Caplan Syndrome

Zielonka TM, Bareła D, Zukowska M, Langfort R, Rudziński P, Chazan R. · Katedra i Klinika Chorób Wewnetrznych, Pneumonologii i Alergologii Akademii Medycznej w Warszawie. · Pol Arch Med Wewn. · Pubmed #16808317 No free full text.

Abstract: A case of a 56-years old male with rheumatoid arthritis and unclear tumoral radiological changes in the lungs was described. Since noninvasive diagnostic procedures failed to explain nature of the pulmonary changes, an open pulmonary biopsy was performed. Pathological examination revealed presence of rheumatoid nodules and pneumoconiosis, typical for Caplan's syndrome. In the past, the patient had been working in foundry industry for 16 years and he had been exposed to silica and iron dust. Articular symptoms were revealed prior to finding the lung changes even dust exposure had occurred many years earlier. In spite of the fact that rheumatoid arthritis is a relatively common disease and pneumoconiosis is also not rare entity, the coexistence of both conditions i.e. Caplan's syndrome has been rarely diagnosed and described.

Rizzo G, Scaglione C, Lopane G, Pollini A, Tommasoni E, Martinelli P. · Department of Neurological Sciences, University of Bologna, Via Ugo Foscolo 7, I-40123 Bologna, Italy. · Neurol Sci. · Pubmed #16688603 No free full text.

Abstract: Parkinsonism is a rare feature of immunological diseases. We describe a 67-year-old man with Caplan's syndrome (CS) and parkinsonism. CS is an immunologic disease characterised by the presence of rheumatoid arthritis associated with a specific form of pneumoconiosis. Parkinsonism as a manifestation of involvement of the central nervous system in this condition has never been reported. Following immunosuppressive treatment both the CS and the parkinsonian signs and symptoms showed a marked improvement. The role of immune mechanisms in these parkinsonian syndromes is discussed.

Caplan A, Walker L, Rasquin A. · Department of Psychology, Université de Montréal, Montreal, Canada. · J Pediatr Gastroenterol Nutr. · Pubmed #16131985 No free full text.

Abstract: OBJECTIVE: To validate the pediatric Rome II criteria for functional gastrointestinal disorders (FGIDs) using the Questionnaire on Pediatric Gastrointestinal Symptoms (QPGS). METHODS: Subjects were 315 consecutive new patients, 4 to 18 years of age, seen in a tertiary care clinic and classified by pediatric gastroenterologists as having a functional problem. Patients and parents separately completed the QPGS before medical consultation. Diagnoses were derived using computer algorithms reflecting the Rome II criteria for pediatric FGIDs. Convergent validity was assessed by prevalence of diagnoses and internal validity using factor analysis to confirm symptom clusters of the criteria. Separate analyses were performed for 4 to 9 and 10 to 18 year olds, and for diagnoses based on parent and child reports. RESULTS: In both age groups, the most prevalent diagnoses were irritable bowel syndrome (IBS) (22.0%, 35.5%), functional constipation (19.0%, 15.2%), and functional dyspepsia (FD) (13.6%, 10.1%). Parent-child concordance on diagnoses was generally poor. Factor analyses supported the internal validity of FD and of IBS symptoms except for relief with defecation. Although functional abdominal pain syndrome and abdominal migraine occurred rarely, symptom clustering within each diagnosis supports their validity. Among patients with abdominal pain, duration was of at least 3 months in most, and pain was of long duration and severe in at least one third. CONCLUSION: More than half of patients classified as having a functional problem met at least one pediatric Rome II diagnosis for FGIDs. This study offers initial support for the validity of several of the criteria.

Walker LS, Lipani TA, Greene JW, Caines K, Stutts J, Polk DB, Caplan A, Rasquin-Weber A. · Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee 37232-3571, USA. · J Pediatr Gastroenterol Nutr. · Pubmed #14734882 No free full text.

Abstract: 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.

Caplan J, Angel M, Parent A. · Divisions of Plastic Surgery and Neurosurgery, University of Mississippi Medical Center Jackson, Mississippi 39214, USA. · J Craniofac Surg. · Pubmed #12140411 No free full text.

Abstract: Encephalocele is a rare late complication of craniosynostosis repair. An undiagnosed nick to the dura is the etiology of this complication. It usually presents within a few months to years after repair. This case report describes a patient with Crouzon's Disease who had a craniosynostosis repair as an infant and presents 22 years later with an encephalocele. The patient had a thorough physical exam, 2D, and 3D CT Scans as a preoperative workup. Surgical intervention included repair of the encephalocele, cranialization of the frontal sinus with bone grafting, and Lefort III osteomies for mid face advancement. The patient benefited from a two-team simultaneous approach between Neurosurgery and Plastic Surgery. The patient also benefited from an aggressive one-stage repair of all her defects and deformities.

Westbury C, Bub D, Chertkow H. · Department of Psychology, University of Alberta, Edmonton, Canada. · Brain Cogn. · Pubmed #12030517 No free full text.

Abstract: Mesulam's (1982) report describing six patients with a slowly progressive aphasia without accompanying signs of dementia led to the recognition of a syndrome now known as Primary Progressive Aphasia (PPA). Many more patients have been described since Mesulam's description was published (see Westbury & Bub, 1997, for a review). However, the published literature is both unsystematic and incomplete, making it difficult to place the findings into a coherent theoretical framework. In addition, little previous work (see Mesulam, 1987) has specifically attempted to specify the difference between PPA and dementia of Alzheimer's type (DAT), although the two disorders are easily confused since many language deficits can masquerade on early presentation as memory or cognitive deficits. In this paper, the linguistic deficits of 11 PPA patients are analyzed, and contrasted with the linguistic deficits of a group of 11 DAT patients. Patients in both groups were tested using an extensive battery of language tests, the Psycholinguistic Assessment of Language Battery (Caplan & Bub, 1990; Caplan, 1992). We consider seven linguistic symptom clusters that differentiate the two groups.

Caplan A. · No affiliation provided · Med Lav. · Pubmed #11899941 No free full text.

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Caplan R, Austin JK. · Department of Psychiatry, University of California at Los Angeles, Los Angeles, California, USA. · Ment Retard Dev Disabil Res Rev. · Pubmed #11107194 No free full text.

Abstract: Epilepsy and mental retardation, two relatively common childhood conditions, are both associated with a wide range of behavioral disorders. This article reviews the behavioral disturbances found in children with epilepsy, mental retardation, and both conditions. The behavioral disturbances found in children with epilepsy are associated with seizure-related, cognitive, developmental, and psychosocial factors. Although children with mental retardation also demonstrate a broad spectrum of behavioral disturbances, children with specific mental retardation syndromes have better-defined patterns of psychopathology. The presence of epilepsy and mental retardation seems to increase the severity of psychopathology. Further studies are needed, however, to define better the interaction of these two conditions and how they impact the behavior of children.

Caplan S, Dell'Angelica EC, Gahl WA, Bonifacino JS. · Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 18T Room 101, 18 Library Dr. MSC 5430, 20892-5430, Bethesda, MD, USA. · Immunol Lett. · Pubmed #10841946 No free full text.

Abstract: The major histocompatibility complex class II subunits (MHC-II) alpha and beta assemble with the invariant chain (Ii) in the endoplasmic reticulum and are transported to endosomal-lysosomal organelles known as MHC class II compartments (MIICs). Although it has been shown that two dileucine-based signals in the cytosolic tail of Ii, as well as a dileucine-based signal in the tail of the beta chain mediate sorting to MIICs, the molecular mechanisms by which alphabetaIi complexes are sorted have yet to be resolved fully. The AP-3 adaptor complex stands out as a particularly good candidate for mediating this targeting because: (i) it has a proven role in the trafficking of membrane proteins to lysosome-related organelles; and (ii) it has the ability to interact with dileucine-based signals in vitro. To investigate the potential role of AP-3 in transport of MHC-II to MIICs, we have examined MHC-II trafficking in human B-lymphoblast lines from patients with Hermansky-Pudlak syndrome type 2 (HPS-2), which are deficient in the AP-3 complex. Pulse-chase analyses revealed no significant alteration in the kinetics of synthesis and degradation of either MHC-II subunits or Ii. Moreover, we observed neither impairment of the formation of compact SDS-resistant alphabeta dimers, nor delay in the appearance of a conformational epitope indicative of a mature, Ii-free alphabeta dimer. Finally, we demonstrated that in HPS-2 patients' cells, there was no delay in the expression of the alphabeta dimers on the cell surface. Thus, AP-3 does not seem to be essential for normal trafficking of MHC-II. These findings have important implications for HPS-2 patients, because they suggest that the recurrent bacterial infections suffered by these patients are not likely due to impaired antigen processing and presentation by MHC-II.

Caplan JP. · No affiliation provided · Psychosomatics. · Pubmed #16959937 links to  free full text

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Gyger M, Rosenberg A, Shamy A, Hercz A, Caplan S, Sebag IA, Brisson ML, Roy DC. · No affiliation provided · Bone Marrow Transplant. · Pubmed #15558044 No free full text.

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Caplan RH. · No affiliation provided · J Clin Endocrinol Metab. · Pubmed #10372747 links to  free full text

This publication has no abstract.