Endocrinologic profile of patients with idiopathic melasma.

Complete endocrinologic evaluation of 9 women (ages 24-41) with idiopathic melasma (melasma not associated with pregnancy nor ingestion of oral contraceptives) was performed and compared to age- and sex-matched normal controls. Serum cortisol, adrenocorticotropin, plasma immunoreactive alpha and beta melanocyte-stimulating hormones, luteinizing hormone, follicular-stimulating hormone, estradiol and progesterone levels were performed in the basal state. Additionally, total T4, T3RU, FTI, prolactin, 2-h postprandial blood sugar, and 24-h urine for 17-hydroxysteroids and 17-ketosteroids were done and found to be normal. The melasma patients presented statistically significant increased levels of LH (p less than 0.001) and lower levels of serum estradiol (p less than 0.025) than normal controls. It is proposed that these hormonal alterations may represent subclinical evidence of a mild ovarian dysfunction which may underlie the pathogenesis of some cases of idiopathic melasma.

External beam irradiation for T1, T2-3 and T4 transitional cell carcinoma of the urinary bladder.

Fifty-seven patients with T1 transitional cell carcinoma of the bladder, 57 patients with T2-3 and 10 patients with T4 were treated by radical irradiation +/- salvage cystectomy. Thirty-five patients with T1 were treated by transurethral resection (TUR) plus local chemotherapy +/- salvage cystectomy. Eleven patients with T2-3 were treated with 5 X 4 Gy in one week followed by cystectomy while four patients with T2-3 were treated with 40 Gy in 4 weeks also followed by cystectomy. Five patients with T4 were treated with 40 Gy in 4 weeks plus cystectomy. Seven year survival rates are: 69% for T1 treated by radical irradiation, 43% for T1 treated by TUR (p less than 0.02), 36% for T2-3 treated either by radical irradiation or 5 X 4 Gy plus cystectomy, and 18% for T4 treated by radical irradiation. All the survival rates indicated are free of disease and include the cases in which salvage cystectomy was successfully performed. Sixty per cent of the cured patients conserved very good functional bladders. We believe that external beam irradiation plus salvage cystectomy is indicated in the treatment of T1 and T2-3 tumours of the bladder.

Pericardial effusion. Unusual complication in thyroid cancer.

A case in which a prolonged hypothyroid state resulted in development of a pericardial effusion is presented. The compensatory mechanism for protein homeostasis is lost in severe hypothyroidism. Iodine is transported to the pericardial space, which is filled with a high-protein content fluid and remains trapped. The mechanism for the accumulation of the fluid in the pericardial space is discussed. To prevent this unusual but dangerous complication, the authors recommend close monitoring of the patient's clinical status and serum thyroid stimulating hormone. The use of a short acting thyroxine substitute for at least 2 weeks can decrease occurrence of this complication and will shorten the time the patient needs to be hypothyroid.

Recurrent ectopic adrenocorticotropic hormone producing thymic carcinoid detected with octreotide imaging.

Primary thymic carcinoids are rare tumors in which the tumor cells retain functional somatostatin receptors. In-111-labeled octreotide imaging has been used to diagnose abdominal carcinoids with a sensitivity rate of approximately 87%. The authors describe a case of a recurrent, ectopic cortisol-releasing hormone that produced thymic carcinoid localized as a focal area of increased activity in the upper mediastinum when planar and tomographic octreotide scintigraphy was used. Chest CT and MRI failed to localize the tumor. This may be the first reported case of In-111-labeled octreotide used to identify Cushing's syndrome caused by a cortisol-releasing hormone that produced thymic carcinoid.

Validation of single photon absorptiometry in estimating bone mineral mass in rhesus monkey skeletons.

Recent emphasis on age-related morbidity addresses the increasing life expectancy of Western countries. Osteopenia is a multi-factorial process affecting a sizeable number of elderly individuals, with bone fractures producing significant morbidity and related mortality. A non-human primate would be a very useful experimental model for the study of age-related osteopenia, if it could be shown that it is similarly afflicted by age and if non-invasive methods utilized among humans could give a valid estimation of bone mineral content in the animal. Our previous studies utilizing SPA (single-photon absorptiometry) suggest age-related decrease in BMC (bone mineral content) among rhesus macaque skeletons, similar to that observed in humans. The present studies were done in order to further validate the utilization of bone densitometry (SPA) in the rhesus macaque, comparing their BMC and bone density (BD) with the size and weight of their corresponding bones. Both radii and the right femur of 102 adult rhesus monkey skeletons (5 to 23 years old), were obtained from the Caribbean Primate Research Center (CPRC) Skeletal Collection. There were 55 females and 47 males. Bone parameters were obtained using a Norland Digital Bone Densitometer, model 2780 at 2 scanning sites: distal 1/3 of radii and proximal 1/3 of femurs. Bone lengths (cm) and weights (gm) were compared and correlated with densitometric parameters. Excellent correlations were consistently found, with r values 0.74 to 0.96 and p less than 0.0001. Thus it appears that SPA at distal 1/3 radius or proximal 1/3 femur are a valid estimate of total bone mineral mass in rhesus monkeys.

Bone densitometric profile among young adults in Puerto Rico: morphometric and SPA results.

Through many contributing factors (ethnicity, diet, exercise, sun exposure, sex, etc.), bone density has traditionally been considered to reach a peak (PBD), during the third decade of life. More recently, data suggests this might occur earlier, mostly mediated by onset and duration of sexual maturation. In order to ascertain such contention, we studied 45 young (20-24 yr) adults utilizing single-photon absorption densitometry (SPA, Norland 2870), at the distal 1/3 of non-dominant arms, a site with 75% cortical bone. A uniform questionnaire on their life styles, ethnic/racial background, & biochemical/hormonal tests established their healthy state, as usually found among medical student volunteers. All had reached Tanner V stage. BD was obtained dividing BMC (bone mineral content, gm/cm) by BW (bone width, cm), and expressed as gm/cm2. Wt (weight) of Males (n = 24, age = 22.8 +/- 0.8 yr) was 77.9 +/- 8.9 Kg, height, (Ht) was 176.1 +/- 5 cm, both significantly higher than for Females (n = 21, Wt = 58.4 +/- 9.8 Kg, Ht = 159.2 +/- 5 cm), p < 0.0001). BMI (body mass index, kg/m2) were: M = 25.3 +/- 2.5 while F = 23.0 +/- 2.9 (p < 0.008). Ages were similar: M = 22.8 +/- 0.83, F = 22.5 +/- 0.81 years. Results were analyzed using SAS (IBM PS) & Cricket-graph (Macintosh), expressed as arithmetic means +/- SD or sem. LR (regressions) were applied whenever feasible.(ABSTRACT TRUNCATED AT 250 WORDS)

Incidence of insulin dependent diabetes mellitus in Puerto Rico: the University Pediatric Hospital experience.

The incidence of newly-diagnosed Insulin-dependent diabetes mellitus (IDDM) in a pediatric population has been found to increase with colder seasons in countries at latitudes where a marked weather change presumably triggers a higher prevalence of viral infections (especially respiratory), that may induce an insulitis which could be etiologically related with IDDM. In order to assess whether the opposite is true for a country with rather uniform climate, like Puerto Rico, we have studied the seasonal pattern of newly diagnosed IDDM seen at our institution during a 10 year period (1973-1982). Also, recent reports on a higher mortality from diabetes in Puerto Rico going from 16.6 in 1977 to 31.0 in 1983, prompted us to look at a possible secular trend of increasing incidence of IDDM. There were a total of 269 new cases of IDDM or 27 cases/year, with standard deviation (SD) of +/- 5.3, range 18 to 33, and no secular trend at all. Sex distribution was quite variable, having male:female (m/f) ratios from 1:2 to 3:1, and an over-all m/f of 13.3/13.6. Incidence of IDDM per month of year went from a low of 1.7 for April and November to a high of 3.0 for March and 2.9 for September, 2.8 for October, with a mean of 2.24 +/- 0.48 cases/month. Again, no significant seasonal differences were noted. Mean incidence for our "cold" months (November to April was 2.25/month; for our "hot" season (May to October) was 2.3/month; for our "rainy" season (July to December) 2.3 and for our "dry" season (January to April) 2.3/month. Thus, it appears that there are no such seasonal trends of IDDM In Puerto Rico.

A syndrome of familial intrathyroidal primary parathyroid hyperplasia: case reports and critical review of literature.

Intrathyroidal hyperplastic parathyroid glands were responsible for primary hyperparathyroidism (PHPT) in two of three members in a family. The third had an extrathyroidal parathyroid "adenoma". Both intrathyroidal parathyroid (IThP) hyperplastic glands were the largest ones removed at the time of surgical cure. A review of the literature confirmed our postulate of a higher incidence of familial cases among patients with hyperparathyroidism and IThP with an incidence of 10.34% of IThP in familial cases versus a 4.2% in non-familial cases with PHPT. This contrasts with an incidence of 0.1% of IThP in normal patients. We hypothesize that stimulation of IThP tissue by surrounding calcitonin-producing C-cells might play a role in the seemingly preferential IThP hyperplasia. Recognition of this syndrome of Familial IThP Hyperplasia is important in order to avoid unnecessarily aggressive surgery for hyperparathyroidism.

An update on the discovery, pathophysiological actions, clinical manifestations and possible physiology of parathyroid related peptide.

PTHrP has had an unidentified role in medicine since 1930, when Albright described a patient with renal cortical cell carcinoma with hypercalcemia. Since then hypercalcemia has been recognized as the most common paraneoplastic syndrome. At that time the concept of "ectopic PTH syndrome" was introduced, and remained in literature until the true etiology was finally described. In the early 1970's Roof and Benson presented evidence that PTH in humoral hypercalcemia differed from "authentic" PTH. This marked the starting point for researchers to try identifying the molecule that mimicked PTH action and structure. This molecule, named parathyroid-related peptide, has been associated to hypercalcemia seen with solid tumors, such as squamous cell carcinoma of the lung and renal cortical cell carcinoma. PTHrP has been demonstrated to have similar actions to PTH but to differ in decreasing osteoblastic activity while increasing osteoclastic activity. The more fascinating finding was the presence of the PTHrP genes throughout the body, mostly the lactating breast as well as the heart, lungs and skin among others. Despite its identification, finding its physiological roles on normal tissue still remains to be clarified.

Fine needle thyroid aspiration: university hospital experience.

Fine needle thyroid aspiration (FNTA) as a diagnostic tool of thyroid disease was started at the University District Hospital (UDH) in 1983. FNTA was performed in 54 willing patients from December 1984 through December 1985. If no medical contraindications existed, thyroid surgery was recommended in order to assess diagnostic accuracy. Surgery was done in 34 (63%) patients; while not in 20:3 for medical reasons, 2 lost to follow-up, 3 refused, 7 had inappropriate FNTA, and 5 for unknown reasons. None had complications to FNTA. Cytology was classified as Class O: inadequate; Class 1: benign; Class 2: indeterminate; and Class 3: malignant. "Non-benign" cytology was obtained in 14 patients: 7 indeterminate (class 2) and 7 malignant (class 3). Four of these had carcinoma confirmed by surgery. Of the 19 with benign FNTA (class 1) only one has thyroid carcinoma. One patient had inadequate sample (Class O). The sensitivity was 80%, specificity 65%, and diagnostic accuracy 67%. All these statistical values are within the range reported in fifteen reviewed series. Thus, FNTA at UDH had a diagnostic value comparable to that described in the literature. It may be utilized as a safe, reliable tool complementary to clinical data in order to reduce the amount of unnecessary thyroid surgery, carry out earlier diagnoses of malignant nodules and increase the yield of carcinoma among those operated.

The lumbar and femoral bone mineral densities in a normal female Puerto Rican population.

The aim of this study was to establish the reference values of the bone mineral density (BMD) of the lumbar spine and the proximal femur in a normal adult female Puerto Rican population. In the selection of the population, exclusion criteria included diseases, use of drugs and toxic habits (smoking and alcoholism) known to affect bone and mineral metabolism. The study population comprised 131 healthy females, ages 20 to 69 years. Spinal radiographs were taken of women age 60 or more to exclude spinal defects or artifacts. The bone mineral densities were measured using the dual energy X Ray absorptiometer (DEXA, Hologic model 1000). The peak BMD was found in the lumbar spine at the age 30 to 39 years and at the age 20 to 29 years in the femoral neck, trochanter and Ward's triangle. The decrease in BMD from peak values to those at age 60 to 69 years were 18% for the lumbar spine, 16.3% for the femoral neck, 30.1% for the Ward's triangle and 12.4% for the trochanter. The major fall in the BMD was related to the menopause and compares with that of other series. There was no statistical difference from Hologic reference values in a mixed population of the USA.

Clinical profile of 128 subjects operated for primary hyperparathyroidism.

From 1960 to 1990, one hundred twenty eight (128) subjects with primary hyperparathyroidism were operated in the University Hospital. The medical records were reviewed. Serum and urine chemistries were done by conventional methods, serum PTH was done by RIA's (N-, C-, and midregion) and intact by IRMA and 1,25 dihydroxycholecalciferol by a non equilibrium receptor assay from calf thymus and preceded by double Sep-Pak chromatography. The distal third of the radius (nondominant arm) was used to evaluate radial bone density (RBD), using single photon absorptiometry (Norland) and the lumbar bone density (LBD) was measured by dual energy X Ray absorptiometry (DEXA). The RBD was done in 41 females and 15 males and the LBD in 12 females and 4 males. The series comprised 95 females, age range from 15 to 79 years, and 33 males, age range from 14 to 69 years. Prominent clinical features included nephrolithiasis in 72 subjects (56%), osteitis fibrosa cystica in 2, isolated familial hyperparathyroidism in 4 subjects in one family, 7 subjects with MEN-1 in 3 families, and 4 subjects with MEN-2 in one family. Only 7 subjects were asymptomatic. Serum calcium was elevated in all, serum alkaline phosphatase was elevated in 24% and urinary hydroxiproline was increased in 48%. Serum phosphorus was low in 92%. PTH assay was either elevated or inappropriately normal for the serum calcium in all patients tested. Serum 1,25 D was elevated in 57%. The PTH level was positively correlated with the serum calcium (r = 0.70), but had no significant correlation with the serum phosphorus and the 1,25 D. The RBD expressed as the standard deviation from that of the mean for age and sex matched controls was > or = 2 SD below the mean in 39% of females and in 40% of males. In contrast to the RBD none of the subjects tested had a LBD > or = 2 SD below the age and sex adjusted mean. 103 subjects had adenomas, 20 primary hyperplasia, 2 carcinomas and in 3 surgical exploration was unsuccessful. As to the outcome of Surgery, 117 (93%) were cured. Thus, in this series, successful surgery for primary hyperparathyroidism is the rule. Primary hyperparathroidism is rarely asymptomatic and appendicular bone disease and nephrolithiasis are commonly seen.

Pheochromocytoma: a twenty year experience at the University Hospital.

During the past 20 years (1970-90), we had 24 patients with pheochromocytoma: 19 diagnosed clinically and 5 post-mortem. Their ages ranged from 17 to 74 (mean, 43.2 years). Males (n = 14) outnumbered females (n = 10), a 1.41:1 M:F ratio. A majority were symptomatic (95%), with a typical triad of headaches, palpitations and diaphoresis. Most frequent finding was hypertension (95%). It was sustained in 60% and paroxysmal in 35%. In 6 patients (25%) pheochromocytomas were bilateral, all familial. Fifteen were solitary adrenal tumors (63%); 3 (12.5%) were extra-adrenal: 2 intra-abdominal, and 1 cardiac paraganglioma of right atrium. Of 6 familial cases, 4 were associated to Von Hippel-Lindau (VHL) disease, while 2 were multiple endocrine neoplasia (MEN-II) patients. All familial cases were bilateral and in the adrenals. There were no malignancies. Among the 19 clinical cases pre-operative Dx was made by positive urine VMA or catecholamines urine levels: (95 and 100% sensitivity respectively). Preoperative visualization by CT or MRI was done in 62% of the most recent patients. In 5 earlier cases the diagnosis was made post mortem: 3 died of cerebral hemorrhage, 1 with a pons infarct and 1 with congestive heart failure (CHF). There were 2 post-operative deaths and another died 13 years later from thyroid medullary carcinoma. Of the 19 operated, 13 (68%) were cured. Thus pheochromocytomas retain considerable morbidity and some mortality. These rare tumors constitute a clinical diagnostic challenge yet a rewarding therapeutic experience for the alert physician.

Unusual cases of hyperparathyroidism.

The history, physical and radiologic findings, treatment and pathology in five unusual cases of hyperparathyroidism is presented. The hyperparathyroidism was caused by a large (113 grams) mediastinal adenoma in the first patient, who is alive 25 years after surgery. A parathyroid carcinoma with compression of the esophagus was documented in the second patient. This patient is alive and normocalcemic 23 years after surgical treatment. A third patient with hyperplasia returned with hypercalcemia 20 years postsurgery requiring reoperation. A fourth patient with advanced bone findings was found to have a parathyroid adenoma. The fifth case is a patient with tertiary hyperparathyroidism secondary to hypophosphatemic rickets.

[Hormonal treatment of cancer of the prostate]. / Tratamiento hormonal del cáncer de próstata.

We set forth the results obtained after application of a hormonal protocol in prostate carcinoma (P.C.) on 257 patients controlled between June 1976 and June 1987, of whom we have selected 160 who fulfilled the following requirements: under 80 years old, confirmed anatomopathological diagnosis, state equal to or above the T1 of the Union International contre le Cancer (U.I.C.C.) classification, tolerance of treatment applied, clinical, analytical and complete, systematic iconographic follow up and minimum survival of more than one year. We treated those patients with localised P.C. (they have no demonstrable metastases), who in our series numbered 78 (78/160), with Diethylethylbestrol (D.E.S.) at an orally administered dose of 1 mgr. a day. In these the plasma testosterone dropped below 100 nanograms/l. in 57 cases (57/79). In this case metastases appeared in 22 cases (22/78). We treated those patients with metastatized P.C., who in our series numbered 59 (59/160), with orally administered Estramustine Phosphate (Estracyt) at a dose of 560 mgr. every 24 h. in two goes. In these the plasma testosterone dropped below 100 nanograms/l. in 50 cases (50/59). In this group the metastases disappeared in 7 patients, became stabilized in 30 and worsened in the other 22 patients. We carried out surgical orchiectomy on 49 patients (49/160): in 17 cases due to associate vascular pathology, in 13 cases for sociocultural reasons, in 5 cases because of advanced age and in 14 cases it was conducted on patients with a poor response to D.E.S.

[Renal mass as the initial manifestation of a metastasized oat-cell]. / Masa renal como primera manifestación de un oat-cell metastatizado.

Renal metastasis of localized tumours in the economy are rare. In our series of 162 renal tumours only 3 were metastatic. Even more unusual is that the metastasis should be the presenting form. Such is the case we are reporting: a 59 year-old male complaining from back pain and toxic syndrome. Supplementary examinations were performed sequentially until arriving at an open biopsy which confirmed the oat cell histology. The paper explains the results from the various examinations. The ominous prognosis of this histology is illustrated.

[Malignant neoplasms and kidney transplantation]. / Neoplasias malignas y transplante renal.

In 661 renal transplantations, 2 potentially migrated tumours (0.38%), 5 preexisting neoplasias (0.76%), and 31 "de novo" tumours were seen in 29 patients (4.4&). Although of very low incidence, the likelihood of tumour migration from elderly donors, given the circumstances surrounding removal, offers a high risk. None of the preexisting neoplasias relapsed following transplant. The highest prevalence was seen in skin (40%), lung (13%), kidney (13%) and bladder (6.6%) "de novo" tumours. Incidence of lymphoma was low. Dominant etiological factors of the recipient were older age, effective and tolerated immunosuppression, viral infections, environmental agents and antigenic stimulation of the graft. Skin lesions have responded well to local treatment, without need to discontinue immunosuppression, a measure that is mandatory in other malignant tumours. Also, the conclusions of a round table during the 25th National Meeting of Urotransplantation of the Spanish Association of Urology held in 1994 on "Oncology and Renal Transplantation" are presented.

[BCG in superficial carcinoma of the bladder. Authors' experience]. / BCG en el carcinoma vesical superficial. Nuestra experiencia.

Contribution of 95 patients with surface vesical tumour managed with UTR and endovesical BCG. The response was absence of recurrence in 68.4% cases in Ta stage, 63% in T1 and 66.7% in 'in situ' carcinoma. Therapy tolerability was good, with few side-effects. We conclude that BCG endovesical instillation as co-adjunctive therapy to UTR in surface vesical tumours has been shown to be effective for the period under study.

Localization of parathyroid lesions: blind study for surgeons.

Success in the treatment of primary hyperparathyroidism rest in the accurate localization and removal of the diseased gland or glands. Computerized tomography and nuclear imaging scans are being used to localize abnormal parathyroid tissue. In the present study, fifteen consecutive patients undergoing surgery for primary hyperparathyroidism were all subjected to these ancillary studies. Results were not revealed to the operating team. In all instances an adenoma was localized during neck exploration. CT Scan failed to localize 73% of the affected glands. Nuclear scans missed almost fifty percent of the parathyroid adenomas. The low yield of these ancillary localizing tests makes them unnecessary in the routine evaluation of patients undergoing surgery for primary hyperparathyroidism.

Burkitt's lymphoma treatment in a rural hospital in Sierra Leone.

BACKGROUND: Sierra Leone is a low-income sub-Saharan country in the endemic Burkitt's lymphoma (BL) belt. We performed a prospective trial of a reduced-intensity chemotherapy protocol for the treatment of paediatric BL. PATIENTS AND METHODS: The trial included all children clinically diagnosed with BL between 2005 and 2008. Biopsy, bone-marrow aspiration, analysis of cerebrospinal fluid, abdominal ultrasound and plain x-ray of involved sites were performed when feasible. The treatment protocol was a first i.v. dose of cyclophosphamide (CPM) 40 mg/kg, followed by oral CPM weekly for two doses and then bimonthly to a total of six doses. Treatment was based on clinical diagnosis as it was several weeks before pathology results were available. RESULTS: Eighty-seven patients were included, with a median age 7 years and 4 months; 59/87 (67.8%) were boys. Nearly half (n = 17, 42.5%), presented with moderate or severe malnutrition. Biopsy was performed in 44 patients, BL being verified in 36 (41.4% of all patients). Most children presented with advanced disease: 28 (32%) at stage II, 47 (54%) at stage III and 12 (13.8%) at stage IV. Most patients (71/87, 82%) initially responded to treatment, but just over half (47/87, 54%) experienced relapse and refractory disease. Forty patients (46%) in complete or partial clinical response were lost to follow-up. CONCLUSION: The outcome for BL in rural Sierra Leone according to this protocol is poor. Low-dose CPM was ineffective. Constraints on performing complete diagnosis and staging, frequency of advanced disease at presentation and a high drop-out rate might explain our poor results.