Age Of Onset In Children Research Articles

Differences in response to treatment in children with severe IgA nephropathy according to patient age.

To clarify whether the response to treatment of IgA nephropathy (IgAN) differs depending on patient age, we examined the response to treatment according to age of onset in children with IgAN. We collected data for 44 children with severe IgAN. The children were retrospectively divided into three groups based on their age at disease onset. Group 1 consisted of 24 children under 11 years old, group 2 consisted of 9 children aged 12 to 13 years, and group 3 consisted of 11 children aged over 14 years old. The clinical features and prognosis were analyzed for each group. The urinary protein excretion and serum IgA values in group 3 were higher than those in groups 1 and 2 at the most recent follow up, and histological findings showed that the MESTCG scores in group 3 were higher than those in group 1. Furthermore, the incidence of patients with persistent nephropathy or renal insufficiency in group 3 was higher than those in groups 1 and 2. Patients aged 14 years and older with IgAN may respond poorly to treatment compared with those younger than 14 years old. Therefore, care must be taken regarding response to treatment and relapse when treating older children.

A Girl with Limp and Rash on the Palms and Soles.

A Girl with Limp and Rash on the Palms and Soles.

Enterovirus Induced Opsoclonus-Myoclonus Syndrome in a 16-Month-Old Girl

Background/Purpose: Opsoclonus-Myoclonus Syndrome (OMS) is a rare neurological disorder characterized by jerking conjugated bulbar movements, ataxia with myoclonus, and psychiatric symptoms. OMS results from various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. OMS can appear at any age, the average age of onset in children is 18 to 22 months.

402 – An association study on children with gilles de la tourette’s syndrome and COMT gene polymorphism

Objective To investigate the association between catechol-O-methyl transferase (COMT) gene polymorphism and Gilles de la Tourette’s syndrome (GTS). Methods Using Amplification Refractory Mutation System(ARMS) PCR genotyping assay method, a polymorphism (val158met) of COMT gene was genotyped in 112 of all GTS patients including 54 GTS-alone patients, 48 GTS+ADHD patients among of them and 71 healthy controls. The correlation between positive association of polymorphism (val158met) of COMT gene in GTS and the age of onset in patients with GTS was also analyzed. Results When compared with healthy controls, genotype of val158met did not differ in total GTS patients or alone- GTS patients (x2=0.56, P = 0.756; x2= 1.05, P = 0.600 respectively). There is also no significant difference (P>0.05) in allele distribution of val158met in total GTS patients or alone- GTS patients when respectively compared with controls (x2= 0.18, P = 0.669; x2= 0.29, P = 0.593 respectively). However, Genotype distribution of val158met was significantly different between GTS+ADHD patients and controls (x2=6.35, P = 0.041). The frequency of the val allele of this locus was significantly higher in GTS+ADHD patients than those in controls (x2= 5.49, P = 0.019).The age of onset in children with GTS having the val/val genotype of COMT gene val158met polymorphism were significantly earlier than no val/val genotype (P = 0.016). Conclusion Polymorphism (val158met) of COMT gene may be associated with GTS children with comorbid ADHD, which may play an important role to make the age of onset in children with GTS become earlier.

1334 – Age of onset and degrees of cognitive deficit in children and adolescents with endogenous mental disorders

Introduction Cognitive deficit in endogenous mental disorders in children and adolescents is studied less than in adults, particularly age of onset influence. There is very few data about the connection between degree of cognitive deficit and age of psychiatric illness onset in children and adolescents. It’s difficult to determine the specificity of cognitive deficits depending on the clinical form of endogenous mental disorders (schizophrenia). Objectives 93 patients (11.8 ± 2.3 years) with childhood schizophrenia (F20.8, 26 patients), schizotypical disorders (F21, 59 patients), unspecified schizophrenia (F2x.x, 8 patients). We used adaptation of ICD-10 for RF. Aims Assessment of degree of cognitive deficit relative to age of onset and diagnosis. Methods Tests assessing cognitive processes: memory, attention, thinking. Z-scales were used for estimation of cognitive deficit. Results Age of onset was divided into three periods: early, preschool and school age. The least degree of all cognitive functions occurs in patients with school age of onset. F20.8 and F2x.x with early and preschool age of onset have severe thinking deficit. F21 demonstrates decreasing of cognitive deficit degrees in all functions with increasing of age of onset. Degree of cognitive deficit is connected with clinical diagnosis. Patients with F20.8 have strong deficit in all cognitive functions (memory, attention, thinking). F21 patients demonstrate a variety of cognitive deficit degrees including age norm outstrip. F2x.x patients keep intermediate position. Conclusions Age of onset might be important in determination of degrees of cognitive deficit. The last depends on diagnosis, age of onset & kind of cognitive process.

Clinical and electroencephalographic characteristics of Jeavons syndrome

The study was designed to examine the clinical and electroencephalographic characteristics of children with Jeavons syndrome. Video-electroencephalography (VEEG) monitoring was carried out in 9 patients with Jeavons syndrome. The clinical and electroencephalographic characteristics, treatment and prognoses were analyzed. Of the 9 patients, 8 were female, and 1 was male. The onset age of children with eyelid myoclonia (EM) was from 3 to 9 years old. It was obtained through the chief complaint, prosecution or VEEG monitoring. Three cases were misdiagnosed and 2 cases were overlooked initially. Seven out of 9 patients had generalized tonic clonic seizures (GTCS) during the course of disease, of whom 5 experienced only one episode. GTCS was the cause for the first visits to hospital in 5 patients. Since the clinical manifestations of EM with or without absence were often slight, VEEG monitoring with eye closure and intermittent photic stimulation tests helped to induce discharges and seizures. Eye closure was more potent than intermittent photic stimulation as a triggering factor. Ictal EEG showed 3 - 6 Hz generalized spike and waves and polyspikes burst. The main treatment option was valproate monotherapy (6 cases) or combined with other antiepileptic drugs (1 case). Levetiracetam, lamotrigine and topiramate were also used in patients and effective to some degree. Two patients lost follow up. The age of 7 patients at follow-up ranged from 9 y to 15 y. Seizures were controlled in 1 case, suspiciously controlled in 1 case, decreased in frequency in 4 cases and were still frequent in 1 case. During follow-up, normal intelligence was found in the former 2 cases, difficult learning in 2 cases, and slightly intellectual impairment in 2 cases. Jeavons syndrome is one of the idiopathic generalized epilepsies characterized by EM with or without absence. The age of seizure onset might be difficult to be exactly established, as EM was often misinterpreted and overlooked initially. Clinical history combined with VEEG monitoring with eye closure and intermittent photic stimulation tests could diagnose this disease. Valproate and other new antiepileptic drugs were effective for this disease. Jeavons syndrome is a lifelong disorder. Seizures sometimes could be well controlled. When seizures were resistant to treatment, cognitive and intellectual impairment might occur.

An association study on Gilles de la Tourette's syndrome and catechol-O-methyl transferase gene polymorphism

Objective To investigate the association between catechol-O-methyl transferase (COMT) gene polymorphism and Gilles de la Tourette’s syndrome(GTS). Methods Using Amplification Refractory Mutation System(ARMS) PCR genotyping assay method, a polymorphism (val158met) of COMT gene was genotyped in 112 of all GTS patients (total GTS group) including 54 GTS-alone patients group, 48 GTS+ ADHD patients group among of them and 71 healthy controls. The correlation between positive association of polymorphism (val158met) of COMT gene in GTS and the age of onset in patients with GTS was also analyzed. Results Compared with healthy controls group, genotype of val158met did not differ in total GTS patients group or alone-GTS patients group (χ2=0.56, P=0.756; χ2=1.05, P=0.600 respectively). There was also no significant difference (P>0.05) in allele distribution of val158met in total GTS patients group or alone-GTS patients group compared with controls group respectively (χ2=0.18, P=0.669; χ2=0.29, P=0.593 respectively). However, genotype distribution of val158met was significantly different between GTS+ ADHD patients group and controls group(χ2=6.35, P=0.041). The frequency of the val allele of this locus was significantly higher in GTS+ ADHD patients group than those in controls group (χ2=5.49, P=0.019). The mean age of onset (6.80±1.54) in 36 children within GTS+ ADHD patients group with the val/val genotype of COMT gene val158met polymorphism was significantly earlier than the mean age of onset (8.04±1.54) in 12 children in val/val genotype(P=0.016). Conclusion Polymorphism (val158met) of COMT gene may be associated with GTS children with comorbid ADHD, which may play an important role to make the age of onset in children with GTS become earlier. Key words: Gilles de la Tourette's syndrome; Catechol-O-methyl transferase; Association analysis; Age of onset

Differential Diagnosis of Lower Extremity Enlargement in Pediatric Patients Referred with a Diagnosis of Lymphedema

There are many causes for a large lower limb in the pediatric age group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to determine the differential diagnosis in pediatric patients referred for lower extremity "lymphedema" and to clarify management. The authors' Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of the lower extremity. Records were studied to determine the correct cause for the enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies were also analyzed. A referral diagnosis of lower extremity lymphedema was given to 170 children; however, the condition was confirmed in only 72.9 percent of patients. Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic lymphatic malformation (19.6 percent), noneponymous combined vascular malformation (13.0 percent), capillary malformation (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema (6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3 percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children with lymphedema was older than in patients with another diagnosis (p = 0.027). "Lymphedema" is not a generic term. Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema; the most commonly confused causes are other types of vascular anomalies. History, physical examination, and often radiographic studies are required to differentiate lymphedema from other conditions to ensure the child is managed appropriately.

Young age as a risk factor for complicated course and visual outcome in intermediate uveitis in children

ObjectiveTo identify prognostic factors in intermediate uveitis (IU) in children.MethodsRetrospective case series of 35 patients with onset of IU ≤16 years and a minimum follow-up of 1 year. Demographic and...

Disentangling chronological age from age of onset in children and adolescents with obsessive--compulsive disorder.

Although paediatric obsessive--compulsive disorder (OCD) is increasingly recognized as a putative developmental subtype of the disorder, it remains uncertain as to whether additional subtyping by age at onset in childhood or adolescence is warranted. Subjects included children and adolescents meeting DSM-III-R and DSM-IV criteria for OCD referred to a specialized OCD clinic. All youth were systematically evaluated with structured diagnostic interviews and clinical assessment by an OCD expert. Irrespective of current age, an earlier age at onset predicted increased risk for attention deficit hyperactivity disorder, simple phobia, agoraphobia and multiple anxiety disorders. In contrast, mood and psychotic disorders were associated with chronological age and were more prevalent in older subjects. Tourette's disorder showed associations with both chronological age and age at onset. Chronological age and age at onset predicted different patterns of comorbidity and dysfunction in children and adolescents with OCD. Considering the heterogeneity of OCD, age at onset may help identify meaningful developmental subtypes of the disorder beyond chronological age.

Familial spastic paraparesis: evaluation of locus heterogeneity, anticipation, and haplotype mapping of the SPG4 locus on the short arm of chromosome 2.

Familial spastic paraparesis (SPG) is a clinically and genetically heterogeneous group of disorders. At least three loci have been implicated in autosomal dominant pure SPG and mutations in either of two loci may cause the X-linked form. Although the penetrance is high for all forms by age 60, there is wide variation in clinical characteristics, including age of onset. Two-point and multi-point linkage analyses in nine families provided supportive evidence that the most common form of SPG is linked to chromosome 2 (SPG4). Haplotype analysis localized the critical region to a 6 cM interval between D2S392 and D2S367. By haplotype analysis, the disease in at least one family does not appear to be linked to any of the presently known SPG loci, suggesting that there is at least one additional SPG gene. Evaluation at ages of onset in 11 families gave suggestive evidence for anticipation with mean age of onset in parents (41.3 years) being older than mean age of onset in children (26.9 years; P < 0.005).