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Query: UMLS:C0000737 (abdominal pain)
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Cyclic hematopoiesis, (CH, or cyclic neutropenia) is a rare disease manifested by transient severe neutropenia that recurs approximately every 21 days. The hematologic profile of families with the autosomal dominant form (ADCH) has not been well characterized, and it is unknown if the phenotype is distinct from the more common sporadic congenital or acquired forms of CH. We studied nine ADCH families whose children displayed typical CH blood patterns. Pedigrees confirmed dominant inheritance without evidence of heterogeneity or decreased penetrance; three pedigrees suggested new mutations. Families were Caucasian with exception of one with a Cherokee Native American founder: A wide spectrum of symptom severity, ranging from asymptomatic to life-threatening illness, was observed within families. The phenotype changed with age. Children displayed typical neutrophil cycles with symptoms of mucosal ulceration, lymphadenopathy, and infections. Adults often had fewer and milder chronic neutropenia without distinct cycles. While CH is commonly described as "benign", four children in three of the nine families died of Clostridium or E. coli colitis, documenting the need for urgent evaluation of abdominal pain. Misdiagnosis with other neutropenias was common but can be avoided by serial blood counts in index cases. Genetic counseling requires specific histories and complete blood counts in relatives at risk to assess status regardless of symptoms, especially to determine individuals with new mutations. We propose diagnostic criteria for ADCH in affected children and adults. Recombinant human granulocyte colony-stimulating factor treatment resulted in dramatic improvement of neutropenia and morbidity. The differential diagnosis from other forms of familial neutropenia is reviewed.
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PMID:Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis. 898 58

Castelman disease (CD) is a rare lymphadenopathy that usually presents as a solitary, slow growing mass; its etiology is still unknown. The authors described a case of CD localized in the retro-pancreatic space. A 33-year old patient, female, underwent abdominal ultrasonography because of upper right abdominal pain. The ultrasound showed gallbladder stones and a mass of 4 cm in diameter behind the pancreatic head, modifying the shape of the inferior vena cava. The patient underwent surgical excision and histological examination showed a hyaline type of CD. In the 70% of the cases, the disease is located in the mediastinum, only in 14% of the cases in the retroperitoneal space. The case we report is a hyaline type of CD that is the most frequent histological type, constitutes 90% of the cases, occurs usually in the young population and after surgical excision has a very good prognosis. Different is the clinical behaviour of the plasma cell type of CD that has an aggressive and often fatal clinical outcome. The patient we treated is still free of any symptoms one year after the operation.
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PMID:[Retroperitoneal Castleman's disease. Presentation of a clinical case]. 900 77

The clinicopathological and immunohistochemical findings in 25 cases of inflammatory pseudotumor of lymph nodes (IPT) are presented. The patients were 13 women and 12 men between 8 and 81 years of age. Clinically, symptoms of prior infection, fatigue, abdominal pain, weight loss, fever of unknown origin, pelvic inflammatory disease, or nausea and night sweats were obtained in 15 patients, whereas six patients presented with asymptomatic lymphadenopathy. In four additional patients, no clinical information was obtained. The involved nodes included cervical, supraclavicular, inguinal, mesenteric, and mediastinal lymph nodes. In two cases, there was synchronous involvement of separate lymph node groups (inguinal and cervical in one case and cervical and mediastinal in another case), whereas in a third patient there was synchronous involvement of the spleen and a paraaortic lymph node. Histologically, the lesions were characterized by a fibrosing/inflammatory process that showed marked heterogeneity and striking variation from case to case. Based on their histological features, the lesions could be classified into three different groups: Stage I was characterized by the appearance of single or multiple small foci containing a spindle cell proliferation admixed with a prominent inflammatory background, with complete preservation of the remainder of the nodal architecture; stage II was characterized by more diffuse involvement of the lymph node with a marked inflammatory response admixed with a prominent myofibroblastic proliferation leading to subtotal effacement of the nodal architecture, often with extension of the process beyond the capsule into perinodal fat; and stage III was characterized by almost complete replacement of the lymph node by diffuse sclerosis with scant residual inflammatory elements and total loss of the normal nodal architecture. Immunohistochemical studies in 20 cases showed a striking number of vimentin- and actin-positive myofibroblastic cells with moderate increase in CD20/CD45+ small lymphocytes and polyclonal plasma cells in the stage I lesions, the emergence of numerous CD68+ histiocytes admixed with lymphocytes, plasma cells, and abundant fibromyofibroblastic cells in the stage II lesions, and only few remaining scattered CD68+ histiocytes and fibroblasts in the stage III lesions. Our findings suggest that inflammatory pseudotumor of lymph node represents an evolving, dynamic process that may adopt different morphological appearances depending on its stage of evolution. Recognition of the various stages of this process may be of importance for differential diagnosis with other fibrosing/inflammatory conditions of lymph nodes.
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PMID:Inflammatory pseudotumor of lymph nodes: a study of 25 cases with emphasis on morphological heterogeneity. 904 98

Enlarged anorectal lymph nodes can cause colic in young horses by obstructing the caudal aspect of the rectum. Dyschezia and clinical signs consistent with abdominal pain were the predominant reasons for evaluation of the 5 young (3 to 15 month old) horses of this report. Digital transrectal palpation revealed a firm mass obstructing the caudal aspect of the rectum in each horse. Results of cytologic evaluation of the masses revealed a lymphoid population of cells in 4 of 5 horses. These nodes regressed over time or became abscesses and drained into the rectum. In 1 horse, detection of a mature abscess and concomitant dysuria necessitated immediate surgical drainage of the mass; however, the other 4 horses were successfully managed medically, thereby avoiding risks associated with surgery of the perirectal area. Anorectal lymphadenopathy should be considered as a differential diagnosis in young horses with colic.
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PMID:Anorectal lymphadenopathy causing colic, perirectal abscesses, or both in five young horses. 907 84

The disease is named after George H. Whipple who, in 1907, was the first to describe an intestinal "lipodystrophy". Although Whipple's disease is generally recognized as a multisystem chronic granulomatous disease, primarily involving the digestive system, it can also appear as a primary neurological disorder in rare cases. Most often it is manifested with loss of weight, diarrhea, malabsorption, abdominal pain, lymphadenopathy, cardiopathy, hyperpigmentation and hypotension. The presence of periodic acid-Schiff (PAS)-positive macrophages in biopsy specimens (not only jejunal) and demonstration of "Whipple's bacilli" visible by electron microscopy, are diagnostic signs of active Whipple's disease. Whipple's disease confined to the CNS is rare. It is rarely found in the differential diagnosis of patients with progressive neurological deterioration. The most common neurological picture includes progressive dementia, external ophalmoplegia, myoclonus, seizures, ataxia, hypothalamic dysfunction (sleep disorders, hyperphagia, polydipsia) and meningitis. Oculofacial-skeletal myorhythmia as a movement disorder, associated with Whipple's disease, is reported. Fulminant course of cerebral Whipple's disease is unusual and unfavourable. The confusing and nonspecific clinical appearance is typical for primary CNS involvement. It has recently been suggested that CNS involvement occurs in all cases, although only 10-20% of patients may show it. The CNS is the most common site of disease relapse. The CT scans and MRI of the brain are often normal, but may show cortical/subcortical atrophy, hydrocephalus, focal or intracerebral mass lesions. The cerebrospinal fluid can sometimes contain PAS-positive macrophages. Brain biopsy is suggested as a diagnostic method in cases of high suspicion of CNS Whipple's disease. However, the lesions are frequently inaccessible and false negative. Without extended antibiotic therapy, the course of Whipple's disease is lethal. Now, the prognosis is good, although the optimal antimicrobial regimen is not clearly established. Initial parenteral therapy (tetracycline, penicilline, streptomycine, chloramphenicol, ampicilline) and peroral long-term treatment with trimetoprime-sulphametoxasole, are recommended. As CNS relapse of Whipple's disease may occur after several years, long-term treatment should include antibiotics that are able to cross the blood-brain barrier. The CNS relapse, in contrast to the systemic ones, is resistant to the treatment. Appropriate therapy instituted earlier in the course of the disease is associated with a better neurological outcome. Early recognition can be critical in Whipple's disease because of irreversible neurological sequelae seen later in the course of this potentially treatable condition. In cases with high clinical suspicion in which Whipple's disease cannot be diagnosed with procedures such as jejunal biopsy, antibiotic therapy is recommended. Recovery of an established neurological deficit may rarely occur. Longterm follow-up studies would help to identify the optimal antibiotic regimen and duration of treatment.
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PMID:[Neurologic disorders in Whipple's disease]. 910 28

A 57 year-old woman was seen after a three-week period of upper abdominal pain, nausea, fever, headache and exertional dyspnoea. Laboratory examination showed an elevated ESR and serum gamma-GT activity. The chest X-ray showed cardiomegaly resulting from a pericardial effusion as was demonstrated by echocardiography. An abdominal CT-scan disclosed multiple hypodense lesions in the liver and spleen and lymphadenopathy along the hepatoduodenal ligament. Liver biopsy showed a necrotising granulomatous hepatitis. A recent infection with Bartonella, presumably B. henselae, was demonstrated serologically. The patient was treated with clarithromycin and recovered.
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PMID:[Visceral granulomas and pericardial effusion caused by a Bartonella henselae infection]. 915

We describe a case of peripheral T cell lymphoma that is remarkable for its fulminate course and selective targeting of both kidneys. The patient was a 6-year-old girl who was in her usual state of good health until the onset of abdominal pain and fever. She was treated for acute oliguric renal failure and visual disturbances. A renal biopsy was performed. Biopsy findings were interpreted as suggestive of a vasculitic process, and treatment was initiated for a presumptive diagnosis of Wegener's granulomatosis. The patient died 3 days following admission, and autopsy revealed extensive bilateral kidney infiltration by a peripheral T cell lymphoma. The remainder of the body was spared with the exception of mild infiltration of the pulmonary parenchyma and choroid plexus by neoplastic lymphocytes. The neoplastic nature of the disease was confirmed utilizing immunoperoxidase stains and T cell receptor gene rearrangement. Primary renal lymphoma and renal failure attributable to involvement by lymphoma are rare findings that should be considered when other more common causes of renal insufficiency have been excluded. The presenting clinical complaints are generally of short duration, nonspecific, and atypical. Most patients exhibit oliguria. Physical examination may reveal hepatosplenomegaly, lymphadenopathy, and flank and/or abdominal mass(es). Laboratory findings frequently include an elevated serum creatinine, blood urea nitrogen, lactate dehydrogenase, and a mild proteinuria. Electrolyte abnormalities are variably present. Possible radiographic findings include hypodense or hypoechoic renal lesions and diffuse bilateral renal enlargement. Although the prognosis is dismal, survival may be prolonged utilizing current treatment modalities, and rare patients may be "cured" of disease. The clinical presentation, radiological findings, and prognosis of patients with clinically evident renal involvement by non-Hodgkin's lymphoma are discussed.
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PMID:Rapidly progressive T cell lymphoma presenting as acute renal failure: case report and review of the literature. 918 23

A survey of physicians in private practice in Jamaica was conducted between March and September 1993 to provide a descriptive analysis of the occurrence of patients with sexually transmitted diseases (STDs) seen in their practices. Questionnaires were delivered to 371 physicians of whom 127 (34%) responded, completing 683 (men 353, women 330) individual patient questionnaires. Each physician collected data over a period of one week. The median ages of the men and women were 27 years and 26 years, respectively. 464 (68%) patients were being seen for the first time for symptomatic STDs, and the visit was a follow-up for 132 (19%) who had been previously diagnosed; 40 (6%) patients were asymptomatic contacts referred by a sex partner, and 12 (2%), who were asymptomatic, asked for a 'checkup'. A history of previous STD was given by 358 (52%) patients. 470 (69%) patients had a genital discharge (M, 65%; F, 73%; p = 0.017), 52 (8%) had anogenital lesions (M, 10%; F, 5%; p = 0.013) and 45 (7%), inguinal lymphadenopathy (M, 10%; F, 3%; p = 0.002). Among women, 131 (40%) had lower abdominal pain on examination and 105 (32%) had cervical excitation tenderness or pain suggesting pelvic inflammatory disease (PID). A working diagnosis of gonorrhoea was made in 273 (40%) patients, trichomoniasis in 121 (18%), nongonococcal infection in 114 (17%), syphilis in 60 (9%), herpes genitalis in 20 (3%) and chancroid in 11 (2%). PID was diagnosed in 121 (37%) women and nongonococcal urethritis in 98 (28%) men. The most frequently prescribed treatments were for chlamydia, gonorrhoea and trichomoniasis. In general, working diagnoses correlate well with clinical observations and treatment given, matched with diagnosis according to national guidelines. A comparison of the STD burden between the public and private sectors was not possible because of sample bias.
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PMID:Survey of patients with sexually transmitted diseases seen by private physicians in Jamaica. 926 May 33

Gastrointestinal histoplasmosis complicated by intestinal obstruction and peritonitis has not been reported. We report a case of gastrointestinal histoplasmosis in a 27-year-old patient with acquired immunodeficiency syndrome (AIDS). The patient was a Chinese man from Thailand with a history of intravenous drug use and unprotected sex with female prostitutes. He was admitted for prolonged fever, abdominal pain, and diarrhea. Colonoscopy revealed volcano-like ulcers and tumors, while computed tomography of the abdomen showed a colon tumor and hypoattenuated lymphadenopathy of the retroperitoneum. Histopathologic examination as well as cultures of colon biopsy specimens and an aspirate from the retroperitoneal lymphadenopathy revealed Histoplasma capsulatum. Intestinal obstruction and peritonitis requiring surgical intervention developed, despite amphotericin B therapy. Histoplasmosis should be included in the differential diagnosis in AIDS patients who present with colon tumors, retroperitoneal lymphadenopathy, and peritonitis.
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PMID:Intestinal obstruction and peritonitis resulting from gastrointestinal histoplasmosis in an AIDS patient. 974 71

We reported a case of primary macroglobulinemia with stomach and pulmonary invasion. The patient was 71 years-old who had cervical lymphadenopathy and abdominal pain. Biopsy material of cervical lymph node showed non-Hodgkin's lymphoma, and he was diagnosed primary macroglobulinemia by IgM immunological histo-chemical staining of materials of stomach biopsies. Combination chemotherapies were not effective for the reduction of IgM-lambda protein, and organ invasion seemed to be progressive, so we tried interferon-alpha (IFN-alpha) to control M component. Daily injection of 6 megaunits of IFN-alpha induced significant reduction of M component and pulmonary invasion. This favorable changes were observed for 1 year. However, his pulmonary invasion on X-ray films relapsed and he died of respiratory failure by reason of severe pneumonia. IFN-alpha is currently available for myeloproliferative disease, especially chronic myelogenous leukemia and multiple myeloma. This case report showed that IFN-alpha was also available for primary macroglobulinemia.
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PMID:[Interferon-alpha treatment for chemotherapy-resistant primary macroglobulinemia with stomach and lung invasion]. 975 16


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