Pediatric-aged Patients Research Articles

Acute mastoiditis in children — our experience

The incidence of acute mastoiditis and the number of complications has changed since the 1950s, despite the increasing antibiotic effectiveness. Other series concluded that the incidence of acute mastoiditis is rising in the recent years, which can be justified by the antibiotic resistance of the microorganisms and the absence of paracentesis in the treatment of acute otitis media. Our aim is to approach risk factors, clinical presentation, diagnosis and treatment of acute mastoiditis. We reviewed 62 clinical records of patients in pediatric age, observed in D. Estefânia Hospital — Lisbon, between January 1993 and December 1997. There was a relative homogenous distribution during the 5 years of the study period. The patient age ranged from 5 months to 14 years. They all were treated with intravenous antibiotics. The mean duration of treatment was 7.4 days. We registered 15 complications: 14 retroauricular subperiosteal abscesses and one subdural empyema. The most common isolated microorganism was Streptococcus pneumoniae. We found no statistic difference ( P>0.1) in the incidence of acute mastoiditis between the 5 years of the study

SULINDAC VERSUS ADENOMATOUS POLYPOSIS COLI IN PRETEEN AND TEENAGE PATIENTS: SO FAR, SO GOOD!

42 APC is an autosomal dominant disorder with an estimated prevalence of 1 in 5,000 - 7500. Affected children rarely present before the age of 10. Management has classically been surgical. The observation that the NSAID, sulindac, causes regression of APC polyps has generated hope that colectomy can be delayed in patients with polyps <1 cm at the time the medication is initiated. Aim: To report an experience with sulindac in pediatric-age patients with APC. Methods: Retrospective chart reviews. Results: 9 patients were identified (7-RCH; 2-CHM); 7 females. The mean age at diagnosis 10.3 years (range 7-15). Two patients had already undergone colectomy; both had multiple polyps in the stomach and duodenum. Polyps were identified in the UGI tracts of 7/9 patients. Five were APC gene positive. The age at initiation of sulindac was 11.7 years (range 9-16 years). These 9 patients were followed a mean of 22.6 months (range 6-53). All patients demonstrated an initial regression in polyp size and number which was maintained throughout the period of observation. The largest polyp removed was .8 cm, and this was from a girl who discontinued the sulindac 5 months prior to colonoscopy. High grade dysplasia was not detected in any polyp or biopsy. There have been no reported complications with the medication (150 or 200 mg bid). Discussion: The mechanism of action of sulindac is debated but it appears that the metabolites, especially the sulfide metabolite, causes cell cycle arrest in proliferating cells resulting in apoptosis. Conclusion: Sulindac seems effective in producing regression of UGI and colorectal adenomas in pediatric-age APC patients. It is hoped that treatment with careful monitoring will delay surgery beyond the teenage years.

Paediatric cardiac anaesthesia in a developing country. Guatemala Heart Team.

During the week of October 15-24, 1995 a team of 65 medical, anaesthesiology, surgical, nursing and paramedical personnel travelled to Guatemala City, Guatemala to perform cardiac surgery on children with complex congenital and acquired valvular heart disease. During this mission 42 patients had their lesions surgically repaired. Cardiopulmonary bypass was required in 36 cases. There were no anaesthetic or surgical deaths. All six patients who did not require cardiopulmonary bypass were extubated in the operating room. Of the patients who required cardiopulmonary bypass, 23 were extubated in the operating room (64%). There was no intraoperative anaesthetic morbidity nor postoperative respiratory complications. No patients was reintubated after planned extubation. Cardiac surgery in paediatric age patients can safely be performed in developing countries if close attention is paid to proper patient selection and one maintains the standards of care practised in developed countries.

Status epilepticus increases CSF levels of neuron-specific enolase and alters the blood-brain barrier.

Neuron-specific enolase (NSE) is a sensitive marker of brain damage in stroke, global ischemia, and coma. Serum NSE is also correlated with the duration and outcome of status epilepticus (SE). CSF-NSE levels have not been previously reported in SE. We report the CSF concentrations of NSE in 11 patients with cryptogenic/remote symptomatic SE. CSF obtained within 24 hours of SE showed increased concentrations of NSE in 9 of 11 patients. The mean CSF-NSE for the group was elevated compared with the levels for normal control subjects (30.8 +/- 18.33 versus 10.76 +/- 3.08 ng/mL; p = 0.002). Further, CSF-NSE levels were elevated compared with simultaneous serum levels in the same group of patients (p = 0.01). In addition, the CSF/serum albumin ratio (QAlb), a measure of the integrity of the blood-brain barrier, was increased in SE patients compared with control individuals (33.4 versus 4.79 x 10(-3); p = 0.0001). An increase of QAlb correlated with CSF-NSE (rs = 0.66, p = 0.04) and serum NSE levels (rs = 0.83, p = 0.004). CSF-NSE is a promising in vivo marker for brain injury after SE.

Upper Respiratory Tract Complications of Gastroesophageal Reflux in Adult and Pediatric-Age Patients

Upper airway complications of gastroesophageal reflux occur much less frequently than those aborad to the upper esophageal sphincter; however, laryngitis, laryngeal and/or tracheal stenosis, globus syndrome, oropharyngeal dysphagia, otitis media, sinusitis, and rhinitis can all be associated with significant morbidity and occasional mortality in both adult and pediatric patients. Sudden infant death and apparent life-threatening events, both found only in pediatric patients, are even less frequently associated with gastroesophageal reflux. Today, excellent diagnostic methods are available, such as proximal 24-hour pH probe evaluations or scintigraphy, making proper diagnosis much easier than previously. Although today’s medical and surgical methods do not affect the underlying pathophysiology, they are frequently very effective in controlling signs and symptoms, allowing the patients to return to resume their normal life-styles and livelihoods.

Síndrome del túnel carpiano en la infancia. A propósito de un caso de debut precoz

The carpal tunnel syndrome (CTS) is the commonest neuropathy due to compression to be seen in adults. There are very few cases in the literature referring to patients of paediatric age, particularly those under ten years old. Most of these young patients had a metabolic disorder (mucopolysaccharidosis (MPS) or mucolipidosis (ML). In fact, as many as 90% of the MPS had CTS, sometimes subclinically. This syndrome is caused by compression of the median nerve at the level of the carpal tunnel, to which multiple factors may contribute, both local and systemic, as reviewed in this paper. The clinical findings differ from those in adults, but the appearance of suggestive symptoms and signs should make one suspect the condition and request an electromyographic study (EMG) which would be diagnostic. We describe the case of a five year old girl, with a clinical history suggesting the presence of a carpal tunnel syndrome for 12 months and characterized by paraesthesia and limitation of flexon-extension movements of the fingers of the affected hand, with pain on movement. The symptoms appeared on waking in the morning, gradually improved as the day advanced and became bilateral over a period of six months. The diagnosis was confirmed by EMG and MR helped to clarify the aetiology. The interesting aspect of this article is the youth of the patient, the absence of known etiological factors and the presence of tenosynovitis detected on MR as has been described in some idiopathic/familial forms.

O-11-175 - Clinical features of gliomas involving the corpus callosum

Along with malformations of cortical development or cortical dysplasia, hippocampal sclerosis, and remote ischaemic damage, tumours are among the more commonly identifiable causes of medically intractable seizures in paediatric age patients. This study reviews one institution’s 20 year experience with tumours arising in this clinical setting.Retrospective review of 129 paediatric patients [less than 19 years of age, 65 females (50.4%)] with tumours and medically intractable seizures encountered during a 20 year period of time (1989–2009). Using the most recent World Health Organization (WHO) classification of brain tumours, tumour type and grade were assessed.The most common sites of origin included temporal lobe (n = 77, 59.7%), parietal lobe (n = 20, 15.5%), and frontal lobe (n = 15, 11.6%). WHO grade included 73 (56.6%) grade I tumours, 32 (24.8%) grade II tumours, and 18 (14%) grade I/II tumours. In six cases (4.7%), a WHO grade was not associated with mass. Tumour types included: ganglioglioma (n = 48, 37.2%), dysembryoplastic neuroepithelial tumour (n = 17, 13.2%), low grade astrocytoma (n = 15, 11.6%), low grade mixed glioma (n = 8, 6.2%), low grade oligodendroglioma (n = 5, 3.9%), meningioangiomatosis (n = 4, 3.1%) angiocentric glioma (n = 3, 2.3%), and dysembryoplastic neuroepithelial tumour/ganglioglioma composite tumour (n = 3, 2.3%). Less frequently observed lesions (n = 1 or 2) included pilocytic astrocytoma, protoplasmic astrocytoma, pleomorphic xanthoastrocytoma, and glioneuronal hamartoma. In 18 cases, distinction between low grade glioma and low grade glioneuronal tumour could not be definitively made. Coexisting malformation of cortical development/cortical dysplasia was noted in 29.8% of evaluable cases. In four tumours, coexistent hippocampal sclerosis was identified. Ki-67 labelling indices were less than 5% in all (n = 51) cases assessed. Of 25 tumours evaluated for chromosome 1p status, only one low grade mixed glioma demonstrated evidence of deletion; only one of 22 evaluated tumours (a low grade mixed glioma) showed evidence of chromosome 19q deletion.Collectively, WHO grade I glioneuronal tumours account for slightly more than half of all neoplasms which cause intractable epilepsy in paediatric patients. A significant minority of tumours (n = 18, 14%) were difficult to definitively classify as glioma versus glioneuronal tumour, due to extent of sampling. Coexistent pathologies including malformation of cortical development/cortical dysplasia may be observed in a significant minority of tumours, suggesting a possible developmental origin for some tumours arising in this setting.

Magnetic Deflection Forces from Atrial Septal Defect and Patent Ductus Arteriosus-Occluding Devices, Stents, and Coils Used in Pediatric-Aged Patients

Magnetic deflection forces and imaging artifact were assessed for 13 pediatric interventional cardiology devices. The magnetic deflection forces incurred by these devices are small compared with physiologic forces experienced in the cardiovascular system.

Subclinical peripheral neuropathy in HIV infection: an electrophysiological study

To investigate visual habituation – a measure of visual cortical excitability – in photosensitive patients in pediatric age and compare the findings with a matched sample with idiopathic generalized epilepsies without photosensitivity and with normally developing children.We presented a full-field black-and-white checkerboard pattern, at 3 reversal/s with 100% contrast binocularly for 600 consecutive trials and measured the N75–P100 and P100–N145 pattern-reversal visual evoked potential inter-peak amplitudes and N75, P100, N145 latencies for the six blocks of 100 responses. As a measure of habituation we used the slope of the linear regression line of the N75–P100 and P100–N145 peak-to-peak amplitudes. The slope of the linear regression line of the N75–P100 and P100–N145 latencies was also analyzed.Statistical analysis revealed significant differences between the three groups in the slope index of N75–P100 PR-VEP amplitude, with increased or constant amplitude in the PS group compare to the IGE and ND across the six blocks.Our results support the notion that photosensitivity is associated with altered control of excitatory and inhibitory cortical processes. The causal relationship between habituation deficit and photo-paroxysmal response needs to be further investigated with longitudinal studies.This study supports the hypothesis that suppression of PR-VEP is a sensitive intermediate phenotype, which discriminates patients with photosensitivity from those with generalized epilepsies in pediatric age.

Main trends in the study of altered states of consciousness

To investigate visual habituation – a measure of visual cortical excitability – in photosensitive patients in pediatric age and compare the findings with a matched sample with idiopathic generalized epilepsies without photosensitivity and with normally developing children.We presented a full-field black-and-white checkerboard pattern, at 3 reversal/s with 100% contrast binocularly for 600 consecutive trials and measured the N75–P100 and P100–N145 pattern-reversal visual evoked potential inter-peak amplitudes and N75, P100, N145 latencies for the six blocks of 100 responses. As a measure of habituation we used the slope of the linear regression line of the N75–P100 and P100–N145 peak-to-peak amplitudes. The slope of the linear regression line of the N75–P100 and P100–N145 latencies was also analyzed.Statistical analysis revealed significant differences between the three groups in the slope index of N75–P100 PR-VEP amplitude, with increased or constant amplitude in the PS group compare to the IGE and ND across the six blocks.Our results support the notion that photosensitivity is associated with altered control of excitatory and inhibitory cortical processes. The causal relationship between habituation deficit and photo-paroxysmal response needs to be further investigated with longitudinal studies.This study supports the hypothesis that suppression of PR-VEP is a sensitive intermediate phenotype, which discriminates patients with photosensitivity from those with generalized epilepsies in pediatric age.

Steady-state pharmacokinetics and bioavailability of total and unbound disopyramide in children with cardiac arrhythmias.

We studied the pharmacokinetics of the total (protein-bound plus -unbound and unbound forms of disopyramide (DP) at steady-state in six children (aged 5.2 to 12.2 years) with cardiac arrhythmias who had received repeated oral DP therapy. Maximum concentrations after the oral dose were reached at 2.5 +/- 1.1 h (mean +/- SD) for both total and unbound DP. The bioavailabilities calculated from total and unbound plasma concentration-time curves were 99 +/- 23 and 89 +/- 27% of the dose, respectively. These parameters seen in our children are similar to those reported from adult subjects. The mean elimination half-lives (t1/2), volumes of distribution, and total body clearances (CL) of total and unbound DP were 3.15 +/- 0.64 and 2.50 +/- 0.37 h, 1.02 +/- 0.25 and 2.60 +/- 0.38 L/kg, and 3.79 +/- 0.82 and 13.12 +/- 2.60 ml/min/kg, respectively. These mean CL and t1/2 values are considerably greater and shorter, respectively, than those reported from adult subjects. The findings indicate that the greater doses of DP per kg of body weight reportedly required for attaining a therapeutic plasma drug level in pediatric age patients should be due to a greater drug CL in children than in adults. A sustained-release preparation of DP may be required for pediatric patients to minimize a large fluctuation of plasma drug levels during the dosing intervals.

Intraoperative radiotherapy in the multidisciplinary treatment of bone sarcomas in children and adolescents

From September 1984 to December 1989, 38 patients of pediatric age with localized bone sarcomas received intraoperative radiotherapy (IORT) as part of a multidisciplinary treatment program. The age ranged from 6 to 21 years. The tumor histologies were 22 osteosarcomas and 16 Ewing's sarcomas. Thirty-four had initial primary disease (90%) and 4 were treated for local recurrence (10%). IORT was used on 32 untreated patients and in 6 previously treated with external beam radiotherapy (EBR). The IORT field included the surgically exposed tumor bed area. Single radiation doses ranging from 10 to 20 Gy were delivered, using 6-20 MeV electron beams. The median follow-up time for the entire group is 25 months (2-65+ months). The projected 5-year disease-free and overall survival rates are 65% and 69%, respectively. One patient developed a local recurrence in each histological group: one chondroblastic osteosarcoma and one cervical Ewing's sarcoma. Six patients died from metastatic progression: 3 initially recurrent tumors and three primary disease cases. Severe neuropathy and soft tissue necrosis were seen in some patients as IORT related complications. IORT is a feasible technique to be integrated in multidisciplinary programs that may promote local control in pediatric and adolescent patients with bone sarcomas. Peripheral nerves are dose-limiting tissue structures for IORT.

Arterial aneurysms in children: Clinicopathologic classification

Thirty-one arterial macroaneurysms in 23 pediatric-aged patients (16 boys and 7 girls) were treated at the University of Michigan. The average age at time of diagnosis was 10.2 years (range 6 months to 18 years). Vessels involved the aorta (4), as well as hepatic (1), splenic (2), gastroepiploic (1), renal (12), iliac (1), superficial femoral (4), popliteal (1), brachial (1), radial (2), and ulnar (2) arteries. Twelve children exhibited overt clinical manifestations including presence of a mass (7), local pain (3), hematemesis (1), and painless obstructive jaundice (1). Eleven children had asymptomatic lesions. Aneurysm existence was confirmed by arteriography or operation. All but one child underwent surgical therapy, with 20 long-term survivors (mean follow-up 3.5 years). One operative death occurred and one death occurred 6 years after surgery. This experience and a review of previously reported cases served as a basis for categorization of childhood aneurysmal disease as true aneurysms associated with (I) arterial infection, (II) giant-cell aortoarteritis, (III) autoimmune connective tissue disease, (IV) Kawasaki's disease, (V) EhlersDanlos syndrome or Marfan's syndrome, (VI) other forms of noninflammatory medial degeneration, (VII) arterial dysplasias, (VIII) congenital-idiopathic factors, as well as (IX) false aneurysms associated with extravascular events causing vessel wall injury or disruption. Knowledge of the varied dinicopathologic characteristics of arterial aneurysms in children is important in treating these patients.

Hypoglycemia in Infants and Children

Although the conditions that cause hypoglycemia in adults may also be present in infants and children, there are many entities unique to the pediatric age group. This reflects the delicate balance that exists in the newborn and young child between glucose production and utilization. During fasting in infants and children, hepatic glucose production is normally two to three times that of adults when expressed on the basis of weight. In the newborn and young infants, hypoglycemia usually presents with irritability, feeding difficulties, lethargy, cyanosis, tachypnea, and/or hypothermia rather than the typical adrenergic or neuroglucopenic symptoms seen in the adult. The hypoglycemia may be due to abnormalities in hormone secretion, substrate interconversion, or mobilization of metabolic fuels. The hypoglycemia associated with hyperinsulinemia may be transient neonatal, sustained, or drug-induced. Inborn errors of metabolism caused by enzymatic defects are responsible for hypoglycemia associated with abnormalities of production and utilization of metabolic fuels. These can involve carbohydrate, protein, and fat metabolism. In addition, there may be acquired or transient defects in carbohydrate metabolism secondary to other diseases or ingestion of certain substances. Finally ketotic hypoglycemia appears to be due to abnormalities in substrate availability. A variety of tests are useful for establishing the etiologic basis of the hypoglycemia, and the appropriate treatment depends upon the underlying cause.

Percutaneous Drainage of Splenic Abscess: Case Report and Review of Literature

Splenic abscess is an uncommon entity, reported in 0.14% to 0.70% of several autopsy series. Untreated, the mortality approaches 100%.1 When the diagnosis is made preopenatively and treated by antibiotics and splenectomy, there is a 17% mortality, but preoperative diagnosis was made only in 33% of the cases in one review.2 High degree of clinical awareness and aggressive diagnostic approach are essential for early diagnosis. percutaneous drainage of splenic abscess has been demonstrated to be a safe and effective method of treatment in adults in eight cases,3-5 without mortality or significant complications. We present the first pediatric age patient who was successfully treated by percutaneous drainage.

Toluidine Blue in the Detection of Perineal Lacerations in Pediatric and Adolescent Sexual Abuse Victims

Posterior fourchette lacerations are suggestive of sexual assault, and toluidine blue dye has increased the detection of these lacerations in adult rape victims. This study investigated the use of toluidine blue dye in the pediatric (0 to 10 years) and adolescent (11 to 18 years) patients to detect posterior fourchette lacerations in sexually abused and control populations. Application of toluidine blue dye increased the detection rate of posterior fourchette lacerations from 4% (1/25) to 28% (7/25) (P less than .05, Fisher exact test) in adolescent sexually abused patients and from 16.5% (4/24) to 33% (8/24) (P = .318, Fisher exact test) in pediatric sexually abused patients. Posterior fourchette lacerations occurred with the same frequency in sexually abused adolescents and sexually active controls adolescents. In the pediatric aged population, 33% of the sexually abused group had lacerations detected, whereas none of the control patients had lacerations. The presence of posterior fourchette lacerations in the pediatric aged patient is strongly suggestive of sexual abuse. Toluidine blue increases the detection of posterior fourchette lacerations in children and adolescents (P less than .001, Fisher exact test). The application of toluidine blue dye to highlight posterior fourchette lacerations is an important addition to tools already used in the evaluation of the sexually abused patient.

Expression of Normal Myeloid-Associated Antigens by Acute Leukemia Cells

Monoclonal antibodies that react with hematopoietic cells and their precursors in a stage and lineage restricted fashion were used in indirect immunofluorescence assays to examine leukemic cells from 105 pediatric age patients. The differentiative states of blasts from 42 patients with acute nonlymphocytic leukemia (ANLL) were defined by these antibodies. When these were compared to their morphologic and histochemical levels of differentiation as defined by the French-American-British (FAB) classification, no direct relationship was found. The reactivity of these antibodies with leukemic cells from 63 patients with acute lymphocytic leukemiA (ALL) was also investigated, and the usefulness of these antibodies in distinguishing leukemias of myeloid from those of lymphoid origin was demonstrated.

Expression of normal myeloid-associated antigens by acute leukemia cells

Monoclonal antibodies that react with hematopoietic cells and their precursors in a stage and lineage restricted fashion were used in indirect immunofluorescence assays to examine leukemic cells from 105 pediatric age patients. The differentiative states of blasts from 42 patients with acute nonlymphocytic leukemia (ANLL) were defined by these antibodies. When these were compared to their morphologic and histochemical levels of differentiation as defined by the French- American-British (FAB) classification, no direct relationship was found. The reactivity of these antibodies with leukemic cells from 63 patients with acute lymphocytic leukemiA (ALL) was also investigated, and the usefulness of these antibodies in distinguishing leukemias of myeloid from those of lymphoid origin was demonstrated.

The method and management of thyroid surgery in the pediatric patient

Thyroid surgery in the pediatric age patient accounts for a small minority of all thyroid surgery. Batsakis and Nishiyama reported only 136 patients under the age of 18 who underwent thyroid surgery in 27 years at the University of Michigan. In most series involving the pediatric age group, the majority of thyroid procedures are performed on adolescents. Thyroid surgery in the young child requires special precautions in addition to those routinely associated with thyroidectomy in the adult. The techniques and perioperative management of the pediatric thyroidectomy employed at our institution has evolved as experience is gained. Our experience with five such patients with adequate follow-up will be presented.

The role of sigmoidoscopy, radiographs, and colonoscopy in the diagnostic evaluation of pediatric age patients with suspected juvenile polyps

Juvenile polyps are the most common cause of painless hematochezia in pediatric Age patients after the first year of life. This study evaluated the role of rigid proctosigmoidoscopy, air contrast barium enema examinations, and colonoscopy in the diagnostic approach to 43 such patients. During sigmoidoscopy, polyps were removed from 31 children. On subsequent barium enema examination, more proximal lesions were found in only 4 of these 31 patients, but were seen in 7 of the 12 children who had negative sigmoidoscopic evaluations. Fourteen children had colonoscopy performed. This group included the latter 12 patients plus 2 of the former 4 who again developed hematochezia. Polypectomies were done during 11 of these procedures. Eight of these 11 children had proximal lesions seen during radiographic studies. The diagnostic approach to pediatric age patients with painless hematochezia should include an initial rigid sigmoidoscopic examination. Barium enema evaluation should be reserved for those patients requiring colonoscopy. The latter examination should be performed in all children who have had negative sigmoidoscopic examinations plus those who have had polyps removed from the rectum and then again develop hematochezia.