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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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PMID:Sickle cell anemia- clinical manifestations in 100 patients and review of the literature. 113 Apr 36

In the US and northern Europe, the prevalence of pregnant syphilitic women is estimated at .1-.6%, while in South Africa it was 7.6% in 1982. In 1978, there 108 cases in the US which increased to 268 reported cases in 1985. The increase of congenital syphilis (CS) by 25% from 1985 to 1988 was attributed to the spread of crack cocaine in the US. The rate was 10.5 cases/100,000 live births in the US during this period, a 21% increase. In contrast, in the Netherlands there were 2.5 cases/100,000 live births during 1982-85. Clinical symptoms appear 3 weeks after birth, but some are present at birth such as hepatosplenomegaly, bloated abdomen, cutaneous lesions, and nasal discharge turning into purulent rhinitis. Anemia occurs in 90% of children with CS. Generalized lymphadenopathy, splenomegaly with hepatomegaly, and syphilitic hepatitis may also occur. Syphilitic skeletal abnormalities include osteochondritis, periostitis, osteomyelitis, and osteitis. Meningovascular syphilis produces nervous system effects. CS complications include nephrotic syndrome and acute glomerulonephritis. Ocular abnormalities are caused by treponemes found in the cornea, sclera, uvea, retina and the optic nerve. Chorioretinitis and iridocyclitis are common ocular lesions. The pathogen Treponema pallidum can be diagnosed by dark field microscopy, by immunofluorescence, or by histopathological examination of silver-stained preparations. Pregnancy women with syphilis are treated with penicillin although failures have been reported after single or 2 or 3 in administrations of 2.4 MU benzathine penicillin and after giving tetracycline in 3rd trimester pregnancy. The CDC recommendation for treating infants with CS is iv 50,000 U/kg penicillin G every 8-12 hours for 10-14 days or im 50,000 U procaine penicillin once daily for 10-14 days. Single administration of 50,000 U/kg benzathine penicillin is recommended for newborn children whose mothers have been treated with erythromycin.
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PMID:Congenital syphilis. 161 61

From 1956 through 1989, 38 men and 26 women were seen at the Mayo Clinic with biopsy-proven AA. The underlying disorder was rheumatic disease in 42, infectious disease in 11, inflammatory bowel disease in 6, and other causes in 5. All patients were symptomatic at the time of diagnosis. Fifty-eight of the 64 patients had proteinuria or renal insufficiency. Fourteen also had significant symptoms of gastrointestinal amyloid, and 6 had amyloid goiter. None of the patients had symptomatic cardiac involvement, and only 3 had palpable hepatomegaly. Renal, gastric, rectal, fat, and marrow biopsies were positive for amyloid in 100%, 94%, 82%, 58%, and 46% of tested patients, respectively. The median survival of the entire group was 24.5 months. Thirty-five of the 47 deceased patients died as a direct result of their amyloidosis, primarily from complications of renal failure. Nine were successfully treated and had regression of the disease. Two with bronchiectasis responded to long-term cyclic antibiotic therapy, as did 1 patient with osteomyelitis. One patient with inflammatory bowel disease responded to surgical resection, and 1 with familial Mediterranean fever responded to colchicine. Four patients with rheumatic disease were treated with cyclophosphamide (in 2) and methotrexate (in 2), with complete resolution of their renal disease. All 9 successfully treated patients are alive, with a median follow-up of 58 months. Statistical analysis revealed that creatinine values greater than or equal to 2.0 mg/dl (P less than 0.003) and a serum albumin value less than 2.5 g/dl (P less than 0.02) were associated with a poorer survival. The single strongest variable associated with poor survival was a serum creatinine level greater than 2 mg/dl at presentation, with a median survival of 11.2 months compared to patients with a creatinine level less than 2.0 mg/dl, with a median survival of 56.9 months.
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PMID:Secondary systemic amyloidosis: response and survival in 64 patients. 206 9

The pattern of illness in 60 consecutive children with homozygous sickle cell disease who attended the Paediatric Emergency Room of a busy Lagos hospital with acute illness was studied prospectively. Their ages ranged from 3 months to 13 years with a peak in the 2nd year. There were twice as many boys as girls. The commonest symptoms were fever, limb or abdominal pain and cough, and the commonest signs were pallor and hepatomegaly. Painful crises occurred in 27, anaemic crises in 11, and a combination of these in 12 children. Infection was detected in 76% of subjects in crises. Infection was found in 82% of all the children and was mainly bacterial. The commonest infections were pneumonia (35%), bacteraemia (32%), tonsillitis/pharyngitis (17%) and osteomyelitis (8%). The predominant bacteria isolated were Klebsiella spp (38%), E. coli (23%), Staph. aureus (23%), Staph. albus (23%) and Pseudomonas spp (23%). Some children had multiple isolates. Bacterial infection was a major cause of morbidity in very young children and merits appropriate control and preventive measures in this age group. The spectrum of bacteria isolated makes it unlikely that the specific anti-pneumococcal measures widely advocated in Europe and America for young children with SCA would be appropriate in Nigeria.
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PMID:Acute illness in Nigerian children with sickle cell anaemia. 244 66

Gaucher's disease involves the liver, the spleen, and the bone. Liver-spleen and bone scintigraphy were used during an 8-yr period to evaluate changes caused by this disease. Patients were investigated with a liver-spleen scan for abdominal pain, mechanical discomfort, enlarged liver or spleen on physical examination, abdominal mass, abnormal liver function tests, and symptoms of hypersplenism. Fourteen liver-spleen scans were performed in nine patients. Liver scintigraphy showed various degrees of enlargement and inhomogeneous uptake. In two patients focal defects were detected. In one, focal defects were due to liver involvement with Gaucher's disease, but in the other they were caused by metastatic pancreatic carcinoma. The study was also useful in detecting splenic infarction and in following enlargement of the spleen after partial splenectomy. The main indication for bone scintigraphy in six patients was bone pain. This was found to be caused by either aseptic necrosis of the head of the femur, bone infarction, pathological fractures, or osteomyelitis. Loosening after total hip replacement was ruled out in three patients and missed in one patient. Scintigraphy appears to be a simple, sensitive test for evaluation of the liver, spleen, and bony skeleton in patients with symptomatic Gaucher's disease.
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PMID:Scintigraphic findings in Gaucher's disease. 376 Sep 80

2 children with pyogenic liver abscesses were hospitalized during the past 2 years. A 6-year-old boy had high fever and hepatomegaly, and a large liver abscess was found in the right hepatic lobe. Streptococcus milleri was isolated from the pus. Treatment with a combination of prolonged drainage of the abscess and antibiotic therapy was successful. A 4-month-old girl who had prolonged fever was found to have osteomyelitis of 3 thoracic vertebrae and 2 liver abscesses in the right lobe. She was treated successfully with broad spectrum antibiotics. Additional workup revealed that she had chronic granulomatous disease.
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PMID:[Pyogenic liver abscess in children]. 945 70

A total of 1300 birds in flock of breeder Pharaoh quail (Coturinix coturnix) experienced a moderate rate of mortality (13%) during a 7-day period. Clinical signs included depression, ruffled feathers, prostration, lameness, inapetence, diarrhea, and periorbital sinus swelling with mucoid discharge and lameness. Gross lesions observed in dead quail were emaciation, carcass congestion, mild hepatomegaly with green discoloration, congested intestinal mucosa, caseous purulent arthritis-osteomyelitis, and thickened crop mucosal epithelium. Histopathologic examination revealed mild hepatic amyloidosis, proliferative parabronchitis, splenic reticular cell hyperplasia, thymic cortical atrophy, subacute bacterial osteomyelitis, periarthritis, and crop mycosis. Pasteurella multocida was isolated from the joints of these birds and the isolates were serotype 3 x 4. These findings suggest that Pharaoh quail are susceptible to P. multocida and are likely to develop subacute to chronic fowl cholera.
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PMID:Subacute to chronic fowl cholera in a flock of Pharaoh breeder quail. 953 3

Hepatic sarcocystosis was diagnosed in a horse in association with refractory bacterial osteomyelitis and plasma cell tumor of the maxilla and hepatic salmonellosis. Gross lesions included pleural, pericardial, and peritoneal effusions, hepatomegaly, gastric ulceration, colonic edema, and proliferative tissues filling 2 maxillary dental alveoli. Histologically, liver was characterized by severe suppurative, necrotizing, periportal hepatitis, and severe periacinar necrosis. Hepatocytes frequently contained protozoal schizonts in various stages of development. In mature schizonts, merozoites were often arranged radially around a central residual body, consistent with asexual division by endopolygeny. Ultrastructural features of merozoites included an apical conoid and polar ring, anterior micronemes, central nuclei, and absence of rhoptries. These protozoa did not react to antisera raised against Neospora caninum, Sarcocystis neurona, Toxoplasma gondii, or Hammondia hammondi. The microscopic and ultrastructural characteristics and immunoreactivity of this organism are consistent with a Sarcocystis sp. other than S. neurona. This is the first report of Sarcocystis-associated hepatitis in a horse. The life cycle of this organism and source of infection are unknown.
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PMID:Hepatic sarcocystosis in a horse. 1057 37

We report an analysis of clinical course of 18 patients presenting with Staphylococcus aureus sepsis. Community acquired infection was caused by Methicillin susceptible S. aureus (MSSA) in 11 patients. MSSA in 3 and Methicillin Resistant S. aureus strains (MRSA) in 4 patients, were the etiologic factor in 7 patients with nosocomial infection. From anamnestic data patients presented with: elevated body temperature--18/18, arthralgia and myalgia--9/18, headache--8/18, nausea--6/18, chills--2/18. Physical examination on admission revealed: meningismus--12/18, hepatomegaly--11/18, purulent and haemorrhagic skin lesions--7/18 and impaired neurological status (Glasgow Coma Scale < or = 12)--6/18. The mean APACHE III score, calculated from data collected at diagnosis of sepsis was 47 (7-114). Several complications had been observed: endocarditis--10, purulent meningitis--5, focal CNS lesions--5, pneumonia--8, pulmonary abscess--3, hydrothorax--1, abscesses of the spleen--5, renum--4, osteomyelitis--2. 11/18 patients required ICU treatment. Ventilator assistance of respiration was necessary in 7/18. Acute thrombocytopenia (< 100,000/ml) was diagnosed in 60%. In 5 patients suppurative meningitis had been diagnosed with a mean pleocytosis-837 (173-1898) microL. The results of treatment were satisfactory in 11 patients, 3 patients required further surgical treatment (2--cardiosurgery, 1--orthopedic surgery), 4 patients died. Infection caused by community acquired MSSA strains had been characterized by severe clinical course with increased incidence of endocarditis, organ failure and abscess forming. We conclude that Staphylococcus aureus sepsis is still a life-threatening disease, which should be treated at centers with immediate access to imaging techniques of CNS and circulatory system as well as intensive care and cardiosurgery. Community acquired S. aureus sepsis compared with nosocomial infection is characterized by more severe clinical course and higher mortality, despite of a great susceptibility to most antibiotics of causative S. aureus strains.
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PMID:[Staphylococcus aureus sepsis--still life threatening disease]. 1177 Mar 18

Inherited neutropenia is characterized by a decrease in the absolute number of circulating neutrophils and an increased susceptibility to infections. The current study was performed to determine the clinical and laboratory findings of Iranian patients with inherited neutropenias. Records of 26 patients (14 male, 12 female) with inherited neutropenia were reviewed in this study. The patients had been referred to Children's Medical Center, a referral center for immunodeficiency disorders in Iran, during a 22-year period (1981-2003). Primary immunodeficiency disorders of these patients were as follows: cyclic neutropenia (8 patients), Shwachman-Diamond syndrome (7 patients), Kostmann syndrome (6 patients), and Chediak-Higashi syndrome (5 patients). The mean absolute neutrophil count of patients was 398.2 +/- 259.3 cells/mm (range 74-1,152/mm) at the first visit. Twenty-one patients showed severe, four moderate, and one mild neutropenia. Sixteen of these patients had leukopenia, seven anemia, two thrombocytopenia, and one monocytosis. The most common presenting complaints in these patients were oral ulcer, otitis, pneumonia, diarrhea, cutaneous abscess, and oral candidiasis. The patients first manifested symptoms of infection suggesting neutropenia at a median age of 7.5 months (range 1 month to 10 years). During follow-up, respiratory infections developed in 24 cases, oral manifestations in 20 patients. The most common infections, in descending order of frequency, were otitis media, abscesses, pneumonia, oral ulcers, acute diarrhea, cutaneous infections, oral candidiasis, and periodontitis. Less frequent infections were sinusitis, cystitis, conjunctivitis, meningitis, and osteomyelitis. Nonspecific symptoms (hepatomegaly and splenomegaly) were also detected in 10 patients and 1 patient, respectively. Three patients died of recurrent infections. The infectious manifestations both at presentation and during follow-up in inherited neutropenia were similar. Although inherited neutropenias are rare, recurrent infections always deserves further evaluation for detecting such disorders.
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PMID:Congenital neutropenia and primary immunodeficiency disorders: a survey of 26 Iranian patients. 1601 23


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