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| | Turkish Journal of Haematology |
 | | The patients had hepatosplenomegaly and bone marrow examinations revealed Iymphohistiocytosis. | | | The pathognomonic granules of Chediak-Higashi syndrome were not observed in peripheral blood smear. |
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http://www.tjh.com.tr/text.php3?id=160
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| | Tropical Medicine Central Resource |
| | Congenital toxoplasmosis showing interstitial pneumonitis in the medial aspects of the lung bases, as well as hepatosplenomegaly in an infant with generalized T. | | | There are no reports of calcifications in the spinal cord or canal in toxoplasmosis acquired congenitally. | | | Infants with severe, generalized congenital toxoplasmosis frequently show evidence of interstitial pneumonia and hepatosplenomegaly ( Fig. |
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http://tmcr.usuhs.mil/tmcr/chapter45/imaging2.htm
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| | Fact Sheet - Neiman Pick Types A and B |
| | On autopsy, he noted that the affected child had evidence of lipid storage in the reticuloendothelial system and that it was similar to that seen in Gaucher disease. | | | The neurologic exam showed a peripheral neuropathy characterized by decreased deep tendon reflexes but no sensory abnormalities. | | | There are very little or no neurologic symptoms and some patients remain entirely asymptomatic until adulthood. |
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http://www.lsdn.com/glance_fact_niemann.htm
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| | Human schistosomiasis - immunology in a foreign field.& |
| | Thus, it is possible that misinterpretation of the relationship between murine immune responses and human disease has given Th2-type responses an unjustified 'bad press' in regard to schistosomiasis. | | | And how much of what we are learning from the mouse is applicable to human schistosomiasis? | | | This suggests that Th2-type responses, while damaging to a mouse suffering an overwhelming level of infection, may have an important regulatory role in man, suppressing the fibrogenic potential of chronic hepatic inflammation. |
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http://www.path.cam.ac.uk/~schisto/Articles/General.html
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| | neurologic Epstein-Barr and co-infections |
| | RESULTS: Despite repeated trials of the antiviral agents prednisolone and gamma-globulin, his condition deteriorated. | | | Infection with cytomegalovirus (CMV) in infants can be congenital or perinatal. | | | Organomegaly was found in 3 out of 6 CMV-positive patients (1 hepatomegaly, 1 splenomegaly, 1 hepatosplenomegaly), and 4 out of 5 EBV-positive patients (lymphadenopathy in all 4 cases, hepatosplenomegaly in 2 cases). |
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http://members.jorsm.com/~binstock/ebv-case.htm
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| | eMedicine - Niemann-Pick Disease : Article by Margaret McGovern, MD, PhD |
| | With advancing age, the loss of motor function and deterioration of intellectual capabilities are progressively debilitating, and in later stages, spasticity and rigidity are evident, and affected infants are completely unable to interact with their environment. | | | In mildly affected patients, splenomegaly may not be noted until adulthood, and disease manifestations may be minimal. | | | In contrast, patients with type B disease survive to adulthood, although most present in childhood with hepatosplenomegaly. |
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http://www.emedicine.com/ped/topic2889.htm
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| | Turkish Journal of Haematology |
| | In this report we present the clinical and laboratory findings and the outcome of two children with IAHS to emphasize the importance of early diagnosis and the effectiveness of intravenous immunoglobulin therapy in these patients. | | | IVIG therapy 400 mg/kg for five days was added to therapy; after the second dose of IVIG therapy, fever, hepatosplenomegaly, and DIC features disappeared and hematologic parameters was in normal limits. | | | Hemophagocytic syndrome is a rare disorder characterized by a group of clinical, laboratory and histopathological findings such as fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, lymphohistiocytosis and hemophagocytosis in the bone marrow, spleen, and lymph nodes[ 1 ]. |
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http://www.tjh.com.tr/text.php3?id=260
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| | An 8 |
| | Which of the following conditions is most likely the cause of the patient's peripheral blood smear findings? | | | This patient's jaundice is most likely due to which of the following? | | | None of the information in this patient's history and clinical examination except the jaundice and hepatosplenomegaly suggests that she has hepatitis (choices B, C, and D) ; more specifically, the marked anemia and marrow expansion of the cranium would not be seen in hepatitis. |
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http://iuhs-isa.org/Jaundice.htm
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| | Lysosomal Storage Diseases MEDSTUDENTS-METABOLIC DISORDERS |
| | The infant form is characterized by early onset, marked hepatosplenomegaly and severe neurologic manifestations that result in early death. | | | This condition is due to deficiency of glucosylceramidase, leading to a glucosylceramide lipidosis. | | | Diagnosis may be given by finding distinctive Niemann-Pick cells in bone marrow, but is necessary to confirm it with specific enzime assay. |
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http://www.medstudents.com.br/metdis/metdis6.htm
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| | Kim's Oriental Medicine Clinic |
| | On July 20, 1988 my medical examination showed virtually no bone pain, no hepatosplenomegaly and no peripheral edema, lungs clear, and blood pressure 170/90 with minimal sternal tenderness. | | | The acupuncture and moxibustion treatments which I was given by Dr. Kim progressively and significantly benefited my health condition. | | | On May 18, 1988, my medical examination showed the sternal pain almost completely gone with blood pressure 160/80. |
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http://www.acupuncturekim.com/new/testimonial.asp?type=0&name=Melanoma&id=25
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| | Unexplained Immunodeficiency and Opportunistic Infections in Infants -- New York, New Jersey, California |
| | Of the nine mothers for whom information is available, seven are reported to be intravenous drug abusers. | | | She responded to antifungal therapy, but at 5 months, candidiasis recurred, and she had hepatosplenomegaly. | | | Clinical features seen in these 12 infants include: failure to thrive (83%), oral candidiasis (50%), hepatosplenomegaly (92%), generalized lymphadenopathy (92%), and chronic pneumonitis without a demonstrable infection (83%). |
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http://www.cdc.gov/mmwr/preview/mmwrhtml/00001208.htm
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| | Indian Pediatrics - Images in Clinical Practice |
| | The baby had a classical presentation with laetescent plasma, hepatosplenomegaly (inges-tion of chylomicrons by reticuloendothelial system) and lipemia retinalis. | | | The syndrome is produced by the deficiency of either lipoprotein lipase or Apoprotein C-II. | | | Diagnosis is by demonstrating elevated levels of triglycerides (>1000 mg/dl) and cholesterol. |
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http://www.indianpediatrics.net/aug2001/aug-925.htm
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| | CHEST: Simultaneous Presentation Of Pulmonary Sarcoidosis And Cytomegalovirus Pneumonitis In An Immunocompetent Patient ... |
| | She did not smoke, drink nor use drugs. | | | Introduction: CMV pneumonitis can be a severe life threatening disease in certain immunosuppressed patients, most notably bone marrow and solid organ transplant patients and to lesser degree, AIDS patients. | | | Here we present a patient who likely had an indolent, smoldering progression of undiagnosed and untreated pulmonary sarcoidosis for years who suffered a subacute decline in pulmonary function accompanied by fevers and hepatosplenomegaly and was found to have histologic evidence of both sarcoidosis and CMV pneumonitis as an etiology. |
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http://www.findarticles.com/p/articles/mi_m0984/is_4_116/ai_57563286
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| | Diagnostic Dilemma - Pediatric Oncall |
| | Disclaimer:The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. | | | A 2 year old child with fever, hepatosplenomegaly and generalised lymphadenopathy | | | A 2½ years old girl with hepatosplenomegaly, anemia and generalized lymphadenopathy |
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http://www.pediatriconcall.com/fordoctor/diagnosisdilemma/listdilemma.asp
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| | Infections in Oncology:Toxoplasmic Lymphadenopathy Clinically Presenting as Lymphoma |
| | In addition, retroperitoneal or mesenteric lymphadenopathy may produce abdominal pain. | | | Physical examination was unremarkable with no fever, lymphadenopathy, or hepatosplenomegaly. | | | Some of the symptoms are malaise, night sweats, myalgias, sore throat, maculopapular rash, hepatosplenomegaly, and an atypical lymphocytosis (<10%). |
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http://www.moffitt.usf.edu/pubs/ccj/v4n5/department4.html
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| | Hepatosplenomegaly |
| | To find more information on specific conditions, please visit our partner sites: | | | Liver and spleen enlargement (hepatosplenomegaly) can occur as the result of an inherited disorder in which the liver cannot process glucocerebroside. | | | The buildup of this substance in body tissues can cause severe damage to the central nervous system in infants. |
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http://www.healthcentral.com/ency/408/ImagePages/17215.html
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| | Jornal de Pediatria |
| | Dyslipidemia, observed in seven patients, was characterized by an increase in triglyceride levels and a decrease in HDL. | | | The morphological and functional study of the skeletal musculature of patients with this disease suggests that increased muscle mass results from hyperplasia instead of hypertrophy, showing no association with increased strength ( 8). | | | Hepatosplenomegaly, often accompanied by abnormal liver function tests, may cause triglyceride and glycogen levels to increase in hepatocytes, being associated with cirrhosis ( 3,4). |
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http://www.jped.com.br/conteudo/04-80-04-333/ing.asp?cod=1209
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| | Gaucher Disease: A Diagnosis, Evaluation and Treatment Summary |
| | HEPATOSPLENOMEGALY - painless splenomegaly is often coupled with hepatomegaly, which can lead to liver dysfunction. | | | Three different patient profiles are associated with distinct disease classifications. | | | Gaucher cells lead to the displacement of healthy, normal cells in bone marrow, hepatosplenomegaly, organ dysfunction, and skeletal deterioration. |
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http://gaucher.mgh.harvard.edu/diag.html
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| | THE MERCK MANUAL, Sec. 2, Ch. 16, Hypolipidemia And The Lipidoses |
| | Type A patients have less than 5% of normal sphingomyelinase activity. | | | Type B is more variable phenotypically than type A. Xanthomas, skin pigmentation changes, hepatosplenomegaly, and lymphadenopathy may occur. | | | A familial autosomal recessive disorder of lipid metabolism resulting in an accumulation of abnormal glucocerebrosides in reticuloendothelial cells and manifested clinically by hepatosplenomegaly, skin pigmentation changes, skeletal lesions, and pingueculae. |
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http://www.merck.com/mrkshared/mmanual/section2/chapter16/16b.jsp
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| | Final Diagnosis -- Case 39 |
| | However, other Type I patients (and this case in particular) may have no symptoms and can be identified only by screening or during evaluation for other diseases(e.g. | | | Hepatosplenomegaly (Inconspicuous or absent), Adenopathy (Minimal), Ascites (Rare), Hemmorhage from nose/gums (Common), Petechiae/Puperic spots (Occasional), Skin Pigmentation (45-75% of patients), Pingueculae, Bone Destruction (75% esp. the femur), Onset is early in life with mild/minimal symptoms/signs, Anemia, thromboctopenia, and/or bone lesions often lead to diagnosis | | | For example, the Adult form (Type I), which is the most common type of Gaucher's disease, often presents with a combination of anemia, thrombocytopenia, hepatosplenomegaly, and extensive skeletal disease. |
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http://path.upmc.edu/cases/case39/dx.html
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| | www.whonamedit.com |
| | A rare disease of unknown cause in which lipids accumulate in the body and manifest as histiocytic granuloma in bone, particularly in the skull; the skin; and viscera, often with hepatosplenomegaly and lymphadenopathy. |
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http://www.whonamedit.com/syndlist.cfm/48
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| | Hemolytic Uremic Syndrome MEDSTUDENTS PEDIATRICS |
| | In 10% of cases rectal prolapse may occur, and 2% of patients may need surgery because of gangrene. | | | Other signs and symptoms may include jaundice, convulsions, dehydration, edema, petechiae, hepatosplenomegaly. | | | This is followed in a week (it may vary from 1 to 14 days) by the sudden onset of irritability, pallor, weakness, lethargy and oliguria or anuria. |
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http://www.medstudents.com.br/pedia/pedia5.htm
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| | Gaucher Disease - Diagnosis |
| | Gaucher disease can also be associated with heart and lung problems (interstitial lung disease, pulmonary hypertension). | | | Rarely, Gaucher disease can present in the newborn with skin changes, in addition to hepatosplenomegaly and eventual neurologic complications. | | | The diagnosis should be suspected in patients of all ages who present with the following signs and symptoms, in the absence of an alternative explanation: enlarged liver and spleen (hepatosplenomegaly), low blood counts (anemia and thrombocytopenia), and bone problems (osteonecrosis, fracture, pain). |
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http://www.childrensgaucher.org/about/diagnosing.htm
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| | Familial Lipoprotein Lipase Deficiency |
| | Affected individuals have childhood-onset episodic abdominal pain, recurrent acute pancreatitis, eruptive cutaneous xanthomata, and hepatosplenomegaly. | | | Familial lipoprotein lipase deficiency is suspected in individuals with childhood-onset marked hypertriglyceridemia with episodic abdominal pain, recurrent acute pancreatitis, eruptive cutaneous xanthomata, hepatosplenomegaly, and chylomicronemia. | | | Familial lipoprotein lipase deficiency usually presents in childhood with episodes of abdominal pain, recurrent acute pancreatitis, eruptive cutaneous xanthomata, and hepatosplenomegaly. |
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http://www.geneclinics.org/profiles/lpl/details.html
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| | ACR Learning File Web |
| | The hepatosplenomegaly of Hurler does not occur in either Morquio's syndrome or achondroplasia. | | | Progressive mental and physical deterioration is the rule, and death results at a younger age than in other mucopolysaccharidoses. | | | Dwarfism is common in Hurler syndrome and may be mistaken for achondroplasia, but shortened extremities are not present at birth. |
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http://www.learningfile.com/learning_file/viewcase.php?section=nu&case_num=903
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| | Gaucher Disease |
| | Type 1 is characterized by the presence of clinical or radiographic evidence of bone disease (osteopenia, focal lytic or sclerotic lesions, osteonecrosis), hepatosplenomegaly, anemia and thrombocytopenia, lung disease, and the absence of primary central nervous system disease. | | | The perinatal lethal form is associated with hepatosplenomegaly, pancytopenia, and microscopic skin changes (i.e., abnormalities in the stratum corneum attributed to altered GL1-to-ceramide ratio), and may present clinically with ichthyosiform or collodion skin abnormalities or as non-immune hydrops fetalis [ | | | Oculomotor involvement may be found as an isolated sign of neurologic disease in individuals with a chronic progressive course and severe systemic involvement (i.e., massive hepatosplenomegaly). |
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http://www.geneclinics.org/profiles/gaucher/details.html
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| | Zinc deficiency and development |
| | Urinary excretion of zinc in patients with the syndrome of anemia, hepatosplenomegaly, dwarfism, and hypogonadism. | | | Changes in height, triceps skin fold, and arm circumference were apparently similar in the three groups that received nutrients. | | | Prasad A, Miale Jr A, Farid Z, Sandstead H, Schulert A. Zinc metabolism in patients with syndrome of iron deficiency anemia, hepatosplenomegaly, dwarfism and hypogonadism. |
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http://www.zinc-health.org/Health/Art-02.cfm
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| | WOLMAN'S DISEASE: THE KING FAISAL SPECIALIST HOSPITAL |
| | She had continuous vomiting and greasy stools from birth, with gradual abdominal distention, severe anemia, thrombocytopenia, shortness of breath and repeated chest infections, for which she was worked up for cystic fibrosis and malabsorption syndromes. | | | All patients were referred for abdominal distention, tachypnea and chronic lung disease due to distended abdomen, and severe failure to thrive. | | | Results : All patients were failing to thrive with progressive hepatosplenomegaly. |
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http://www.kfshrc.edu.sa/annals/182/97-264.html
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| | OMIM Entry 257200 |
| | Type E patients are adults with moderate hepatosplenomegaly and some increase in sphingomyelin in the liver and spleen as well as from cell in the marrow. | | | Hepatosplenomegaly, retarded physical and mental growth and severe neurologic disturbances are features. | | | A 'new' variety of Niemann-Pick disease was observed in 9 children in 5 families by |
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http://www.hgmp.mrc.ac.uk/cgi-bin/wrapomim?257200
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| | Dr. Koop - Cytomegalovirus Infection- Health Encyclopedia and Reference |
| | This syndrome is similar to infectious mononucleosis (caused by Epstein-Barr virus) and is characterized by fever, malaise, and muscle and joint pain but without sore throat. | | | Cytomegalic inclusion disease of the neonatal (newborn) period is characterized by variable severity ranging from an asymptomatic infection to a disease with fever, hepatosplenomegaly (enlarged liver and spleen), mental or motor retardation, and sometimes death. | | | Cytomegalovirus (CMV) is a type of herpes virus that often causes asymptomatic infection in otherwise healthy persons. |
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http://www.drkoop.com/encyclopedia/93/552.html
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| | Multiple Sulfatase Deficiency |
| | Abnormalities of the skeleton may occur, such as curvature of the spine (lumbar kyphosis) and the breast bone. | | | Multiple sulfatase deficiency is a very rare hereditary metabolic disorder in which all of the known sulfatase enzymes (thought to be seven in number) are deficient or inoperative. | | | Major symptoms include mildly coarsened facial features, deafness, and an enlarged liver and spleen (hepatosplenomegaly). |
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http://my.webmd.com/hw/raising_a_family/nord552.asp
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| | Hunter's syndrome (Charles A. Hunter) (www.whonamedit.com) |
| | Rare hereditary disorder characterised by dwarfism, a coarse facies, hepatosplenomegaly, digital contractures and mental retardation and deafness. | | | Speech and mental development are delayed, the child has frequent respiratory infections, and as the disease progresses a typical constellation of physical signs becomes evident: protuberant abdomen, claw hands, excessive hair growth, coarsening of the face with grotesque facial features; retarded growth, and behaviour problems. |
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http://www.whonamedit.com/synd.cfm/1109.html
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| | Anemia Due to Hemolysis |
| | Fetal anemia from hemolysis in utero is also associated not only with hepatosplenomegaly but also with myocardial dysfunction, resulting in fluid accumulation, anasarca or generalized edema, and low serum albumin. | | | When anemia becomes severe, cardiorespiratory distress may be evident. | | | Hepatosplenomegaly results because of extramedullary hematopoiesis triggered by rapid destruction of RBCs. |
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http://www.heisz.com/Harald/Technik/NDM/html/p180.html
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| | Intermittent Fever and Pancytopenia in a Young Mexican Man |
| | Waxing and waning fever (undulant fever) is also commonly seen.5 Many patients have gastrointestinal complaints that can be suggestive of ileitis or colitis.6,7 Pancytopenia is seen in a significant percentage of patients. | | | The patient continued to have intermittent fevers ( Figure). | | | The disease is characterized by generalized somatic complaints, which can include fever, sweats, anorexia, fatigue, weight loss, and depression. |
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http://www.sma.org/smj1997/octsmj97/13text.htm
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| | Hemophagocytic Lymphohistiocytosis (HLH) |
| | Neurological symptoms may complicate and sometimes dominate the clinical course. | | | In its most typical form, it is clinically characterized by prolonged fever, hepatosplenomegaly and cytopenia (most commonly thrombocytopenia and anemia). | | | If the diagnosis is not made in time, the child may die or develop permanent disabilities. |
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http://www.histio.org/society/HLH/henter1.shtml
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| | GAUCHERS DISEASE // Medical-Conditions.org // Medical Conditions |
| | The most common subtype is the non-neuronopathic form, a slowly progressive condition characterized by hepatosplenomegaly and skeletal deformities. | | | The infantile form presents at 4-5 months of age with anemia, loss of cognitive gains, neck retraction, dysphagia, and hepatosplenomegaly. | | | An autosomal recessive disorder caused by deficiency of the enzyme glucocerebrosidase (see GLUCOSYLCERAMIDASE) featuring the pathological storage of glycosylceramide in mononuclear PHAGOCYTES (Gaucher Cells). |
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http://www.medical-conditions.org/?q=Gauchers+Disease
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| | Haema/Áßìá |
| | Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, rapidly fatal, autosomal recessive disorder of the mononuclear phagocytic system, characterized by a benign, generalized histiocytic proliferation, with marked hemophagocytosis | | | Principle clinical features are fever, hepatosplenomegaly and cytopenias accompanied by hypertriglyceridemia and hypofibrinogenemia. | | | An infant who was admitted with fever, dyspnea, marked pancytopenia and hepatosplenomegaly, was diagnosed as familial hemophagocytic lymphohistiocytosis (FHL) is presented. |
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http://www.mednet.gr/eae/haema/h32-6.htm
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| | Glossary |
| | It has been suggested that the syndrome may result from an imbalance between the GABA-ergic and catecholaminergic pathways that control motor unit activity; the imbalance may be caused by autoantibodies against glutamic acid decarboxylase. | | | It differs from classical rheumatoid arthritis in that it is more often marked by acute febrile onset, and generalized lymphadenopathy and hepatosplenomegaly are more prominent. | | | A classification of T-lymphocytes, especially into helper/inducer, suppressor/effector, and cytotoxic subsets, based on structurally or functionally different populations of cells. |
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http://nationaljewish.org/fp10.html
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| | Health Care Professionals - Diagnosis |
| | There are some early clinical signs and symptoms, which alone are not diagnostic, but may warrant more definitive testing. | | | Analysis of urinary glycosaminoglycans (heparan sulfate and dermatan sulfate) was the first method available for diagnosis of MPS I and may still be used as a preliminary investigative test. | | | Although these findings at presentation will vary with the severity of the disorder, MPS I should be suspected in individuals with coarse facial features, hepatosplenomegaly, and characteristic skeletal, joint, or ocular findings. |
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http://www.aldurazyme.com/hc/mps/az_us_hc_diagnosis.asp
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| | Lab bibliogrpahy 1995 - 2001/title> |
| | Exposure to malaria affects the regression of hepatosplenomegaly after treatment for Schistosoma mansoni infection in Kenyan children. | | | Regression of hepatosplenomegaly in Kenyan school-aged children after praziquantel treatment and three years of greatly reduced exposure to Schistosoma mansoni. | | | Mwatha, J.K., Jones, F.M., Mohamed, G., Naus, C.W., Kenty, L., Riley, E., Kimani, G., Ouma, J.K. and Dunne, D.W. Associations between anti- Schistosoma mansoni and - Plasmodium falciparum antibody responses and hepatosplenomegaly in Kenyan schoolchildren. |
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http://www.path.cam.ac.uk/~schisto/lab_papers2001.html
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| | September Surveillance Bulletin |
| | Pta:- HBsAg and anti-HBc were demonstrated in the blood specimens of two patients, aged 61 and 20 years respectively, with ascites. | | | Test for hepatitis A and B were negative. | | | A further seven infants under three months of age were found to be CMV IgM positive. |
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http://www.niv.ac.za/survbul/current/sep2.htm
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