scholarly journals A case study of three patients with mucopolysaccharidoses in Hue Central Hospital

2020 ◽  
Vol 8 ◽  
pp. 2050313X2093824
Author(s):  
Tran Kiem Hao ◽  
Nguyen Thi Diem Chi ◽  
Nguyen Thi Hong Duc ◽  
Nguyen Thi Kim Hoa

Mucopolysaccharidosis is a group of rare metabolic disorders characterized by a deficiency of enzymes in the degradation of glycosaminoglycans. The incomplete degradation process leads to the accumulation of glycosaminoglycans in lysosomes of various tissues, which interferes with cell function. We report three cases that were classified as Hurler—Mucopolysaccharidosis I, Morquio—Mucopolysaccharidosis IV A, and Maroteaux–Lamy—Mucopolysaccharidosis VI. Clinical presentations of these cases vary, depending on each type of enzyme defect. All the patients appeared healthy at birth, and symptoms appear at around 1 or 2 years. Clinical features, radiological findings, and especially enzyme assays have allowed us to establish a definitive diagnosis in these cases. These cases highlight that abnormal clinical symptoms, such as growth failure, coarse facial features, and joint problems, are key points for further investigation relating to mucopolysaccharidosis disease. However, in low- and middle-income countries, it is difficult to have a definitive diagnosis of one of the mucopolysaccharidoses due to lacking enzyme assays.

Author(s):  
Jade Benjamin-Chung ◽  
Andrew Mertens ◽  
John M Colford ◽  
Alan E Hubbard ◽  
Mark J van der Laan ◽  
...  

AbstractGlobally 149 million children under five are estimated to be stunted (length more than 2 standard deviations below international growth standards). Stunting, a form of linear growth failure, increases risk of illness, impaired cognitive development, and mortality. Global stunting estimates rely on cross-sectional surveys, which cannot provide direct information about the timing of onset or persistence of growth failure— a key consideration for defining critical windows to deliver preventive interventions. We performed the largest pooled analysis of longitudinal studies in low- and middle-income countries to date (n=31 cohorts, 62,993 children, ages 0-24 months), allowing us to identify the typical age of linear growth failure onset and to investigate recurrent faltering in early life. The highest incidence of stunting onset occurred from birth to age 3 months. From 0 to 15 months, less than 5% of children per month reversed their stunting status, and among those who did, stunting relapse was common. Early timing and low reversal rates emphasize the importance of preventive intervention delivery within the prenatal and early postnatal phases coupled with continued delivery of postnatal interventions through the first 1000 days of life.


2007 ◽  
Vol 191 (S50) ◽  
pp. s71-s77 ◽  
Author(s):  
Mohan Isaac ◽  
Prabhat Chand ◽  
Pratima Murthy

BackgroundOutcome of schizophrenia has been described as favourable in low-and middle-income countries. Recently, researchers have questioned these findingsAimsTo examine the outcome studies carried out in different countries specifically looking atthose from low-and middle-income countriesMethodsLong-term course and outcome studies in schizophrenia were reviewedResultsA wide variety of outcome measures are used. The most frequent are clinical symptoms, hospitalisation and mortality (direct indicators), and social/ occupational functioning, marriage, social support and burden of care (indirect indicators). Areas such as cognitive function, duration of untreated psychosis, quality of life and effect of medication have not been widely studied in low-and middle-income countriesConclusionsThe outcome of schizophrenia appears to be better in low-and middle-income countries. A host of sociocultural factors have been cited as contributing to this but future research should aim to understand this better outcome. There is a need for more culture-specific instruments to measure outcomes


2007 ◽  
Vol 37 (3) ◽  
pp. 477-500 ◽  
Author(s):  
Mark Weisbrot

This article looks at Latin America's political shift over the last several years. The author argues that these changes have largely been misunderstood and underestimated in the United States for a number of reasons. First, Latin America's unprecedented growth failure over the past 25 years is a major cause of these political changes and has not been well-understood. Second, the collapse of the International Monetary Fund's influence in Latin America, and in middle-income countries, is an epoch-making change. Third, the availability of alternative sources of finance, especially from the reserves of the Venezuelan government, has become very important. Finally, the increasing assertion of national control over natural resources is an important part of the new relationship between Latin America and the United States. For these and other reasons, the relationship between Latin America and the United States has undergone a fundamental and possibly irreversible change, and one that opens the way to new and mostly more successful economic policies.


Nature ◽  
2020 ◽  
Vol 577 (7789) ◽  
pp. 231-234 ◽  
Author(s):  

AbstractChildhood malnutrition is associated with high morbidity and mortality globally1. Undernourished children are more likely to experience cognitive, physical, and metabolic developmental impairments that can lead to later cardiovascular disease, reduced intellectual ability and school attainment, and reduced economic productivity in adulthood2. Child growth failure (CGF), expressed as stunting, wasting, and underweight in children under five years of age (0–59 months), is a specific subset of undernutrition characterized by insufficient height or weight against age-specific growth reference standards3–5. The prevalence of stunting, wasting, or underweight in children under five is the proportion of children with a height-for-age, weight-for-height, or weight-for-age z-score, respectively, that is more than two standard deviations below the World Health Organization’s median growth reference standards for a healthy population6. Subnational estimates of CGF report substantial heterogeneity within countries, but are available primarily at the first administrative level (for example, states or provinces)7; the uneven geographical distribution of CGF has motivated further calls for assessments that can match the local scale of many public health programmes8. Building from our previous work mapping CGF in Africa9, here we provide the first, to our knowledge, mapped high-spatial-resolution estimates of CGF indicators from 2000 to 2017 across 105 low- and middle-income countries (LMICs), where 99% of affected children live1, aggregated to policy-relevant first and second (for example, districts or counties) administrative-level units and national levels. Despite remarkable declines over the study period, many LMICs remain far from the ambitious World Health Organization Global Nutrition Targets to reduce stunting by 40% and wasting to less than 5% by 2025. Large disparities in prevalence and progress exist across and within countries; our maps identify high-prevalence areas even within nations otherwise succeeding in reducing overall CGF prevalence. By highlighting where the highest-need populations reside, these geospatial estimates can support policy-makers in planning interventions that are adapted locally and in efficiently directing resources towards reducing CGF and its health implications.


Nutrients ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 2044
Author(s):  
Ritu Rana ◽  
Marie McGrath ◽  
Paridhi Gupta ◽  
Ekta Thakur ◽  
Marko Kerac

(1) Introduction: Current evidence on managing infants under six months with growth failure or other nutrition-related risk is sparse and low quality. This review aims to inform research priorities to fill this evidence gap, focusing on breastfeeding practices. (2) Methods: We searched PubMed, CINAHL Plus, and Cochrane Library for studies on feeding interventions that aim to restore or improve the volume or quality of breastmilk and breastfeeding when breastfeeding practices are sub-optimal or prematurely stopped. We included studies from both low- and middle-income countries and high-income countries. (3) Results: Forty-seven studies met the inclusion criteria. Most were from high-income countries (n = 35, 74.5%) and included infants who were at risk of growth failure at birth (preterm infants/small for gestational age) and newborns with early growth faltering. Interventions included formula fortification or supplementation (n = 31, 66%), enteral feeds (n = 8, 17%), cup feeding (n = 2, 4.2%), and other (n = 6, 12.8%). Outcomes included anthropometric change (n = 40, 85.1%), reported feeding practices (n = 16, 34%), morbidity (n = 11, 23.4%), and mortality (n = 5, 10.6%). Of 31 studies that assessed formula fortification or supplementation, 30 reported anthropometric changes (n = 17 no effect, n = 9 positive, n = 4 mixed), seven morbidity (n = 3 no effect, n = 2 positive, n = 2 negative), five feeding (n = 2 positive, n = 2 no effect, n = 1 negative), and four mortality (n = 3 no effect, n = 1 negative). Of eight studies that assessed enteral feed interventions, seven reported anthropometric changes (n = 4 positive, n = 3 no effect), five feeding practices (n = 2 positive, n = 2 no effect, n = 1 negative), four morbidity (n = 4 no effect), and one reported mortality (n = 1 no effect). Overall, interventions with positive effects on feeding practices were cup feeding compared to bottle-feeding among preterm; nasogastric tube feed compared to bottle-feeding among low birth weight preterm; and early progressive feeding compared to delayed feeding among extremely low birth weight preterm. Bovine/cow milk feeding and high volume feeding interventions had an unfavourable effect, while electric breast pump and Galactagogue had a mixed effect. Regarding anthropometric outcomes, overall, macronutrient fortified formula, cream supplementation, and fortified human milk formula had a positive effect (weight gain) on preterm infants. Interventions comparing human breastmilk/donor milk with formula had mixed effects. Overall, only human milk compared to formula intervention had a positive effect on morbidity among preterm infants, while none of the interventions had any positive effect on mortality. Bovine/cow milk supplementation had unfavourable effects on both morbidity and mortality. (4) Conclusion: Future research should prioritise low- and middle-income countries, include infants presenting with growth failure in the post-neonatal period and record effects on morbidity and mortality outcomes.


2020 ◽  
Vol 4 (4) ◽  
pp. 599-602
Author(s):  
Rahul Thakur ◽  
Rajshree Singh ◽  
Sabin Nepal ◽  
Prasanna Ghimire

Introduction: Tetanus is an acute onset neurological disease that is often lethal. It has a high disease burden in low and middle-income countries. Tetanus is caused by a toxin made by spores of the bacterium Clostridium tetani, which are found in soil, dust, and animal feces. The toxin impairs the motor neurons leading to muscle stiffness. However, with the development of a toxoid vaccine, the incidence has sharply declined and is now categorized as a vaccine-preventable disease. The treatment of tetanus is primarily supportive and focuses on managing the complications until the effects of toxins resolve. Case Report: We report the case of a 67-year-old farmer who previously sustained a laceration injury approximately 45 days prior to presenting to the emergency department with abdominal pain and rigidity. After a comprehensive evaluation to rule out other items in the differential diagnoses, he was diagnosed with tetanus based on clinical symptoms and ultimately required mechanical ventilation. The patient was then managed in the intensive care unit and later made an uneventful recovery. Conclusion: This case illustrates an uncommon presentation of tetanus and the latency of the infectious process. Often when patients present with atypical symptoms, it poses a diagnostic dilemma to the clinicians. Thus, it is very important to carefully elicit a history of contaminated injury. This case also highlights the importance of prophylactic vaccine in low and middle-income countries, which can reduce disease-related mortality and morbidity.


PEDIATRICS ◽  
2018 ◽  
Vol 141 (3) ◽  
pp. e20172183 ◽  
Author(s):  
Iván Mejía-Guevara ◽  
Daniel J. Corsi ◽  
Jessica M. Perkins ◽  
Rockli Kim ◽  
S.V. Subramanian

2021 ◽  
Author(s):  
Fredman González ◽  
Omar Zepeda ◽  
Christian Toval-Ruiz ◽  
Armando Matute ◽  
Hernan Vanegas ◽  
...  

ABSTRACTNew information is emerging about SARS-CoV-2 epidemiology and immunity, but little of this information comes from low- and middle-income countries or from patients receiving care in the outpatient setting. The current study investigated the SARS-CoV-2 infection status and antibody responses in 157 patients seeking care for a respiratory disease suggestive of COVID-19 in private healthcare clinics during the first wave (June–October 2020) of infections in Nicaragua. We examined nasal swabs for the presence of viral RNA via RT-PCR and longitudinally collected sera for the changes in SARS-CoV-2 Spike antibody levels over six months. Among patients with confirmed SARS-CoV-2 infections, we evaluated if clinical symptoms were associated with age, hematological parameters and co-morbidities. The combination of PCR and paired serology identified 60 (38%) of the 157 outpatients as acute COVID-19. While both PCR and serology identified the majority (n = 38, 64%) of the acute infections, a notable number of outpatients were identified by RT-qPCR (n = 13, 22%) or by serology (n = 9, 14%) only. During the longitudinal study, we identified 6 new infections by serology among the 97 non-COVID-19 subjects. In conclusion, this study report that more than one third of the outpatients seeking care for acute respiratory disease during the first epidemic wave of SARS-CoV-2 in Nicaragua had an acute mild COVID-19 infection that correlate with prolonged humoral response. This immune response to the RBD antigen, more likely IgG dependent, significantly increased between the acute to convalescent and decay in the late convalescent but still remained seropositive.


2012 ◽  
Author(s):  
Joop de Jong ◽  
Mark Jordans ◽  
Ivan Komproe ◽  
Robert Macy ◽  
Aline & Herman Ndayisaba ◽  
...  

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