1/9. hepatomegaly and cholestasis as primary clinical manifestations of an AL-kappa amyloidosis.A 53-year-old man, who presented with weight loss over a period of 10 months, hepatomegaly, markedly raised cholestatic enzymes and Ca 19-9, was initially suspected of suffering from metastatic cholangio-carcinoma. Liver biopsy revealed depositions of AL-amyloid. Further investigations confirmed a generalized amyloidosis. Biopsies taken from the gastric, colonic, and bronchial mucosa all showed depositions of amyloid. A nephrotic syndrome was interpreted as being secondary to the renal involvement. echocardiography identified changes which were consistent with cardiac involvement. A plasmacytoma or lymphoma was excluded. At the time of diagnosis the patient was in a good physical condition with normal renal function. Within a few weeks the renal function deteriorated and after 2 months the patient developed ascites and became jaundiced. Four months after initial presentation the patient died from cardiac failure.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/9. Constrictive pericarditis with dwarfism in two siblings (mulibrey nanism).Two siblings with marked dwarfism, now 11 and 19 years of age, have been followed from infancy. The girl had frequent episodes of pneumonitis and presented at age 4 years with hepatic enlargement and ascites which proved to be due to constrictive pericarditis. The boy presented with growth failure and pseudohydrocephalus. He had fibrous dysplasia of the tibia and a pathologic fracture; acute hepatic congestion followed physical activity at age 13 years and led to the diagnosis of constrictive pericarditis. Muscle function was normal, there was no evidence for a primary liver disorder, and mental development was normal so that the coined word "mulibray" seemed inappropriate. pericardiectomy produced only partial improvement; both patients have hepatic enlargement and continue to need diuretics. A third patient with dwarfism, frequent respiratory infections, and pericardial calcification has certain features of the syndrome.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/9. New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis b. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.- - - - - - - - - - ranking = 17.816819818379keywords = physical examination, physical (Clic here for more details about this article) |
4/9. Inflammatory myofibroblastic tumor of the liver.We report a case of hepatic inflammatory myofibroblastic tumor in a 26-year-old African American man who presented with right upper quadrant pain, weight loss, and fatigue during the previous year. hepatomegaly was found on physical examination. Laboratory findings were significant for mild normocytic, normochromic anemia and elevated erythrocyte sedimentation rate. Imaging studies showed 2 contiguous masses suspicious for malignancy. A left partial hepatectomy was performed; the preoperative differential diagnosis was for angiosarcoma and hepatocellular carcinoma. The resected liver specimen showed 2 contiguous, firm, tan-white nodules that microscopically represented a proliferation of spindled myofibroblast cells set in an inflammatory and collagenized background. The spindle cells were strongly reactive for smooth muscle actin but negative for ALK-1. The morphologic and immunophenotypic findings, coupled with the clinical presentation, were consistent with an inflammatory myofibroblastic tumor of the liver.- - - - - - - - - - ranking = 17.816819818379keywords = physical examination, physical (Clic here for more details about this article) |
5/9. hepatomegaly as a single presenting sign of stage IV-S neuroblastoma.This article presents the case of an infant who on routine physical examination at the age of 5 months was found to have hepatomegaly. The initial investigation revealed borderline liver function tests, and ultrasonography showed multiple space-occupying lesions. Further workup using different imaging techniques, urinary vanillylmandelic acid, serum catecholamines, and liver biopsy led to the diagnosis of stage IV-S neuroblastoma. No primary site outside of the liver could be demonstrated. The tumor regressed spontaneously.- - - - - - - - - - ranking = 17.816819818379keywords = physical examination, physical (Clic here for more details about this article) |
6/9. adult respiratory distress syndrome associated with miliary tuberculosis.Three patients with respiratory failure resulting from miliary tuberculosis had a characteristic clinical presentation that included a long history of a prominent cough, dyspnea, weight loss, tachycardia, tachypnea, pulmonary adventitious sounds, and hepatomegaly. Hematologic investigation showed a normal white cell count with marked left shift in the morphology of white cells in all three patients, and evidence of disseminated intravascular coagulation in one patient. In only one patient was the initial sputum positive for acid-fast bacilli; in the others, invasive diagnostic procedures including lumbar puncture, bone marrow trephine, and open-lung biopsy were necessary for diagnosis. Miliary tuberculosis should be suspected in patients with adult respiratory distress syndrome of unknown etiology. Simple diagnostic procedures such as sputum, bronchial brushings, and urine examination should be followed by bone marrow trephine, liver biopsy, transbronchial lung biopsy, and lumbar puncture if physical signs of meningitis are present.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/9. Transient neonatal pustular melanosis.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.- - - - - - - - - - ranking = 17.816819818379keywords = physical examination, physical (Clic here for more details about this article) |
8/9. Amebic liver abscess in children.Five children with amebic liver abscesses are presented, and the distinctive clinical and laboratory features for these and 119 other children are described. The majority (91%) were less than 3 years old, and 77% had an isolated abscess in the right hepatic lobe. Each child presented with a history of fever and gastrointestinal symptoms, and two-thirds presented with cough or difficulty breathing. Most (81%) patients had hepatomegaly on physical examination and some had a well-defined mass. Hematologic abnormalities including anemia, neutrophilia and an increased ratio (greater than 0.15) of immature neutrophils to total neutrophils were commonly observed at the time of admission. Of interest, intravenous pyelograms revealed deviation of the right kidney due to hepatomegaly in each of three children studied. All patients evaluated had one or more filling defects demonstrated by liver-spleen scan or abdominal ultrasound. Most patients underwent either an open (9%) or closed (77%) drainage procedure. Fifty-six children (46%) died, in some cases before specific therapy was instituted. Of those who recovered all received therapy with metronidazole or a combination of chloroquine with emetine or dehydroemetine.- - - - - - - - - - ranking = 17.816819818379keywords = physical examination, physical (Clic here for more details about this article) |
9/9. Clinical and morphologic features of hepatic angiosarcoma in vinyl chloride workers.Fifteen male workers exposed to vinyl chloride developed angiosarcoma of the liver. Thirteen died of disease and two are currently living for short periods after diagnosis. Their ages ranged from 36 to 58 years (average 47.5 years). Their exposure time ranged from 4 to 27.8 years (average 16.9 years). The most common presenting symptoms were fatigue, weight loss, and abdominal pain. hepatomegaly followed by splenomegaly were the most common physical findings. Biochemical profiles yielded variable results and proved to be of little value in the detection or diagnosis. Of eight patients autopsied, distant organ involvement was present in two cases, duodenal involvement in one, and direct extension of tumor to adjacent organs or tissues in four additional ones. The remainder, diagnosed by open liver biopsy, revealed no tumor extension. The gross features of the tumors were hemorrhagic necrosis, cystic degeneration, fibrosis, and apparent multicentricity. The histologic features were those of the typical angiosarcoma found in a variety of sites with a wide range of cellular differentiation. The histologic diagnosis was often impaired by the extensive tumor necrosis. Elsewhere in the liver subcapsular fibrosis, a distinct type of portal fibrosis, and endothelial cell hyperplasia with or without sinusoidal dilatation were noted. The reduction of industrial chemical exposure has already been achieved and will hopefully eliminate this chemically related tumor in the future. There is, however, a significant group of previously exposed workers who will require careful monitoring to detect functional abnormalities of the liver and possible early neoplastic changes.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |