Primary adrenal lymphoma presenting as Addison's disease. Case report and review of the literature.

Primary Adrenal Lymphoma (PAL) is a very rare clinical entity. Adrenal insufficiency is a common complication of this pathology. Most patients present with clinical and laboratory findings of adrenal insufficiency and bilateral enlargement of the adrenal glands. We present a 78-year-old woman admitted to our institution with typical clinical and laboratory findings of adrenal insufficiency. Computerized tomography (CT) of the abdomen revealed bilateral enlargement of the adrenal glands. The patient was eventually diagnosed with a diffuse large B-cell lymphoma after a CT-guided needle adrenal biopsy and treated with combined immuno-chemotherapy (R-LPD-COP). Twenty months after the initial evaluation, she is in good condition, with no signs of adrenal insufficiency.

Thyroid dysfunction in adult long-term survivors after hemapoeitic stem-cell transplantation (HSCT).

Thyroid function was evaluated in 72 adult survivors (41 females and 31 males) at 16 to 56 years of age, 1.5 years mean time (range 0.2 - 9.8) after hemapoeitic stem cell transplantation (HSCT) with no known prior history of thyroid dysfunction. Thyroid stimulating hormone (TSH) and free thyroxin levels (FT4) were determined before and after stimulation with thyrotropin releasing hormone (TRH). Conditioning regimens for HSCT did not include TBI. Overt hypothyroidism (basal TSH > 8 microIU/ml, FT4 < 0.8 ng/dl) was observed in 6% of male patients and 5% of female patients; subclinical hypothyroidism (basal TSH 4 - 8 microIU/ml, low normal FT4 0.8 - 1.9 ng/dl) was observed in 13% of males and 5% of females. A significant number of euthyroid patients (40% males and 54% females) with normal basal TSH and FT4 levels overresponded to TRH stimulation; the finding being statistically significant (p < 0.005). A heavy TSH response after TRH stimulation indicates compensated subclinical dysfunction of the thyroid gland. Chemotherapy-only conditioning regimens may have an adverse effect on thyroid gland function not always detected by determination of basal TSH and FT4 levels. This finding warrants long-term evaluation of thyroid function in HSCT patients.

Effectiveness of home-centered care through telemedicine applications for overweight and obese patients: a randomized controlled trial.

OBJECTIVE: To determine if home-centered monitoring through telemedicine has an impact on clinical characteristics, metabolic profile and quality of life in overweight and obese patients. DESIGN: Randomized controlled trial, 6-month duration. SETTING: Tertiary care academic hospital. SUBJECTS: A total of 122 patients were eligible to participate as they met the inclusion criteria of increased body mass index (BMI>25 kg/m(2)), age>18 and <70 y and ability to operate electronic microdevices. INTERVENTIONS: All patients in the control group (n=77) received standard hospital care. Patients in the intervention group (n=45), additionally, measured three times a week, for 6 months, their blood pressure and body weight and transmitted them to an automated call center. These values were not shared with the patients' physician or dietician. MAIN OUTCOME MEASURES: Clinical (body weight, BMI, blood pressure), laboratory (fasting plasma glucose, triglycerides, HDL-cholesterol, total cholesterol) and quality of life parameters (SF-36((R)), Visual Analog Scale of European Quality-5 Dimensions, Obesity Assessment Survey). Data were analyzed in an intention-to-treat-way (last observation carried forward). RESULTS: Drop-out rate was similar in the control and intervention groups: 12 vs 11 percent, respectively, P=NS. There were no significant differences at baseline between intervention and control groups in all main outcome parameters. There were significant decreases for patients in the intervention group in body weight (from 101.6+/-22.4 to 89.2+/-14.7 kg, P=0.002, P=0.05 vs controls at 6 months), total cholesterol (from 247.6+/-42.0 to 220.7+/-42.6 mg/dl, P=0.002, P=0.05 vs controls at 6 months) and triglycerides (from 148.4+/-35.0 to 122.3+/-31.4 mg/dl, P=0.001, P=0.01 vs controls at 6 months). Intervention group patients made a total of 1997 phone contacts. The number of phone contacts was correlated positively with Social Functioning (SF), Vitality (VT) and Mental Health (MH) scores of SF-36((R)) at baseline (r=0.48, r=0.41, r=0.41, respectively, P=0.05) but not with weight loss. CONCLUSIONS: Home-centered, intense treatment through the use of telemedicine can be effective in improving short-term obesity outcomes.

Home care delivery through the mobile telecommunications platform: the Citizen Health System (CHS) perspective.

Health delivery practices are shifting towards home care. The reasons are the better possibilities for managing chronic care, controlling health delivery costs, increasing quality of life and quality of health services and the distinct possibility of predicting and thus avoiding serious complications. For the above goals to become routine, new telemedicine and information technology (IT) solutions need to be implemented and integrated in the health delivery scene, and these solutions need to be assessed through evidence-based medicine in order to provide solid proof for their usefulness. Thus, the concept of contact or call centers has emerged as a new and viable reality in the field of IT for health and telemedicine. In this paper we describe a generic contact center that was designed in the context of an EU funded IST for health project with acronym Citizen Health System (CHS). Since the generic contact center is composed by a number of modules, we shall concentrate in the modules dealing with the communication between the patient and the contact center using mobile telecommunications solutions, which can act as link between the internet and the classical computer telephony communication means. We further elaborate on the development tools of such solutions, the interface problems we face, and on the means to convey information from and to the patient in an efficient and medically acceptable way. This application proves the usefulness of wireless technology in providing health care services all around the clock and everywhere the citizen is located, it proves the necessity for restructuring the medical knowledge for education delivery to the patient, and it shows the virtue of interactivity by means of using the limited, yet useful browsing capabilities of the wireless application protocol (WAP) technology.

Post-partum thyroiditis in a mediterranean population: a prospective study of a large cohort of thyroid antibody positive women at the time of delivery.

Post-partum thyroiditis (PPT) is a common disease with important clinical sequelae. Its clinical features, association with antimicrosomal antibodies (TPOAb) and long-term prognosis are not thoroughly described in Mediterranean populations. The aims of this study were: 1) to describe clinical features of PPT in a Greek population, 2) to associate TPOAb with PPT presence, and 3) to identify long-term prognosis of PPT. 1,594 consecutive post-partum women were studied within 3 days of their delivery. Women with positive TPOAb were followed-up for one yr for clinical and biochemical evidence of PPT. Incidence of positive TPOAb was 5.2% whereas incidence of PPT was 2.4%. PPT was presented as hyperthyroidism alone (18%), hypothyroidism alone (40%) and hyperthyroidism followed by hypothyroidism (42%). A titer of 1:1600, immediately post-partum, could predict PPT with 97% sensitivity and 91% specificity. History of PPT in previous pregnancies was associated with PPT in the current pregnancy. This association did not exist with maternal age, gestation duration, parity, abortions, sex of the newborn, birth-weight or smoking. Long-term hypothyroidism developed in 21% of women. Observation and post-partum thyroid function testing, where PPT is suspected, seems to constitute the best clinical approach. There is need for long-term follow-up in PPT patients.

Pituitary abscess presenting with cranial nerve paresis. Case report and review of literature.

Non-adenomatosus lesions of the pituitary represent a small part of the intrasellar processes and they have heterogeneous presentation. Making a precise diagnosis is of great importance, as it may lead to more efficient management. A 65-year-old man was admitted to the hospital because of headache and right cranial nerve III palsy. Basic laboratory work-up was normal whereas endocrinological assessment revealed hypopituitarism without diabetes insipidus. Plain radiography showed an enlarged sella and frontal and paranasal sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the sella revealed an intrasellar lesion with extension to the sphenoid and cavernous sinuses as well as the suprasellar region, exerting pressure on the optic chiasm. On T1-weighted images the mass had a low-intensity signal with a smooth enhancing rim with bright signal. Given the presence of multiple sinusitis and imaging characteristics a pre-operative diagnosis of pituitary abscess was made. The patient was operated via transphenoidal route and purulent material was drained out. Cultures of the material were positive for Staphylococcus aureus. Antibiotics as well as cortisol replacement therapy were given. Three months later hypopituitarism persisted but there was significant improvement in the neurological findings. We report a case of an unusual presentation of a pituitary abscess. High index of suspicion, the presence of associated conditions such as pituitary tumors, meningitis or sinusitis, as well as diabetes insipidus and specific imaging features are the main diagnostic clues. Pre-operative diagnosis, which will lead to prompt antibiotic therapy and transphenoidal drainage, can decrease high mortality and morbidity associated with this disease.

Stapedial reflex: a biological index found to be abnormal in clinical and subclinical hypothyroidism.

There is only limited evidence for biological parameter abnormalities in subclinical hypothyroidism. The aim of this study was to investigate the impact of varying degrees of thyroid failure on the stapedial reflex as a biological index, and establish its role in the evaluation of the hypothyroid patient. We studied 10 patients with subclinical hypothyroidism, 10 patients with clinical hypothyroidism, and 20 controls. All three parameters of stapedial reflex (amplitude, decay, and threshold) were measured before and after restoration of euthyroidism through thyroxine administration. Data are given as mean +/- SEM. Stapedial reflex maximal amplitudes were different among the groups studied (p < 0.0001), as values in subclinical (4.3 +/- 0.4 mm) and clinical (3.7 +/- 0.3 mm) groups before treatment were lower (p < 0.05) than those of control (5.7 +/- 0.3 mm), and subclinical (6.4 +/- 0.5 mm) and clinical (5.6 +/- 0.4 mm) groups after treatment. Similarly, stapedial reflex decays were different among the groups studied (p < 0.001), as values in subclinical (81 +/- 7 ms) and clinical (89 +/- 4 ms) groups before treatment were higher (p < 0.05) than those in control (65 +/- 2 ms), subclinical (56 +/- 8 ms), and clinical (61 +/- 8 mm) groups after treatment. There was no significant difference among the groups for stapedial reflex threshold or significant correlation between stapedial reflex parameters and thyroid function tests. Stapedial reflex, a biological parameter that reflects neuromuscular status, is abnormal in patients with subclinical and clinical hypothyroidism and returns to normal when clinical and biochemical euthyroidism has been achieved through thyroxine administration.

TSH-secreting pituitary macroadenoma in an 11-year-old girl.

Graves' disease, multinodular toxic goiter or toxic adenoma are the usual causes of hyperthyroidism in children as well as in adults. We report a case of hyperthyroidism due to TSH-secreting pituitary macroadenoma in an 11-year-old girl. The patient was admitted to the Endocrine Department for pituitary function evaluation, five months after transfrontal adenomectomy and pituitary irradiation for a macroadenoma. On admission the patient was clinically hyperthyroid and the work-up established a diagnosis of hyperthyroidism due to TSH-secreting adenoma (high levels of TSH in the face of hyperthyroidism, no TSH response to TRH stimulation, diffuse thyroid goiter without eye signs or pretibial myxedema). Of interest in this case was the fact that: (a) she is the youngest patient reported with hyperthyroidism due to a TSH-secreting macroadenoma and (b) hyperthyroidism was diagnosed after adenomectomy.

Visual evoked potentials in hypothyroid and hyperthyroid patients before and after achievement of euthyroidism.

Thyroid hormone deficiency is frequently associated with central nervous system (CNS) disturbances such as mental retardation, convulsions, coma etc. Studies of quantitative changes in CNS in hypo- or hyperthyroidism are scarce. Evoked potentials is a good method of assessing the electrical response of the brain to different (visual, acoustic, somatosensory) stimuli and has been used extensively in the study of brain disturbances and to a lesser degree in metabolic diseases. We studied the visual evoked potentials (latency and amplitude) in 12 patients with hyperthyroidism and 15 patients with hypothyroidism, before treatment and after they became euthyroid. Four of the hyperthyroids (33%) had abnormally prolonged (> 104 msec) latencies before therapy. Two of them had clinical exophthalmos. No change was observed after euthyroidism was achieved. On the contrary 7 out of 15 (47%) hypothyroids had abnormally prolonged latencies which became normal in 4 when euthyroidism was achieved. Amplitude was lower than normal in 6 and became normal only in one of them after treatment. None of the hyperthyroid patients had amplitude changes. In conclusion, hypothyroid patients may have changes in the amplitude and/or the latency of visual evoked potentials which are reversible to a great extent with thyroxine. Evoked potentials is another method of studying in humans the metabolic effects of thyroxine deficiency in CNS.

Large parathyroid functioning carcinoma (1,200 g) presenting as a substernal goiter.

Parathyroid carcinoma is a rare tumor responsible for 0.5-5% of primary hyperparathyroidism. It is usually small (not more than 27 g) and the precise diagnosis of malignancy is made when local or distant metastases are found. We describe a case of a 37 yr old male presenting with a substernal goiter and no specific symptoms except hypertension. This mass had cysts and calcifications and it was in the anterior upper mediastinum. The patient had severe hypercalcemia (Ca greater than 14 mg/dl), high PTH levels and mild renal failure. Bone scanning showed signs of hyperparathyroidism. The patient was subjected to total thyroidectomy and removal of the mass en block. The tumor was circumscribed lobulated and mostly cystic. It weighed 1,200 g (380 g after evacuation of cysts) and measured 12 x 9 x 4.5 cm. Histologic examination showed a highly differentiated adenocarcinoma of parathyroid with metastasis in a regional lymph node. Almost 4 years later the patient is alive and well without hypercalcemia and without evidence of distant metastases.

Endocrine studies in a patient with functioning adrenal rest tumor of the liver.

We report a case of a calcified liver tumor in a 23 year old female patient who presented with virilization and a mild degree of Cushing's syndrome. Androgen levels were elevated; there was loss of cortisol circadian rhythm and marked increase in urinary 17-ketogenic and 17-ketosteroids which failed to suppress with administration of dexamethasone. Venous sampling by inferior vena cava catheterization showed that the highest steroid hormone levels were in blood from the right hepatic vein. After death, in vitro studies revealed that the tumor contained testosterone and cortisol as determined by immunofluorescence techniques. The adrenals and ovaries were atrophic. Results of metyrapone testing indicated dyshormonogenesis. To our knowledge, this is the first case of an adrenal rest tumor of the liver proved to be functionally active.

Three adult cases resembling hereditary bone dysplasia.

Hereditary bone dysplasia with hyperphosphatasemia is a generalized disorder of bone formation which begins in infancy, uniformly involves the skull and long bones and results in progressive deformities and short stature. This entity has been described 27 times under various names, including juvenile Paget's disease, but only two case reports have described the condition in adults. In the present report two siblings and an unrelated individual are described with features resembling hereditary bone dysplasia. In all three the condition developed in infancy but was first recognized in middle age. Clinical and radiographic features of short stature, extensive thickening of the calvarium with areas of "cotton wool sclerosis", and bowed deformities of the long bones were present. The serum alkaline phosphatase was elevated in one case and normal in two. One patient demonstrated a marked clinical and biochemical response to a six month course of disodium etidronate after failing to respond to a trial of salmon calcitonin. There were significant differences between these three cases and classic hereditary bone dysplasia as described in infants and children. The patients themselves also had variable features. These observations suggest that either hereditary bone dysplasia is indeed variable, especially as afflicted children pass into adulthood, or different skeletal diseases are presently being included under the general term hereditary bone dysplasia with hyperphosphatasemia.

Neurologic disturbances in Paget disease of bone: response to calcitonin.

The neurologic manifestations of Paget disease and the therapeutic effect of calcitonin were studied in 49 patients. Twenty-four patients (49%) had neurologic disorders involving cranial nerves other than the auditory system, brainstem, spinal cord, or spinal roots and nerves. Eighteen of the 24 patients (75%) showed significant subjective or objective improvement after calcitonin treatment. The effect of calcitonin treatment on spinal cord compression was dramatic in three of six patients. The observations made of these patients support previous data suggesting that the neurologic signs and symptoms of Paget disease have their pathogenesis in both mechanical impingement and vascular distortion. The importance of early detection of neurologic signs and symptoms is emphasized, since prompt treatment with calcitonin may prevent severe complications.

Brown tumors associated with secondary hyperparathyroidism of chronic renal failure.

Two cases of brown tumors associated with secondary hyperparathyroidism and chronic renal failure are reported. Both patients underwent excision of the tumor because of mechanical interference. Subsequent parathyroidectomy has substantially improved the general status of the patients.

Cardiac tamponade in primary myxedema and review of the literature.

A case of cardiac tamponade secondary to primary myxedema is described. The nature of the patient's pericardial fluid and clinical course compared with other cases in the literature is reviewed. The patient had no recurrence of cardiac tamponade. Complete resolution of the pericardial effusion occurred 10 months followint initial pericardiocentesis and L-thyroxine therapy.

Primary hyperparathyroidism and breast cancer.

Three cases demonstrating the coexistence of primary hyperparathyroidism and breast carcinoma with the disappearance of hypercalcemia following removal of parathyroid adenomas are presented. In these cases, the patients had the typical diagnostic findings of primary hyperparathyroidism. Reluctance to perform neck explorations in such patients does not appear warranted.

Paget's disease of the bone: observations after cessation of long-term synthetic salmon calcitonin treatment.

Thirteen patients with Paget's disease of the bone were treated with subcutaneous injections of synthetic salmon calcitonin (SCT) for a mean period of 22 months at doses of 50-100 MCR units daily or 3 times a week. They manifested symptomatic improvement and significant reductions in serum alkaline phosphatase and urinary hydroxyproline excretion during SCT administration. Following discontinuation of SCT, symptomatic improvement was maintained in 10 patients for up to one year, whereas a recurrence of symptoms was seen in only 3 patients. The serum alkaline phosphatase generally showed a return toward pretreatment values 6 months after discontinuation of SCT, whereas urinary hydroxyproline remained depressed for up to a year.