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Neurologic disorders in pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Robert Burger, Terry Rolan, David Lardizabal, Upinder Dhand, Aarti Sarwal, Pradeep Sahota
Fetal/neonatal complications in myasthenic pregnancies include higher perinatal mortality (6–8%), stillbirth (2%), pulmonary hypoplasia, polyhydramnios, neonatal MG (10–20%), and arthrogryposis multiplex congenita (AMC) (31,41,42). Neonatal MG is related to placental transfer of acetylcholine receptor antibody. The neonate may develop hypotonia, poor sucking and swallowing, and respiratory difficulties. The onset is within 1 to 4 days and weakness resolves over 4 to 6 weeks. Intrauterine exposure to acetylcho-line receptor antibody is also the basis for poor fetal movement and consequent AMC (27). There is a high risk of recurrence of AMC in subsequent pregnancies.
Miscellaneous Topics
Published in Nirmal Raj Gopinathan, Clinical Orthopedic Examination of a Child, 2021
Prateek Behera, Karthick Rangasamy, Nirmal Raj Gopinathan
The term “arthrogryposis multiplex congenita” (AMC) refers to a heterogeneous group of muscular, neurological, and connective tissue anomalies that present with two or more joint contractures at birth as well as muscle weakness (Figure 18.2).3 Arthrogryposis is not a diagnosis per se but rather is a descriptive term with many etiologies and clinical presentations. Of all the etiologies, neurological factors account for 70–80% of cases. Based on the clinical presentation, signs, and etiology, AMC is divided into subgroups. Amyoplasia and distal arthrogryposis are the two most common subgroups.3 Children with amyoplasia are often bedridden. They tend to have a midfacial hemangioma. They primarily affect the limbs. Table 18.1 summarizes the clinical features of these two subgroups.
The future starts now – the identity and competence of doctors and the impact of accreditation
Published in Roger Ellis, Elaine Hogard, Professional Identity in the Caring Professions, 2020
Julie Gustavs, Theanne Walters
In 2000, the AMC established connections with consumer groups and updated its standards to include explicit reference to the need for medical education providers and their members to engage with the community to ensure that medical programs meet the needs of the communities they serve.
Accelerating the development of vaccine microarray patches for epidemic response and equitable immunization coverage requires investment in microarray patch manufacturing facilities
Published in Expert Opinion on Drug Delivery, 2023
Tiziana Scarnà, Marion Menozzi-Arnaud, Martin Friede, Kerry DeMarco, George Plopper, Melinda Hamer, Ajoy Chakrabarti, Philippe Alexandre Gilbert, Courtney Jarrahian, Jessica Mistilis, Renske Hesselink, Kristoffer Gandrup-Marino, Jean-Pierre Amorij, Birgitte Giersing
The most recent example is, of course, the response to the COVID-19 pandemic. Funding to manufacturing at risk was a key component in accelerating the availability of COVID-19 vaccines. The COVAX mechanism (which brings together governments, global health organizations, manufacturers, scientists, the private sector, civil society, and philanthropic organizations with the aim of providing innovative and equitable access to COVID-19 vaccines) used ‘push incentives,’ subsidizing developers and manufacturers directly for pharmaceutical research and development and manufacturing capacity while vaccine candidates were still in early development [30]. COVAX also provided ‘pull incentives,’ paying for performance via a reward price and/or delivery of products. These included the Advanced Purchase Commitment (APC), which provided individual vaccine developers and manufacturers with ‘volume guarantees’ for vaccines before they were licensed, as well as the Advance Market Commitment (AMC), a market-wide demand guarantee, available to any manufacturer, to buy an overall quantity of vaccines if and when they were ready. These mechanisms incentivized developers and manufacturers to invest in vaccine development and manufacturing at risk [31,33].
COVID-19 vaccine equity: a health systems and policy perspective
Published in Expert Review of Vaccines, 2022
Remco Van De Pas, Marc-Alain Widdowson, Raffaella Ravinetto, Prashanth N Srinivas, Theresa J. Ochoa, Thierno Oumar Fofana, Wim Van Damme
In the last 20 years, inequity of routine vaccination has further decreased, in large part because of new financing frameworks and procurement mechanisms, and the establishment of Gavi, the Vaccine Alliance, in 2000, bringing together private and public partners. The Gavi Alliance, via its Advanced Market Mechanism (AMC) and partnership model, has driven down prices for the procurement of routine vaccines. In 2002, Gavi funded three ‘Accelerated Development and Introduction Plans’ for rotavirus, pneumococcus (PCV), and Haemophilus influenzae type b (Hib) vaccines to reduce the time lag between their introduction in rich countries and poor countries and increasing coverage. These plans have supported the introduction of four vaccines in 43 countries since 2002 [21]. Of note are the newer vaccines such as rotavirus, human papillomavirus (HPV), pentavalent (diphtheria, pertussis, tetanus, hepatitis B, and Hib), and PCV vaccines. Although these have prices ranging from USD 1.6–14.0/dose – a price range that is often over 10 times as those in the original EPI scheme [20] – global expansion of the use of these vaccines continues because of public subsidies and international aid.
A 7-year old female with arthrogryposis multiplex congenita, Duane retraction syndrome, and Marcus Gunn phenomenon due to a ZC4H2 gene mutation: a clinical presentation of the Wieacker-Wolff syndrome
Published in Ophthalmic Genetics, 2021
Deena Godfrey, Alcy Torres, Gena Heidary, Hovra Zahoor, Arthur Lee, Gerard Berry, Elizabeth Engle
DRS is a form of congenital strabismus characterized by limited horizontal eye movement coupled with globe retraction and reduction in palpebral fissure height on attempted adduction and, in some cases, up or downshoots of the eye (1). DRS is caused by failure of abducens motor neurons to innervate the lateral rectus muscle, which is innervated instead by an aberrant branch of the oculomotor nerve (1). In DRS, co-contraction of the medial rectus and lateral rectus muscles occurs as a result of this aberrant neuronal innervation (1). AMC refers to congenital non-progressive joint contractures involving at least two different body areas (2). Most AMC cases are secondary to nervous system defects that arise from environmental or genetic factors (3). Thus, motor neuron maldevelopment can lead to contractures in AMC and restricted horizontal eye movement in DRS (4). Both DRS and AMC have an incidence of approximately 1:1500–1:3000 live births (1). Significant research has been directed towards uncovering the genetic etiology of DRS and AMC. Familial DRS can follow autosomal dominant or recessive inheritance patterns, with pathogenic variants being identified CHN1, MAFB, and SALL4 among other genes (5). AMC has been associated with autosomal dominant, autosomal recessive, and X-linked inheritance, and over 400 AMC genes have been reported (6). Here, we report a child with both AMC and DRS who harbors a de novo ZC4H2 mutation, thereby confirming this association that was previously reported in two teenaged females (7).