[Relapsing polychondritis : A rare differential diagnosis in clinical practice]. / Rezidivierende Polychondritis : Eine seltene Differenzialdiagnose im klinischen Alltag.

Relapsing polychondritis (RPC) is a chronic immune-mediated inflammatory, systemic disease primarily leading to structural damage and impaired function of cartilage tissue. However, the systemic inflammatory process in RPC can also affect sensory organ structures, the respiratory tract, the nervous and cardiovascular systems as well as the kidneys. The immune-mediated disease leads to recurrent inflammatory attacks causing a progressive degradation of elastic and hyaline cartilage structures, especially in the ears, nose, larynx, trachea and diarthrodial joints. However, other connective tissue structures in the eye and the heart valves may also be involved. The RPC is regarded as an orphan disease as the number of reported cases has so far remained confined to approximately 600 worldwide. The rarity of the disease has limited systematic clinical studies and the available empirical data are exclusively derived from casuistic studies or evaluation of small case series. The therapeutic interventions depend on the extent and localization of the disease manifestation. Thus, nonsteroidal anti-inflammatory drugs (NSAID), glucocorticoids and immunosuppressive agents with conventional synthetic disease-modifying antirheumatic drugs (DMARD) have been demonstrated to be beneficial. More severe and refractory diseases may require a targeted pharmacological intervention with biologic DMARDs.

Clozapine does not induce a motor impairment in operant responding for heat reinforcement.

This experiment examined the effects of intraperitoneal (i.p.) clozapine (CLZ) compared to haloperidol (HAL) on operant responding for heat reinforcement in a cold environment (-8 degrees C). Three doses of CLZ (1, 3 and 5 mg/kg) were found to dose-dependently increase responding for heat while lowering core temperature (T(c)) only at the highest dose. Three doses of HAL (0.1, 0.3 and 0.5 mg/kg) dose-dependently decreased operant responding which resulted in a dose-dependent decrease in T(c). The highest dose of CLZ was then tested in two other paradigms: a reinforcement schedule in which heat was available as long as the lever was held down, and a temperature gradient (range 7-45 degrees C) in which access to heat required minimal motor effort. The ad libitum reinforcement schedule still did not provide enough heat to overcome the hypothermic effects of CLZ. However, in the gradient, rats receiving CLZ selected a warmer region of the gradient, and T(c) was higher than that of controls. These data support CLZ's reputation for having minimal motor side effects. Unlike HAL, the hypothermic effects of CLZ appear to be unrelated to effects of the drug on movement.

Nonhepatosplenic gamma delta T-cell lymphoma with initial testicular compromise.

We report here a case of nonhepatosplenic gammadelta T-cell lymphoma with undescribed initial localization in testis, without hepatosplenomegaly or adenopathies, and subsequent development in the maxillary sinus. The maxillar mass biopsy revealed a T-cell infiltration, and its immunologic characterization by flow cytometry showed a gammadelta T-cell phenotype (CD45+, CD3+, CD2+, TCR gammadelta+), without expression of CD7, CD5, CD1a, TdT, CD4, CD8, TCR alphabeta, or NK antigens (CD16, CD56, and CD57). Clonal gamma-chain gene rearrangement by polymerase chain reaction (PCR) was detected in testicular and maxillar biopsies. Epstein-Barr virus type 1 (EBV) sequences were detected by molecular biology in the biopsy material, suggesting that this oncogenic virus may play a role in the genesis of the clonal expansion of gammadelta T-cells. The patient was initially treated with standard chemotherapeutic protocols, with poor response and aggressive course.

A hyperglycemic peptide hormone from the Caribbean shrimp Penaeus (litopenaeus) schmitti.

From a crude extract of the sinus glands of the shrimp Penaeus (litopenaeus) schmitti a peptide with hyperglycemic activity in a homologous bioassay was isolated and characterized by a combination of automatic Edman degradation, enzymatic digestions, TLC of dansyl-amino acids, and mass spectrometry. Its M(r) is 8359.4 Da by MS, which coincides with the deduced sequence. Its N-terminus is free and its C-terminus is amidated. It has 6 Cys residues in conserved positions compared with other known CHHs. This is the first sinus gland hormone from an Atlantic Ocean shrimp characterized to date. It has a remarkable 90% sequence similarity to the Indo-Pacific shrimp P. (marsupenaeus) japonicus Pej-VII hyperglycemic hormone.

Promyelocytic blast crisis of chronic myelogenous leukaemia with translocations (9;22) and (15;17).

The promyelocytic blast crisis is a rare form of transformation during the evolution of chronic myeloid leukaemia (CML). We report a case of promyelocytic blast crisis with t(15;17) in addition to t(9;22). The morphology and immunophenotype of the blasts were similar to those seen in acute promyelocytic leukaemia (APL). The t(15;17) was confirmed by FISH. The patient had evidence of coagulopathy with clinical and laboratory findings of disseminated intravascular coagulation (DIC). This report highlights the importance of correlating the results of multiple diagnostic methods in order to establish a correct diagnosis of the promyelocytic blast crisis of CML.

[Chronology of portal hypertension, decreased sodium excretion, and activation of the renin-angiotensin system in experimental biliary cirrhosis]. / Cronología de hipertensión portal, disminución de excreción de sodio y activación del sistema renina-angiotensina en cirrosis biliar experimental.

OBJECTIVES: 1) To evaluate the biochemical, renal, histological and splanchnic and systemic hemodynamic abnormalities induced by bile duct obstruction in rats, and 2) to study the temporal relationships between the start of portal hypertension, decrease of urinary sodium excretion and activation of the renin-angiotensin system. METHODS: Bile duct obstruction was induced in 127 male Wistar rats, and renal function, hemodynamic, biochemical and liver histology were evaluated at weeks 1, 2, 3 and 4 after complete bile duct obstruction; the data were compared to that in 30 control rats. RESULTS: Portal pressure significantly increased at week 1 (11.7 +/- 1.5. vs. 7.8 +/- 1.5 mmHg, p < 0.05) while the mean arterial pressure remained stable until week 4 when a slight decrease was observed (91.3 +/- 6.6 vs. 96.1 +/- 8.6 mmHg in control rats). A significant decrease in urinary sodium excretion was observed at week 1 (1.1 +/- 0.5 mEq/24 h) compared to control rats (2.3 +/- 0.6 mEq/24 h). In addition, hyperreninemia was observed at week 1 (5.1 +/- 0.2 vs. 2.4 +/- 1.3 ng Ang l/mL/h, p < 0.05) and hyperaldosteronism at week 2 (103 +/- 46 vs. 25.1 +/- 8.8 ng/24 h, p < 0.05) compared to control rats. CONCLUSION: A temporal relationship between the beginning of portal hypertension and a decrease of renal sodium excretion, hyperreninemia and hyperaldosteronism was observed in bile duct ligated rats. This experimental model could be used to evaluate the effects of new drugs to prevent biliary cirrhosis including the abnormalities in the renal handling of sodium.

Pigmentation of the rainbow trout (Oncorhynchus mykiss) with oil-extracted astaxanthin from the langostilla (Pleuroncodes planipes).

The effect of oil-extracted astaxanthin from the red crab or langostilla (Pleuroncodes planipes) on the growth and pigmentation of forty five rainbow trouts (Oncorhynchus mykiss) was investigated by feeding a test diet supplemented with 75 mg/kg of astaxanthin during six weeks and compared with a control diet. The oil-extraction process of the pigment is described. Weight, flesh astaxanthin concentration, and color (L*, a*, b*) of the flesh were measured at 0, 3, and 6 weeks. No apparent effect of astaxanthin supplementation was observed on fish development. In spite of the low free astaxanthin amount in the diet (8%), an acceptable carotenoid concentration in the flesh (3.60 +/- 0.78 mg/kg, w/w), and a red hue (H(ab)o = 44.13 +/- 2.36) were obtained at the end of the study. The red hue was strongly correlated with the carotenoid concentration (r = 0.98).

Complete primary structure of the molt-inhibiting hormone (MIH) of the Mexican crayfish Procambarus bouvieri (Ortmann).

The amino acid sequence of MIH was elucidated by means of digestions with specific proteases, manual Edman degradation, and mass spectrometry. MIH consists of a 72-residue peptide chain (molecular mass 8322 Da) with six cysteines forming three disulfide bridges that connect residues 7-43, 23-39, and 26-52. It has blocked N- and C-termini and lacks tryptophan, histidine, and methionine. MIH shows striking similarity to the crustacean hyperglycemic hormone (CHH) isomorphs of Procambarus bouvieri (90% identity) and to the MIH from Homarus americanus (79% identity) and Penaeus vannamei (46% identity). It is also related to the MIH from Carcinus maenas (28% identity) and Callinectes sapidus (28% identity).

Amino acid sequence of the minor isomorph of the crustacean hyperglycemic hormone (CHH-II) of the Mexican crayfish Procambarus bouvieri (Ortmann): presence of a D-amino acid.

The primary structure of the neurohormone crustacean hyperglycemic hormone (CHH-II) was determined by means of enzymatic digestions, manual Edman degradation, and mass spectrometry. CHH-II is a 72 residue peptide (molecular mass 8388 Da), with six cysteines forming three disulfide bridges that connect residues 7-43, 23-39, and 26-52. The peptide has blocked N- and C-termini, and lacks tryptophan, histidine, and methionine. The CHH-I and CHH-II of Procambarus bouvieri have identical sequences and elicit levels of hyperglycemia that are not distinguishable. The difference between the two isomorphs consists in a posttranslational modification of a L-Phe in CHH-I to a D-Phe in CHH-II at the third position from the N-terminus.

Primary structure of the major isomorph of the crustacean hyperglycemic hormone (CHH-I) from the sinus gland of the Mexican crayfish Procambarus bouvieri (Ortmann): interspecies comparison.

The amino acid sequence of this neuropeptide was elucidated by means of a combined approach of enzymatic digestions, manual and automatic Edman degradations, and mass spectrometry. It is a 72 residue peptide (molecular mass 8388 Da), with six cysteines forming three disulfide bridges connecting residues 7-43, 23-39, and 26-52, with blocked N- and C-termini, and lacking the amino acids histidine, methionine, and tryptophan. The CHH-I of Procambarus bouvieri is compared with the other known CHHs from Orconectes limosus (98.6% identity), Homarus americanus isomorph A (83.3% identity), Homarus americanus isomorph B (79.2% identity), and Carcinus maenas (61.1% identity).

Diastema closure: a case report.

This clinical study describes two combined modalities of treatment, orthodontic and porcelain laminate placement, to facilitate diastema closure. Geristore, a dual-cure fluoride-releasing composite was mixed to bond orthodontic brackets in place. H6 elastic bands were used with the orthodontic brackets to close the diastemas sufficiently and to allow the placement of Cerinate porcelain laminates to produce a beneficial cosmetic effect.

Actinomycin D blocks the hepatic functional albumin mRNA increase in aminonucleoside-nephrotic rats.

Hepatic functional albumin-mRNA was measured in the following groups of rats: (a) puromycin aminonucleoside (PAN)-nephrotic rats, (b) PAN-nephrotic rats treated with actinomycin D prior to sacrifice, (c) control rats, and (d) control rats treated with actinomycin D. Albumin mRNA was translated in an mRNA-dependent cell-free system from rabbit reticulocyte lysate. Albumin-mRNA increased about 2-fold in PAN-nephrotic rats. This increase was abolished in vivo in PAN-nephrotic rats treated with actinomycin D. Albumin mRNA was not significantly modified in control rats treated with actinomycin D. These data suggest that the increased level of hepatic functional albumin mRNA observed in PAN-nephrotic rats in vivo was due mainly to the increased rate of albumin gene transcription.

Thyroid dysfunction in a prospectively followed series of patients with progressive systemic sclerosis.

Thirty-nine patients with progressive systemic sclerosis (PSS) in stable clinical conditions were extensively evaluated for the presence of thyroid disease. Two patients had previously undetected hypothyroidism while 7 additional patients had normal serum thyroid hormone levels but an exaggerated TSH response to thyrotropin-releasing hormone (TRH) administration, consistent with subclinical hypothyroidism. Four of the 9 subjects with abnormal TRH responses had positive antithyroid antibodies and of the remaining 5, 4 had been on chlorambucil or prednisone. Basal TSH and TSH response to TRH were significantly higher in PSS patients as a group when compared to a control group and increased with increasing duration of PSS. Serum antithyroid antibodies (antithyroglobulin and/or antimicrosomal antibodies) were positive in 18% and thyroid scans were abnormal in 18% of the patients. The euthyroid sick syndrome was not seen. Our findings indicate an increased frequency of, sometimes previously unsuspected, clinical and subclinical hypothyroidism in stable PSS patients which appears to be autoimmune in nature and becomes more prevalent with increased PSS duration. Careful and regular monitoring of the thyroid function in PSS patients is advisable.