Screening Programme for Phenylketonuria in the Gaza Strip: Evaluation and Recommendations

Abstract

Phenylketonruia (PKU) is an inherited metabolic disorder that results in progressive mental retardation. PKU is a paradigm of a disease that can be identified by proper screening of newborns and medical follow-up in order to prevent serious complications. The present study was designed to evaluate the Palestinian national screening programme for PKU in the Gaza Strip. Data about the screening of PKU in the Gaza Strip were obtained from the records of the healthcare centers of the Palestinian Ministry of Health (MOH) during the year 2000. In addition, PKU patients and families were interviewed. The results showed that the prevalence of PKU in the Gaza Strip varied considerably between the different regions with an overall prevalence of 6.35/100,000, while the maximum prevalence of 28.3/100,000 occurred in the rural areas. Coverage of PKU testing in the Gaza Strip is limited to about 35.3 per cent of the total newborns, who are delivered and receive health care at the government clinics. Among those newborns delivered at the government clinics, the percentage of PKU screening is about 87.8 per cent. However, PKU testing is not carried out at UNRWA clinics where about two-thirds of newborn deliveries take place. On average, 61 per cent of PKU testing is made in the infant's second week, ranging between 11 and 17 days, and the remaining (39 per cent) are tested thereafter. Approximately 60 per cent of PKU patients had consanguine parents (first cousins), while 7.7 per cent had no consanguinity. Only 43.1 per cent of PKU patients were fed on the specialized low phenylalanine milk. An inverse correlation was reported between the use of low phenylalanine milk and age. A total of 35.4 per cent of the PKU patients were regularly monitored by blood tests each month, 47.7 per cent had not been tested for the previous year. It was concluded that the PKU screening programme has to be improved, the screening methods should be reviewed, and the screening coverage should include all the newborns in the Gaza Strip.

The Prevalence of Phenylketonuria in Arab Countries, Turkey, and Iran: A Systematic Review

“…Other studies conducted in Iraq [37] indicated that all 7 cases detected with PKU had consanguineous parents. These findings were consistent with previous studies where 57% of PKU patients in Egypt [26], 60% of PKU cases in Gaza Strip [19], 86.6% of PKU patients in Iran [22], and 34 patients out of 43 PKU cases from Iran [31] had consanguineous parents. Similarly, a recent study conducted in 2017 in Mazandaran Province in Iran indicated that parental family relationships among confirmed PKU cases were 53.6% [24].…”

“…In some of the prevalence/incidence studies, prevalence was self-calculated in 9 articles and corrected in 11 articles. Prevalence/incidence studies were further categorized to the type of study whether it was a national NBS program ( n = 5 articles) [6, 15–18], regional/governorate NBS program ( n = 6 articles) [19–24], selective screening for newborns ( n = 6 articles) [25–30], selective screening of sick/symptomatic newborns and/or infants, children, and adults ( n = 9 articles) [31–39], selective screening for both newborns and sick/symptomatic newborns and/or infants, children, and adults ( n = 4 articles) [40–43], or selective screening for sick/symptomatic children and adults from institutions for mentally challenged ( n = 3 articles) [44–46]. In addition, a study conducted in Turkey addressed PKU prevalence among newborns, sick/symptomatic subjects, and mentally challenged individuals.…”

“…These results concur with Moammar et al [25] findings in Saudi Arabia revealing all detected cases to have consanguineous parents. A study conducted in Gaza in contrast confirmed that some PKU cases were not consanguineous [19]. Nevertheless, it fails to mask the fact that most of the studies reporting the cases arose from consanguineous marriages.…”

Scope and efficiency of the newborn screening program in identifying hemoglobin S

“…Research conducted in the Gaza Strip, in the Palestinian Territories, showed that 61% of the collections occurred between the 11th and 17th day of life 18 . In Brazil, in Porto Alegre city, the average was 11 days; 19 in Sergipe State 17 days, with 50% of samples being collected in children older than 28 days; 20 in Campina Grande city, the average was 29.8 days, 21 and in Marília, São Paulo State, the average was 27 days 22 …”

Gaza Enclave: Victim, Enemy, Rival

“…40 As far as legitimacy is concerned, one should read carefully the reports produced by scholars associated with the two parties. Thus for instance the report produced by Ali Abu-Shahla, Anat Cygielman, and Ata Qaymari seems to be part of an official Fatah discourse that attempts to delegitimise Hamas rule in Gaza.…”