Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An infant was vaccinated at the age of 3 days with BCG vaccine. At the age of 3 years 10 months he developed an infection by Salmonella typhimurium. The infection persisted with recurrent episodes of fever, peri-nephritic abscess, abscesses of lymph nodes, hepatomegaly, splenomegaly and paravertebral and retro-peritoneal abscesses, from which Salmonella were isolated. At the age of 6 years and 2 months later. At post-mortem examination there were widespread histiocytic nodules in many organs, from which Mycobacterium bovis BCG were cultured. One previous case has been reported from Czechoslovakia. The mother of that child was the sister of the father of the child reported here. It was not possible to investigate the possibility of abnormalities of humoral or cellular immunity in the family.
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PMID:Fatal generalized BCG histiocytosis. 32 38

An etiologic study was made of 107 cases of granulomatous hepatitis which were observed in a Department of Internal Medicine between January, 1971 and December, 1977 (excluding the hepatobiliary diseases). The most common etiology was tuberculosis (30 cases, 28 percent) followed by sarcoidosis (19 cases, 17.7 percent), Mediterranean exanthematous fever (13 cases, 12.1 percent), brucellosis (8 cases, 7.4 percent) typhoid fever (7 cases, 6.5 percent) and the idiopathic forms (8 cases, 7.4 percent). A lower rate of incidence was among Hodgkin's disease, toxoplasmosis, adenocarcinomas, leprosy, and those of unknown etiology, classified in this way because the study and follow-up of the patients could not be completed. There were, moreover, individual cases caused by mononucleosis, BCG reaction, hypogammaglobulinemia, celiac disease, and temporal arteritis. From a clinical point of view 50 percent of the patients had hepatomegaly and moderate disturbance of the liver enzymes. The most important enzymatic increases were detected in the cases caused by brucellosis; in the cases which were secondary to sarcoidosis the liver enzymes were normal. A comparison is established between the etiologic incidence of the present series and of others published in the literature. The causes and diagnostic problems of this type of lesion are discussed.
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PMID:[Granulomatous hepatitis. Etiologic study of 107 cases (author's transl)]. 45 94

A retrospective study on severe malnutrition concerning children hospitalized at the Pediatric ward of Dr. Pirngadi Hospital, Medan from January 1 to December 31, 1988 was conducted. Patients less than five years old were included in this study. The purpose of this study was to know the incidence of severe malnutrition, its symptoms and signs, the immunization status, feeding pattern and socio-economic factors. Out of the 3370 hospitalized patients, 2453 (72.78%) were children under five years old. Of these, 312 (12%) suffered from severe malnutrition. It consisted of marasmus 131 (41.9%), marasmic kwashiorkor 94 (30.1%) and kwashiorkor 87 (27.8%). The highest incidence was found in the age group of 0-2 years (58%). Clinical manifestation of marasmus were old man face (131 or 100%), muscular hypotrophy (118 or 71.9%) and decreased subcutaneous fat (116 or 71.1%) in marasmic kwashiorkor children 46 or 50% had their hair easily picked out, 45 or 46.3% showed hyperpigmentation and 48 or 52% had pretibial edema in the kwashiorkor group 29 or 63% had moon face, 52 or 60.4% showed crazy pavement dermatosis, 77 or 51.3% had hepatomegaly and 87 or 48% pretebial edema. Moon face was seen in 29 (63%), crazy Pavement Dermatosis in 52 (60.4%), hepatomegaly in 77 (51.3%), and pretebial edema in 87 (48%) of kwashiorkor cases. The accompanying diseases were mostly diarrhea (95%) and bronchopneumonia (22%). Immunization status showed that BCG comprised 50.6%, while DPT III and OPV III in 13.7% and 10.5% respectively and measles only 0.64%. More than half (59.6%) of them were breast-fed up to 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical features of severe malnutrition at the pediatric ward of Dr. Pirngadi Hospital Medan. 207 61

Eighty cases of miliary tuberculosis admitted to our hospital between January 1981 and December 1984 were reviewed. The age of the patients ranged from 3 months to 12 years, with an average of 2 years 2 months (26.5 months). Nine cases (11.25%) died during hospitalization due to the severe condition at the time of admission. Only 8 patients (10%) were in good nutritional condition. Seventy-two patients (90%) had been visiting the primary health care clinic for several times since 2-3 months but were never diagnosed as suffering from tuberculosis. Fever or recurrent fever were found in 78 cases (97.5%), anorexia in 65 cases (81.3%), chronic and/or recurrent cough in 72 cases (90%) and malaise in 43 (53.8%). Forty-one (51.3%) denied the presence of a close contact with source of infection. Hepatomegaly was found in 44 cases (55%), 19 (23.8%) of which were associated with splenomegaly. Choroidal tubercle was found in 4 cases; 1 case with coxitis, 1 with brain tuberculoma, 1 with ascites, 1 with endobronchitis and 1 with hepatitis. Forty-three (53.8%) were tuberculin negatives, 24 of which become positives after treatment. Fourteen cases had BCG scar. History of measles was found in 21 cases. Children with longterm and recurrent fever, anorexia, decrease of body weight and recurrent cough should be suspected of having TB thus enabling to get an early diagnosis.
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PMID:Miliary tuberculosis in children. A clinical review. 207 67

A 3-month-old child with a grossly symmetrical monomorphic papular acral-located skin eruption, a voluminous lymphadenopathy post-BCG vaccination, and hepatomegaly was seen. The diagnoses of Gianotti-Crosti's syndrome and generalized tuberculous infection have been discarded. The appearance of these lesions 48 hours after a tuberculin patch test, their tuberculoid structure, and the absence of any systemic involvement favored the diagnosis of papular tuberculids, a rarely reported complication of BCG vaccination.
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PMID:Papular tuberculids post-BCG vaccination. 361 Apr 34

Adjuvant chemo- and immuno-therapy with dacarbazine (1st to 5th day 250 mg/m2 daily) and BCG (6th day 0.01 ml intracutaneously)was administered to a 50-year-old male patient in a three-week cycle after surgical removal of a superficially spreading malignant melanoma. Metoclopramide was used as an antiemetic. During the second therapy cycle sudden severe vascular collapse with increasing hepatomegaly and signs of acute hepatic failure occurred leading to death after two days. At necropsy a Budd-Chiari syndrome with thombosed hepatic veins and congestive liver parenchyma necroses was found. The cause was hyperergic endophlebitis combined with severe infiltration of the vascular walls by eosinophilic granulocytes. In association with 5 more similar cases from other clinics (personal communications) this picture must be assumed to be a complication of dacarbazine treatment.
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PMID:[Budd-Chiari syndrome during treatment with dacarbazine (DTIC) (author's transl)]. 698 51

One hundred and thirteen children suffering from tubercular lymphadenitis proven histopathologically, were studied for clinical and laboratory features. Age distribution was equal in all age groups except during infancy when it was rare. Sex ratio indicates a male preponderance with male to female in the ratio of 1.5:1 (67:46). Family history of contact with known tuberculous patient was positive in 19 (17%) children, 44 (40%) had received BCG, and 98 (88%) were either normal nourished or had mild malnutrition. Cervical, axillary and inguinal nodes were found in 90 (80%), 14 (12%), and 8 (7%) children respectively. Epitrochlear node was found in only 1 child. 11 (10%) children had discharging sinus, all being in cervical group. The consistency of nodes was firm in 98 (87%), fluctuation was present in 15 (13%). In 87 (77%) lymph nodes were matted. Hepatomegaly of more than 2 cm was present in 13 (11%) and spleen was enlarged (> 2 cm) in 4 (4%) only: Mantoux test was positive in 96 (85%) children and chest X-ray was abnormal in 25 (22%) cases. Findings suggest that tubercular adenitis occurs in all age groups with equal frequency. It can occur in vaccinated children also. It may be a sole manifestation of tubercular infection. The cervical nodes are predominantly involved. There is no typical location of nodes in individual groups but multiplicity and matting of nodes are characteristic features of tubercular adenitis in children.
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PMID:Tubercular lymphadenitis: clinical manifestations. 1082 23

Bloody stools, diarrhea and perianal abscesses were observed from the age of two months infant. The boy received a BCG vaccination at the age of four months. The patient was diagnosed as having Crohn's disease at the age of six months by intestinal endoscopy. Based on the diagnosis, he was treated with nutrition therapy, salazosulfapyridine, and prednisolone. Fever of unknown origin occurred two months after he had taken azathioprine at the age of two years and two months. Mycobacterium tuberculosis was detected from a gastric aspirate, and he was diagnosed as having disseminated BCG infection by means of the multiplex PCR method. Chest CT showed miliary pulmonary nodules in both lungs on admission. Physical examination revealed enlarged lymphnodes, which were palpable around the neck and groin, and hepatomegaly. Laboratory data were within normal ranges except a slightly increased peripheral white blood cell and serum CRP level. He was treated with rifampicin (15 mg/kg/day), isoniazid (15 mg/kg/day) for 12 months, and streptomycin (25 mg/kg/day) for two months. He became afebrile a week after starting the treatment, and the miliary pulmonary nodules in both lungs had disappeared by 5 months after starting the treatment. An abnormality of the NEMO gene, which is the gene responsible for ectodermal dysplasia and immunodeficiency, was identified at the age of three years. It is assumed that an abnormality of the NEMO gene caused a latent BCG infection over a period of one year and ten months, and immunosuppressive medicine (azathioprine) induced a disseminated BCG infection. This case report supports that anti-tuberculosis medicine should be given to prevent disseminated BCG infection if an infant who receive immunosuppressive therapy is found to have an immune deficiency characterized by a mycobacterium infection after BCG vaccination.
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PMID:[A case of disseminated BCG infection found during treatment of an infant with Crohn's disease]. 1976 66

Hemophagocytic syndrome (HS) is a life-threatening condition of hyperinflammation. Main symptoms are: prolonged fever, cytopenia, hepatosplenomegaly, hemophagocytosis, hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia. Primary genetic form and secondary HS associated with infections, malignancies or autoimmune disorders can be distinguished. Untreated HS in most cases leads to death. We analyzed retrospectively 7 cases of HS in children (3 girls, 4 boys; aged 10 days -14 years) treated in 3 different pediatric centers from 2004 to 2009. In 3 cases HS was associated with infections (EBV, CMV, Bacillus Calmette Guerin - BCG), in 1 child with non-Hodgkin anaplastic large cell lymphoma (ALCL), in 1 patients probably with side effect of antiepileptic drug. In 2 cases cause of HS remained unknown. Fever, hepatomegaly, pan- or bicytopenia and hyperferritinemia were present in all children. In addition, splenomegaly was noted in 6 cases, hemophagocytosis in 6 children, impaired function or decreased number of NK cells in 4 cases, hypofibrino-genemia in 5 and hypotriglyceridemia in 4 patients. Among other symptoms and signs we observed: lymphadenopathy, hepatic failure, oedema, rash, neurological symptoms, increased level of LDH and inflammatory markers. In one child acute pancreatitis occurred. Among others, antibiotics, antiviral and immunosuppressive drugs were used in therapy. HLH-2004 protocol was applied in 4 cases. Patient with ALCL was treated with chemotherapy and allogeneic stem cell transplantation. Four patients are alive, 2 died because of HS, child with ALCL died because of generalized infection in peritrans-plantation period. In case of prolonged fever, splenomegaly and cytopenia diagnosis of HS should be considered. Following tests are recommended: complete blood count, ferritin, triglycerides, fibrinogen, bone marrow aspiration and NK cell assessment. Patients should be also screened for infections and malignancies. Early diagnosis of HS and underlying condition is crucial to start lifesaving therapy.
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PMID:[Hemophagocytic syndrome in children with different underlying conditions]. 2134 76