Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors are reporting a new case of widespread scabies in a baby. They take this opportunity to emphasize on the atypical erythematous and excoriated papular rash which sometimes may be vesicular and hyper-keratotic. This widespread eruption may mimic generalised dermatitis, pustular psoriasis and even histiocytosis X. They also underline importancy of longlasting ointment with fluorinated steroid being responsible for this widespread eruption.
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PMID:[Extensive scabies in a baby (author's transl)]. 23 38

Monozygotic twin boys presented at 1 year of age with seborrheic skin rash, otorrhea, and hepatosplenomegaly. Skin biopsy confirmed Langerhans cell histiocytosis. Treatment with conventional antineoplastic drugs and with calf thymus extract was ineffective. The disease remained refractory to recombinant human alpha-interferon and to low-dose total body irradiation, and the children died between 3 and 3 1/2 years of age.
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PMID:Disseminated Langerhans cell histiocytosis in identical twins unresponsive to recombinant human alpha-interferon and total body irradiation. 151 Jan 99

Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Congenital cutaneous Langerhans histiocytosis. Apropos of 7 cases]. 160 6

Forty-one patients with Langerhans Cell Histiocytosis (LCH) were treated over a thirty-one year period in our institution. These children were classified according to the number of systems involved: twenty-two had unisystem disease while nineteen had multisystem disease. A histological diagnosis was reached in 82% of cases, the remainder being diagnosed on both radiological and clinical grounds. 68% of those with multisystem disease had a rash at diagnosis whilst 64% had a persistent ear discharge. The diagnosis was established accidentally in 25% of those with unisystem disease. The mortality rate was 21% and was confined to those who were under two years of age at diagnosis, all of whom had multisystem disease. Morbidity was 20% and was restricted to patients with multisystem disease. Only one patient died within the last 10 years; there were no therapy related deaths. Treatment related morbidity was seen in only three children. In keeping with other series, our review has identified the following adverse prognostic factors a) age under 2 years at presentation, b) multisystem disease and c) major organ dysfunction. In view of the natural history of the disease, it is suggested that chemotherapy only be used in those patients who have major organ dysfunction or progressive disease and that radiotherapy is rarely indicated.
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PMID:Langerhans cell histiocytosis--a 31 year review. 176 7

Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus formation secondary to lymph node disease has never before been recorded as a manifestation of histiocytosis X. Histiocytosis X should therefore be considered in the differential diagnosis of "suppurative" lymphadenopathy so that appropriate treatment may be given without delay.
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PMID:Chronic dermal sinuses as a manifestation of histiocytosis X. 308 14

The records of 70 patients presenting to this hospital since 1961 with histiocytosis X confirmed by biopsy examination have been reviewed. The patients were subdivided into three groups: group A, those under 2 years of age at diagnosis; group B, those between 2 and 5 years; and group C, those over 5 years. All eight patients who died (11% overall mortality) were under 2 years of age at diagnosis. Involvement of lung, liver, and bone marrow were confirmed as poor prognostic features. The presence of bone disease and absence of skin rash were identified as favourable features.
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PMID:Favourable prognostic features in histiocytosis X: bone involvement and absence of skin disease. 349 68

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
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PMID:New insight into the causes of immunodeficiency disorders. 638 1

Twenty-eight patients with histiocytosis X (HX) who had bone marrow aspirates and/or trephine biopsies taken at diagnosis were studied. Five of the 28 patients (18%) had bone marrow specimens with clusters of benign histiocytes characteristic of histiocytosis X. Several statistically significant clinical and laboratory differences were found between those patients with positive marrows versus those with uninvolved bone marrows. Presence of HX in the bone marrow was more frequently associated with fever (P less than 0.01), splenomegaly (P less than 0.01), rash (P = 0.04), diabetes insipidus (P = 0.09), and platelet counts less than 175 X 10(9)/L(P = 0.01). Two of the five patients with bone marrow involvement died, whereas there were no deaths due to HX in the other 23 patients. From this review, it is suggested that bone marrow involvement at diagnosis may be associated with more extensive and potentially fatal disease. HX of the marrow was more easily diagnosed from trephine biopsy sections than aspiration smears.
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PMID:Bone marrow involvement in histiocytosis X. 660 70

The authors experienced a case of histiocytosis X with a large intracranial mass resulting in a convulsive seizure. The patient showed left exophthalmos and a skin rash one year and two months after birth. Histiocytosis X was diagnosed from a skin biopsy, and predonine, endoxan and vincristine were administered. The rash disappeared, but the exophthalmos remained. At the age of two years and nine months, punched-out lesions appeared in the skull and 4,000 rads of radiation was applied. Thereafter, the exopthalmos persisted but there was no particular problem in the course. However, a convulsive seizure with fever suddenly appeared at nine years and ten months of age and the patient was hospitalized. At the time of admission, the general condition was good and there were no abnormalities in neurological tests. In neuroradiological examinations, a calcified and poorly vascularized mass 8 cm in maximum diameter was found to occupy the left middle cranial fossa. Chondrosarcoma was strongly suspected from these findings, but there was also symmetrical thickening of bone cortex in the peripheries of the long bones of the extremeties which appeared to be the recovery process from bone destruction caused by histiocytosis X. Therefore, the formation of an intracranial mass by histiocytosis X was diagnosed and surgery was performed. When left osteoplastic fronto-temporal craniotomy was performed, the mass was found to be raising the temporal lobe and it could be easily separated from the surrounding tissue. However, these was firm adherence to dura mater of the middle cranial fossa (especially that of the superior orbital fissure). Histologically, there were many cells with small nuclei, no polymorphism, abundant and clear cytoplasm which were darkly stained and slightly atypic. These findings matched those for histiocytosis X. Cases of histiocytosis X rarely show symptoms of the central nervous system or infiltration of the central nervous system. Only 31 such cases were seen in the literature investigated by the authors. Neurological symptoms include pyramidal symptoms such as hemiparesis and impairment of the cranial nerves, particularly paresis of the optic, trigeminal, facial and acoustic nerves. Convulsive seizures were seen in only five cases including the one reported here. It is also rare for intracranial masses to be formed in cases of histiocytosis X and only six cases, including the authors', have been found with masses of a maximum diameter of more than 5 cm.
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PMID:[Case of histiocytosis X with a large intracranial mass]. 660 36


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