PubMed HealthSearch

Biomedical subjects

S A Pedersen

Publications and source records attributed to S A Pedersen.

At least 19 recordsLinked to original sources

Comprehensive 1-year cost analysis of open antireflux surgery in Nordic countries. Nordic GORD Study Group. Gastro-oesophageal reflux disease.

BACKGROUND: Antireflux surgery is a highly effective treatment option in patients with severe gastro-oesophageal reflux disease. However, because of the increasing pressure of cost containment within health care, cost aspects must also be added to the decision-making process. METHODS: The aim of this analysis was to assess the total cost of open antireflux surgery during the first year after operation in 178 patients with chronic reflux, who were recruited into a controlled, prospective clinical trial. The study was carried out in 17 hospitals in the Nordic countries. RESULTS: The cost of the operation represented more than 90 per cent of the direct medical costs which amounted to approximately US $5700. For a patient in the work force the indirect cost, i.e. loss of production, represented 47 per cent of the total cost, which was about US $10800. CONCLUSION: The total cost profile of open antireflux surgery has now been established prospectively and can form a basis for future comparisons.

Adolescent

Effects of growth hormone replacement therapy on IGF-related parameters and on the pituitary-gonadal axis in GH-deficient males. A double-blind, placebo-controlled crossover study.

It has been suggested that growth hormone (GH) may play a regulatory role in male reproductive function. To express full anabolic effect in immature boys testosterone apparently requires the presence of GH. In GH-deficient adults, GH replacement therapy exerts a variety of anabolic actions, some of which are similar to the effects of gonadal steroids. However, little is known about the potential effects of GH on gonadal steroids and on dynamic tests of pituitary-gonadal function in adults with GH deficiency. We evaluated the pituitary-gonadal axis in a 4-month double-blind, placebo-controlled GH study in 13 young males with childhood-onset GH deficiency of which 6 had isolated GH deficiency. GH treatment significantly increased serum levels of total IGF-I from 98 (68) to 323 (126) microg/l, free IGF-I from 0.48 (0.47) to 2.24 (1.66) microg/l, IGFBP-3 from 1,874 (1,178) to 3,520 (778) microg/l and ALS levels from 9,182 (5,524) to 16,872 (6,278) microg/l (all p < 0.0001). We found no differences in basal testosterone levels in the 13 patients between the GH and placebo treatment periods (21.9 (5.1) vs. 24.5 (8.1) nmol/l, nonsignificant). Furthermore, no effect of GH on the testicular response to hCG after 72 h was seen compared to placebo (36.2 (6.4) vs. 38.8 (10.3) nmol/l). In addition, no differences existed in basal SHBG, DHT, free testosterone, delta4-adion and DHEA-S levels. There were no statistically significant differences in maximal FSH and LH response to a GnRH challenge between the GH and the placebo periods (15.7 (5.3) vs. 18.0 (8.8) U/l and 47.0 (26.4) vs. 40.4 (26.5) U/l, respectively). Furthermore, there was no effect on cortisol responses after ACTH between the GH and the placebo periods. However, significantly higher estradiol levels were seen after GH treatment (110 (50) pmol/l) compared to after placebo (89 (34) pmol/l, p = 0.03). Prostate-specific antigen levels decreased after GH treatment compared to after placebo (0.42 (0.54) vs. 0.47 (0.48) microg/l) and this difference almost reached statistical significance (p = 0.059). Inhibin-B levels were significantly lower in hypogonadal patients substituted with androgens, but GH had no effect on inhibin-B levels. In conclusion, GH replacement therapy in 13 GH-deficient young adult males resulted in significant increases in total and free IGF-I as well as in ALS levels in all patients, but had no significant effect on: (1) the pituitary FSH and LH response to GnRH; (2) basal and hCG-stimulated levels of androgens and SHBG; (3) basal inhibin-B levels; (4) ACTH-stimulated cortisol secretion. By contrast, GH administration had subtle anti-androgenic effects in terms of elevated elevated estradiol levels and decreased prostate-specific antigen levels, although both parameters remained within the normal range. Thus, at the level of blood chemistry the effects of GH administration do not appear to involve major alterations in the pituitary-gonadal axis.

Adrenocorticotropic Hormone

A new peroxisomal beta-oxidation disorder in twin neonates: defective oxidation of both cerotic and pristanic acids.

Twin brothers were born with clinical symptoms indicating that they were suffering from Zellweger syndrome. However, instead of a generalized peroxisomal dysfunction, only very long-chain fatty acids and the pristanic acid/phytanic acid ratio were elevated in plasma and decreased oxidation of very long-chain fatty acids and pristanic acid was the only impairment found in fibroblasts. The other peroxisomal parameters tested were normal, including normal oxidation of phytanic acid and normal activity of dihydroxyacetonephosphate acyltransferase in fibroblasts as well as normal plasma bile acids. Although the biochemical results point to a defect in peroxisomal beta-oxidation, the isolated finding of impaired oxidation of very long-chain fatty acids and pristanic acid has to our knowledge not been reported previously and is difficult to explain by a deficiency of a known peroxisomal beta-oxidation enzyme.

Diseases in Twins

Growth hormone (GH) provocative retesting of 108 young adults with childhood-onset GH deficiency and the diagnostic value of insulin-like growth factor I (IGF-I) and IGF-binding protein-3.

Serum levels of total insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) reflect the endogenous GH secretion in healthy children and exhibit little diurnal variation, which makes them good diagnostic markers for screening of GH deficiency (GHD) in short children, although some controversy still exists. In adults, the diagnostic value of IGF-I and IGFBP-3 suspected of GHD has been reported in only a few studies. We performed a GH provocative test, using oral clonidine, in 108 patients who had previously been treated with GH during childhood (73 men and 35 women). Basal IGF-I and IGFBP-3 levels were compared to those in 1237 healthy controls (312 controls > 18 yr) as well as to peak GH levels. Seventy-nine patients had peak GH values below a cut-off value of 7.5 micrograms/L (34 with isolated GHD), whereas 29 patients had a normal GH response (28 with previous isolated GHD), i.e. 45% of patients treated with GH during childhood because of isolated GHD had a normal GH response when retested in adulthood. Multiple regression analysis revealed that peak GH levels were dependent on the degree of hypopituitarism, body mass index, and duration of disease. IGF-I levels were below -2 SD in 60 of 79 GHD patients and above -2 SD in 21 of 29 patients with a normal GH response. IGFBP-3 levels were below -2 SD in 54 of 79 GHD patients and above -2 SD in 23 of 29 patients with a normal GH response. Multiple linear regression analysis demonstrated that IGF-I and IGFBP-3 were significantly dependent on peak GH levels and the number of other pituitary axes affected. In this analysis, duration of disease was significantly associated with both IGF-I and IGFBP-3, whereas body mass index was significantly associated with IGFBP-3, but not with IGF-I. We conclude that IGF-I and IGFBP-3 determinations predict the outcome of a GH provocative test in adults suspected of GHD and believe that IGF-I as well as IGFBP-3 serum concentrations are valuable diagnostic parameters in the evaluation of GHD in adults with childhood-onset disease. We suggest that children who have been treated with GH should undergo reassessment of their GH secretory status as young adults by provocative testing as well as by IGF-related peptides before continued adult GH replacement therapy is considered. However, our data suggest that it is not necessary to reconfirm GH deficiency by GH provocative testing in young adults who have two or more pituitary hormone deficiencies in addition to GHD.

Administration, Oral

Free insulin-like growth factor I serum levels in 1430 healthy children and adults, and its diagnostic value in patients suspected of growth hormone deficiency.

Serum levels of total insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) reflect endogenous GH secretion in healthy children, which makes them good diagnostic markers for screening of GH deficiency (GHD) in short children, although some controversy still exists. Only a minor fraction of the total IGF-I circulates in its free form, which is believed to be the biologically active form. However, our knowledge of the clinical or physiological value of determination of free IGF-I in serum is limited at present. In adults, the diagnostic value of total IGF-I and IGFBP-3 determinations in patients suspected of GHD has only been reported in a few studies, whereas no previous reports on the diagnostic value of free IGF-I levels in adults suspected of GHD exist. Serum levels of free IGF-I were determined in 1430 healthy children, adolescents, and adults by a newly developed, commercially available immunoradiometric assay (Diagnostic Systems Laboratories) to establish valid normative data for this analysis. We studied the diagnostic value of free IGF-I in relation to total IGF-I and IGFBP-3 determinations in adults who were suspected of GHD. A GH provocative test, using oral clonidine, was performed in 108 adult patients who had previously been treated with GH in childhood. In healthy subjects, free IGF-I levels increased during childhood, with the highest mean values during puberty. After puberty, a subsequent decline in serum levels of free IGF-I was apparent. We found, unmeasurable free IGF-I values in 34 of the prepubertal children (3.3%). All individuals over 8 yr of age had measurable free IGF-I levels that amounted to approximately 1% of the total IGF-I concentrations. Free IGF-I levels were below--2 SD in 56 of 79 GHD patients (sensitivity, 71%) and above--2 SD in 24 of 29 patients with a normal GH response (specificity, 83%). Multiple linear regression analysis demonstrated that free IGF-I was significantly dependent on peak GH levels, duration of the disease, and number of other pituitary axes affected. We conclude that free IGF-I serum levels increase during childhood with a peak in puberty, whereafter free IGF-I levels return to prepubertal levels. Three percent of healthy prepubertal children had unmeasurable free IGF-I levels using this assay. We found that determination of the free IGF-I serum concentration may predict the outcome of a GH provocative test in adults suspected of GHD, but that a single determination of free IGF-I offered no significant advantage compared to determination of total IGF-I or IGFBP-3 serum levels.

Adolescent

A double-blind placebo-controlled study on the effects of omeprazole on gut hormone secretion and gastric emptying rate.

BACKGROUND: The present study was designed to investigate whether an effect of omeprazole on gastric emptying is related to changes in the secretion of selected gut hormones. METHODS: The studies were performed in healthy men after 10 days' treatment with 40 mg omeprazole daily/placebo. Food ingestion took place in a duodenal phase, I and the meal consisted of an omelette labelled with technetium Tc 99m, followed by 150 ml water labelled with indium In 111. Plasma concentrations of gastrin, cholecystokinin (CCK), and motilin were measured. RESULTS: Pretreatment with omeprazole reduced gastric emptying rates. This applied to all variables and was most pronounced with regard to amounts of solid (median (95% confidence interval)) emptied at 180 min (71% (48 - 86) for omeprazole versus 96% (87 - 100) for placebo; P < 0.01). All median values of plasma gastrin concentrations from the omeprazole series were higher than the corresponding values from the placebo series, and omeprazole induced a tenfold increase in incremental integrated area (IIA, pmol/l x 180 min) of the gastrin concentration curve (5250 (2570 - 9680) versus 575 (240 - 1485); P < 0.01). New findings include a lower postprandial secretion of CCK in the omeprazole series, and consequently, a difference in total integrated area (TIA, pmol/l x 180 min) (88 (21 - 147) versus 217 (104 - 267); P < 0.05) and IIA (pmol/l x 180 min) (52 (2 - 142) versus 165 (104 - 195); P < 0.05), respectively; a difference in IIA-30 (pmol/l x 30 min) of plasma motilin concentrations (270 (140 - 595) (omeprazole) versus 460 (285 - 655); P < 0.05); and a direct relationship between the amounts of liquid emptied at 30 min and the corresponding TIA-30 of plasma motilin in the omeprazole (Rs = 0.677; P < 0.05) and the placebo series (Rs = 0.767; P < 0.05). CONCLUSION: Pretreatment with 40 mg omeprazole daily decreases the gastric emptying rates and has a substantial influence on the secretion of gastrin, motilin, and CCK. The finding of an omeprazole-induced decrease in CCK release may have clinical implications. Further investigation into the possible effect of proton-pump inhibitors on biliary tract motility and cholesterol solubilization in gallbladder bile is warranted.

Adult

Heartburn without oesophagitis: efficacy of omeprazole therapy and features determining therapeutic response.

BACKGROUND: Data are limited on the value of effective antisecretory therapy in the relief of heartburn in patients without oesophagitis. METHODS: Patients with heartburn, without endoscopic signs of oesophagitis, were randomized to double-blind treatment with omeprazole, 20 or 10 mg once daily, or placebo, for 4 weeks (n = 509). Pre-treatment oesophageal acid exposure was assessed using 24-h intra-oesophageal pH monitoring. Heartburn was assessed at 2 and 4 weeks. RESULTS: At 4 weeks the proportion of patients with complete absence of heartburn was 46% (95% confidence interval, 39-53%) with 20 mg omeprazole, 31% (25-38%) with 10 mg omeprazole, and 13% (7-20%) with placebo. Satisfaction with therapy was reported by 66%, 57%, and 31% of the patients, respectively. CONCLUSION: Omeprazole, 20 and 10 mg once daily, provides rapid relief of heartburn in patients without endoscopic oesophagitis.

Anti-Ulcer Agents

Gastric antrectomy with selective gastric vagotomy does not influence gallbladder motility during interdigestive and postprandial periods.

Fasting gastrointestinal motility and gallbladder motility during the interdigestive state and in the postprandial period was studied in eight patients who were operated for ulcer disease with an antrectomy and selective gastric vagotomy. Nocturnal motility recording revealed all three phases of the migrating motor complex (MMC) in all but one patient, where no phase III activity was recorded. In the rest of the patients 3-10 events with phase III activity were recorded. At scintigraphy ([75Se]HCAT) a cyclic gallbladder filling and emptying in relation to the MMC cycle was found. Episodes with emptying were confined to phase II and a total of 13 episodes with a median duration of 25 min (range 10-70 min) were observed. A median of 10.7% (6.1-17.7%) of the gallbladder contents was emptied. In a control group of eight healthy young men the values were 13.5 min (9-36 min) and 6.9% (3.7-31.1%), respectively. These differences were not significant. During the postprandial period, a lag period in gallbladder emptying of median 15 min (5-20 min) was observed when food ingestion took place during phase I of the MMC. Thereafter a gradual emptying occurred with a rate of 0.95% min (0.71-1.15%/min). In a control group of healthy young males, the lag period was 13.5 min (9-22.5 min) and the emptying rate 0.61%/min (0.08-0.77%/min). When food ingestion occurred during phase II of the MMC, the lag period of gallbladder emptying in the patient group was median 0 min (0-5 min) and the emptying rate was 0.77%/min (0.33-0.86%/min). The values in the control group were 0 min (-9 to 13.5 min) and 0.76%/min (0.54-2.25%/min), respectively. These differences between the patients and controls were not significant. In conclusion, antrectomy and selective gastric vagotomy do not influence fasting gastrointestinal motility or gallbladder motility during the interdigestive state or in the postprandial period.

Aged

The relationship between gut hormone secretion and gastric emptying in different phases of the migrating motor complex.

BACKGROUND: No studies are available on the relationship between the response of gut hormones and gastric emptying in different phases of the migrating motor complex. This study examined whether basal gut hormone concentrations in plasma before food ingestion are predictors of emptying characteristics and whether different hormone secretion patterns are associated with specific alterations in emptying rate. METHODS: Twelve healthy men were examined on two occasions: one with meal ingestion in phase I and the other with meal ingestion in phase II. The meal consisted of an omelette labelled with 99mTc followed by 150 ml water labelled with 111In. Plasma concentrations of gastrin, cholecystokinin, motilin, and peptide YY were measured in the fasting state, immediately after food ingestion, and at 15-min intervals in the postprandial period. RESULTS: New findings from the present study include a higher incremental integrated postprandial motilin response in phase I than in phase II (998 pmol/l*30 min (495 to 2010) versus 210 pmol/l*30 min (-270 to 2323), p < 0.05), and a linear relationship between median total integrated motilin response and solid emptying at 120 min in phase I (Rs = 0.58; p < 0.05). Furthermore, in phase I a linear relationship between total integrated area of cholecystokinin and solid emptying at 120 min was demonstrated (Rs = 0.62; p < 0.05). CONCLUSION: The findings from the present investigation have to be considered in the future design of studies that focus on postprandial release of gastrointestinal hormones. The transition from phase III to phase I is a reproducible and easily recognized pressure event. Therefore, we recommend the use of food ingestion immediately after termination of duodenal phase III.

Adult

Inhalation of nitric oxide as a treatment of pulmonary hypertension in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) still has a mortality risk of around 40%. The concomitant pulmonary hypoplasia and the persistent pulmonary hypertension are of major prognostic importance. The use of a selective pulmonary vasodilator may revert this vicious circle that is fatal to many children. Inhalation of nitric oxide (NO) has been suggested as a rational treatment of this condition. The authors report three cases of high-risk infants with CDH where NO was used successfully. It is concluded that hypoxemia in CDH can be treated successfully with NO inhalation when conventional treatment fails, and it may in some cases prove to be an alternative to ECMO.

Administration, Inhalation

Increases in plasma motilin follow each episode of gallbladder emptying during the interdigestive period, and changes in serum bile acid concentration correlate to plasma motilin.

BACKGROUND: The relationship between each single period of gallbladder emptying during the migrating motor complex (MMC) cycle and changes in concentration of plasma motilin and serum bile acids is unknown. METHODS: The variations in the concentration of plasma motilin and serum bile acids in relation to interdigestive gallbladder motility and the MMCs was studied in nine healthy male volunteers. A method combining biliary scintigraphy (99mTc-labelled dimethyl-iminodiacetic acid) and continuous pressure recording from the antroduodenal region was used. RESULTS: During 9 MMC cycles a total of 15 episodes of gallbladder emptying were observed with a median (range) duration of 25 min (15-45 min). Each episode of gallbladder emptying was followed by a steep increase in plasma motilin, reaching a median value of 30 pmol/l (13-43 pmol/l), corresponding to an increase of 18 pmol/l (4-33 pmol/l). The increase in plasma motilin started at the beginning of gallbladder emptying, but the peak value was not reached until a median of 20 min (10-45 min) later. Low plasma motilin concentrations were found between the emptying periods in cases with two or more emptying during the MMC cycle. The serum concentration of bile acids also showed a cyclic variation in relation to gallbladder motility. During periods of gallbladder emptying serum bile acid concentration had a median value of 1.78 mumol/l, as compared with a median value of 1.17 mumol/l during periods of gallbladder filling. This difference did not reach significance, however. In the pooled data from all subjects, a significant correlation (p < 0.01) between the serum concentration of bile acids and plasma concentration of motilin was found. CONCLUSION: Gallbladder emptying was followed by a steep increase in plasma motilin concentration, and in cases of two or more emptying periods during the MMC cycle the concentration decreased in between. The shape of the serum bile acid profile is dependent on the intestinal transport and absorption of bile acids, and the significance of the cyclic variation in serum concentration of bile acids in relation to plasma motilin, gallbladder motility, and MMC needs further investigation.

Adult

Absence of correlation between hepatic function and characteristics of migrating motor complexes in the gastrointestinal tract.

BACKGROUND: Cyclic changes in gallbladder filling and emptying during the migrating motor complex (MMC) cycle have been demonstrated by scintigraphy. However, a possible cyclic change in the hepatic function and handling of the pharmacologic agents used for scintigraphy during the MMC cycle could have an influence on these results. The aim of the present study was to investigate the hepatic handling of cholic acid and mebrofenin in relation to the MMCs of the gastrointestinal tract. METHODS: The plasma disappearance rate of 14C-cholic acid and the hepatic uptake and excretion of 99mTc-mebrofenin were examined during phase I and phase II of the MMC in six healthy male volunteers. RESULTS: The plasma disappearance rate of 14C-cholic acid showed a biexponential course with an initial rapid and late slow phase after a bolus injection. There were no significant differences between the initial or late plasma disappearance rate of 14C-cholic acid during phase I as compared with phase II. The results of the time-activity curves from the 99mTc-mebrofenin scintigraphy showed an exponential rapid increase in radioactivity followed by an almost linear slow decrease after a bolus injection. There was no significant difference between phase I and phase II in any of the variables studied. CONCLUSION: The lack of a relationship between hepatic handling of cholic acid and mebrofenin and MMC excludes this as a possible source of error in the investigations of the dynamic function of the enterohepatic circulation and especially gallbladder motility by the use of either cholic acids or iminodiacetic acid derivatives as investigative agents.

Adult

CATCH: classroom process evaluation in a multicenter trial.

The purpose of this paper is to describe the process evaluation model for the classroom curricula of the Child and Adolescent Trial for Cardiovascular Health (CATCH) Project. The process evaluation plan specifically targets how much each curriculum was implemented, to what degree it was implemented as designed, and the barriers to implementation. Additionally, the rationale for each of the process evaluation measures and the instrument development process are presented. Data resulting from these measures will be essential in order to answer questions regarding the internal validity of the main outcomes of the project. Specific examples and sample results are provided from the CATCH third-grade classroom curriculum, which was implemented the first year of CATCH. A discussion also is presented of how the findings from a sample of these measures were used to gain additional insight on the salient features of the curriculum, and how those features may be related to student outcomes.

Adolescent

Growth hormone (GH) treatment increases serum insulin-like growth factor binding protein-3, bone isoenzyme alkaline phosphatase and forearm bone mineral content in young adults with GH deficiency of childhood onset.

Recent studies have demonstrated that growth hormone (GH)-deficient adults have a markedly decreased bone mineral content compared to healthy adults. However, there are conflicting results regarding the effects of GH treatment on bone mineral content in GH-deficient adults. Therefore, we evaluated the effect of GH treatment on a marker of bone formation (bone alkaline phosphatase), hepatic excretory function and distal forearm bone mineral content in GH-deficient adults. Growth hormone was administered subcutaneously in 21 adults (13 males and 8 females) with GH deficiency of childhood onset for 4 months in a double-blind, placebo-controlled GH trial, while 13 of the patients then received further GH for an additional 14 months. Serum insulin-like growth factor I (IGF-I) increased significantly from 100 to 279 micrograms/l and IGF binding protein-3 (IGFBP-3) from 1930 to 3355 micrograms/l after 4 months of GH treatment (p < 0.0001). In addition, the molar ratio between IGF-I and IGFBP-3 increased significantly from 0.22 to 0.33 after GH treatment (p < 0.0001). Bone alkaline phosphatase increased significantly from 38.6 to 92.9 U/l during GH therapy in male patients (p < 0.0001), whereas liver-derived alkaline phosphatase was unaltered by GH. In the females, the increase in bone alkaline phosphatase did not reach statistical significance (19.1 vs 40.0 U/l, p = 0.06). The GH-induced increase in bone alkaline phosphatase correlated significantly with the increase in serum IGFBP-3 (r = 0.46, p = 0.04) but not with the increase in serum IGF-I (p = 0.16).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Preoperative assessment of resectability in gastroesophageal carcinoma by linear array endoscopic ultrasonography.

Preoperative endoscopic ultrasonography (EUS) was performed in 51 consecutive patients with biopsy-verified esophageal or gastric cancer, to judge resectability. A comparison between preoperative EUS and surgical (and histologic) findings was possible in 39 patients (76%). In 33 of these 39 patients (85%) EUS made a correct preoperative assessment. In three cases misinterpretation was due to metastasis outside the viewing field of the echoendoscope. Although based on preliminary experience, we are convinced that EUS will help us to select patients who will ultimately benefit from surgery. Further prospective studies are necessary to clarify the possible benefits in terms of costs, effects of palliation in selected patients, and long-term survival.

Adenocarcinoma

[Endoscopic ultrasonography of malignant tumors in the upper abdomen].

Endoscopic ultrasonography (EUS) of the upper gastrointestinal tract is a newly developed, non-invasive investigational method. It combines the direct optical picture of the endoscope with a simultaneous ultrasonographic image of the whole wall of the oesophagus, stomach and duodenum as well as organs and other structures with anatomical relations to the upper gastrointestinal tract. EUS is evaluated for its capacity with respect to cancer of the oesophagus, stomach, pancreas, biliary tree and neuroendocrine tumours, the emphasis being laid on TNM-classification, assessment of resectability and comparison with other imaging techniques. It is concluded that EUS is well-suited for assessing tumour infiltration and thereby also the resectability of these cancer types. Judging whether lymph nodes visualized by EUS are malignant or not is difficult when operating solely from endosonographic and quantitative criteria, and while the sensitivity of EUS for detecting lymph node metastases is in most situations superior to other investigational methods, the specificity and ability to visualize distant metastases is poor. For these purposes the optimal solution appears to be a combination of EUS with CT and/or ultrasound scanning. Future possibility of EUS-guided biopsy will strengthen the method's position in the diagnostic armoury concerning malignant disease in the upper gastrointestinal tract.

Digestive System Neoplasms

Autosomal recessive microcephaly, microcornea, congenital cataract, mental retardation, optic atrophy, and hypogenitalism. Micro syndrome.

Three affected children from an inbred family had microcornea, microcephaly, congenital cataract, severe mental retardation, retinal dystrophy, optic nerve atrophy, hypothalamic hypogenitalism, and agenesis of the corpus callosum. The disorder is presumably autosomal recessive; no identical syndrome has been described, but we consider syndromes with similar features.

Adolescent

[Ileus of the small intestine during the neonatal period. Treatment and prognosis].

During 13 years, 47 infants were treated for mechanical ileus resulting from intestinal obstruction located proximally to the coecum (duodenal obstruction, malrotation, jejuno-ileal obstruction and meconium ileus). The mortality and morbidity were low. A total of three infants died; all of them in the immediate postoperative period. One infant died on account of rupture of the anastomosis; in two infants, death was caused by pulmonary complications and disseminated intravasal coagulation. Seven reoperations were performed and among these five infants on account of adhesions-/fibrous band ileus. At follow-up examination five infants were slightly underweight and retarded in growth; only one child had troublesome gastrointestinal problems. We recommend the use of antenatal ultrasound in the 30th week of gestation more frequently and always when polyhydramnios is present. This should raise the suspicion of gastrointestinal obstruction. Postnatal screening for associated congenital anomalies should also be done, since these are the most frequent cause of death in this patient category.

Duodenal Obstruction