Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemophagocytic syndrome, splenic microabscesses and pulmonary cavitary lesions were presented in a 17-month-old boy with prolonged fever, hepatosplenomegaly and a history of tuberculous lymphadenitis. Clinical course mimicked tuberculosis. Blood cultures were negative. Ultrasound-guided, percutaneous aspiration from splenic microabscesses grew Burkholderia cepacia. He was treated successfully with trimethoprim-sulfamethoxazole. This child with chronic granulomatous disease had an unusual clinical manifestation of B. cepacia infection.
 ...
PMID:Hemophagocytic syndrome and Burkholderia cepacia splenic microabscesses in a child with chronic granulomatous disease. 1536 35

Bacillus Calmette Guerin (BCG) lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC) of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH). FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB). In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.
 ...
PMID:FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis. 1561 35

We report a case of systemic phaeohyphomycosis in a 13-year old girl. The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy. The patient was diagnosed initially as tuberculous adenitis and was on antituberculous therapy, but with worsening of symptoms. Lymph node and liver biopsy showed granuloma with fungal hyphae suggestive of phaeohyphomycosis. The patient responded to antifungal therapy. Later she developed secondary biliary cirrhosis with cholangitis due to long stricture of the common bile duct and common hepatic duct requiring biliary stenting. To the best of our knowledge, this is the first report on phaeohyphomycosis causing biliary cirrhosis in the English lilterature.
 ...
PMID:Hepatobiliary phaeohyphomycosis with secondary biliary cirrhosis. 1591 77

Chronic granulomatous disease (CGD) is a rare immunodeficiency disorder. The inability of phagocytic cells to kill catalase-positive organisms, such as Staphylococcus and Aspergillus species, causes recurrent infections, persistent inflammation, and granuloma formation. The imaging findings in nine cases of CGD were studied. Recurrent pulmonary infection was the most common abnormality (seven cases). Its complications included pulmonary abscesses, bronchiectasis, mediastinal abscesses, osteomyelitis, sepsis, and brain abscesses. Suppurative cervical adenitis was the second most common abnormality (four cases) and was also the presenting abnormality in the youngest patient (aged 31 days). Abdominal manifestations included hepatosplenomegaly, recurrent hepatic and splenic abscesses, necrotic mesenteric adenopathy, and gastric outlet obstruction. Osteomyelitis occurred in two cases secondary to hematogenous spread or spread of contiguous infection from the lung. Persistent infections led to formation of chronic inflammatory masses and granulomas in five cases. With improvements in therapy, the prognosis of CGD patients has improved and the general consensus is that most patients will survive into adulthood. Hence, radiologists are more likely to encounter the complications of CGD and should familiarize themselves with the spectrum of imaging findings.
 ...
PMID:Imaging of chronic granulomatous disease in children. 1616 Jan 5

Kikuchi-Fujimoto Disease (KFD) was first described in Japan in 1972. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. We searched literature records beginning from 1991 and analyzed epidemiological, clinical, and laboratory data of 244 patients (including cases diagnosed in our institution) reported in 181 publications. Of the 244 cases, 33% were male and 77% were female. Mean age was 25 (1-64) and 70% was younger than 30. Most of the cases were reported from Taiwan (36%), USA (6.6%), and Spain (6.3%). Fever (35%), fatigue (7%) and joint pain (7%) were the most frequent symptoms, while lymphadenomegaly (100%), erythematous rashes (10%), arthritis (5%), hepatosplenomegaly (3%), leucopenia (43%), high erythrocyte sedimentation rate (40%), and anemia (23%) being the most common findings. KFD was associated with SLE (32 cases), non-infectious inflammatory diseases (24 cases), and viral infections (17 cases). SLE was more frequent in cases from Asia than Europe (28 and 9%, respectively). The disease was self-limiting in 156 (64%) and corticosteroid treatment was necessary in 16 (16%) of the cases. The mortality rate was 2.1%. Early diagnosis is crucial as the clinical and laboratory presentation generally imitates situations needing lengthy and costly diagnostic and therapeutic interventions. Additionally, association with SLE needs further investigation.
 ...
PMID:Kikuchi-Fujimoto Disease: analysis of 244 cases. 1653 88

In this study, the clinical and laboratory features of children with lymphadenopathy were evaluated. Over a 3-year period, 126 patients were referred to the clinic for lymphadenopathy. Twenty-eight of cases have diseases mimicking lymphadenopathy; 98 (mean age: 86+/-55 months) have lymphadenopathy. Localized, limited, and generalized involvement was found in 52%, 30%, and 18% of patients. The most common localization was the head and neck region. The causes of lymphadenopathy were benign diseases in 75 patients. Sixty percent were reactive lymphadenopathy, 39% were lymphadenitis. Lymphadenitis was more frequently localized and bigger than 3 cm compared with reactive adenopathy (p=.02, p=.004). Twenty-three patients have malignant diseases whose mean age was higher than others (p=.002). The enlargement of supraclavicular nodes was more likely due to malignant disease (p=.001). The risk of malignant disease was higher in patients who had generalized lymphadenopathy, lymph nodes bigger than 3 cm, hepatosplenomegaly, and high lactate dehydrogenase levels. In conclusion, this study pointed out the important clues for the differential diagnosis, which were present in the history, physical, and laboratory findings.
 ...
PMID:Analysis of children with peripheral lymphadenopathy. 1689 60

In this study which was carried over a period of 2 years, from 2003 to 2004, 270 paediatric patients with active Tuberculosis (TB) disease attending the OPD of S.N. Medical College, Agra were screened for Human Immunodeficiency Virus (HIV)-1/2 antibodies. Of these, 23 were found to be HIV-positive. Seroprevalence of HIV infection among paediatric TB patients in Agra is 8.51% (23/270). The HIV infection was found to be significantly higher, i.e. 82.61% in male children than in female children, i.e. 17.39%. Among the age groups, which were divided into < or =1, 2-5, 6-10 and 11-15 years, maximum cases of HIV-positivity, i.e. 65.22% was observed in the age group, 2-5 years of age. Among the HIV-positive children with TB, 86.75% were of pulmonary and 13.04% were of extra-pulmonary type. Among the vaccinated children, 65.22% were found to be HIV-positive, while 34.78% of the HIV-positive children were not BCG vaccinated. HIV-positive children are more likely to suffer from prolonged fever, weight loss, failure to thrive, developmental delay, stunted growth, cough, anorexia, lethargy, lower respiratory tract infections (LRTI) and hepatosplenomegaly while HIV negative are more likely to suffer from fever, diarrhoea, lymphadenitis, pallor and LRTI. 82.60% (19/23) of these TB patients had a history of positive contact with HIV, i.e. one of the parents was HIV-infected. The mode of transmission of HIV infection among paediatric TB patients was perinatal as revealed during the counselling sessions (pre-test and post-test) of both the parents.
 ...
PMID:Seroprevalence of HIV infection among paediatric tuberculosis patients in Agra, India: a hospital-based study. 1690 56

Kikuchi-Fujimoto's disease (KFD) or histiocytic narcotising lymphadenitis is a febrile benign lymphadenopathy of unknown etiology, involving more frequently cervical lymph nodes and diagnosed on biopsy of an affected node. It is sometimes associated with auto-immune diseases such as systemic lupus erythematosus (SLE). However less frequent symptoms including involvement of extracervical nodes, hepatosplenomegaly and systemic symptoms (weight loss, night sweats) are possible and suggest an infectious disease. We report the case of a Senegalese patient with positive Ag HBs who developed SLE and KFD mimicking tuberculosis. Atypical pseudo-infectious appearance of an inflammatory systemic disease is a rare occurrence and the diagnosis can be difficult.
 ...
PMID:[Systemic lupus erythematosus and Kikuchi-Fujimoto disease mimicking tuberculosis]. 1856 9

A 58-year-old man was referred to the internal medicine outpatient department because of abdominal pain and lymphadenopathy. CT imaging revealed multiple mediastinal, para-aortic, mesenteric and pelvic lymphoma. Biopsy of an inguinal lymph node for histology purposes revealed granulomatous lymphadenitis. A watchful waiting approach was adopted. Two and a half years after first presentation, the patient presented with weight loss, fever and night sweats. CT imaging showed progressive lymphadenopathy and hepatosplenomegaly. Histopathological examination of a laparoscopically removed lymph node showed extensive infiltration with macrophages with stained rod-shaped bacteria (periodic acid-Schiff staining), which is typical of Whipple disease. PCR and biopsies of the small intestine confirmed the diagnosis. Antibiotic therapy was started with rapid clinical and biochemical improvement. A year later PCR of the duodenal biopsies was negative and the antibiotics were discontinued. Whipple disease is a rare chronic infection caused by Tropheryma whipplei. Clinical symptoms can vary, so it often takes a long time to diagnose.
 ...
PMID:[Unexplained lymphadenopathy: Whipple disease]. 1993 Jul 47

Here, we describe a patient with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) who simultaneously presented with generalized erythroderma and dermatopathic lymphadenitis (DL). A 63-year-old Korean woman presented at our hospital with fever, hepatosplenomegaly, axillary lymphadenopathy, and generalized erythrodermic eruption. The bone marrow biopsy findings were consistent with the diagnosis of HLH, and EBV DNA was detected using the polymerase chain reaction. Based on serologic tests that indicated a primary EBV infection, the patient was diagnosed with EBV-associated HLH. Histopathologic analysis of enlarged lymph nodes was consistent with DL, and EBV-encoded small nuclear RNA-positive mononuclear cells were detected. We assume that activated histiocytes, lymphocytes, and proinflammatory cytokines in HLH may have important roles in the development of generalized erythroderma and DL. Disrupted epidermal/dermal junctions owing to erythroderma may also be involved in the development of DL.
 ...
PMID:Dermatopathic lymphadenitis with generalized erythroderma in a patient with epstein-barr virus-associated hemophagocytic lymphohistiocytosis. 2001 Feb 84


<< Previous 1 2 3 4 Next >>