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Biomedical subjects

S L Werlin

Publications and source records attributed to S L Werlin.

At least 73 records · Page 4Linked to original sources

Maturation of secretory function in rat pancreas.

In contrast to morphologic maturity, the newborn rat pancreas is unresponsive to carbachol. At 24 h, carbachol-stimulated secretion of exportable proteins markedly increases. Responsiveness to the calcium ionophore, A23187, is present at term. At age 24 h, pancreas from fasted newborn rats is also unresponsive to carbachol. When cholecystokinin octapeptide or hydrocortisone was administered to fasted newborn rats secretory function developed normally. Thus, responsiveness of newborn rat pancreas to secretagogues matures rapidly and can be induced by hormones.

Amylases↗

Pharyngoesophageal motor function in patients with myotonic dystrophy.

Six adults with myotonic dystrophy underwent detailed radiological and manometric studies of pharyngoesophageal function. Four of the patients had no history of swallowing dysfunction, while two had very mild symptoms. All patients showed abnormalities of pharyngoesophageal function. Pharyngeal peristalsis was present but of low amplitude. Resting upper esophageal sphincter pressure was diminished, but the sphincter relaxed normally. Peristalsis in the esophageal body was invariably absent in the striated muscle segment of the esophagus and was either absent of decreased in amplitude in the smooth muscle segment. The lower esophageal sphincter was normal. Myotonia did not occur in the sphincters or the esophageal body. These findings suggest that pharyngoesophageal dysfunction is common in patients with myotonic dystrophy, even when clinical symptoms are absent. The abnormalities are probably related to muscle weakness rather than to myotonia in the pharyngeal and esophageal musculature.

Adult↗

Esophageal function in esophageal atresia.

In order to determine the spectrum of esophageal dysfunction in repaired esophageal atresia, 14 patients were evaluated with esophageal manometry, intraluminal pH recording, and radiology. Nine patients had no difficulty in swallowing but six had symptoms suggestive of gastroesophageal (GE) reflux. On pH recording, six had evidence of GE reflux. Basal sphincter pressure was 22 mm Hg in both reflux and nonreflux patients. No patient had manometric evidence of peristalsis in the proximal esophagus, but six had peristalsis in the distal esophagus. On radiology all had a normal peristaltic stripping wave in the cervical esophagus, and peristalsis was absent in the proximal thoracic esophagus in all patients but present in the distal esophagus in five of the 10 patients studied. Esophageal dysfunction is present in all patients with repaired esophageal atresia even when symptoms are absent.

Adolescent↗

The megacystis microcolon intestinal hypoperistalsis syndrome: report of a case.

An infant girl with the megacystis microcolon intestinal hypoperistalsis syndrome is reported. The presence of polyhydramnios and the absence of upper urinary tract abnormalities, differentiate this patient from similar cases previously reported. Although the etiology of this disease is unknown, partial success with the use of bethanechol suggests that the deranged intestinal motility may be related to dysfunction of the autonomic nerve endings of the gut.

Colon↗

Gastrointestinal structure and function in Fabry's disease.

We evaluated gastrointestinal structure and function in 13 hemizygous males and 17 heterozygous females, five to 67 years old, from four kindreds with Fabry's disease. Gastrointestinal symptoms, noted in 62% (8/13) of the males and 29% (5/17) of the females, were present prior to the diagnosis of Fabry's disease in five patients; were discovered at the time of study in six patients and were associated with multiple other symptoms in two patients. Serum protein, albumin, folate, Vitamin B12, calcium, phosphorous, cholesterol and iron were normal in all 30 patients. Xylose absorption was normal in 2/2 males and 13/13 females studied. HLA B8 antigen was present in none of the males and 2/17 females. Peroral duodenal (one male), jejunal (six males, two females) and rectal (one male) biopsies on light microscopy demonstrated a normal villous pattern and luxol-fast blue positive "foamy" cell deposits in all males, while no deposits were visualized in the females. In all males and females studied, electron microscopic examination showed electron dense, intralysosomal "zebra-like" (0.5-0.75 micrometer.) bodies in the vascular endothelial and perithelial cells and in the cytoplasm of the small unmyelinated neurons, and perineurial cells. Despite the frequency of gastrointestinal symptoms, both malabsorption and celiac disease were absent.

Adolescent↗

Mechanisms of gastroesophageal reflux in children.

We studied the lower esophageal sphincter in 29 children with known or suspected gastroesophageal reflux using a sleeve sensor and micro pH electrode. Mean lower esophageal sphincter pressure for a ten-minute monitoring period was 19 +/- 13 mm Hg; however, considerable temporal variation occurred in each child. Gastroesophageal reflex was infrequently associated with a low basal sphincter pressure (< 5 mm Hg) but usually associated with an increase in intra-abdominal pressure or with transient inappropriate relaxation of the lower esophageal sphincter.

Child, Preschool↗

Sandifer syndrome: an unappreciated clinical entity.

Five cases of Sandifer syndrome are reported, and the authors suggest that frequently the diagnosis may be overlooked, especially when the patient has a milder or incomplete form of the syndrome. None of the children had hiatal hernia, so this is not an essential requirement for the syndrome. The findings in these five cases suggest that the abnormal posturing associated with this syndrome may be the result of extreme sensitivity of the esophagus to refluxed gastric acid.

Female↗

Laboratory measures of copper metabolism in the differentiation of chronic active hepatitis and Wilson disease in children.

The accuracy of the serum ceruloplasmin level in distinguishing chronic active hepatitis from Wilson disease was compared to the 24-hour urinary copper excretion and hepatic copper content in 20 untreated patients with chronic active hepatitis and 25 with Wilson disease. Serum ceruloplasmin levels were decreased in five patients (25%) with chronic active hepatitis and were normal in seven patients (28%) with Wilson disease at the time of diagnosis. The 24-hour urinary copper excretion failed to provide accurate discrimination between the two groups, being elevated in all patients with Wilson disease and in five of eight patients with chronic active hepatitis studied. All patients with Wilson disease had hepatic copper levels greater than 400 microgram/gm dry weight, whereas patients with chronic active hepatitis had levels less than 300 microgram/gm dry weight. The discriminatory value of hepatic copper concentration makes this the most reliable test for differentiating chronic active hepatitis and Wilson disease in children and adolescents. The serum ceruloplasmin level may not be significantly accurate for definitive diagnosis in this age group.

Adolescent↗

Caroli disease: the diagnostic approach.

Two new pediatric examples of Caroli disease are reported; one was diagnosed by operative cholangiography and one by percutaneous transhepatic cholangiography. Although a variety of approaches may lead one to suspect the diagnosis, percutaneous transhepatic cholangiography is now the method of choice to establish the diagnosis.

Bile Ducts, Intrahepatic↗