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Biomedical subjects

E Cutz

Publications and source records attributed to E Cutz.

At least 109 records · Page 6Linked to original sources
PubMed1989

Acrodermatitis enteropathica with normal serum zinc levels: diagnostic value of small bowel biopsy and essential fatty acid determination.

We report a patient with acrodermatitis enteropathica and a normal serum zinc level in whom the diagnosis was confirmed by plasma phospholipid fatty acid and a small bowel biopsy response to oral zinc therapy. Acrodermatitis enteropathica is a rare autosomal recessive condition of zinc deficiency characterised by chronic diarrhoea associated with failure to thrive, periorificial dermatitis and alopecia, susceptibility to infections and behavioural changes. Diagnosis is usually established by reduced serum zinc levels (classical acrodermatitis enteropathica). Paneth cell abnormalities on electron microscope of a small bowel biopsy can be supportive. A few cases with the typical picture of acrodermatitis enteropathica without hypozincaemia (variant acrodermatitis enteropathica) have been described. The diagnosis of variant acrodermatitis enteropathica to date has been based on an entirely empiric, but nonetheless convincing clinical response to oral zinc therapy. Laboratory aids to diagnosis have been lacking.

Acrodermatitis↗
PubMed1988

Treatment of Campylobacter pylori-associated antral gastritis in children with bismuth subsalicylate and ampicillin.

We evaluated the efficacy of medical therapy, consisting of liquid bismuth subsalicylate prescribed either in combination with oral ampicillin (n = 15) or alone (n = 1), in the clearing of bacterial colonization on the antrum in 16 children with Campylobacter pylori-associated antral gastritis. We also examined the effects of medical treatment on altering the severity of associated antral inflammation. Eight patients had upper gastrointestinal tract hemorrhage, two had acute gastric outlet obstruction, and 10 had symptoms of episodic epigastric abdominal pain. Duodenal ulcers were demonstrated in 10 of the 16 patients; in the other six, C. pylori-associated antral gastritis was documented without evidence of acute peptic ulceration. Seven days after a 6-week course of medical therapy, repeat upper endoscopy plus mucosal biopsy specimens showed that C. pylori colonization of the antrum had cleared in 12 of the 16 (75%) patients. Inflammation in the antrum improved in all patients in whom colonization by C. pylori was eradicated. In contrast, in the four with persistent colonization of the antrum, the severity of antral gastritis had not improved (p less than 0.01). Clinical symptoms improved in 9 of 12 patients in whom C. pylori colonization was no longer present, whereas subjective symptoms were unaffected in those with continued bacterial colonization of the antrum (p less than 0.05). We conclude that oral bismuth subsalicylate, in conjunction with ampicillin, can eradicate C. pylori colonization of the antrum, and that clearing of C. pylori is correlated with an improvement in the associated antral gastritis and clinical symptoms. These findings provide additional support for the hypothesis that these gastric organisms could play an etiologic role in primary antral gastritis and peptic ulcer disease.

Adolescent↗
PubMed1987

Association of Campylobacter pylori on the gastric mucosa with antral gastritis in children.

We investigated the presence of Campylobacter pylori colonization of the gastric mucosa and of histologic evidence of gastritis in a prospective study of 71 consecutive children undergoing upper gastrointestinal tract endoscopy and gastric biopsies because of gastrointestinal symptoms. Two tissue samples from the gastric antrum were obtained from 67 of the 71 children (mean age [+/- SD], 11.4 +/- 3.8 years). One sample was evaluated for evidence of gastritis and stained with silver to detect organisms morphologically resembling campylobacter. The second sample was cultured for C. pylori, and a portion was used to perform a urease-screening test for the presence of C. pylori. Antral gastritis was diagnosed histologically in 18 of 67 patients. C. pylori was identified by both culture and silver staining on the antral mucosa in 7 of 10 patients with unexplained gastritis (primary gastritis) but in none of 8 patients with gastritis associated with an identifiable underlying cause (secondary gastritis). C. pylori was not identified in any of the 49 cases with normal histologic features. The urease-screening test was positive in only three of six patients with a positive culture for C. pylori. Duodenal ulcers were diagnosed by endoscopy in five patients. Each of the five had C. pylori on the antral mucosa, but organisms were not identified on the duodenal mucosa. We conclude that the presence of C. pylori on the antral mucosa is specifically associated with primary antral gastritis and may also be associated with primary duodenal ulceration.

Adolescent↗
PubMed1987

Effect of granulocyte depletion in a ventilated surfactant-depleted lung.

In a previous paper Cutz, Bryan et al. showed that in rabbits after repetitive lung lavage high-frequency oscillatory ventilation maintained excellent gas exchange and did not cause hyaline membrane formation (J. Appl. Physiol. 55: 131-138, 1983). In contrast, conventional mechanical ventilation had poor gas exchange and extensive hyaline membrane formation and we attributed these differences to mechanical barotrauma. However, we completely overlooked the large number of granulocytes in the damaged lung. To investigate this using the same model we have used mechanical ventilation on two groups of rabbits, one with normal granulocytes, the other depleted of granulocytes by pretreatment with nitrogen mustard. The nondepleted rabbits had poor gas exchange, a substantial protein leak into the lung and extensive hyaline membranes. The depleted animals had good gas exchange, a very small protein leak and no hyaline membranes. Repletion of granulocytes from donor rabbits lead to poor gas exchange and hyaline membrane formation. It is concluded that lung lavage causes prompt margination of granulocytes which become activated by the ongoing epithelial barotrauma of conventional ventilation.

Animals↗
PubMed1987

Perinatal hemochromatosis. Clinical, morphologic, and quantitative iron studies.

Three sibling and two isolated-case perinates (4 newborn, 1 stillborn) died with siderotic cirrhosis and widespread parenchymal siderosis, the latter similar to that seen in both hereditary and secondary hemochromatosis. Reticuloendothelial siderosis was absent, as occurs in primary hemochromatosis. Studies of iron metabolism were performed antemortem in two of the siblings and ante-, post- and internatally in their mother, who showed hyperferremia antenatally. The only finding in the affected family suggestive of hereditary hemochromatosis was the commonly associated HLA haplotype (A3, B7) in the mother and an infant. Liver morphology, including immunocytochemistry and ultrastructure, was similar in the 5 infants and suggested that liver disease commenced as massive necrosis in midfetal life. Histologic grading and chemical assays for iron and copper on liver and spleen of the 5 index cases were compared with 26 controls; placentas were compared with 12 control placentas. Hepatic iron concentration, but not hepatic copper concentration, was significantly increased in index cases, compared with controls. Hepatic iron to copper ratio was significantly increased in index cases, compared with controls, but this ratio was unaltered in spleen and placenta. Total hepatic iron, but not total hepatic copper, was significantly increased in index cases, compared with a subgroup of 11 controls of low gestational age, similar to the fetal stage when liver disease commenced in utero. The results suggest that, irrespective of the fetal liver disease being genetic or acquired, hepatic iron overload was directly involved in pathogenesis.

Copper↗
PubMed1987

Campylobacter pyloridis-associated primary gastritis in children.

Campylobacter pyloridis are spiral-shaped Gram-negative bacteria that have recently been associated with gastritis and peptic ulcer diseases in adults. The organisms have been identified in biopsy specimens of the gastric antrum by staining sections with silver stain. To determine the frequency of colonization of the antral mucosa with C pyloridis in association with gastritis in childhood, we retrospectively reviewed 53 cases in patients (mean age 11.9 years) in whom upper endoscopy with antral biopsy was performed for the evaluation of symptoms referable to the upper gastrointestinal tract. A silver impregnation method was used to detect the presence of C pyloridis. Nineteen of 53 antral biopsy specimens showed evidence of mucosal inflammation. Ten of these 19 patients had no apparent predisposing etiology for gastritis, and six of these ten had C pyloridis identified on antral biopsies. Nine of the 19 patients had secondary causes of gastric inflammation (two gastroduodenal Crohn disease, two eosinophilic gastroenteritis, four drug related, and one dys-gamma-globulinemia and lymphonodular hyperplasia). In contrast, none of these nine patients had C pyloridis demonstrated on antral biopsies. Silver stain for C pyloridis was negative in all 34 of the 53 children who had normal antral histologic findings. C pyloridis was not demonstrated by silver stain on any of the duodenal sections, although eight of the 19 children with gastritis and nine of the 34 children with normal antral histologic findings had histologic evidence of duodenal inflammation. We conclude that C pyloridis is associated with primary antral gastritis but not with secondary gastritis in the pediatric population.

Adolescent↗
PubMed1986

Serotonin in the human infant carotid body.

By immunocytochemistry serotonin was localized in the chief cells of the carotid body in human infants. Radioenzymatic measurement of the serotonin concentration revealed that it represents a significant proportion of the total amine content of the carotid body.

Carotid Body↗
PubMed1986

Association of changes in bombesin immunoreactive neuroendocrine cells in lungs of newborn infants with persistent fetal circulation and brainstem damage due to birth asphyxia.

The pulmonary neuroendocrine (NE) cells, from 16 term infants dying at 1-4 days of age from birth asphyxia, were immuno stained for bombesin-like immunoreactivity by the immunoperoxidase method. The distribution and frequency of bombesin-immunoreactive NE cells were quantified morphometrically and correlated with the presence or absence of brainstem function and persistent fetal circulation (PFC). In infants with loss of brainstem function, the frequency of bombesin immunoreactive NE cells was significantly increased compared to infants with intact brainstem function, i.e. meconium aspiration with PFC. Infants with brainstem injury, with one exception, failed to develop PFC. Pathological changes in the tegmentum of the brainstem, i.e. containing the respiratory center, correlated in nine of 10 cases with loss of brainstem function. These data suggest an inverse relationship between brainstem function, release of bombesin-like peptide from the pulmonary NE cells and the functional state of the pulmonary vasculature. Intact brainstem function appears to be essential for both the release of bombesin-like peptide from the NE cells and for pulmonary vasoconstriction leading to PFC; absence of brainstem function is, on the other hand, associated with failure to release bombesin-like peptide and loss of PFC type reactivity in the pulmonary vasculature. However, it appears unlikely that bombesin itself is a direct mediator of pulmonary vasoconstriction.

Asphyxia Neonatorum↗
PubMed1986

Complications of parenteral nutrition.

There have been no controlled studies that clearly document a beneficial effect of TPN on neonatal mortality and morbidity. Beneficial effects such as reducing the time required to achieve some targeted weight and enhancing positive nitrogen balance are used in support of TPN. The positive opinion about TPN is strongly supported by the experience in surgical patients with GI disorders. It is not surprising that use of TPN is a complex therapy in newborn patients. It is an essential therapy in many patients if nutrition is to be maintained that permits growth and promotes resolution of other chronic problems. Accurate determination of the requirements for TPN is difficult, while complications are not always preventable by simply monitoring blood chemistries. Because of the acute or long-term and potentially lethal complications, there is need for further understanding in parenteral nutrition.

Avitaminosis↗
PubMed1986

Pulmonary artery endothelial abnormalities in patients with congenital heart defects and pulmonary hypertension. A correlation of light with scanning electron microscopy and transmission electron microscopy.

Scanning electron microscopy and transmission electron microscopy were applied to lung biopsy specimens from patients with congenital heart defects, and pulmonary artery endothelium was analyzed for alterations in surface characteristics and intracytoplasmic composition which might reflect abnormal function. The patients were divided into four groups distinguished by increasing severity of pulmonary vascular changes on light microscopy graded both morphometrically and by the Heath-Edwards classification; group 1, normal vasculature or only abnormal extension of muscle into peripheral arteries; group 2, medial hypertrophy; group 3, medial hypertrophy +/- decreased artery number + intimal hyperplasia; group 4, decreased artery number + occlusive intimal hyperplasia. On scanning electron microscopy, the pulmonary artery endothelial surface in group 1 patients was "crinkled" or "corduroy-like", i.e., composed of narrow, even ridges; in groups 2 and 3, it was "cable-like", i.e., comprised of deep intertwined ridges; in group 4 it was "chenille" in texture, i.e., high ridges alternated with low, uneven, and twisted ones. There was significant increased density of surface microvilli in groups 2 and 3 patients when compared to groups 1 and 4 (p less than 0.05 for each comparison). On transmission electron microscopy pulmonary artery endothelial cells in groups 2 and 3 patients were also characterized by a significant increase in the volume density of rough endoplasmic reticulum (p less than 0.01) and microfilament bundles (p less than 0.05). The coarse endothelial surface characteristics associated with pulmonary vascular changes may result in abnormal interaction with blood elements and release of vasoactive substances. The increased microvilli, rough endoplasmic reticulum, and microfilament bundles in patients with moderate but not advanced arterial changes suggest a phase where increased endothelial metabolic function and alterations in the cytoskeleton may also contribute to heightened pulmonary vascular reactivity.

Actin Cytoskeleton↗
PubMed1985

Diagnosis and therapy of necrotizing tracheobronchitis in ventilated neonates.

From January 1983 to September 1984 our neonatal ICU (NICU) treated eight endotracheally intubated infants who had suspected airway obstruction characterized by hypercarbia dissonant with severity of lung disease and difficulty in ventilation with lack of chest movement, both on conventional intermittent mandatory ventilation and high-frequency oscillation. Bronchoscopic removal of necrotic tissue was possible in six infants, two of whom survived. Bronchoscopy showed desquamation of epithelial surfaces, leaving encrusted exudations considered to be characteristic of necrotizing tracheobronchitis (NTB). The four nonsurvivors of bronchoscopy and one of the infants not submitted to bronchoscopy had NTB confirmed at autopsy. NTB was not associated with any specific lung disease, humidifier, or ventilator. The autopsy frequency of NTB during this period was 5 per 160 NICU admissions. A separate chart review of unselected autopsied cases in 1981 and 1982 showed that 12 of 284 neonates admitted to the NICU had NTB. NTB appears to be a rediscovered condition related to endotracheal intubation and mechanical ventilation using high mean airway pressures.

Bronchitis↗
PubMed1985

In vitro characteristics of pulmonary neuroendocrine cells isolated from rabbit fetal lung. I. Effects of culture media and nerve growth factor.

Pulmonary neuroendocrine (NE) cells, dispersed throughout the airway mucosa as single cells and as innervated clusters (neuroepithelial bodies), were isolated from rabbit fetal lung and studied in short-term culture. The effects of culture media and nerve growth factor (NGF) on in vitro maintenance, differentation, and cell kinetics of isolated NE cells were examined. For demonstration of NE cells in intact lung, during cell separation and after culture, immunostaining for serotonin, formaldehyde-induced fluorescence method, histochemical reaction for acetylcholinesterase, and electron microscopy were used. The isolation procedure consisted of mechanical and enzymatic dissociation of lung tissue followed by separation of isolated cells on a discontinuous gradient of Percoll, resulting in 5- to 10-fold enrichment in NE cells. Cell fractions enriched in NE cells were cultured up to 7 days either in supplemented alpha-minimal essential medium with fetal bovine serum or in defined, hormone-supplemented, serum-free medium. NGF (2.5 S 5 to 50 ng/ml) was added to both serum-supplemented and serum-free media; cultures without NGF served as control. The number of serotonin-immunoreactive NE cells maintained in serum-supplemented medium (0.5% fetal bovine serum) increased significantly (p less than 0.05) on days 4 and 7 compared with cultures grown in serum-free medium. NE cells maintained in serum-supplemented medium incorporated [3H]thymidine and their labeling index was significantly increased (p less than 0.01) on day 7, whereas few or no NE cells were labeled in cultures grown in serum-free medium. NGF had no effect on the maintenance or kinetics of NE cells. Cultured NE cells formed elongated (unipolar or bipolar) neurite-like cytoplasmic processes with a button-like ending, regardless of the presence of NGF. Amine accumulated in perinuclear cytoplasm and in button-like endings. Staining for acetylcholinesterase (strongly positive in intact neuroepithelial bodies) was not detectable after separation into single cells, culture, or exposure to NGF. This study demonstrates that NE cells can be isolated from rabbit fetal lung and maintained in short-term culture. Low concentrations of fetal bovine serum enhanced the in vitro maintenance of NE cells, whereas NGF had no such effect. A feeder layer may be also important, since NE cells were closely associated with lung epithelial or fibroblast-like cells. The formation of neurite-like processes appears to be an expression of paracrine/paraneuron-type cell differentiation not mediated by NGF.(ABSTRACT TRUNCATED AT 400 WORDS)

APUD Cells↗
PubMed1984

Sudden infant death syndrome: increased carotid-body dopamine and noradrenaline content.

Carotid bodies from infants dying of sudden infant death syndrome contained significantly higher concentrations of dopamine (10-fold) and noradrenaline (3-fold) than those from age-matched control infants. Administration of dopamine inhibits respiration by direct action on the carotid body, and it is suggested that the elevated levels of endogenous catecholamines found in victims of sudden infant death syndrome may compromise the normal function of the carotid body, particularly the ventilatory response to hypoxia.

Carotid Body↗
PubMed1984

Neonatal intestinal pseudoobstruction.

In the past 10 years, the diagnosis of pseudoobstruction lasting more than two months was confirmed in 10 infants after sepsis, meconium ileus, and Hirschsprung's disease were excluded, and surgery or autopsy failed to demonstrate a site of mechanical intestinal obstruction. Four infants had undergone prior operation for another anomaly: gastroschisis (2) and ileal atresia (2). Five of the remaining infants also had megacystis. The lack of coordinated intestinal motility was best appreciated by radiocontrast small bowel studies, which showed degrees of aperistalsis or segmentation. Rectal manometric studies were not helpful. Histology of the intestine was normal in seven, while a gross deficiency of nerve fibres was noted in one patient and a myopathy of smooth muscle in another. A variety of drugs used to stimulate peristalsis were ineffective. Seven patients had 25 operations, often to exclude mechanical causes of obstruction. The mainstay of treatment was TPN and intestinal decompression. Six children survived; their ages ranged between 8 months and 9 years (median age, 16 months). There has been improvement in intestinal peristalsis in five children, three of whom now tolerate a regular diet and two of whom are on TPN and are currently increasing oral intake. Four infants died, two from sepsis, two from TPN-related hepatic failure. In contrast to previous reports, we conclude that intestinal pseudoobstruction may be self-limited in some neonates, including those with megacystis. Therapy should consist of long-term nutritional support and treatment of other anomalies that may be present.

Combined Modality Therapy↗