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Biomedical subjects

R Boehm

Publications and source records attributed to R Boehm.

At least 19 recordsLinked to original sources

Recurrent intussusceptions in an infant that were terminated by laparoscopic ileocolonic pexie.

For children with ileocolic intussusceptions, laparoscopy has been proposed as an emergency intervention, but it has not been elaborated for elective prevention of recurrencies. We report about an infant who developed his first ileocolic intussusception at the age of 12 months. Radiologic devagination was successful, but had to be repeated for two consecutive recurrences within several days. Six months later, he presented with another episode of intussusception, which again was managed conservatively. At this time, preventive surgery seemed indicated. Diagnostic laparoscopy using three trocars and 5-mm instruments showed an insufficient closure of the ileocecal valve, allowing the surgeon easily to provoke an intussusception. Consequently, the distal ileum was attached to the ascending colon with several interrupted 3-0 sutures. The infants postoperative course was uneventful. Oral feeding was started immediately, and he could be discharged after 3 days. Within a follow-up period of 1 year, no evidence of intussusception was noted. We conclude that for children with recurrent episodes of intussusception, laparoscopic ileocolonic pexie presents a beneficial strategy for protective surgery.

Humans↗

Postpericardiotomy syndrome after minimally invasive pectus excavatum repair unresponsive to nonsteroidal anti-inflammatory treatment.

A 14-year-old boy developed postpericardiotomy syndrome after an otherwise uneventful minimally invasive pectus excavatum repair. Dyspnoea, chest pain, and pericardial effusion progressed despite nonsteroidal anti-inflammatory treatment. The symptoms rapidly resolved with intravenous methylprednisolone, and pericardiocentesis was thus avoided. This is the first report of postpericardiotomy syndrome after the Nuss procedure treated with systemic steroids.

Adolescent↗

Ileoileal intussusception caused by a Ewing sarcoma tumour. An unusual case report.

Intussusception typically occurs in childhood, presenting with a well-known medical history and clinical symptoms. Pathologically, a "leading point" may be attributed to lymphadenomatosis, polyps, or a tumour. In older patients and adolescents, the diagnosis can be complicated due to the lower incidence and variable subacute symptoms. We report on an 18-year-old patient with increasing abdominal discomfort over several weeks. External diagnostics showed no pathological signs or were misinterpreted as a malfunction of intestinal motility. The patient experienced increasing colics, recurrent vomiting, dehydration and weight loss. Finally he was transferred to our paediatric surgical department and laparotomy had to be performed for the clinical and radiological signs of an ileus. An ileoilealic intussusception was found, caused by a small bowel tumour, which almost completely obstructed the intestinal lumen. It was resected and bowel continuity was re-established. Histopathology revealed a very rare, highly malignant mesenchymal Ewing sarcoma, infiltrating the complete bowel wall. After the postoperative course, the patient was transferred to our oncological department for chemotherapy. In older children or young adults, intestinal malignancies are extremely rare. Nevertheless, if these patients suffer from unspecific complaints of chronic intestinal obstruction, a tumour must be ruled out. A Ewing sarcoma may be responsible for an intussusception.

Adolescent↗

Critical validation of colour Doppler ultrasound in diagnostics of acute scrotum in children.

Acute scrotum in children is generally a symptom. Several pathologies of the testis, scrotum or groin may cause it, including inflammatory as well as ischaemic processes. Testicular torsion with ischaemia of the testis is an emergency requiring prompt surgical intervention. Even experienced paediatric surgeons and urologists may have difficulties differentiating an ischaemic from an inflammatory aetiology solely on the basis of clinical signs and symptoms. In cases of aetiologic uncertainty, operative exploration has to be performed to rule out ischaemia, which led in the past to a high rate of surgery. More recently, colour Doppler ultrasonography (CDUS) has become an imaging modality for the examination of the acute scrotum with the purpose of detecting ischaemia, thus reducing the need for explorative surgery. In this study we examined the reliability of CDUS, comparing the sonographical diagnoses with both the intraoperative findings and the clinical course. We present here 132 consecutive cases of acute scrotum in children, who were admitted from 01/98 through 10/02 to our hospital. All patients underwent CDUS. Patients with ultrasonographic signs of ischaemia of the testis (group A) and patients with demonstrated perfusion and suspicious or severe clinical symptoms (group B) were operated immediately. Patients with demonstrated perfusion without suspicious or severe symptoms (group C) were treated conservatively. Thirty-eight patients (28.8%) were treated operatively. Eleven cases of testicular torsion in group A (12 pat.) could be confirmed intraoperatively, in one case there was no testicular torsion but a severe inflammation of the testis with torsion of the appendix testis. In group B (26 pat.) operative exploration revealed one case of testicular torsion. In group B two cases of sonographically suspected torsions of the appendix testis also emerged as epididymitis intraoperatively. In the other cases postoperative diagnosis was in agreement with the preoperative CDUS. The clinical course of 94 conservatively treated patients (71.2%) (group C) confirmed in all cases a non-ischaemic aetiology. There was a total of 12 cases (9.1%) where acute scrotum was caused by testicular torsions. This relatively low rate underlines the necessity to differentiate between ischaemic and other aetiologies, thus avoiding operation in many patients. 71.2% of our patients could be treated conservatively. However, it is essential to exclude an ischaemic cause before opting for conservative therapy. Even though CDUS is a very valuable diagnostic tool in this sense, this exclusion cannot be ensured in every case and is highly dependent on the expertise and technique of the investigator. Therefore in cases with any doubts an operative exploration has to be performed to avoid the most dreaded complication in the treatment of acute scrotum.

Acute Disease↗

Laparoscopic resection of a congenital liver cyst and simultaneous closure of a diaphragmatic defect in a 5-month-old infant.

Congenital liver cysts are an unusual finding and present a difficult diagnostic challenge. In a 5-month-old infant, routine echocardiography detected a cystic lesion (3 cm in diameter) just below the diaphragm. All conventional diagnostics, including magnetic resonance imaging (MRI), failed to clarify its etiology definitively. Moreover, a phrenic irregularity was suspected at the site of Morgagni. Finally, laparoscopy (5-mm scope and instruments) confirmed the diagnosis of a solitary liver cyst and a diaphragmatic defect. Using a Harmonic scalpel, the cyst was resected and the diaphragmatic defect was closed with interrupted nonabsorbable sutures. The patient's postoperative course was uneventful. Pathological analysis showed a true liver cyst with mesothelial lining and hepatic residues. Retrospective speculation suggests a common embryologic origin for both malformations. In conclusion, laparoscopy allowed final diagnosis and simultaneous treatment of an infant with the rare combination of a liver cyst and a diaphragmatic defect. To our knowledge, this case represents the first endosurgical experience of such a simultaneous procedure.

Cysts↗

Laparoscopic repair of an extrahiatal hernia caused by congenital dysplasia: a case report.

A 40-year-old woman with complaints of relapse in the upper abdomen and dysphagia was referred for laparascopic hiatal hernia repair. Chest radiograph, barium-swallow, and upper endoscopy revealed a paraesophageal hernia. Esophageal manometry and 24-h-pH study showed no pathological findings. A laparoscopic gastropexy was planned. Intraoperatively, in contradiction to the preoperative findings, an extrahiatal hernia containing most of the stomach was found. After resection of the hernia sac, the beating heart without covering pericardium was seen. These findings were confirmed by an additional thoracoscopy at the end of the operation. The defect was closed by direct suturing. The postoperative course and 2-month follow-up were uneventful. The resected parts of the hernia sac showed an embryonic and dysgenetic etiology. This rare malformation has been reported in combination with complex syndromes, which appear with serious clinical and morphological signs in the neonatal period. In adults, the pericardial aplasia can be observed during diagnostic or surgical interventions. In these patients, complaints are usually not caused by the malformation but may be due to the occasional herniation of abdominal organs. We consider laparoscopic repair to be a gentle and safe procedure for the treatment of extrahiatal hernias.

Adult↗

Multiple intestinal stenoses and peripheral gangrene: a combination of two rare surgical complications in a child with Kawasaki disease.

Kawasaki disease (KD) often presents with a challenging variety of clinical symptoms. Severe gastrointestinal complications are rare and mainly appear as pseudo-obstruction. However, the authors report the unique case of a 4-month-old girl with KD suffering from a mechanical ileus. The optimal timing of surgery presented a dilemma, because she received lytic treatment for gangrenes of both her hands and feet and additionally had large coronary artery aneurysms. Finally laparotomy had to be performed while the patient was on an anticoagulant medication, and it showed a 30-cm-long jejunal segment with multiple filiforme stenoses, requiring resection and anastomosis. The postoperative course was uneventful regarding the abdominal situation; however, the left hand and left foot remained gangrenous and had to be amputated. In patients with KD, not only pseudo-obstruction, but irreversible intestinal obliteration has to be encountered. This combination of intestinal stenosis and acral gangrene has not been described before. J Pediatr Surg 36:651-653.

Amputation, Surgical↗

Proliferation, zonal maturation, and steroid production of fetal adrenal transplants in adrenalectomized rats.

The present study investigated the histologic maturation, proliferative capacity, and steroid production of fetal adrenal transplants (Tx) in adrenalectomized rats. A pair of fetal adrenal glands (18-20 days of gestation) was transplanted into the omentum of syngeneic Lewis rats (n = 45). Four weeks later, in 5 animals the grafts were excised for morphologic evaluation. Proliferation was investigated by immunohistochemical staining for KI-67 protein and quantified by the proliferation index (PI = positive cells/100 counts). All other hosts (Tx; n = 40) underwent bilateral adrenalectomy (AE) to induce Addisonian crisis. Postoperatively, survival and concentrations of potassium, sodium, aldosterone, and corticosterone were recorded for 6 months. These data were compared to controls (C=only AE; n = 30) and a sham group (S; n = 10). At the end of the study period all surviving hosts were killed for histologic examination of grafts. At 4 weeks post-Tx the adrenal grafts demonstrated a distinct zona glomerulosa and frequent proliferation with a PI of 0.084, comparable to normal control (0.092). Following AE survival was significantly prolonged in Tx (86% vs 12% of C, P < 0.05). Control animals developed severe hyponatremia and hyperkalemia, whereas in Tx only transient signs of Addisonian crisis were recorded. Levels of aldosterone dropped within 7 days in the Tx and C groups, but returned to normal for Tx within 8 weeks. Corticosterone levels of Tx animals fell to 25% within week, but steadily increased to 70% by the end of the study. At 6 months, grafts revealed a mature adrenocortical structure with little proliferative activity, which was comparable to controls. In a syngeneic rat model fetal adrenal transplants thus mature and proliferate to provide sufficient steroid production for adrenalectomized hosts.

Adrenal Glands↗

Active expression of the ubiA gene from E. coli in tobacco: influence of plant ER-specific signal peptides on the expression of a membrane-bound prenyltransferase in plant cells.

The ubiA gene from E. coli codes for 4-hydroxybenzoate: polyprenyldiphosphate 3-polyprenyltransferase, an integral membrane protein involved in ubiquinone biosynthesis. This prokaryotic membrane protein was stably expressed in tobacco using Agrobacterium tumefaciens-mediated transformation. Transgenic lines containing a direct fusion of the ubiA structural gene to a 35S-derived promoter gave very low enzyme activity levels (average 0.16 pkat/mg). Inclusion of an N-terminal ER-specific signal peptide from a lectin gene from Phaseolus vulgaris resulted in an average activity of 1.08 pkat/mg in the transgenic tobacco lines. The additional inclusion of a C-terminal HDEL tetrapeptide, responsible for the retention of proteins in the endoplasmic reticulum of eukaryotic cells, increased the activity to 18.6 pkat/mg. When the promotor of this construct was changed from the 35S derivative to the recently described very strong plant promoter (ocs)3mas, the activity increased further to 128.6 pkat/mg. The most active tobacco line showed activities of the introduced enzyme which exceeded those of wild-type E. coli (the source of ubiA) by a factor of 1100. These results demonstrate the efficacy of plant ER-specific signal peptides for the active expression of a prokaryotic membrane protein in plants.

Alkyl and Aryl Transferases↗

Inhibition by adenosine A(2A) receptors of NMDA but not AMPA currents in rat neostriatal neurons.

Whole-cell patch clamp experiments were used to investigate the transduction mechanism of adenosine A(2A) receptors in modulating N-methyl-D-aspartate (NMDA)-induced currents in rat striatal brain slices. The A(2A) receptor agonist 2-p-(2-carboxyethyl)phenethylamino-5'-N-ethylcarboxamidoadenosine (CGS 21680) inhibited the NMDA, but not the (S)-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) current in a subset of striatal neurons. Lucifer yellow-filled pipettes in combination with immunostaining of A(2A) receptors were used to identify CGS 21680-sensitive cells as typical medium spiny striatal neurons. Dibutyryl cyclic AMP and the protein kinase A activator Sp-cyclic AMPs, but not the protein kinase A inhibitors Rp-cyclic AMPS or PKI(14 - 24)amide abolished the inhibitory effect of CGS 21680. The phospholipase C inhibitor U-73122, but not the inactive structural analogue U-73343 also interfered with CGS 21680. The activation of protein kinase C by phorbol 12-myristate 13-acetate or the blockade of this enzyme by staurosporine did not alter the effect of CGS 21680. Heparin, an antagonist of inositol 1, 4,5-trisphosphate (InsP(3)) and a more efficient buffering of intracellular Ca(2+) by BAPTA instead of EGTA in the pipette solution, abolished the CGS 21680-induced inhibition. The calmodulin antagonist W-7 and cytochalasin B which enhances actin depolymerization also prevented the effect of CGS 21680; the calmodulin kinase II inhibitors CaM kinase II(281 - 309) and KN-93 but not the inactive structural analogue KN-92 were also effective. The calcineurin inhibitor deltamethrin did not interfere with CGS 21680. It is suggested that the transduction mechanism of A(2A) receptors to inhibit NMDA receptor channels is the phospholipase C/InsP(3)/calmodulin and calmodulin kinase II pathway. The adenylate cyclase/protein kinase A and phospholipase C/protein kinase C pathways do not appear to be involved.

Adenylyl Cyclases↗

Genetic engineering on shikonin biosynthesis: expression of the bacterial ubiA gene in Lithospermum erythrorhizon.

The naphthoquinone pigment shikonin from Lithospermum erythrorhizon Sieb. et Zucc. (Boraginaceae) was the first plant secondary metabolite produced in industrial scale from plant cell cultures. We have now manipulated the biosynthetic pathway leading to shikonin in L. erythrorhizon by introduction of the bacterial gene ubiA. This gene of Escherichia coli encodes 4-hydroxybenzoate-3-polyprenyltransferase, a membrane-bound enzyme that catalyzes a key step in ubiquinone biosynthesis. Using geranyl diphosphate (GPP) as substrate, it is able to catalyze the formation of 3-geranyl-4-hydroxybenzoate (GBA), a principal step of shikonin biosynthesis. The prokaryotic ubiA gene was fused to two signal sequences for targeting of the resulting peptide to the endoplasmic reticulum (ER). Constructs with different constitutive promoters were introduced into L. erythrorhizon using Agrobacterium rhizogenes-mediated transformation. In the resulting hairy root lines, high UbiA enzyme activities could be observed, reaching 133 pkat mg(-1). Expression of ubiA resulted in an accumulation of GBA in an amount exceeding that of the control culture by a factor of 50. However, the ubiA-transformed lines showed only a marginal (average 22%) increase of shikonin production in comparison to the control lines, and there was no significant correlation of UbiA enzyme activity and shikonin accumulation. This suggests that overexpression of ubiA alone is not sufficient to increase shikonin formation, and that further enzymes are involved in the regulation of this pathway.

Alkyl and Aryl Transferases↗

Laparoscopy combined with conventional operative techniques.

In order to establish minimal invasive methods further, it should be guaranteed that laparoscopy is not only performed in a few pediatric surgical centers. A simple approach to gain experience would be the performance of diagnostic laparoscopy. However, benefit could be increased if the surgeon combines the minimal invasive laparoscopy to establish the diagnosis with the conventional surgical technique to continue. This approach for example applies to complicated cases of appendicitis. In a combined procedure, even bowel resections and tumor extirpations can be performed with minimal invasion. In twenty-seven cases--11 appendicectomies, 14 resections of cystic ovarian tumors and 2 resections of Meckel's diverticulum--we applied this technique and found no complications so far. The surgical method presented is convenient and safe.

Appendectomy↗

Ventriculoscopy-aided implantation of ventricular shunts in patients with hydrocephalus.

Ventriculoscopy represents a new concept in the surgical treatment of children with hydrocephalus. Optimal catheter position can now be achieved with the help of a new method consisting of endoscopically controlled implantation of ventricular shunts. In addition, interventional ventriculoscopy enables or improves new operative procedures such as the fenestration of intracranial cysts or the removal of dislodged parts of catheter tips from the corresponding ventricle under direct optical control. Ventriculoscopy has been performed on 17 pediatric patients, with no complications to date.

Catheterization↗

Changes in the nature of inpatient medical services--impact on medical education and patient care.

One hundred sequential admissions to an internal medicine department in a 765-bed teaching hospital in 1973 were compared to 100 admitted in 1987. Mean age in 1973 was 50.3 years as compared to 57.4 in 1987. Length of stay shortened from 8.8 days in 1973 to 4.7 days in 1987. The overwhelming majority of the admissions during both periods had circulatory and/or respiratory disease. The number of diagnoses on admission increased from 2.3 in 1973 to 3.8 in 1987. In 1973, 22% of the patients received no drugs as compared to 6.3% in 1987. X-ray studies per hospital day doubled and invasive procedures more than quintupled. Intravenous fluids were given on 2.5% of days in 1973 and on 22.3% in 1987. Thus medical patients are now older and sicker, yet stay for much shorter periods of time. This radical change in the intensivity and tempo of work raises serious questions about the appropriateness of these sites as major loci for undergraduate teaching.

Diagnosis-Related Groups↗

Assessment of nonarticular tenderness and prevalence of fibromyalgia in children.

Fibromyalgia syndrome (FS) is most common in midlife, but may be seen at any age. Its prevalence and assessment of tenderness in healthy children is not known. We assessed 338 healthy schoolchildren for tenderness thresholds and prevalence of FS. In all children a point count of 18 tender points (TP) was conducted by thumb palpation and tenderness of some of the TP sites as well as control point sites was further assessed using a Chatillon dolorimeter. All children and their parents were questioned about the presence of widespread pain or aching. Children were considered to have FS if they met the American College of Rheumatology (ACR) criteria for diagnosis of FS. Of the 338 children, 21 (6.2%) had FS. Thresholds of tenderness of 9 TP were 5.0 (1.2) (kg) [mean (standard deviation)] for boys vs 3.6 (0.8) (kg) for girls (p < 0.001). Thresholds of tenderness of the control point sites were 7.1 (1.4) (kg) for boys vs 5.5 (1.1) (kg) for girls (p < 0.001). Thresholds of tenderness of TP and control points in the children with FS were 2.5 (0.4) (kg) and 4.2 (0.5) (kg) vs 4.5 (1.2) (kg) and 6.6 (1.4) (kg) respectively in the children without FS (p < 0.001). We suggest that FS is common in the pediatric age group. Boys have lower tenderness than girls; children with FS have lower thresholds for tenderness both at control and TP compared to the subjects without FS.

Adolescent↗

[Assessment of diabetic autonomic neuropathy by heart rate monitoring].

Assessment of diabetic autonomic neuropathy in a representative sample of 91 noninsulin-dependent diabetics was performed in 3 community clinics. A difference of less than 10 beats/min in heart rate between deep inspiration and deep expiration determined by ECG recording served as the criterion for autonomic neuropathy. By this test, 86% of the patients had diabetic autonomic neuropathy, a slightly higher proportion than that reported by others using similar criteria in more selected populations. The test is very sensitive and can be used for screening.

Adult↗

Endorphin suppresses FSH-stimulated proliferation of isolated neonatal Sertoli cells by a pertussis toxin-sensitive mechanism.

During perinatal development, when the size of the Sertoli cell population is determined, Leydig cells produce beta-endorphin, a peptide which may interact with Sertoli cells to modify their FSH-responsiveness, as suggested by our previous work. The goal of the present study was first, to test directly the possibility that beta-endorphin modifies the proliferative response of neonatal Sertoli cells to FSH, and second, to gain information on a mechanism(s) involved in any observed effect. We treated isolated 6-day-old Sertoli cells with FSH or vehicle in vitro and measured their incorporation of exogenous, radiolabeled thymidine with quantitative autoradiography. After 2 days in culture with FSH, we detected a 10-fold increase in the rate of Sertoli cell proliferation. The level of cell division in these FSH-treated cultures was identical to that in other cultures exposed to cAMP under similar conditions. In addition, inclusion of beta-endorphin 3 hr prior to FSH or cAMP decreased the effect of the hormone by 50% but left the cAMP response unchanged. Thus, beta-endorphin acts on isolated, neonatal Sertoli cells at a point prior to intracellular production of cAMP to suppress their response to FSH. When other cultures were treated with pertussis toxin, a blocker of intracellular GTP-binding proteins such as Gi, before sequential addition of endorphin and FSH, the effect of beta-endorphin on FSH-responsiveness was abolished. Moreover, when other cultures were exposed to pertussis toxin in the absence of endorphin, followed by FSH, their response to the hormone was unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗