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)

A Mexican-American boy presented at birth with an extensive eruption consisting of 0.5 to 1.0 cm hyperpigmented macules with a distinct peripheral scale involving primarily the forearms, abdomen and lower back (Fig. 1). Rare intact vesicopustules were also identified. There was an unremarkable prenatal history, and the infant was a product of a normal vaginal delivery. With the exception of the skin lesions and moderate hepatosplenomegaly, the physical examination was normal. Gram stains of the pustules showed numerous neutrophils but no bacteria. Bacterial cultures, of the skin and blood, TORCH screen (toxoplasmosis, rubella, cytomegalic virus, and herpes virus) and a VDRL were negative. On the second day of life, the patient developed several pustules with surrounding erythema consistent with erythema toxicum neonatorum. Wright-stained smears of these lesions showed abundant eosinophils. Hepatosplenomegaly resolved by the third day of life and at the time of discharge only hyperpigmented macules persisted. Follow-up visit six weeks later showed no evidence of skin lesions.
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PMID:Transient neonatal pustular melanosis. 51 27

The paper reports on a case of "late onset disease" (congenital German measles). Diagnostically significant for this disease in an infant appearing perfectly healthy when new-born, were mainly the findings of an interstitial pneumonia, hepatosplenomegaly, as well as findings indicating the presence of a meningoencephalitis and retinopathy. The diagnosis of this rare course of congenital German measles was finally established via the identification of specific IgM rubeola antibodies, of a raised hemagglutination inhibition titer, and of the German measles virus itself. An attempt to treat the interstitial pneumonia with cortisone preparations remained unsuccessful. The clinical and diagnostic problem complexes are discussed briefly.
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PMID:["Late onset disease" (congenital german measles) (author's transl)]. 56 31

Although rubella virus and cytomegalovirus (CMV) are important causes of congenital infections, information on their prevalence in our country is scarce. We studied a total of 249 infants suspected of having congenital infections from January 1988 to September 1989. Serum samples of these infants were tested for rubella and cytomegalovirus specific IgM antibodies by mucapture ELISA. Thirty (12%) infants were positive for rubella IgM antibody, and 50 (20%) had CMV specific IgM antibody. In the group presenting with hepatosplenomegaly (n = 56) rubella and CMV specific IgM antibodies were detected in 1 (1.7%) and 25 (44.6%) infants respectively. In the group presenting with congenital malformations (n = 90), 23 (25.5%) were positive for rubella, and only 9 (10%) had CMV IgM antibodies. Of the infants presenting with mental retardation (n = 39), only CMV infection was detected in 3 (7.7%) infants, whereas amongst the group showing intrauterine growth retardation (n = 16), 5 (31.25%) had CMV specific IgM antibodies and 2 (12.5%) had rubella specific IgM antibodies. In the miscellaneous group (n = 48), 4 (8.3%) and 8 (16.6%) infants had rubella and CMV IgM antibodies respectively. CMV infection was prevalent in a significantly higher number of children with hepatosplenomegaly than rubella while in infants with congenital malformations a significantly higher number had rubella infection. It is concluded that rubella and CMV infections are commonly seen in children with intrauterine infections in our population.
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PMID:Prevalence of rubella virus and cytomegalovirus infections in suspected cases of congenital infections. 165 69

We have reviewed sixty three cases of patients with hepatosplenomegaly admitted at the internal medicine service at the Hospital Infantil del Estado de Sonora over a period of the ten years. We didn't found differences in the number of male and female children. The larger groups were from 1 month to 6 years old age. The nutrition status were normal in more of 80% of the patients. The time that children spent at the hospital for diagnosis was less than 15 days in more than half of the cases. The symptoms predominating and clinical features were paleness fever and adenomegalies. Basic laboratory and other studies were made in a initial period, phase I (CBS, urinalysis serum, urea serum creatinine, serum glutamic oxalacetic and piruvic transaminases, and coccidioidine skin test, monotest, ASL, cytomegalovirus and rubella antibodies, Sabin-Feldman test, VDRL and chest, abdominal and paranasal sinus x-Ray) we reached a definitive diagnosis with this laboratory methods in 41.2% of the cases. A percutaneous hepatic biopsy was a rewarding accurate diagnostic procedure in 19 of 21 patients. The diagnosis that we established in general groups were: infections diseases, oncologic disease, metabolic disturbances, primary unknown hepatic disease, hematologic disorders and congestive diseases. In this letter we want to show our experience in a second level pediatric hospital in a mexican province so that this work can be reviewed by other Physicians and they can offer a better attention for this patients.
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PMID:[Hepatosplenomegaly of unknown origin. A study of 63 cases]. 179 Aug 37

Eight cases of acute human herpesvirus type 6 (HHV6) infection in infants were diagnosed serologically by the demonstration of IgM anti-HHV6 (8/8) and a significant change in total anti-HHV6 antibody titre (6/8). Four infants were sufficiently ill to require admission to hospital and further investigations: one with encephalitis and three with gross hepatosplenomegaly, two of whom had evidence of simultaneous infection with another herpes-virus. The remaining four infants had an illness compatible with roseola infantum, although this diagnosis had not been made clinically. Sera from two of those infants with rash had been sent for analysis to exclude rubella because the infants' mothers were pregnant. The other two had received antibiotics when febrile, and the subsequent appearance of the roseola rash had raised the possibility of antibiotic allergy. The data suggest that there are clinical syndromes in addition to roseola infantum associated with the presence of IgM anti-HHV6, in which serological screening for evidence of acute HHV6 infection may be useful.
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PMID:Roseola infantum and other syndromes associated with acute HHV6 infection. 217 78

We conducted a retrospective survey of children who were born with congenital rubella syndrome (CR) resulting from a recent rubella epidemic. Sources of information were hospital and laboratory records and data collected in an active search for deaf children born following the epidemic and attending rehabilitation centers for the deaf (Micha). Criteria for inclusion in the survey were: 1) major clinical defects, and 2) one or more of the following positive laboratory findings--virus isolation, presence of rubella-specific IgM antibodies, or the presence of hemagglutination inhibition (HI) antibodies in children beyond the age of 1 year. Excluded from the study were 28 children with persistent HI antibodies, but without clinically detected defects. CR was identified in 45, among them 43 with deafness. Other major defects were psychomotor retardation, microcephaly, cataracts and heart defects. Transient abnormalities included encephalitis, hepatosplenomegaly, jaundice, thrombocytopenia, intrauterine grown retardation and failure to thrive. Thirty-one mothers (70%) reported a history of clinical rubella in pregnancy, the others having experienced subclinical infection. Multiple defects were found in children born following early gestational rubella (less than 2 months); abnormalities also occurred as a consequence of rubella as late as the fifth month of gestation.
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PMID:Congenital rubella in Israel following the 1978-79 rubella epidemic. 666 76

Adult Still's Disease (SD) has evolved into a well-characterized nosologic entity. This categorization allows physicians to place a unifying label on the rare, puzzling patient who presents with a systemic illness characterized by high spiking fever of unknown cause associated with intense arthralgias or arthritis, an evanescent erythematous macular or maculopapular rash, and other less constant features of systemic illness including lymphadenopathy, hepatosplenomegaly, sore throat, leukocytosis, anemia and elevated concentration of hepatic enzymes. The diagnosis of Adult SD is syndromic, based solely on compatible clinical findings; serologic or other diagnostic tests do not aid in diagnosis. The diagnostic problem presented by these patients with such severe systemic illness and the insecurities inherent in diagnosis based solely on clinical features make the availability of the diagnosis, Adult SD, useful in patient care. The cause of Adult SD is unknown. Some have speculated that the disease has features of non-necrotizing immune complex vasculitis. Rubella infection has been reported to be associated with Adult SD, but no clear-cut etiologic relationship has been established. Neither rubella infection nor any other potential antigen has been identified consistently in association with the disease. Management of patients with the disease depends on the correct diagnosis. Diagnosis should include recognition of the syndrome as well as exclude other possible diseases. Control of systemic manifestations may require unusually high doses of aspirin, indomethacin or other non-steroidal anti-inflammatory drugs, prednisone or combinations of these drugs. Some adults appear to require both high-dose prednisone and indomethacin to control disease manifestations. Fortunately, systemic attacks are usually episodic; steroid toxicity can be minimized by use of alternate day doses and attempts to discontinue steroids between episodes. The current series and other reports of long-term follow-up indicate that Adult SD may be more disabling than was originally reported. At least three patterns of recurrences occur: 1) systemic attacks with or without arthritis, 2) pauciarticular disease, and 3) disabling deforming chronic arthritis, which may require surgery and long-term anti-inflammatory, gold, or cytotoxic therapy.
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PMID:Adult Still's disease. Evolution of a clinical syndrome and diagnosis, treatment, and follow-up of 17 patients. 670 Apr 35

Indirect enzyme immunoassay, performed with Labsystems (Helsinki) kits, in 30 mothers and their newborns, revealed that 100% of parturient women present IgG antibody to hepatitis A (HAV), herpes simplex 1, and measles viruses, constantly transferring these antibodies to their newborns. 78.6% of the women had IgG to rubella (German measles) virus, passively transmitting them to their offsprings. Serological markers (HBsAg and anti-HBc) of hepatitis B virus infection were present in 42% of the investigated women, anti-HBc being also present in the serum of the newborns. Between the identified risk factors (in the past obstetrical history, current pregnancy, labor and early postnatal period) and the spectrum of IgG antibodies present in mothers no significant correlations were revealed. The same obvious lack of correlation between IgG antibodies and risk factors in the neonate (prematurity, low birth weight, malformations) was also found. However, the presence of IgG anti-measles (2 case), IgM anti-rubella (1 case) and IgM and HAV (4 cases) was associated, in the same order, with interstitial pneumonia, hepatosplenomegaly and death, icterus neonatorum, cardiovascular and neurologic malformations. Neither mothers nor their offsprings presented anti-HIV antibodies, suggesting that in the investigated patients no perinatal transmission had occurred.
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PMID:[The presence in pregnant women of the risk factor of serum antibodies against 9 viruses with significance in materno-infantile pathology and the transfer of these antibodies to the newborns]. 799 65

We report an infant with autoimmune neutropenia (AIN), idiopathic thrombocytopenia (ITP), and IgG2/IgA deficiency. The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly. A complete blood count revealed a platelet count of 2.0 x 10(3) cells/microliters, and a white cell count of 3,600 cells/microliters, with severe neutropenia (less than 1% bands and segmented cells). Neutrophils and platelets adhering to megakaryocytes were decreased in the bone marrow. Tests for serum neutrophil-binding IgG (NB-IgG) and platelet-associated IgG (PA-IgG) were positive. A diagnosis of both AIN and ITP was made and therapy with intact-type gamma-globulin and prednisolone was initiated. Improvement occurred, but was temporary. A lack of serum IgA and IgG2 was noted during the clinical course. The patient has not been susceptible to bacterial infections but has had a severe clinical course with rubella and chickenpox.
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PMID:An infant with both autoimmune neutropenia and idiopathic thrombocytopenia with IgG2/IgA deficiency. 847 60

Haemophagocytic syndrome is a systemic clinicopathological entity characterized by systemic proliferation of benign haemophagocytic histiocytes, fever, cytopenia, abnormal liver function and, frequently, coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections, a wide spectrum of malignant neoplasms, autoimmune diseases and drugs. We report a case of rubella virus-associated haemophagocytic syndrome in a previously healthy 29-year-old woman. Blood tests showed cytopenia, especially severe thrombocytopenia, liver dysfunction, hyperferritinaemia and hypercytokinaemia. Bone marrow examination showed many mature histiocytes with active haemophagocytosis. A skin biopsy from the rash revealed perivascular lymphohistiocytic infiltrates with haemophagocytic histiocytes in the upper and mid-dermis. The patient was treated with antibiotics and immunoglobulin, and by supportive measures including platelet transfusion, and recovered completely.
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PMID:Virus-associated haemophagocytic syndrome caused by rubella in an adult. 989 58


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