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Hereditary persistence of fetal hemoglobin (HPFH) is a benign condition in which significant fetal hemoglobin production continues well into adulthood, disregarding the normal shutoff point after which only adult-type hemoglobin should be produced. The percentage of incorrect expression might be as low as 10%-15% or as high as 100% of the total hemoglobin, usually higher in homozygotes than in heterozygotes. The present case is a typical example of homozygous HPFH.

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OBJECTIVES: To investigate variability in pediatric tracheostomy tube care practice patterns and access to resources across Canada. METHODS: Canadian pediatric otolaryngologists-head & neck surgeons reported their own practice patterns for children with chronic tracheostomy tubes using a web-based, 29-item multiple choice and short answer questionnaire. Domains investigated included tracheostomy team membership, inpatient care practices, caregiver education, homecare resources, speech and communication, and completeness of emergency tracheostomy kits. RESULTS: The response rate was 86.4% (38/44). Most respondents care for children with tracheostomy tubes as part of an inter-professional team (25/36; 69.4%) and arrange routine follow-up with a speech and language pathologist (22/36; 61.1%). However, the majority (23/34; 67.6%) of respondents do not formally reassess caregiver competencies (i.e. cardiopulmonary resuscitation, emergency tracheostomy care). Notably, respondents were also unsure 36.1% (13/36) of how frequently Shiley tracheostomy tubes should be washed and reused with the majority (15/36; 41.7%) reporting never. Most (15/36; 41.7%) respondents were also unsure of reuse recommendations for Bivona tracheostomy tubes. One third (12/36; 33.3%) of respondents were unsure about government-funded homecare services being provided in their community to children with tracheostomy tubes. CONCLUSION: There is much variability in pediatric tracheostomy tube care practice patterns across Canada. Results suggest that an evidence-based Canadian clinical practice guideline may help to streamline care provided to Canadian children with tracheostomy tubes.

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Can you diagnose this patient and identify the uncommon cause of his illness? http://ow.ly/t4nX30bzJFz.

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OBJECTIVES/HYPOTHESIS: Adductor spasmodic dysphonia (AdSD) is a voice disorder characterized by variable symptom severity and voice disability. Those with the disorder experience a wide spectrum of symptom severity over time, resulting in varied degrees of perceived voice disability. This study investigated the longitudinal variability of AdSD, with a focus on auditory-perceptual judgments of a dimension termed laryngeal overpressure (LO) and patient self-assessments of voice-related quality of life (V-RQOL). STUDY DESIGN: Longitudinal, correlational study. METHODS: Ten adults with AdSD were followed over three time periods. At each, both voice samples and self-ratings of V-RQOL were gathered prior to their scheduled Botox injection. Voice recordings subsequently were perceptually evaluated by eight listeners for LO using a visual analog scale. RESULTS: LO ratings for all-voiced and Rainbow Passage sentence stimuli were found to be highly correlated. However, only the LO ratings obtained from judgments of AV stimuli were found to correlate moderately with self-ratings of voice disability for both the physical functioning and social-emotional subscores, as well as the total V-RQOL score. Based on perceptual judgments, LO appears to provide a reliable means of quantifying the severity of voice abnormalities in AdSD. CONCLUSIONS: Variability in self-ratings of the V-RQOL suggest that perceived disability related to AdSD should be actively monitored. Further, auditory-perceptual judgments may provide an accurate index of the potential impact of the disorder on the speaker. Similarly, LO was supported as a simple clinical measure that serves as a reliable index of voice change over time.

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OBJECTIVE: To evaluate the non-inferiority of a lower therapeutic dose (300,000 IU) in comparison to standard dose (600,000) IU of Vitamin D for increasing serum 25(OH) D levels and achieving radiological recovery in nutritional rickets. DESIGN: Randomized, open-labeled, controlled trial. SETTING: Tertiary care hospital. PARTICIPANTS: 76 children (median age 12 mo) with clinical and radiologically confirmed rickets. INTERVENTION: Oral vitamin D3 as 300,000 IU (Group 1; n=38) or 600,000 IU (Group 2; n=38) in a single day. OUTCOME VARIABLES: Primary: Serum 25(OH)D, 12 weeks after administration of vitamin D3; Secondary: Radiological healing and serum parathormone at 12 weeks; and clinical and biochemical adverse effects. RESULTS: Serum 25(OH)D levels [geometric mean (95% CI)] increased significantly from baseline to 12 weeks after therapy in both the groups [Group 1: 7.58 (5.50­10.44) to 16.06 (12.71­ 20.29) ng/mL, P<0.001]; Group 2: 6.57 (4.66­9.25) to 17.60 (13.71­22.60, P<0.001]. The adjusted ratio of geometric mean serum 25(OH)D levels at 12 weeks between the groups (taking baseline value as co-variate) was 0.91 (95% CI: 0.65­1.29). Radiological healing occurred in all children by 12 weeks. Both groups demonstrated significant (P<0.05) and comparable fall in the serum parathormone and alkaline phosphatase levels at 12 weeks. Relative change [ratio of geometric mean (95% CI)] in serum PTH and alkaline phosphatase, 12 weeks after therapy, were 0.98 (0.7­1.47) and 0.92 (0.72­1.19), respectively. The serum 25(OH)D levels were deficient (<20 ng/mL) in 63% (38/60) children after 12 weeks of intervention [Group 1: 20/32 (62.5%); Group 2: 18/28 (64.3%)]. No major clinical adverse effects were noticed in any of the children. Hypercalcemia was documented in 2 children at 4 weeks (1 in each Group) and 3 children at 12 weeks (1 in Group 1 and 2 in Group 2). None of the participants had hypercalciuria or hypervitaminosis D. CONCLUSION: A dose of 300,000 IU of vitamin D3 is comparable to 600,000 IU, administered orally, over a single day, for treating rickets in under-five children although there is an unacceptably high risk of hypercalcemia in both groups. None of the regime is effective in normalization of vitamin D status in majority of patients, 3 months after administering the therapeutic dose.

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PURPOSE OF STUDY: Study was aimed to observe benefits of transfusion of neocytes/pooled neocytes over packed RBCs by super transfusion regimen in ß-thalassemic patients and its comparison with related studies. MATERIALS AND METHODS: Twelve cases were selected and were equally divided into control and study group in which packed RBCs and neocytes were transfused respectively for one year. In study group, average 8 transfusions were required over 14 in control group. RESULTS: A marked extension of transfusion interval was observed in study group patients receiving neocytes transfusion (avg. 45.8 days; range, 32-54) over control group patients i.e. those receiving conventional packed RBCs (avg. 26.1; range 22-30). There is an average 40.8% reduction in the volume of RBCs required in the study group compared to control group and this difference is statistically significant (p = 0.00075). In study group, 20.1% reduction in iron overload and increase of iron by 9.6% in control group was reported. CONCLUSION: We observed that neocytes transfusion is effective in extending the transfusion interval as well as in reducing the blood requirement and iron over load.

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This study was aimed to review and establish the practice of exchange transfusion (ET) with reconstituted blood in neonates and to observe fall of bilirubin and its comparison with related studies. Twenty-five neonates diagnosed as hemolytic disease of newborn (HDN) were selected for this study, in which exchange transfusion was carried out as one of the treatments for hyperbilirubinemia. Out of the 25 cases, 15 were of Rhesus (Rh) HDN, while ABO and other blood groups constituted 6 and 4 HDN cases respectively. First, the neonates's and mother's blood samples were subjected to relevant investigations. After that, for neonates having Rh HDN, O Rh negative cells suspended in AB plasma were given, O Rh positive cells suspended in AB plasma were given to ABO HDN; and O positive cells, which were indirect Coomb's cross-matched compatible with neonates' and mother's serum / plasma, suspended in AB plasma were given to the neonates having HDN because of other blood group antibodies. The exchange transfusion (ET) was carried out taking all aseptic precautions by Push-Pull technique with double-volume exchange transfusion method. The average post-exchange fall in serum indirect bilirubin was (52.01%) in all 25 cases, which was found to be more significant than the previous studies. Looking into the superiority of the exchange transfusion in HDN by reconstituted blood, the reconstituted blood can be modified and supplied as per the requirement and conditions.

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We assessed the effect of Azadirachta indica (A. indica), a plant that has been reported to possess antioxidant, anti-inflammatory and anxiolytic properties, on cerebral reperfusion injury and long term cerebral hypoperfusion. When blood flow to brain region that has undergone critical period of ischemia is re-established, additional injury is to be expected from the reperfusion. In the present study, bilateral common carotid artery (BCCA) occlusion for 30 min followed by 45 min reperfusion resulted in increase in lipid peroxidation, superoxide dismutase (SOD) activity and fall in total tissue sulfhydryl (T-SH) groups. A. indica pretreatment (500 mg/kg/day x 7 days) attenuated the reperfusion induced enhanced lipid peroxidation, SOD activity and prevented fall in T-SH groups. Moreover, A.indica per se increased brain ascorbic acid level, which was unchanged during reperfusion insult. Long-term cerebral hypoperfusion induced by permanent BCCA occlusion has been reported to cause behavioral and histopathological abnormalities. In the present study, as tested by open field paradigm and Morris' water maze, a propensity towards anxiety and disturbances of learning/memory were observed in animals subjected to hypoperfusion for 2 weeks. A. indica (500 mg/kg/day x 15 days) significantly reduced these hypoperfusion induced functional disturbances. Reactive changes in brain histology like gliosis, perivascular lymphocytic infiltration, recruitment of macrophages and cellular edema following long term hypoperfusion were also attenuated effectively by A. indica. We conclude that our study provides an experimental evidence for possible neuroprotective potentiality of A. indica.

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Free radicals are implicated in causation of cerebral reperfusion injury and chronic cerebral hypoperfusion in rats is associated with functional and histopathological disturbances. Ocimum sanctum (OS), a plant widely used in Ayurveda, has been shown to possess anti-inflammatory, antioxidant and cognition-enhancing properties. In the present study, we investigated the effect of methanolic extract of OS leaves in cerebral reperfusion injury as well as long-term hypoperfusion. Occlusion of bilateral common carotid arteries (BCCA) for 30 min followed by 45 min reperfusion caused increase in lipid peroxidation and up-regulation of superoxide dismutase (SOD) activity accompanied by fall in tissue total sulfhydryl groups (TSH) in rat forebrains. Ascorbic acid levels were unchanged, however. OS pretreatment (200 mg/kg/day for 7 days) prevented this reperfusion-induced rise in lipid peroxidation and SOD activity. OS pretreatment also stabilized the levels of TSH during reperfusion. Long-term cerebral hypoperfusion (a model of cerebrovascular insufficiency and dementia) induced by permanent occlusion of BCCA for 15 days demonstrated altered exploratory behavior in open-field testing and memory deficits as tested by Morris' water maze. Histopathological examination of hypoperfused animals revealed reactive changes, like cellular edema, gliosis and perivascular inflammatory infiltrate. OS treatment (200 mg/kg/day for 15 days) significantly prevented these hypoperfusion-induced functional and structural disturbances. The results suggest that OS may be useful in treatment of cerebral reperfusion injury and cerebrovascular insufficiency states.

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Herein we are presenting the clinical, morphological and cytochemical characteristics of five cases of acute megakaryoblastic leukaemia (AML-M7) seen by us over a period of five years (Jan 1996-Dec 2000). Morphological assessment revealed marked polymorphism of blast cells and platelets both in the peripheral blood and bone marrow smears in all cases. Size of the blast cells ranged from very small to very large multinucleated cells, with variable chromatin pattern and number of nucleoli. More differentiated megakaryocytic cells showing cytoplasmic blebs, protrusions and platelet budding with bizarre platelet morphology were characteristic features suggesting the diagnosis. Cytochemical stains like myeloperoxidase, sudan black and PAS were positive in 5-15% of blast cell. Coagulation studies revealed a normal coagulation profile, whereas platelet studies showed marked impairment in aggregation of platelets with ADP and adrenalin with a normal PF-3 availability.

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Restoration of blood flow to an ischemic brain region is associated with generation of reactive oxygen species with consequent reperfusion injury. Chronic cerebral hypoperfusion induced by permanent occlusion of bilateral common carotid arteries in rats is associated with behavioral and histopathological alterations. Nimodipine, a dihydropyridine calcium channel antagonist, has potent vasodilatory effect on cerebral vessels and increases cerebral blood flow. We analyzed whether nimodipine reduces injury caused by transient forebrain ischemia and long-term cerebral hypoperfusion. Bilateral common carotid occlusion for 30 min followed by 45 min reperfusion resulted in a two-fold increase in lipid peroxidation and superoxide dismutase activity. Nimodipine pretreatment (4 mg/kg, i.p.) brought down these levels by 30 and 23%, respectively. Long-term cerebral hypoperfusion in rats caused a propensity towards anxiety and listlessness (open field paradigm) accompanied by deficits of learning and memory (Morris' water maze testing). Additionally, histopathological observation in hypoperfused brains revealed reactive changes in the form of perivascular inflammation, gliosis and astrocytosis. Nimodipine treatment significantly alleviated these changes in behavioral and histopathological parameters. Our data confirm the protective role of nimodipine in ischemia reperfusion injury. Moreover, it suggests the beneficial role of nimodipine in cerebrovascular insufficiency states and dementia.

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