Evolving phenotype of Marfan's syndrome

AIM: To examine evolution of the physical characteristics of Marfan's syndrome throughout childhood. METHODS: 40 children were ascertained during the development of a regional register for Marfan's syndrome. Evolution of the clinical characteristics was determined by repeat evaluation of 10 patients with sporadic Marfan's syndrome and 30 with a family history of the condition. DNA marker studies were used to facilitate diagnosis in those with the familial condition. RESULTS: Musculoskeletal features predominated and evolved throughout childhood. Gene tracking enabled early diagnosis in children with familial Marfan's syndrome. CONCLUSIONS: These observations may aid the clinical diagnosis of Marfan's syndrome in childhood, especially in those with the sporadic condition. Gene tracking has a role in the early diagnosis of familial Marfan's syndrome, allowing appropriate follow up and preventive care.     

The clinical course and echocardiographic features of Marfan's syndrome in childhood

The clinical and echocardiographic manifestations in 25 patients with Marfan's syndrome diagnosed during infancy and childhood (mean [+/- SD] age, 8.1 +/- 4.8 years; range 0 to 16 years) were evaluated. Twenty-one patients (84%) had a midsystolic click, 11 patients (44%) had mitral regurgitation (MR), and five patients (20%) had combined MR and aortic regurgitation (AR). Echocardiography demonstrated mitral valve prolapse in all 25 patients, aortic root dilatation in 20 patients (80%), AR in seven patients (28%), and aortic aneurysm in five patients (20%). During the follow-up period (mean, 5 +/- 4.5 years), progressive AR and aortic aneurysm were documented in four patients, progressive MR in three patients, and progressive aortic root dilatation in two patients. Five patients (22%) died during the follow-up period. Among patients with a positive family history of Marfan's syndrome, MR was less frequent as compared with sporadic cases (29.4% vs 75%, respectively). Progressive cardiovascular involvement was more frequent among patients diagnosed before 10 years of age compared with those diagnosed later (60% vs 12.5%, respectively). Cardiovascular involvement was a common feature of childhood Marfan's syndrome, causing significant morbidity and mortality. Sporadic cases and children diagnosed before 10 years of age represented a particularly high-risk group.     

Adulthood outcome of tic and obsessive-compulsive symptom severity in children with Tourette syndrome

BACKGROUND: Tourette syndrome (TS) is a childhood-onset neuropsychiatric disorder that is characterized by both motor and phonic tics. One half to two thirds of children with TS experience a reduction or complete resolution of tic symptoms during adolescence. At least one third of adults with TS have comorbid obsessive-compulsive disorder (OCD). OBJECTIVES: To clarify the clinical course of tic and OCD symptoms in children with TS and determine if baseline clinical measurements in childhood are associated with future symptom severity in late adolescence and early adulthood. DESIGN: Prospective cohort study. SETTING: Yale Child Study Center tic and OCD outpatient specialty clinic. PARTICIPANTS: Forty-six children with TS who received a structured clinical evaluation prior to age 14 years. MAIN OUTCOME MEASURES: Expert-rated tic and OCD symptom severity at follow-up interview an average of 7.6 years later (range, 3.8-12.8 years). RESULTS: Eighty-five percent of subjects reported a reduction in tic symptoms during adolescence. Only increased tic severity in childhood was associated with increased tic severity at follow-up. The average age at worst-ever tic severity was 10.6 years. Forty-one percent of patients with TS reported at one time experiencing at least moderate OCD symptoms. Worst-ever OCD symptoms occurred approximately 2 years later than worst-ever tic symptoms. Increased childhood IQ was strongly associated with increased OCD severity at follow-up. CONCLUSION: Obsessive-compulsive disorder symptoms in children with TS became more severe at a later age and were more likely to persist than tic symptoms.     

Cyclical vomiting syndrome

Cyclical vomiting syndrome (CVS) was described over 100 years ago, but it is often underdiagnosed and undertreated, even after a diagnosis is made. It is relatively common, affecting almost 2% of school-age children in some studies. Although it is traditionally seen as a childhood disease related to migraine, CVS does occur in adults. The main characteristic of CVS is the stereotypical recurrent nature of episodes of intense nausea and vomiting lasting from few hours to few days and followed by a complete resolution of symptoms. The diagnosis is predominantly a clinical one and there are internationally accepted criteria for diagnosis. The management of acute attacks of CVS aims to relieve symptoms, reduce the duration of attacks and prevent dehydration and hospital admission. Management also includes appropriate counselling on healthy lifestyle, provision of individual management plans and preventive medications. The aim of management is to reduce the number of attacks and improve quality of life. About half the children with CVS start to have migraine with or without aura in late adolescence and around 40% continue with CVS into early adult life. This article is aimed at healthcare professionals looking after children with CVS and describes the clinical presentation, the criteria required for diagnosis and outlines the different treatment options.     

Epileptic syndromes in childhood and adolescence

The syndromic approach to the diagnosis of epilepsy has been one of the most significant advances in clinical epileptology in the past few decades. An epilepsy syndrome is defined by the clustering of signs and symptoms, including investigational results, which define a unique epilepsy condition. There is no completely satisfactory way of grouping the very large number of epilepsy syndromes that occur in childhood and adolescence. In this paper the following are reviewed: the idiopathic epilepsy syndromes in infancy; the idiopathic focal epilepsies of childhood and the familial (autosomal dominant) focal epilepsies; the idiopathic generalised epilepsies; the symptomatic and probably symptomatic focal epilepsies; and the epileptic encephalopathies. Although a syndrome diagnosis is not possible in all children with epilepsy, where it is, it is likely to offer the best guide to management and prognosis.     

Clinique de l’enfance d’une population d’adultes schizophrènes. Étude rétrospective à propos de 50 cas

ObjectivesThe early stages of schizophrenia have been particularly investigated in Northern Europe and in the English-speaking countries, aiming at creating screening programs, in order to improve the disease prognosis. Due to a lack of specificity, knowledge about the early manifestations of schizophrenia must be supported to implement effective prevention strategies. Our retrospective study aims at describing, in a dimensional approach, childhood symptoms of an adult schizophrenic population.MethodsThe sample consists of 50 schizophrenic adults, diagnosed with ICD-10 criteria, aged from 18 to 30, treated in the Psychotherapeutic Center of Nancy (CPN) in 2008 and previously in a child psychiatric department within the CPN. Various symptoms were collected from children psychiatric records using a dimensional reading grid created by the authors. Childhood and adolescence were dichotomized using the limit of 12 years old. Continuity between the dimensions of childhood and adolescence was researched.ResultsOur sample consists of 72% male patients and includes a majority of early schizophrenia forms. The diagnosis was established in average around 21. Symptoms have been described in their medical records for 31 subjects during childhood and for 46 during adolescence. In our sample, the childhood semiology is characterized by six clinical dimensions (each with several signs): functional, cognitive, impulsivity, negative, anxiety and positive/dissociation. During their childhood, 25 patients had symptoms from more than two dimensions concurrently. The most frequent items belong to the functional dimension, which is not specific; but also, in 40% of cases, to the cognitive dimension. These symptoms correspond to the vulnerability markers of the premorbid stage of schizophrenia. In addition, eight patients were followed during their childhood for pervasive developmental disorders classified as « other » in ICD-10 (F 84.9). These disorders also seem to belong to premorbid manifestations, showing increased vulnerability to adult schizophrenia. Every childhood dimensions are stable through adolescence in our population with more statistical power for the items of impulsivity, anxiety and positive/dissociation dimensions. This result may suggest the existence of an evolutionary dimensional continuum, from childhood to adolescence in our population.ConclusionThe continuum highlighted by our study enables to show the emergence of various dimensions at different stages of the development of future schizophrenic patients. Other studies seem to be necessary to confirm our results. Although the described symptoms lack of specificity up to now, their presence can inform the therapist about the risk of later schizophrenia development.     

Tumor development in three patients with Noonan syndrome

The diagnosis of Noonan syndrome is essentially clinical, based upon the distinct phenotype and the involvement of the cardiovascular system. Tumor development is a rare manifestation of Noonan syndrome but can be explained by the molecular pathophysiology involved in the disorder. We present three Noonan patients who developed solid tumors. The first patient, a 4-year-old girl, developed granular cell tumors as did her mother in childhood. The second patient, a 1-year-old boy, had a low grade pilocytic astrocytoma, the clinical expression of which was persistent headache. MRI showed a pituitary mass in the posterior lobe. It was surgically removed. The third patient, a 7-year-old boy was found to have Sertoli tumors in his right cryptorchid testis. All three patients fulfilled the clinical criteria for Noonan syndrome. However, genetic testing was negative in patients 1 and 3. The diagnosis of Noonan syndrome was made based on distinct phenotypic findings in three patients who had different types of tumors.     

Churg-Strauss syndrome in a 15-year old boy

A 15 year-old boy was admitted in our hospital with the clinical signs of cardiopulmonary deterioration. His history showed bronchial asthma since 3 years. Furthermore he revealed signs of polyneuropathy, exanthema and a transitory pulmonary infiltration. Elevation of eosinophilic granulocytes and IgE confirmed the diagnosis of a Churg-Strauss syndrome. A rapid improvement of the symptoms was achieved by high-dose steroids and symptomatic therapy. The impaired systolic and diastolic myocardial function improved, but a cardiomyopathy with a shortening fraction below 25% persisted. The Churg-Strauss syndrome is a rare disease in childhood and is classified as a systemic granulomatosis of the vessels. The marked perimyocarditis with consecutive restrictive cardiomyopathy is a rare manifestation in childhood and adolescence. The prognosis of this disease is mainly influenced by the cardiac involvement.     

Clinical features of pathologic childhood aerophagia: early recognition and essential diagnostic criteria

OBJECTIVE: This study investigated the early recognition and diagnosis of pathologic childhood aerophagia to avoid unnecessary diagnostic approaches and serious complications. METHODS: Between 1995 and 2003, data from 42 consecutive patients with pathologic childhood aerophagia, aged 2 to 16 years, were reviewed. An esophageal air sign was defined as an abnormal air shadow on the proximal esophagus adjacent to the trachea on a full-inflated chest radiograph. RESULTS: Of the 42 patients, the chief complaints were abdominal distention (52.4%), recurrent abdominal pain syndrome (21.4%), chronic diarrhea (11.9%), acute abdominal pain (7.1%) and others (7.2%). Mean symptom duration before diagnosis was 10.6 months (range, 1 to 60 months), and it exceeded 12 months for 16 (38.1%) patients. The clinical features common to all patients were abdominal distention that increased progressively during the day, increased flatus on sleep, increased bowel sound on auscultation and an air-distended stomach with increased gas in the small and large bowel by radiography. Visible or audible air swallowing (26.2%) and repetitive belching (9.5%) were also noted. Esophageal air sign was observed in 76.2% of the patients and in 9.7% of the controls (P=0.0001). The subgroups of pathologic childhood aerophagia divided by underlying associations were pathologic childhood aerophagia without severe mental retardation (76.2%), which consisted of psychological stresses and uncertain condition, and pathologic childhood aerophagia with severe mental retardation (23.8%). CONCLUSIONS: The common manifestations of pathologic childhood aerophagia may be its essential diagnostic criteria, and esophageal air sign may be useful for the early recognition of pathologic childhood aerophagia. Our observations show that the diagnostic clinical profiles suggested by Rome II criteria should be detailed and made clearer if they are to serve as diagnostic criteria for pathologic childhood aerophagia.     

Posttraumatic stress disorders in children and adolescents.

Millions of children are exposed to traumatic experiences each year. Over 30% of these children develop a clinical syndrome with emotional, behavioral, cognitive, social, and physical symptoms called posttraumatic stress disorder. The symptoms of posttraumatic stress disorder fall into three clusters: reenactment of the traumatic event: avoidance of cues associated with the event or general withdrawal; and physiological hyperreactivity. Significant physical and medical problems in childhood, adolescence, and adulthood appear to be related to childhood trauma. Current treatment approaches include postacute psychoeducation, individual psychotherapy, pharmacotherapy, and cognitive-behavioral therapy. Despite increasing attention over the past 10 years, childhood posttraumatic stress disorder remains an understudied public health problem.     

Natural history of cardiovascular manifestations in Marfan syndrome.

AIMS: To investigate the natural history of mitral valve and aortic abnormalities in patients with Marfan syndrome during childhood and adolescence. METHODS: Fifty two patients with Marfan syndrome were followed for a mean of 7.9 years. Occurrence of adverse cardiovascular outcomes was measured clinically and by ultrasound examination. RESULTS: Mitral valve prolapse (MVP) was diagnosed in 46 patients at a mean age of 9.7 years, more than 80% of whom presented as "silent MVP". Mitral regurgitation (MR) occurred in 25 patients, aortic dilatation in 43, and aortic regurgitation (AR) in 13. Both MVP and aortic dilatation developed at a constant rate during the age period 5-20 years. In 23 patients MVP was diagnosed before aortic dilatation, in 18 the reverse occurred, and in 11 patients the two abnormalities were diagnosed simultaneously. During follow up, 21 patients showed progression of mitral valve dysfunction; progression of aortic abnormalities occurred in 13. Aortic surgery was performed in 10; two died of subsequent complications. Mitral valve surgery was performed in six. In sporadic female Marfan patients the age at initial diagnosis of MVP, MR, aortic dilatation, and AR was lowest, the grade of MR and AR most severe, the time lapse between the occurrence of MVP and subsequent MR as well as between dilatation and subsequent AR shortest, and the risk for cardiovascular associated morbidity and mortality highest. CONCLUSIONS: During childhood and adolescence in Marfan syndrome, mitral valve dysfunction as well as aortic abnormalities develop and progress gradually, often without symptoms, but may cause considerable morbidity and mortality by the end of the second decade, especially in female sporadic patients.     

Clinical features of paediatric AIDS in Uganda

A total of 177 children seen at two hospitals in Kampala are described who were strongly suspected of having acquired immunodeficiency syndrome (AIDS), either on clinical grounds or because they fulfilled the World Health Organization (WHO) case-definition criteria for diagnosis of paediatric AIDS. Blood was taken from the 177 children and 154 of their mothers and tested for antibody to human immunodeficiency virus (HIV) by an enzyme-linked immunoassay (ELISA). Altogether, 119 (67%) children were seropositive, but only 85 (71%) fulfilled the WHO case-definition criteria, and they were significantly older than the 34 who did not fulfil the criteria. A further 58 children were seronegative but fulfilled the WHO criteria. Of the 119 seropositive children, only 3 had a history of previous blood transfusion, but 103 (98%) of 105 mothers were HIV seropositive: consequently, their children were considered to have been infected in utero or perinatally. Thirteen (26%) of 49 mothers of seronegative children were seropositive. Eighty per cent of HIV-infected children were under 2 years of age at diagnosis and 23% died within 3 months of diagnosis. None of the parents was known to be an intravenous drug user, a prostitute or bisexual. The difficulty of accurate diagnosis of AIDS presents a major problem in Africa, as the WHO clinical case-definition criteria alone are clearly not adequate.     

Early and reliable diagnosis of non-Hodgkin lymphoma in childhood and adolescence: contribution of cytomorphology and flow cytometric immunophenotyping

Non-Hodgkin lymphoma (NHL) represents one of the most rapidly growing malignancies in childhood and adolescence. About 80% of patients now are cured with adequate treatment. Serious complications at presentation due to tumor lysis syndrome or local tumor effects are commonly observed. Thus, a rapid diagnosis with the least invasive procedure enabling the initiation of early and specific therapy is necessary to diminish early fatality or persistent impairment. In 56 centrally registered patients with NHL, cytomorphologic analyses (FAB criteria) of May-Grünwald-Giemsa-stained touch imprints or malignant effusions and flow cytometric immunophenotyping (EGIL criteria) of fresh cell suspensions with a standardized panel of monoclonal antibodies were performed. The authors identified 23 patients with Burkitt lymphoma by the combination of FAB L3 morphology and a mature B-cell phenotype and 22 patients with lymphoblastic lymphoma by FAB L1/L2 morphology and a T-/B-cell precursor phenotype. They also found 11 patients with large cell lymphomas, 3 of them with anaplastic large cell lymphoma (T-cell phenotype; NPM/ALK-positive). In the remaining 8 patients diffuse large B-cell lymphoma was suspected by the combined use of cytologic and immunophenotypic findings (mature B-cell phenotype). In all cases with available solid tumor material (n = 42/56) the preliminary diagnosis was confirmed by histopathology. Burkitt lymphoma, lymphoblastic lymphoma, and, in a few cases, some large cell lymphomas could be classified reliably by cytomorphology and immunophenotyping of freshly obtained tumor cell material, enabling an early start of specific lymphoma treatment.     

Raynaud's syndrome in children. Study of 23 cases]

AIM OF THE STUDY: To analyze the epidemiological characteristics of Raynaud's syndrome in childhood. PATIENTS AND METHODS: We conducted a nine-year-retrospective survey of children up to 17 y seen with Raynaud's syndrome. Charts were retrieved from pediatrics and dermatology outpatient units, and from the registry of capillaroscopy. A specific questionnaire was designed and missing data were completed after a phone interview of the parents. RESULTS: A definite diagnosis of Raynaud's syndrome was ascertained in 23 patients with marked female predominance (SR = 0.27). Triggering factors were essentially a cold environment and emotions. The mean age at the diagnosis was 11 y (5 to 16 y) with an onset after ten years in 65% of cases. Eleven of these Raynaud's syndromes were secondary with ten connectivitis, eight remained essential and four were suspected to be secondary. DISCUSSION: This series of pediatric Raynaud's syndromes was important according to scarcity of literature on this topic. The diagnosis is made upon exclusive clinical basis and we underline the high frequency of serious underlying conditions. Further etiologic investigations are mandatory for any pediatric patient with Raynaud's syndrome. The sensitivity (78%) and specificity (80%) of capillaroscopy were of interest in our patients; its prognosis value needs to be evaluated in a larger group of patients.     

Hemoglobin A1c is a reliable criterion for diagnosing type 1 diabetes in childhood and adolescence

Ehehalt S, Gauger N, Blumenstock G, Feldhahn L, Scheffner T, Schweizer R, Neu A for the DIARY‐Group Baden‐Wuerttemberg. Hemoglobin A1c is a reliable criterion for diagnosing type 1 diabetes in childhood and adolescence. Background: Little is known about the use of the hemoglobin A1C (HbA1c) test for the diagnosis of diabetes in childhood and adolescence. The aim is to investigate sensitivity and specificity of HbA1c at onset of childhood type 1 diabetes. Methods: A total of 184 children and adolescents with blood glucose levels above 200 mg/dL (11.1 mmol/L) were included: 84.8% (n = 156, mean age 9.0 yr) with new onset of type 1 diabetes, 15.2% (n = 28, mean age 6.1 yr) with transient hyperglycemia. HbA1c was measured using the Bayer^® DCA2000 analyzer. Results: Patients with new onset of type 1 diabetes (n = 156) had HbA1c values between 6.6% and > 14% (mean (SD) 11.4 (2.0)%; IQR, interquartile range 9.8–13.3%). All patients suffered from typical symptoms of hyperglycemia, i.e., polyuria and polydipsia. In the patient group with transient hyperglycemia (n = 28), HbA1c values were between 4.5 and 6.1% (mean (SD) 5.3 (0.4)%; IQR 5.0–5.6%). None of these patients reported typical symptoms of diabetes. All patients with HbA1c values greater than 6.35% had new onset of type 1 diabetes. Sensitivity of HbA1c at the onset of childhood type 1 diabetes was calculated to be 100%. In patients with HbA1c values less than 6.35%, the diagnosis of type 1 diabetes could be excluded. Thus, specificity of HbA1c as diagnostic criterion was 100%. Conclusions: Childhood type 1 diabetes can be diagnosed and excluded with high reliability by means of HbA1c testing.     

Kelley-Seegmiller syndrome

BACKGROUND: Hypoxanthinguanin-phosphoribosyltransferase (HPRT) deficiency, an x-linked inherited disease can cause two presentations: A complete deficiency (Lesch-Nyhan syndrome) accompanies with grave renal and neurological symptoms. A second form is the partial enzyme deficiency (Kelley-Seegmiller syndrome). PATIENT: We present a thirteen year old boy with relapsing hyperuricemia and hypercreatininemia. In the framework of postoperative renal insufficiency the diagnose of a Kelley-Seegmiller syndrome was elaborated. RESULT: About 25% of patients with Kelley-Seegmiller syndrome present mild neurological symptoms but never with self-destructive behavior. Pathophysiological an increased de novo purinsynthesis is present. Therefore, it comes to an overproduction of urine acid. Urolithiasis is one clinical manifestation. CONCLUSION: Relapsing urolithiasis and renal insufficiency has to be worked up. In the differential diagnosis a disorder of purine metabolism has to be discussed.     

Neugeborenes mit Arachnodaktylie, Arthrogryposis und Aortendilatation

Marfan’s syndrome is an autosomal dominant connective tissue disorder. The diagnosis of Marfan’s syndrome relies on defined revised criteria (Ghent nosology 2010) and the typical clinical features encompass aortic root dilatation, ectopia lentis and disproportionate long bone overgrowth. The clinical phenotype is highly variable and the most severe forms become symptomatic in the neonatal period and early infancy. Early diagnosis confirmed by detection of a causative mutation in the fibrillin-1 gene is important for exclusion of other diagnoses, optimal treatment and genetic counselling of the families.     

Delayed diagnoses of Turner's syndrome: proposed guidelines for change

OBJECTIVE: To measure the delays in diagnosis of Turner's syndrome (TS) and to propose strategies for earlier screening and diagnosis. METHODS: The medical records of 81 girls with TS were reviewed for age at diagnosis, reason(s) for karyotype analysis, and clinical features including growth failure. Delay in diagnosis was calculated as equal to age at diagnosis for children born with lymphedema and/or 2 or more of the following dysmorphic features: webbed neck, nail dysplasia, high palate, and short fourth metacarpal. For all others, delay in diagnosis was calculated as the difference between the age at which height fell below the 5th percentile and the age at which the diagnosis of TS was made. RESULTS: Lymphedema was the key to diagnosis in 97% of the girls diagnosed with TS in infancy, and short stature was the key to diagnosis for 82% of the girls diagnosed in childhood or adolescence. For girls diagnosed in childhood or adolescence, the delay in diagnosis averaged 7.7 +/- 5.4 years. Many had dysmorphic features and/or a history of lymphedema at birth, and diagnosis was made an average of 5.3 years after patients had fallen below the 5th percentile for height. By the time of diagnosis, patients were very short, averaging -2.9 SD in height. CONCLUSIONS: The diagnosis of TS is often delayed. We recommend karyotype analysis for all girls with unexplained short stature, delayed puberty, webbed neck, lymphedema, or coarctation of the aorta. Furthermore, karyotype analysis should be strongly considered for those who remain above the 5th percentile for height but have 2 or more features of TS, including high palate, nail dysplasia, short fourth metacarpal, and strabismus.     

ADHS im Jugend- und Erwachsenenalter

The diagnosis of attention deficit hyperactivity disorder (ADHD) in adolescence and adulthood has just recently been considered. The expression of main symptoms changes as the individual matures. Adults typically display attention deficits, restlessness, impulsivity, organizational deficits, and poor affect regulation. Research indicates a prevalence of 1–2.5%. Corresponding to ADHD in children, high rates of comorbidity and affected psychosocial functioning are reported. It is recommended to use clinical interviews to find out about actual and former symptoms as well as questionnaires for diagnosis. However, it is critical to note that obligatory diagnostic criteria are still lacking. To ascertain the beginning of the disorder, objective documentation from childhood must be considered. Stimulants accompanied by psychotherapy are thought to be the most effective treatment. Further research is urgently needed on therapeutic methods and their combinations with psychotropic drugs.     

Recurrent abdominal pain: symptom subtypes based on the Rome II Criteria for pediatric functional gastrointestinal disorders

OBJECTIVES: Recurrent abdominal pain (RAP) is a common childhood complaint rarely associated with organic disease. Recently, the Pediatric Rome Criteria were developed to standardize the classification of pediatric functional gastrointestinal disorders (FGIDs) using a symptom-based approach. The authors tested the hypothesis that most patients with childhood RAP could be classified into one or more of the symptom subtypes defined by the Pediatric Rome Criteria. METHODS: Using a prospective longitudinal design, new patients with RAP (n = 114) were studied at a tertiary care children's medical center. Before the medical evaluation, parents completed a questionnaire about their child, assessing symptoms defined by the Pediatric Rome Criteria. RESULTS: Of the 107 children for whom medical evaluation revealed no organic etiology for pain, 73% had symptom profiles consistent with the Pediatric Rome Criteria for one of the FGIDs associated with abdominal pain (irritable bowel syndrome, 44.9%; functional dyspepsia,15.9%; functional abdominal pain, 7.5%; abdominal migraine, 4.7%) CONCLUSIONS: This study provides the first systematic empirical evidence that RAP, originally defined by Apley, includes children whose symptoms are consistent with the symptom criteria for several FGIDs defined by the Rome criteria. The pediatric Rome criteria may be useful in clinical research to (1) describe the symptom characteristics of research participants who meet Apley's broad criteria for RAP, and (2) select patients with particular symptom profiles for investigation of potential biologic and psychosocial mechanisms associated with pediatric FGIDs.