Challenges in diagnosis and management of neonatal hyperparathyroidism in a resource-limited country: a case series from a Sudanese family.
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ABSTRACT:
SUBMITTER: Hassan SS
PROVIDER: S-EPMC8627147 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Apert syndrome or acrocephalosyndactyly is a rare genetic disease characterized by craniofacial dysmorphism and syndactyly of the hands and feet. We report an observation in a 4-month-old female infant, whose father was 65 years old. The infant was admitted to the neonatology of Sourô Sanou University Hospital (Burkina Faso) for respiratory distress in a congenital malformation disorders context with the notion of resuscitation for 10 minutes at birth. Her clinical examination revealed a craniofacial dysmorphism, syndactyly, choanal atresia, a cleft palate and a retardation of the psychomotor development. The paraclinical assessment consisted of a radiograph of the skeleton and a cerebral tomodensitometry confirming bicoronal synostosis and bone syndactyly; an abdominopelvic, cardiac ultrasound didn't reveal any abnormalities; toxoplasmic serology was negative and rubella serology positive. The association of Apert syndrome with positive rubella serology seems fortuitous. Also, the association of choanal atresia and cleft palate has not commonly been reported in Apert syndrome. In the absence of surgical the infant has been followed until 9 months with therapeutic prospects.
| S-EPMC6908957 | biostudies-literature
Project description:BackgroundThere is continued debate as to the optimal strategy for diagnosis and management of primary hyperparathyroidism (PHPT).AimTo compare the strategies used for the diagnosis and management of PHPT by physicians in five European countries.DesignQuestionnaire-based survey.MethodsPhysicians in France, Germany, the UK, Italy and Spain were invited to participate in the survey which was conducted using a web-based interface and were included in the evaluation if they had treated a minimum of four patients suffering from PHPT in the past year.ResultsA total of 421 physicians completed the survey. The majority of respondents were endocrinologists (68%) but other specialities included rheumatologists (10.9%), internists (11.8%) and urologists (9.2%). Diagnostic methods were similar across different countries and specialities but there were significant differences in the proportion of physicians who recommended parathyroidectomy in asymptomatic patients with indications for surgery according to the 2002 National Institutes of Health (NIH) consensus conference statement (?(2?)=?26.1, P?<?0.001). The proportion of patients referred for surgery ranged from 32% in Italy to 66% in Spain with intermediate values in Germany (64%), France (55%) and the UK (53%). Conversely, pharmacological therapy was used most frequently for these patients in Italy (32%) and least frequently in Spain (14%).ConclusionSignificant differences exist in the management of patients with asymptomatic PHPT in countries across Europe who have accepted indications for surgery according to the NIH consensus statement. Further research will be required to explore the reasons for this and to determine if these differences affect the clinical outcome of PHPT.
| S-EPMC3359504 | biostudies-literature
Project description:There are many challenges to performing clinical research in resource-limited settings. Here, we discuss several of the most common laboratory issues that must be addressed. These include issues relating to organization and personnel, laboratory facilities and equipment, standard operating procedures, external quality assurance, shipping, laboratory capacity, and data management. Although much progress has been made, innovative ways of addressing some of these issues are still very much needed.
| S-EPMC3893068 | biostudies-literature
Project description:PurposeAdequate cancer pain management (CPM) is challenging in resource-limited settings, where current international guideline recommendations are difficult to implement owing to constraints such as inadequate availability and accessibility of opioids, limited awareness of appropriate opioid use among patients and clinicians, and lack of guidance on how to translate the best evidence into clinical practice. The multinational and multidisciplinary CAncer Pain managEment in Resource-limited settings (CAPER) Working Group proposes a two-step initiative to bridge clinical practice gaps in CPM in resource-limited settings.MethodsA thorough review of the literature, a steering committee meeting in February 2017, and post-meeting teleconference discussions contributed to the development of this initiative. As a first step, we developed practical evidence-based CPM algorithms to support healthcare providers (HCPs) in tailoring treatment according to availability of and access to resources. The second part of the initiative proposes a framework to support an effective implementation of the CPM algorithms that includes an educational program, a pilot implementation, and an advocacy plan.ResultsWe developed CPM algorithms for first-line use, breakthrough cancer pain, opioid rotation, and refractory cancer pain based on the National Comprehensive Cancer Network guidelines and expert consensus. Our proposed educational program emphasizes the practical elements and illustrates how HCPs can provide optimal CPM according to evidence-based guidelines despite varied resource limitations. Pilot studies are proposed to demonstrate the effectiveness of the algorithms and the educational program, as well as for providing evidence to support a draft advocacy document, to lobby policymakers to improve availability and accessibility of analgesics in resource-limited settings.ConclusionsThese practical evidence-informed algorithms and the implementation framework represent the first multinational step towards achieving optimal CPM in resource-limited settings.
| S-EPMC6499735 | biostudies-literature
Project description:PurposeTo provide clinicians practicing in resource-limited settings with a framework to improve the diagnosis and treatment of pediatric and adult patients with sepsis.MethodsThe medical literature on sepsis management was reviewed. Specific attention was paid to identify clinical evidence on sepsis management from resource-limited settings.ResultsRecommendations are grouped into acute and post-acute interventions. Acute interventions include liberal fluid resuscitation to achieve adequate tissue perfusion, normal heart rate and arterial blood pressure, use of epinephrine or dopamine for inadequate tissue perfusion despite fluid resuscitation, frequent measurement of arterial blood pressure in hemodynamically unstable patients, administration of hydrocortisone or prednisolone to patients requiring catecholamines, oxygen administration to achieve an oxygen saturation >90%, semi-recumbent and/or lateral position, non-invasive ventilation for increased work of breathing or hypoxemia despite oxygen therapy, timely administration of adequate antimicrobials, thorough clinical investigation for infectious source identification, fluid/tissue sampling and microbiological work-up, removal, drainage or debridement of the infectious source. Post-acute interventions include regular re-assessment of antimicrobial therapy, administration of antimicrobials for an adequate but not prolonged duration, avoidance of hypoglycemia, pharmacological or mechanical deep vein thrombosis prophylaxis, resumption of oral food intake after resuscitation and regaining of consciousness, careful use of opioids and sedatives, early mobilization, and active weaning of invasive support. Specific considerations for malaria, puerperal sepsis and HIV/AIDS patients with sepsis are included.ConclusionOnly scarce evidence exists for the management of pediatric and adult sepsis in resource-limited settings. The presented recommendations may help to improve sepsis management in middle- and low-income countries.
| S-EPMC3307996 | biostudies-literature
Project description: Not available
| S-EPMC8965955 | biostudies-literature
Project description:Neonatal disease severity scoring systems are needed to make standardized comparison between performances of different units and to give prognostic information to parents of individual babies admitted. Existing scoring systems are unsuitable for resource-limited settings which lack investigations like pH, pO2/FiO2 ratio, and base excess. This study was planned to evaluate Modified Sick Neonatal Score (MSNS), a novel neonatal disease severity score designed for resource-constrained settings. It was a facility-based cross-sectional analytical study, conducted in the "Special Newborn Care Unit" (SNCU) of government district hospital, attached to Kasturba Medical College, Mangalore, India from November 2016 to October 2017. A convenience sample of 585 neonates was included. Disease severity was assessed immediately at admission using MSNS. MSNS had 8 parameters with 0, 1, and 2 scores for each. 41% of study population was preterm (n=240), and 84.1% had birth weight less than 2500 grams (n=492). The mean (SD) of the total MSNS scores for neonates who expired and discharged was, respectively, 8.22 (2.96) and 13.4 (2.14), a difference being statistically significant at P < 0.001. Expired newborns had statistically significant frequency of lower scores across each of the parameters. An optimum cutoff score of ≤10 with 80% sensitivity and 88.8% specificity in predicting mortality was obtained when the ROC curve was generated with the MSNS score as the test variable. Area under the curve was 0.913 (95% CI: 0.879-0.946). In conclusion, MSNS is a practicable disease severity score in resource-restricted settings like district SNCUs. It is for application in both term and preterm neonates. Total score ≤10 has a good sensitivity and specificity in predicting mortality of admitted neonates when used early during the course of hospitalization. MSNS could be used as a tool to compare performance of SNCUs and also enable early referral of individual cases to units with better facilities.
| S-EPMC6532318 | biostudies-literature
Project description:BackgroundMobile communication has been found to improve antiretroviral therapy (ART) adherence among people living with HIV. In an ongoing randomized clinical trial, 2 mobile communication strategies (ie, sending SMS text messages and real-time medication monitoring [RTMM]) were used to improve adherence to ART among people living with HIV in Tanzania. We noticed remarkable discrepancies between self-reported adherence and adherence recorded by SMS text messaging or RTMM among some of the first trial participants.ObjectiveOur objective was to describe these cases and the observed discrepancies in more detail, to serve as a useful illustration of some of the challenges in using mobile health in resource-limited settings.MethodsIn an ongoing randomized trial, adults living with HIV from two HIV treatment centers in Tanzania who were suspected of low levels of adherence were randomly assigned in a 1:1:1 ratio to receive (1) SMS text message reminders, (2) an RTMM device, or (3) no additional intervention to standard HIV care. During bimonthly study visits, the participants self-reported their level of adherence, received feedback about their level of adherence based on SMS text messaging or RTMM, and discussed strategies to overcome adherence problems with nurses providing HIV care. For the purpose of this report, we selected people living with HIV who had completed 5 follow-up visits and consistently reported more than 95% adherence, while SMS text messaging or RTMM recorded lower than 75% adherence. The participants were invited for a short, face-to-face in-depth interview to explore reasons for this discrepancy.ResultsAt the time of this analysis, 26 participants had completed follow-up. Six of these evidenced the above-mentioned discrepancies, with an average adherence of 46% based on SMS text messaging or RTMM, while self-reported adherence was 98%. Five of these 6 participants insisted that their adherence to ART was good, with 4 reporting that their adherence to properly using the monitoring device was low. Three participants mentioned concerns about involuntary disclosure of HIV status as a main reason for low adherence to using the device. Two participants were still depending on other reminder cues despite receiving SMS text message or RTMM reminders. Poor network coverage caused low adherence in 1 participant.ConclusionsPsychosocial barriers were reported as importantly contributing to low adherence, both with respect to use of ART and proper use of the adherence-monitoring device. This case series illustrates that when introducing new digital adherence monitoring technology, researchers should consider psychosocial barriers and distinguish between adherence to device use and adherence to treatment.Trial registrationPan African Clinical Trials Registry PACTR201712002844286; https://tinyurl.com/y98q4p3l.
| S-EPMC7315367 | biostudies-literature
Project description:BackgroundHand hygiene (HH) is a cornerstone of programmes to prevent healthcare associated infections (HAI) globally, but HH interventions are seldom reported from African neonatal units.MethodsWe conducted a quasi-experimental study evaluating the impact of a multi-modal intervention (SafeHANDS) on HH compliance rates, alcohol-based handrub (ABHR) usage, the Hand Hygiene Self-Assessment Framework (HHSAF) score, and healthcare-associated bloodstream infection (HA-BSI) rates at a 132-bed South African neonatal unit (4 wards and 1 neonatal intensive care unit [NICU]). The intervention included a campaign logo, HH training, maternal education leaflets, ABHR bottles for staff, and the setting of HH performance targets with feedback. Three 5-month study phases were completed in July 2020 (baseline), December 2020 (early) and May 2021 (intensive).ResultsA total of 2430 HH opportunities were observed: 1002 (41.3%) at baseline, 630 (25.9%) at early and 798 (32.8%) at intensive study phases. At baseline, the overall neonatal unit HH compliance rate was 61.6%, ABHR use was 70 mL/patient day, and the baseline HHSAF score was 'basic' (165). The overall neonatal unit HH compliance rate was unchanged from baseline to intensive phases (617/1002 [61.6%] vs. 497/798 [62.3%]; p = 0.797). The ABHR use remained similar between phases (70 versus 73 mL/patient day). The HHSAF score improved to 'intermediate' level (262). There was no change in the neonatal unit HA-BSI rate.ConclusionDespite improvement in the HHSAF score, no improvement in overall HH compliance rates, ABHR usage, or HA-BSI rates was observed. Future HH interventions in resource-limited neonatal units should incorporate implementation science and behaviour modification strategies to better understand the barriers and facilitators of HH best practice.
| S-EPMC9867086 | biostudies-literature
Project description:ImportanceUntreated primary hyperparathyroidism impairs quality of life and incurs substantial costs. Parathyroidectomy is a low-risk, high-success, definitive intervention.ObjectivesTo determine the appropriateness of diagnostic evaluation for primary hyperparathyroidism in patients with hypercalcemia and the use of parathyroidectomy for the treatment of primary hyperparathyroidism across the Veterans Affairs (VA) health care system.Design, setting, and participantsA retrospective cohort study of veterans with hypercalcemia and primary hyperparathyroidism was conducted from January 1, 2000, through September 30, 2015, using the VA Corporate Data Warehouse, a national electronic health record-based repository. The study included 371 370 veterans with chronic hypercalcemia and 47 158 veterans with biochemical evidence of primary hyperparathyroidism diagnosed by hypercalcemia, elevated serum parathyroid hormone levels, and near-normal serum creatinine levels. Statistical analysis was performed from April 21, 2017, to April 10, 2019.Main outcomes and measuresThe proportion of veterans with hypercalcemia who have parathyroid hormone levels evaluated, the proportion of veterans with hyperparathyroidism who are treated surgically, and the factors associated with parathyroidectomy using generalized linear latent and mixed model regression.ResultsOf 371 370 patients with chronic hypercalcemia, 86 887 (23.4%) received further testing with parathyroid hormone level. Of 47 158 patients meeting diagnostic criteria for primary hyperparathyroidism (42 737 men [90.6%] and 4421 women [9.4%]; mean [SD] age, 67.3 [11.8] years), 6048 (12.8%) underwent parathyroidectomy. Of 5793 patients with primary hyperparathyroidism presenting with a serum calcium level more than 1 mg/dL above the upper limit of normal, 1501 (25.9%) underwent parathyroidectomy. There was a decreasing trend in the use of parathyroidectomy over time. Factors positively associated with parathyroidectomy were nephrolithiasis (odds ratio [OR], 2.23; 95% CI, 1.90-2.61) and non-Hispanic white race/ethnicity (OR, 1.31; 95% CI, 1.17-1.46), while age (OR, 0.95; 95% CI, 0.95-0.96), Elixhauser Comorbidity Index score (OR, 0.76; 95% CI, 0.72-0.80), decreased estimated glomerular filtration rate (OR, 0.52; 95% CI, 0.45-0.60), and diagnosis of osteoporosis (OR, 0.65; 95% CI, 0.52-0.80) were inversely related to surgery.Conclusions and relevanceFrom this study's findings, parathyroid hormone level is infrequently tested in patients with hypercalcemia, suggesting underdiagnosis of primary hyperparathyroidism. Patients meeting diagnostic criteria for primary hyperparathyroidism are undertreated with recommended parathyroidectomy. Similar gaps have previously been observed in non-VA care of primary hyperparathyroidism, suggesting the need for a systematic evaluation of barriers to diagnosis and treatment that informs intervention design.
| S-EPMC6632180 | biostudies-literature