INSULIN AND GLUCAGON SECRETION IN HEPATIC GLYCOGENOSES

ABSTRACT. Okada, S., Seino, V., Kodama, H., Yutaka, T., Inui, K., Ishida, M., Yabuuchi, H. and Seino, Y. (Department of Pediatrics, Osaka University Hospital, Fukushima-ku, Osaka and The Third Division, Department of Medicine, University of Kobe, Kobe, Japan). Insulin and glucagon secretion in hepatic glycogenoses. Acta Paediatr Scand 68: 735, 1979.—Insulin and glucagon secretion was investigated in ten patients with hepatic glycogenosis, types I and III, in order to understand the relationship between hypoglycemia and pancreatic function. In all patients, both oral glucose tolerance and intravenous arginine infusion tests revealed hypoinsulinemia. Decreased urinary C-peptide levels with standard food intake also supported hypofunction of pancreatic beta cells. On the contrary, the normal secretion pattern of glucagon in both types indicated in the arginine loading test, intact alpha cells in the pancreas. Persistent hypoinsulinism, which is apparently an adaptation to hypoglycemia, could be an important cause of nutritional dwarfism in both types of glycogenosis. The usefulness of the measurement of urinary C-peptide, which evaluates the pancreatic function and provides management for normal body growth, is discussed.     

Electrophysiological Effects of Intravenous Disopyramide Phosphate on the Wolff-Parkinson-White Syndrome

The effects of a single intravenous infusion of 2mg/kg body weight disopyramide phosphate (DP) on the mode of initiation of reentrant supraventricular tachycardia were assessed in seven patients with Wolff-Parkinson-White (WPW) syndrome using bundle of His electrograms and the ventricular extrastimulus method. The delta wave disappeared in three patients after DP. However, retrograde conduction via the accessory pathway persisted even after DP administration in all patients. These effects contributed to the induction of reciprocating tachycardia after DP. The retrograde functional refractory period of the His-Purkinje system (HPS) and the effective refractory period of the accessory pathway were increased in all cases and contributed to the development of reentry HPS. After DP, the zone of reentry HPS widened in four cases (including a newly developed case) and remained unchanged in three cases. Reentrant supraventricular tachycardia zones widened in three cases; these widened reentrant supraventricular tachycardia zones were induced by the widened reentry HPS, that is, reentry HPS was followed by the reentrant supraventricular tachycardia. This study demonstrates that persistence of retrograde accessory pathway conduction and widened reentry HPS which might be dose-related after DP could be the retrograde determinants affecting the reentrant supraventricular tachycardia zone.     

Torsion of the hernia sac within a hydrocele of the scrotum in a child

Torsion of the hernia sac is a rare disease that presents as acute scrotum in children. Including the present case, only six cases have been reported in the English literature. We report a 10-year-old boy who presented with pain and swelling of his right scrotum. Ultrasonography revealed a hypoechoic region adjacent to the normal right testis. The inflammatory changes of the right scrotum deteriorated. The patient underwent surgery and a necrotic cyst was recognized within a hydrocele of the scrotum. The cyst was not connected with the testis or epididymis and was twisted at an angle of 270 degrees. The cause of the necrotic cyst observed was anatomical and pathological torsion of the hernia sac.     

Megacystis and megacolon in an infant with Ehlers-Danlos syndrome

We report a 14 month old male infant with Ehlers-Danlos syndrome who became ‘anuric’ due to an acutely dilated urinary bladder. Although the patient was also found to have megacolon, no diverticulum was seen in his gastrointestinal tract or urinary bladder. In order to decompress the urinary bladder and colonic wall, we put an indwelling urinary catheter in place for 2 months, and carried out daily glycerin enema for 3 months. All urological and gastrointestinal symptoms subsided with this intensive medical treatment. The diagnosis of megacystis and megacolon was made very early in life for this patient. This may indicate that the striking extension of gastrointestinal and bladder wall may lead to the development of diverticula of gastrointestinal and urinary tracts in later life.     

Long-Term Results of Cryoablation with a New Cryoprobe to Eliminate Chronic Atrial Fibrillation Associated with Mitral Valve Disease

The purpose of this study was to examine the performance of a new cryoprobe in the treatment of chronic atrial fibrillation (AF) associated with mitral valve disease. The study included 66 patients undergoing mitral valve replacement. The mean AF duration was 9.0 ± 9.0 years and mean left atrial (LA) was diameter 57 ± 10 mm. Cryoablation (−60°C) was applied to four pulmonary vein (PV) orifices over 2–3 minute. The spherical tip (2-cm in diameter) of the cryoprobe is capable of ablating the left atrium near the PV, as well as the PV ostium with a single cryoablation. After cryoablation, mitral valve surgery or a combined surgical procedure were performed in 66 patients. There were no intraoperative complications. Sinus rhythm was restored in 60 patients (91%) immediately after the operation. Recurrent AF was treated with antiarrhythmic drugs and/or direct current cardioversion in 43 patients (72%). At discharge, 48 patients (72%) were in sinus rhythm. During a mean follow-up period of 31 ± 16 months, 40 patients (61%) were in sinus rhythm with (29) or without antiarrhythmic drugs (11). In patients in sinus rhythm at the end of the follow-up period, the duration of preoperative AF duration was significantly shorter (P < 0.05) and the preoperative LA diameter and cardiothoracic ratio were significantly smaller than in patients who were in AF (both for P < 0.005). Using this new cryoprobe, sinus rhythm was restored and maintained in 61% of patients with chronic AF and mitral valve disease with a 12–15 minute cryoablation procedure.     

Alpha-delta sleep in a case with non-24 h sleep-wake syndrome: Quantitative electroencephalogram analysis of alpha and delta band waves

Abstract  Four all-night polysomnograms of a 39-year-old male patient with non-24 h sleep-wake syndrome were recorded. We analysed electroencephalograms (EEG) with the power spectrum method and the wave pattern recognition analysis of Fujimori. The EEC of the rest waking condition showed normal patterns. High-voltage diffuse alpha band waves were observed in sleep stages 2, 3 and 4. The integrated area of the alpha band waves in the analysis epochs showed a strong positive correlation to the delta hand components in the power spectrum of the same epoch during sleep (correlation coefficients r = 0.762–0.815). Alpha hand waves during sleep were clearly different from the alpha waves in the rest waking condition, with respect to slower peak frequency and the frontal dominant voltage distribution.     

N-Acetyl-L-γ-glutamyl Derivatives of p-Nitroaniline,Sulphamethoxazole and Sulphamethizole for Kidney-specific Drug Delivery in Rats

Kidney-specific delivery of p-nitroaniline, sulphamethoxazole and sulphamethizole after either intravenous administration of the L-γ-glutamyl or N-acetyl-L-γ-glutamyl derivatives or the parent drugs has been examined in a rat model. All L-γ-glutamyl derivatives were converted to the corresponding parent drugs within 60 min whereas the N-acetyl-L-γ-glutamyl derivatives were fairly stable in the systemic circulation after parenteral administration. Concentrations of p-nitroaniline and sulphamethoxazole 20 min after administration of the parent drugs were somewhat higher in the kidney than in the liver and lung. The concentration of sulphamethizole in the kidney was dramatically higher than those in the hepatic and pulmonary tissue. Kidney-specific delivery of the drugs of interest was evaluated by determining the tissue concentrations of the released parent drug and the total drug levels (i.e. drug levels after hydrolysis of all conjugate to the parent drug). For L-γ-glutamyl-p-nitroaniline released renal levels of p-nitroaniline and total p-nitroaniline concentrations were both higher than those obtained after p-nitroaniline dosing. Use of L-γ-glutamylsulphamethoxazole resulted in higher total sulphamethoxazole concentrations in the kidney, but did not lead to an increase in released (unconjugated) sulphamethoxazole levels. In contrast, no kidney-selective distribution was observed for L-γ-glutamylsulphamethizole. Markedly increased kidney distribution was observed for both N-acetyl-L-γ-glutamyl-p-nitroaniline and N-acetyl-L-γ-glutamylsulpha-methoxazole and the liver and lung concentrations were correspondingly reduced in comparison with parent drug dosing. Use of the N-acetyl-L-γ-glutamyl-p-nitroaniline conjugate increased the concentration of p-nitroaniline in the kidney to the same extent as did L-γ-glutamyl-p-nitroaniline. In conclusion, N-acetyl-L-γ-glutamyl derivatization of certain compounds seems to be useful for kidney-specific ***drug delivery and preliminary data suggests that lipophilic drugs are better substrates than hydrophilic compounds. Results related to the selectivity of tissue distribution of the derivatives and species differences are discussed.