Impaired glucose tolerance leads to delayed diagnosis of Alström syndrome.

OBJECTIVE: To emphasis the need for timely diagnosis of Alström syndrome. CASE: We report a case of late-detected Alström syndrome in a boy 15 1/2 years of age who was admitted because of poor wound healing and hyperglycemia. RESULTS: Diagnosis was made and the patient's impaired glucose tolerance improved by diet. CONCLUSIONS: Regular follow-ups are needed to recognize and possibly prevent late-appearing complications.

Phase I metabolism of imipramine by microsomes of small intestine in comparison with metabolism by liver microsomes.

The metabolism of imipramine was investigated by the incubation of C-14 labelled compound in Krebs-Ringer bicarbonate buffer with microsomes of small intestine and of liver from guinea pigs. Imipramine and its metabolites were extracted with chloroform, separated by TLC and determined quantitatively by direct scanning with a TLC Linear Analyzer. With intestinal microsomes, the following metabolites could be identified: DMI, 2-OH-IMP, IMP-N-oxide. DMI is the main metabolite. The same metabolites appeared after incubation with liver microsomes, but the proportions were different: the N-oxide formation was predominant, followed by N-demethylation and hydroxylation. The formation of DMI and 2-OH-IMP seems to follow Michaelis-Menten kinetics, both in assays with intestinal and with liver microsomes. The liver/intestine ratio of DMI and 2-OH-IMP formation is proportional to the cytochrome P-450 ratio in the microsomes, in contrast to N-oxide formation.

[The value of MRT of the thorax in congenital tracheal stenoses]. / Wertigkeit der KST des Thorax bei kongenitalen Trachealstenosen.

MRI of the thorax was performed in 24 children aged between six weeks and five years, in whom a tracheal stenosis had been demonstrated by bronchoscopy. Since bronchoscopy can only demonstrate the interior of the trachea, various imaging methods, such as CT and angiography, were used to demonstrate the topography and cause of the tracheal stenosis. MRI has shown that the most common cause of a stenosis in the central section of the trachea is focal compression by an aberrant brachiocephalic trunk (10 cases). Stenosis of the distal trachea could be due to anomalies of the aortic arch (5 cases), a dilated pulmonary artery (4 cases) or a soft tissue mass (3 cases). In all these patients, MRI was greatly superior to the conventional methods. By using a special technique, MRI made it possible to clarify the cause and localisation of a tracheal stenosis by a non-invasive examination.

Quality of paediatric IDDM care in Germany: a multicentre analysis. German Paediatric Diabetology Group.

Quality management has been applied in recent years to improve the care of children and adolescents with insulin dependent diabetes mellitus (IDDM). In 1995 the German Paediatric Diabetology Working Group published standards on quality control, in which relevant parameters on structure, process and outcome of care were defined. A computer software programme-developed at the University of Ulm under the auspices of the German Secretary of Health-has been used for quality control with central anonymous analysis in a nationwide survey. Data from 23 paediatric centres with 2407 patients seen between January and June 1996 were evaluated. The results showed an admission rate to hospital of 23.8 per 100 patient-years with an average duration of in-patient stay of 2.74 days/year. 80% of the patients were treated with an intensive insulin therapy regimen comprising three or more injections daily. The overall metabolic control was reasonably good with a mean HbA1c value of 7.8%. The rate of severe hypoglycaemia complicated by coma and/or convulsions was six per 100 patient-years and of ketoacidosis one per 100 patient-years. Unfortunately screening for diabetic retinopathy and nephropathy was not carried out consistently. The incidence was 44% and 33% respectively.

Reverse smooth muscle plasty: a new method of treating anorectal incontinence in infants with high anal and rectal atresia.

A new technique of treating anorectal incontinence in infants with high anal and rectal atresia is described. In the first stage of this new operation an abdominosacroperineal pull-through procedure according to Rehbein, Romualdi, and Kiesewetter is performed. In the next step of this new method the circular and longitudinal muscle layers of the pulled-through colon are turned back 180 degree and fixed to the pulled-through bowel. The mucosa of the turned back bowel is removed. Then the whole muscle cuff is re-pulled into the puborectalis sling. Pressure studies as well as X-ray investigations showed a good sphincter function of the newly established internal sphincter equivalent.

Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy.

Three cases of male infants with hypoplasia and aplasia of the right lung and dextrocardia are reported. The infants developed increasing obstructive respiratory distress in the first 4 months of life. In all three cases, there was an opaque right hemithorax with overinflation of the left lung, a posterior deviation of the trachea with pulsatile stenosis, and a posteriorly located aortic arch. Hypoplasia (two cases) or aplasia (one case) of the right pulmonary artery and an absence or a remnant of the right main bronchus were also observed. Associated malformations were esophageal atresia with an abnormal high pouch of the proximal esophagus in case 1, and hypertelorismus syndrome in case 2. Because of severe respiratory distress in all three patients, an aortopexy was performed at the ages of 5 months (one infant) and 10 months (two infants). The obstructive respiratory signs disappeared postoperatively in the following weeks. It has been observed that tracheal stenosis in aplasia or hypoplasia of the right lung may be caused by the dislocated aortic arch. An aortopexy can release the airway compression in such cases. Because of associated malformations, a careful evaluation of the airway and vessels is recommended.

Ileus in the newborn: a study of decreasing mortality.

The authors' experience with operative therapy for ileus-duodenal, small intestinal, colon, and rectal-in newborns is presented with an analysis of the improvements in prognosis over the last 30 years. For the time period 1980-1989, the following survival rates were achieved: duodenum: 100%; small bowel: overall 96.8%, for uncomplicated cases 100%; congenital megacolon: overall 89%, for uncomplicated cases 100%; ileus of all intestinal portions together: 97.5%, for uncomplicated cases 100%.

Lobar emphysema and atelectasis syndrome, a nosological unity.

Shrinking and atelectatic pulmonary processes--atelectasis syndrome--and lobar emphysema show morphological similarities: congenital malformations of the bronchial structure, external compression of the bronchi as well as inflammatory processes. Ventilation mechanics determine whether in the described processes an atelectasis or an emphysema manifests itself clinically. Usually, one finds over-expansions in connection with atelectasis syndrome and atelectases together with lobar emphysema. Both processes can be reversible, if we are not dealing with bronchial malformations. Longer lasting processes as well as acute, life threatening forms require operation. The belief that atelectasis syndrome and lobar emphysema constitute a nosological unity is illustrated by our cases of 9 patients with atelectasis syndrome and 16 patients with lobar emphysema.

Clinical findings in middle lobe syndrome and other processes of pulmonary shrinkage in children (atelectasis syndrome).

Processes of atelectasis and pulmonary shrinkage are not confined to the right middle lobe. This fact is illustrated by case reports of five of our nine such patients. All cases shared common clinical and morphologic similarities: congenital malformations of the bronchial skeleton, compression, and chronic inflammation produce comparable pulmonary morphology that always includes atelectasis. Atelectasic processes of longer duration require surgical resection; this led to cures in all the authors' patients.

New morphological findings in so-called negative appendectomies.

Appendices of children operated on because of clinical signs of acute appendicitis and presenting normal histological findings were revised by means of special assays. These findings were then compared with appendectomies during abdominal surgery, catarrhal and early phlegmonous appendicitis. We found pathological changes of the epithelium and the lymphatic tissue in more than two thirds of the specimens originally classified as normal. Therefore the term of "negative appendectomy" has to be redefined.

[Invagination in childhood]. / Zur Invagination im Kindesalter.

This paper is a retrospective study of all children with intussusception, who were treated in the pediatric surgical clinic in the Dr. v. Hauner'sches Kinderspital of the University of Munich from 1970 to 1988. In this time we evaluated 99 cases out of 101 children. Points of interest were epidemiological data, such as sex ratio, average age, place of residence, exact documentation of the anamnesis typical of this pediatric surgical entity, of diagnosis, site of intussusception as well as of frequency of conservative barium enema reduction and surgical treatment respectively. 68 patients of these evaluated 99 cases had to be operated. As far as postoperative courses are concerned bowel obstruction occurs in 6 patients, 4 cases developed a relapse of intussusception and one child died.

[Postoperative pneumothorax following interventions for diaphragmatic hernias and defects]. / Postoperativer Pneumothorax nach Eingriffen wegen Zwerchfellhernien und -defekten.

Basing on the fact that postoperative pneumothorax occurred in a third of 47 patients with diaphragmatic hernias and defects, the mechanisms of progressive respiratory insufficiency and the occurrence of postoperative pneumothorax are debated as well as therapeutical measures for prevention. It is suggested as an important therapeutical principle in order to prevent any negative pressure on the operated thoracic side and to keep by all means the mediastinum in the middle. By the aid of these therapeutical principles, an improvement of the still high lethality of 40% - which postoperative pneumothorax is decisive for - can be achieved.

[Surgical closing of recto-vaginal, recto-urethral, urethro-vaginal and vesico-cutaneous fistulas by means of interposition of the gracilis muscle]. / Der operative Verschluss recto-vaginaler, recto-urethraler, urethro-vaginaler und vesico-cutaner Fisteln durch Interposition des Musculus gracilis.

Ten cases of recto-vestibular, recto-vaginal, recto-urethral, urethro-vaginal, and vesico-cutaneous fistulas in children are reported. The fistula was closed by an interposition of the gracile muscle in every case. No relapse has been observed to date, 1.5 to 6 years after the operation. The surgical technique is described.

[Surgical therapy of neonatal necrotizing enterocolitis]. / Die operative Therapie der neonatalen nekrotisierenden Enterocolitis.

Between 1972 and 1988, a total of 57 patients with an average birth weight of 1988 g and an average gestational age of 34.1 weeks were operated on for neonatal necrotizing enterocolitis. In 48% of the cases, the critical indication for surgery was determined by clinical examination; in 52% of the cases, the indication was based upon a radiological diagnosis of pneumoperitoneum. The affected intestinal portions were first resected. Then, the proximal and distal limbs were sewn together and brought out through a separate short incision. Utilizing this procedure, the lethal rate sank from above 80% to below 30%. Taking into account unrelated causes of death, no significant increase in risk has been associated with the use of this procedure on prematurely born patients and patients exhibiting acute intestinal perforations.

Validation of dry heat inactivation of bacterial endotoxins.

Bacterial endotoxins (ETs) are lipopolysaccharides from the cell wall of Gram negative bacteria. ETs get into the environment as a result of autolytic desintegration of the bacterial cells. There exist a number of depyrogenation methods, either serving to remove or to inactivate ET. The most common means of ET inactivation is dry heat. Unfortunately no uniform regulation exists describing the conditions for sufficient ET inactivation. While the USP and FDA require an ET reduction of 3 log steps, no European regulation exists regarding depyrogenation of final containers for parenterals. However, the Ph. Eur. specifies the temperature and time conditions for depyrogenation of glassware in the pyrogen test monograph, allowing to choose between the two variants 250 degrees C/30 minutes or 200 degrees C/60 minutes which are not equivalent. In the present study those conditions for depyrogenation of glass containers in production of parenterals were investigated which, on the one hand, are technically feasible and, on the other hand, comply with the requirements of the main Pharmacopeias; furthermore, an ET preparation suitable for validation studies was selected. The preparation of ET indicators, the dry-heat inactivation and the recovery of ET are described in detail. Based on the results obtained, it is recommended to follow a defined treating temperature and period for safe depyrogenation of glass containers for parenterals, which results in a 4 log step reduction in ET without fillers. Thereby the USP/FDA requirement for a 3 log step reduction as well as the 200 degrees C/60 minutes requirement variant given in the Ph. Eur. can be fulfilled.