SM Journal of Pediatrics

Archive Articles

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Intraperitoneal Abscess in a Preterm Infant: a Case Report

Developments in empiric antibiotic therapy in recent decade shave led to a decrease in idiopathic primary peritonitis in neonates. Furthermore, intraperitoneal abscesses are extremely rare in neonates. We report a case of an intraperitoneal abscess without an apparent cause, such as necrotizing enterocolitis or surgery, in a preterm infant. The main contribution of our report is a reminder that intra-abdominal abscesses, although rare, can and do occur in neonates, and that diagnosing them can be difficult.

Susumu Yamazaki¹*, Natsuki Ohkawa¹, Masato Kantake¹, Mariko Koyama², Naoto Urushihara², Minoru Hamazaki³, and Toshiaki Shimizu⁴

 


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Angiotensin Converting Enzyme Inhibition in those with Functionally Univentricular Hearts: is there a Problem with Overuse?

Angiotensin converting enzyme inhibitors are being increasingly used in those with functionally univentricular hearts with minimal evidence of their efficacy in this population. Additional medications, such as angiotensin converting enzyme inhibitors can be a practical inconvenience to patients, increase the cost of care, and may even be detrimental to these patients in some respects. This short communication reviews the current evidence for angiotensin converting enzyme inhibition in patients with functionally univentricular hearts.

Rohit S Loomba*


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Classifying Injury Causation in Interim Medico-Legal Reports Following Forensic Evaluation of Suspicious Childhood Injury

Paediatricians with child protection responsibilities evaluate allegations of physical harm and sexual abuse which are subject to both child protection and criminal investigations. This evaluation requires the production of an interim medico-legal report which contributes to interim decisions regarding the safety of the child whilst investigations are still in progress. A second report may be needed at a later time once investigations are considered complete. The interim medico-legal report contributes an expert opinion about injury causation including direct harm or harm through neglect or substandard care which has relevance to mitigating future risk of harm and understanding the support needs of a family. This paper defines key principles in relation to the structure and content of an interim medico-legal report, which must conform to forensic standards of practice. A classification of injury causation for interim medico-legal reports is proposed which can be useful to systematically define outcomes for data collection, audit and research purposes.

Catherine Skellern¹,²* and Jan Connors¹,²


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Growth Retardation and General Hypotonia Revealing Vitamin B12 Deficiency

Vitamin B 12 has important consequences for hematopoiesis and the central nervous system, its deficiency leads to clinical hematological, neurological and psychiatric manifestations. We report the case of a ten months old male infant, exclusively breastfed, with a significant delay in the developmental and communicative reactions. Physical examination confirmed the pallor, the axial hypotonia and revealed that the child was below the 2nd percentile for weight and the 3rd percentile for length. He had a hemoglobin level of 6.4g/dL with an MCV of 100.2fL, His serum vitamin B12 level was less than 50pg/mL and serum folate level 20.4ng/mL. No intrinsic factor antibodies were found. Hormonal and nutritional screening revealed no other deficiency. Thalassemia screening resulted negative. Maternal investigations showed anemia and maternal vitamin B12 deficiency. The research for anti-parietal antibodies of the stomach returns very positive. Vitamin B12 supplementation permitted with weight gain and rapid improvement of interactions and muscle tone.

Salahiddine Saghir*, Toufik Meskini, Said Ettair, Naima Erreimi and Nezha Mouane


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A Case of Reverse Shapiro Syndrome Responding to Cyprohepatadine

Reverse Shapiro syndrome consists of the association of unexplained hyperthermia and corpus callosum agenesis. The genetic basis is still unidentified; yet, neurochemical abnormalities as well as hypothalamic dysfunction have been proposed as possible etiologies. Only four cases of reverse Shapiro Syndrome have been reported to date and many therapeutic managements have been proposed without any radical success. Here we report a new case of a 6 months old male patient, with controlled cryptogenic epilepsy who is also presenting with a reverse Shapiro syndrome. The patient presented with recurrent episodes of hyperthermia that responded completely to a treatment by cyproheptadine.

Hicham Mansour*, Maha Sabouneh and Ghassan Hmaimess


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Severe Staphylococcal Scalded Skin Syndrome Following Purulent Conjunctivitis

Staphylococcal Scalded Skin Syndrome (SSSS) is an uncommon toxin-mediated disease which causes blistering and desquamation of the skin and induced by Staphylococcus aureus (S. aureus). SSSS in neonates is a rare condition and most commonly seen in children aged 6 months to 5 years, with the highest probability of disease being between 2-3 years. This report presents a 35-day-old infant who developed exfoliation and peeling of the skin after purulent conjunctivitis.

Esra Türe¹*, Abdullah Yazar¹, and Fatih Akın¹


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Commentary: Environmental Medicine: The Role of Epigenetic Mechanisms

With the current emphasis on delivering accurate diagnosis, prognosis and treatments to prevent and treat human diseases, it is critical that one understands the mechanistic bases for the pathogenesis of both acute and chronic diseases. Although “Environmental Medicine” involves the whole spectrum of clinical disciplines from obstetrics, pediatrics to geriatrics and involves the potential roles of genetics in the onset of any diseases that to be associated with some environmental factor(s), the roles of “epigenetic” toxicological mechanisms seems to be largely ignored in “personalized medicine”, or “precision medicine” where the use of genetic information has played a major role in certain aspects of personalized medicine. With the recent development of sophisticated molecular technologies, various current paradigms, some having origins in past insights to the origin of “genetic or heredity diseases” or in the discovery of environmental agents (radiation, toxic chemicals or pathogenic biological organisms), there has been some confusions of how these agents contribute to these diseases.

A short “Commentary” is proposed to assist in sorting out these various factors contributing to “genetic” and “environmental” causes of these diseases. In brief, it will be assumed that the singular concept that genes or environmental factors, alone, are not “the” cause of any disease, but that complex interactions are needed to affect disease pathogeneses. This will involve our current understanding that mutations (gene or chromosomal), cell death and alterations of gene expression at the transcriptional, translational and posttranslational levels (epigenetic change) are involved in the toxicological mechanisms of the pathogenesis of birth defects, cancer, cardiovascular diseases, inflammatory diseases, reproductive- and neurological-disorders. Much of our global disease burden is the result of a collision of glacial-speed biological evolution of genes needed for survival and reproduction with the laser speed cultural evolution. Lastly, with the recent discovery of organ-specific adult stem cells, alteration of the numbers (increase or decrease) of these organ-specific stem cells could provide the mechanistic basis for the risk of various stem-cell related diseases later in life (The Barker hypothesis)

James E Trosko*


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Development of a Method to Use a Color Tracker for Motor Therapy for Individuals with Rett Syndrome

Background and Objectives: Rett Syndrome (RTT) is a development disorder with a known genetic origin that causes a child to lose purposeful use of their hands and develop characteristic stereotypical repetitive hand movements. Through the use of Virtual Reality (VR), the frequency, length, and intensity of therapy sessions can be enhanced, leading to improved upper extremity function.

Methods: The objective was to develop a VR system, namely VR_Color Tracker, specifically tailored for persons with RTT and designed to decrease the characteristic stereotypical repetitive hand movements and increase the use of hands in skilled function. The VR_Color Tracker was created, tested, revised, and retested through the use of a multi-level, iterative development process. Limitations such as technological restrictions and effective tracking were addressed prior to testing with a RTT participant.

Results: A feasibility investigation was carried out, a System Usability Scale (SUS) was completed with the researchers, and the system’s usability was determined to be in the seventieth percentile.

Conclusions: The VR_Color Tracker is feasible to use for an individualized approach to RTT therapy, but more research is needed to determine its clinical efficacy

Nicole E. McAmis¹, Matthew H. Foreman¹, Molly D. Himmelrich², Pamela S. Diener², and Jack R. Engsberg¹*


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Outcomes and Prognostic Factors for Non-metastatic Osteosarcoma of the Extremity

Purpose: To assess the impact of several patient-related and treatment-related prognostic factors in patients with localized osteosarcoma of the extremities treated at a single institution with the same chemotherapy protocol over a three-year period.

Patients and Methods: We reviewed 57 patients with localized osteosarcoma of the extremities treated at the Children’s Cancer Hospital, Egypt, between July 2007 and December 2010, with a minimum follow-up of one year. Gender, age, tumor site and size, type of surgery, surgical margins, serum level of alkaline phosphatase and histologic response to preoperative treatment were retrospectively evaluated.

Results: With a median follow-up of 23 months, the three-year Event-Free Survival (EFS) and overall survival rates were 70.5% and 77.8 %, respectively. In univariate analysis, EFS was significantly related to the age of patients, serum level of alkaline phosphatase, and tumor volume but not to histologic subtype or histologic response to preoperative treatment. In multivariate analysis, only gender and serum alkaline phosphatase were statistically significant. Local and systemic recurrences occurred in 12 patients (21%) (10 developed pulmonary metastasis, one patient developed both bone and pulmonary metastases, and one patient had local recurrence). Median time to recurrence was 9.2 months, and recurrence correlated with the histologic response to preoperative treatment, tumor volume and serum alkaline phosphatase.

Conclusion: The three- year EFS and overall survival were 70.5% and 77.8%, respectively. Age, tumor volume, and the serum level of alkaline phosphatase significantly influenced EFS. These factors should be considered when deciding on risk-adapted treatments for osteosarcoma patients. However, gender and serum alkaline phosphatase were the only significant variables in the multivariate analysis

Manal A. Zamzam¹, Emad Ali H. Moussa², Ahmed El Ghoneimy³, Magdy El Sherbiny³, Mohamed S. Zagloul⁴, Hala Taha⁵, Iman Goda⁵, Sameera Ezzat⁶, and Ranin Soliman⁷*


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Association of Post-Surgical Lactate Levels with Morbidity and Mortality in Pediatric Patients Undergoing Extracorporeal Circulation Pump

Lactate is a final product of anaerobic metabolism its accumulation can produce cellular and organic dysfunction of all systems leading to lactic acidosis [1]. Access to its measurement in hospitals has allowed it to be used as a marker of tissue hypoperfusion in multiple pathologies. This marker has been studied as a prognostic factor in patients with sepsis, septic shock, but has not been studied in patients undergoing surgical correction of cardiac malformations [2].

María del Pilar Cejudo-García de Alba¹, Jaime Valle Leal¹*, Jesús Gabriel Sánchez Beltrán², Amado Jesús Francisco Vázquez-Amparano¹, and Jesús Manuel Armenta Velderrain¹