Abstract
Phenylketonuria (PKU) is a correctable inborn error of metabolism which causes lethal intellectual delay and neurobehavioral anomalies. A screening package, especially for early recognition can support to regulate the PKU process of most patients. New-born screening program in any country focuses at the earliest detection of inheritance deficiency disorders in order to avoid the most severe repercussion by appropriate medication. This screening program needs a concomitant diagnosis and involves additional clinical research. Strategies from developed countries recommend that new-born screening should be done as soon as possible after birth before hospital/clinic discharge because if detected later, it conveys to significantly increase in disability as well as morbidity. Although exact protocol differs among different countries, testing procedures for PKU should be followed universally recognized in the developed world. Unfortunately, new-born screening program in Bangladesh is in lying-in room or possibly in pilot study in particular hospital, because the health-care system is classically targeted mortality (like childbirth complications) and transmittable morbidities (such as COVID-19) but not inborn frailties. Although policies and management of childbirth complications have been successfully lowered infant and mother mortality rates, the number of disabled babies increased tremendously. The study aims to investigate the current status of new-born screening (NBS) program of PKU in the Rajshahi Division Bangladesh, and focus on future plans to manage with life-long treatment. The primary challenges such as financial support for newborn screening, publicity, should be identified and implemented for national PKU-NBS policy as a role model of Bangladesh for developing countries.
Highlights
Alma-Ata Convention, 1978 declared that every child has identical right to acquire the basic healthcare facility regardless of sex, race/ethnic, society or nationality as a worldwide birth-right [1]
Most of the developing countries have focused on quality of health services and abundant evidence proposes that quality of health care is almost absent in case of inherent newborn deficiency disorders [2–4]
For establishing new-born screening programs of PKU patients in the United Kingdom, Robert Guthrie developed a classical method for the detection of high levels of Phe in blood shortly after a baby born by using a bacterial inhibition assay [10]
Summary
Alma-Ata Convention, 1978 declared that every child has identical right to acquire the basic healthcare facility regardless of sex, race/ethnic, society or nationality as a worldwide birth-right [1]. For establishing new-born screening programs of PKU patients in the United Kingdom, Robert Guthrie developed a classical method for the detection of high levels of Phe in blood shortly after a baby born by using a bacterial inhibition assay [10]. Given the priority of higher birth rate and socioeconomical considerations in countries like Bangladesh, introducing new-born screening (NBS) program for these metabolic deficiency disorders would be a substantial but valuable challenge. These therapeutics demonstrate the choice of solutions that can be organized to address the basic problem of PKU, the lack of catalytic action of the PAH enzyme Majority of these therapies have shown high efficacy in the animal models (such as murine) of PKU, all of the therapies need pharmacokinetic analysis and clinical trials to ensure the safety and longevity of treatment, as well as eliminating the rigorous dietary restrictions in case of patients with PKU [29]. Patients at home countries should be disable entitled and government can provide a disability allowance and/or a disability certificate to cover out of package costs as well as social security of PKU patients
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