Clinical significance of hyperparathyroidism in familial multiple endocrine adenomatosis type I (MEA I).

In order to investigate the suggestion that hyperparathyroidism in patients with familial MEA I has a mild and nonprogressive clinical course, we have compared clinical, biochemical, roentgenologic and histologic features of 29 patients with hyperparathyrodism originating from six families with the MEA I syndrome with those of 28 unselected patients with isolated nonfamilial hyperparathyroidism. The patients from the families with MEA I were significantly younger, had lower serum calcium and inorganic phosphate concentrations and a lower incidence of elevated alkaline phosphatase levels. Furthermore, they had multiple enlarged parathyroid glands and recurrence of the disease significantly more often. There was, however, no significant difference in the incidence of renal impairment, urolithiasis, subperiosteal resorption or large bone cysts on roentgenograms, histologic changes in bone biopsy specimens or mortality due to hyperparathyroidism. Therefore, the suggestion that this type of hyperparathyroidism has a milder clinical course is not confirmed in the present study.

Autosomal dominant idiopathic hypoparathyroidism and nervous system dysfunction: report of three cases and review of the literature.

The neurological manifestations of idiopathic hypoparathyroidism in a father, his son, and his daughter are reported. In all three epilepsy was the first manifestation of the disease. Father and son also showed mental deterioration and striocerebellar symptoms; their CT scans revealed symmetrical calcification in the basal ganglia and dentate nuclei. The extent of this calcification increased during normocalcemia, which was produced by dihydrotachysterol therapy. This indicates that other factors than merely hypocalcemia influence the intracerebral calcifying process. Somatosensory evoked potentials (SSEP) showed an abnormal nonspecific complex, indicating dysfunction of the cortical gray matter. It is suggested that in the evaluation of idiopathic hypoparathyroidism one also must be beware of the possibility of epilepsy, mental deterioration, striocerebellar symptoms, intracerebral calcification and SSEP disturbances.

No volatile N-nitrosamines detected in blood and urine from patients ingesting daily large amounts of ammonium nitrate.

Whole blood and urine from 23 patients ingesting ammonium nitrate daily in amounts varying from 2 . 5-9 g were analysed for volatile N-nitrosamines. By contrast with reports in the literature N-nitrosodimethylamine and N-nitrosodiethylamine were not found in blood (limit of detection 0 . 1 micrograms/kg) but a trace of N-nitrosopyrrolidine was found in the blood of one subject. Small amounts of N-nitrosomorpholine in some blood samples and in one blank were believed to be formed during the analysis since morpholine had been added to the samples to indicate artefactual nitrosamine formation. Traces of N-nitrosodimethylamine (0 . 1 microgram/kg) were found in six of the 23 urine samples.

Calcium-phosphate metabolism in autosomal recessive idiopathic strio-pallido-dentate calcinosis and Cockayne's syndrome.

In three siblings with autosomal recessive idiopathic strio-pallido-dentate calcinosis (SPDC) and in three other siblings with Cockayne's syndrome (CS) studies on plasma values of calcium and phosphate, intestinal calcium absorption, radiograms of the hands and studies on the influence of parathyroid hormone (PTH) on the renal threshold for phosphate revealed no abnormalities. In one of the SPDC patients and one of the CS patients the effect of PTH on the cyclic adenosine monophosphate (cAMP) concentrations in urine and cerebrospinal fluid (CSF) were determined. In both a normal response of urinary cAMP was noted. In the CS patient the response of CSF cAMP was also normal. The SPDC patient, however, had a significantly decreased response of CSF cAMP. It is suggested that a decreased sensitivity of the cerebral adenylate cyclase complex is involved in the etiology of autosomal recessive idiopathic SPDC. Subsequently this disorder could be considered as cerebral pseudohypoparathyroidism. The etiology of CS remains unknown.

Study to assess quality of life (morale and happiness) in two continuing care facilities - a comparative study in the UK and The Netherlands.

The elderly in a nursing home in Nijmegen are less disabled and have higher morale and happiness scores than the elderly in a continuing care facility in London. These scores, however, do not correlate with physical or mental disabilities within each group. In Nijmegen, religion (practising and importance of) is associated with high happiness scores in the elderly, whereas use of sedatives lead to reduced happiness or low morale in both groups of elderly.

[Pseudolymphoma syndrome following use of carbamazepine]. / Pseudolymphoma-syndroom na gebruik van carbamazepine.

This article describes the clinical history of a woman aged 75 with a pseudolymphoma syndrome that was probably a consequence of the use of carbamazepine. This syndrome is characterised by lymphadenopathy, fever and a generalised rash. These signs can easily be confused with the existence of a true malignant T-cell lymphoma or Hodgkin's disease. Chemotherapy may be given to these patients if this adverse reaction, which mimicks a true malignant lymphoma, is not recognised. After withdrawal of the drug the symptoms disappear mostly in a few days.

[The course of Alzheimer's disease in a group of nursing home patients: epidemiology, (co)morbidity and mortality]. / Het beloop van de ziekte van Alzheimer bij een groep verpleeghuispatiënten: epidemiologie, (bijkomende) morbiditeit en sterfte.

One hundred medical charts of patients of a psychogeriatric nursing home with 'possible' (senile) dementia of the Alzheimer type were reviewed. Eighty-seven patients had died and 13 were alive at the time of analysis. The aim of the study was to obtain epidemiological information and to investigate (co)morbidity and mortality of these patients. All the data of this group of nursing home patients appeared to have a wide range. The process of dementia started at a mean age of about 75 years and patients remained in their homes for more than five years before admission. The average time spent in the nursing home was 3.0 years. These patients had a shorter life expectancy, counted from the time of admission, compared with the Dutch population. At admission a large percentage of patients suffered from circulatory system disease, musculoskeletal and connective tissue disease and/or sensory deprivation. During the stay the most frequent diseases were urinary and respiratory tract infections, constipation and chronic ulcers of the skin. Symptoms specific of Alzheimer's disease also occurred: feeding problems, disorders of gait and neurological symptoms such as paratonia. A large percentage of patients showed adverse effects of drugs or suffered from the consequences of trauma. Patients died of cachexia or bronchopneumonia. It is unclear whether the population studied is representative of the total population of Alzheimer patients.

[Indicators for progression of dementia in nursing home patients]. / Indicatoren voor progressie van de dementering bij verpleeghuispatiënten.

OBJECTIVE: To investigate the relationship of progression of dementia with patients' characteristics, comorbidity and psychotropic drug use in nursing home patients. SETTING: Psychogeriatric nursing home 'Joachim en Anna', Nijmegen, the Netherlands. DESIGN: Retrospective analysis of medical charts. PATIENTS AND METHODS: In 645 patients, admitted between 1980 and 1989, progression of dementia was expressed by means of a functional status questionnaire developed in the nursing home. Severity of dementia at entry was expressed by a rating scale for elderly patients. Comorbid conditions were classified according to the ICHPPC-2-defined. Psychotropic drug use was expressed as the ratio between days of usage and days of the observation period. Statistical analysis was done by analysis of (co)variance and regression analysis. RESULTS: In univariate as well as in multivariate analysis progression of dementia was related to time spent in the nursing home, severity of dementia at entry and comorbidity and to a lesser degree psychotropic drug use. Patients who stayed for a short time and had a high frequency of concomitant diseases and psychotropic drug use had a faster progression of dementia. Gender, age at entry and type of dementia were of no significance. The factors mentioned, however, accounted for only 20% of the variance. CONCLUSION: Progression of dementia is a complex process which can only partly be explained by the factors mentioned. Nursing home physicians nevertheless should consider these factors when predicting progression.

Dissolution of bilateral renal calculi via percutaneous nephrostomy. Report of a case.

Both kidneys in a patient with multiple, bilateral renal calculi were treated consecutively by irrigation with a stone-dissolving solution through percutaneously placed nephrostomy catheters. Almost complete dissolution of the calculi was obtained. An intercurrent ureteral calculus necessitated introduction of a second catheter in one kidney, but was also successfully treated.

Adult-onset Still's disease complicated by hypercalcaemia: possible relationship with rapidly destructive polyarthritis.

A 34-year-old man with adult-onset Still's disease developed a striking hypercalcaemia during a rapidly destructive polyarthritis with extensive osteoporosis. The hypercalcaemia seemed to be primarily caused by inflammation-induced bone resorption. On prednisone the polyarthritis went into remission and the plasma calcium levels became normal. Other remarkable features in this case were a subluxation of the atlantoaxial joint, a brain-stem haemorrhage, transient hyperuricaemia and hyperuricosuria, and urolithiasis.

Volatile N-nitrosamines, nitrate and nitrite in urine and saliva of healthy volunteers after administration of large amounts of nitrate.

Twelve healthy volunteers ingested orally 7-10.5 g of NH4NO3 in a single dose and 12 others received 9.5 g of NaNO3 intravenously in about 60 min. Samples of blood, saliva and urine were collected just before administration of nitrate and at various times afterwards during a 24 h period. Saliva and urine were analysed for volatile NA, nitrate and nitrite. Blood was analysed for nitrate. Neither in urine nor in saliva were NA other than NDMA detected. Of the 188 urine samples, only 13% contained more than 0.1 microgram NDMA/kg, the highest level being 0.5 microgram/kg. In 92% of the 179 saliva samples, less than 0.5 microgram NDMA/1 was found. The saliva of 1 person contained 0.7-8.9 microgram NDMA/1. Nitrate levels in blood rose sharply during the first hour after intravenous nitrate administration and 2 hours after oral intake, reaching peak values of ca. 6 mmol/1. Peak values for NO-3 + NO-2 in saliva, which were reached 2-6 hours after intake, varied strongly between individuals, ranging from 4 to 43 mmol/1. Nitrite was detected in 26% of the urine samples. There was no correlation between NDMA contents and nitrite in urine. An average of 75% of administered nitrate was excreted in urine in 24 h. However, nitrate contents in blood, urine and saliva after 24 h were still higher than before the nitrate intake.