Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of symptomatic Rathkes' cleft cyst are described. Case 1. A 51-year-old man was admitted to the hospital for evaluation of intermittent headache, in April, 1985. He was neurologically free, but skull films disclosed a ballooning of the sella with thinning of the dorsum sellae. CT scan showed enlargement of the sella, but no abnormal density area in the sellar region. MRI revealed a round mass with high signal intensity located just posteriorly to the pituitary body. By a transsphenoidal approach, a thin-walled cyst was found at the posterior portion of the sella. Turbid mucinous fluid and the capsule of the cyst were subtotally removed. Histologic section of the specimen demonstrated that its wall was composed of a loose fibrous tissue lined by a single layer of ciliated cuboidal epithelium containing some goblet cells. Electron microscopy showed ciliated cuboidal cells, cells with microvilli, cells with light and large granules, and basal cells abutting on the basal lamina. Case 2. A 33-year-old female was referred to the hospital with complaints of severe headache and decreased visual acuity 0.02 in the left and 0.1 in the right, in July, 1985. Visual field examination revealed bitemporal hemianopsia. She had amenorrhea, galactorrhea and polyuria. CT scan and metrizamide CT cisternography detected a low density mass in the suprasellar region. Endocrinological studies disclosed hyperprolactinemia with partial hypopituitarism. She had a right frontal craniotomy and a suprasellar cyst was subtotally removed. Histologically, a cystic wall was lined by pseudostratified columnar epithelium supported by a loose fibrous tissue.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Rathke's cleft cyst--report of three cases]. 361 39

Two young siblings had a syndrome of growth retardation, severe rickets, anemia, renal insufficiency, and renal tubular dysfunction, the last including acidosis, aminoaciduria, and polyuria. There was moderate psychomotor developmental delay. Neither child had cystinosis. Renal biopsy in the older child revealed severe glomerular abnormalities, with capillary wall thickening reminiscent of the hemolytic-uremic syndrome. The proximal convoluted tubules were lined with short, cuboidal cells containing mildly abnormal mitochondria. There was also thinning of brush border microvilli and basolateral infoldings, perhaps as the result of regressive changes, and interstitial fibrous tissue was moderately increased. The etiology of the tubular and glomerular changes is uncertain. We believe these patients represent a previously unreported hereditary syndrome sharing certain clinical features with severe nephropathic cystinosis.
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PMID:A familial syndrome of growth retardation, severe Fanconi-type renal disease and glomerular changes--a new entity? 372 23

Kidneys from 7 mutant Southdown sheep with congenital hyperbilirubinemia, aged from 1 to 5 years were examined. Renal biopsies were taken from another mutant at 3 months and 12 months of age. At 3 months, lesions consisted of thin radial bands of myxomatous tissue in the medullary rays and atrophy of the adjacent collecting tubules. By 1 year collagen had replaced myxomatous tissue. Grossly, the kidneys were normal until 2 years when they became red and gray mottled and stained with bilirubin. The capsules stripped readily to reveal fine granular surfaces in sheep over 2 years of age. On the cut surface of the cortex were 0.5 mm wide radial gray streaks of fibrous tissue. Progressive fibrosis in sheep 2 to 5 years old resulted in a thinning of the cortex. With increasing fibrosis, the number of cystic tubules increased progressively. Protein casts and hyaline droplet degeneration were numerous in sheep over 2 years of age. Plasma cells and lymphocytes were frequently seen in the fibrous bands, and bile pigment was visible in the macrophages in the fibrous tissue and in the epithelium of the proximal tubule cells. Polyuria, low specific gravity urine and reduced effective renal plasma flow and glomerular filtration rates resulted from the replacement of specialized proximal tubule cells by low cuboidal cells, fibrous tissue separating the capillaries from the loops of Henle, destruction of glomeruli and segregation of glomeruli in fibrous bands. The kidney lesions may be determined by the same gene responsible for the hepatic excretion defect for bilirubin.
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PMID:Renal radial fibrosis in mutant Southdown sheep with congenital hyperbilirubinemia. 503 62

The present study was undertaken to devise an electrophysiological method for detecting diabetic retinopathy in rats. The electroretinogram (ERG) and visual evoked potential (VEP) were recorded from unanesthetized and unrestrained rats rendered diabetic with a single i.v. injection of streptozotocin (STZ) at 35 or 40 mg/kg. The STZ-treated rats showed signs of diabetes: hyperglycemia, glucosuria, hypoinsulinemia, polyuria and increased water intake. Amplitudes of the ERG a- and b-waves and oscillatory potentials (OPs) on the b-wave were decreased and latencies of these waves were prolonged gradually after STZ was administered. Especially, latencies of the OPs became significantly different from the pre-treatment values. Latency of the VEP N1 wave showed a slight prolongation, which might be secondary to the depression of retinal function. Histological examination showed swelling and proliferation of the lens epithelium and swelling and vacuolization of the lens fiber were observed in the eyeball 9 weeks after STZ-treatment. Moreover, thinning of each retinal layer was observed in a few rats. Daily s.c. injection of insulin at 10 units/rat/day started from the 4th week. The ERG values returned to the control values after 2-3 weeks of insulin therapy. These results indicate that the ERG and VEP recording procedure used in the present study is useful for early detection of the diabetic retinopathy in rats and that the OP of the ERG appears to be vulnerable to diabetes in the rat as it is in the human.
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PMID:[An electrophysiological method for detecting diabetic retinopathy in rats]. 639 51

An adrenal tumour was diagnosed in a 12-year-old female cross-bred terrier. The dog was presented to the veterinary clinic because she had been gaining weight and had started urinating in the owners' house. Clinical findings included obesity, abdominal enlargement, thinning of the hair coat, seborrhoea sicca, and polydipsia and polyuria. The diagnosis was made by clinical pathology, endocrine function tests and abdominal radiography. Surgical removal of the neoplastic right adrenal gland resulted in resolution of the clinical signs, including regrowth of the hair coat.
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PMID:Adrenal adenoma in a cross-bred terrier. 1603 24

An 8-year-old, male neutered, domestic longhair cat was referred for investigation of insulin-resistant diabetes mellitus. Routine haematology, serum biochemistry, urinalysis (including culture), total T4 and urine creatinine:cortisol ratio were unremarkable, but markedly increased insulin-like growth factor-1 concentration was identified and a pituitary mass was subsequently documented. The cat was treated conservatively with the dopamine agonist L-deprenyl and was re-presented 16 months later for worsening polyuria, polydipsia, polyphagia, marked lumbar muscle atrophy, development of a pendulous abdomen and marked thinning of the abdominal skin. Hyperadrenocorticism was diagnosed based on abdominal ultrasonography, dexamethasone suppression testing and endogenous adrenocorticotropic hormone (ACTH). The cat was treated with trilostane (30 mg q24h PO) and showed some clinical improvement, but developed an opportunistic fungal infection and skin fragility syndrome 4.5 months after commencing treatment, and was euthanased. A double-pituitary adenoma comprising a discrete somatotroph adenoma and a separate plurihormonal adenoma (positive immunoreactivity for ACTH, melanocyte-stimulating hormone and follicle-stimulating hormone) was identified on post-mortem examination. These two pituitary adenomas were suspected to have arisen as independent neoplastic entities with the plurihormonal tumour either being clinically silent at the initial presentation or having developed over the subsequent 16 months.
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PMID:Concurrent somatotroph and plurihormonal pituitary adenomas in a cat. 2355 10

Mammalian class IX myosin Myo9a is a single-headed, actin-dependent motor protein with Rho GTPase-activating protein activity that negatively regulates Rho GTPase signaling. Myo9a is abundantly expressed in ciliated epithelial cells of several organs. In mice, genetic deletion of Myo9a leads to the formation of hydrocephalus. Whether Myo9a also has essential functions in the epithelia of other organs of the body has not been explored. In the present study, we report that Myo9a-deficient mice develop bilateral renal disease, characterized by dilation of proximal tubules, calyceal dilation, and thinning of the parenchyma and fibrosis. These structural changes are accompanied by polyuria (with normal vasopressin levels) and low-molecular-weight proteinuria. Immunohistochemistry revealed that Myo9a is localized to the circumferential F-actin belt of proximal tubule cells. In kidneys lacking Myo9a, the multiligand binding receptor megalin and its ligand albumin accumulated at the luminal surface of Myo9a-deficient proximal tubular cells, suggesting that endocytosis is dysregulated. In addition, we found, surprisingly, that levels of murine diaphanous-related formin-1, a Rho effector, were decreased in Myo9a-deficient kidneys as well as in Myo9a knockdown LLC-PK1 cells. In summary, deletion of the Rho GTPase-activating protein Myo9a in mice causes proximal tubular dilation and fibrosis, and we speculate that downregulation of murine diaphanous-related formin-1 and impaired protein reabsorption contribute to the pathophysiology.
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PMID:Rho GAP myosin IXa is a regulator of kidney tubule function. 2613 56

Autoimmune polyendocrine syndrome type II (APS II) is a rare endocrine disorder that involves the adrenal gland (Addison's disease), thyroid (autoimmune thyroiditis), pancreas (type 1 diabetes), and other non-endocrine organs. Herein, we report a case of a 58-year-old woman with a past medical history of systemic lupus erythematosus (SLE) and Addison's disease, who initially presented with nocturia, polyuria, abnormal sweating, fatigue, hair thinning, heat and cold intolerance, and progressive darkening of the skin for the last few months. After a thorough evaluation, she was diagnosed with autoimmune thyroiditis, and thus, she met the criteria for APS II. This report highlights the unusual presentation of APS II in a patient with SLE. We also discuss common pathophysiological mechanisms that can explain the concurrence of SLE and APS II in this patient.
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PMID:Co-Occurrence of Systemic Lupus Erythematosus and Autoimmune Polyendocrine Syndrome II: Is There a Pathologic Link? 3326 18