Hamonet C*, Ducret L and Bahloul H

DOI : 10.32474/PAPN.2019.02.000139

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Ehlers-Danlos syndrome is a frequent hereditary disease that affects all the connective tissue and is transmitted to all the children of an affected person. A diagnosis is possible, from birth, or shortly thereafter, when observing a clubfoot, hip dislocation, intestinal intussusception, acute umbilical or inguinal parietal hernia, hemorrhages [cutaneous, oral, gastric, intestinal, nasal], persistent constipation, regurgitation and vomiting during bottle-feeding, or false roads. These symptoms are often the cause of false allegations of abuse with children withdrawal and wrongful parents or false accusations of Munchausen disease “by delegation” in a parent, the mother most often...... ReadMore

RF Sartori*, N. CValentini, GC Nobre and RP Fonseca

DOI : 10.32474/PAPN.2019.02.000138

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Objective: The aim of this study was to describe the tests used to assess working memory, inhibition, and cognitive flexibility, and analyzed the main research results related to executive functions in children with Developmental Coordination Disorder or with poor motor skills. Methods: This review used as data sources studies in MEDLINE, Web of Science, APA PsycNET, EMBASE, and Google Scholar with children with poor motor skills, DCD, and typical development. Quality of the studies was conducted using the Newcastle Ottawa Scale. Results: 1475 papers found, 31 matched the review criteria; 31 different executive function tests used to assess children with poor motor skills and DCD. Across the 31 studies, included in this systematic review, eleven studies examined only the working memory; ten studies measured only inhibition, a single study..... ReadMore

ZhihuiRong, Wei Liu, Ling Chen and Liwen Chang*

DOI : 10.32474/PAPN.2019.02.000137

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Pulmonary hemorrhage (PH) is a life threatening condition in newborns, especially preterm infants. Surfactant replacement might be helpful to improve lung function and reduce the ventilation support in infants with PH. We report two premature infants (one with early onset PH and one with late onset PH) who have been successfully rescued by administering surfactant (Calsurf ) after an acute episode of bleeding. Both of the 2 infants survived, and surfactant administration showed improvement in mean airway pressure and oxygenation indices. In the current study Calsurf was found effective in the treatment of both early PH and late PH in premature infants. There were no potential side effects were found in our study...... ReadMore

Mehrdad Mirzarahimi, Manouchehr Barak*, Shima Sarfarazi Moghaddam and Efsaneh Enteshari Moghaddam

DOI : 10.32474/PAPN.2019.02.000136

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Objective: To investigate the effect of USDA in the treatment of indirect hyperbilirubinemia of neonates. Study design: This randomized clinical trial was conducted on neonates with indirect hyperbilirubinemia whom were hospitalized at Ardabil city hospital. 100 newborns were enrolled in the study and randomly divided into two groups. The treatment group (n=50) received 10 mg/kg/day divided q12h USDA in addition to phototherapy, while the control group (n=50) only received phototherapy. Total bilirubin levels were measured every 12 hours until reaching bilirubin below 10mg/dL. Also, duration of phototherapy and hospitalization in both groups were recorded. Data were analyzed in SPSS.21 using statistical methods. Results: There was no difference in the mean of total bilirubin between treat..... ReadMore

Santosh Kumar K*

DOI : 10.32474/PAPN.2018.02.000134

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Apnoea of prematurity (AOP) is a common problem affecting premature infants. Apnoea is defined as cessation of breathing for more than 20 seconds or for lesser duration when associated with bradycardia and desaturation. The most likely and accepted pathogenesis is the “physiologic” immaturity of respiratory control in the neonates. The physiological immaturities include altered ventilatory responses to hypoxia, hypercapnia, and altered sleep states. Other hypotheses include gastroesophageal reflux and anaemia which are still controversial. Three types have been identified namely obstructive, central and mixed types of apnoea. Management options include the use of supplemental oxygen, position chages and drug therapy with methyxanthines. Other supportive therapies include kangaroo care, packed cell transfusions. The long term neu..... ReadMore

Edwin Dias* and Anusha Dias

DOI : 10.32474/PAPN.2019.02.000133

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Ifosfamide encephalopathy is a fatal toxicity of Ifosfamide therapy. 13 cases have been reported till the year of 2018 worldwide in children and none among Ewing’s sarcoma cases. Here we are discussing a unique case of Ifosfamide encephalopathy during the course of treatment of Ewing’s sarcoma and was on Ifosfamide therapy and developed encephalopathy. Child improved symptomatically with MESNA and mannitol. Child was found to be normal on follow up and chemotherapy treatment was continued....... ReadMore

Ghassan Saleh Ahmed Salama* and Yasser Fouad Mahmoud Amer

DOI : 10.32474/PAPN.2018.02.000132

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Objective: To examine effects of early amino acid (AA) infusion on serum glucose and calcium levels of very low birth weight (VLBW) preterm newborns, through the first 72 hours of life. Study Design: hospital designed prospective study of total 110 VLBW newborns with < 32 weeks gestation, and less than 1500 g was performed. 55 newborns received AA within 6 hours of life (early group) while 55 newborns did not receive AA during the first 72 hours of life (late group). Results: Average glucose levels were significantly lower in the early group during the first 72 hours of life. Average serum Ca levels during first 72 hours of life were significantly higher in newborns with early group. Conclusions: Early amino acid infusion improve serum glucose and Ca levels in VLBW newborns, and thus decreasing the risk of hyperglycemia and hypoca..... ReadMore

Jeanne M Cartier*

DOI : 10.32474/PAPN.2019.02.000131

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Within the practice of pediatric medicine, a family and social history has historically been a part of the assessment of children. However, the scope of said history was usually limited to understanding the family composition, family health history and developmental behavior and activities of the child. Ongoing advances in basic and clinical sciences have increased knowledge of underlying disease etiology, including the role of genetics and genomics, brain physiology and immune functioning in disease origins and development, have led to the discovery of new and effective pharmacological agents and have informed the development of evidenced based treatment protocols. These advances have clearly improved t..... ReadMore

Ponnambalam Arun*, Kaveri Krishnasamic, Palani Gunasekeranc and Vidya Padmanabhan

DOI : 10.32474/PAPN.2019.02.000130

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Diarrheal diseases continue to be largest and deadliest ones globally especially affecting children. From earlier days there is renewed interest in the discovery of novel compounds that can be used to fight these kinds of diseases using plants and its materials. Rotavirus has been recognized as the major etiological agent of diarrheal diseases of young children in developed as well as developing countries. The presence of various phytochemicals like flavonoids, tannins, saponins, alkaloids and terpenes present in the active extracts increase the colonic water and electrolyte reabsorption and result in treatment of diarrhea. This study was undertaken to know about traditional herbal plant having medicinal value. Euphorbia hirta was analyzed for the presence of phytochemical and cytotoxic activity using MA104 cell line. Non toxic dose..... ReadMore

Anwar A Mithwani*, Adnan A Mithwani, Muhammad Ziad Shama, Assem Ahmad Kadrey, Abdullah Alomar Almeshrif and Amerullah Malik

DOI : 10.32474/PAPN.2019.02.000129

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Grieg cephalopolysyndactyly syndrome (GCPS) is a rare congenital genetic disorder present at birth, characterized through physical abnormalities, primarily affecting the development of the limbs, head, and face (craniofacial malformations). We report a case of a full term, newborn female, large for gestational age with 4.2 kg. Symptoms included transient tachypnea of the newborn and craniofacial dysmorphism. The mother was G5P4 with gestational diabetes, and a reported case of polyhydramnios but did not have any previously affected babies. The craniofacial dysmorphism anomalies consisted of macrocephaly (Head circumference of 39 cm with a standard deviation of 2+ for her age), frontal bossing with a broad forehead, a groove between the frontal bone, widely spaced eyes, bilateral polysyndactyly, a thumb and an extra digit fu..... ReadMore

Khalil Salameh*, Abedal khalik Ahmad Khedr, Rajesh Pattu Valappil and Ahmed Hosny Ahmed Tomerak

DOI : 10.32474/PAPN.2019.02.000128

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ver use of antibiotics lead to resistance and unwanted side-effects in newborns. Since 1992, professional societies or public health agencies have issued several generations of recommendations for prevention or management of early-onset neonatal sepsis (EOS) [1-5]. Despite these efforts, recommendations remain inconsistent, clarifications are necessary, local adaptations are common, and compliance rates are low [6-7]...... ReadMore

Shubhankar Mishra*

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Juvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against components of the postsynaptic membrane of the neuromuscular junction. The patients present with a wide range of symptoms-from isolated intermittent ocular symptoms to general muscle weakness with or without respiratory insufficiency. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. It must be differentiated from congenital myasthenia which is a channelopathy rather than autoimmune disease. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunogl..... ReadMore

Naveen Ganapan and Charul Mehta*

DOI : 10.32474/PAPN.2018.02.000126

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Aim: To study the disease control via pulmonary function test with usage of bronchodilators in bronchial asthma patients. Setting and design: Prospective cohort study done at Asthma clinic, Dept of pediatrics, BJ medical college, Ahmedabad. Materials and Methods: Patients enrolled in this study were from asthma clinic BJ medical college and Civil hospital. Pulmonary function test was done on total of 91 known asthma patients. Here three parameter of lung function is taken in to account. Percentage OD FEV1 of predicted, FEV1:FVC Ratio and PEFR , its effect on bronchodilators on PEFR. Results: Out of 91 patients, 72 (79.25%) patients had almost normal pulmonary function. Only 1 patient had severe restriction of pulmonary function and 18 patients had moderate restriction of lung function. All patients shows improvement in PEFR more than 2..... ReadMore

Yaëlle Chaudy*, Thomas M Connolly and Brian Magowan

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Clinical Decision Support (CDS) is a growing field and the technology is increasingly used by both clinicians and patients. In maternity care, numerous guidelines exist on risk assessment and proposed care plans during pregnancy and labour. However, as new evidence arise, these guidelines are subject to change. It is time consuming for clinicians to a. Compile all this information and b. Keep it up to date. This paper presents our approach to overcoming these two issues: the SAFER (Safe Assessment Form to Evaluate Risk) maternity system. This CDS system contains rules extracted from current guidelines on maternity care and generates care plans at various stages of gestation based on patient data. The system includes a mobile application and a web interface. The mobile application can be used to visualise care plans and edit patient dat..... ReadMore

Shubhankar Mishra

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Neurodegenerative disorders of childhood are complicated diseases with wide range of systematic involvement. These diseases often pose great challenge to clinicians in terms of diagnosis and management. The purpose of this article is to outline a systematic approach to a child presenting with suspected neurodevelopmental regression. Many inherited metabolic disorders present with neural regression. The clinical approach depends upon the age of presentation, site of involvement in brain. Sound clinical knowledge and better approach leads to early diagnosis, better management and above all genetic counselling. As the medical science is in the track of rapid progression several treatment modalities are in the pipeline for neurodegenerative syndromes, early diagnosis and referral to higher centres can bring a better future to the child...... ReadMore

Khatuna Lomauri*

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Neonatal chylothorax results from the accumulation of chyle in the pleural space and may be either congenital or an acquired condition. Congenital chylothorax is most likely due to abnormal development or obstruction of the lymphatic system and often associated with hydropsfetalis. It can be idiopathic or may be associated with various chromosomal and other genetic abnormalities. It is important to identify infants with chylothorax, as there are specific issues that need to be addressed in the management of these patients...... ReadMore

Wala Ben Kridis*, Ines Werda, Rim Kallel, Nabil Toumi, Tahia Boudawara, Afef Khanfir and Mounir Frikha

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Neuroblastoma is a relatively common pediatric pathology of the sympathetic nervous system. The particularity of neuroblastoma lies in its development from cells whose embryological maturation into adult sympathetic nerve cells or chromaffin cells is incomplete. In the literature, many cases of spontaneous regression of neuroblastoma have been reported, but the mechanisms for which they are responsible remain ambiguous. The specificity of our case is the benign evolution of an osteomedullary stage 4 neuroblastoma and its maturation into a ganglioneuroma 6 years after the end of chemotherapy. This transformation depends on various factors related to the tumor (such as the over expression or not of N-myc, the presence or absence of Treks and their receptors), the host (the intervention of the immune system) and to other external..... ReadMore

George Gregory Buttigieg KM

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Modern medico-legal evaluation of Neonatal Cerebral Palsy (NCP) demands recognition of Neonatal Hypoxic Ischaemic Encephalopathy (NHIE) as an indispensable proof of peri-partum fetal hypoxia. Without proven NHIE, modern jurisprudence should not even consider obstetric/paediatric liability of causative peri-partum hypoxia of negligence on the grounds of medically responsible fetal/newborn hypoxia leading to Cerebral Palsy...... ReadMore

Santosh Kumar K*, Mangalabharathi S and Kamalarathnam CN

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Objective: To study the spectral characteristics of the phototherapy systems and to identify variations in spectral properties with changes in height, voltage, tilt and temperature output. Setting: Research laboratory at a tertiary care hospital. Methods: The spectral characteristics were measured from two compact fluorescent lamp (CFL) and eight light emitting diode (LED) phototherapy devices in a dark room with an irradiance of <0.1µW/cm2 /nm. The variations in the spectral characteristics with changes in height, tilt and voltage were measured. Heat output was documented...... ReadMore

İsmail Aytac

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Loeys-Dietz Syndrome is a otosomal dominant connective tissue disorder which has characteristic triad with a) Arterial tortuosity, aneurysms or dissections; b) Hypertelorism; and c) Bifid uvula or cleft palate. This review summarizes the features of anesthesia management in this syndrome..... ReadMore