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Asploro Journal of Pediatrics and Child Health (ASJPCH) is an international, peer-reviewed open access journal in which it addresses not only health related issues (disease, infection, genetic disorder, growth impairment etc.), but also aims to cover topics related to the physical, emotional and social well-being of children. Child psychology, behavioral aspects, societal apathy, exposure to the criminal and violent environment, parental negligence, and cultural background, are some of the factors that influence the development of a child; and any research related to these issues will be accepted for publication….
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Introduction: Cystic Fibrosis has been reported before in almost all Arab countries with an incidence ranges from 1:2500- 1:7000. Presentations varied, but mainly due to recurrent chest infection and Pancreatic Insufficiency. Median survival has been far below North American countries. Delayed diagnosis and delayed management account for the low median survival.
Objectives: To present the demographic data of Cystic Fibrosis patients and their families, that involves their social status and education.
Methodology: A retrospective chart review as part of the Cystic Fibrosis registry data from the period January 1998 to December 2018. All confirmed CF patients of all age groups who contributed their demographic information were included and analyzed.
Results: A total of 430 confirmed Cystic Fibrosis patients. 236 (96%) patients survived, and 10 (4%) died. Two hundred and thirteen (49.5%) were males, and 217 (50.5%) were females. Eighty-three percent consanguinity rate. Forty-five had a family history of Cystic Fibrosis, and the diagnosis was suggested by family history in 9.5% of patients. 415 (98.1%) were of Saudi nationality. 156 (36.5%) were from the Eastern province. The mean age at diagnosis was 3.46 years (SD±5.547). Median survival around 22 years. Mean Sweat chloride was 92.04 mmol/ L (17.343). In reviewing the educational level of 247 patients, the level of elementary school accounted for 90 (36.1%) of patients, 24 (9.7%) of mothers, and 21 (8.5%) of fathers. Similarly, 43 (17.4%)/ 22 (8.9%)/ 102 (41.3%) were in the preparatory level, 35 (14.3%)/ 43 (17.4%)/ 51 (20.6%) were in the high school level, and 23 (9.3%)/ 39 (15.8%)/ 46 (18.6%) were in the college level, respectively. Regarding the employment: 145 (58.7%) patients are students, 3 (1.2%) are part-time employees, and 15 (6.0%) are full time employees. 207 (83.8%) mothers are housewives, 2 (0.8%) are students, and 29 (11.7%) have full-time employment. Paternal employment showed that 210 (85.0%) are full time, and 7 (2.8%) are part-time employees. Regarding their accommodation: 77 (31.2%) of Cystic Fibrosis parents owned a villa, 81 (32.8%) rented an apartment, and 79 (32%) owned their own apartment.
Conclusion: More than 2/3 of CF patients are students at the elementary school level, and only 6.0% have a full-time job, which makes them completely dependent on both parents for their care. Median survival improved from 8 years in 1984 to 22 years. Further efforts need to be applied to different aspects of care to further improve median survival.
Introduction: Cholelithiasis has been reported in 12%-24% of Cystic Fibrosis (CF) patients, and is usually made up of cholesterol gallstones. These abnormalities are frequently asymptomatic and can include intra and extrahepatic ducts, gallbladder thickening and contraction, micro gallbladders, and cholelithiasis. Abdominal sonography is routinely used in order to detect these abnormalities.
Objectives: To obtain the prevalence of gall stones (Cholelithasis) in CF patients and its relation to other clinical, laboratory, radiological, and genetic data.
Methodology: A retrospective chart review as part of the CF registry data from the period 1st January 1984 – 1st June 2018. All confirmed CF the patients of all age groups that have US studies done were included in the study. Patients with positive gallstones or sludge were evaluated and discussed.
Results: A total of 391 confirmed CF patients were involved. Out of them, 252 patients had an abdominal ultrasound, 7 patients (3%) had gallstones on the abdominal US, 8 patients (3%) were revealed to have sludge and 237 patients (94%) had normal gallbladders. Pancreatitis was found in 4 patients (2%). 191 patients (76%) had pancreatic insufficiency.77 patients had follow up abdominal ultrasounds and 5 patients (7%) were found to have persistent gallstones, 4 patients (5%) had persistent sludge and 68 patients (88%) remained negative for gallstones. 2 patients required cholecystectomy.
Conclusion: Cholelithiasis is a common complication of CF disease; its incidence is more than the general population. Thus, we recommend that every CF patient get an ultrasonography study as part of liver disease screening to rule out any Gallbladder pathology.
Heller’s medial canaliform dystrophy (MCD) is a rare entity characterized by the paramedian crest formation of one or both thumbnails.
We report a case of a 13-year-old child who was used to biting thumbnails during several stressful events and diagnosed as medial ductal dystrophy of the thumbnail.
Mesenteric cyst is presented as lump abdomen or detected incidentally in ultrasonography for other reason or when it becomes symptomatic due to complications. Spontaneous infection or rupture of the mesenteric cyst and disseminated infections are very rare complications in children. Expeditious diagnosis and surgical excision of the cyst are imperative for fruitful outcome. We report a case of a perforated mesenteric cyst with sepsis and intracerebral haemorrhage in a 9 month child who was managed successfully in our institute.
An umbilical cord hernia (UCH) is a form of abdominal wall defect, affecting 6 out of every 10,000 newborns. The persistence of urachus is an embryonic remnant that connects the bladder to the abdominal wall at the level of the umbilicus, being yet more uncommon. We reviewed the literature, searching in PubMed, under the terms “Hernia of umbilical cord”, “Congenital hernia of cord” and “Persistent Urachus”. Only a few similar cases of both pathologies associated described were found. Our main objective is to highlight the distinct clinical features, embryogenesis, prognosis and associated anomalies of two infrequent embryopathies. And to describe a infrequent case of both abnormalities presenting simultaneously.
UCH are often misdiagnosed with other abdominal wall deffects, such as omphalocele, umbilical hernias, gastroschisis and umbilical cord cysts. The normal cord insertion, adequate muscle development of the abdominal wall and a wall defect less than 5cm is what differentiates it from an omphalocele. UCH has a low morbidity overall, as it is not associated with other anomalies. The most frequently observed urachal malformations are the persistence of a urachus and urachal cyst. The prenatal diagnosis of patent urachus is made by ultrasound or magnetic resonance, being easily mistaken with abdominal wall defects, confirming the diagnosis with an ultrasound at birth. The persistence of urachus may resolve spontaneously, if not, surgical resolution is recommended. Similar to a UCH, a patent urachus shows little association with other malformations.
It is important to know the clinical presentation and the diagnostic perinatal methods employed for appropriate management and favorable results for both pathologies. This relies on knowing when to suspect possible associated anomalies and when complementary studies might be needed. It is also important to be aware that there is the possibility of a UCH and a patent urachus existing simultaneously.