Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Schistosoma mekongi is a newly recognized species of Schistosoma that is closely related to Schistosoma japonicum and is pathogenic to humans. Like the other forms of schistosomiasis in which the parasites reside in the mesenteric vasculature, S. mekongi infection has hepatosplenomegaly and portal venous hypertension as its most common serious sequelae. Schistosomiasis, although endemic in certain areas of Southeast Asia, has not been recognized as a health problem in Asian refugees who are currently entering the United States. In the present study 12 Laotian immigrants with S. mekongi infection were examined. The clinical, laboratory, and parasitologic findings in these patients are discussed. The diagnosis was made by examination of stool specimens in 11 patients and rectal biopsy specimens in one patient. All patients were asymptomatic, although the six youngest children had hepatomegaly. It is suggested that specific serologic tests may be useful in screening Asian refugees for infection with Schistosoma.
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PMID:Infection with Schistosoma mekongi in Southeast Asian refugees. 731 Jan 75

A patient with early infantile galactosialidosis presenting as congenital adrenal hyperplasia with clitoral hypertrophy and arterial hypertension is reported. Serum 17-alpha-OH-progesterone and plasma renin levels were elevated. Adrenal hyperplasia and thickening of the cardiac septum were detected by sonography; however, progressive hepatosplenomegaly, increasingly coarse features, and vacuolization of bone marrow and liver cells suggested a storage disorder. Combined deficiency of beta-galactosidase and sialidase enzyme activity in both lymphocytes and cultured fibroblasts was detected. This patient with early infantile galactosialidosis is the first reported who presented with congenital adrenal hyperplasia.
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PMID:Infantile galactosialidosis presenting with congenital adrenal hyperplasia and renal hypertension. 821 48

The clinical picture of twelve patients with sarcoidosis commencing under 4 years of age is presented. Follow-up ranged from 5 to 30 years with a mean of 11.5 years. Two patients were mother and daughter, the other cases were sporadic. All children developed the typical symptom triad of rash, polyarthritis and uveitis with onset before their 4th birthday. Further symptoms comprised fever, hepatosplenomegaly, parotid swelling and hypertension. Eight patients suffered from cardiac and four from cerebral involvement; one girl died from cerebral involvement at the age of 12. Two developed severe lung changes. The prognosis was poor with sequelae in all patients. Several children seem to have improved on immunosuppressive therapy. Early onset sarcoidosis is a multisystemic granulomatous disease; since polyarthritis is a dominant manifestation, many patients are diagnosed as having juvenile chronic arthritis. The early onset form must be distinguished from later onset sarcoidosis, which mainly affects children over 8 years of age or young adults. The disease types differ in their presentation, symptoms, course and prognosis; some of the clinical features and the histology, however, are identical.
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PMID:Sarcoidosis of early onset. A challenge for the pediatric rheumatologist. 829 66

We describe two cases of POEMS syndrome, both with polyneuropathy, monoclonal gammopathy of the IgG lambda type, thickening of the skin with Raynaud phenomenon, multiple osteosclerotic lesions and hypothyroidism. One of them, also had papilledema, elevated cerebrospinal fluid protein, intracranial hypertension and phrenic nerve palsy; the other one had ascites and hepatosplenomegaly. Phrenic nerve palsy associated to this syndrome has not been described previously.
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PMID:[The POEMS syndrome, apropos of 2 cases and review of the literature]. 896 62

Bone marrow necrosis (BMN) is a relatively rare entity and has been associated with a poor prognosis. It is most commonly found in patients with neoplastic disorders, severe infections and sickle cell anemia. An unusual case of antiphospholipid syndrome (APS) with extensive bone marrow necrosis is described in a 27 year old woman. The patient presented with severe pancytopenia, some cognitive impairment resulting from a previous cerebrovascular accident, fever, hypertension, dyspnoea, tachycardia, hepatosplenomegaly, and vaginal bleeding. Her laboratory findings included a strongly positive Coombs' test (anti-IgG and anti-C3d), a prothrombin time of 23 seconds and an activated partial thromboplastin time of 45 seconds. Anticardiolipin antibody tests were positive. Antinuclear and anti-DNA antibodies were negative but the anti-SM test was positive. A bone marrow biopsy specimen was reported as showing extensive necrosis. The patient was treated with steroids, transfusion, and plasma exchange with some clinical improvement but her pancytopenia did not respond and necessitated frequent transfusions. This case lends further support to the association between APS and BMN.
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PMID:Bone marrow necrosis in antiphospholipid syndrome. 915 83

Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and prompt therapy have the potential to reduce mortality. Leukemias, lymphomas and central nervous system tumors account for more than one half of new cancer cases in children. Early in the disease, leukemia may cause nonspecific symptoms similar to those of a viral infection. Leukemia should be suspected if persistent vague symptoms are accompanied by evidence of abnormal bleeding, bone pain, lymphadenopathy or hepatosplenomegaly. The presenting symptoms of a brain tumor may include elevated intracranial pressure, nerve abnormalities and seizures. A spinal tumor often presents with signs and symptoms of spinal cord compression. In children, lymphoma may present as one or more painless masses, often in the neck, accompanied by signs and symptoms resulting from local compression, as well as signs and symptoms of systemic disturbances, such as fever and weight loss. A neuroblastoma may arise from sympathetic nervous tissue anywhere in the body, but this tumor most often develops in the abdomen. The presentation depends on the local effects of the solid tumor and any metastases. An abdominal mass in a child may also be due to Wilms' tumor. This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. A tumor of the musculoskeletal system is often first detected when trauma appears to cause pain and dysfunction out of proportion to the injury. Primary care physicians should be alert for possible presenting signs and symptoms of childhood malignancy, particularly in patients with Down syndrome or other congenital and familial conditions associated with an increased risk of cancer.
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PMID:Recognition of common childhood malignancies. 1077 55

Many infants with intrauterine growth retardation (IUGR) are screened for TORCH infections. The yield and costs of such a practice may not be justifiable. Medical charts of infants with IUGR who had a workup for toxoplasmosis, other (infections), rubella, cytomegalovirus (infection), and herpes (simplex) (titer) (TORCH) infections were reviewed for the presence of clinical findings, laboratory and head ultrasound abnormalities associated with intrauterine infections. Maternal charts and reports of placental pathology were reviewed for identifying maternal illnesses and placental causes associated with IUGR. Seventy-five out of 182 infants (41%) with IUGR had a workup for TORCH infection. Maternal conditions associated with IUGR included: pregnancy-induced hypertension (19%), tobacco use (43%), alcohol abuse (21%), illicit drug use (24%), chronic hypertension, diabetic vasculopathy or collagen vascular disease (12%), and multiple gestation (3%). Placental pathology was available in 53/75 cases. Thirty-six of fifty-three (67%) placentae had abnormalities associated with IUGR: placental infarcts (22 of 36), vasculitis/villitis (15 of 36), placenta previa (1 of 36), abruptio placenta (2 of 36), and velamentous insertion of umbilical cord (1 of 36). Clinical findings among infants included hepatosplenomegaly, cataract or rash (1 of 75), thrombocytopenia and/or neutropenia and/or direct hyperbilirubinemia (11 of 75). Seven out of 75 infants had dysmorphic features. None of the infants (0 of 75) had positive IgM titers for toxoplasma, rubella, cytomegalovirus (CMV), or herpes simplex virus (HSV). No infants (0 of 43) had elevated total IgM titers; one infant (1 of 57) had a positive urine culture for CMV. One infant had evidence of calcifications on head ultrasound and a second infant had hydrocephalus (2 of 43). The costs associated with workup for TORCH infections among 75 infants included: TORCH titers determination: $17,816, total IgM titers: $1318, urine culture for CMV: $5734, and head ultrasound: $28,165. The yield of workup for TORCH infection among infants with IUGR is poor and does not justify the incurred costs.
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PMID:Yield and costs of screening growth-retarded infants for torch infections. 1101 37

A 77-year-old woman was referred to our hospital because of leukocytosis and leukoblastosis in September 1999. She was healthy except for hypertension, and no abnormal findings in the peripheral blood had been observed up to December 1998. Physical examination revealed neither hepatosplenomegaly nor superficial lymphadenopathy. A bone marrow film showed massive proliferation of blast cells (87.8%), some of which contained coarse basophilic granules (38.6%). The cells were negative for peroxidase and esterase (alpha-naphtyl butyrate and ASD-chloroacetate) staining, but the granules showed metachromasia upon toluidine blue staining. As immunophenotypic analysis of the cells showed double positive for CD13/CD19 but negativity for CD33, this case did not meet the diagnostic criteria for biphenotypic acute leukemia. Chromosome and gene analysis showed positivity for the Ph1 chromosome with minor bcr/abl chimeric mRNA. A homogenate of the peripheral mononuclear cells demonstrated a high concentration of histamine. Electron microscopy analysis confirmed that some of the blast cells contained dense granules, which closely resembled "immature basophil granules" morphologically. These results suggested that the blast cells showed basophilic differentiation. As the clinical course and peripheral blood findings were different from blastic crisis of chronic myelogenous leukemia (CML) and CML with minor bcr/abl chimeric mRNA, the present case was diagnosed as "multiphenotypic acute leukemia", a type of acute basophilic leukemia classified by Duchayne.
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PMID:[Basophilic differentiation of leukemic cells in a patient with acute leukemia carrying minor bcr/abl chimeric mRNA]. 1152 47

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.
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PMID:POEMS syndrome revealed by multiple glomeruloid angiomas. 1207 35

Infectious aneurysm is a rare event, especially after the introduction of antibiotic therapy. However, its early detection is very important for timely treatment with antibiotics and surgical intervention. This pathology may generally be due to mycotic endocarditis or septic embolization, prevailing in the preantibiotic era, and to aortitis, whose incidence is actually increasing, mainly in subjects with preexisting large-vessel atherosclerosis and intimal defects. This clinical entity is usually defined as microbial arteritis and recognizes Salmonella spp as the microorganism most frequently isolated from blood or vascular tissue cultures. The authors present the case of a 56-year-old man with a history of hypertension that some weeks before admission manifested as hyperpyrexia and episodic lumbar pain, associated with hepatosplenomegaly and with a pulsing mass in the periumbilical region. Abdominal computed tomography (CT) scan documented a voluminous infrarenal aortic aneurysm with a markedly reduced and irregular vessel wall. The patient underwent surgical excision of the aneurysm, during which marked periaortic inflammation phenomena, complete absence of the posterior aortic wall for a length of 5-6 cm, and the exposure of the correspondent vertebral bodies were observed. Histopathologic examination of the aneurysmal tissue showed atheromatous and thrombotic aspects and confirmed strong signs of inflammation. This case may suggest that the occurrence of microbial aortitis, especially from Salmonella spp, should be taken into account in the presence of a septic status associated with back, abdominal, or thoracic pain.
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PMID:Salmonella aortic aneurysm: suggestions for diagnosis and therapy based on personal experience--a case report. 1554 58


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