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Biomedical subjects

I Halperin

Publications and source records attributed to I Halperin.

At least 37 records · Page 2Linked to original sources

Congenital panhypopituitarism of late onset.

A 29-year-old male was diagnosed congenital panhypopituitarism due to pituitary hypoplasia, absence of pituitary stalk and ectopic neurohypophysis. This report, together with bibliographic review, may induce to reconsidering isolated GH failures, partial hypopituitarisms and panhypopituitarisms which have up to now been interpreted as idiopathic. The availability of new image techniques has allowed a better definition of the anatomic substrate of these alterations, and more subtle hormonal studies have pointed out, in some cases, their possible hypothalamic origin. On this basis, some of these cases may be interpreted as presenting the same alteration as those described in necropsy of newborns dead due to severe panhypopituitarism. Therefore, a unique clinical picture with various degrees of severity may be postulated.

Adult↗

Primary localized cutaneous amyloidosis and familial medullary thyroid carcinoma.

We have studied a family with an autosomal dominant inheritance of primary localized cutaneous amyloidosis (PLCA) and familial medullary thyroid carcinoma (MTC). Ten family members were screened for multiple endocrine neoplasia (MEN) 2; five were found to have MTC and two had C-cell hyperplasia. None had evidence of phaeochromocytoma or parathyroid abnormalities. Five of these seven patients presented characteristic interscapular hyperpigmented lesions, showing dermal amyloid deposits in two of the four patients in which a biopsy was performed. The data are analysed in the light of two recent reports of MEN 2A associated with identical lesions. We conclude that PLCA should be sought in MTC patients, even if no other endocrinopathies are present. This may be informative of the familial character of MTC in index cases and also of the tumour gene status in family members who are being screened.

Adult↗

Persistent B19 parvovirus infection in patients infected with human immunodeficiency virus type 1 (HIV-1): a treatable cause of anemia in AIDS.

OBJECTIVE: To determine the role of B19 parvovirus in red cell aplasia of patients infected with human immunodeficiency virus type 1 (HIV-1). DESIGN: Uncontrolled clinical trial, with assay of serum, peripheral blood cells, and bone marrow for virus using DNA hybridization and immunocytochemistry techniques; these assays were then correlated with clinical findings, results of immunoassays for antivirus antibodies, and with immunoglobulin (Ig) therapy. SETTING: Government medical referral center, and university and private hospitals. PATIENTS: Seven patients with pure red cell aplasia and serologic evidence of infection with HIV-1. MEASUREMENTS AND MAIN RESULTS: All patients had giant pronormoblasts in the bone marrow (present in transient aplastic crisis caused by acute B19 parvovirus infection). High concentrations of B19 parvovirus were demonstrated in sera, in several cases in samples separated by weeks or months. Viral DNA and capsid protein were present in the bone marrow of three patients studied, and active viral replication was detected by southern analysis. There was no antivirus IgG in capture immunoassay and no or very low levels of antivirus IgM. The patients did not have symptoms of fifth disease, the illness caused by this virus in immunologically normal persons. Six patients were treated with a regimen of intravenous commercial immunoglobulin. In all cases, this therapy resulted in rapid reduction in serum virus concentrations and full recovery of erythropoiesis. Relapses in two cases were predicted by DNA hybridization studies, and these cases were successfully retreated. CONCLUSIONS: The B19 parvovirus is a remediable cause of severe chronic anemia in HIV-infected patients. Recognition of and therapy for parvovirus in this population will avoid erythrocyte transfusion and should prevent transmission of the virus to other persons, including immunosuppressed persons and women of child-bearing age.

Acquired Immunodeficiency Syndrome↗

Acute water intoxication after intranasal desmopressin in a patient with primary polydispsia.

Only a few cases of severe acute water intoxication (AWI) due to intranasal desmopressin have been reported, none of which occurred in patients with primary polydipsia. We describe a case of AWI with semicoma and convulsions, due to intranasal desmopressin, in a 32-year-old patient with dipsogenic diabetes insipidus. Previous reported cases of AWI due to desmopressin are discussed. The importance of ruling out primary polydipsia when this drug is used, not only for central diabetes insipidus but also for other current indications such as classic hemophilia, is stressed.

Administration, Intranasal↗

Evaluation of clinical and hormonal effects in hirsute women treated with ketoconazole.

Seven hirsute women were treated with 200 mg/12 h po of ketoconazole during 6 months. We evaluated clinical and hormonal effects during this period. Ketoconazole treatment decreased testosterone, androstenedione and dehydroepiandrosterone sulfate, while 17-alfahydroxyprogesterone increased. This finding suggests a steroidogenic blockade at the level of C17-20 lyase. We did not observe changes in LH, SHBG and cortisol values. In spite of this findings after 6 months treatment, hirsutism remained unmodified. We conclude that ketoconazole inhibits androgen synthesis in hirsute women, but the use of this drug as an alternative and effective treatment of hirsutism warrants further evaluation.

17-alpha-Hydroxyprogesterone↗

[Evaluation of the treatment of Cushing's syndrome using ketoconazole].

The effectiveness of ketoconazole for the therapy of Cushing's syndrome, both of adrenal and pituitary origin, was retrospectively evaluated. To this end, the clinical and hormonal results in 5 patients were assessed. There were 4 females and one male, with ages ranging between 17 and 64 years. Three of them, who had Cushing's disease, received sustained treatment with ketoconazole when other therapeutic measures had failed. The two remaining patients (one with Cushing's disease and the other with adrenal adenoma) were treated with this drug in the preoperative period to alleviate the deterioration in the clinical condition caused by hypercortisolism. The clinical evolution of the patients is described, as well as the plasma adrenocorticotropin (ACTH) and the free cortisol in 24 hour urine in the subsequent controls. In view of the study results, we feel that ketoconazole is an effective treatment for the long term control of Cushing's syndrome. In addition, it may be also useful as preoperative therapy in those patients severely deteriorated by the sustained hypercortisolism.

Adolescent↗

Chronic lymphocytic leukemia with hypothalamic invasion.

Headaches and a state of confusion developed in a patient with chronic lymphocytic leukemia (CLL). A computed tomography (CT) scan of the head showed a hyperdense lesion in the hypothalamus with obstruction of the foramina of Monro. Leukemic cells with monoclonal lambda B-cell markers were identified in the ventricular fluid at the time of surgical decompression. Similar cells were identified in the peripheral blood. This lesion has not been described as a complication of CLL, a disease that, unlike other leukemias, rarely has central nervous system manifestations.

Aged↗

[Cushing's disease in a patient with primary empty sella turcica].

Primary empty sella is a neuroanatomical condition which is more common in middle aged obese multiparous females with long-standing hypertension. Usually there are no symptoms, but occasionally nonspecific headache may be present. Hormone studies are commonly normal in these patients, although several functional hypothalamic and pituitary abnormalities have been reported. We report a 64-year-old female with primary empty sella in whom Cushing's disease was diagnosed. The association of both disorders is exceptional.

Cushing Syndrome↗

Late-onset hypocalcemia appearing years after thyroid surgery.

We revised four cases of hypocalcemia diagnosed yr after thyroid surgery, including 1 man and 3 women, operated 5-23 yr before the onset of symptoms, which ranged from mild paresthesia to convulsive seizures. Total serum calcium levels ranged from 1.1 to 2.05 mmol/l, and PTH levels were low in the 3 cases in which they were measured. Hypoparathyroidism appearing as late-onset hypocalcemia after extense thyroid surgery is a poorly understood condition, perhaps not as unfrequent as it is considered.

Adult↗

Diabetogenic effect of nifedipine.

This case report describes a 60-year-old diabetic patient whose daily insulin requirements increased by 30 percent following nifedipine administration. Glucagon and intravenous glucose tolerance tests were performed with and without nifedipine treatment, in order to evaluate the roles of decreased pancreatic beta islet cell function and augmented insulin peripheral resistance in the diabetogenic effect of nifedipine. Insulin and calculated glucose peripheral utilization extrapolated from the glucose concentration curves were not significantly different. C-peptide levels tended to be lower with nifedipine treatment at baseline and during the glucagon tests. This may suggest that the altered glycemic control associated with nifedipine was mediated by a suppressed islet beta cell function. The effect of calcium channel-blockers upon glycemic control and the possible mechanisms involved are discussed.

Blood Glucose↗

A case with persistent nephrogenic diabetes insipidus following parathyroidectomy for hyperparathyroidism.

A 25-year-old woman with persistent nephrogenic diabetes insipidus (NDI) following parathyroidectomy for primary hyperparathyroidism is described. NDI is a well recognized complication of primary hyperparathyroidism, generally imputed to hypercalcemia, and promptly reversible after correcting it. In our case, the impaired concentrating ability of the renal tubule was irreversible after the removal of the parathyroid adenoma and the correction of the hypercalcemia, presumptively due to the morphological irreversible changes in the kidney. In addition, hypercalciuria persisted and was the cause of a compensatory hyperparathyroidism. Treatment with thiazide diuretic was effective to decrease relative hypercalciuria, thus reversing the compensatory hyperparathyroidism.

Adenoma↗

Mediastinal lymph node metastases from gastrointestinal carcinoma.

Mediastinal and hilar lymphadenopathy secondary to gastrointestinal metastases rarely have been reported and have been considered to occur only in conjunction with lymphangitic lung spread. The current report is of 15 cases of hilar and mediastinal metastases secondary to pancreatic, gastric, and colonic primary tumors. In only three of these cases was lymphangitic lung involvement noted. Possible pathways of spread of these tumors to the mediastinal lymph nodes are discussed.

Autopsy↗

Diagnosis of lung carcinoid with cutaneous hyperpigmentation eight years after bilateral adrenalectomy.

A 26-yr-old male was submitted to bilateral adrenalectomy in 1977 for Cushing's syndrome. Some months later he developed intense skin hyperpigmentation together with increased ACTH levels (149 to 4000 ng/l). The sellar region was always normal in X-ray studies. In April 1985, when the patient complained of chest pain, a chest x-ray showed a polycyclic mass in the upper left lobe of the lung. ACTH ranged from 20,000 to 100,000 ng/l, with no response to CRF or cyproheptadine administration. Urinary 5-OH-indolacetic acid was negative. Thoracotomy was performed in July 1985 with resection of two intrapulmonary masses. Histologic study demonstrated a carcinoid tumor, with positive neuron-specific enolase and ACTH immunochemical stain. ACTH concentration in tumoral tissue was 91 pg/g tissue. After surgery ACTH fell dramatically to 37 ng/l, and has remained at this level since then, associated with resolution of the skin hyperpigmentation.

Adrenalectomy↗

Treatment of pituitary macroadenomas secreting PRL, HGH or ACTH with long-acting bromocriptine.

Long-acting im bromocriptine was administered to 7 patients with pituitary macroadenomas (4 acromegalics, 1 Nelson's syndrome and 2 prolactinomas), with good tolerance except during the first 24 h. During a 42-day period hormonal, CT-scan and visual field variations were followed. In acromegalics HGH decrease was not evident, except in some isolated sample. In Nelson's syndrome ACTH showed a 94% fall on day 14, even though a spontaneous oscillation cannot be ruled out, and recovery took place from day 21 on. PRL remained undetectable in both. In prolactinomas, PRL suffered a great decrease (91.8% and 96.3% on days 21 and 28 respectively) and remained well below its initial values up to the end of the study, in spite of partial recovery. In these 2 patients CT-scan evidenced shrinkage of tumor mass, which was not observed in the remaining 5 cases. Visual fields did not improve in the 2 cases initially affected (Nelson's syndrome and 1 prolactinoma). Long-acting bromocriptine seems to have the same therapeutic uses of the oral form with the possible advantage of a better tolerance of full initial doses.

Acromegaly↗