Objectives To investigate the relationship between congenital nephrotic syndrome (CNS) and neonatal polycythemia, and clinical diagnosis and treatment. Methods The clinical manifestations, diagnosis, and treatment of a case of CNS with neonatal polycythemia were retrospective analyzed, and the related literature were reviewed. Results A male infant had abdominal distention after his birth, followed by feeding intolerance and poor response. On the third day of birth, he was diagnosed of neonatal polycythemia according to the levels of hemoglobin (249 g/L) and hematocrit (0.714 L/L). And after a partial exchange transfusion, the symptoms were improved. On the sixth day of birth, the infant had edema, urinary protein +++, serum albumin at 12.7 g/L and blood cholesterol at 8.84 mmol/L, and was clinical diagnosed of CNS. Oral hormone therapy was ineffective and he died 32 days after birth. Conclusions CNS combined with neonatal erythrocytosis is rare in clinic. The co-existing of the two is more likely to induce thromboembolism and organ dysfunction, and the clinical prognosis is poor.

This study sought to probe into the mechanism of spontaneous contraction of portal vein. The morphological and electrophysiological characteristics of the freshly isolated interstitial cells (ICs) of rabbit portal vein were investigated by using immunohistochemical and conventional whole-cell patch clamp techniques. The isolated interstitial cells exhibited stellate-shaped or spindle-shaped bodies with a variable number of thin processes projecting from cell bodies, and these cells were noted to be c-Kit immunopositive. Under conventional whole-cell patch clamp configuration, the membrane potential was held at -60 mV, the spontaneous rhythmic inward currents were recorded in ICs, and the frequencies of which were similar to those of spontaneous contraction of portal vein. The inward currents were insensitive to nicardipine (an L-type calcium channel blocker) but could be abolished by gadolinium (a non-selective cation channel blocker). The results suggested that the spontaneous rhythmic inward currents recorded in freshly isolated ICs may be pacemaker currents which elicit the spontaneous contraction of portal vein.
Action Potentials ; Animals ; Electrophysiology ; Female ; Interstitial Cells of Cajal ; physiology ; Male ; Muscle, Smooth, Vascular ; physiology ; Periodicity ; Portal Vein ; cytology ; physiology ; Rabbits ; Transient Receptor Potential Channels ; metabolism

Objective To explore the role of adenosine monophosphate-activated protein kinase (AMPK), a key regulator of cellular energy metabolism, in vascular smooth muscle cell (VSMC) migration in response to physiological cyclic stretch. Methods The Flexcell-5000T mechanical loading system was applied with a physiological cyclic stretch at 10% amplitude and 1.25 Hz frequency to primary rat VSMCs, to simulate mechanical stimulation of VSMCs in vivo. The protein expression of p-AMPK in VSMCs was detected by Western blotting, and VSMC migration was detected by wound healing test. Results Compared with the static group, physiological cyclic stretch loading for 24 h significantly decreased the area of wound healing, indicating that physiological cyclic stretch inhibited VSMC migration. The protein expression of p-AMPK in VSMCs was increased significantly after physiological cyclic stretch loading for 3 h, and was decreased significantly after 24 h. Under physiological cyclic stretch loading conditions, incubating AMPK inhibitor could significantly reduce the protein expression of p-AMPK after 3 h, and promote VSMC migration after 24 h; incubating AMPK activator AICAR under static conditions significantly increased the protein expression of p-AMPK after 3 h, and weakened VSMC migration after 24 h. Conclusions Physiological cyclic stretch inhibits VSMC migration by increasing the protein expression of p-AMPK, indicating that VSMC migration regulated by physiological cyclic stretch is of great significance for maintaining vascular homeostasis.