Abnormalities In Pediatric Patients Research Articles

Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections

Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by cardiac catheterization. Although angiography has been the gold standard for evaluation it carries certain inherent risks, especially in small and sick infants. In this study we retrospectively assessed the utility of magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the evaluation of pulmonary venous abnormalities in pediatric patients. The results were compared with operative findings. We reviewed nine patients with total APVC and four with partial APVC. Twelve patients were infant (age range: 1 day to 8 months) and the other was 16 years old. MRA and/or CTA clearly visualized the anomalous drainage of pulmonary veins in all patients. Cardiac catheterization was performed five patients with one total anomalous pulmonary venous connection and four with partial anomalous pulmonary venous connection. Ten patients were operated on. Diagnoses of patients were confirmed by operative findings. In Conclusions, MR and CT angiography allow detailed and comprehensive evaluation of the APVC and are good diagnostic modalities for use in the preoperative assessment of the anomaly in pediatric patients.

Noninvasive detection of coronary abnormalities in pediatric patients with Kawasaki disease using multi-slice spiral CT

目的 评价多层螺旋CT(MSCT)在小儿川崎病冠状动脉损害中应用的可行性及价值.方法 34例川崎病患儿进行16层或64层CT冠状动脉增强扫描,其中18例有同期二维超声心动图检查对照.计算各支冠状动脉的显示情况.观察病变形态,测量左、右冠状动脉主干的直径,计算2种方法的相关系数.结果 除4例左旋支及8例右冠状动脉中段显示不清外,其余冠状动脉均可清楚显示.MSCT发现34例患者中,14例有冠状动脉损害,共22支冠状动脉受累.冠状动脉主干扩张6例,主干扩张伴动脉瘤形成3例,主干未扩张而中远段有动脉瘤形成2例,冠状动脉狭窄1例,钙化2例,串珠状改变3例.MSCT及二维超声在冠状动脉主干测量值上差异无统计学意义(P＞0.05),左、右冠状动脉主干测量值的相关系数分别为0.85、0.91(P＜0.05).3例右冠状动脉中远段的动脉瘤及1例冠状动脉狭窄超声未发现.2例轻至中度二尖瓣反流及5例血管壁增厚MSCT未发现.结论 MSCT用于探查小儿川崎病冠状动脉损害是可行的,可以作为心脏超声的补充和替代方法用于川崎病冠状动脉损害的诊断及随访。

Approval Summary: Nelarabine for the Treatment of T-Cell Lymphoblastic Leukemia/Lymphoma

To describe the clinical studies, chemistry manufacturing and controls, and clinical pharmacology and toxicology that led to Food and Drug Administration approval of nelarabine (Arranon) for the treatment of T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma. Two phase 2 trials, one conducted in pediatric patients and the other in adult patients, were reviewed. The i.v. dose and schedule of nelarabine in the pediatric and adult studies was 650 mg/m2/d daily for 5 days and 1,500 mg/m2 on days 1, 3, and 5, respectively. Treatments were repeated every 21 days. Study end points were the rates of complete response (CR) and CR with incomplete hematologic or bone marrow recovery (CR*). The pediatric efficacy population consisted of 39 patients who had relapsed or had been refractory to two or more induction regimens. CR to nelarabine treatment was observed in 5 (13%) patients and CR+CR* was observed in 9 (23%) patients. The adult efficacy population consisted of 28 patients. CR to nelarabine treatment was observed in 5 (18%) patients and CR+CR* was observed in 6 (21%) patients. Neurologic toxicity was dose limiting for both pediatric and adult patients. Other severe toxicities included laboratory abnormalities in pediatric patients and gastrointestinal and pulmonary toxicities in adults. On October 28, 2005, the Food and Drug Administration granted accelerated approval for nelarabine for treatment of patients with relapsed or refractory T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma after at least two prior regimens. This use is based on the induction of CRs. The applicant will conduct postmarketing clinical trials to show clinical benefit (e.g., survival prolongation).

Diffusion-weighted MR imaging abnormalities in pediatric patients with surgically-treated intracranial mass lesions

Diffusion-weighted imaging (DWI) is a magnetic resonance imaging (MRI) technique that measures the degree of water diffusion in vivo. DWI abnormalities are frequently observed on immediate postoperative imaging following surgical resection of gliomas in adults. These abnormalities subsequently demonstrate contrast enhancement, which may be confused with lesion recurrence. The purpose of this study was to investigate the occurrence of these postoperative abnormalities in pediatric patients with intracranial mass lesions. Thirty-three consecutive patients <or=18 years old with a newly diagnosed intracranial mass lesion underwent MRI, including DWI, before and immediately after surgical treatment. The median patient age was 9.9 years (range 0.2-18 years). Supratentorial and infratentorial lesions were identified in 22 and 11 patients, respectively. Infiltrative and noninfiltrative, as well as benign and malignant lesions, were included. Postoperative imaging demonstrated areas of reduced diffusion adjacent to the resection cavity in 20 (61%) cases. The median volume of these areas was 1.7 cm3 (range 0.3 cm3-12.0 cm3). Subsequent imaging studies in 9 of the 18 cases showed contrast enhancement in the area corresponding to the DWI abnormality. There were no clinical deficits attributable to any of the diffusion abnormalities. There was no association between the occurrence of these abnormalities and whether the lesion was infiltrative, non-infiltrative, benign, or malignant. DWI abnormality on immediate postoperative MRI is common following surgery for newly diagnosed intracranial mass lesions in pediatric patients. Focal contrast enhancement in the postoperative period may be confused with recurrence for some lesions. Our study suggests that immediate postoperative DWI is useful in interpreting new areas of focal contrast enhancement on subsequent imaging in children who have had surgery for brain tumors.

A Comparison of MRCP and ERCP for Evaluation of Bile Duct and Pancreatic Duct Abnormalities in Pediatric Patients

Endoscopic Retrograde Cholangiopancreatography (ERCP) is considered as the gold standard in the evaluation of bile duct (BD) and pancreatic duct (PD) abnormalities. Magnetic Resonance Cholangiopancreatography (MRCP) is a viable alternative to ERCP. While MRCP is an established modality for evaluating pancreaticobiliary disorders in adults, there are no data in establishing its accuracy in the pediatric population. Method: Retrospective study comparing MRCP and ERCP findings of 15 studies done in 13 children (8 male & 7 female) obtained from 6/2002-5/2005.

The complex nature of the interaction between disease activity and therapy on the lipid profile in patients with pediatric systemic lupus erythematosus

To determine the prevalence of lipid abnormalities at different times and to determine the influence of both the disease and corticosteroid therapy on lipid abnormalities in pediatric patients with systemic lupus erythematosus (SLE). Lipid measurements were obtained in an inception cohort of 139 pediatric patients with SLE (114 females). Fasting levels of total cholesterol, triglycerides, low-density lipoprotein (LDL) cholesterol, and high-density lipoprotein (HDL) cholesterol in the SLE patients were compared with those in age- and sex-matched control subjects. Disease activity levels and medication dosages were obtained at the time of lipid measurements. At the time of diagnosis, the mean levels of total cholesterol, LDL cholesterol, and triglycerides were highest, whereas the mean levels of HDL cholesterol were lowest. The percentage of patients with abnormal triglyceride values was highest at diagnosis, decreased at year 1, and then remained relatively constant thereafter. The mean total cholesterol and LDL cholesterol levels decreased at year 1 as compared with the time of diagnosis and then remained relatively constant. The lowest mean HDL cholesterol levels were found at the time of diagnosis, and these values rose with time. Comparison of lipid levels at different prednisone dosages and disease activity levels revealed that changes in triglyceride levels were mainly associated with changes in disease activity, changes in both total cholesterol and LDL cholesterol levels were associated with changes in the prednisone dosage and not disease activity, and low levels of HDL cholesterol were associated with active SLE, whereas the prednisone dosage was associated with increased levels of HDL cholesterol. Factors intrinsic to SLE appear to alter lipid levels. Control of SLE may be the most important factor in improving abnormal lipid profiles, and paradoxically, prednisone therapy may improve abnormal lipid levels.

Magnetic resonance spectroscopic studies of pediatric mood disorders: a selective review

This review article presents a summary of the recent magnetic resonance spectroscopic studies of pediatric mood disorders. Magnetic resonance imaging is a promising tool for investigating the biologic substrates of neuropsychiatric disorders without the use of ionizing radiation. This novel technique has been recently applied to pediatric patients with mood disorders. Structural, chemical, and functional abnormalities in pediatric patients with mood disorders have been reported. To date, only modest numbers of pediatric studies using magnetic resonance imaging have been conducted. Most of these studies have small sample sizes and heterogeneous subject groups. These studies have provided important information regarding the underlying neuro-circuitry and pathophysiology of these complex and disabling disorders, however.

The usefulness of dynamically balanced FFE WATS-MR imaging with kinematic display for studying pediatric gastrointestinal disease

Abnormalities in gastrointestinal movement underlie various symptoms in the pediatric population. Although fluoroscopic, manometry, and ultrasonography remain important tools for studying gastrointestinal movement, none can depict the whole gastro-intestine full-size without radiation. The purpose of this study was to evaluate whether kinematic water selective excitation MR imaging (WATS-MR cine) could reveal gastrointestinal movement in childhood. Thirty-six patients (age: 3 to 17 years; 15 male and 22 female) underwent dynamically balanced first field echo (FFE) imaging with a thick slice of water selective excitation without breath hold. The WATS-MR cine was taken at three different times (preprandial, immediately postprandial, and 30-min postprandial) for sequential 5 min. Images were displayed in a cine mode at 10 times the normal speed, and the motion of water in each organ was evaluated. Movement of the stomach, small intestine, and colon could be observed together in a field. This study made it possible to evaluate both preprandial and postprandial gastrointestinal water movement. WATS-MR cine is useful for visualizing gastrointestinal water movement in accessible children older than 6 years without sedation. A cine mode at 10 times the normal speed provided characteristic images reflecting gastrointestinal water movement abnormalities in pediatric patients.

Cytogenetic effects in children treated with methylphenidate

In recent years there has been a surge in methylphenidate (Ritalin) use for treatment of attention deficit/hyperactivity disorder (ADHD) in children. However, there is a paucity of information on whether this drug poses any potential health risks, such as mutagenicity or carcinogenicity, for humans. To address this issue, we investigated whether this central nervous system stimulant produces cytogenetic abnormalities in pediatric patients at therapeutic levels. In a population composed of twelve children treated with therapeutic doses of methylphenidate, we analyzed three cytogenetic endpoints in peripheral blood lymphocytes obtained before and three months after initiation of treatment with this drug. In all participants, treatment induced a significant 3, 4.3 and 2.4-fold increase in chromosome aberrations, sister chromatid exchanges and micronuclei frequencies, respectively ( P=0.000 in all cases). These findings warrant further investigations of the possible health effects of methylphenidate in humans, especially in view of the well-documented relationship between elevated frequencies of chromosome aberrations and increased cancer risk.

Surface electromyographic studies of swallowing in normal children, age 4–12 years

Objectives: Surface electromyographic (sEMG) studies were performed on 100 normal children, age 4–12 years, to establish normative database for duration and amplitude of muscle activity during swallowing and continuous drinking. Study design: Prospective observational study of healthy volunteers. Methods: Parameters evaluated during swallowing include the timing and amplitude (in μV) of activity of m. orbicularis oris, masseter, submental and infrahyoid (laryngeal strap muscles (LSM)) groups covered by platisma. Four tests were examined: voluntary single swallows of saliva (“dry” swallow), voluntary single water swallows as normal, voluntary single swallows of excessive amount of water (up to 15 ml), continuous drinking of 50 ml of water (duration and number of swallows). Duration of muscle activity during swallowing (mean plus standard deviation (S.D.)) was measured for two age groups: 4–8 and 9–12 years old. The group of 40 adults, age 18–30 years, was taken as a control group. Results: Normative data for duration and amplitude of muscle activity during single swallowing and continuous drinking are established for healthy children. The duration of muscle activity during swallows and drinking in all tests showed decrease with the age, and this tendency is statistically significant (one-dimensional analysis of variance, SPSS, χ 2 criterion, 95% confidence interval). There was no statistically significant difference in amplitude (range) measurements between children and adults ( P=0.05). There was no statistically significant difference between male and female children duration of muscle activity during single swallowing and continuous drinking in all age groups ( P≥0.05). Conclusion: Surface EMG of swallowing is a simple and reliable noninvasive method for screening evaluation of swallowing with low level of discomfort of the examination. The normative timing of events data can be used for evaluation of complaints and symptoms, as well as for comparison purposes in pre- and postoperative stages and in electromyography (EMG) monitoring during ENT or neurological treatment. These parameters represent normal deglutition, and can be used to identify abnormalities in pediatric patients, and provide a basis for comparison of swallowing performance, both within and between patients.

Paranasal sinus computed tomographic findings of children with chronic cough

Objective: Chronic cough is a frequent problem in the practice of clinical pediatrics, and sinusitis is a common etiologic factor. In this study, our aim was to determine the prevalence of sinus abnormalities in pediatric patients presenting with chronic cough. Methods: Forty-two patients, ranging from 3 to 16 years of age from both sexes completed the study. Paranasal sinuses were examined by coronal paranasal sinus limited computed tomographic scan. The CT scan findings were categorized as no disease, minimal, moderate and severe sinusitis. Results: The most frequent symptoms after cough were rhinorrhea, sniffling, and halitosis. We found sinus abnormalities in 66.6% and no abnormality in 33.3% of the children. Age and sex were not important factors in sinus disease. There was no correlation between sinus findings and any of symptoms. The most common anatomical abnormalities were concha bullosa, paradoxical middle turbinate, Haller's cells, and deviated nasal septum. These abnormalities were not correlated with sinus disease. Conclusion: Paranasal sinus abnormalities are common among pediatric patients with chronic cough and imaging studies of the sinuses should be considered in these children.

Artificial neural network predicts CT scan abnormalities in pediatric patients with closed head injury.

Artificial neural networks (ANNs) use nonlinear statistical modeling techniques to explore relationships in complex clinical situations. This study compared predictive ability of a trained ANN model to that of physician prediction of cranial computed tomographic (CT) scan abnormalities in children with head injury. A prospective cohort of 351 patients who presented with head trauma and underwent CT scans were studied. All pertinent data on historical and demographic information, and clinical features were recorded. Emergency department physicians used clinical judgment to record pretest probability of abnormal CT scans for all patients prospectively. Similar data from a retrospective chart review of 382 patients with head injury in the immediate preceding year were collected and used to train the ANN. Data from the prospective study was used to validate the ANN, construct a logistic regression model, and compare physician prediction. Forty-five (12.9%) of 351 patients had abnormal CT scans. In predicting CT scan abnormality, the ANN model was more sensitive (82.2%) compared with physician prediction (62.2%). ANNs may serve as a useful aid for decision support for emergency physicians in predicting intracranial abnormalities in closed head injury.

Cloning and Initial Characterization of the Human DPYD Gene Promoter

Dihydropyrimidine dehydrogenase (DPD) is the rate-limiting enzyme in the degradation of pyrimidine bases and pyrimidine-based antimetabolites. Reduced DPD activity is associated with toxicity to 5-fluorouracil (5FU) therapy in cancer patients and with neurological abnormalities in paediatric patients. Although variant DPYD alleles have been identified in DPD-deficient patients, they do not adequately explain polymorphic DPD activity or associated clinical phenotypes in vivo. DPD may be transcriptionally regulated as mRNA levels correlate with activity and are differentially regulated in human tissues. A 1.85 kb 5′ flanking region of the human DPYD gene was cloned and has transcriptional activity in cultured cells. Analysis of this 5′ flanking region in rhesus and cynomolgus monkeys demonstrated conservation (>96%) between humans and primates. Putative binding sites for ubiquitous and cell-specific factors were identified. A polymorphism that disrupts a putative γ-interferon response element was identified in a cancer patient with reduced DPD activity and severe 5FU toxicity. Further insight into regulation of DPD expression may identify new avenues for the treatment of clinical problems associated with DPD deficiency.

Congenital airway abnormalities in patients requiring hospitalization.

To determine the cause of congenital airway abnormalities in pediatric patients requiring hospitalization for their respiratory status. Case series in a tertiary care center. A 5-year retrospective chart review was conducted at our institution. A total of 174 patients were identified who required hospitalization for their respiratory status as a result of a congenital airway abnormality. Of the 174 patients, 114 (65.5%) were male and 60 (34.5%) were female. Eighty patients (47%) presented within the first 3 months of life. Forty-six patients (26%) were born prematurely, and 49 patients (28%) were diagnosed as having gastroesophageal reflux. The majority of patients (139 [80%]) had multiple presenting symptoms or signs. Stridor was the most common (129 [74%]), followed by accessory respiratory effort, cyanosis, apnea, and failure to thrive. Diagnosis was made at the time of surgical evaluation in 91% of the patients, with the remaining diagnoses made using radiological findings and/or clinical evaluation. Sixty-five patients (37%) had multiple sites of airway abnormalities; laryngeal abnormalities were noted almost 3 times as often as tracheal abnormalities (161 vs 62, respectively). Of the laryngeal abnormalities, laryngomalacia was the most common, followed by glottic web, subglottic stenosis, vocal-cord paralysis, and subglottic hemangioma. Tracheomalacia was the most common tracheal abnormality, followed by external compression and tracheal stenosis. Thirty-three patients (19%) required tracheotomy for management of recurrent respiratory decompensation. While congenital airway abnormalities are usually self-limited, those patients requiring hospitalization represent a group with a more severe respiratory status who have a greater chance of requiring tracheotomy. The recognizable percentage of patients with gastroesophageal reflux and prematurity accounts for comorbid factors in the need for hospitalization for respiratory issues related to congenital airway abnormalities.

TSH Surge Abnormalities in Pediatric Patients with Diabetes Mellitus

TSH Surge Abnormalities in Pediatric Patients with Diabetes Mellitus

Metabolic bone disease of total parenteral nutrition.

Parenteral nutrition-associated metabolic bone disease in children is manifested primarily as osteopenia and, on occasion, fractures. The etiology is likely multifactorial, with calcium and phosphate deficiency playing a major role in the preterm infant and with the role of aluminum toxicity yet to be clearly defined in this population. Lack of normal values of bone histomorphometry in the premature infant as well as lack of normal data for biochemical markers of bone turnover in these patients contribute to the uncertainty. Other factors that may play a role in the pathogenesis include lack of periodic enteral feeding; underlying intestinal disease, including malabsorption and inflammation; the presence of neoplasms; and drug-induced alterations in calcium and bone metabolism. The true incidence and prevalence of parenteral nutrition-associated bone abnormalities in pediatric patients remain unknown.

Total body mineral mass measured with dual photon absorptiometry in healthy children.

Using dual photon absorptiometry, bone mineral content (BMC) and bone mineral density (BMD) of the total body and the lumbar spine were assessed in 97 healthy, Caucasian children aged 3-14 years. Excellent correlations were found between BMC and BMD on the one hand and age, body height and body weight on the other. No differences were found between boys and girls. There was a strong correlation between lumbar spine measurement as compared to those of the total body. Regression equations for total body and the different parts of the skeleton were calculated with either BMC or BMD as the dependent variable, and age, body height and body weight as independent variables. High variation coefficients were obtained in these multiple regressions, except for the head. For total body BMC and total body BMD, growth charts were constructed using Tanner and Whitehouse data on body height and body height and body weight. Conclusions. The increase in total body mineral content is an important feature of normal growth. Normal data for BMC and BMD in childhood are essential for bone mineralisation abnormalities in paediatric patients.

EKG abnormalities in pediatric patients with myotonic dystrophy

Electrocardiographic (EKG) abnormalities are frequent in patients with myotonic dystrophy; cardiac complications may lead to significant morbidity and mortality. The charts of 17 pediatric patients with myotonic dystrophy were reviewed to ascertain the frequency of EKG abnormalities and cardiovascular symptoms. Fifteen of 17 patients had abnormal EKGs with sinus bradycardia being the most common abnormality. Only 1 of 17 patients had cardiovascular symptoms. Four patients had moderate to severe weakness and 3 of them had a conduction disturbance (i.e., first-degree AV block or intraventricular conduction delay). Two of the remaining 13 patients with mild weakness had conduction disturbances. No pediatric patients had progressive EKG abnormalities during follow-up. Baseline EKG study of pediatric patients with myotonic dystrophy is recommended because abnormalities are frequent and usually asymptomatic. Frequent follow-up EKGs are probably unnecessary unless the patient is symptomatic or has heart block.

Absence of serum chemistry abnormalities in pediatric patients presenting with seizures.

To determine the utility of the routine practice of obtaining serum chemistry values on children presenting after a seizure, we reviewed the emergency department records of 241 episodes of seizures in pediatric patients. One hundred fifty-five nonfebrile (49 initial, 106 recurrent) and 86 febrile (53 initial, 33 recurrent) convulsive episodes were analyzed. At least one serum chemistry value was obtained in 149 (64%) patients. Clinically significant abnormalities were found in 0/149 serum sodium, 0/148 glucose and blood urea nitrogen, 0/86 calcium, and 0/61 magnesium studies. We concluded that routine determination of serum chemistry values in pediatric patients presenting with a seizure is unnecessary unless specific clinical data strongly suggest otherwise.

The contribution of perfusion scintigraphy in the evaluation of children suffering from recurrent localized pneumonia

The value of perfusion scintigraphy as a screening test for children who have suffered from several episodes of recurrent localized pneumonia was evaluated in 32 patients aged 1-15 years. Perfusion studies were carried out using macroaggregated albumin (MAA) labeled with technetium 99m. In 9 patients (28%), large lobar or multisegmental perfusion defects were demonstrated. Their final diagnoses proved to be bronchiectasis (5 patients), bronchomalacia (2 cases), agenesis of a lobe (1 subject), and lobar sequestration (1 patient). In 23 children (72%), the perfusion scintigraphic patterns were normal or diffusely nonhomogeneous. All of these patients improved clinically on a 1- to 2.9-year follow-up. We conclude that a normal perfusion scintigraphy is a useful screening test for excluding structural lung abnormalities in pediatric patients with recurrent localized pneumonia. Children showing a pattern of lobar or multisegmental perfusion defects should be further investigated to rule out structural abnormalities as an underlying cause of disease.