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Gallium-67-citrate scintiscanning in the search for occult primary malignant tumours.

Fifty consecutive patients in whom occult primary malignant disease was suspected underwent scintiscanning of the head, neck and trunk with gallium-67-citrate. In 17 patients a carcinoma was correctly identified as to presence and approximate location. In another seven patients the site of the primary tumour was identified from the scan and confirmed by radiologic study but no histopathological diagnosis, except for metastatic anaplastic carcinoma, was made. In another five patients the many abnormalities on the scan did not permit identification of a possible site of the primary tumour. Eleven patients had normal scintiscans and no evidence of malignant disease on follow-up. In seven patients with proven carcinoma false-negative results were obtained: no uptake of the radioisotope was detected, even at the sites of known disease. In three patients false-positive uptake of the radiotracer was observed; two had benign disease and one had a malignant tumour remote from the scan abnormality.

Adult↗

[Value of stabilization of the tarsus in certain pseudo-vascular predominantly anterolateral leg pains].

The authors have studied 18 cases of patients presenting podiatric disorders as well as a moderate varicose or lymphatic involvement. The functional symptomatology, dominated by antero-lateral leg pain, was important enough to disturb professional or leisure activities. By preferring the study of walking abnormalities (varus effect, dominant varus or equal varus-valgus instability) and the search for an elective involvement of the lateral peroneus brevis, an accurate podiatric diagnosis was made along with its ensuing therapy: adequate fitting of shoes and prescription of well adjusted ortheses which correct a dynamic hollow foot. With this treatment, the functional disorders were regularly improved or they disappeared. This result could not have been obtained by treating only the vascular impairment, which was treated conjointly in function of its own indications which do not appear to be modified by the podiatric evaluation.

Adolescent↗

Severe lower limbs lymphedema of late onset revealing polysplenia syndrome--a case report.

Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.

Abnormalities, Multiple↗

Vertebral collapse and normal peripheral blood cell count at the onset of acute lymphatic leukemia in childhood.

Acute lymphatic leukemia presenting with bone pain and spine involvement is a recognized clinicopathologic complex that can mimic a wide range of orthopaedic conditions. Bone pain as the presenting complaint is common, with a reported incidence of 27% to 50%. Radiologic abnormalities associated with leukemia in children has been described previously. In the literature, the incidence of spinal involvement is controversial, but there is agreement that the spine is less commonly involved than are the long bones. At the onset of the disease, only 10% of children have normal peripheral blood counts. If the patient has spinal involvement and a normal leukocyte count, the diagnosis is often unclear. Only three of these patients have been described in the literature; this article adds one more patient with acute lymphatic leukemia with back pain as the main symptom, vertebral collapse, and a normal peripheral blood cell count at the time of initial presentation. It illustrates that delay in diagnosis frequently occurs, with the classic features of the disease being uniformly absent.

Blood Cell Count↗

The significance of routine duodenal biopsies in pediatric patients undergoing upper intestinal endoscopy.

GOALS: To determine the significance of performing routine duodenal biopsies during upper intestinal endoscopy in a pediatric population and to evaluate their contribution to the overall diagnosis. BACKGROUND: Performing duodenal biopsy during every upper endoscopy regardless of the indication for endoscopy and the macroscopic findings, is a controversial topic. Advocates of performing routine biopsies argue that unexpected pathology such as villous atrophy, may have significant clinical implications. Opponents argue that the yield of performing a biopsy on an apparently normal mucosa is low. STUDY: Duodenal biopsies, routinely taken from 201 pediatric patients during upper endoscopy over a 26-month period were retrospectively reviewed. Duodenal biopsies taken during this period for suspected mucosal lesions were not included in the analysis. Indications for endoscopy included suspected peptic disease, gastroesophageal reflux, unexplained vomiting, abdominal pain, iron deficiency anemia and Crohn disease. RESULTS: Of the 201 sets of biopsies reviewed, 159 (79.1%) were normal, 7 had insufficient material for evaluation and 35 (17.4%) carried abnormalities that included: 10 Giardia lamblia (4.9%), 13 mild chronic inflammation (6.5%), and 8 increased intraepithelial lymphocytes (3.9%). Two biopsies showed mixed acute and chronic inflammation, 1 showed lymphatic dilatation and 1 had a mild mucosal lesion. The risk for microscopic pathology in the duodenum was higher when Helicobacter pylori was present in the gastric biopsy (25.98% vs. 12.16% P < 0.02). The negative predictive value of a normal appearing duodenal mucosa was 81.5%, implying that a normal appearing mucosa does not rule out pathology. No complications were encountered in our series. CONCLUSION: We suggest that the inclusion of routine duodenal biopsies as part of upper endoscopy in pediatric patients should be considered favorably. This practice may yield additional pathologic findings that otherwise could have been missed. It should be done regardless of the indication for endoscopy or the gross appearance of the mucosa. This practice does not increase the risk of the procedure.

Adolescent↗

Sudden hearing loss as the sole manifestation of neurosarcoidosis.

Sarcoidosis is a chronic, idiopathic granulomatous disease with frequent pulmonary, ocular, and lymphatic systemic manifestations. Central nervous system involvement is rare (1% to 5%), yet the most frequent presenting symptoms (48%) in patients with neurosarcoidosis are neurologic. Eighth cranial nerve abnormalities occur in 20% of these patients, typically in association with other cranial neuropathies or overt systemic disease. Isolated eighth nerve disease is very rare. We report two cases of neurosarcoidosis manifest as isolated, sudden onset sensorineural hearing loss, one of which had a cerebellopontine angle (CPA) granuloma that mimicked an acoustic neuroma. Both patients experienced marked steroid-induced hearing improvement that persisted after termination of therapy. Brainstem evoked response audiometry (BSER) abnormality persisted despite normalization of audiologic parameters. The implications for diagnosis and treatment of sarcoidosis-induced hearing loss are described. Neurosarcoidosis must be considered when evaluating patients with sudden hearing loss. Prompt steroid therapy offers hope for significant hearing recovery in these patients.

Adult↗

Normal and abnormal intestinal absorption by humans.

Adults eating a Western diet digest and absorb ingested food containing approximately 100 g fat, 350 g carbohydrate, and 75 g protein daily. Normal fat absorption requires adequate gastric, pancreatic, liver-biliary, mucosal, and lymphatic function. Carbohydrate and protein absorption is much less dependent on liver-biliary and lymphatic function. The intestine has a large reserve capacity for digestion and absorption of nutrients which is due to both excess function and to adaptive changes which increase function in one segment of the digestive-absorptive system when it is decreased or lost in another segment. The large reserve capacity explains why most of the prevalent intestinal diseases seldom cause clinically detectable changes in absorption. However, there are more than 30 less-common human diseases which cause malabsorption of one or more nutrients. Those that cause the malabsorption syndrome, i.e., steatorrhea and weight loss, can be conveniently categorized according to the major deficiency leading to the absorptive defect as follows: insufficient pancreatic enzyme activity, insufficient bile acid, disease of the small intestinal wall, multiple defects, mechanism unknown, and drug-induced malabsorption. A few diseases, most of which are congenital, cause malabsorption of only one or a few related nutrients such as lactose malabsorption in lactase deficiency. Most of the tests currently in use for detecting and diagnosing the cause of malabsorption are relatively insensitive and nonspecific. Chemical analysis of the fat in a three-day stool collection remains the single best test for diagnosing the malabsorption syndrome. However, a breath test using Triolein labeled with either the radioactive or stable isotope of carbon may be an important recent advance. Other breath tests are also currently being investigated for quantitating absorption or malabsorption of various substances including bile acids and various sugars. Studies of the function of the intestinal epithelial cells are usually best accomplished using tissue obtained by per oral biopsy. Biopsy specimens are used for many types of study including light and electron microscopic examination, chemical and enzymatic assays, tissue culture, and uptake of various radiolabeled compounds.

Bile Acids and Salts↗

Subcutaneous and intravenous delivery of diagnostic agents to the lymphatic system: applications in lymphoscintigraphy and indirect lymphography.

Lymph node status is important in the staging of many malignancies. Although tissue characterization by histologic analysis of biopsy samples may improve staging, noninvasive staging is more acceptable to both patients and clinicians. Several imaging techniques may serve this goal. Modern noninvasive techniques such as computed tomography and magnetic resonance detect lymph node abnormality by nodal enlargement, but that does not always imply malignant involvement. On the other hand, many nodes are infiltrated or replaced by tumour without change in size. This becomes a serious diagnostic defect by these modalities. Consequently, attention has been focused to develop contrast agents and radiolabelled complexes for better cancer detection as well as characterization of individual tumours in lymph nodes. For delivery of such materials to regional lymph nodes one can take advantage from the distinct physiological function of the lymphatic capillaries. The thin-walled and fenestrated lymphatic microvessel is easily penetrated by particulate and macromolecular agents after injection into the extracellular space. Once inside the vessel, materials that are transported with the lymph either specifically target certain nodal elements (e.g. neoplastic cells) or become cleared by macrophages located in the lymph nodes. Indeed, interstitial delivery of diagnostic agents have been of benefit in determining regional spread of cancer and assessing lymphatic function either by lymphoscintigraphy or indirect lymphography. On the other hand, development of contrast materials that can reach lymph nodes after a single intravenous injection is highly desirable because of the large number of lymph nodes in the body and access being difficult to most of them. Today, a number of contrast agents exist that can reach a vast array of lymph nodes in the body, particularly those that are not readily accessible for histologic evaluation, after a single intravascular injection to help distinguish between normal and tumour-bearing nodes or reactive and metastatic nodes with magnetic resonance. In this article we critically examine advantages and limitations of both subcutaneous and intravenous routes of injection for the delivery of diagnostic agents to the lymphatic system.

Journal Article↗

Thoracic duct-cutaneous fistula in a patient with cirrhosis of the liver: successful treatment with a transjugular intrahepatic portosystemic shunt.

Patients with cirrhosis of the liver have increased hepatic and gastrointestinal lymph flow that may contribute to the formation of ascites and pleural effusions. Increased lymph flow, which is due to postsinusoidal portal hypertension, causes a high rate of flow through the thoracic duct. Because of the high flow rates, disrupted lymphatic vessels in patients with cirrhosis of the liver may fail to close, a situation that results in chylous ascites, pleural effusions, or chylous fistulas. Chylous fistulas deplete proteins, fluid, and lymphocytes and thus lead to volume depletion and coagulopathy. Herein we describe an unusual case in which a high-output traumatic thoracic duct-cutaneous fistula developed in a patient with cirrhosis and led to volume depletion and coagulopathy. Correction of the portal hypertension with placement of a transjugular intrahepatic portosystemic shunt led to closure of the fistula and normalization of accompanying metabolic abnormalities.

Blood Coagulation Disorders↗

Primary adenocarcinoma of the gallbladder presenting as primary gynecologic malignancy: a report of two cases.

BACKGROUND: Carcinoma metastatic to the uterus from extragenital sites is rare. Such metastatic disease is typically diagnosed at autopsy or in patients with known primary malignancies. This report discusses two cases of primary carcinoma of the gallbladder presenting as abnormalities in gynecologic screening procedures. CASES: A 71-year-old woman presented with postmenopausal bleeding. Uterine curettage revealed poorly differentiated adenocarcinoma of presumed endometrial origin. Intraoperative frozen-section analysis of the uterus showed carcinoma involving the lymphatics, but no primary tumor. Further exploration revealed primary adenocarcinoma of the gallbladder, with widespread metastases. The second case was a 67-year-old asymptomatic woman. Routine cervical cytology showed adenocarcinoma, but tissue studies were negative. She developed jaundice 1 month later. Computed tomography of the upper abdomen revealed a mass in the gallbladder fossa, and needle biopsy of the lesion showed adenocarcinoma. CONCLUSIONS: Metastatic carcinoma of non-genital tract origin may present as primary gynecologic malignancy. The physician should be aware of the implications of both the common and unusual interpretations of screening and diagnostic procedures. When the clinicopathologic presentation is atypical, a thorough knowledge of the differential diagnoses of abnormal test results allows appropriate and expeditious patient management.

Adenocarcinoma↗

Expression of interleukin-1 beta, interleukin-6 and tumour necrosis factor alpha by macrophages in canine lymph nodes with mineral-associated lymphadenopathy, granulomatous lymphadenitis or reactive hyperplasia.

The distribution of CD3+ T lymphocytes and the expression of interleukin-1 beta (IL-1 beta), interleukin-6 (IL-6) and tumour necrosis factor alpha (TNF alpha) by macrophages in canine lymph nodes with mineral-associated lymph-adenopathy, granulomatous lymphadenitis or reactive hyperplasia was examined immunohistochemically. In areas of preserved lymph node architecture, CD3+ T lymphocytes were located in the paracortex, with scattered cells in the follicular mantle and germinal centre, and small aggregates in the medullary cords. T cells were prominent within areas of diffuse granulomatous inflammation and formed a peripheral rim around microgranulomas. TNF alpha expression was demonstrated in macrophages from seven of 11 dogs with mineral-associated lymphadenopathy, one of five dogs with granulomatous lymphadenitis and three of five with reactive hyperplasia. Positive macrophage staining for IL-1 beta and IL-6 was seen in two lymph nodes with reactive hyperplasia. Lymph nodes from control (clinically normal) dogs showed no evidence of cytokine expression. Neutrophils expressing IL-1 beta were observed within the medullary sinuses of two lymph nodes with mineral-associated lymphadenopathy and one with reactive hyperplasia, and in the inflammatory infiltrate in one lymph node with granulomatous lymph-adenitis. The sensitivity of immunohistochemical examination as a means of detecting cytokines in abnormal tissue is discussed.

Animals↗

Pagetoid reticulosis: a disease of histiocytic origin.

A patient with Pagetoid reticulosis has been investigated using standard transmission electron microscopy and an immunohistochemical technique for identifying T lymphocytes in situ in light and electron microscopy. Our results indicate that the majority of the cells in the underlying dermal infiltrate are T lymphocytes but that only very few T lymphocytes are found in the extensive epidermal infiltrate. Ultrastructurally the majority of the abnormal cells in the epidermis are of histiocytic origin.

Histiocytes↗

Kawasaki syndrome (the mucocutaneous lymph node syndrome).

Kawasaki syndrome is a newly-recognized clinical entity characterized by multisystem involvement. It has an acute onset and triphasic clinical course. Although essentially a self-limiting disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically, the disease is characterized by widespread vasculitis. There is a monomodal age distribution, with peak occurrence during the first two years of life and few affected over the age of 8 years. Males outnumber females 1.5:1; persons of Japanese extraction are overrepresented compared with other races, caucasians underrepresented. Community-wide epidemics have been reported from diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy is supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.

Age Factors↗