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Biomedical subjects

A Schärli

Publications and source records attributed to A Schärli.

At least 19 recordsLinked to original sources

A correlative morphometric and clinical investigation of hypoganglionosis of the colon in children.

Hypoganglionosis of the myenteric plexus of the colon is not clearly defined and seldom investigated. Colon segments from 15 children with an extended oligoeuronal hypoganglionosis up to the proximal resection end were morphometrically studied and compared to normally innervated colon segments. The study was performed with resected specimens from 7 children with isolated hypoganglionoses, 8 children with a Hirschsprung-associated hypoganglionosis, and 12 colon segments with normal innervation. The resected colon specimens were caudo-cranial coiled. The native tissue was frozen at -80 degrees C on a cryostat carrier and cut at -20 degrees C in 15 microns-thick sections (equivalent to 4-5-micron-thick paraffin sections). The air-dried sections underwent an enzyme-histochemical procedure for an acetylcholinesterase reaction to stain the parasympathetically innervated myenteric plexus. For histological identification and morphometric measurements, ganglia and nerve cells were selectively stained using a lactic dehydrogenase reaction. The morphometric measurements were performed with an optic-electronic image analysis system that determined ganglion size, ganglion distances, nerve cell number per ganglion, and ganglion number per mm colon. The results showed that hypoganglionosis of the myenteric plexus is characterised by a 42% decrease in plexus area and a 55% decrease of the nerve cell number per mm length of colon. The number and area of myenteric ganglia showed a decrease of 59% and a doubling of the ganglion distances. The histopathological diagnosis of a hypoganglionosis of the colon was not necessarily an indication of a chronic constipation, but rather an indication of a disposition for constipation. A chronic constipation is often caused by a long hypoganglionic segment proximal to a resected short Hirschsprung segment.

Cell Count↗

[Neuronal intestinal dysplasia. Critical 10-years' analysis of clinical and biopsy diagnosis].

61 cases of neuronal intestinal dysplasia are compared in a follow-up study. Two clinically and bioptically different types of intestinal dysplasia can be distinguished. One type with involvement of the sympathetic nerves and the clinical signs of intestinal spasticity, ulcerative colitis with haemorrhagic stools. Histotopochemically, this disease pattern shows aplasia or hypoplasia of the sympathetic nerves with enhanced parasympathetic activity (elevated acetylcholinesterase activity in the lamina propria mucosae and orbicular musculature). One type with involvement of the plexus submucosus clinically accompanied by adynamia of the colon with megacolon formation. This type becomes manifest usually around the 6th month of life. Bioptically there are large groups of ganglion cells and Schwann's cells, but there is also acetylcholinesterase activity as in the other type. This 2nd form is seen more frequently. A third form is a combination of both diseases. The incidence rate of neuronal intestinal dysplasia is equal to that of Hirschsprung's disease.

Acetylcholinesterase↗

[Hemangio-endothelioma of the liver (case report) (author's transl)].

The infantile hemangio-endothelioma (IHE) of the liver, a rare neoplasm, may lead the clinician and the surgeon to suspect the presence of a malignant tumor, whereas histologic examination often reveals a benign condition; our case report illustrates this discrepancy in a 3 1/2-month-old girl suffering from multiple nodules in the liver. Conservative steroid treatment resulted in a marked reduction in tumor size; the girl is in good health more than two years after diagnosis. More than 80% of hepatic IHE are diagnosed during the first six months of life. Hepatomegaly, congestive heart failure and hemangiomas of the skin combine to the classic symptomatic triad. Since the mortality is high in untreated cases, early diagnosis is of importance. IHE may regress spontaneously. Therefore, conservative therapy, such as steroid medication and treatment of the secondary, tumor-induced complications appear indicated. Alternatively, embolization and/or resection of the tumor may be considered. Signs of cardiac or hepatic insufficiency at diagnosis and a low degree of histologic differentiation are of unfavorable prognostic significance.

Biopsy↗

Congenital duodenal membrane as cause for hiatus hernia, stricture of the oesophagus, and stricture carcinoma in an adult with Down's syndrome.

The case of a 31 year old male mongoloid patient is reported, where a congenital duodenal membrane, asymptomatic in childhood, had led to hiatal hernia, stricture of the oesophagus, and finally to carcinoma of the stricture (Fig. 1). Observation time until death was 26 months. In a first operation, the membrane was resected (Fig. 2) and Nissen's fundoplication performed. 31/2 months after that stricture, carcinoma was diagnosed. In the second operation, carcinoma was resected and continuity reestablished by oesophagogastrostomy. The patient died 8 months after the second intervention.

Adult↗

[Ulcerating duplication of the ileum as rare cause of massive intestinal hemorrhage in the adult].

In this paper is presented the case of a 27 year old woman in whom a congenital duplication of the distal ileum led to a massive hemorrhage. Heterotopic gastric mucosa was found in the ileal lesion and ulcer formation histologically proven. This case shows that congenital lesions of this sort may not come to the attention of the pediatrician or pediatric surgeon but become the concern of the surgeon in adult life.

Abnormalities, Multiple↗