Case report: Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt

We present a case of portal-systemic encephalopathy due to a congenital splenorenal shunt. A 69 year old woman was admitted to hospital because of recurrent episodes of disturbed consciousness. The present episode had begun 3 months prior to admission. Although the patient demonstrated mildly slurred speech, the remainder of her neurological examination was unremarkable. She showed no clinical signs of portal hypertension and her liver function, except for a serum hepaplastin test of 58% and an ICG retention rate of 28% at 15 min, was normal. Her serum ammonium level was 210 μg/dL. The venous phase of a superior mesenteric arteriogram revealed a splenorenal shunt and narrowing of the portal vein, which was 4 mm in diameter. The histological findings, demonstrated by a needle liver biopsy specimen, were consistent with mild fibrosis and lymphocytic infiltration. Following the diagnosis of a splenorenal shunt in the absence of liver cirrhosis, ligature of the shunt was performed with a splenectomy. The portal vein pressure after ligature of the shunt rose from 12.5 to 18.8 mmHg. This shunt was thought to be of congenital origin. The high preoperative serum ammonia concentration decreased to the normal range postoperatively and the serum hepaplastin test and ICG retention rate similarly improved postoperatively. A follow-up superior mesenteric arteriogram was performed during the venous phase, demonstrating resolution of the shunt and decreased portal vein narrowing. The patient has suffered no further episodes of disturbed consciousness postoperatively.     

Successful differentiation of Spitz naevus from malignant melanoma by microfluorometric analysis of cellular DNA content

Two cases of presumed Spitz naevus, whose diagnosis on clinical and histological grounds was uncertain, were examined for cellular DNA content using the technique of DAPI-DNA microfluoromety. They were compared with 20 cases, respectively, of clinically and histologically confirmed, Spitz, naevus, malignant melanoma and acquired pigmented naevus. The two Spitz naevi showed a diploid pattern in a distribution histogram of cellular DNA content. The pattern was similar to that of confirmed Spitz naevi and of acquired pigmented naevi but different from the aneuploid pattern of malignant melanomas. DNA index values of the two cases were within the range of confirmed Spitz naevi and different from those of malignant melanomas. The DAPI-DNA microfluorometric method thus provided confirmatory evidence for the diagnosis of Spitz naevus. The method appears to reflect sensitively the biological behaviour of tumour cells, and is a useful aid to the diagnosis of uncertain Spitz naevi.     

A girl having congenital chloride diarrhea treated with spironolactone for seven years

We report on a girl having congenital chloride diarrhea (CCD) who has been followed for 7 years and 6 months sequentially. Dilated intestinal loops, marked enlargement of the abdominal circumference of the fetus and hydramnios were noted by ultrasound examination at 31 weeks of gestation. After delivery by cesarean section for hydramnios, she excreted profuse watery yellow green stools with marked abdominal distension. At 4 months of age, hypochloremia, hyponatremia and a high concentration of chloride in the stool were identified. She was diagnosed as having CCD. Because it was difficult to administer a large volume of potassium chloride (KCl), and sodium chloride (NaCl), we decided to administer spironolactone. After administration of spironolactone, we could generate correct serum electrolytes using less amounts of KCl. At 7 years and 6 months of age, her body size was within normal limits and her intellectual, mental and physical development had been normal. In spite of normal serum electrolytes, blood pH and the presence of chloriduria, secondary hyperaldosteronism was noted. We consider that spironolactone may be useful to decrease the amount of KCl administration in the neonatal period, but frequent measurements of renin, angiotensin and aldosterone would be necessary for adequate control in CCD cases.     

Effect of beclomethasone dipropionate on nasal metachromatic cell sub-populations

Abstract. The effect has been investigated of local administration of beclomethasone dipropionate (BDP) on cell numbers of nasal epithelial metachromatic cell (NMC) sub-populations. Twenty-one patients with perennial allergic rhinitis were studied in four groups according to the duration of treatment or after treatment with BDP. Nasal scrapings were taken after 1 week (Group 1) or 2 weeks (Group 2) of BDP treatment, or after discontinuing BDP for 1 week (Group 3) or 2 weeks (Group 4). Cells were fixed with Mota's lead acetate or 10% buffered formalin followed by toluidine blue staining to count the number of NMC and to classify these according to morphological subtypes (basophils or mast cells). Formalin-sensitive mast cells and basophils in nasal scrapings were reduced more than formalin-resistant mast cells with BDP treatment. Formalin-sensitive mast cells were also more prompt to recover from BDP than formalin-resistant mast cells. The results suggest that the formalin-sensitive NMC is a sub-population of cells which responds to BDP treatment in allergic rhinitis.     

The nocturnal secretion of cardiac natriuretic peptides during obstructive sleep apnoea and its response to therapy with nasal continuous positive airway pressure

The nocturnal secretion profile of the newly identified natriuretic peptide (NP), brain natriuretic peptide (BNP), was studied in 14 patients with obstructive sleep apnoea syndrome (OSAS) (apnoea hypopnoea index: 60.5±3.4, mean±SE) during two separate nights before and during nasal continuous positive airway pressure (NCPAP) therapy. Plasma levels of NPs (atrial natriuretic peptides; ANP and BNP) were measured at 2-h intervals during sleep. Simultaneously, blood pressure was measured by a non-invasive method (Finapres^®, Ohmeda, Englewood, CO, USA) and urine was collected for determing volume and catecholamine levels. Urinary and serum sodium concentration were determined before and after the study. Eight non-snoring subjects were also studied for the investigation of normal nocturnal profiles of BNP levels. To understand the discrete secretion profiles of the two NPs during sleep, blood was sampled from an additional seven patients every 5 min over a 30-min period around 00.00 and 04.00 hours before NCPAP. In patients with OSAS, plasma BNP levels increased from the beginning of sleep (22:00 h) to the morning (06:00 h) before NCPAP therapy (P< 0.01, anova ). Baseline BNP levels were not significantly correlated with patient's clinical and poly- somnographic parameters. However, in the latter half of the sleep period (02:00–06:00 h), increases in BNP levels during the night before NCPAP therapy were significantly correlated with blood pressure elevations (systolic: r=0.784 P< 0.01, diastolic: r=0.587 P< 0.01) and with apnoea duration (r=0.582 P< 0.01). In normal subjects BP and BNP levels were not changed significantly during sleep. Plasma BNP levels were well correlated with concomitant ANP levels (P< 0.001). NCPAP therapy reduced ANP and BNP levels during sleep and in the morning (P< 0.01). Plasma levels of BNP at 5 min intervals before NCPAP therapy revealed few variations. On the other hand, ANP levels fluctuated over the 30-min period. Changes in BNP levels during sleep in the patients with OSAS may be related to blood pressure variations, but may be too small to play a significant physiological role in regulating diuresis in OSAS. Further work is required to determine the precise role of dual natriuretic system in cardiovascular load and natriuresis in OSAS.     

Pemphigoid vegetans

A Japanese case of pemphigoid vegetans is described. The clinical, histopathological and immunological features were similar to the previously reported cases. The patient also developed vesicular lesions. Indirect immunoelectronmicroscopy revealed that the autoantibody in this patient's serum reacted with basal cell hemidesmosomes. This study provides further evidence that pemphigoid vegetans is a subtype of bullous pemphigoid.     

Muscarinic Receptor Binding of Imidafenacin in the Human Bladder Mucosa and Detrusor and Parotid Gland

Objectives: The current study aimed to characterize comparatively the binding of imidafenacin to muscarinic receptors in the human bladder mucosa and detrusor muscle and parotid gland. Methods: The muscarinic receptor in homogenates of human tissues (bladder mucosa and detrusor muscle and parotid gland) was measured using a radioligand binding assay with [N‐methyl‐³H]scopolamine methyl chloride ([³H]NMS). Results: Imidafenacin competed with [³H]NMS for binding sites in the bladder mucosa and detrusor muscle and parotid gland, and its affinity was significantly (2.6–8.7 times) higher than that of oxybutynin. Also, the affinity of imidafenacin for muscarinic receptors was approximately two‐fold higher in the parotid gland than bladder tissue. The affinity of imidafenacin in the mucosa was similar to that in the detrusor muscle, suggesting that this agent exhibits therapeutic effects by blocking muscarinic receptors in the mucosa as well as detrusor muscle. Scatchard analysis revealed that imidafenacin increased significantly (approximately four‐fold) K_d values for [³H]NMS binding in the human detrusor muscle and parotid gland, with little effect on B_max values. This observation indicates that imidafenacin binds to the muscarinic receptors in human tissues in a competitive and reversible manner. Conclusion: Imidafenacin binds to muscarinic receptors in the human bladder mucosa and detrusor muscle and parotid gland with high affinity. This agent was considered to exhibit therapeutic effects on the lower urinary tract symptoms due to an overactive bladder by blocking muscarinic receptors in the urothelium as well as detrusor muscle.     

Calcium staining by the glyoxal-bis-(2-hydroxyanil)-method in the livers of rats treated with CCl4, diltiazem,and with both agents together

ABSTRACT— We studied the histochemistry of Ca in livers treated with CCl₄, diltiazem (one of the Ca antagonists), and both agents together to determine whether hepatocytes or other parts of the liver in liver lesions show Ca staining and whether the grade or location of Ca in these injuries varies. For Ca staining, cryostat sections were treated by the glyoxal-bis-(2-hydroxyanil) (GBHA)-method using O.C.T. imbedding compound instead of paraffin. Diltiazem-treated rats showed Ca granules in the bile canaliculi around the terminal hepatic veins and Kupffer cells 6 h after intragastric injection. Rats treated with CCl₄ showed fine red granules in the cytoplasm of the hepatocytes around the terminal hepatic veins as soon as 5 min mildly and 2 h moderately after intraperitoneal injection. Hepatocytes around the terminal hepatic veins showed positive Ca granules 6 to 30 h after intragastric injection of CCl₄. Hepatocytes stained by Ca showed acidophilic degeneration and coagulative necrosis. The hepatocytes of rats treated with both diltiazem and CCl₄ revealed fewer Ca granules than those treated with CCl₄ alone. In summary, Ca was stained by the GBHA method from the early stage of liver injury by CCl₄ and was closely involved in acidophilic degeneration and coagulative necrosis of hepatocytes. The Ca staining in liver cells in CCl₄-treated rats was decreased by diltiazem.