving 100 anovulatory women with polycystic ovarian syndrome (PCOS) who had laparoscopic ovarian drilling (LOD) between January 1995 and May 2002 at the Royal Berkshire Hospital, Reading, a large district general hospital (DGH). The aim was to evaluate the efficacy of LOD in the treatment of women with anovulatory PCOS in a DGH setting. We also looked at the factors predicting the clinical outcome to be able to counsel the patients pre-operatively. The study showed that the spontaneous pregnancy rate after LOD was 32.46%. A further 28.5% conceived after induction of ovulation with clomiphene citrate (CC) or purified follicular stimulating hormone (Metrodin HP), with a cumulative pregnancy rate of 74%. We did not find a significant difference in the luteinising hormone to follicular stimulating hormone (LH:FSH) ratio of greater than 2.5, LH level of greater than 10 IU/l, body mass index (BMI), age or duration of infertility between the group of women who conceived and those who failed to conceive, in response to LOD.     

Predicting cubitus varus in supracondylar fractures of the humerus by Baumann's angles in post reduction X-rays

Objectives: The present study presents the technique to predict cubitus varus by post reduction Affected Side and Normal Side Baumann's angle difference (ASBA and NSBA) respectively. It intends to correlate the Baumann's angle to the final carrying angle of the injured elbow and presents the relevant mathematical clinical rule along with its prediction test characteristics. Material and Methods: Total 57 patients of 6.5+/-1.67yrs, 22 were males and 8 females with 19/30 having left side injury. Isolated closed supracondylar fractures of humerus up to 5 days duration included and previous trauma, pathological fracture, other injury, elbow disease were excluded .30/57 completed >1 year follow-up. Results: The Mean NSBA was 74.4+/-4.14 masculine. The mean normal side carrying angles (NSCA) were 9.56 +/- 2.2 masculine. The NSCA IQR (Inter Quartile Range) was 8.8-10 masculine. The ASBA was 79.9+/-9.1 masculine and affected side carrying angles (ASCA) was 0.20+/-8.7 masculine. The ASCA was best predicted by the difference between ASBA-NSBA (ASCA=3.87-0.65(ASBA- NSBA; F=15.91). At a cut off of 8.8 masculine (the lower limit of IQR for NSCA), a value >0 masculine for ASBA- NSBA was 80% predictive of cubitus varus. With pre test probability of varus at 70%, sensitivity was 0.94 and specificity 0.42. Discussion: A prediction rule to predict the final carrying angle from ASBA NSBA difference is presented with a positive predictive value 0.80, specificity of 0.42, and sensitivity of 0.94 at a pre test probability of 0.70.When the diagnosis of cubitus varus is ASCA<8.8 masculine (Lower limit of the IQR for NSCA). Conclusion: If affected side Baumann's Angle - Normal Side Baumann's Angle is equal to or greater than 0 then there was 80% probability of having cubitus varus. Key words: Supracondylar fractures of humerus, Baumann's angle, Complications, Carrying angle, Cubitus Varus.     

Osteogenic pulmonary metastases originating from a phyllodes tumour of the breast with osteosarcomatous differentiation

Malignant phyllodes tumour is an uncommon breast neoplasm which undergoes osteosarcomatous differentiation in 1.3% of cases. We document the plain film, CT and scintigraphic features of a case of pulmonary metastases from this rare primary tumour.     

Taking Telemedicine to the Next Level in Diabetes Population Management: a Review of the Endo ECHO Model

Worldwide increases in diabetes prevalence in the face of limited medical resources have prompted international interest in innovative healthcare delivery models. Project ECHO (Extension for Community Healthcare Outcomes) is a “telementoring” program which has been shown to increase capacity for complex disease management in medically underserved regions. In contrast to a traditional telemedicine model which might connect a specialist with one patient, the ECHO model allows for multiple patients to benefit simultaneously by building new expertise. We recently applied the ECHO model to improve health outcomes of patients with complex diabetes (Endo ECHO) living in rural New Mexico. We describe the design of the Endo ECHO intervention and a 4-year, prospective program evaluation assessing health outcomes, utilization patterns, and cost-effectiveness. The Endo ECHO evaluation will demonstrate whether and to what extent this intervention improves outcomes for patients with complex diabetes living in rural New Mexico, and will serve as proof-of-concept for academic medical centers wishing to replicate the model in underserved regions around the world.