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Biomedical subjects

J Engert

Publications and source records attributed to J Engert.

At least 19 recordsLinked to original sources

Large supraumbilical pseudocystic tumour due to ectopic pancreatic tissue located in a rest of the omphaloenteric duct.

Ectopic umbilical pancreatic tissue is extremely rare. We report on a case of a two-year-old boy who suffered from a large recurrent supraumbilical tumour with central cystic degeneration. Ectopic pancreatic tissue was located within the submucosal layer of an umbilical rest of the omphaloenteric duct. Peptic erosion and inflammatory alteration of tissue surrounding the umbilical vein caused recurrent bleeding and formation of a pseudocyst as well as chronical inflammatory granulations within the abdominal wall.

Choristoma↗

Treatment of recurrent malignant sacrococcygeal germ cell tumors: analysis of 22 patients registered in the German protocols MAKEI 83/86, 89, and 96.

PURPOSE: To evaluate therapeutic options for recurrent malignant sacrococcygeal germ cell tumors (GCT) following three-agent, cisplatinum-based, first-line chemotherapy and tumor resection. PATIENTS AND METHODS: Twenty-two patients were evaluated in 22 first-, 14 second-, five third-, and two fourth-relapse situations. One patient, who relapsed with pure teratoma, was excluded from the analysis of adjuvant treatment. RESULTS: Seventeen patients presented with an isolated local recurrence, two patients showed a distant relapse, and three patients suffered from a combined local and distant recurrence. Twelve patients achieved complete remission (CR) after surgery (n = 12) and adjuvant platinum chemotherapy (n = 10). Seven of these patients remain in continuous CR, and five patients relapsed. All patients who achieved only a partial remission developed a second relapse. Three of 14 patients could be cured after a second (or further) relapse. Altogether, 10 patients survived disease free, and 12 patients died as a result of tumor progression (n = 11) or therapy-related complications (n = 1). The completeness of salvage surgery and clinical remission status after first salvage treatment were the most important prognostic parameters. In addition, patients in first or second relapse with locally advanced or poorly responding tumors benefited from preoperative chemotherapy in combination with regional hyperthermia (RHT). In some patients after microscopically incomplete resection, irradiation at doses > 45 Gy contributed to a favorable outcome. CONCLUSION: The complete resection of the local recurrence represents the cornerstone of salvage treatment. Preoperative platinum-based chemotherapy, combined with RHT in some patients, facilitates complete tumor resection. Radiotherapy should be reserved for those patients with microscopically incomplete tumor resection. As the chance of cure decreases with further relapses, it is important to establish a stringent therapeutic strategy to avoid significant treatment delays and, most importantly, insufficient local therapy.

Actuarial Analysis↗

Nephritogenic ochratoxin A interferes with mitochondrial function and pH homeostasis in immortalized human kidney epithelial cells.

The ubiquitous nephritogenic and carcinogenic fungal metabolite ochratoxin A (OTA) has been shown to interact with renal cell function at low nanomolar concentrations. This is possibly brought about through changes in cellular pH (pHc) homeostasis and mitochondrial function. We assessed the effect of nanomolar concentrations of OTA on pHc homeostasis and the possible involvement of mitochondria using immortalized human kidney epithelial (IHKE1) cells. Within seconds OTA evoked a decrease of pHc with a threshold concentration of 0.1 nmol/l, followed by a sustained alkalinization. Acidification was the same in bicarbonate and non-bicarbonate Ringer solution. When Ca2+ entry across the plasma membrane was prevented, virtually no OTA-induced pH changes could be observed. Inhibition of Na+/H+-exchange (NHE, Na+-free solution) and H+-ATPase (bafilomycin A1) did not reduce the OTA-induced acidification. By contrast, determination of NHE activity as a function of pHc revealed that OTA stimulates NHE (maximal flux increases) in a Ca2+-dependent manner. OTA exposure did not increase lactic acid production, indicating that anaerobic glycolysis was not enhanced. Inhibiting complexes I, III and IV of the mitochondrial electron transport chain (ETC) with rotenone, antimycin A and CN- prevented the OTA-induced acidification almost completely. Completely inhibiting F1FO-ATPsynthase with oligomycin reduced the effect of OTA by approximately equal 50%. In addition, OTA induced a hyperpolarization of the mitochondrial membrane potential (psim) in a Ca2+-dependent manner. Furthermore, OTA exposure resulted in a mitochondria-dependent increase of the cellular ATP content. We conclude that OTA activates mitochondria and NHE by interfering with cellular Ca2+ homeostasis. Stimulation of mitochondrial metabolism leads to enhanced "proton production". Anaerobic glycolysis is not enhanced.

Antimycin A↗

A pancreatic pseudocyst with acute hemorrhage in a child--secondary to a spherical duodenal duplication? A case report.

Duodenal duplications are rare congenital malformations with variable and non-specific symptoms. A spherical duodenal duplication associated with a pancreatic pseudocyst causing an acute bleeding due to a vascular erosion in a 16-month old child is presented. The possibility of a causal relationship between duodenal duplication and pancreatic pseudocyst is discussed.

Common Bile Duct↗

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↗

Teratomas in infancy and childhood.

BACKGROUND AND PROCEDURE: Outcomes in children with teratomas collected between October 1982 and December 1995 in cooperative protocols of the German Society of Pediatric Oncology and Hematology (GPOH) were analyzed. Teratomas were diagnosed in 329 (42%) of 780 registered patients with germ cell tumors. The annual incidence was 0.24/100,000. Main primary sites were coccygeal (n = 132, 2.2:1 female predominance), ovary (n = 81), testis (n = 40) and brain (n = 15, 2.8:1 male predominance). RESULTS: Two hundred seventy cases of extracranial non-testicular teratoma were evaluated: In mature teratomas (n = 154) the observed relapse rate was 10%. Incomplete resection was the main risk factor for relapse. After complete resection, the relapse-free survival (RFS, Kaplan-Meier-estimation) was 0.96 +/- 0.01 (n = 126, observation time 18-155 months) in comparison to an RFS of 0.56 +/- 0.09 in incompletely resected teratomas grade 0 (n = 28, observation time 28-94 months) (P < 0.01). Im-mature teratomas were treated by surgery alone in 76 cases and by surgery and adjuvant chemotherapy in 40 cases. The observed relapse risk was 18%. Main risk factors for relapse were incomplete tumor resection (n = 38) as well as immaturity in incompletely resected teratomas. Fifteen of 29 relapsing patients presented with malignant tissue in the recurrent tumor (mainly yolk sac tumor); in contrast, seven of 40 patients with immature teratoma relapsed despite adjuvant chemotherapy without showing malignant components (P = 0.014). Nine of 36 (25%) relapsing patients died of disease. Eleven of the 27 (41%) surviving children suffered from mutilation after repeated surgery. COMMENTS: It is suggested that an international randomized trial for patients with incompletely resected high risk teratoma be initiated to evaluate the effect of adjuvant chemotherapy on specific end-points: 1) influence on relapse rate in general; 2) reduction of the proportion of malignant relapses; 3) avoidance of mutilating surgery.

Adolescent↗

Molecular genetic analysis and human chorionic gonadotropin stimulation tests in the diagnosis of prepubertal patients with partial 5 alpha-reductase deficiency.

UNLABELLED: Reduced conversion of testosterone (T) to dihydrotestosterone (DHT) results in defective virilization in karyotypic males. Different mutations in the 5 alpha-reductase type 2 gene cause the phenotypic variability of the disease. In this report we describe four prepubertal patients with a predominantly male phenotype who carry homozygous point mutations in the 5 alpha-reductase type 2 gene and address the specific T and DHT response to different human chorionic gonadotropin (hCG) stimulation tests. For molecular genetic analysis, DNA from peripheral blood leucocytes was studied. The coding region of the 5 alpha-reductase type 2 gene was characterized by exon-specific polymerase chain reaction amplification, non-radioactive single strand polymorphism analysis, and direct sequencing. Three different homozygous point mutations (Gly196-Ser, Arg227-Gln and Ala228-Thr) were identified in the patients. In contrast, in the DNA from 100 phenotypically normal males only two heterozygous abnormalities (Ile196-Ile, delta Met157) were characterized. For hormonal studies, T and DHT were measured in serum before and after hCG stimulation employing different protocols. HCG stimulation with 5000 IU/m2 once and prolonged stimulation with seven injections of 1500 IU hCG per single dose every other day were used. CONCLUSION: While abnormal T/DHT ratios were identified with both hCG protocols in the patients, prolonged stimulation lead to higher T values and to higher T/DHT rations, and hence to a better discrimination of pathologic results.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗

Echocardiographic proof of pulmonary hypertension with irreversible increased resistance in the pulmonary circulation as a complication after placement of a ventriculo-atrial shunt for internal hydrocephalus.

Increased resistance in the pulmonary vessels in children with ventriculo-atrial shunts is a rare and often unrecognized permanent complication. We report 2 children in whom this diagnosis was detected by two-dimensional echocardiography. The first patient received a ventriculo-atrial shunt at age 9 days for congenital internal hydrocephalus. At 17 months it had to be replaced because of infection of the efferent catheter limb. At 22 months at a routine follow-up the echocardiographic diagnosis of pulmonary hypertension was made. Invasive studies confirmed the presence of irreversible increased resistance in the pulmonary circulation. The second patient received a ventriculo-atrial shunt at age 13 months because of a cerebral cyst. After repeated catheter infections, at 28 months a ventriculo-peritoneal shunt was placed. At age 4 years the diagnosis of pulmonary hypertension was made by routine echocardiography. This finding was confirmed by invasive studies. The left pulmonary artery was completely occluded. Both patients had developed microemboli, caused or aggravated by catheter sepsis, in the second case probably through contiguous clot growth up to complete occlusion of the left pulmonary artery. Therapeutic measures seemed not to be indicated. Two-dimensional echocardiography proved to be a reliable method for diagnosing increased resistance and pulmonary hypertension. We recommend routine echocardiography for follow-up in all children with ventriculo-atrial shunts.

Cerebrospinal Fluid Shunts↗

[Intestinal triplication--a case report of embryogenesis of tubular and cystic small intestine duplication].

Case report of a triplication of the ileum that has not been described so far in this form, with tubular as well as additional cystic duplication within the first malformation, in a boy of 3 years of age. Due to recurrent massive bleeding per anum and the impossibility of separation, resection of the entire involved intestine was performed. The cause of bleeding was found to be a 10 to 8 mm ulcer within the dystopic gastric mucosa that extended into the submucosa. The theories of pathogenesis are discussed, implying that there is no uniform genesis of this disorder. The clinical pattern, diagnosis and therapy are that of the duplication.

Child, Preschool↗

[Thoracic surgery emergencies in early childhood].

Thoracic surgical emergencies in early childhood refer mainly to congenital malformation. To enable a successful treatment, disclosing of provable malformations by prenatal ultrasound screening and gradual sonographic diagnostics are necessary. Management of thoracic emergency situations should take place within an interdisciplinary team. Progress can only be expected by improved ultrasonographic examinations, planning of dilivery, intrapartum management, post partal differentiated diagnostic and therapy. As those prerequisits are not provided everywhere, medical care of those newborns effected in qualified centers is to be recommended.

Abnormalities, Multiple↗

Surgical correction of virilised female external genitalia.

Recent investigations and reports on late results indicate that vaginal orgasm is more the exception than the rule, so that, for a woman, preservation of clitoral sensitivity is essential to a satisfying sexual life. All techniques involving total clitoridectomy, plastic imitations, or displacement of the clitoris under the symphysis must therefore be discarded. Even if plication or trapping of an enlarged clitoral shaft under the mons veneris can be regarded as sensitivity-maintaining procedures, they nevertheless do not yield satisfactory results, since painful sensations or a feeling of pressure may occur during erection. Hence, reduction-planess should use techniques which shorten the erectile parts of the clitoris and reduce its size, while still maintaining sensitivity. Good cosmetic and tactile results may be achieved by means of selective excision of the corpora cavernosa and lateral clitoral excisions. Reconstruction of the labia minora out of clitoral shaft skin is combined with separate creation of a neo-preputium clitoridis. Vaginal enlargement plasties have always been problematic, since shrinking particularly of the vaginal introitus, occurs in up to 25% of patients who undergo this operation. However, a sufficiently large pediculated perineal skin flap inserted into the "defect" of the posterior vaginal wall provides sufficient width of the vaginal introitus and canal. Partial vaginal aplasia, with the vagina opening into a urogenital sinus near the bladder, calls for additional abdominal mobilisation. For psychological reasons, vaginal dilatations are not to be recommended. If necessary, a second vaginal enlargement plasty should instead be performed later; this may be carried out without problems before puberty. To avoid the disadvantage of a dry skin flap which does not assimilate to normal vaginal mucosa even after many years and with oestrogen treatment, mobilisation of the posterior vaginal wall with displacement of real vaginal mucosa towards the perineum can be carried out. However, one-stage reconstruction of clitoris, vulva and vagina during early childhood is preferable in every case, in order to avoid the psychological damage which can undoubtedly otherwise be caused. An exception is the late onset form of congenital adrenal hyperplasia.

Adolescent↗

[Bladder exstrophy and quality of life].

The primary objectives of operative management of exstrophy of the urinary bladder are to obtain secure closure of pelvic girdle and abdominal wall, unimpeded voiding, urinary continence, absence of reflux, preservation of renal function, functional and cosmetically acceptable external genitalia, and the absence of malignancy. These objectives cannot be fully achieved by only one of the established procedures such as functional bladder closure, internal or external urinary diversion and epispadias repair. Early individual and creative surgical management including changing concepts when needed and careful life-long followup are thought to provide the basis for a satisfying social adjustment and quality of life.

Adolescent↗

[Psychotrauma associated with ambulatory and inpatient surgery in infants. A prospective study].

The presumed advantages of day-case surgery are often based on the argument that this kind of surgery induces less psychic harm in children than inpatient care. However, medical psychologists in particular increasingly prefer short-term hospitalization with rooming-in or daily visits by the mother. Neither opponents nor supporters of day-case surgery could produce valid findings from controlled and methodically valid studies of this subject. MATERIAL AND METHODS. A controlled, prospective study of the psychogenic effects of non-acute surgery under general anesthesia in children between 2 and 6 years of age was performed. The childrens' psychologic alterations were measured 6 and 12 weeks after the operation using a structured interview of the mother. Twenty-four children were treated by day-case surgery, 28 as inpatients. All children belonged to ASA groups I and II. The interviewer was "blind" to the indication, kind of surgery and duration of hospitalization. The mothers' answers concerning the postoperative behavior of their children were coded in terms of frequency of changes (seldom, unchanged, more frequent) or intensity (vigorous, unchanged, slight) in comparison to the time before operation. The data were compared to the anxiety of the children and mothers before premedication. The childrens' anxiety was measured using the scoring system of Büttner and Breitkopf; the mothers' anxiety was measured using a modified scoring system according to Groeger and Grosse-Aldenhövel. RESULTS. 1. There were no significant differences between the day-case and the stationary groups, in either preoperative anxiety assessments or postoperative psychological findings. 2. In comparison with the total number of possible psychologic effects, the number that really occurred was very small; only in 6 of 81 items did more than one-third of the mothers report any changes. 3. Most items were reported as minimal changes. Therefore, all items had to be analyzed using the method of analysis of factors (mean components, varimax rotation, Scree-test). Within 44.7% of extracted variance there were 2 factors involving behavior. Items applying to factor 1 included disturbed sleep and increased attachment of the child to the parents, indicating "emotional confusion" or "troublesome behavior." Factor 2 indicated a more flexible manner of playing and increased ability to concentrate.4+

Ambulatory Surgical Procedures↗

[Indication and use of the fixateur externe in childhood].

The use of the fixateur externe in childhood is an indispensable extension of all measures taken by the paediatric traumatologist to preserve the skeletal system. In recent decades external fixations by means of Kirschner wires in association with plaster casts or in the Küntscher distractor had become necessary on account of increasing severity of the injuries to the extremities. Now a reduction in size and weight as well as multidimensional applicability in conjunction with microsurgical vascular reconstructions and neurovascular grafting of soft parts have increased the indications and considerably improved the results achieved. The article demonstrates by means of own examples how the indication for internal and external stabilisation must be weighed, taking into consideration the special features of the growing skeleton, the age, the type of injury, the localisation and extension of the injury. Choosing a particular type of osteosynthesis does not necessarily exclude transition to another type. The type of fixateur externe described here (Hoffmann) represents just one possibility of a system which is possible in principle.

Adolescent↗

[Secondary surgery for restoration of nerve function (author's transl)].

The technique of nerve-suturing belongs in modern times to the domain of microsurgery. Without implementing the posibilities offered by microsurgery, nerve-suturing - even directly end-to-end - is obsolete. In order to obtain a sufficient functional end-result, suturing under tension must be avoided at all costs. Knowledge of this has given rise to the common interposition nerve grafts. Even with interposition grafts, no cases of growth-disturbances, including a failure of the nerve to grow or disturbances in function because of growth, have been observed in children. For a good post-op. results, a continual therapy with neurotropic medicaments, physiotherapy and faradisation, and neurophysiological checks on progress made in renervation are absolutely essential.

Child↗