Journal of Comprehensive Pediatrics
Volume:4 Issue: 3, Aug 2013

Liver disease is increasingly common in pediatric cystic fibrosis (CF). Liver dysfunction in CF patients is an early complication and relatively common which may progress silently.. The purpose of this study was to determine the prevalence of abnormal liver architecture by Ultrasonography (US) and their associations to abnormal liver function tests particularly abnormal Gamma Glutamyl Transpeptidase (GGT), Alanine Aminotransferase (ALT), and Aspartate Aminotransferase (AST) level for its early detection before the complications occur..Patients and This study as a cross-sectional study was performed at the Children''s Medical Center Hospital, Pediatrics Center of Excellence in Tehran, Iran. In all, 114 patients with cystic fibrosis (70 boys, 44 girls) were enrolled. Sample blood test including AST, ALT, GGT and abdominal Sonography was obtained from all patients. Abnormal liver function test was defined by two consecutive occasions; ALT and/or AST levels were ≥ 2 times the upper limit of normal values. GGT normal values were defined by patient age. Data were analyzed using χ2 test and independent T test. Statistical significance was defined as P values of < 0.05 by SPSS ver.19 software.. Abnormal liver function test was detected in CF patients. As well, liver sonogram was abnormal in approximately one-third of the patients. This study showed a higher prevalence of biochemical abnormality in patients with abnormal livers ultrasonography.. Noninvasive paraclinical evaluation methods could be recommended in the patients with CF for early detection of silent liver abnormalities before progression to end stage liver disease..

Febrile convulsion (FC) is the most common type of seizure that occurs in children aged 6-60 months. It occurs in two forms including simple and complex febrile seizure.. The purpose of this study was to determine the clinical, epidemiologic and laboratory features of patients admitted to Ali-ebne-Abitaleb hospital of Zahedan with febrile seizure in 2010-2011..Patients and In this cross-sectional study, the patients with diagnosis of febrile seizure were the target population. We obtained patient’s data based on clinical examination, history and information registered in hospital medical files including demographic, clinical and laboratory findings.. During the study period, 160 children with febrile seizures and mean age of 26.2 ± 19.5 months were studied. Male to female ratio was 1.2:1. Ninety-nine (61.9%) children had simple seizures and 61 (38.1%) children had complex seizures. Thirty-one (19.4%) of the patients had family history of febrile seizures and family history of epilepsy was positive in 6.3% of cases. Ninety one percent of cases were born with normal vaginal delivery. Also, only 5 patients (3.1%) had less than 37 weeks of gestational age at birth. The mean rectal temperature of the patients was 38.5 ± 0.67 ºC. Gastroenteritis was the most common cause of fever in our patients.. The highest frequency of FC was seen in younger than 20-month-old children. Except for the lower incidence of positive history of prematurity and higher prevalence of gastroenteritis, results of the present study are relatively similar to other studies..

Oral mucositis is a frequent problem after high-dose chemotherapy which could impair patient’s quality of life, higher rates of infections, and delay in subsequent chemotherapy.. The present study was performed to assess the effect of topical application of pure natural honey in prevention of chemotherapy induced mucositis in patients with cancer..Patients and In this randomized clinical trial 48 patients with acute leukemia requiring chemotherapy were assigned to three equal groups. During induction or reinduction period of chemotherapy, first group received honey plus normal saline; the second one received just normal saline and third, (in control group) did not receive any prophylaxis. Patients were evaluated weekly for progression of mucositis according to the WHO mucositis scale. Data were analyzed with the Pearson Chi-Square and Fisher Exact test, by fifteen edition of SPSS software.. In group of ‘honey plus normal saline’ no patients developed mucositis, while in normal saline group 4 patients and in control group 12 patients developed mucositis. The rate of mucositis was significantly lower in both intervention groups compared to control (P < 0.001).. This study demonstrates that using either honey or normal saline can reduce the rate and severity of chemotherapy induced mucositis..

Wilson’s disease is an autosomal recessive disorder, characterized by a disturbance in copper metabolism that leads to copper overload in different tissues of the body. Because of various manifestations of Wilson’s disease, physicians should have high index suspicion when patients manifest any type of liver disease, neurologic and psychiatric signs and symptoms.. A 7.5-year-old boy was referred, presenting with generalized pruritus and stammer since the age of 4. Initial evaluation showed abnormal liver enzymes. Abdominal sonography revealed multiple echogenic lesions without acoustic shadow in the gallbladder, which was suggestive for gallstone. After about two years, he suffered from weakness, drowsiness, fever, nausea, epistaxis and abnormal liver function tests. With through clinical and laboratory work up, Wilson’s disease was finally diagnosed and appropriate treatment was started. Acceptable response to treatment was achieved.. Wilson’s disease has a wide range of manifestation so physicians should have high index suspicion when patients present any type of liver disease, neurologic and psychiatric signs and symptoms. Any delay in diagnosis or management can result in catastrophic outcomes..

Ectopic cervical thymic cyst is a very rare cause of neck masses in children. The diagnosis is sometimes very difficult and preoperative, correctly diagnosed thymic cysts in the neck are very low.. We report a 5-year-old boy with a left sided cervical mass and respiratory distress following a common cold, and ultrasound and CT scan revealed a cystic mass in the left neck with extension to the anterior mediastinum, suggesting cystic hygroma. After complete resection of the cyst and its extension, pathology report demonstrated simple thymus cyst.. Thymic cyst must be considered in differential diagnosis of a young child with a cervical mass..

Bronchial adenoma is rare in primary tumors of the lung and produce non-specific respiratory symptoms.. A 4-year-old boy referred to our clinic with chronic cough and wheezing. Foreign body aspiration was recommended for him on the base of radiologic and clinical manifestation. In bronchoscopy, foreign body was not detected, but an obstructing tumor was found at left lower lobe bronchial orifice. Trans bronchial biopsy, revealed a histopathologically low grade mucoepidermoid carcinoma. He underwent left lower lobectomy with safe margin.. However, respiratory tract tumors are rare in children but can masquerade foreign body in this group. We suggest that in children of recurrent pneumonia with persistent lung infiltration, bronchial tumor should be considered..

Primary pulmonary hypertension (PPH) is an uncommon disease in children. This pulmonary vascular disease may be idiopathic, familial or a kind of hereditary systemic vasculopathy. Multisystemic vascular diseases in children are rare and may be under-recognized. A few reports have described the involvement of cerebral vessels such as moyamoya disease (MD) in patients with PPH. MD is a unique cerebrovasculopathy, characterized by occlusion of the internal carotid arteries.. We reported a 2-year-old boy with PPH, who presented unusual and intractable seizure which suggested the diagnosis of MD. We also reviewed a few previous reports about the association of PPH with moyamoya disease.. Moyamoya disease is a kind of cerebral vasculopathy and should be considered as a differential diagnosis for patients with unusual seizure attacks who are suffering from primary pulmonary hypertension..

Lumbar puncture (LP) is a commonly needed procedure in pediatric medicine. In teaching hospitals, it is usually taught to junior residents by senior residents.. The goal of this study was to determine the dexterity of pediatrics residents in performing this procedure..Patients and All pediatric residents of Shiraz University of Medical Sciences were enrolled in this study. A presumptive case, a 6-year-old patient, suspected of meningitis was presented to them and they were asked to perform lumbar puncture on a manikin while they were observed by two attending physicians. A check list containing 14 items was completed for each participant and finally data were analyzed statistically.. The part of lumbar punctures least considered by pediatric residents was using sedation. There were significant differences between residents of different levels regarding sedating the patients and performing LP. However, there were no significant differences between them in regards to infection control and preparing the patient before starting lumbar puncture. Male and female residents were not different in performing any part of lumbar puncture.. Pediatrics residents’ dexterity in performing lumbar punctures is not ideal. Many of them do not consider using of sedation prior to performing lumbar punctures. With increasing years of education, their attention to sedation of the patient and their technique of LP improves, but their competence regarding infection control does not. It is necessary for them to learn this procedure by frequent theoretical and practical learning sessions..