Cytogenetic analysis of hypophyseal adenoma. Study of 9 cases and review of the literature]

We present an easy alternative to enhance rhinoplasty in patients with wide nasofrontal angles. By implanting PTFE (GORE-TEX) in the glabella region, we obtained a significant change in nasal shape. Twenty-seven patients were treated with this technique with no complications and with good results. The material showed excellent biocompatibility, matching the host tissues perfectly.     

Heterotopic replantation

In multidigital amputations, it is sometimes better to replant an amputated finger to a different proximal part if a better function can be expected in this position. In our clinical material between October 1991 and March 1994, heterotopic replantation was performed in twelve digits in eleven patients. Three fingers were replanted to the thumb, three to the index, four to the middle, and two to the ring fingers. The functional results were satisfying. The total active range of motion was on an average 24% of a normal finger. Static two-point discrimination was 8.5 mm on an average, and the values for the Semmes-Weinstein test ranged between 3.61 and 6.5. A heterotopic replantation is of special value for primary thumb reconstruction.     

Heterotopic ossification

Acetyl-CoA carboxylase (ACC1) catalyzes the first and rate limiting step of de novo fatty acid synthesis. Defects in Acc1p were recently correlated with an altered structure/function of the nuclear envelope in yeast. The subcellular distribution of the enzyme was determined in wild-type and mutant cells by cell fractionation and confocal immunofluorescence microscopy. Even though fatty acid synthesis is generally considered to be a cytosolic reaction, we found that Acc1p cofractionated with nuclei and the ER (endoplasmic reticulum) marker BiP/Kar2p. Membrane-bound Acc1p was susceptible to proteinase K digestion and was solubilized by mild salt treatment indicating that it is loosely associated with the cytosolic surface of the nuclear ER membrane. Consistent with these observations, immunofluorescence analysis revealed that Acc1p was distributed in a gradient within the cytoplasm that had its highest concentration around the ER. Possible association of Acc1p with the nuclear pore complexes (NPCs) was investigated in strains that display NPC clustering. Results of these experiments suggest that Acc1p localization is independent of NPC distribution. We propose that association of Acc1p with the cytoplasmic surface of the ER membrane is physiologically relevant to "channel" the enzymatic product of Acc1p, malonyl-CoA, to a putative ER-localized fatty acid chain elongase complex.     

Unusual approaches to hypophyseal adenomas

For pituitary adenomas surgery, rhinoseptal transsphenoidal approach is used in 98 to 99% of the cases. Although this approach is fitting for microadenomas and the majority of macroadenomas, some of them develop extensions in the nasal fossas, the posterior cranial fossa, the suprasellar region, or into the cavernous sinus and will require other approaches. For the superior routes, the frontopterional approach gives good control of the suprasellar region, the anterior and middle base of the skull. The tumor dissection is performed inside the concavity of the chiasm and between the internal carotid artery and the optic nerve (optico-carotid approach). The frontopterional approach is used for superolateral extensions, especially in the lateral fissure. The bifrontal basal inter hemispheric approach, through a medial frontal bone flap tangential to the base, gives a good route to the suprasellar region and behind the dorsum, and also for tumors extended in the third ventricle in case of prefixed chiasm. For the inferior routes, the participation of ENT or craniofacial surgeons is a great help. The transfacial or transethmoidal approach performs a hollowing of the nasal fossas and gives a large interorbital tunnel adapted for tumors extended in the rhinopharynx and the ethmoid. The Le Fort I maxillary osteotomy offers also a large approach for adenomas extending in the rhinopharynx. The transcavernous approach from Dolenc, for adenomas progressing in the cavernous sinus requires a long and difficult procedure. The progression of some adenomas in many directions may require a combined approach in one or two procedures.     

Medical treatment of secretory hypophyseal adenomas

Despite much research on the relationship between marital status and health, the confounding effects of marital selection are not well understood. Even less is known about 'adverse selection', the phenomenon that people with poor health or health related attributes have a higher chance of marriage. Using data from the National Child Development Study, a longitudinal study of the 1958 British birth cohort, this paper examines the effects of factors that can select single people into early or later marriages. The selection factors are from three domains, namely, health status, socioeconomic status, and risk-taking behaviour. It is found that, from age 16-23 y, adverse selection is prevalent. People from a lower socio-economic background and smokers are more likely to be married. This has the potential to suppress any association between marriage and health. Meanwhile, men with medical problems are less likely to marry. At ages from 23-33 y, adverse selection reduces and those who have better life chances are more likely to get married. This has the potential of creating a spurious relation between marriage and health. These findings shed light on the understanding of the confounding effects of marital selection according to different life stages.     

Idiopathic hypophyseal hyperplasia: diagnosis by magnetic resonance

INTRODUCTION: Hypophyseal hyperplasia is an uncommon disorder in which the gland increase in size due to excessive proliferation of strings of normal cells, which usually secrete prolactin. Different aetiologies may cause this disorder of the hypophyseal gland. However, in a small number of cases the hyperplasia is not due to any of these aetiologies and is therefore known as idiopathic hypophyseal hyperplasia. There are few references in the literature to idiopathic hypophyseal hyperplasia with hyperprolactinaemia. Usually diagnosis is reached after treatment for a hypophyseal adenoma, since the clinical features are similar. CLINICAL CASES: We present three cases seen in our department, in which hormone and endocrine studies were done to exclude known causes of hyperplasia, together with CT and MR scans. We analyzed the behaviour of hypophyseal hyperplasia by using imaging techniques, and the differential aspects with regard to hypophyseal adenomas. Firm diagnosis is only made on anatomopathological study of the hypophysis. However, we consider that sound knowledge of the characteristics of this condition may help to establish the correct diagnosis and thus avoid unnecessary surgery. CONCLUSIONS: We review the information published in the literature on this subject, emphasizing the importance of differential diagnosis by means of imaging techniques.     

Heterotopic ossification after femoral intramedullary rodding

Heterotopic ossification (HO) after femoral intramedullary rodding is a significant complication of the procedure. One hundred eighteen cases of femoral roddings performed on 113 patients were available for review. The data were computerized and evaluated using univariate analysis and multivariate regression analysis. A statistically significant increase of HO was found with male gender, increased delay to surgery, and in patients requiring prolonged intubation because of their multiple injuries. HO was classified using a modified version of the method of Brumback et al. (grades 0-IV). A strong correlation of HO with brain injury documented by computed tomography scan was also found to be statistically significant for the more severe grades of HO. This group of patients had not previously been identified as being at high risk for HO.     

Hormones of the posterior region of the hypophyseal gland

Arginine vasopressin is the main hormone involved in the regulation of body fluid osmolality. The hormone is released by the posterior pituitary whenever water deprivation causes an increased plasma osmolality or whenever the cardiovascular system is challenged by hypovolaemia and/or hypotension. The main site of action of this hormone is the renal collecting duct, but vasopressin is also a potent vasopressor and neurotransmitter, it has a role in the secretion of corticotrophin, in the regulation of the cardiovascular system, temperature and other visceral functions. Vasopressin also promotes the release of coagulation factors by vascular endothelium and increases platelet aggregability. In addition to its classical contractile effect on uterine myometrial and mammary glandular myoepithelial cells, oxytocin acts as neurotransmitter, stimulates endometrial prostaglandin production, pituitary prolactin secretion, luteolysis, sperm transport and natriuresis, and may play a role in immune function. Sensorial stimuli arising from the cervix and vagina as well as stimulation of the breast can induce secretion of oxytocin from the posterior pituitary. There are many vasopressin and oxytocin analogues (agonists and antagonists) that are synthetized with the goal of increasing duration of action and selectivity for the receptor subtypes, while non-peptide antagonists are orally active. The oxytocin and the vasopressin V1a, V1b and V2 receptors have recently been cloned and shown to form a sub-family within the large superfamily of G-protein-linked receptors. Renal V2 receptors mediate vasopressin-induced water reabsorption via induction of intracellular cAMP production in collecting duct cells. Most remaining actions of vasopressin on blood vessel constriction, liver glycogenolysis, platelet adhesion, adrenal angiotensin II secretion and certain brain functions are mediated via V1a-type receptors that are coupled to a Gq/11 protein. V1 receptor activation leads to stimulation of phospholipases C, D and A2, and an increase in intracellular calcium. Vasopressin stimulates pituitary corticotrophin release via a third vasopressin receptor type (V1b) which is present in corticotrophs. Oxytocin induces myometrial contraction, endometrial prostaglandin F2a production, mammary gland milk ejection, renal natriuresis and specific sexual, affilitative and maternal behaviours via oxytocin receptors which are also coupled to a G1/11 protein. Although only one oxytocin receptor type has been cloned so far, recent binding studies indicate that uterine endometrial oxytocin receptors may constitute a distinct receptor subtype. Expression of oxytocin receptors have relevant up- and down-regulation by oestrogens and progesterone.     

Thyrotropin-secreting pituitary adenoma

Thyrotropin (TSH)-secreting pituitary adenoma (TSPA) is a rare cause of hyperthyroidism and detailed reports of this entity in Taiwan are uncommon. We report a patient with TSPA with symptoms of hyperthyroidism and describe the presentation, endocrine and histologic findings, and treatment. The patient, a 42-year-old man, presented with a 2-year history of weight loss, palpitation, anxiety, and bad temper. He had increased basal serum thyroxine (T4, 18.3 micrograms/dL) and triiodothyronine (T3, 250 ng/dL) concentrations. The TSH concentration was normal (4.6 microIU/mL) and showed impaired response to stimulation by TSH-releasing hormone. Tests for antithyroid antibodies were negative. Thyroid scintigraphy showed mild thyroid enlargement. The thyroid uptake of radioactive iodine (131I) was high at 2 hours (34%) and 24 hours (63%) after 131I administration. Other serum hormone concentrations were within normal limits. Magnetic resonance imaging of the brain showed a microadenoma in the pituitary region. Octreotide and bromocriptine tests showed 78.4% and 58.3% inhibition of TSH, respectively. The patient underwent trans-sphenoidal pituitary tumor excision, and the symptoms of hyperthyroidism subsided after surgery. Six months after the operation, there was no evidence of recurrence of the tumor or symptoms of hyperthyroidism. Hormonal supplements were also not necessary. In conclusion, TSPA is a rare cause of hyperthyroidism. However, in patients with symptoms of hyperthyroidism and increased basal serum T1 and T3 concentrations, but normal or even elevated serum TSH concentrations, TSPA should be considered in the differential diagnosis.     

Neonatal pharyngeal pseudodiverticulum mimicking esophageal atresia

Pseudodiverticulum of the hypopharynx is an infrequent but potentially serious complication of orogastric or nasogastric tube insertion and endotracheal intubation. We report two cases of injury to the hypopharynx resulting in a pseudodiverticulum of the hypopharynx that was initially diagnosed as esophageal atresia. Both cases were managed successfully by conservative therapy. We also review the literature regarding neonatal pharyngeal pseudodiverticulum.     

Endoscopic stapling diverticulotomy of pharyngeal pouch

PURPOSE: Homozygosity for Hb D-Punjab (or Hb D-Los Angeles; codon 121; GAA-->CAA) is rare among Arabs. The co-inheritance of Hb D with beta(0)-thalassemia trait is even rarer, with only 10 previous cases reported worldwide. PATIENTS AND METHODS: We present clinical and hematological data for two Hb D homozygotes and three compound heterozygotes for Hb D-Punjab and beta(0)-thalassemia (IVS-II-1; G-->A). All the individuals belong to a consanguineous Kuwaiti Arab family. The hemoglobin variant and the beta-thalassemia allele were characterized by sequencing, allele-specific amplification, and oligonucleotide hybridization. RESULTS: The hematology was unremarkable except for a moderate elevation of Hb F (3-4%) and significant hypochromia and microcytosis in the subject with Hb D/beta(0)-thalassemia. CONCLUSION: This report confirms the benign nature of homozygosity for Hb D.     

Tubular apocrine adenoma

Two cases of Gilbert's syndrome and three cases of other diseases showed abnormally low plasma indocyanine green (ICG) clearance as contrated with normal or nearly normal plasma sulfobromphthalein (BSP) clearance. To evaluate the role of serum proteins in pathogenesis of these cases, ICG-binding patterns of serum proteins of the patients were compared with that of normal persons by the method of Sephadex G200 column chromatography. No qualitative differences in binding patterns were observed between serum proteins of the patients and normal persons. From these results, serum proteins were thought to be minor determinants of the hepatic ICG uptake mechanism. Because of normal or nearly normal plasma BSP clearance in these patients, the contents of hepatic organic anion-binding proteins, Y and Z, were thought to be sufficient. Possible qualitative abnormalities of the Y and/or Z proteins were also discussed.     

Heterotopic periarticular ossification after total hip replacement]

Fractures of the proximal femur represent a significant health concern especially in the elderly. Fatigue damage and microfractures have been implicated in the etiology of hip fractures; however, the extent to which these factors are sufficient to bring about significant reductions in proximal femur strength and stiffness is unknown. This study examined the hypothesis that fatigue loading of the proximal femur results in highly correlated decreases in bone stiffness and strength through the accumulation of bone microdamage. One canine femur from each of 10 pairs was monotonically loaded to failure to determine the ultimate strength. The contralateral femur was then cyclically loaded at 50% of the ultimate load value for either 3600 cycles or until a 40% reduction in stiffness was achieved. This femur was then monotonically loaded to failure. For two additional femur pairs, the fatigued femur was histologically processed to reveal bone microdamage. In support of the hypothesis, the data demonstrated a linear relationship between strength loss and stiffness loss (Adj. R2 = 0.79, p < 0.0004) with significant decreases in residual whole bone strength (p < 0.004) following cyclic loading. In addition, damage (microcracks) in the cortical bone and broken trabeculae were observed in the neck and head region of the femur fatigued until its stiffness was reduced by 40% but not fractured subsequent to cyclic loading.     

Seasonal regulation of prolactin secretion in hypophyseal stalk transected beef calves

Conformational transitions of holo-alpha-lactalbumin in a hydro-ethanolic cosolvent system was studied by spectrofluorescence, CD in near- and far-uv regions, and high-sensitivity differential scanning calorimetry. Experimental results allow us to propose that in isothermal conditions alpha-lactalbumin undergoes a number of conformational transitions with increasing ethanol concentration: N<=>I<=>D<=>H. The existence of I-state was deduced from spectrofluorometric and near-uv CD data. In this state the aromatic chromophores of the amino acid side chains are more accessible to the solvent displaying higher local mobility. The H-state was detected from far-uv CD spectra as a state corresponding to the content of alpha-helices higher than originally found in native protein. However, calorimetric measurements provide data revealing only the two-state mechanism of alpha-lactalbumin unfolding in both water and in aqueous ethanol solutions. This indicates that the energy levels of N- and I-states as well as of D- and H-states are similar. Thermodynamics of the unfolding of alpha-lactalbumin in hydroethanolic solutions was analyzed with the help of the linear model of solvent denaturation. Unfolding increments of enthalpy, entropy, and Gibbs energy of transfer of the protein from a reference aqueous solution to hydro-ethanolic solutions of different concentrations were determined from the calorimetric data. They are linear functions of molar ethanol fraction. The slope of the unfolding increment of Gibbs energy of transfer was calculated from data on transfer of amino acid residues taking into account the average solvent accessibility of amino acid residues in the native structure of small globular proteins, using the additive group contribution method.     

Thyroid dysfunction in uremia: evidence for thyroid and hypophyseal abnormalities

Disturbances in thyroid function and a high prevalence of goiter develop in patients on chronic hemodialysis. This study shows that in patients on dialysis, mean serum thyroxine and triiodothyronine levels are lower than normal. Patients with chronic renal failure not on dialysis, have mean serum thyroxine levels similar to normal subjects and low mean serum triiodothyronine levels. However, both serum thyroxine and triiodothyronine concentrations decrease as the renal failure worsens. In addition, both groups of patients with renal failure have a decreased serum thyroxine response to oxogenous thyrotrophin and a diminished serum thyrotrophin response to thyrotrophin-releasing hormone. These data suggest the presence of an intrathyroidal and an hypophyseal defect in uremic patients. Although serum iodide concentrations are elevated, there is no correlation between the level of serum iodide and the degree of renal failure. Therefore, we have no direct evidence that iodide excess is responsible for the abnormalities observed.     

Hypopituitarism following extirpation of a pharyngeal pituitary

Vaginal blood flow changes in women were evaluated qualitatively by heat-flow discs mounted on cooled vaginal probe. Statistically significant increases in vaginal blood flow, systolic blood pressure, and pulse rate were found in seven normal subjects during a 4 minute immersion of one hand in ice water.