Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 52-year-old patient was admitted to the hosptial for evaluation of hypertension. He had two skin lesions, one on the forehead and one in the postauricular area, which had been present since birth. The forehead lesion was a nevus sebaceous and the postauricular lesion was a syringocystadenoma papilliferum. Except for a few patients with widespread nevus sebaceous and syringocystadenoma papilliferum associated with neurologic abnormalities, most of the previously reported patients with these nevi have had solitary lesions of one or the other. An association of nevus sebaceous and syringocystadenoma papilliferum in the same lesion is not uncommon. Despite bleeding and crusting in one of the lesions and despite informing the patient that a malignant neoplasm may develop in these nevi, he refused excision of either of the lesions.
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PMID:Nevus sebaceous and syringocystadenoma papilliferum. 18 8

The clinical, laboratorial and radiological features of a 3-month-old child with neurocutaneous melanosis are described. The patient was born with multiple disseminated benign cutaneous nevi, proven by skin-biopsy, and presented with drug-resistant seizures and psycho-motor retardation. Serial cerebrospinal fluid studies showed high protein and low glucose levels, with pleocytosis and malignant cells in the fluid. Pneumoencephalogram showed mild non-obstructive hydrocephalus. Immunological studies showed normal immunological activity at three months of age and very poor activity at one year. The patient died at 16 months of age, after the sudden onset of intracranial hypertension and meningeal signs. Immunotherapy and chemotherapy were suggested but not accepted by the family. Some comments are made based on this and other 43 cases described in the literature; the value of laboratory studies in detecting malignant transformaiton in the meninges in patients with benign skin nevi is stressed.
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PMID:[Neurocutaneous melanosis. Report of a case]. 65

The authors describe two children with anomalous intracranial venous return associated with bilateral facial nevi, macrocrania, and cephalic venous hypertension. Both children had functional absence of the jugular bulbs, forcing the intracranial venous effluent to exit through persistent emissary pathways. Both children had sustained intracranial hypertension, with one child developing symptomatic communicating hydrocephalus that responded satisfactorily to shunting. The relationship between these patients and those with Sturge-Weber syndrome is discussed. The embryologic abnormality producing the anomalous venous return is characterized. The link between venous hypertension and the development of hydrocephalus is discussed. The increased cranial compliance seen in this age group may predispose certain pediatric patients to develop hydrocephalus when stressed by venous hypertension.
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PMID:Facial nevi associated with anomalous venous return and hydrocephalus. 93 97

Plasma HCS levels have been measured in normal and pathological pregnant women. In the normal group HCS levels increased from 6--8 weeks till 33-34 weeks and then felt significantly. HCS pattern in prediabetic and chemical diabetic pregnant women was similar to the normal group. However HCS levels in chemical diabetics were significantly higher during the first two trimesters. HCS levels increased in twin pregnancy, diminished in cases of eclampsia, hypertension, fetal growth retardation, mole and blighted ovum, and disappeared after intrauterine death. Nothing could be deduced from the obese and Rh-isoimmunization groups. It is confirmed the value of HCS determination as an index of placental maturation. Also, insulin/HCS ratio may be of some aid in the study of carbohydrate intolerance in pregnancy.
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PMID:Human chorionic somatommamotropin (HCS) and pregnancy. Its relation with insulin. 103 1

To elucidate the role of Met-enkephalin in the hypertension in spontaneously hypertensive rats, the depressor response to Met-enkephalin was compared between normotensive (Wistar) and spontaneously hypertensive rats by administration of bestatin (1.25 mg) as a component of a mixture of enkephalinase inhibitors. All experiments were performed under anesthesia by intraperitoneal injection of pentobarbital (50mg/kg). Catheters were connected to the arterial and venous lines for blood pressure recording and administration of drugs. Hypotensive effects of injected Met-enkephalin were noticeably enhanced by administration of bestatin compared with pre-bestatin responses at each dose of Met-enkephalin(10, 25, 50, and 100n mol). We could not find any statistically significant difference between blood pressure responses to 25n mole Met-enkephalin in Wistar and spontaneously hypertensive rats both before and after administration of bestatin. Our present study dose not suggest the active role of Met-enkephalin in the hypertension of spontaneously hypertensive rats.
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PMID:A comparison of the hypotensive effects of Met-enkephalin in normotensive and spontaneously hypertensive rats. 274 99

The etiology of the Klippel-Trenaunay syndrome (KTS) remains obscure. Although venous hypertension secondary to deep venous obstruction has been suggested as a cause, recent studies have demonstrated that most patients have unimpeded venous drainage. Calf blood flows have been measured in 33 patients with KTS using venous occlusion plethysmography. Although all flow rates were within normal limits, flow in affected limbs was invariably greater than in normal limbs (p less than 0.001), and this is related to the presence of the nevus. Biopsies of subcutaneous veins demonstrate the histological features of a response to chronically raised flow. The authors suggest that KTS is caused by mesodermal abnormality during fetal development, leading to the maintenance of microscopic arteriovenous communications in the limb bud, as a result of which the triad of nevus, hypertrophy, and superficial varices is produced. Deep venous abnormalities occur pari passu with the triad and are not responsible for its development.
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PMID:The etiology of the Klippel-Trenaunay syndrome. 299 51

The authors reported the clinical course and the postmortem examination of a unique case of neurocutaneous melanosis with numerous anomalies and complications, which included congenital dislocation of lenses, hypogonadism, ectopia of prostatic duct, genuine phimose, retentio testis, psina bifida and neurogenic bladder. This 13-year-old boy with a large hairy nevus in a bathing trunk configulation and multiple small nevi over the whole body since his birth was admitted to our hospital for evaluation of headache and vomiting. Neurological examination showed bilateral papilledema and slight left hemiparesis. A CT scan revealed a large right frontal mass and craniotomy was performed with subtotal removal of this tumor which was confirmed as a malignant leptomeningeal melanoma. He initially made uneventful postoperative recovery, and two courses of chemotherapy with DTIC, ACNU and VCR were given; however, the currence of brain tumor ensued shortly thereafter, and he died in approximately six months after the onset of intracranial symptoms despite of the third course of chemotherapy. Thirty five cases of neurocutaneous melanosis associated with or without malignant melanoma have been reported in Japan. Twenty-eight cases were male and 7 female. Two cases showed the evidence of primary malignant melanoma outside of the central nervous system, whereas twenty eight leptomeningeal melanoma, in which 22 were solid and 6 diffuse, were shown intracranially. Other 5 cases had epileptic seizure and/or hydrocephalus caused by wide spreaded leptmeningeal melanosis. This high incidence of intracranial malignant melanoma in this disorder was remarkable compaired with the previous reports in other countries. Mean duration between deaths and the onset of symptoms of intracranial hypertension or focal neurological signs was 7 months, ranging from 1 to 24 months, showing the rapidly deteriorating course in this disorder.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case of neurocutaneous melanosis associated with intracerebral malignant melanoma]. 332 33

Using strains of spontaneously hypertensive rats with different degrees of hypertension, the influence of the duration of hypertension on platelets was examined through changes in platelet serotonin contents. The blood pressures of these strains were in the descending order of m-SHRSP greater than SHRSP greater than SHR greater than WKY. Serotonin content in normotensive WKY platelets was maintained in the range of 0.715 +/- 0.048(17) n mole/10(8) through ages 5-50 weeks in both sexes. In contrast with WKY of the same age and sex, a significant decrease in platelet serotonin content began to be observed in male m-SHRSP at 18-weeks of age, in female m-SHRSP and male SHRSP at 22-weeks of age, and in female SHRSP at 32-weeks of age, respectively. The content in SHR platelets of both sexes was unaltered up to 40-weeks of age. The time of the appearance of these exhausted platelets coincided with the reported time of scanning electron microscopic observation of vascular injuries in each strain of rats. It has been concluded that a long duration of hypertension causes platelets to become degranulated and exhausted due to in vivo activation of platelets at sites of arterial injury. Thus the changes of platelet contents could be an indicator of vascular injuries.
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PMID:The appearance of exhausted platelets due to a duration of hypertension in stroke-prone spontaneously hypertensive rats. 398 98

Several mechanisms can be invoked in order to explain the appearance in a limb of the characteristic triad : naevus, varices and osteodystrophy. Many cases can be explained by the haemodynamic disturbance associated with venous hypertension caused by a venous malformation combined with arteriovenous microcommunications. These are the true Klippel and Trenaunay Syndromes. In a few rare cases the haemodynamic disturbance is caused by one or more arteriovenous fistulae with major flows. These are the Parkes-Weber-Syndromes. Finally, in the more complex syndromes there is the combined triad of naevus, varices and osteodystrophy, together with other genetic disturbances. These are the Phacomatoses.
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PMID:[Klippel-Trenaunay syndrome and phacomatoses]. 624 51

A total of 16 normal subjects and 30 patients with IB (labile hypertension) and IIB (stable hypertension) stages of essential hypertension during excessive salt intake as well as 13 normal persons and 43 patients at the labile and stable stages of essential hypertension on usual salt diet were examined. Renin activity, plasma aldosterone and cortisol levels were studied using radioimmunoassay in the basal conditions and 1 and 5 hours later after intravenous administration of 5% saline solution (3,6-3,8 mu mole of sodium (1 kg body mass). It was demonstrated that the prolonged excessive intake of sodium hydrochloride resulted in the alteration of the functional state of the renin-angiotensin-aldosterone system even in the normal persons. The above alterations are characterized by the loss of the adequate response of the renin-angiotensin system and the adrenal cortex to the increased sodium concentration, and extracellular fluid volume in the body, as well as by the separation of the functions of these two humoral systems.
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PMID:[Effect of salt on the functional state of the renin-angiotensin-aldosterone system in healthy persons and hypertensives]. 702 16


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