National control programme against nutritional blindness due to vitamin A deficiency: Current status & future strategy

Vitamin A deficiency (VAD) among 1-5 yr old children is reported to be widely prevalent in Southeast Asia and some parts of Africa. It is the leading cause of preventable blindness in young children in the low-income countries in the world. Children even with milder signs of VAD have higher risk of morbidity and mortality. Inadequate dietary intakes of vitamin A with poor bioavailability associated with frequent infections are the primary contributory factors. Currently available approaches to control VAD are ensuring adequate intakes of vitamin A in daily diets, fortification of foods consumed regularly particularly among the low-income communities and periodic administration of massive dose of vitamin A supported by public health interventions and reinforced by behaviour change communication. Under the National Programme in India, six monthly administration of mega dose of vitamin A to 6-59 month old children has been implemented since 1970, to prevent particularly blindness due to VAD and control hypovitaminosis A. Despite inadequate coverage and poor implementation of the programme, blindness due to VAD in children has almost disappeared, though subclinical VAD is still widely prevalent. Based on the results of meta-analysis of eight trials, which indicated that vitamin A supplementation to children aged 6-59 months reduced child mortality rates by about 23 per cent, the World Health Organization made a strong recommendation that in areas with VAD as a public health problem, vitamin A supplementation should be given to infants and children of 6-59 months of age as a public health intervention to reduce child morbidity and improve child survival. At present, in India, there is a need for change in policy with respect to the national programme to opt for targeted instead of universal distribution. However, NITI (National Institution for Transforming India) Aayog, which formulates policies and provides technical support to the Government of India, recommends strengthening of the National Programme for control of VAD through six monthly vitamin A supplementation along with health interventions. Eventually, the goal is to implement food based and horticulture-based interventions harmonizing with public health measures, food fortification and capacity building of functionaries for elimination of VAD.

Controversies in neuro-ophthalmology: Steroid therapy for traumatic optic neuropathy.

Background: There is an increase in the incidence of traumatic optic neuropathy (TON) due to increasing urbanization and rapid spurt in the number of motor vehicles on the road. Despite early presentation and ease of diagnosis the visual outcomes in TON are still limited. There is also significant confusion about the timing, dose and efficacy of steroid treatment in its management. Purpose: To provide a clinical update of the pros and cons of steroid therapy for TON. Design: The paper is a retrospective review of the currently available literature in the English language indexed in PubMed. Methods: A PubMed search was conducted by the authors using the following terms: Traumatic optic neuropathy, megadose, steroids, methylprednisolone. Relevant original articles, review articles, and case reports related to the topic of discussion were evaluated and discussed in the paper. Results: There is no prospective randomized control trial evaluating the effect of steroids in TON. There are varying reports on the effect of steroid therapy from significant improvement to no difference compared to observation. Conclusion: The decision to give steroids to patients with TON has to be on an individual case to case basis and must involve informed consent from the patient. There are documented advantages and disadvantages of steroid therapy and a prospective, randomized, controlled trial is necessary comparing steroids, surgery and observation before definitive management can be evolved.

The Treatment of Liver Hemangioma Associated with Kasabach-Merritt Syndrome in Neonates

Hemangioma is the most frequent liver tumor in infancy. The treatment of hepatic hemangioma includes medical, surgical, and non-operative interventional therapy. There are no standard medical regimens currently considered consistently effective. MDMP (megadose methylprednisolone) and alpha-interferon can be used for medical treatment. Interventional occlusion of feeding arteries in symptomatic hepatic hemangioma is considered a safe and effective alternative to early open surgery. Untreated symptomatic patients with heart failure have a high mortality rate. For this reason symptomatic patients with heart failure may require non-operative treatment such as interventional embolization, because hepatic resection is burdened with high risk. We report our experiences of two patients with neonatal liver hemangiomas whose clinical courses were complicated by cardiac failure to whom medical treatment and/or interventional vascular occlusion were done. Both patients had Kasabach-Merritt syndrome complicated with cardiac failure. Initially, the masses were considered unresectable. In one case, we performed interventional therapy in addition to medical treatment. In another case, we tried medical therapy with megadose steroid and alpha-interferon. Unfortunately, in spite of the treatments, the patients died of severe hemorrhage.

Optic Nerve Damage Complicating Repair of Orbital Floor Fracture

PURPOSE: The authors report a devastating complication, visual loss following the orbital wall fracture repair. Its occurrence results from orbital compartment syndrome, or direct intraoperative injury to the optic nerve from surgical manipulation, bony fragment, orbital implants, or inferior retinal arteriolar occlusion. METHODS: A patient who was 15-year old was fisted on his right eye. When he visited a hospital, both visual acuity was 1.0 and his pupillary light reflex was normal. He complained diplopia in primary position and his eyeball showed motility limitation to both upward and downward gaze. Anterior and posterior segments of the eye were normal. Orbital CT showed right inferior orbital wall fracture and herniated inferior rectus muscle sheath and soft tissue. Orbital floor implantation after systemic steroid therapy for 1 week was done. However postoperative visual acuity was decreased to light perception and relative afferent pupillary defect(RAPD) was developed. So immediately orbital decompression and mega-dose steroid therapy was carried out. RESULTS: After 2 month, visual acuity was recovered to 1.0 and RAPD was decreased. There remained hyperdeviation and exodeviation in right eye but was no limitation of eyeball movement. The color vision test was normal but visual field test, visual evoked potential test showed abnormal findings CONCLUSIONS: Visual loss following the repair of a orbital wall fracture was gradually recovered by immediate decompression and mega-dose steroid therapy but remained visual field defect associated with optic nerve atrophy.

Allogeneic Peripheral Blood Stem Cell Transplantation from An HLA-identical Sibling Donor in Children / 대한소아혈액종양학회지

PURPOSE: G-CSF-mobilized peripheral blood is one of the sources of allogeneic hematopoietic stem cells. We report our experiences on allogeneic peripheral blood stem cell transplantation (allo-PBSCT) from an HLA-identical sibling donor in children. METHODS: From August 1998 to January 2003, 8 patients underwent allo-PBSCT. Median age of recipients and donors were 4 yr 10 mo (range 3 yr 2 mo 15 yr) and 5 yr 1 mo (range 1 yr 11 mo 19 yr 8 mo), respectively. Seven patients (3 ALL, 2 neuroblastomas, 1 AML, 1 Gaucher disease) had failed from previous allogeneic or autologous transplant and 1 patient had refractory acute biphenotypic leukemia. Only 2 patients were in complete remission at allo-PBSCT. G-CSF 10mug/kg/day was injected subcutaneously to the donor for 5 days and large volume leukapheresis was performed on 4th and 5th days. RESULTS: Median number of CD34 and CD3 cells infused was 18.55 106 (range 9.47 84.76) /kg and 8.26 108 (range 0.88 24.58) /kg, respectively. All patients achieved ANC > 0.5 109/L after a median of 9 days and 6 patients eventually achieved platelet engraftment. There was no grade II-IV acute GVHD but limited chronic GVHD developed in 6 evaluable patients. There was no CMV antigenemia. Three patients died from either transplant-related mortality (n=2) or relapse (n=1). The remaining 5 patients are alive disease-free for 7, 8, 15, 16, and 19 months from allo-PBSCT, respectively. CONCLUSION: Our results suggest that mega-dose allo-PBSCT from an HLA-identical sibling donor is expected to improve transplant outcome especially in very high risk pediatric patients.

Megadose Steroids and Intranasal Optic Nerve Decompression in the Treatment of Traumatic Optic Neuropathy

BACKGROUND AND OBJECTIVES: Traumatic optic neuropathy (TON) is a relatively rare complication associated with closed head injury. However, it represents an extremely poor prognosis, and its management remains controversial. We present the treatment results of 15 patients with immediate and complete TON who were treated with megadose steroids (MDS), and, in cases where MDS produced no response, intranasal optic nerve decompression (OND). PATIENTS AND METHODS: The diagnosis of TON was based on evidence of the following : complete loss of vision, absence of direct pupillary light reflex and intact consensual response. All of the patients underwent high resolution CT scans of the orbit and received a complete neuro-opthalmologic examination. MDS was started immediately after the diagnosis. If no response occurred by 48 hours, an intranasal OND was conducted. RESULTS: Two of the 15 patients exhibited improved vision after treatment with MDS, and six of the remaining 13 patients who were unresponsive to MDS demonstrated improved vision after OND. Overall, eight out of the 15 patients experienced improved vision. CONCLUSION: This study is uncontrolled, but suggests that our protocol of MDS and, in cases where this produced no response, OND may be an effective and valid treatment modality for patients with immediate complete TON, which is generally believed to represent an extremely poor prognosis regardless of treatment.

Endoscopic Optic Canal Decompression

The visual loss owing to acute optic nerve injury after blunt head trauma has been reported about 5% at the incidence. But it has been real circumstances that we still don`t have the exact knowlege about surgical indication, operation time and steroid dosage for this candidate. Lately, endoscopic surgery was introduced for treatment of orbital disease instead of classic external approach and we have performed and report endoscoic endonasal optic canal decompression followed by megadose steroid injection in 5 patients who experienced visual loss due to optic canal fracture following trauma.

Evaluation of Methylprednisolone Dosing Regimen in Experimental Diffuse Brain Injury

The present study was undertaken to examine the ability of a single large intraperitoneal dose of methylprednisolone(15, 30, 40, 60mg/kg) to modify the evolution of diffuse brain injury(DBI) in rats undergoing a contusion injury of 400gm-1m. Twenty five Sprague-Dawley rats were divided to five groups. Group 1 was not treated after cranial impact as control. Rats in group 2, 3, 4, 5 were treated with 15-mg/kg, 30-mg/kg, 40-mg/kg, 60-mg/kg methylprednisolone dose at 1 hour after injury, respectively. After 24 hours, these rats were sacrificed, and the water content of the whole brain were measured. When the water content was assumed as the criterion of brain edema, there was significant difference between control group(71.03+/-.42%) and group 2(67.34+/-.07%), group 3(66.43+/-.36%), group 4(64.52+/-.11%)(p0.01). This study shows that megadose of methylprednisolone in 15, 30, or 40mg/kg reduces the brain edema, and the its effect is biphasic in that the 60-mg/kg methylprednisolone dose is ineffective.