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Primary nasal, paranasal, oral and pharyngeal (NPOP) lymphoma, thought to be a distinct entity among childhood lymphomas, may present with a wide variety of common ENT symptoms such as nasal obstruction, rhinorrhoea, epistaxis or sinusitis. The diagnosis may only be recognized when the disease results in symptoms such as visual loss, facial paraesthesia or lymphadenopathy, or systemic symptoms, such as fatigue, bone pain or abdominal pain. Full radiological assessment plays a vital part in making the diagnosis and planning treatment. Computerized tomography (CT) gives excellent bony detail but magnetic resonance imaging (MRI) using T2-weighted images, allows differentiation of mucosal thickening and retained sinus secretions from the tumour. Extension into the surrounding spaces and the cranial fossa is best assessed by coronal and sagittal T2 images. MRI is the best technique for follow-up because no radiation is involved and better soft tissue delineation improves the distinguishing of tumour from fibrosis.
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PMID:B-cell non-Hodgkin's lymphoma of the paranasal sinuses. 816 17

For proper use of systemic GCS, a basic knowledge of the normal HPA axis, as well as knowledge of the pharmacology, clinical usage guidelines, and adverse reactions of these agents is imperative. Both short-term (acute) and long-term side effects should be well known by the physician. The pros and cons of oral and parenteral therapy for various disorders and in various situations should be recognized. For long-term therapy, an intermediate-acting agent such as prednisone in single, early morning doses is most commonly used to minimize suppression of the HPA axis. Alternate-morning doses produce even less suppression if the disease process will respond. A through patient history, including general medical history and medications the patient is taking, is important to anticipate any potential problems. Weight and blood pressure should be checked initially and every 1 to 3 months thereafter. Blood glucose, electrolytes, and lipid studies, including triglycerides, should be done approximately every 6 months. An ophthalmology examination should be performed every year, and stool examination for occult blood and chest radiography can be obtained as indicated. Bone density studies might be necessary in patients who are at high risk for osteoporosis. Specific acute situations may dictate other studies. The patient on long-term GCS should be kept as active as possible, as mild-to-moderate exercise helps prevent certain side effects, such as osteoporosis. The dose of oral GCS is best given with food to prevent gastrointestinal irritation, and agents to decrease gastric acidity might be needed in certain situations. Exposure to infections should be prevented, where possible, and treatment initiated at the first sign of systemic or cutaneous infection. Pain should be evaluated early, especially abdominal pain or bone pain; MRI is indicated if aseptic necrosis of bone is suspected. Both trauma and severe sun exposure should be avoided. Consultation with other specialists is strongly recommended when the situation dictates. Diet is one of the most important strategies to minimize side effects from long-term GCS therapy. Vegetable protein should be increased in the diet, and fats and carbohydrates limited. Adequate calcium is imperative, and calcium supplementation is recommended for high-risk osteoporosis patients. Small amounts of vitamin D may be necessary to increase absorption of calcium. Restriction of sodium is also important, as is maintainance of dietary potassium. Supplemental potassium may be necessary in some patients, and a thiazide diuretic might be useful in patients with hypertension, edema, or osteoporosis. Vitamin C can be given to promote wound healing. A good doctor-patient relationship is important in managing the patient on long-term GCS. The patient must return for regular visits and be encouraged to promptly report any adverse reactions to the physician. If these criteria are maintained and the strategies noted previously are followed, problems from long-term therapy with GCS will be minimized.
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PMID:Minimizing complications from systemic glucocorticosteroid use. 878 96

During the months of September 1993 through February 1994, an outbreak of hemorrhagic fever occurred in the city of Jayapura, the provincial capital of Irian Jaya, Indonesia. Seventy-two patients (age range = 1-41 years) with suspected dengue hemorrhagic fever (DHF) were enrolled into the outbreak investigation conducted during October-November 1993. The pediatric patient population consisted of 36 individuals ages 1-12 years of age with a similar male to female ratio. From clinical histories obtained from the children diagnosed with DHF (n = 23), the predominant complaints were fever (100%), headache (96.7%), vomiting (47.8%), abdominal pain (39.1%), back/bone pain (39.1%), cough (39.1%), sore throat (21.7%), convulsions (17.4%), and eye pain (13.0%). Clinical findings of the same pediatric patients included a positive tourniquet test result (100%), thrombocytopenia (100%), hemoconcentration (100%), skin petechiae (43.5%), epistaxis (39.1%), and maculopapular rash (26%). All four of the children diagnosed with DHF grade IV had hepatomegaly, pleural effusion, ascites, cold perspiration, and confusion. Serologic data demonstrated that a majority (46 of 70, 68.7%) of the individuals assessed did not have significant levels of IgM specific for dengue viruses at the time of their admission. However, the nine successful dengue virus isolations were only from these serononreactive cases (19.6%). From the other patients assessed, 11.4% had a primary (or first exposure) serologic response to dengue virus antigen (predominantly IgM); 17.1% had a secondary (or subsequent exposure) serologic response to the same dengue antigens (predominantly IgG response) and 5.7% (four adults) had indeterminate serologic data that could not differentiate between reactivity to dengue or Japanese encephalitis virus antigen preparations. Virus culture of blood samples produced nine dengue virus isolates: DEN- 1 (2), DEN-2 (1), and DEN-3 (6). Japanese encephalitis and influenza viruses were not isolated from blood and pharyngeal specimens, respectively, from any of the patients. Thus, this first reported outbreak of DHF in Irian Jaya, Indonesia was found to be attributed to dengue viruses types 1, 2, and 3.
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PMID:The first reported outbreak of dengue hemorrhagic fever in Irian Jaya, Indonesia. 924 17

Although hypercalcemia is a well-recognized complication in malignant disorders, neither the incidence and prognostic significance of hypercalcemia, nor the role of parathormone related peptide (PTHrP) in pediatric acute lymphoblastic leukemia (ALL) have been clarified. Of 83 newly diagnosed pediatric ALL patients with early pre-B cell phenotype treated at our hospital during the last 8 years, four patients were diagnosed as having hypercalcemia (> 14 mg/dl). In these 4 hypercalcemic ALL patients at onset, serum calcium levels ranged from 14.6 to 20.8 mg/dl (normal 7.4-9.0 mg/dl), and serum PTHrP levels were markedly elevated to 112-240 pmol/l (normal range: 17.6-61.2 pmol/l). Unlike patients with ordinary ALL in childhood, gastrointestinal symptoms (nausea, vomiting, abdominal pain) and skeletal symptoms (bone pain, gait disturbance) were the chief complaints. Because of these characteristic symptoms, bone marrow aspiration was carried out in two patients in an attempt to diagnose ALL before leukemic cells appeared in peripheral blood. Serum calcium levels were promptly normalized by induction chemotherapy. The four patients have been in complete remission from 35+ to 125+ months. Based on these results, the incidence of hypercalcemia in pediatric ALL patients with early pre-B cell phenotype at our institute is calculated to be about 4.8%. Gastrointestinal and skeletal problems are the characteristic initial symptoms, and hypercalcemia does not seem to be significant in the prognosis of these patients.
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PMID:Hypercalcemia in children presenting with acute lymphoblastic leukemia. 940 Dec 81

A 67-year-old woman has a 20-year history of recurrent abdominal pain, diarrhea and diffuse bone pain. During the course of numerous hospitalisations the diagnoses "iron deficiency anemia", "iron absorption disorder", "osteoporosis" and "hyperparathyroidism" had been made. Despite treatment with vitamin D3, calcium, fluorides and iron, the patient's condition deteriorated to such a degree that she became in need of constant care. After 20 years of illness, nontropical sprue (celiac disease) with secondary intestinal osteopathy was identified. High-dose parenteral treatment with vitamin D3, oral calcium supplementation and a gluten-free diet resulted in an improvement of the patient's condition within three months, and the patient can now largely look after herself again.
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PMID:[Severe osteomalacia in endemic sprue. An important differential diagnosis in osteoporosis]. 957 65

Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and prompt therapy have the potential to reduce mortality. Leukemias, lymphomas and central nervous system tumors account for more than one half of new cancer cases in children. Early in the disease, leukemia may cause nonspecific symptoms similar to those of a viral infection. Leukemia should be suspected if persistent vague symptoms are accompanied by evidence of abnormal bleeding, bone pain, lymphadenopathy or hepatosplenomegaly. The presenting symptoms of a brain tumor may include elevated intracranial pressure, nerve abnormalities and seizures. A spinal tumor often presents with signs and symptoms of spinal cord compression. In children, lymphoma may present as one or more painless masses, often in the neck, accompanied by signs and symptoms resulting from local compression, as well as signs and symptoms of systemic disturbances, such as fever and weight loss. A neuroblastoma may arise from sympathetic nervous tissue anywhere in the body, but this tumor most often develops in the abdomen. The presentation depends on the local effects of the solid tumor and any metastases. An abdominal mass in a child may also be due to Wilms' tumor. This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. A tumor of the musculoskeletal system is often first detected when trauma appears to cause pain and dysfunction out of proportion to the injury. Primary care physicians should be alert for possible presenting signs and symptoms of childhood malignancy, particularly in patients with Down syndrome or other congenital and familial conditions associated with an increased risk of cancer.
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PMID:Recognition of common childhood malignancies. 1077 55

Systemic mastocytosis (SM), as opposed to cutaneous-only mastocytosis, implies the presence of neoplastic mast cell infiltration in extracutaneous tissue. Mast cell disease in adults is often systemic and often involves the bone marrow. Typical clinical and laboratory features of SM include urticaria pigmentosa, mast cell mediator symptoms (eg, headache, flushing, lightheadedness, urticaria and pruritus, nausea, diarrhea, abdominal pain, and vasodilatory shock), bone pain (eg, osteoporosis, lytic bone lesions, and fractures), hepatosplenomegaly, cytopenia, eosinophilia, elevated serum tryptase and histamine, and bone marrow fibrosis and angiogenesis. SM may be indolent (no evidence of organ dysfunction), aggressive (presence of organ dysfunction), associated with another often chronic myeloid hematologic disease (SM-AHD), or present as mast cell leukemia or sarcoma. Mast cell-mediator symptoms are treated with histamine antagonists and cromolyn sodium. Indolent SM does not require cytoreductive therapy. Aggressive SM and SM-AHD are managed based on their molecular profile. Recent information suggests that FIP1-like-1-platelet-derived growth factor receptor-alpha(+) SM responds well to imatinib mesylate, whereas interferon-alpha should be considered as a first-line treatment in all of the other cases, including patients with Asp816Val(+) SM. Cladribine has been shown to be effective in patients who develop resistance to interferon treatment.
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PMID:Systemic mastocytosis: current concepts and treatment advances. 1508 68

A dengue outbreak occurred in Kaohsiung City starting in July in 2001. We studied the clinical profile of all patients admitted to Kaohsiung Veterans General Hospital during this outbreak from July 2001 to January 2002. A total of 25 cases of clinically suspected dengue fever were treated during this period, and 13 of them were confirmed by laboratory results (13/25; 52%). Eleven of the 25 patients (11/25; 44%) were admitted. The mean age of the patients with laboratory confirmation of infection was 53 years (range, 7 to 85 years). Headache (7/13; 53.8%), bone pain (8/13; 61.5%), myalgia (10/13; 76.9%), abdominal pain (7/13; 53.8%), and skin rash (9/13; 69.2%) were the most common presentations. A high proportion of patients were classified as having dengue hemorrhagic fever (DHF) [6/13; 46.2%] and 2 of these patients had dengue shock syndrome (DSS) based on the World Health Organization criteria. Pretibial petechia (6/13; 46.1%), gastrointestinal bleeding (6/13; 46.1%), and hemoptysis (4/13; 30.8%) were the most common hemorrhagic manifestations. The average hospital stay was 7.1 days. Thrombocytopenia was very common and 84.6% patients had a platelet count less than 100,000/mm3. Monocytosis was found in all patients. Few patients required blood or platelet concentrate transfusion. The 2 patients who developed DSS both survived. All patients recovered completely without any obvious sequela. In conclusion, there was a high percentage of DHF among patients in the dengue outbreak in 2001. Increasing rates of DHF compared to previous reports from Taiwan may be a sign of hyperendemicity (multiple serotypes present) of the dengue virus in Kaohsiung City and its greater likelihood elsewhere in Taiwan. Prevention and control of both dengue fever and DHF have thus become increasingly important.
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PMID:Characteristics of a dengue hemorrhagic fever outbreak in 2001 in Kaohsiung. 1549 6

A census is currently being carried out of the French cohort of Gaucher disease patients. This article describes its preliminary results, obtained by analysing the records of 101 patients for whom clinical and laboratory data were accessible while they were receiving enzyme therapy. At the time of diagnosis, all patients presented with splenomegaly, 70% had asthenia and one in three was already affected by major bone damage. After 1 year of enzyme therapy, splenomegaly had diminished by half and the different scores (asthenia, bone pain and abdominal pain, etc.) had markedly improved, as had the biochemical markers. As for the 6 patients affected by type 3 Gaucher disease and treated with enzyme therapy after the onset of neurological signs, a stabilisation or even some improvement of the disease was observed. In-depth study of the French cohort should make it possible to formulate consensus recommendations for the future, based on well-established data.
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PMID:[Clinical study of the French cohort of Gaucher disease patients]. 1664 96

(1) In dialysis patients with chronic renal failure, hyperphosphataemia can cause osteorenal dystrophy, leading to bone pain, fractures and excess cardiovascular mortality. In addition to a low-phosphorus diet and dialysis, phosphorus chelators are usually needed to control blood phosphorus levels. The first choice is calcium carbonate, and sevelamer is an alternative. (2) Lanthanum carbonate, a phosphorus chelator, is now also licensed for the treatment of hyperphosphataemia in dialysis patients with chronic renal failure. (3) In addition to three dose-finding placebo-controlled studies, clinical evaluation includes 2 comparative randomised unblinded trials: one 6-month trial versus calcium carbonate and a 2-year trial versus other phosphorus chelators. During these trials, lanthanum was no more effective than the comparators in terms of effects on the mortality rate, incidence of fractures, or blood phosphorus level. (4) During these trials, adverse events attributed to treatment were more frequent with lanthanum than with the other phosphorus chelators. The main problems were gastrointestinal disorders (nausea, vomiting, diarrhoea, constipation and abdominal pain), headaches, seizures, and encephalopathy. (5) The accumulation of lanthanum in the bones and brain is troubling. The known long-term adverse effects of aluminium, another trivalent cation with weak gastrointestinal absorption, suggest that caution is also required with lanthanum. (6) In practice, when a phosphorus chelator is needed to treat hyperphosphataemia in dialysis patients with chronic renal failure, calcium carbonate is the first choice and sevelamer remains the best alternative.
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PMID:Lanthanum: new drug. Hyperphosphataemia in dialysis patients: more potential problems than benefits. 1745 39


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