Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002453 (amenorrhea)
 6,245 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reviewed a series of 109 reported Japanese cases of "plasma cell dyscrasia with polyneuropathy and endocrine disorder." This syndrome shows 1) polyneuropathy with increased protein level in the cerebrospinal fluid and sometimes papilledema, 2) endocrinological symptoms, including skin pigmentation, sclerosis, hypertrichosis, gynecomastia, impotence, amenorrhea, decreased glucose tolerance, edema, pleural effusion and ascites, 3) hepatomegaly, splenomegaly and lymphadenopathy, 4) polycythemia, leukocytosis and thrombocytosis, 5) osteosclerotic changes and 6) plasma cell dyscrasia. Plasma cell dyscrasia is considered to be the cardinal change in this syndrome. Most of the patients have low levels of IgG lambda or IgA lambda M-protein in the serum and a slightly increased number of plasma cells in the bone marrow. The clinical course is usually chronic. Surgical excision or irradiation of the local lesion and administration of corticosteroids and/or anti-cancer drugs are effective in improving polyneuropathy and other systemic symptoms. This syndrome is apparently more common in Japan than in the United States and European countries. The pathogenesis of the association of a variety of symptoms in this syndrome is still unclear.
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PMID:Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. 631 93

The authors examined 200 consecutive patients with pituitary adenomas admitted to the neuroendocrine service at the Montreal General Hospital between 1976 and 1981. The main presenting signs and symptoms were amenorrhea/impotence (70%), headache (46%), and typical acromegalic or cushingoid features (28%). Only 9% had visual field defects, 2% had optic atrophy, and 1% had ocular motility problems. A comparison of our findings with four previous studies has demonstrated an increasing incidence of reproductive system abnormalities and a decreasing incidence of visual abnormalities in patients with pituitary tumor. The reasons for this changing pattern are discussed and the role of the ophthalmologist in the care of these patients is redefined.
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PMID:Pituitary tumors and the ophthalmologist. 666 64

Twelve patients with pituitary tumor whose prior treatment included surgery and radiotherapy in four, surgery alone in four, radiotherapy alone in one, and none in three were studied. Nine had hyperprolactinemia, two had elevated serum growth hormones, and one had no pituitary hormone excess. Visual field defects were present in six. All had pituitary-gonadal insufficiency manifested as impotence or amenorrhea. All were tested with bromocriptine, 7.5 to 25 mg daily, and followed up for eight to 27 (mean 15) months. Serum prolactin levels decreased to normal in seven of nine patients. Serum growth hormone values were normalized in both acromegalics. When hormone levels were reduced to normal, pituitary tumor size decreased. Vision was restored to normal in five of six patients, including one patient with pituitary tumor but no pituitary hormone excess. Bromocriptine corrects the physiological defects associated with pituitary tumors that have been incompletely treated with surgery, radiotherapy, or both and may be a useful primary treatment for patients with pituitary tumors.
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PMID:Bromocriptine reduces pituitary tumor size and hypersection. Requiem for pituitary surgery? 705 32

One hundred and seventy patients with endocrine abnormalities, 29 males and 141 females, were studied by computerized tomography (CT) scanning of the pituitary and by pituitary hormone assays including basal and stimulated hormone concentrations in the blood. Pituitary macroadenoma was observed in 40 of the 170 patients, microadenoma in 49, primary empty sella in 46, secondary empty sella in 25, including 20 of the 40 patients with macroadenoma after surgical removal or radiation therapy of the tumor. Suprasellar tumor was present in four patients. In 23 women with menstrual abnormalities and 3 men with impotence and hyperprolactinemia, the results of CT scanning were normal. Of the 40 patients with macroadenoma, 24 presented with a hyperfunctioning syndrome (acromegaly or hyperprolactinemia). Among the 49 patients with microadenomas, 46 had the amenorrhea-galactorrhea syndrome, one male presented with acromegaly and one with Cushing's disease, and one female also had Cushing's disease. Primary empty sella was associated with the amenorrhea-galactorrhea syndrome in females and impotence in males. Only four female patients demonstrating an empty sella were asymptomatic. Distinctive biochemical findings were identified in certain groups of patients with different pituitary pathologic features. CT scanning was found very useful in the assessment of the size of the tumor, its suprasellar or parasellar extension and response to therapy, and in differential diagnosis of tumor and empty sella. Disappointing results were obtained in patients with endocrine abnormalities and normal results of CT scanning, and in patients following surgical treatment for a pituitary tumor, when the identification of recurrent tumor was impossible from a single examination. It is concluded that CT scanning is the most advanced technique in the diagnosis of anatomic pituitary abnormalities and it should replace the traditional invasive diagnostic procedures.
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PMID:Diagnosis of pituitary tumors by hormone assays and computerized tomography. 709 Nov 57

Prolactin-producing pituitary adenomas known to cause amenorrhea and galactorrhea in the female have been implicated recently in male infertility and impotence. Knowledge of the pathophysiology of prolactin and of the choices of treatment for these tumors is an important adjunct to the urologist's armamentarium.
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PMID:Prolactin--added dimension in male genitosexual disorders. 735 44

Over 15 yr, 24 patients underwent transsphenoidal pituitary surgery for Cushing's disease with a median follow-up of 12 months. Cures included 7 patients with normal sella turcicas (6 microadenomas), 6 patients with focal depressions (6 microadenomas) of the sella (grade I), and 3 patients (3 adenomas) with enlarged sellas (grade II). Three patients with sella destruction (grades III and IV), 2 with normal sellas, and 1 with focal sella depression (grade I) were not cured. Two apparent cures (microadenomas) recurred. Tumor histology revealed 19 basophilic adenomas; electron microscopy (14 tumors) and immunochemical studies (10 tumors) revealed only ACTH cells. Circadian rhythm returned in 6 cured patients. Impotence (in 2), amenorrhea (in 7), and galactorrhea (in 3) resolved in affected cured patients. The major surgical complication was hemorrhage at the operative site (3 patients). Transsphenoidal pituitary surgery is a valuable method for managing Cushing's disease in many patients.
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PMID:Cushing's disease: management by transsphenoidal pituitary microsurgery. 735 19

The ability to measure serum prolactin levels has led to the characterization of hyperprolactinemia as a clinical entity. In women galactorrhea and amenorrhea are symptoms of elevated prolactin levels; in men, impotence seems to be a clinical correlate. In the differential diagnosis, concern about the presence of a pituitary adenoma is preeminent. Management of microadenomas is controversial; both active therapy and conservative follow-up have been advocated. For larger lesions, neurosurgery or radiation becomes necessary. Drug therapy to reduce prolactin levels is now available.
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PMID:Hyperprolactinemia. 746 1

There is now substantial evidence that coeliac sprue is associated with infertility both in men and women. In women it can also lead to delayed menarche, amenorrhoea, early menopause, recurrent abortions, and a reduced pregnancy rate. In men it can cause hypogonadism, immature secondary sex characteristics and reduce semen quality. The real mechanism by which coeliac sprue produces these changes is unclear, but factors such as malnutrition, iron, folate and zinc deficiencies have all been implicated. In addition in men gonadal dysfunction is believed to be due to reduced conversion of testosterone to dihydrotestosterone caused by low levels of 5 alpha-reductase in coeliac sprue. This leads to derangement of the hypothalamic-pituitary axis. Hyperprolactinaemia is seen in 25% of coeliac patients, which causes impotence and loss of libido. Gluten withdrawal and correction of deficient dietary elements can lead to a return of fertility both in men and women.
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PMID:Infertility, obstetric and gynaecological problems in coeliac sprue. 798 65

POEMS syndrome has been defined as an association of plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. Although certain authors do not distinguish this syndrome from osteosclerosing myeloma, syndromes comparable to POEMS have been observed without bone lesions. Others have described the clinical features involved under the terms of Crow-Fukase's syndrome, PEP syndrome (pigmentation, oedema, plasma cell dyscrasia, or Takatsuki's syndrome. Seen in men twice as often as in women, usually between the ages of 40-50 years, all five clinical features are not always present or may be accompanied by other signs. The first sign of the peripheral polyneuropathy is usually sensorial impairment followed by distal then proximal motor deficit. The deficit is usually severe and 50% of the patients become unable to walk. Cranial nerves are rarely involved. Liver, spleen and lymph node enlargement are observed. The most frequent signs of an endocrinopathy are gynaecomastia, atrophy of the testicules, impotence and amenorrhoea. Testosterone levels are low and oestrogen levels are increased in men together with luteinizing hormone, follicle stimulating hormone and prolactin. Hypothyroidism is frequent and diabetes mellitus is observed in 50% of the patients. Protein M is a monoclonal immunoglobulin (IgG or IgA), almost always with a light lambda chain. Skin changes include hyperpigmentation, hypertrichosis, hyperhidrosis, thickening of the skin suggestive of sclerodermia and papillary angiomas. Other signs, especially peripheral oedema often occur early in the disease course and may precede the peripheral neuropathy. POEMS syndrome is often associated with a myeloma (up to 50% of the cases in certain series). Although immunoglobulin deposit on myelin sheaths, anti-endocrine antibodies and receptors of lambda chains have been proposed as playing a role, no mechanism of pathogenesis has been determined. The natural history of the disease leads to a severe polyneuropathy. The patients become totally bedridden and death results from complications of decubitus rather from the direct effect of the underlying dyscrasia. When bone lesions are minor, radiotherapy or surgery can improve the neuropathy and resection of a solitary plasmocytoma can lead to total remission. Chemotherapy or corticosteroids may improve the polyneuropathy in certain cases. Plasma exchange has not been successful.
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PMID:[POEMS syndrome]. 807 60

A case-control study was carried out in the Congo to define a scoring system based on a number of clinical and epidemiological criteria of African trypanosomiasis due to Trypanosoma brucei gambiense which could be used by peripheral health services to establish a diagnosis. The survey comprised 163 cases and 326 controls. Clinical signs and symptoms were fever, headache, pruritus and skin lesions due to scratching, diarrhoea, oedema, cervical adenopathies, sleep rhythm disturbances, changes in appetite, amenorrhoea or impotence, mental confusion, neurological signs, and other minor clinical disturbances. Other criteria were a history of previous trypanosomiasis and the presence of domestic animals in the home environment. Analysis of the results showed that neither a single criterion nor a group of criteria is pathognomonic for the disease. The selected criteria do not allow discrimination of sleeping sickness patients among suspected individuals who present themselves. A scoring system is therefore of little use at the peripheral level of health services, particularly when considering the additional workload involved. The low diagnostic value of these clinical signs and symptoms and other indicators in African trypanosomiasis stresses the difficulty in developing an early warning tool for an integrated control strategy in primary health care.
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PMID:[African human trypanosomiasis: study of a scoring system of presumptive diagnosis in the Congo]. 849 Sep 85


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