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[A case of Crow-Fukase syndrome with extramedullary plasmacytoma: marked clinical deterioration following a biopsy to plasmacytoma].

A 66-year-old man developed paresthesia of the distal parts of the bilateral lower limbs a week after his upper respiratory infection, followed by the weakness with the legs and paresthesia with the lip area, tongue and finger tips. Those symptoms gradually became worse to the point that he was unable to walk 10 days later. Although skin pigmentation, edema, and lymph node swelling were not found, we made a diagnosis of Crow-Fukase syndrome (CFS) because of clinical features of polyneuropathy, IgG-lambda type M proteinemia, endocrinological abnormality, elevated plasma level of vascular endothelial growth factor (VEGF) and extramedullary plasmacytoma in his abdomen. Following intravenous immunoglobulin therapy (IVIg), he showed marked improvement. However, his neurologic symptoms deteriorated acutely just after open biopsy together with the elevation of VEGF level, and a few days later he was in the state of flaccid quadriparesis. We tried IVIg therapy again and his neurologic symptoms were markedly improved. We speculated that an elevated VEGF, released from plasma cells induced by the bioprocedure, might have caused an increase in microvascular permeability and affected the blood-nerve-barrier, thereby his neurologic symptoms deteriorated. It is thought that this case may support the hypothesis that a significant role is played by VEGF in the pathomechanism of the development of CFS. Additionally we experienced that IVIg was very effective to the neurologic symptoms, and we think that IVIg will be able to be one of the future therapy of the CFS. To our knowledge, there has been no report of CFS which manifested acute deterioration of his neurologic symptoms just after open biopsy with acute onset with Guillain-Barré syndrome like symptoms.

Aged↗

[Orbicularis oculi muscle and crow's feet. Pathogenesis and surgical approach].

Crow's feet is one of the characteristic signs of the aging face. Minor techniques designed to obliterate these lesions rapidly demonstrate their limits. The orbital fibers of the orbicularis oculi muscle play a fundamental role in the pathogenesis of wrinkles. Their contracture will create wrinkles in a similar way to the spokes of a wheel perpendicularly to the orientation of the muscle fibers. The dynamic action of the zygomatic muscles contributes to exaggerate these wrinkles. Statically, ptosis of the lateral brow and the aging skin also contributes to these lesions. Surgical treatment can be accomplished by a temporal lift or via a blepharoplasty approach. The vertical fibers of the orbicularis oculi muscle must be corrected by muscle incision or resection, muscle incision and suspension, muscle redraping and fixation, covering of the orbicularis oculi muscle with the malar SMAS. In patients with ptosis of the tail of the eyebrow one must either do a forehead lift or a mask-lift that will redrape the skin. The surface of the skin may require peeling by dermabrasion. Of course, these surgical techniques require a detailed knowledge of the anatomy of this region and especially the distribution of the branches of the facial nerve. The author analyses and compares the results of the different techniques.

Esthetics↗

[A case of Crow-Fukase syndrome showing improvement following excision and irradiation of bone lesions].

A 57-year-old woman suffering from pleural and pericardial effusion, pulmonary hypertention, lymphadenopathy, hepatosplenomegaly, edema, hypertrichosis, small hemangioma and polyneuropathy was diagnosed as Crow-Fukase syndrome. Osteoctomy of the left second rib and irradiation of this rib and the left iliac bone were performed. Serum vascular endothelial growth factor (VEGF) level decreased to less than one-half the level before the operation (from 5,180 to 2,150 pg/ml). Immediately after the operation, pleural and pericardial effusions due to hyperpenetration improved, and polyneuropathy and hypertrichosis due to hypervasularity also gradually improved. The resected lesion was histopathologically found to be of a plasmacytoma of the IgG lambda type. Since the level of VEGF in the tissue specimen was much lower (116 pg/ml) than that in the serum, VEGF could not have been produced by the plasmacytoma.

Bone Neoplasms↗

[Five cases of Crow-Fukase syndrome].

We presented five cases of Crow-Fukase syndrome. Plasma cell hyperplasia or dyscrasia in bone marrow were recognized in all cases and localized bone lesion was seen in three cases. Thyroid dysfunction was seen in three cases; hyperthyroidism in one case and hypothyroidism in two cases, which was considered to be one of the characteristics though it has seldom been described in this disease. Two of four cases treated with prednisolone had good responses but two cases treated with interferon had no effect.

Adult↗

[Crow-Fukase syndrome. A report of 2 cases].

Two cases of crow-fukase syndrome were reported. Both cases showed the characteristic polyneuropathy, organomegaly, endocrinologic disturbances and skin involvement. There was no evident response to cortico--steroid therapy.

Adult↗

[A case of Crow-Fukase syndrome associated with idiopathic thrombocytopenic purpura].

A 40-year-old man was admitted to our hospital because of paresthesia and weakness of the limbs. At the age of 38, he was diagnosed as having an idiopathic thrombocytopenic purpura (ITP) which have been refractory to oral administration of prednisolone and splenectomy. Platelet-associated IgG was elevated markedly at that time. It was, however, only mildly elevated on this admission. He showed polyneuritis, generalized pigmentation, hirsutism, and marked edema on the legs. The bone X-ray disclosed a lytic lesion in the left iliac bone, which was confirmed as a plasmacytoma by bone biopsy. Axonal degeneration with marked loss of myelinated figure was seen on sural nerve biopsy. Serum immunoelectrophoresis revealed his monoclonal IgG was lambda type. Then, he was diagnosed as having a Crow-Fukase syndrome associated with ITP. Plasma exchange, pulse therapy, and irradiation to plasmacytoma resulted in a slight improvement of the polyneuritis and the skin symptoms, and a disappearance of edema. However, ITP has not responded to these therapies. Although the same autoimmune mechanism is suggested in these conditions, we could not clarify how this monoclonal IgG produce both polyneuritis and ITP.

Adult↗

[Crow-Fukase syndrome (POEMS syndrome). The first Italian presentation of a case and review of the literature].

A 42-year-old Italian man affected with a multisystemic disease is presented. The main features were: polyneuropathy, organomegaly (liver, spleen and lymph nodes enlargement), endocrinopathy (loss of libido, low plasmatic levels of testosterone), monoclonal protein (k-light chains only in 100-fold-concentrated urine sample, without other signs of plasmocytic proliferation), skin changes. Clinical skin alterations were striking: diffuse thickening, hyperpigmentation, hyperhidrosis , hypertrichosis, while histo- and immunopathological examination of skin biopsies showed proliferation of dermal collagen fibers and deposition of melanin in the epidermis. These findings were suggestive for the diagnosis of Crow-Fukase (POEMS) syndrome, associated with peculiar angiofollicular lymph node hyperplasia (Castleman's disease). The patient was followed up for 15 months with steroids, systemic chemotherapy, plasma-exchange and immunomodulating drugs. A massive anasarca complicated the picture leading him to death. Actually the pathomechanisms of this rare disease have not been fully elucidated. The relations between this syndrome and some malignant lymphoproliferative diseases (i.e. osteosclerotic myeloma) are controversial; at least a part of its features could be either reactive or tissue-specific-antibody mediated. A genetic influence should be suggested from the Japanese reports.

Adult↗

[Juvenile Crow-Fukase syndrome with response to bolus of methylprednisolone after failure of treatment by plasma exchange].

A 20-year-old woman was hospitalized because of abdominal distention. She had developed facial edema about one year earlier, and recently amenorrhea and red verrucae on the chest and abdomen. Neurological examination disclosed hypesthesia, paresthesia, and diminished tendon reflexes in the arms and legs. The level of serum immunoglobulin A (IgA) was elevated and an M protein was detected. Examination of the bone marrow disclosed abnormal increase in plasma cells. Results of glucose tolerance test were mildly abnormal. The patient was diagnosed as Crow-Fukase syndrome. Plasma exchange was done four times and melphalan was given orally for two weeks, but the level of serum IgA increased further. Then one bolus injection of methylprednisolone decreased the serum IgA with improvement in other signs. The disorder is now controlled satisfactory with a low dose of prednisolone.

Adult↗

[Crow-Fukase syndrome--the first case report in Bulgaria].

A case of a 53 years old woman with Crow-Fukase's syndrome, the first case diagnosed and described in Bulgaria, is presented. The patient presented with polyneuropathy (predominantly locomotor, better expressed in the legs and less expressed in the hands, with hyperproteinorachia), anasarca (peripherial edema, ascites, hydrothorax), skin changes (hyperpigmentations), endocrinopathy (transitory carbohydrate intolerance), dysglobulinemia, organomegaly (hepatomegaly).

Bulgaria↗

[Heritability of fertility in human populations and the structure of Crow's index].

Heritability of fertility was determined in populations with natural character of reproduction (Middle Asia Republics) and in populations with family planning (Middle Russia). The material was collected by interviewing the married women of postreproductive age. The heritability of fertility was estimated as a doubled coefficient of correlations between sisters for effective fertility. In populations with natural character of reproduction the heritability was about 50% and it did not differ from O in the populations with family planning. The values of heritability were used in the components of the Crow index analysis. The main contribution into broad-sense heritability of fertility comes from additive component, as shown in an Asia population (Turkmen). The values of heritability of fecundability (86%) and of postpartum sterility (66%) have been calculated for the same population.

Asia, Central↗

The skin changes in the Crow-Fukase (POEMS) syndrome. A case report.

A man with a six-year history of intermittent burning sensations in his legs, hypothyroidism, and peripheral neuropathy also had hypertrichosis, hyperpigmentation, Terry nails, and taut thickened skin of the extremities. Discovery of a plasma cell tumor led to recognition of the Crow-Fukase syndrome, and appropriate treatment with melphalan and prednisone.

Aged↗

[Crow-Fukase syndrome (POEMS syndrome) and osseous mastocytosis secondary to Castleman's angiofollicular lymphoid hyperplasia].

In a case of Crow-Fukase (POEMS) syndrome there was a chronic, progressive, and eventually lethal polyradiculoneuropathy. In addition, adenomegaly, oedema and pleural effusions, gonadic atrophy, serum monoclonal IgA, and skin pigmentation were present. Plain x-rays and CT scan of the pelvis and lower vertebrae showed multiple poorly defined lesions. At postmortem there was no myeloma and a bone mastocytosis was found. In addition, next to T11, there was an abdominal nodule, 2 cm in diameter, with histological characteristics of Castleman's angiofollicular lymphoid hyperplasia. Immunohistochemical studies showed that plasmocytes of this lesion secreted polyclonal immunoglobulins with a high prevalence of IgA. Thus, the primary interest of this case lies in the association of bone mastocytosis with a POEMS syndrome. Also, one single localisation of Castleman angiofollicular lymphoid hyperplasia was found, assumed to be in this case the cause of the POEMS syndrome. Therefore, a minute and benign hyperplasic lesion, which was only discovered at autopsy, secreted the protein responsible for the symptoms and signs, and eventually the patient's death.

Aged↗

Morphological and histochemical observations on the ovarian surface epithelium during the reproductive cycle of crow (Corvus splendens) and myna (Acridotheres tristis).

A morphological and histochemical study has been made of ovarian surface epithelium during the sexual cycle of seasonally breeding birds: crow (Corvus splendens) and common myna (Acridotheres tristis). The surface epithelium is composed of a single layer of compactly arranged columnar and flat cells in the quiescent ovary. It develops numerous villi during the breeding season. The formation of villi has been correlated with the proliferation of cells which are subsequently incorporated into the ovarian stroma where they appear to form follicle and thecal cells around the growing oocytes as evidenced by the close similarities in the morphological and histochemical characteristics of these cell types. As the ovarian activity increases, the surface epithelial cells show increasing amounts of RNA and proteins, which are indicative of their rapid multiplication. No lipids and enzyme activities of acid and alkaline phosphatases, ATPase. DPN- and TPN- diaphorases and delta5-3beta HSDH have been detected in the surface epithelium of both quiescent and active ovaries.

Animals↗

Crow-Fukase syndrome: a case associated with vasospastic angina.

The patient is a 60-year-old man who developed numbness of the extremities, paralysis, hyperpigmentation of the skin, hypertrichosis, anasarca and chest pain at the age of 58 years. The diagnosis of Crow-Fukase syndrome was made and myeloma was not found. Prednisolone therapy was effective but chest pain reappeared every morning when prednisolone was tapered to 30 mg alternate day. Coronary arteriogram showed no stenosis but administration of acetylcholine into the coronary artery produced ST elevation in electrocardiogram, chest pain and coronary artery stenosis which were relieved by administration of nitrates into the coronary artery.

Acetylcholine↗

[A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis].

Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with membranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (lambda). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/dl and creatinine 0.8 mg/dl. Light microscopic examinations showed enlargement of glomeruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposition and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.

Adult↗

Surgical correction of the crow's feet deformity.

Improved methods and important adjuncts for rejuvenation of the aging face have evolved as surgeons have recognized the limitations of the techniques they employ. Presented here is a simple, safe, and straightforward technique for correction of the "crow's feet" deformity that has proven to produce a predictable and long-lasting improvement.

Female↗