Persistent hyperinsulinemic hypoglycemia of infancy: case report

OBJECTIVE: To report a case of Persistent Hyperinsulinemic Hypoglycemia in twins which is a situation not yet reported in the literature. METHODS: Report of seizures in identical twins, from consanguineous parents, with persistent hypoglycemia as cause of the seizures. Laboratory tests, performed for etiological investigation of the hypoglycemia, included thyroid hormones (T4/TSH), insulin, cortisol, growth hormone, stimulation test with glucagon (to evaluate the insulin/glucose relation), and histopathological study of the pancreas. RESULTS: Laboratorial investigation revealed a persistent hypoglycemia with hyperinsulinism which were confirmed with the stimulation test with glucagon. The histopathological exam showed a persistence of first generation pancreatic islet, confirming the diagnosis of Persistent Hyperinsulinemic Hypoglycemia in Infancy (the new denomination of Nesidioblastosis). CONCLUSION: Although rare, this condition must be early suspected early in the evaluation of hypoglycemia of the young infant, even out of the neonatal period, specially if the parents are consanguineous. The adequate therapy must be quickly initiated in order to prevent neurological damage.     

A bronchogenic cyst of the right ventricular endocardium

In a 73-year-old male patient with a history of prostate cancer, a right ventricular endoluminal tumor was diagnosed by echocardiography. An endocardial papillary fibroelastoma or myxoma appeared possible; a malignant tumor could not be ruled out. The tumor was resected using extracorporeal circulation and cardioplegic arrest. Histopathology study revealed a bronchogenic cyst with ciliated epithelium.     

Possibilities in improvement of glucocorticoid treatments in asthma with special reference to loteprednol etabonate

Allergic conditions contribute significantly to the burden of chronic disease in the industrialized world. The increasing prevalence has lead research into the discovery and development of various new therapeutic strategies. Despite considerable efforts of the pharmaceutical industry, the leukotriene antagonists were the only new class of asthma treatments to be licensed in the past 30 years. Topical glucocorticoids (GCs) are the most potent and effective therapy for treating allergic diseases. However, their use is limited by diverse undesired effects. Changes in pharmacokinetic parameters of GCs may be an interesting and promising approach to improve efficacy and safety of inhaled GCs. Loteprednol etabonate has been developed on the basis of the retrometabolic drug design. In animal studies, it has been demonstrated to have long-lasting anti-allergic (anti-asthmatic) effects without influencing the hypothalamic-pituitary axis (HPA). This soft steroid is now in phase III of the clinical development. Recently, loteprednol has been proven to be effective in the management of allergic rhinitis (400 microg once daily). No suppression of HPA was observed at clinically effective and higher doses. In conclusion, loteprednol as the first representative of soft steroids elicits marked anti-inflammatory effects, but has no impact on endocrine responses. It may represent a promising new therapy in the treatment of allergic rhinitis and asthma.     

Polycythaemia vera: will new markers help us answer old questions?

The publication of the first molecular markers for polycythaemia vera (PV) has led to a renewed interest in finding the mutations leading to the development of this myeloproliferative disorder. A substantial amount of cell biological data on the PV stem cell, however, have been available for over two decades. This review will summarise, combine and integrate both fields of investigation. Based on this analysis, two alternative models for the development of myeloproliferative disorders are proposed.     

Usefulness of troponin I as a marker of myocardial injury after pediatric cardiac catheterization

Cardiac troponin I (cTnI) is a sensitive and specific marker of myocardial injury. The degree of myocardial injury associated with pediatric cardiac catheterization is unknown. We sought to investigate cTnI after pediatric cardiac catheterization, and to evaluate the degree of elevation observed with specific types of interventions. Seventy-three pediatric catheterizations were evaluated. Diagnostic procedures and interventions not expected to cause myocardial injury were assigned to group I, whereas interventional procedures expected to be associated with cardiac injury were assigned to group II. Group II procedures were further subdivided based on type of intervention. Serum samples were obtained before and after all procedures and analyzed for cTnI. Postprocedure cTnI levels were compared across groups and correlated with age and weight. Procedures in group II were associated with significantly higher cTnI levels than group I (median 2.65 ng/ml; interquartile range 0.9 to 4.9 ng/ml for group II vs 0.3; 0.3 to 1.6 ng/ml for group I, p <0.001). Within group II, cTnI was inversely correlated with age (p <0.05) and weight (p <0.05). Radiofrequency catheter ablation (RFA) caused higher cTnI levels than other types of interventions (median 3.7 ng/ml; 1.9 to 9.5 ng/ml for RFA vs 1.75; 0.7 to 4.9 ng/ml for non-RFA, p <0.05). Most pediatric interventional catheterization procedures are associated with myocardial injury, as evidenced by elevation of cTnI, with RFA causing higher levels than other interventions. Conversely, most diagnostic procedures are associated with no detectable myocardial injury. When compared with adult studies, pediatric patients seem to be at higher risk for myocardial injury from interventional cardiac catheterization.     

Submandibular tumor

Ohne Zusammenfassung V.Tr?ger Klinik für Hals-, Nasen-,Ohrenheilkunde, Unfallkrankenhaus,Warener Stra?e 7, 12683 Berlin, E-Mail: Vanessa.Tr?ger@UKB.de     

Modulation of synaptic transmission by nociceptin/orphanin FQ and nocistatin in the spinal cord dorsal horn of mutant mice lacking the nociceptin/orphanin FQ receptor

Nociceptin/orphanin FQ (N/OFQ) and nocistatin (NST) are two neuropeptides derived from the same precursor protein that exhibit opposing effects on spinal neurotransmission and nociception. Here, we have used whole-cell, patch-clamp recordings from visually identified neurons in spinal cord dorsal horn slices of genetically modified mice to investigate the role of the N/OFQ receptor (N/OFQ-R) in the modulatory action of both peptides on excitatory glutamatergic and inhibitory glycinergic and gamma-aminobutyric acid (GABA)-ergic synaptic transmission. In wild-type mice, N/OFQ selectively suppressed excitatory transmission in a concentration-dependent manner but left inhibitory synaptic transmission unaffected. In contrast, NST reduced only inhibitory but not alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) receptor-mediated excitatory synaptic transmission. N/OFQ-mediated inhibition of excitatory transmission was completely absent in N/OFQ-R receptor-deficient (N/OFQ-R(-/-)) mice and significantly reduced in heterozygous (N/OFQ-R(+/-)) mice, whereas the action of NST on inhibitory neurotransmission was completely retained. To test for the relevance of these results for spinal nociception, we investigated the effects of intrathecally injected N/OFQ in the mouse formalin test, an animal model of tonic pain. N/OFQ (3 nmol/mouse) induced significant antinociception in wild-type mice, but had no antinociceptive effects in N/OFQ-R(-/-) mice. These results indicate that the inhibitory action of N/OFQ on excitatory glutamatergic synaptic transmission and its spinal antinociceptive action are mediated via the N/OFQ receptor, whereas the action of NST is independent of this receptor.