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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Female LSH hamsters infected with Treponema pallidum subsp, endemicum before pregnancy or during early pregnancy transmit a form of syphilis to the fetus that is similar to human congenital syphilis. The offspring develops rhinitis, skin rash, failure to thrive, and hepatosplenomegaly. T. pallidum is detectable in their livers, spleens, and nasal secretions. Immunoglobulin M antibodies are detected in the serum.
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PMID:Experimental model of congenital syphilis. 833 90

Testing for syphilis during pregnancy reveals a positive serologic status in 0.02% of cases. However, a 66% rate of stillbirths is noted in women who are infected and who have not benefited from any treatment. Routine screening is at present performed during the early stages of pregnancy but a second serologic test during the third trimester is useful in the diagnosis of a late infection especially in drug users or HIV (human immunodeficiency virus) positive patients. Congenital syphilis is diagnosed in utero when a positive maternal serologic status is associated with ultrasound images showing fetal abnormalities; these include hepatosplenomegaly, hyperechogenic bowel, signs of bowel obstruction or fetal hydrops. Maternal syphilis is treated by delayed action penicillin and is indicated even for patients allergic to the antibiotic which in this particular case is delivered after desensitization. First line therapy by intravenous penicillin is indicated when confronted with the following high risk factors of congenital syphilis: an elevated titre of VDRL (venereal disease research laboratory) at the time of diagnosis or delivery, unknown date of the precise onset of the infection, the appearance of a rash or of a chancre during pregnancy, ultrasound fetal abnormalities or late therapy during the third trimester. Treatment of the new-born child will depend on the results of clinical, serologic and X-ray evaluation. Long term follow-up for at least a year is mandatory.
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PMID:[Maternal and congenital syphilis]. 959 61

A preterm, very low birth weight infant was born to a mother with early latent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU of benzathine penicillin. The infant had clinical, laboratory, and radiographic abnormalities consistent with congenital syphilis, ie, a Venereal Disease Research Laboratory test titer that was fourfold greater than was the maternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonitis, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examination showing a reactive Venereal Disease Research Laboratory test, pleocytosis, and elevated protein content. The infant died on the third day of life, and an autopsy revealed an evolving gumma of the anterior pituitary. Immunoglobulin M immunoblotting of serum and CSF was positive, and polymerase chain reaction detected Treponema pallidum DNA in endotracheal aspirate and CSF. This case highlights the pathologic abnormalities observed in congenital syphilis and focuses on the rare finding of an evolving anterior pituitary gumma. Furthermore, it documents the failure of maternal syphilis treatment during the last 4 weeks of pregnancy to cure fetal infection and supports the recommendation that all infants born to mothers with syphilis treated during the last 4 weeks of pregnancy should receive penicillin therapy.
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PMID:Pituitary gland gumma in congenital syphilis after failed maternal treatment: a case report. 1039 Feb 90

In 2 infants, a girl and a boy, congenital viral infection was diagnosed in the neonatal period. The prenatal examination (serologic investigation for Toxoplasma gondii, rubella virus, cytomegalovirus, herpes simplex virus and syphilis (TORCHES)) was negative. In both cases prenatal ultrasonography was abnormal and suggested intrauterine infection. The infants were born with typical symptoms of multisystem disease, known as symptomatic congenital cytomegalovirus infection (jaundice, petechiae, hepatosplenomegaly, intrauterine growth retardation, microcephaly and cerebral calcifications) and congenital rubella syndrome (intrauterine growth retardation, congenital heart disease, cataract, hepatosplenomegaly and cerebral calcifications), respectively. Both had severe cerebral damage. To diagnose severe congenital infection in the first trimester of pregnancy in presence of congenital anomalies in utero there are other possible methods than TORCHES investigation, such as polymerase chain reaction and virus culture in amniotic fluid or in foetal blood obtained by cord puncture.
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PMID:[Congenital infection: diagnostic serology of the mother not always definitive]. 1121 56

This case report highlights the presentation of syphilis in a small-for-gestational age (SGA) female infant who was born to a mother with no prenatal care. In the first 2 days of life, the infant developed hepatosplenomegaly, conjugated hyperbilirubinemia, thrombocytopenia, and hemolysis. After extensive diagnostic investigation, the case was solved on the infant's day of life (DOL) 12. In retrospect, the health care team had obtained a diagnostic result that could potentially have solved the case on DOL 2. Although this infant suffered no long-term sequelae secondary to the delayed diagnosis, the review of this case presentation may assist other clinicians in an early identification of a potentially lethal diagnosis.
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PMID:Case report of an SGA infant with jaundice, direct hyperbilirubinemia, hepatosplenomegaly, thrombocytopenia, and a negative VDRL. 1513 91

Patients with systemic lupus erythematosus (SLE) have an extremely variable prognosis and mortality. The purpose of this report is to highlight the importance of looking for lupus parameters in patients with autoimmune haemolytic anaemia (AIHA) and/or immune thrombocytopenia (ITP) as this represents a subgroup of systemic lupus erythematosus (SLE) patients with a fairly good prognosis. We report a case of an 8-year-old Malay boy who was admitted to hospital with fever and gum bleeding. Physical examination revealed a malar rash, oral ulcers, petechial haemorrhages and bruises over the limbs, generalised lymphadenopathy and hepatosplenomegaly. Laboratory investigations confirmed the diagnosis of SLE. The patient's serum showing the presence of antinuclear factor, antiphospholipid antibodies and a biological false-positive test for syphilis. Immunological and haematological parameters were in keeping with combined AIHA and ITP (Evans' syndrome). No organ involvement was present and the patient responded well to corticosteroid therapy. This case demonstrates the importance of making an early diagnosis of SLE with haematological complications, in order to ensure full benefit of therapy and emphasises the good prognosis expected in this subgroup of SLE patients.
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PMID:Significance of autoimmune haemolytic anaemia and immune thrombocytopenia (Evans' syndrome) in systemic lupus erythematosus. 1632 81

A 17-year-old girl previously in good health presented with a 2-month history of recurrent, high-grade fever; general fatigue; anorexia; a 10-kg weight loss; and multiple, painful, reddish skin lesions on the lower abdomen. Some lesions were ulcerated, with an oily yellowish brown discharge. A systemic review was unremarkable other than bleeding from the nose. Her medical and family histories were unremarkable. On examination, the patient was pale, jaundiced, and febrile (temperature of 39 degrees C). She had enlarged lymph nodes in the axillary and inguinal areas. There was moderate hepatosplenomegaly. Local skin examination revealed multiple erythematous, tender, and firm subcutaneous nodules of variable size (1-2 cm) on the lower abdomen. Some nodules were ulcerated, with oily yellowish brown discharge and overlying ecchymosis (Figures 1 and 2). Mucous membranes were free of lesions. Laboratory investigations showed pancytopenia, an elevated erythrocyte sedimentation rate (>80 mm/h), normal renal function tests, abnormal hepatic function tests (alanine aminotransferase 172 U/L, aspartate aminotransferase 229 U/L, alkaline phosphatase 725 U/L, and total bilirubin 100 mmol/L [normal range 0-18 mmol/L]), conjugated bilirubin 45 mmol/L (normal range 0-5 mmol/L), and high triglycerides 855 mg/dL (normal range 20-200 mg/dL). Prolonged prothrombin time, 26 seconds (normal range 13-16 seconds); prolonged activated partial thromboplastin time, 61 seconds (normal range 26-38 seconds); positive disseminated intravascular coagulation studies evidenced by low fibrinogen, 74 mg/dL (normal range 160-350 mg/dL); and positive fibrinogen degradation products were also noted. Throat, midstream urine, and blood culture results were negative. Serologic tests for syphilis, HIV, and hepatitis B and C viruses were negative. Epstein-Barr virus and cytomegalovirus serologic values revealed evidence of past infection. Tuberculin and Coombs tests were negative. The alpha1-antitrypsin level was normal. Antinuclear and anti-smith antibodies, rheumatoid factor, and cryoglobulins were negative. CT showed enlarged lymph nodes in the axillary and inguinal areas, bilateral small pleural effusion, moderate hepatosplenomegaly, severe fatty infiltration of the liver, and thickening of lower abdominal subcutaneous tissue. A liver biopsy showed steatohepatitis. Bone marrow aspirate and trephine were normal. A deep punch biopsy of a nodule from the right lower abdomen revealed lobular panniculitis with atypical lymphocytes and large macrophages with cytophagocytosis ("beanbag" cells) (Figures 3 and 4). Immunohistochemistry showed that these atypical cells were positive for CD3, CD8, granzyme B, and perforin, and negative for CD56. T-cell gene rearrangement studies on skin lesions revealed a monoclonal T-cell receptor (gamma-chain) gene rearrangement, supporting the diagnosis of subcutaneous panniculitis-like T-cell lymphoma. On presentation, the initial treatment included 6 U of fresh frozen plasma, 2 U of packed red blood cells, and 2 g IV fibrinogen for 3 consecutive days. The patient was started on prednisolone 60 mg orally once daily and cyclosporine A 5 mg/kg/d orally in two divided doses. The fever and other systemic symptoms and skin lesions resolved within 2 weeks after the treatment. The prednisolone dose was tapered gradually, and a maintenance dose of cyclosporine A was continued. The patient's condition remained in remission at 12-month follow-up; there was no evidence of clinical relapse.
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PMID:Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome successfully treated with cyclosporin A. 1685 14

Neonatal alopecia has been described in many circumstances but never as a part of the constellation of signs in congenital syphilis. Common clinical features of early congenital syphilis include hepatosplenomegaly, skeletal deformities, hematologic disturbances, and mucocutaneous features such as rhinitis and maculopapular rash. Syphilitic alopecia has previously been described only in conjunction with secondary syphilis. In this article, we describe the first occurrence of alopecia in a neonate with congenital syphilis.
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PMID:Early diffuse alopecia in a neonate with congenital syphilis. 1715 99

Hepatitis is a rare clinical manifestation of syphilis. In this report a 50 years old male patient who was diagnosed as secondary syphilis presenting with hepatitis has been discussed. The patient was admitted to the hospital with high fever and skin rash, and his history revealed a suspected sexual contact. He indicated that he had been admitted to a health center eight months ago because of the presence of a penile wound, however VDRL (Venereal Disease Research Laboratory) test was negative at that time. Fever (39.5 degrees C), jaundice in skin and sclera, generalized macular and maculopapular skin rash including palms and soles, lymphadenopathy and hepatosplenomegaly were detected in physical examination. Laboratory tests yielded elevated erythrocyte sedimantation rate, high CRP levels and elevated liver enzyme levels, however viral hepatitis markers together with VDRL and TPHA (Treponema pallidum hemagglutination) tests were found negative. Ceftriaxone therapy was initiated because of the presence of high fever (40 degrees C) and 30 leukocyte/mm3 in urine, and the absence of bacteria in Gram staining of urine sample. However, the antibiotic therapy was discontinued since fever persisted. As the clinical signs and symptoms strongly indicated syphilis, the serological tests were repeated and VDRL positivity at 1/8 and TPHA positivity at 1/1280 titers were detected. Ceftriaxone therapy was restarted and continued for 14 days with complete cure. Since the spouse of the patient was also found VDRL and TPHA positive, she was treated with penicilin. The presentation of this case emphasized the importance of repeating the serological tests for syphilis since they might be negative in the early stages of infection. The case also indicates that syphilis should be considered in the differential diagnosis of hepatitis.
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PMID:[A case of secondary syphilis with hepatitis]. 1768 17

In the UK, diagnosis of syphilis among women of child-bearing age has more than doubled between 1999 and 2007, and each year, around ten cases of congenital syphilis have been reported. Doctors should, therefore, be aware of the potential presentation of congenital syphilis, a deadly disease yet curable when managed correctly. We present a rare case of a premature neonate of 29 weeks displaying manifold clinical signs of congenital syphilis acutely after delivery, including coagulopathy, hepatosplenomegaly, intraventricular ischaemic lesions and metaphyseal bone changes, not previously described in the literature at this age in the UK.
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PMID:Early congenital syphilis in a premature baby. 2110 3


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