Hyponatremia is a frequent occurrence after pituitary surgery, having been described in 9% to 35% of the patients. It is produced by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) or, more frequently, by the cerebral salt-wasting syndrome (CSWS). The clinical presentation of both syndromes is identical and the ...

PURPOSE: To report almost simultaneous onset of Vogt-Koyanagi-Harada (VKH) syndrome in co-workers, friends, and neighbors. METHODS: The onset of VKH syndrome in six patients was examined. RESULTS: Patient 1 and Patient 2 worked in the same room of an office and developed VKH syndrome 3 weeks apart from each other. ...

PURPOSE: To investigate the effectiveness of anterior canthal tendon release in the management of centurion syndrome. METHODS: Four patients diagnosed as having the centurion syndrome were treated over a period of 3 years. They all underwent bilateral anterior canthal tendon release under local anesthesia. RESULTS: None of these patients had ...

Patients with Werner's syndrome have the appearance of premature ageing. Neurological complications are usually regarded as uncommon. The neurological manifestations in three patients with cardinal features of Werner's syndrome, including short stature, premature greying and baldness, thin arms and legs, cataracts and scleroderma-like skin changes, are presented. The neurological features ...

Burning mouth syndrome (BMS) is an enigmatous condition both for the patient and the clinician, and is diagnosed on the basis of the patient's symptoms when they have on examination an apparently healthy looking oral mucosa. A variety of local and systemic factors are known to contribute to burning mouth ...

BACKGROUND: Hyper-immunoglobulin E (IgE) syndrome is a complex immune deficiency characterized by chronic eczematous dermatitis, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variably impaired T cell function. Although decreased interferon-gamma (IFN-gamma) production in patients with hyper-IgE syndrome is pointed out and known as a cause of reduced neutrophil chemotaxis, ...

Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient ...

PURPOSE: To elucidate whether thrombocytopenia in 22q11.2 deletion syndrome patients is associated with the hemizygosity of glycoprotein Ib-beta and to clarify the correlation of phenotype and genotype of this gene in 22q11.2 deletion syndrome patients with thrombocytopenia. METHODS: Platelet number, mean platelet volume, platelet agglutination, and the protein level of ...

Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out ...

Aarskog syndrome is a rare syndrome with a typical triad of facial, digital and genital characteristics. Orthodontic treatment of a patient with Aarskog syndrome has not been documented in literature till date. This case report shows that an individual with Aarskog syndrome can be an excellent orthodontic patient and should ...

We describe a low-risk myelodysplastic syndrome patient who did not respond to erythropoietin alone, but who did show a major erythroid response to combination therapy consisting of erythropoietin and moxifloxacin. This observation was exclusively empirical. The immunomodulatory effects of moxifloxacin may explain the synergy with erythropoietin.

The study evaluated neuroophthalmologic and computerized tomography (CT) findings in 100 patients with somatotrophic adenoma and clinical picture of acromegaly, who underwent transsphenoidal adenomectomy. Prior to the surgery, visual field was normal in 77 patients. The diameter of adenoma in these patients ranged from 8 to 30 mm on CT, ...

The bone marrow failure states, aplastic anemia and myelodysplastic syndrome, are characterized by reticulocytopenic anemia, with variable neutropenia and thrombocytopenia. The bone marrow biopsy is very hypocellular in aplastic anemia, but it is usually hypercellular in myelodysplastic syndrome. Marrow cytogenetic abnormalities are present in approximately half of myelodysplastic syndrome patients ...

BACKGROUND/AIMS: Hepatocellular carcinoma patients with paraneoplastic syndromes usually have a larger tumor volume and a higher serum alpha-fetoprotein. Whether the presence of paraneoplastic syndromes, such as hypercholesterolemia, hypoglycemia, hypercalcemia, and erythrocytosis in hepatocellular carcinoma patients is a significant prognostic factor in patients' survival is of clinical interest. METHODOLOGY: We evaluated ...

BACKGROUND: Crush syndrome has been described as extensive muscle damage, leading to acute renal failure. The aim of this study was to evaluate the possible role of nitric oxide, tumor necrosis factor-alpha (TNF-alpha) and interleukin-1 beta (IL-1 beta) in crush syndrome. PATIENTS AND METHODS: A total of 17 patients suffering ...

We investigated nocturnal secretion pattern of melatonin in patients with cardiac syndrome X and healthy subjects. The present study performed in five patients with cardiac syndrome X and in nine healthy controls. Blood samples from all subjects were collected every 2 h intervals between 22:00 and 08:00 h. Melatonin levels ...

PURPOSE: To estimate the prevalence of Usher syndrome in Heidelberg and Mannheim and to map its geographical distribution in Germany. METHODS: Usher syndrome patients were ascertained through the databases of the Low Vision Department at the University of Heidelberg, and of the patient support group Pro Retina. Ophthalmic and audiologic ...

OBJECTIVE: Review the clinical features of occipital condyle syndrome. BACKGROUND: Occipital condyle syndrome consists of unilateral occipital region pain associated with ipsilateral 12th cranial nerve paresis. It is typically due to metastasis to the skull base and is underdiagnosed. DESIGN: We report a retrospective case series of 11 patients (8 ...

Eisenmenger's syndrome is a complex combination of cardiovascular abnormalities and is defined as pulmonary hypertension at systemic pressure level with reversed or bi-directional shunt through an intracardiac or aortopulmonary communication. Patients with Eisenmenger's syndrome are at high risk for peripartum morbidity and mortality. Multigravid patients with this disease are therefore ...

Kyol goeu (literally, 'wind overload') is an orthostatically triggered syncopal syndrome often found among Khmer refugees in the US. In the present study, 36 of 100 (36%) Khmer patients attending a psychiatric clinic were found to have suffered a kyol goeu episode in the past, whereas 60 of 100 (60%) ...

DiGeorge syndrome is characterized by conotruncal cardiac defects, hypocalcemia, and a hypoplastic thymus. Many, but not all, patients have a heterozygous deletion of chromosome 22q11.2. In its most severe form, it represents a devastating syndrome with high mortality. Patients with severe immunodeficiency are candidates for a thymic transplant or a ...

OBJECTIVES: To examine populations with diagnosed clinical interstitial cystitis (IC) and urethral syndrome and normal controls using the potassium sensitivity test (PST), to determine the incidence of PST-provoked pain and/or urgency, and to document the type and location of IC and urethral syndrome pain, association of pain with sexual intercourse, ...

Dapsone syndrome or sulphone syndrome was noticed within four to six weeks of starting treatment in 10 out of 604 patients (1.6%) on MDT for leprosy treated at Rajah Muthiah Medical College Hospital, South Arcot District, Tamil Nadu State during the period 1995-1998. Patients developed either maculo papular rash or ...

The oculocerebrorenal syndrome of Lowe (OCRL) is an X-linked disorder characterized by major abnormalities of eyes, nervous system, and kidneys. We report two patients with typical intracranial lesions on MRI. The proton spectroscopy study of the periventricular white matter showed a moderate elevation of the signal at 3.56 ppm in ...

Triple A syndrome is characterised by achalasia, alacrima, adrenal insufficiency and progressive neurological abnormalities including impaired autonomic nervous function. We present five patients with triple A syndrome in whom we describe xerostomia for the first time, a symptom which was presumed to be practically exclusive to Sjøgren syndrome and familial ...

Rokitansky syndrome is a developmental defect characterized by agenesis of the uterus and vagina but normal gonads and secondary sexual characters. It is not commonly transmitted as a dominant genetic trait. Surrogacy, which is legally and ethically accepted in the UK and other countries, has made it possible for the ...

Gitelman's syndrome is a renal tubular disorder characterized by a sodium and chloride reabsorption defect in distal tubular cells that determines hypokalemia, metabolic alkalosis, hypomagnesemia, and low calcium excretion. The presence of choroidal calcifications was sought in five patients with Gitelman's syndrome by ophthalmic examination, fluorescein angiography, indocyanine green angiography, ...

OBJECTIVE: To describe an often-unrecognized clinical picture of multiple organ failure in hemophagocytic lymphohistiocytic syndrome (HLS). DESIGN: Retrospective chart review. SETTING: A ten-bed pediatric intensive care unit (PICU) in a tertiary children's university hospital. PATIENTS: A total of 11 children (age, 5 months to 13 yrs) who fulfilled the criteria ...

It is known that patients with Klinefelter's syndrome are inclined to develop concomitant malignant tumours, as well as extragonadal germ cell tumours. The association of a primary spinal germinoma in a patient with Klinefelter's syndrome is reported for the first time, and the coincidence of elevated gonadotropin levels and oncogenesis ...

We report two cases of Schnitzler's syndrome in which anti-interleukin-1alpha autoantibodies and functional autoantibodies against the high affinity IgE receptor (FcepsilonRIalpha) or against IgE were absent. One patient responded well to TL-01 phototherapy, a treatment which may be considered in patients with Schnitzler's syndrome if, as is usually the case, ...

The development of steal syndrome distal to an arteriovenous fistula (AVF) created for hemodialysis access remains a significant clinical problem. This study was undertaken to determine the role of intraoperative noninvasive testing in the prediction and management of steal syndrome following arteriovenous fistula creation. First, in order to determine a ...

Ten patients (6F, 4M) with recurrent Tolosa-Hunt syndrome are reported. Besides ocular motor symptoms, one patient had trigeminal nerve involvement, one had ipsilateral ocular sympathicoplegia with miosis and ptosis, and one tinnitus during an episode of Tolosa-Hunt syndrome, ipsilateral to the pain side. One patient had Bell's palsy, one had ...

Patients with Kartagener's syndrome (KS) are invariably infertile with totally immotile spermatozoa. Intracytoplasmic sperm injection (ICSI) is considered to be the treatment of choice for patients with immotile spermatozoa. We report the second KS case in the literature from whom immotile spermatozoa from the ejaculate failed to fertilize mature oocytes ...

The effects of a beta blocker (atenolol), a calcium antagonist (amlodipine), and a nitrate (isosorbide-5-mononitrate) on anginal symptoms in 10 patients with syndrome X were assessed in a crossover, double-blind, randomized trial. Only atenolol was found to significantly improve chest pain episodes, suggesting that it should be the preferred drug ...

Three patients with Sjogren's syndrome are presented in whom frequent tonic/dystonic spasms of the limbs developed during the course of the illness. These patients' clinical findings suggested spinal cord involvement, a localization that was confirmed by magnetic resonance imaging in two patients. In one patient the painful movements responded to ...

BACKGROUND: Hepatocellular carcinoma (HCC) is the most common malignancy in Taiwan. Some patients may manifest paraneoplastic syndromes during the clinical course of the disease. In this study, the authors evaluated the clinical significance of these paraneoplastic syndromes, compared the prevalence of these syndromes between cases of hepatitis B virus (HBV)-related ...

Four patients had anosognosia for hemiplegia (AHP) as a manifestation of pontine infarction in the mediolateral region. Patients with AHP syndrome had no mental and neuropsychologic disturbances, and all had involvement of the medial or lateral part of the pons (medial or lateral pontine reticular nuclei). Brainstem lesions, which activate ...

An intussusception can easily occur in patients with Peutz-Jeghers syndrome (PJS), and its early detection is crucial. We present a PJS patient in whom sonography (US) helped in detecting intussusception at a time when there was only a very vague symptom and provided successful conservative treatment. Close clinical and US ...

No statistical evaluation of patients with carcinoid syndrome in a reliable number of cases has been available in the past 35 years. To update our knowledge about the syndrome, we have evaluated from various clinicopathologic viewpoints a large series of patients with the syndrome reported up to date. The data ...

BACKGROUND: Optic disc elevation associated with Down syndrome is an uncommon phenomenon and raises the suspicion of an intracranial space-occupying lesion, thus necessitating the consideration of invasive and noninvasive investigations. METHOD OF STUDY: Four patients with Down syndrome and optic disc elevation without an underlying intracranial pathology are reported. Thorough ...

Approximately 150 human immunodeficiency virus (HIV)-infected patients with a thrombotic microangiopathy (TMA)-like syndrome have been reported in the literature since the early 1980s. The prevalence of a TMA-like syndrome in our hospitalized patients was determined to discern whether it is a more common occurrence than previously recognized and, if possible, ...

BACKGROUND AND OBJECTIVES: We describe a new therapeutic modality for sacroiliac joint syndrome that represents an alternative to other treatment modalities. We report on four cases of sacroiliac joint syndrome with severe pain. METHODS: Three patients had undergone operative treatment of the lumbar spine and one patient suffered from severe ...

The present paper describes a patient who exhibited 'Alice in Wonderland' (AIW) syndrome as well as Lilliputian hallucinations. The patient regularly consumed a cough syrup that contained dihydrocodein phosphate and dl-methylephedrine hydrochloride over 3 years. At the age of 46, he developed AIW syndrome. The patient ingested a large dose ...

AIM: Patients with Bartter's syndrome and Gitelman's syndrome have reduced vascular reactivity, normo-hypotension and decreased peripheral resistances in spite of biochemical and hormonal abnormalities typical of hypertension. Since we found that both types of patients have increased urinary NO2-/NO3-, metabolites of NO, that correlated with their increased urinary cGMP, second ...

Clinical and neuroendocrinological studies were performed in 41 patients with arteriovenous malformations (AVM) of the cingulate gyrus. Before surgery, the primary affliction in 38 patients consisted of memory disturbance, 5 of these having signs of Korsakov's syndrome. Autonomic and epileptic fits were not typical. Of 38 patients undergoing surgery, postoperative ...

An 8-year-old boy presented with precocious puberty and a mediastinal mass. A computer search showed that this rare presentation is most common with germ cell tumor of the mediastinum in children with Klinefelter syndrome. The tumor was completely resected after preoperative chemotherapy, and the patient is well 2 years after ...

Two sisters with Imerslund-Gräsbeck syndrome who presented with clinical features of cobalamin deficiency are described. Intrinsic factor-cobalamin receptor (IFCR) activity and protein levels were determined in ileal biopsy specimens by using radioisotope assay and immunoblotting, respectively. IFCR activities in ileal homogenates expressed as femtomoles of ligand binding per milligram of ...

Ten patients with Laurence-Moon-Biedl-Bardet syndrome were investigated. They all belonged to one large family within which several intermarriages had taken place. We found that, apart from the already described abnormalities, these patients suffered from dysgenesis of the following epiphyses: vertebrae, proximal femur, proximal tibia, capitellum, tarsal navicular, and the distal ...

Two cases of acute tumor lysis syndrome occurring in patients with hepatocellular carcinoma are reported. Both patients were treated with transcatheter chemoembolization. Tumor lysis syndrome was not anticipated in the first case and was diagnosed late. However, the second patient's syndrome was detected early and treated appropriately. Subtle changes in ...

We report on the clinical evolution of the Brazilian family with Ramon syndrome described by de Pina-Neto et al. [1986, Am J Med Genet 25:441-443]. Three members (patients IV-2, IV-18, and IV-19) have developed pigmentary changes in the retina and paleness of the optic disk. Patient IV-18 also has developed ...