Hereditary Spherocytosis: Observations on Hemolytic Mechanisms and Iron Metabolism

1. Two healthy patients with hereditary spherocytosis were phlebotomizeduntil they developed iron deficiency and the erythrocytes became hypochromic.The hereditary spherocytes were no longer spheroidal: they became thin, andthe fragility tests improved. However, the life span of the cells in the circulation was not improved. Later, splenectomy corrected the hemolytic disease.

2. In both patients, prior to the experiment, the hemolytic disease wascompensated. There was no anemia despite the rapid turnover of red cells.In one of the patients, whose average red cell life span was only five days, theoutput of hemoglobin must have been exceedingly high. It was computed tobe 135 Gm. per day, or 20 times the normal rate.

3. Some aspects of iron metabolism in hereditary spherocytosis are discussed.

4. The shape of the red cell in HS does not appear to be responsible for itspremature destruction by the spleen. Iron deficiency corrects the spherocytosis,but it does not correct the hemolytic disease. Splenectomy corrects the hemolytic disease, but it does not correct the spherocytosis.

     

Seizure outcome after extratemporal epilepsy surgery in childhood

Aim The aim of the study was to describe seizure outcome following surgery for focal extratemporal epilepsy and identify factors associated with prolonged postsurgical freedom from seizures. Method In this retrospective cohort study, children with drug‐resistant focal extratemporal epilepsy were treated surgically and followed up in a single tertiary care centre between 1997 and 2008. Results Eighty children were identified for inclusion in the study (42 males, 38 females; median age 9y 1mo, range 3mo–18y 7mo). The aetiology was identified as focal cortical dysplasia (n=37), low‐grade tumour (n=22), tuberous sclerosis (n=9), or non‐specific (n=12). Children were followed for a median of 3 years 1 month (range 8mo–10y 7mo) after surgery. Overall, at last follow‐up, 50% of the children had been completely seizure free since surgery (Engel class Ia); of these 40 individuals, 15 had discontinued all antiepileptic drugs. Several presurgical factors were associated with a favourable outcome. However, after controlling for confounding factors, aetiology appeared to be the only determinant of long‐term seizure outcome as non‐specific lesion pathology was associated with seizure recurrence (hazard ratio 10.43; 95% confidence interval 3.26–33.39). Interpretation In 50% of cases, children with surgically treated drug‐resistant extratemporal epilepsies have an excellent long‐term outcome. The aetiology of the epileptogenic lesion appears to be the only significant determinant of surgical outcome in this population of children. It is difficult to correctly identify non‐specific pathology on presurgical magnetic resonance imaging.     

Impact of Arteriogenesis in Plastic Surgery: Choke Vessel Growth Proceeds via Arteriogenic Mechanisms in the Rat Dorsal Island Skin Flap

Objective: We examined the molecular mediators of postoperative choke‐vessel growth. Our focus was the possible overlap between choke‐vessel growth and arteriogenesis. Methods: A rat perforator flap model, encompassing four vascular territories, was used. Flaps were surgically elevated, re‐inset, and allowed to survive for one, three, five, or seven days. Tissue samples for Western and histological analyses were collected from the choke zone along the dorsal midline. Tissue from territories linked by the choke zone was analyzed to distinguish between global and local effects. The proteins examined included CD11b, ICAM‐1, and MMP‐2, three markers associated with arteriogenesis, as well as Hsp70 and vascular endothelial growth factor, markers of physiological stress and hypoxia/ischemia. Results: Arteriogenesis markers, as shown by Western analysis, increased at three and five days after flap elevation, and the increase was localized by immunohistochemistry to the growing arteries and veins. The marker of physiological stress increased at Days 5 and 7. The hypoxia‐ischemia marker did not increase in the choke zone. Conclusions: The growth of choke arteries and veins proceeds in an inflammatory environment that resembles arteriogenesis. Ischemia did not appear to play a role in choke‐vessel changes.     

Complementary and alternative methods in cerebral palsy

There are no published studies specifically addressing complementary and alternative treatments in adults with cerebral palsy (CP). However, national surveys of adults with chronic disabilities document that a majority of them use such treatments, that they are willing to pay out of pocket, if necessary, and that they believe that pursuing such treatment relieves pain, reduces stress and anxiety, and leads to improved feelings of fitness and well-being. Individuals enjoy taking charge of their own health care decisions, and frequently feel more in control with these therapies than with more traditional methods. In contrast to adults, there is some information on complementary and alternative methods (CAM) in children with CP. This article discusses some of the CAM used in children that may be carried over into adulthood, as well as the pitfalls for patients and conventional physicians as they try to sort out what might be helpful and what might be harmful in this arena. Practitioners of both conventional and CAM therapies believe that exercise can be beneficial; accordingly, activities such as recreational sports, yoga, and hippotherapy may be continued from childhood into adulthood. General treatments for stress and anxiety, through such activities as yoga and meditation, though not directed at CP per se, may be more popular for adults than children. Research in this area should first identify what methods are being utilized and then subject these methods to well-designed outcome studies that take into account any associated risks.