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)

VP-16-213, a semisynthetic podophyliotoxin, was tested for antitumor and clinical toxicity in 126 children. The drug was administered iv daily x 5 days every 2 weeks at a starting dose of 75 mg/m2/day. The dose was increased by 25 mg/m2/day/course until clinical response or significant toxicity occurred. The only major toxicity was hematologic, with neutropenia as the most predominant feature. There was one local allergic reaction at the site of injection. No systemic allergic responses were reported. The drug demonstrated significant activity in acute myelomonocytic leukemia with four responses among 19 patients, less activity in acute myelocytic leukemia with two responses among 44 patients, and little activity in acute lymphocytic leukemia with only one partial response among 12 patients. Objective partial responses occurred in ten of 48 patients with solid tumors: two each with Wilms' tumor, lymphoma, and histiocytosis X, and one each with rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and undifferentiated carcinoma. The inclusion of VP-16-213 in combination chemotherapy for childhood acute myelomonocytic leukemia and acute myelocytic leukemia appears indicated in patients relapsing after initial therapy. For solid tumors this is an interim report, with further patient accrual required before specific comments can be made.
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PMID:Phase II study of VP-16-213 in childhood malignant disease: a Children's Cancer Study Group Report. 29 6

We report on two children with malignancy who showed fungemia despite the antifungal treatment with fluconazole. Case 1 was a 7-year-old girl with a recurrence of stage IV neuroblastoma. She had profound neutropenia and fungemia developed after a month-long treatment with fluconazole. Her peripheral blood smear showed phagocytosis in the neutrophils and they were identified as fungi by immunofluorescence method (Fungi flora Y). She died two days after the diagnosis of fungemia. Rhodotorula rubra was isolated after her death. Case 2 was a 2-year-old boy with disseminated Langerhans cell histiocytosis. He had profound neuropenia and fungemia developed after treatment with fluconazole for 6 months. His peripheral blood smear also showed phagocytosis in the neutrophils and they were identified as fungi by Fungi flora Y. He was treated with intravenously administered amphotericin-B. However, he died 13 days after the diagnosis of fungemia. Candida guilliermondii was isolated after his death. Careful observation of the peripheral blood smear is important for early detection of fungi and Fungi flora Y is a quick and useful method to identify fungi. Fluconazole-resistant fungus should be considered when patients with neutorpenia are treated prophylactically with fluconazole for a long time.
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PMID:[Phagocytosis of fungi in the peripheral blood neutrophils of two children with cancer during treatment with fluconazole]. 931 Dec 73

Amifostine protects normal tissues from the cytotoxicity of chemotherapy and radiotherapy and has an effect a growth factor both in vivo and in vitro. To evaluate its possible hemopoietic promoting activity, the authors studied the effect of WR-2721 on cord blood and bone marrow progenitors from children with hematological disorders and normal controls in semisolid cultures after preincubation with 0.1-1000 microM amifostine. Amifostine enhanced the growth of BFU-E in autoimmune neutropenia but inhibited CFU-GM development. It also exerted an inhibitory effect upon growth of committed progenitors in the groups studied (acute lymphoblastic leukemia, Langerhans cell histiocytosis), including cord blood and marrow of healthy controls.
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PMID:Amifostine as differentiating agent in cord blood and bone marrow cultures from children with hematological disorders. 1516 May 11

Congenital neutropenia predisposes to the development of haematopoetic malignancies. We present a 3,5-year child, diagnosed with congenital neutropenia at the age of I month. The diagnosis was based on peripheral blood and bone marrow aspirate analyses, performed after the treatment of multiple axillar abscesses. Recurrent infections were treated with broad-spectrum antibiotics and leukocyte colony stimulating factors. At the age of 19 months during routine check-up the child presented with gingival hypertrophy, fragility and bleeding. The histopathological analysis of gingival biopsy was consisted with the diagnosis of Langerhans cell histiocytosis. The child was treated according to LCH protocol for therapeutic group C. After 12 months of such chemotherapy, the follow-up histopathological analysis of gingival biopsy revealed the presence of Langerhans cells, which was the indication for chemotherapy prolongation (6 additional VP courses). Chemotherapy was completed after 6 additional courses and the disease remission was confirmed by histopathological analysis of gingival biopsy. Congenital neutropenia predisposes to myeloproliferative disorders, particularly to childhood myeloblastic leukemia. Based on the presented case report we would like to emphasize the possibility of other hematological syndromes development.
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PMID:[Langerhans cell histiocytosis in a child with congenital neutropenia]. 1530 29

The hereditary dentine disorders, dentinogenesis imperfecta (DGI) and dentine dysplasia (DD), comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine structure affecting either the primary or both the primary and secondary dentitions. DGI is reported to have an incidence of 1 in 6,000 to 1 in 8,000, whereas that of DD type 1 is 1 in 100,000. Clinically, the teeth are discoloured and show structural defects such as bulbous crowns and small pulp chambers radiographically. The underlying defect of mineralisation often results in shearing of the overlying enamel leaving exposed weakened dentine which is prone to wear. Currently, three sub-types of DGI and two sub-types of DD are recognised but this categorisation may change when other causative mutations are found. DGI type I is inherited with osteogenesis imperfecta and recent genetic studies have shown that mutations in the genes encoding collagen type 1, COL1A1 and COL1A2, underlie this condition. All other forms of DGI and DD, except DD-1, appear to result from mutations in the gene encoding dentine sialophosphoprotein (DSPP), suggesting that these conditions are allelic. Diagnosis is based on family history, pedigree construction and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered. Differential diagnoses include hypocalcified forms of amelogenesis imperfecta, congenital erythropoietic porphyria, conditions leading to early tooth loss (Kostmann's disease, cyclic neutropenia, Chediak-Hegashi syndrome, histiocytosis X, Papillon-Lefevre syndrome), permanent teeth discolouration due to tetracyclines, Vitamin D-dependent and vitamin D-resistant rickets. Treatment involves removal of sources of infection or pain, improvement of aesthetics and protection of the posterior teeth from wear. Beginning in infancy, treatment usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition. Where diagnosis occurs early in life and treatment follows the outlined recommendations, good aesthetics and function can be obtained.
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PMID:Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. 1902 96

From March 1991 through 31st December 2007, 2042 patients underwent stem cell transplantation at the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. These transplantations included 1405 allogeneic stem cell transplantation, 624 autologous stem cell transplantation, and 13 syngeneic stem cell transplantation. Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. We had 105 cellular therapies for postmyocardial infarction, multiple sclerosis, cirrhosis, head of femur necrosis, and renal cell carcinoma. About 30 patients were retransplanted in this center. About 74.9% of the patients (1530 of 2042) remained alive between one to 168 months after stem cell transplantation. Nearly 25.1% (512 of 2042) of our patients died after stem cell transplantation. The causes of deaths were relapse, infections, hemorrhagic cystitis, graft versus host disease, and others.
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PMID:Stem cell transplantation; Iranian experience. 1911 Oct 33

This study evaluates the clinical and laboratory data of children with secondary hemophagocytic lymphohistiocytosis (sHLH) related to malignancy. Charts of patients who met the diagnostic criteria for sHLH associated with malignancy between January 2000-2006 at six different hospitals in Turkey were reviewed retrospectively. The diagnosis of HLH had been established by bone marrow aspiration in 27 patients, cerebrospinal fluid and bone marrow aspiration in one patient and lung-liver biopsy in another. Twenty-nine children were diagnosed as having sHLH related to malignancy. Twenty cases (18 ALL and 2 AML) with acute leukemia (10 girls/10 boys, median age: 8 years [3-14 years]) were found to have sHLH. Five patients with acute leukemia had HLH at the time of diagnosis (Group 1a), and 15 patients with acute leukemia were diagnosed as having sHLH during therapy (Group 1b), namely reactive sHLH associated with the chemotherapy. Nine patients, including two cases each of rhabdomyosarcoma, neuroblastoma, Hodgkin disease, and non-Hodgkin lymphoma (NHL) and one case with Langerhans cell histiocytosis, were diagnosed as having concomitant hemophagocytosis at the initial evaluation of the tumor (Group 2). Fever, anemia, and hypertriglyceridemia were present in all sHLH cases of all three groups. Hepatomegaly was detected in 60.0%, 73.3%, and 88.8% of the three groups, respectively. Splenomegaly was more frequent in patients of Groups 1a (60.0%) and 2 (88.8%) than in those of Group 1b, the reactive ones (13.3%). Hypofibrinogenemia was detected in all patients of Group 1a and Group 2. Low level of fibrinogen was present in 91.6% of patients in Group 1b. All patients in Group 1b (100%) had neutropenia and thrombocytopenia. Neutropenia was found at rates of 60.0% and 55.5% in Group 1a and Group 2, respectively. Thrombocytopenia was detected in 80.0% of patients in Group 1a and 77.7% in Group 2. The overall mortality rate was 34.4% (10 cases) in our series of 29 children with sHLH; 50% of deaths were directly attributable to HLH. Pediatric malignancy-associated HLH patients have been commonly described as case presentations or in a review of the literature. We believe that our cohort, compiling 29 children regarding the association between malignancy and HLH, will be useful for pediatricians who are interested in this still mysterious topic.
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PMID:Malignancy-associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. 1981 62

Bacteraemia and endocarditis are the most frequently reported clinical infections due to Abiotrophia defectiva species. This species has been rarely implicated in infections in neutropenic patients. We report a rare case of long-term venous catheter-related infection caused by A. defectiva that occurred in a febrile child who had neutropenia and Langerhans' cell histiocytosis.
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PMID:Intravascular catheter-related bloodstream infection caused by Abiotrophia defectiva in a neutropenic child. 2337 63

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.
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PMID:Oral manifestion of Langerhans cell histiocytosis mimicking inflammation. 2499 57

Premature exfoliation of primary teeth is an important diagnostic event warranting urgent investigation. The majority of conditions presenting with early loss of teeth are serious and in some cases could be fatal. The most common causes of premature tooth loss are Papillion-Lefevre syndrome, Chediak-Higashi syndrome, hypophosphatasia, neutropenia, leukemia and in some cases Langerhans cell histiocytosis (LCH). LCH is a disorder of unknown cause, characterized by abnormal proliferation of histiocytes. The disease has a predilection for children, although LCH may occur in adults. Owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed or missed and in which many questions remain unanswered, ranging from etiology and pathogenesis to therapy. The purpose of the review is, therefore, to raise awareness of the disease and to highlight the clinical findings that should make the odontologist or primary caregiver suspect the diagnosis.
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PMID:Premature loss of primary teeth with gingival erythema: An alert to dentist. 2660 20


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