Neuropsychological and linguistic follow-up studies of children with galactosaemia from an unscreened population

Many galactosaemics appear to have neuropsychological and/or linguistic problems in spite of dietary treatment. Because the neonatal screening program in Norway does not include galactosaemia, we have re-examined Norwegian galactosaemics. Of 16 known patients, 8 patients participated in the study. They had been diagnosed between 2 and 11 weeks of age, and were between 9 months and 19 years old at the time of this study. All had very low or 0 activity of galactose-1-phosphate uridyl transferase. As part of the study all were examined neurologically, and had an age-appropriate developmental/IQ test, an ABR and an EEG, and a comprehensive psycholinguistic evaluation. The three youngest patients had normal developmental/IQ tests, while the five older patients had IQ scores in or below low range of normal. The majority had delayed language development and three patients were classified as having verbal dyspraxia. ABR and EEG showed mild pathology in the oldest patient only. Galactosaemia appears to be associated with significant risks of developmental and language delays in this unscreened population.     

Indications for laparoscopic colorectal surgery

Background: Between November 1991 and May 1995, a series of laparoscopic colectomies were performed in our hospital. Methods: Our main aim was to define more specifically the indications for laparoscopic colectomy. Results: A total of 69 patients underwent laparoscopic surgery for benign polypoid colorectal disease (n = 10), inflammatory bowel disease (n = 24), and colorectal malignancy (n = 35). Of the latter group, four patients underwent a palliative procedure. The conversion rate of the whole group was 29%. The main reason to convert was infiltrative growth in inflammatory disease or cancer. Respectively, seven (10%) and 12 (17%) patients sustained complications in the perioperative and early postoperative phase. Two patients died perioperatively (3%). The mean hospital stay was 12 days. On follow-up, 11 patients had developed a stenotic anastomosis, which was successfully dilated in all cases. After 3 years, the survival rate according to Kaplan-Meier is 86%, 66%, 68%, and 0% for Dukes' A, B, C, and D color carcinoma, respectively. In one patient with a Dukes B carcinoma, port site metastases were found. Conclusions: Justifiable indications for laparoscopic colorectal surgery include (a) a benign polyp 20–50 cm from the anal ring; (b) mobile, inflammatory large bowel disease; (c) palliation in case of malignant disease, preferably of the left hemicolon. It remains to be proven that laparoscopic colectomy is superior and not just equivalent to open colectomy. This is especially true for resections of colorectal carcinoma with curative intent. Therefore a cost/benefit analysis should be performed in a prospective, randomized setting. Received: 1 November 1996/Accepted: 1 July 1997     

Neonatal brain: color Doppler imaging. Part I. Technique and vascular anatomy

Color Doppler imaging (CDI) can demonstrate the relative direction and velocity of blood flow in color, superimposed on a conventional gray-scale ultrasound image that depicts stationary tissue. Twenty-five infants were studied with portable CDI in the coronal, sagittal, and axial planes. Bilateral antegrade flow was noted in the anterior, middle, and posterior cerebral arteries in all patients. Multiplanar CDI can image flow in the circle of Willis and its tributaries and branches.     

Biplane arteriography in ischemia of the lower extremity

A technique for simultaneous bilateral biplane arteriography of the abdomen, pelvis, and lower extremities is described. The biplane views provided clinically significant information in approximately 40% of cases.     

The effect of enterectomy on gastric secretion in dogs with biliary fistulas

Bile was excluded from the gastrointestinal tract of 5 dogs with Heidenhain pouches by total external biliary fistulas. After a major portion of the jejunum and the ileum was resected Heidenhain pouch secretions increased in all instances. The results indicate that the increase in gastric secretion which regularly follows small-bowel resection is independent of the presence or absence of bile in the gastrointestinal tract.Supported by Grant AM-7750 from the US Public Health Service.The opinions expressed in this publication are those of the authors and not necessarily those of the US Air Force Medical Service.     

Burns caused by monitoring equipment during anaesthesia.

Hazards of monitoring during anaesthesia are burns, cardiac arrest or ventricular fibrillation, electric shock and false alarms caused by interference with the traces. The causative and contributory factors and the measures for the prevention of burns are discussed.     

Abrogation of the beneficial blood transfusion effect in dogs by splenectomy

The role of the spleen in the manifestation of the transfusion phenomenon was studied in beagle dogs. Beagles were transfused 3 times with 100 ml blood from different mismatched mongrel dogs, at 4, 3, and 2 weeks before mongrel kidney transplantation or splenectomy. Seven transfused dogs were splenectomized 1 week before transplantation, 9 transfused animals were not splenectomized, and a group of 9 kidney allografted controls was neither transfused nor splenectomized. All recipients were given postoperative immunosuppression, which consisted of azathioprine and prednisolone. As with earlier demonstrations it was found that 3 pretransplant blood transfusions in the nonsplenectomized group led to a significant prolongation of kidney graft survival. All transfused animals survived for more than 3 weeks, a period in which more than 50% of the kidneys in the control group were rejected. Four out of 9 transfused dogs became long-term survivors. In the transfused-splenectomized group, 70% of the recipients rejected their kidney acutely within 3 weeks, and only one animal became a long-term survivor. The absence of a transfusion effect in most of the splenectomized dogs suggests that, under the experimental conditions employed, the spleen plays a crucial role for the induction and manifestation of the transfusion phenomenon.

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Resumen El efecto benéfico de las transfusiones de sangre sobre la supervivencia de órganos transplantados ha sido confirmado en numerosos estudios en animales y en el hombre. Poco se conoce sobre el mecanismo involucrado en el fenómeno transfusional.El papel del bazo en la manifestación del fenómeno transicional fue estudiado en perros. Los perros fueron transfundidos tres veces con 100 ml de sangre canina 4, 3 y 2 semanas antes del transplante de riñón o la esplenectomía. Siete perros transfundidos fueron esplenectomizados una semana antes del transplante, nueve animales transfundidos no fueron esplenectomizados y un grupo control de nueve alotransplantes no fue transfundido ni esplenectomizado. Todos los recipientes recibieron inmunosupresión con azatioprina y prednisolona.Al igual que lo demostrado previamente, se encontró que la administración de 3 transfusiones pretransplante en el grupo no esplenectomizado resultó en una prolongación significativa de la supervivencia del transplante renal. Todos los animales transfundidos sobrevivieron por más de 3 semanas, periodo durante el cual más del 50% de los riñones en el grupo de control fue rechazado. Cuatro de 9 perros transfundidos se convirtieron en sobrevivientes a largo plazo. En el grupo transfundidoesplenectomizado, el 70% de los recipientes rechazaron el riñón en forma aguda en las primeras 3 semanas, y sólo un animal se convirtió en su perviviente a largo plazo.La ausencia del efecto transfusional en la mayoría de los animales esplenectomizados sugiere que, bajo las condiciones experimentales utilizadas, el bazo juega un papel crucial en la inducción y manifestación del fenómeno transfusional.

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Résumé Les effets bénéfiques des transfusions de sang sur la survie des organes greffés ont été établis par de nombreuses études chez l'animal et chez l'homme. La suppression de ces effets par la splénectomie fait l'objet de cette étude expérimentale menée chez les chiens de race beagle. Ces chiens reçurent 3 fois une transfusion de 100 ml de sang provenant de chiens bâtards, 4, 3, et 2 semaines avant la transplantation d'un rein de chien bâtard ou avant une splénectomie. Sept des chiens transfusés furent splénectomisés une semaine avant la transplantation, neuf ne furent pas splénectomisés et un groupe de contrôle de neuf chiens qui avaient reçu une allogreffe ne furent ni transfusés ni splénectomisés. Tous les chiens greffés reçurent un traitement immuno-suppresseur postopératoire par azathioprine et prednisolone.Comme cela a déjà été démontré, l'étude entreprise a permis de constater que les 3 transfusions préopératoires chez les chiens qui ne furent pas splénectomisés se sont soldées par un prolongement significatif de la durée de survie du rein greffé; en effet les chiens transfusés ont survécu plus de 3 semaines, période au cours de laquelle 50% des reins greffés furent rejetés dans le groupe de contrôle; 4 des 9 chiens qui avaient été transfusés avant l'intervention sont d'ailleurs entrés dans le groupe des survivants à long terme.En ce qui concerne le groupe des chiens transfusés puis splénectomisés, 70% des beagles greffés présentèrent une réaction de rejet au cours des 3 semaines suivant la greffe et un seul animal a survécu à long terme.L'absence des effets bénéfiques de la transfusion chez la majorité des chiens splénectomisés conduit à penser que, dans les conditions expérimentales suivies, la rate joue un rôle crucial dans l'induction et l'expression de ces effets.