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Biomedical subjects

J Hellmann

Publications and source records attributed to J Hellmann.

At least 19 recordsLinked to original sources

Severe aortic thrombosis in the neonate--successful treatment with low-molecular-weight heparin: two case reports and review of the literature.

Small- to moderate-sized aortic thrombi in the sick newborn are frequently demonstrated, however, severe aortic thrombosis is rarely encountered. We report two newborn infants presenting with signs of decreased lower limb perfusion shortly after birth. An occlusive infrarenal aortic thrombus was demonstrated in both infants by ultrasound. No predisposing condition for thrombosis could be found in the first infant; in the second, an umbilical arterial catheter was the likely cause. The infants were successfully treated with low-molecular-weight heparin. In both infants resolution of the thrombus was observed by ultrasound. No complications of treatment were encountered. The clinical spectrum of neonatal aortic thrombosis and treatment options are reviewed.

Anticoagulants↗

Early-infantile galactosialidosis: prenatal presentation and postnatal follow-up.

Galactosialidosis (GS) is an autosomal recessive condition caused by combined deficiency of the lysosomal enzymes beta-galactosidase and alpha-neuraminidase. The combined deficiency has been found to result from a defect in protective protein/cathepsin A (PPCA), an intralysosomal protein which protects these enzymes from premature proteolytic processing. The most severe form of GS, the early-infantile form, results in early onset of edema, ascites, visceromegaly, and skeletal dysplasia. We report a case of early-infantile GS in a male infant who presented with nonimmune fetal hydrops (NIH), "coarse" facial appearance, massive fluid-filled inguinal hernias, multiple telangiectasia, and diffuse hypopigmentation; he subsequently developed visceromegaly. The diagnosis of GS was confirmed biochemically and the defect in PPCA characterized at the protein level. Examination of fetal peripheral blood smears sampled at 30 weeks gestation demonstrated vacuolation of lymphocytes, suggesting blood film examination may be a useful screening tool for cases of NIH where a metabolic disorder is suspected. Skeletal radiography at birth demonstrated punctate epiphyses of the femora, calcanei, and sacrum. We present a discussion of and differential diagnosis for this radiographic finding. To the best of our knowledge, this is the first case of early-infantile GS presenting with stippled epiphyses.

Blotting, Western↗

RITA/Registry of Industrial Toxicology Animal data: a comparative immunohistochemical study of 77 islet cell carcinomas in Sprague-Dawley and Wistar rats using antibodies against insulin, glucagon, somatostatin and gastrin.

UNLABELLED: The objective of this study was to investigate spontaneous islet cell carcinomas with particular reference to possible existing strain differences between Sprague Dawley (SD) and Wistar (W) rats in incidence and immunohistochemical staining pattern. Secondly the occurrence of somatostatin and/or gastrin-positive islet cell tumors should be tested. Islet cell adenocarcinomas (34 from SD, 43 from W-rats) were selected from the RITA-data base and company in-house data base out of an animal pool of 3915 (1681 SD, 2234 W-rats). They were untreated or sham-treated (vehicle) control animals from carcinogenicity studies and whole life-span experiments. Islet cell carcinomas occurred in a higher incidence in male rats (2.98% for SD, 3.23% for W) than in female rats (1.07% for SD, 0.63% for W). All specimens were immunohistologically stained with antibodies against insulin, glucagon, somatostatin and gastrin and, selected specimens with additional antibodies (pancreatic polypeptide, lipase, chymotrypsin, S100-protein, actin and cytokeratin). 94% (SD) and 93% (W), respectively, were insulin-positive and the mean staining intensity (on a scale ranging from 0-4) for insulin was 3.58 (SD) versus 3.37 (W). This high insulin staining incidence and intensity characterized most islet cell carcinomas as malignant insulinomas. 24% (SD) and 37% (W), respectively, were glucagon-positive. Except two tumors in W-rats with a focal strong glucagon expression, the mean staining intensity for glucagon was low (0.38 SD, 0.72 W). 38% (SD) and 44% (W), respectively, were somatostatin-positive, but except for five cases having a focal to multifocal, moderate to marked staining, only a few tumor cells were positive for somatostatin in the other cases and the mean staining intensity for somatostatin was low (0.50 SD, 0.84 W). 6% (SD) and 23% (W), respectively, were gastrin-positive, but only one case of a male Wistar rat exhibited a focal strong staining in parts of the tumor. The other cases showed only a few tumor cells which were positive for gastrin. The mean staining intensity for gastrin was low (0.06 SD, 0.35 W). In all tumors with marked glucagon, somatostatin or gastrin expression, the immunostaining for insulin was still predominating. Thus, insulin was the major hormone produced by most of the tumor cells. Five out of 77 tumors evaluated were immunohistologically negative with all applied antibodies. CONCLUSION: This study presents the first immunohistochemical survey on spontaneous islet cell carcinomas in SD and Wistar rats stained with antibodies against the endocrine pancreas hormones insulin, glucagon, somatostatin and gastrin. No major differences in incidence or immunohistochemical staining pattern between SD and W-rats could be detected. In contrast to SD rats, Wistar rats had multihormonal coexpression in 16.3%. The multihormonal appearance of the neoplasms is well comparable with the findings in other animal species and human insulinomas. Moreover, this is the first study in rats which reports five cases with a marked co-expression of somatostatin and one case with marked focal co-expression of gastrin in malignant islet cell adenocarcinomas.

Animals↗

Neonatal adrenal congestion: a sonographic-pathologic correlation.

We reviewed the clinical, sonographic and pathologic findings in eight neonates in whom diffuse enlargement and abnormal echogenicity of the adrenal glands was documented sonographically. Four of the patients suffered from perinatal asphyxia and two others required mechanical ventilation for other reasons. Six patients died and one suffers from severe development delay, cerebral palsy and failure to thrive. Sonographically, the glands were enlarged, their surface was smooth and there was loss of the central echogenic stripe. Diffuse sinusoidal congestion was found histologically in all five in whom autopsies were performed. These sonographic findings represent part of the spectrum of adrenal changes in neonatal asphyxia and other causes of perinatal stress, and may be associated with poor outcome because of other sequelae of asphyxia.

Adrenal Glands↗

Lethal neonatal Menkes' disease with severe vasculopathy and fractures.

A male neonate presented with an acute onset of severe intra-abdominal bleeding, haemorrhagic shock and multiple fractures leading to death on d 27. Menkes' disease was diagnosed at autopsy and confirmed by copper accumulation studies on cultured fibroblasts. Such an early onset of fatal complications in this condition has not been previously reported. New insights into the pathogenesis of Menkes' disease provided by DNA mutation analysis and difficulties in neonatal diagnosis are discussed. Menkes' disease should be considered in male infants with pathological fractures and other signs of connective tissue disease, even in the neonatal period.

Copper↗

Gastric-outlet obstruction induced by prostaglandin therapy in neonates.

BACKGROUND: An infusion of prostaglandin E1 is widely used to maintain patency of the ductus arteriosus in neonates with congenital heart disease. After gastric-outlet obstruction was recognized in several infants who received prostaglandin E1, we studied the association between the drug and this complication. METHODS: We evaluated all neonates who received prostaglandin E1 in our hospital between October 1, 1989, and September 30, 1991, for clinical, radiologic, or pathological evidence of acute gastric-outlet obstruction. RESULTS: Of the 74 neonates evaluated, 65 had no signs of gastric obstruction and were considered normal; 5 had clinical and radiologic or pathological evidence of gastric obstruction consistent with the presence of antral mucosal hyperplasia. The remaining four neonates had clinical signs of gastric obstruction, but no radiologic or pathological examinations were performed. The 5 neonates with antral hyperplasia had received prostaglandin E1 for longer periods (mean [+/- SD] duration, 569 +/- 341 hours) than the 65 normal neonates (54 +/- 58 hours, P less than 0.001) or the 4 neonates with clinical signs of gastric obstruction (119 +/- 60 hours, P less than 0.05). The cumulative dose of prostaglandin E1 was higher in the neonates with antral hyperplasia (2982 +/- 1392 micrograms per kilogram of body weight) than in the normal neonates (279 +/- 270 micrograms per kilogram, P less than 0.001) or the neonates with signs of gastric obstruction (528 +/- 306 micrograms per kilogram, P less than 0.01). In two neonates with antral hyperplasia, the cessation of therapy lessened the gastric-outlet obstruction. CONCLUSIONS: The administration of prostaglandin E1 to neonates can cause gastric-outlet obstruction due to antral hyperplasia. Neonates who receive prostaglandin E1 at recommended doses for more than 120 hours should be closely monitored for evidence of antral hyperplasia.

Alprostadil↗

Induction of labor as compared with serial antenatal monitoring in post-term pregnancy. A randomized controlled trial. The Canadian Multicenter Post-term Pregnancy Trial Group.

BACKGROUND: The rates of perinatal mortality and neonatal morbidity are higher for post-term pregnancies than for term pregnancies. It is not known, however, whether the induction of labor results in better outcomes than does serial fetal monitoring while awaiting spontaneous labor. METHODS: We studied 3407 women with uncomplicated pregnancies of 41 or more weeks' duration. The women were randomly assigned to undergo induction of labor or to have serial antenatal monitoring and spontaneous labor unless there was evidence of fetal or maternal compromise, in which case labor was induced or cesarean section was performed. In the induction group, labor was induced by the intracervical application of prostaglandin E2. Serial antenatal monitoring consisted of counts of fetal kicks, nonstress tests, and assessments of amniotic-fluid volume. The outcomes we measured were the rates of perinatal mortality, neonatal morbidity, and delivery by cesarean section. RESULTS: Among the 1701 women in the induction group, 360 (21.2 percent) underwent cesarean section, as compared with 418 (24.5 percent) of the 1706 women in the monitoring group (P = 0.03). This difference resulted from a lower rate of cesarean section performed because of fetal distress among the women in the induction group (5.7 percent vs. 8.3 percent, P = 0.003). When two infants with lethal congenital anomalies were excluded, there were no perinatal deaths in the induction group and two stillbirths in the monitoring group (P not significant). The frequency of neonatal morbidity was similar in the two groups. CONCLUSIONS: In post-term pregnancy, the induction of labor results in a lower rate of cesarean section than serial antenatal monitoring; the rates of perinatal mortality and neonatal morbidity are similar with the two approaches to management.

Adult↗

Corneal diameter, axial length, and intraocular pressure in premature infants.

PURPOSE: Seventy premature infants 25 to 37 weeks' postconceptional age were examined during their first week of life to determine the correlation of corneal diameter, axial length, and intraocular pressure with gestational age and birth weight. METHODS: Corneal diameter measurement was determined with corneal templates, total axial length with standardized A-scan ultrasound, and intraocular pressure with a Tonopen II tonometer. RESULTS: Corneal diameter and total axial length showed parallel linear increases from 6.2 mm to 9.0 mm and 12.6 mm to 16.2 mm, respectively; however, no significant correlation was found between intraocular pressure and gestational age or birth weight. The mean intraocular pressure was 10.3 mmHg (standard deviation, 3.5). CONCLUSION: Normative values are established for corneal diameter and total axial length as they relate to birth weight and gestational age, and a mean and standard deviation for intraocular pressure in the premature newborn. These values will aid the ophthalmologist in assessing ocular dimensions in premature infants.

Birth Weight↗

[The occurrence of tumors in large bears (Ursidae)--a literature review and six case descriptions].

Histological findings on two Malayan sun bears and four sloth bears show that malignant neoplasms play an important role in tropic bears. Further, most of the tumors originated from the hepatic and biliary tract. Our results were compared with other investigations on zoo animals during the last 70 years revealing that malignant neoplasms are the most common ones in bears of the family Ursidae. Accordingly to our results, sloth and Malayan sun bears seem to have a disposition to develop malignant neoplasms of the hepatic and biliary tract, but within other species only polar bears seem to suffer predominantly from such neoplasia. The reason for this phenomenon could be an alimentary intake of carcinogens. Furthermore, Malayan sun bears show very often neoplasms of the thyroid gland as it is also observed in other carnivora.

Animals↗

Hyperplasia of somatostatin and pancreatic polypeptide immunoreactive cells in dogs with idiopathic atrophy of the exocrine pancreas.

The pancreatic tissue from 25 dogs with idiopathic atrophy of the exocrine pancreas, and from 6 control dogs, was studied histologically and immunohistometrically. Cells producing insulin (B), glucagon (A), somatostatin (D), and pancreatic polypeptide (PP) were identified using specific antisera and the ABC technique. Histometrical quantitation revealed differences in the distribution of these cell types between the right and left pancreatic lobe. Initial stages of atrophy showed little changes concerning the relative proportions of the four cell types examined. In more advanced stages of atrophy, however, there was significant increase in the percentage of the D cells in the cell population of the left lobe. B and A cells showed no significant changes. In final stages, only tiny tissue spots were considered a secondary and regenerative phenomenon, but an endocrine dysregulation cannot be excluded. Atrophy accompanied by diabetes mellitus and a lack of B cells seem to be due to a deficiency of insulin.

Animals↗

[Abilities and requirements profile of rheumatic patients, basis for suitability assessment as an interdisciplinary task in vocational rehabilitation].

Vocational rehabilitation service provision is always required when current job tasks can no longer be carried out due to disease-related performance restrictions. For social service agencies and employment offices as well as the rehabilitation facilities to intervene, information is needed on the remaining abilities, in order to be able to match future job requirements accordingly. Chronic rheumatic diseases gain increasing importance due to their high incidence and chronic progression; their impact on the capacity for work is considerable, with little attention however so far paid by the rehabilitation community. For 30 rheumatic patients of working age, abilities profiles have therefore been prepared and requirements profiles been established for their current job tasks, based on the methodological concept of Ertomis-Stiftung which had been supplemented by certain items important from an industrial medical angle. The findings show that the rheumatic person experiences limitations in all movement functions, and that lifting and carrying in particular are not possible. A major request is inclusion of a dynamic assessment criterion in the abilities profiles, as the person with a rheumatic disease in principle is capable of carrying out all movements, not however at the speed required to perform the job tasks.

Activities of Daily Living↗

Cerebral blood-flow velocity patterns in post-hemorrhagic ventricular dilation.

To evaluate the effect of ventricular dilation (VD) on cerebral hemodynamics, serial cerebral bloodflow velocity patterns from the anterior and middle cerebral, and circle of Willis arteries were examined by range-gated, pulsed Doppler sonography in premature infants developing post-hemorrhagic VD. Nine infants (25 to 30 weeks gestation) without a patent ductus arteriosus were studied until resolution of VD. Forty-nine cranial sonograms from all nine infants were reviewed independently and grouped cross-sectionally into mild, moderate and severe VD prior to shunt. The corresponding pulsatility index (PI) showed a consistent trend of increase with VD in all three studied vessels. In six infants, absent or reversed diastolic flow was observed at the height of VD. Four of these infants required V-P shunt. Immediate fall in PI occurred in all three vessels. Serial measurement of PI during VD reflects global changes in cerebrovascular resistance. Results confirmed PI could be a useful index in monitoring cerebral hemodynamic changes.

Cerebral Hemorrhage↗

Regional cerebral blood flow velocity patterns in newborn infants.

Using range-gated pulsed Doppler sonography, cerebral blood flow velocity (CBFV) waveforms from the anterior cerebral artery (ACA), middle cerebral artery (MCA) and circle of Willis artery (CW) were examined in a total of 34 newborn infants. We compared the pulsatility index (PI) from the three cerebral arteries sampled in 10 term and 10 preterm (29 +/- 2 weeks) newborn infants without a history of perinatal asphyxia or intracranial pathology. The Pl in the ACA ranged from 0.60 to 1.03. There were no significant differences in Pl between the three vessels by paired comparisons. The Pl of the MCA differed from that of the ACA by 0.00 +/- 0.05. The variation coefficient (CV) was 7%. For CW with ACA, the difference was 0.00 +/- 0.04 and CV was 6%. Both intra- and interexaminer variation in Pl measurements were studied in another 14 infants. The variation coefficients were 5-8% for all three cerebral arteries. We showed that CBFV waveform patterns were similar in regional cerebral arteries, with Pl being a consistent CBFV index. In normal cerebral circulation, the intervessel Pl differences were within observer variations. Deviation from this may suggest abnormal regional cerebral haemodynamics.

Blood Flow Velocity↗

Impact of delayed development in premature infants on mother-infant interaction: a prospective investigation.

We examined events that precede a diagnosis of developmental delay by comparing 16 very low birth weight premature infants whose condition was diagnosed as developmental delay at 18 months corrected age with 16 matched developmentally normal infants. All infants were observed with their mothers during maternal visits to the nursery and during home visits 1, 3, 6, and 9 months after discharge. The mothers' reactions to their infants were rated at these times. All the children were followed up at a neonatal clinic, and detailed clinic records were used to document the time when a physician first suspected a delay and when this delay was first mentioned to the mother. The results indicate that parents had usually been told of their child's handicap by the time the child was 6 to 9 months old; yet mothers of the delayed children changed the interaction with their children as early as 1 month after discharge from hospital. These mothers initially touched, smiled at, and talked to their developmentally delayed infants significantly more often, but by 9 months they did so much less often than mothers of nondelayed children. The degree of change in mothers' behavior was related to their psychosocial background, with better-adjusted mothers showing the most change. We conclude that mothers' statements about their infants are usually valid and that physicians may share their potential concerns about infants more freely.

Adult↗