Sickle Cell Disease

University Hospitals Cleveland Medical Center

Hemoglobin Desaturation in Sickle Cell Disease

Hemoglobin Desaturation and RBC Adhesion: Potential Therapeutic Targets in Sickle Cell Disease

We will test resting SpO2 and night-time oxymetry to obtain Hb saturation results.

We will evaluate 6MWT results and obtain Hb saturation results.

Serum chemistry through a comprehensive panel will be evaluated

Incidence of hypoxia-related symptoms will be obtained from review of the patient's chart and approved symptom questionnaire.

Incidence of nocturnal hypoxia-related symptoms will be obtained from review of the patient's chart and approved symptom questionnaire.

Amount of S-RBC adhesion to LN on the SCD and Hypoxia Biochips will be quantitated, using <400 μL surplus whole blood in EDTA, obtained during routine clinical care (as published previously1,6-8), at the clinic visit immediately prior to night-time oximetry and 6MWT

Fluorescent Activated Cell Sorting (FACS) following incubation with antibodies to CD14, CD16, and CX3CR1 will be performed on 3-400 μL of surplus whole blood.

Simple t-tests will be used to compare RBC adhesion, HEA to LN and monocyte activation in patients with clinically significant hypoxia and those without any hypoxia

At ~2 months after initial testing (and >6 weeks on treatment, if needed), all subjects will be re-evaluated for incidence of hypoxia-related nocturnal symptoms through review of the patient's chart and approved symptom questionnaire

At ~2 months after initial testing (and >6 weeks on treatment, if needed), all subjects will be re-evaluated for amount of RBC adhesion

At ~2 months after initial testing (and >6 weeks on treatment, if needed), all subjects will have incidence of hypoxia-related symptoms re-evaluated through review of the patient's chart and approved symptom questionnaire

Paired t-test on S-RBC adhesion to LN and HEA before and after oxygen therapy, in subjects with and without clinically significant hypoxia will be performed

Amount of WBC activation will be determined

CBC results will be examined for any suggestive changes

Reticulocyte count will be examined for any suggestive changes

LDH level will be examined for any suggestive changes

Serum chemistry through a comprehensive panel will be examined for any suggestive changes

Jane Little

Director, Adult Sickle Cell provider

Intensive care support and Equine F(ab')2 antivenom

Poisoning by Scorpion Sting

A single dose of 4 vials of Equine F(ab')2 antivenom will be administered intravenously over 10 minute

Equine F(ab')2 antivenom

Intensive care support as needed plus placebo

Intensive care support plus placebo

CHU Hassan II de Fès

Hôpital Ibn Zohr, Marrakech

A Trial of Equine F (ab')2 Antivenom for Treatment of Scorpion Envenomation in Morocco

Phase 2/3 Study for Scorpion North Africa Middle East Envenomation With a Immune F(ab')2 (Equine) Antivenom Alacramyn NAMO. A Randomized, Double-Blind, Placebo-controlled, Prospective and Multicenter Study

Comparison between antivenom and placebo groups of the number of cases showing improvement in class of envenomation.

Decrease in plasma venom levels from baseline to one hour after study drug administration; Respiratory rate (breaths per minute); Heart rate (beats per minute); Dose of dobutamine (cumulative, per hour);Incidence of cardiac failure; Incidence of ventilatory failure; Incidence of neurological failure; Mortality

priapism/hypoxia

Hemoglobin Desaturation in Sickle Cell Disease

A Trial of Equine F (ab')2 Antivenom for Treatment of Scorpion Envenomation in Morocco

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