Evaluating the implementation of a multi-level mHealth study to improve hydroxyurea utilization in sickle cell disease.

Background: Sickle Cell Disease (SCD) is a progressive genetic disease that causes organ damage and reduces longevity. Hydroxyurea is an underutilized evidence-based medication that reduces complications and improves survival in SCD. In a multi-site clinical trial, part of the NIH-funded Sickle Cell Disease Implementation Consortium (SCDIC), we evaluate the implementation of a multi-level and multi-component mobile health (mHealth) patient and provider intervention to target the determinants and context of low hydroxyurea use. Given the complexity of the intervention and contextual variability in its implementation, we combined different behavioral and implementation theories, models, and frameworks to facilitate the evaluation of the intervention implementation. In this report, we describe engagement with stakeholders, planning of the implementation process, and final analytical plan to evaluate the implementation outcomes. Methods: During 19 meetings, a 16-member multidisciplinary SCDIC implementation team created, conceived, and implemented a project that utilized Intervention Mapping to guide designing an intervention and its evaluation plan. The process included five steps: (1) needs assessment of low hydroxyurea utilization, (2) conceptual framework development, (3) intervention design process, (4) selection of models and frameworks, and (5) designing evaluation of the intervention implementation. Results: Behavioral theories guided the needs assessment and the design of the multi-level mHealth intervention. In designing the evaluation approach, we combined two implementation frameworks to best account for the contextual complexity at the organizational, provider, and patient levels: (1) the Consolidated Framework for Implementation Research (CFIR) that details barriers and facilitators to implementing the mHealth intervention at multiple levels (users, organization, intervention characteristics, broader community), and (2) the Technology Acceptance Model (TAM), a conceptual model specific for explaining the intent to use new information technology (including mHealth). The Reach Effectiveness Adoption Implementation and Maintenance (RE-AIM) framework was used to measure the outcomes. Discussion: Our research project can serve as a case study of a potential approach to combining different models/frameworks to help organize and plan the evaluation of interventions to increase medication adherence. The description of our process may serve as a blueprint for future studies developing and testing new strategies to foster evidence-based treatments for individuals living with SCD.

Citizen science in hydrological monitoring and ecosystem services management: State of the art and future prospects.

Hydrological monitoring is essential to guide evidence-based decision making necessary for sustainable water resource management and governance. Limited hydrometric datasets and the pressure on long-term hydrological monitoring networks make it paramount to explore alternative methods for data collection. This is particularly the case for low-income countries, where data scarcity is more pronounced, and where conventional monitoring methods are expensive and logistically challenging. Citizen science in hydrological research has recently gained popularity and crowdsourced monitoring is a promising cost-effective approach for data collection. Citizen science also has the potential to enhance knowledge co-creation and science-based evidence that underpins the governance and management of water resources. This paper provides a comprehensive review on citizen science and crowdsourced data collection within the context of hydrology, based on a synthesis of 71 articles from 2001 to 2018. Application of citizen science in hydrology is increasing in number and breadth, generating a plethora of scientific data. Citizen science approaches differ in scale, scope and degree of citizen involvement. Most of the programs are found in North America and Europe. Participation mostly comprises a contributory citizen science model, which engages citizens in data collection. In order to leverage the full potential of citizen science in knowledge co-generation, future citizen science projects in hydrology could benefit from more co-created types of projects that establish strong ties between research and public engagement, thereby enhancing the long-term sustainability of monitoring networks.

Citizen science pioneers in Kenya - A crowdsourced approach for hydrological monitoring.

Although water is involved in many ecosystem services, the absence of monitoring data restricts the development of effective water management strategies especially in remote regions. Traditional monitoring networks can be expensive, with unaffordable costs in many low-income countries. Involving citizens in monitoring through crowdsourcing has the potential to reduce these costs but remains uncommon in hydrology. This study evaluates the quality and quantity of data generated by citizens in a remote Kenyan basin and assesses whether crowdsourcing is a suitable method to overcome data scarcity. We installed thirteen water level gauges equipped with signboards explaining the monitoring process to passers-by. Results were sent via a text-message-based data collection framework that included an immediate feedback to citizens. A public web interface was used to visualize the data. Within the first year, 124 citizens reported 1175 valid measurements. We identified thirteen citizens as active observers providing more than ten measurements, whereas 57% only sent one record. A comparison between the crowdsourced water level data and an automatic gauging station revealed high data quality. The results of this study indicate that citizens can provide water level data of sufficient quality and with high temporal resolution.

Autophagy is essential to suppress cell stress and to allow BCR-Abl-mediated leukemogenesis.

Hematopoietic cells normally require cell extrinsic signals to maintain metabolism and survival. In contrast, cancer cells can express constitutively active oncogenic kinases such as BCR-Abl that promote these processes independent of extrinsic growth factors. When cells receive insufficient growth signals or when oncogenic kinases are inhibited, glucose metabolism decreases and the self-digestive process of autophagy is elevated to degrade bulk cytoplasm and organelles. Although autophagy has been proposed to provide a cell-intrinsic nutrient supply for mitochondrial oxidative metabolism and to maintain cellular homeostasis through degradation of damaged organelles or protein aggregates, its acute role in growth factor deprivation or inhibition of oncogenic kinases remains poorly understood. We therefore developed a growth factor-dependent hematopoietic cell culture model in which autophagy can be acutely disrupted through conditional Cre-mediated excision of the autophagy-essential gene Atg3. Treated cells rapidly lost their ability to perform autophagy and underwent cell cycle arrest and apoptosis. Although Atg3 was essential for optimal upregulation of mitochondrial oxidative pathways in growth factor withdrawal, this metabolic contribution of autophagy did not appear critical for cell survival, as provision of exogenous pyruvate or lipids could not completely rescue Atg3 deficiency. Instead, autophagy suppressed a stress response that otherwise led to p53 phosphorylation and upregulation of p21 and the pro-apoptotic Bcl-2 family protein Puma. Importantly, BCR-Abl-expressing cells had low basal levels of autophagy, but were highly dependent on this process, and rapidly underwent apoptosis upon disruption of autophagy through Atg3 deletion or treatment with chemical autophagy inhibitors. This dependence on autophagy extended in vivo, as Atg3 deletion also prevented BCR-Abl-mediated leukemogenesis in a cell transfer model. Together these data demonstrate a critical role for autophagy to mitigate cell stress, and that cells expressing the oncogenic kinase BCR-Abl appear particularly dependent on autophagy for cell survival and leukemogenesis.

A survey of the prevalence of Giardia in dogs presented to Canadian veterinary practices.

Fecal samples (1216) were collected from dogs presented to 15 veterinary practices across Canada and tested for Giardia by ELISA for group-specific antigen. If positive, the presence of Giardia was confirmed by microscopy. Eighty-eight submissions tested positive by ELISA and microscopy. The overall prevalence rate of Giardia was 7.2%.

Emergency care in patients wearing body casts.

OBJECTIVE: To describe a body cast modification that allows rapid chest exposure for cardiopulmonary resuscitation and alert the medical community to the 3% to 5% incidence of cardiopulmonary arrest in the hospital while wearing such a cast. DESIGN. Single trial timed cast cutting, multiple trial cast shell loading, and clinical observations of perforated casts for cracking or breakage. SETTING: University hospital castroom, mechanical engineering laboratory. INTERVENTION: Body casts mounted on a life-sized torso mold were perforated at 2-inch (50 mm), 1-inch (25 mm), or 1/2 inch (12 mm) intervals around the chest with a 12-mm-diameter vibrating drill. Using a cast saw, the chest piece of each body cast was removed and the time recorded. Engineering studies were performed on two cast shells with and without 12-mm-wide holes up to 1/2" (12 mm) apart, loading the fiberglass to the point of failure and recording the data. Body casts with 1" (25 mm)-interval holes worn by 40 patients were examined after 12 weeks for evidence of failure. MAIN OUTCOME MEASURES: Chest piece removal times of body cast shells with and without holes were compared. Loading data of cast shells were compared to determine if holes as close as 1/2" (12 mm) significantly weakened the cast. Forty casts, perforated at 1-inch (25 mm) intervals, were observed for failure after 12 weeks of wear. RESULTS: Chest exposure time of a mold encased in a fiberglass cast was reduced from 1 minute to 15 seconds; plaster cast removal was reduced from 3 minutes to 1 minute. Engineering studies of perforated casts showed no significant decrease in strength. Casts with and without holes could support chest forces of up to 330 pounds. No failure was observed in casts with perforations 1 inch (25 mm) apart worn for 12 weeks. CONCLUSION: Perforated body casts reduced removal time to 15 seconds without weakening of the cast and provide lifesaving time to perform effective CPR.

Intrauterine onset of a mononeuropathy: peroneal neuropathy in a newborn with electromyographic findings at age one day compatible with prenatal onset.

Mononeuropathies are unusual at birth, and electromyographic (EMG) definition the first day of life has not been reported previously. Although neonatal mononeuropathies may be related to obstetric complications, prenatal mechanisms also merit consideration. We report an infant, born with a peroneal neuropathy, whose EMG was performed 18 h after birth. An isolated peroneal nerve lesion with lack of compound muscle action potential and the presence of fibrillation potentials, confined to the tibialis anterior muscle, suggested a primary intrauterine mechanism for this mononeuropathy. Because of an infant's small size, the temporal profile used in adults for appearance of EMG signs of wallerian degeneration may not apply. Inaccurate conclusions may result if the EMG standards for timing adult nerve injury are applied to newborns. To our knowledge, previous published cases of neonatal mononeuropathies have not included babies whose first EMG was performed before age 4 days. Therefore, an EMG study shortly after birth needed to be accomplished if strong support for the hypothesis of a prenatal onset were to be generated. Our findings are compatible with an intrauterine onset of this baby's peroneal neuropathy.

Future ambulation prognosis as predicted by somatosensory evoked potentials in motor complete and incomplete quadriplegia.

OBJECTIVE: The purpose of this prospective study was to determine the efficacy of tibial somatosensory evoked potentials (SEPs) in predicting ambulation in tetraplegic individuals. DESIGN: This was a prospective study of a cohort of cervical spinal cord-injured patients who had SEPs recorded within 72 hours to 2 weeks post-SCI and whose ambulation outcome was followed up to 2 years post-SCI. SETTING: Regional Spinal Cord Injury (SCI) Center. PATIENTS: All male and female subjects admitted to the center from 1988 to 1991 between the ages of 15 and 60 years who demonstrated C4 through T1 complete and incomplete acute SCIs were asked to participate in this study. MEASUREMENTS: The tibial nerve cortical SEPs were graded as either present or absent. The waveforms were also graded as less than 0.5 microV or > or = 0.5 microV. Quadriceps strength plus touch and pin sensation were tested within 72 hours to 2 weeks post-SCI. Ambulation was rated as absent, exercise, household, or community. The ambulatory and clinical status were assessed monthly for 3 months, and then at 6, 12, 18, and 24 months post-SCI. Statistical analysis using the two-tailed Fisher's exact test was performed relating the initial clinical and SEP data to ambulation outcome up to 24 months post-SCI. RESULTS: All 13 subjects with a right and/or left quadriceps manual muscle test (MMT) greater than 0/5 became ambulatory. Of the 9 subjects with an initial bilateral quadriceps MMT = 0/5, only 1 recovered enough lower limb function to ambulate (p = .0001). One of the 7 subjects with absent touch sensation in the lower limbs became ambulatory, whereas 14 of the 15 subjects with touch sensation present became ambulatory (p = .002). All 7 subjects with absent pin sensation in the lower limbs were nonambulatory, and 14 of 15 subjects with pin sensation present became ambulatory (p < .0001). Of the 9 subjects with bilaterally absent cortical SEP waveforms, 2 became ambulatory. Twelve of the 13 subjects with a cortical SEP wave present became ambulatory (p = .0015). Of the 10 subjects with a cortical SEP wave amplitude less than 0.5 microV, only two became ambulatory, whereas all 12 subjects with an amplitude > or = 0.5 microV became ambulatory (p = .00014). In no subject did the SEP predict future ambulation where the clinical examination did not also predict recovery of ambulation. CONCLUSION: Both the early postinjury clinical evaluation and the SEP predicted ambulation outcome to a significant degree, but the SEP offered no additional prognostic accuracy over that provided by the clinical examination.

Pin sensation as a predictor of extensor carpi radialis recovery in spinal cord injury.

The purpose of this prospective study was to determine if initial preservation of C5 pin sensation could predict extensor carpi radialis (ECR) motor recovery in the zone of partial preservation after spinal cord injury (SCI). The study compared initial C5 pin sensation and initial ECR strength as predictors of ECR motor recovery after C4 and C5 SCI. The initial motor and sensory evaluation was done less than seven days after injury. Motor power was graded from absent (0/5) to normal strength (5/5). Pin sensation was graded as absent (0/2) decreased (1/2) or normal (2/2). The manual muscle test was performed weekly to four weeks and than at three, six, and 12 months after injury. An ECR muscle grade of > or = 3/5 was defined as recovery. C5 pin sensation at the lateral antecubital fossa significantly correlated with ECR recovery to > or = 3/5 (p < 0.001). Only two of nine subjects with absent C5 pin sensation recovered ECR to > or = 3/5, whereas 14 of 15 subjects with 1/2 or 2/2 C5 pin sensation had ECR motor recovery to > or = 3/5. All ten subjects with 1/5 or 2/5 initial ECR strength had ECR motor recovery. Five of six subjects with 0/5 initial ECR strength and subsequent ECR recovery to > or = 3/5 were found to have C5 pin preservation. In conclusion the presence of 1/5 to 2/5 initial ECR strength or 1/2 or 2/2 C5 pin sensation were highly significant predictors of ECR motor recovery to > or = 3/5 in C4 and C5 motor complete quadriplegic subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Extensor carpi radialis recovery predicted by qualitative SEP and clinical examination in quadriplegia.

This prospective study examined the efficacy of the qualitative somatosensory evoked potential (SEP) and the initial clinical neurologic evaluation to predict motor power recovery of the extensor carpi radialis muscle (ECR). Twenty three C5-6 Frankel A-D spinal cord injured (SCI) subjects had SEPs of the median nerve (MN) and superficial radial nerve (SRN) performed within 72 hours to one week post injury. The MN and SRN cortical SEPs were qualitatively graded as either present or absent. Fifteen subjects whose initial ECR muscle strength was less than or equal to 3/5 and eight subjects whose muscle strength was greater than 3/5 were followed up to 12 to 18 months post injury for improvement in ECR muscle strength. The subject's ECR strength was evaluated by manual muscle testing (MMT) at 72 hours, weekly for three weeks, monthly for three months, and then at six, 12, and 18 months. The pin sensation at the C-5 dermatome was also tested at the above intervals and graded as either present or absent. A one tail Fisher Exact test compared the presence or absence of the MN and SRN SEPs to the recovery of the ECR to 3/5. The same one tail test also compared the presence or absence of the 72 hour C-5 pin sensation and the 72 hour MMT to the ECR recovery. Among the 15 subjects with an initial MMT of less than or equal to 3/5, ten subjects had successful ECR recovery (greater than 3/5); 5 did not. The C-5 pin sensation correctly predicted ECR recovery in all subjects studied (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

B cell surface marker analysis of synovial fluid cells in a patient with monoarthritis and chronic lymphocytic leukemia.

We describe a women with clinically quiescent chronic lymphocytic leukemia who developed monoarthritis consistent with her known diagnosis of osteoarthritis. Because of the concurrent chronic leukemia, we were concerned with the possibility of leukemic arthritis. Immunofluorescence of synovial fluid cells demonstrated an increase in B cells that failed to demonstrate monoclonality as determined by light chain class expression. However, biopsy of synovial tissue revealed leukemic infiltration. Local radiation therapy resolved the monoarthritis.

Cutaneous Hodgkin's disease in a patient with human immunodeficiency virus infection.

A 22-year-old man with human immunodeficiency virus infection had skin nodules of the chest wall. When a biopsy was performed on these nodules, Hodgkin's disease was discovered. The patient was subsequently found to have widespread disease involving the lymph nodes and liver. Skin involvement with Hodgkin's disease is rare. In this report, we review previous descriptions of skin involvement appearing in the literature.

Medically reversible quadriparesis in tophaceous gout.

This case report presents a successful nonoperative outcome of a type II odontoid fracture secondary to severe tophaceous gout. The patient presented with spastic quadriparesis and tophaceous involvement of the odontoid process with fracture and C1-C2 instability. He refused surgery. However, his odontoid process stabilized with the used of a collar, and he regained almost normal strength plus independence in self-care and ambulation, demonstrating that an unstable type II odontoid fracture secondary to tophaceous gout can stabilize with conservative management.

Rehabilitation of the person with arthritis of the ankle and foot.

When combined with skillful pharmacologic management, applying the full stock of biomechanical principles available to the rheumatoid foot and ankle is very effective in avoiding the need for surgery and slowing or preventing deformity. The general principles of arthritis rehabilitation also help restore function. When good medical management, joint protection, exercise, and energy conservation techniques are combined with psychosocial vocational therapy as needed, the crippling loss of function so often seen in the past can be largely avoided.

Cytochemical and competitive protein binding assays for estrogen receptor in breast disease. A comparative study of 62 cases.

Estrogen receptor content of breast lesions was estimated using a fluorescent cytochemical technique and a competitive protein binding assay. Of 48 cancers examined, an equal proportion contained significant quantities of receptor by either method (62.5%). The concordance between methods for individual patients was also 62.5%. A greater proportion (26%) of patients younger than 45 years of age had receptor-positive cancers using the cytochemical method than were found by the biochemical method (10%). Benign breast disease was also studied using the fluorescent cytochemical method. A greater proportion of lesions containing estrogen receptors was found compared with that cited in the literature for the competitive protein-binding assay. Because of the methodologic simplicity of the fluorescent cytochemical method, further study for routine use is indicated.

Dysphagia associated with cricopharyngeal dysfunction.

In protracted forms of dysphagia associated with neuromuscular dysfunction, myotomy of the upper esophageal sphincter has been suggested. The literature, however, is unclear about the indications and outcome of this procedure. In this article, 3 cases are presented of dysphagia associated with the failure of relaxation of the cricopharyngeus during swallowing. Two patients had brain stem infarctions and the 3rd had an inflammatory disease of the brain stem. In all patients, barium swallow revealed aspiration of contrast material into the trachea with failure of relaxation of the cricopharyngeal sphincter. Indirect laryngoscopy demonstrated partial paralysis of one or both vocal cords. In one, an emg of the laryngeal muscles showed normal results. A complete evaluation by the speech pathologist failed to reveal abnormality of the oral musculature in all patients. All patients required gastrostomies for their nutritional needs. Therapeutic modification and control of rate, quantity and consistency of food along with counseling for prevention of aspiration. Oral feeding, without aspiration, was achieved in the 3 patients within the treatment period, allowing removal of the gastrostomy. Cricopharyngeal myotomy, therefore, was not necessary in these patients, despite the long duration of the swallowing problem.