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

We report a case of human herpes virus-8-associated multicentric Castleman's disease in an HIV-positive patient with hyponatraemia. A 65-year-old man was admitted with relapsing and remitting fever, scattered skin eruptions and hepatosplenomegaly following combination antiretroviral therapy for his HIV infection. Based on histopathological findings, he was diagnosed as having human herpes virus-8-associated multicentric Castleman's disease and was treated with four-weekly infusions of rituximab. Prior to receiving chemotherapy, we observed several suspected biomarkers of disease activity, positive correlations between plasma human herpes virus-8 viral load and the levels of plasma interleukin-6, C-reactive protein and soluble interleukin-2 receptor, and negative correlations between platelet count, albumin levels and especially serum sodium levels. We hypothesize that non-osmotic release of plasma antidiuretic hormone is a cause of hyponatraemia in human herpes virus-8-associated multicentric Castleman's disease and that relapsing and remitting hyponatraemia could be correlated with plasma human herpes virus-8 viral load.
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PMID:Human herpes virus-8-associated multicentric Castleman's disease in an HIV-positive patient presenting with relapsing and remitting hyponatraemia. 2550 30

An overlap in the distribution of the 2 diseases (leishmaniasis and malaria) was reported in endemic areas, and it can cause significant delay in the diagnosis of leishmaniasis. Here, an 8-year-old Yemeni boy who was initially diagnosed as malaria and schistosomiasis, and later on as leishmaniasis is reported. He presented with prolonged fever, hepatosplenomegaly, and diarrhea. His blood film was positive for Plasmodium falciparum malaria, and his stool was positive for Schistosoma mansoni. Although a full therapeutic course of antimalarial and schistosoma was administered, his fever, weight loss, and increased hepatosplenomegaly continued. Bone marrow aspiration was carried out revealing Leishman-Donovan bodies (amastigote form). He was successfully treated with a full course of sodium stibogluconate. This case stresses the importance of alertness among the treating physicians to this disease occurring in a patient from an endemic area, presenting with prolonged fever, and hepatosplenomegaly.
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PMID:Leishmaniasis, malaria, and schistosomiasis concurrently in an 8-year-old boy. 2643 9

35 cases of Kala-Azar were managed at 200 bedded peripheral hospital, Bihar from Jan 1994 to Jan 1998. Patients presenting with history of fever for more than 3 weeks duration with splenomegaly or hepatosplenomegaly were investigated for Kala-Azar. A confirmative diagnosis of Kala-Azar was made in all cases by demonstrating Leishmania amastigote (LD body) in bone marrow or splenic aspirate. All patients were initially treated by sodium stibogluconate (SSG) 20 mg/kg body wt daily for 20 to 40 days depending on response. SSG induced cardiac toxicity was seen in 6 cases (VT-2, ST-T changes-2, QTc Prolongation-2) out of which 1 patient died of refractory ventricular tachyacarida. 9 patients were unresponsive to SSG,8 patients were treated with pentamidine isoethionate (4 mg/kg body wt IV alternate day) 10-15 dosage. 1 patient was treated with amphotericin-B. All the patients showed clinical and parasitological improvement and no relapse was noted at 6 month follow up. 6 patients had associated tuberculosis (Disseminated TB-2, Miliary TB-1, Pulmonary TB-1. Pleural TB-2). 2 patients had associated pneumonia, 1 patient had HIV infection and 1 patient had erythema nodosum leperosum.
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PMID:PROBLEMS IN MANAGEMENT OF KALA AZAR: EXPERIENCE FROM BIHAR. 2740 12

Adult cases of visceral leishmaniasis (VL), predominantly males, have been reported in the past decade from natives of high altitude areas of North Indian state of Uttarakhand. We report 14 pediatric cases of VL, who were diagnosed and treated successfully over the past 7 years. All these children were born and brought up in this area and had never visited any of the endemic areas. High prevalence of pallor, splenohepatomegaly, thrombocytopenia and poor association with HIV are cardinal features of VL in this region. Although newer drugs have become available, the protozoan continues to be sensitive to sodium stibogluconate. We conclude that the transmission cycle of VL has been established in this region and VL should be considered in the differential diagnosis of any child presenting with fever and hepatosplenomegaly. However, molecular and epidemiological studies are needed to identify the ancestry, vector and animal reservoir if any in this region.
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PMID:Changing Epidemiology: A New Focus of Kala-azar at High-Altitude Garhwal Region of North India. 2758 28

Hepatic hydrothorax, a rare and debilitating complication of cirrhosis, carries high morbidity and mortality. First-line treatment consists of dietary sodium restriction and diuretic therapy. Some patients, mainly those who are refractory to medical management, will require invasive pleural drainage. The authors report the case of a 76-year-old man in a late cirrhotic stage of alcoholic chronic liver disease, presenting with recurrent right-sided hepatic hydrothorax, portal hypertension, hepatosplenomegaly and thrombocytopaenia. After recurrent admissions and complications, the potential for adjusting diuretic therapy was limited. After unsuccessful talc pleurodesis, an indwelling tunnelled pleural catheter was placed with effective symptomatic control. One month later, the patient was readmitted with empyema due to Acinetobacter radioresistens Despite optimised medical and surgical treatment, the patient died 4 weeks later.
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PMID:Hepatic hydrothorax: indwelling catheter-related Acinetobacter radioresistens infection. 3087 55

Drug induced hypersensitivity syndrome (DIHS) is often manifested as severe systemic drug trans-reactions characterized by acute and extensive skin lesions (mostly measles-like rash), fever, enlargement of lymph nodes, multiple organ involvement (hepatitis, nephritis, and pneumonia), eosinophilia and mononucleosis,within 2-6 weeks of the application of sensitizing drugs. In the early stage of the lesion, macular papules or erythema multiforme were common, and in severe cases, exfoliative dermatitis, Stevens-Johnson syndrome and toxic epidermal necrolysis were also common. Most of them developed after taking allergic drugs for 2-6 weeks (average: 3 weeks). Symptoms persisted after discontinuation of allergic drugs. It takes more than one month to alleviate, which may endanger life in severe cases. Documents report that the most common drugs causing DIHS are phenytoin sodium, carbamazepine and phenobarbital aromatic drugs. However, it was reported that phenobarbital sodium was the most common anticonvulsant among allergenic drugs in children, followed by antipyretics, analgesics and antibiotics, which may be related to the spectrum of childhood diseases and the particularity of the drug. Lamotrigine has been reported to cause DIHS in adults in China, but less in children. In order to improve the understanding of clinical diagnosis and treatment of DIHS in children, reduce misdiagnosis, missed diagnosis, and untimely treatment, and prevent the aggravation of the disease, we studied the case of a 4-year-old 7-month-old girl who presented with systemic erythematous papules, fever, hepatosplenomegaly, marked increase of white blood cells, marked decrease of anemia and platelets, abnormal liver function and coagulation routine after taking lamotrigine for one month due to epilepsy seizures. Now, according to the DIHS diagnostic criteria established by Registration of Severe Cutaneous Adverse Reactions Drug Review Group in 2007, plasma exchange was immediately given to replace the toxic metabolites in hemorrhagic plasma, and methylprednisolone was given intravenously for three days. At the same time, after symptomatic supportive treatments, such as loratadine and albumin, the condition gradually improved without recurrence. Through a case report of Drug reaction with eosinophilia and systemic symptoms in a child caused by lamotrigine, we can strengthen our understanding and improve the level of diagnosis and treatment of drug hypersensitivity syndrome in children. Lamotrigine can cause DIHS in children, which is very dangerous. Early diagnosis and early withdrawal of allergenic drugs, plasma exchange and glucocorticoid therapy are the key to treatment.
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PMID:[Lamotrigine induced hypersensitivity syndrome in children: a case report]. 3099 82

y+LAT1 (encoded by SLC7A7), together with y+LAT2 (encoded by SLC7A6), is the alternative light subunits composing the heterodimeric transport system y+L for cationic and neutral amino acids. SLC7A7 mutations cause lysinuric protein intolerance (LPI), an inherited multisystem disease characterized by low plasma levels of arginine and lysine, protein-rich food intolerance, failure to thrive, hepatosplenomegaly, osteoporosis, lung involvement, kidney failure, haematologic and immunological disorders. The reason for the heterogeneity of LPI symptoms is thus far only poorly understood. Here, we aimed to quantitatively compare the expression of SLC7A7 and SLC7A6 among different human cell types and evaluate y+LAT1 and y+LAT2 contribution to arginine transport. We demonstrate that system y+L-mediated arginine transport is mainly accounted for by y+LAT1 in monocyte-derived macrophages (MDM) and y+LAT2 in fibroblasts. The kinetic analysis of arginine transport indicates that y+LAT1 and y+LAT2 share a comparable affinity for the substrate. Differences have been highlighted in the expression of SLC7A6 and SLC7A7 mRNA among different cell models: while SLC7A6 is almost equally expressed, SLC7A7 is particularly abundant in MDM, intestinal Caco-2 cells and human renal proximal tubular epithelial cells (HRPTEpC). The characterization of arginine uptake demonstrates that system y+L is operative in renal cells and in Caco-2 where, at the basolateral side, it mediates arginine efflux in exchange with leucine plus sodium. These findings explain the defective absorption/reabsorption of arginine in LPI. Moreover, y+LAT1 is the prevailing transporter in MDM sustaining a pivotal role in the pathogenesis of immunological complications associated with the disease.
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PMID:y+LAT1 and y+LAT2 contribution to arginine uptake in different human cell models: Implications in the pathophysiology of Lysinuric Protein Intolerance. 3170 28


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