A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned

Mitochondrial diseases have a special predilection to involve the brain in view of its high metabolic demand and the tendency for the formation of excitatory neurotransmitters when there is deficiency of intracellular ATP. These diseases have a great phenotypic variation and need a high degree of suspicion. However, some specific syndromes are well defined, both genotypically and phenotypically. Some of the drugs are potentially fatal mitochondrial poisons and an insight into that may be lifesaving as well as prevent serious morbidities. We report a typical case of myoclonic epilepsy with ragged red fibers (MERRF) with classical phenotype and genotype. There was rapid multiaxial deterioration with the introduction of sodium valproate which partly reversed on introducing mitochondrial cocktail and withdrawal of the offending drug. Sodium valproate, phenobarbitone, chloramphenicol and many anti-viral agents are mitochondrial poisons that increase the morbidity and mortality in patients with mitochondrial disease. More harm to the patient can be avoided with insight into this information.KEY WORDS: Midline lipoma, mitochondrial disease, sodium valproate     

Preoperative determination of tibial nail length: An anthropometric study

Objective: To assess the correlation between five anthropometric parameters and the distance from tibial tuberosity to medial malleolus in 100 volunteers. Methods: Six anthropometric parameters were measured in 50 male and 50 female medical students using a metallic scale: medial knee joint line to ankle joint line (K-A), medial knee joint line to medial malleolus (K-MM), tibial tuberosity to ankle joint (TT-A), tibial tuberosity to medial malleolus (TT- MM), olecranon to 5th metacarpal head (O-MH) and body height (BH). Nail size predicted based upon TT-MM measurement was chosen as ideal nail size. A constant was derived for each of the six anthropometric parameters which was either added or subtracted to each measurement to derive nail size. A regression equation was applied to BH measurements. Nail sizes calculated were compared with that obtained from TT-MM measurement and accuracy was evaluated. Accuracy of O-MH and BH regression equations recommended by other authors were calculated in our data. Results: Adding 11 mm to TT-A distance had highest accuracy (81%) and correlation (0.966) in predicting nails correctly. Subtracting 33 mm from K-MM measurement and 25 mm from K-A distance derived accurate sizes in 69% and 76% respectively. Adding 6 mm to O-MH distance had a poor accuracy of 51%. Nail size prediction based upon body height regression equation derived correct nail sizes in only 34% of the cases. Regression equation analysis by other authors based on O-MH and BH distances yielded correct sizes in 11% and 5% of the cases respectively. Conclusion: TT-A, K-A and K-MM measurements can be used simultaneously to increase accuracy of nail size prediction. This method would be helpful in determining nail size preoperatively especially when one anatomic landmark is difficult to palpate.     

Medical response of a physician and two nurses to the mass-casualty event resulting in the Phi Phi Islands from the tsunami

The Phi Phi Islands are isolated islands located about one hour by ship from the mainland in Krabi province of Thailand. There is a small medical facility where the director is the one physician that provides care to residents and tourists. This small medical facility faced an enormous mass casualty incident due to the 2004 Tsunami. The hospital was damaged by the Tsunami wave and was not functional, one crew member died and another was injured. Medical care and evacuation posed a unique problem in the Phi Phi Islands due to remoteness, limited medical resources, lack of effective communication with the main land and the large number of victims. An alternative medical facility was located in a nearby hotel. The crew included the medical director, two nurses, two additional staff members, 10 local volunteers, and hotel staff members. The medical crew had to treat 600-700 casualties in 24 hours. Most of the victims were mildly injured, but approximately 100 (15%) of the victims could not walk due to their injuries. The medical director, made a conscious decision to initially treat only circulation ("C") problems, by controlling external hemorrhages. This decision was driven by the lack of equipment and personnel to deal with airway ("A") and breathing ("B") problems. In the post-disaster debriefing, the Phi Phi Island hospital physician noted five major lessons concerning disaster management in such extreme situation in a small facility located in a remote area: (1) effective resistant communication facilities must be ensured; (2) clear, simple "evacuation plans" should be made in advance; (3) plans should be made to ensure automatic reinforcement of remote areas with evacuation vehicles, medical equipment and medical personnel; (4) efficient cooperation with medical volunteers must be planned and drilled; and (5) every team member of such a hospital must participate in an educational program and periodic drills should be done to improve the disaster and emergency medicine capabilities. This case report is an example for caregivers all over the world, of an amazing lesson of leadership and courage.