Spontaneous regression of a dura mater arteriovenous fistula of the posterior fossa

A case of spontaneous regression of a posterior fossa dural arteriovenous malformation supplied by the left external carotid artery is reported. The patient, a 68-year-old woman, complained of pulsatile tinnitus most important at night. Two years after the malformation discovery, she presented a transient ischemic attack in the left middle cerebral artery territory. A new angiography showed that the malformation had disappeared; the left transverse sinus could not be opacified. The authors emphasise the interest of Doppler sonography for detection and follow-up of such malformations. They review the few published similar cases and discuss the different possible closure mechanisms of intracranial arteriovenous malformations.     

Orthotopic liver transplantation in a patient with carbamyl phosphate synthetase deficiency and cystic fibrosis

A 15-year-old female with carbamyl phosphate synthetase deficiency, cystic fibrosis, and cystic fibrosis-related diabetes underwent orthotopic cadaveric liver transplantation. Metabolic control was maintained during the procedure with nutritional support and the use of intravenous sodium phenylacetate and benzoate. Her postoperative course was complicated by seizures and a transient decline in her pulmonary function tests, which returned to preoperative levels within one year of the transplant. Now, four years post-transplant, her quality of life has dramatically improved. There are only four Canadian centres with paediatric liver transplantation programs. However, expert medical care for adults with inborn error of metabolism is even more limited, suggesting that access to adult medical care is one of the many factors to be considered when liver transplantation is contemplated for patients with metabolically unstable conditions.     

The treatment of incisional and abdominal hernia with a prosthesis in potentially infected tissues — A series of 47 cases

Summary The aim of this study was to assess the infective risks involved in the placement of a non-absorbable prosthesis in the abdominal wall, in the treatment of abdominal hernias. Two groups of patients were compared over the period 1985 to 1995. Group A (N=47) received non-absorbable (Mersilene) prostheses, placed in a potentially infected area (Altemeier class 2, 3 and 4). Group B (N=47, Altemeier class 1) comprised a similar group of patients to those in group A. The prostheses were usually placed in the retro-muscular prefascial plane. Antibiotics were given either prophylactically or therapeutically, according to the degree of sepsis surrounding the operation. The length of hospital stay was longer in group A (15.6 ± 9 as against 10 ± 6 days. p=0,0006). There were two deaths in group A and none in group B (not significant). The general complication rate was 23.7% in group A as against 8.5% in group B (p=0.016). There was no significant difference between the two groups in regard to surgical complications (group A 23.7%, group B 21.3%). The infection rate in the abdominal wall was 10.6% and 6.4% in groups A and B respectively (not significant). There was only one patient with a prosthetic infection (in group A) requiring revision. We conclude that the use of non-absorbable prostheses placed in the retromuscular prefascial space may be more widely employed, in the course of potentially contaminated intra-abdominal surgery of Altemeier class 2.     

Urdamycins, new angucycline antibiotics from Streptomyces fradiae. VI. Structure elucidation and biosynthetic investigations on urdamycin H

A new angucycline antibiotic has been discovered as a small side product of Streptomyces fradiae (strain Tü 2717), the producer of the urdamycin complex, during screening for biosynthetic relatives of urdamycins C and D. The structure was elucidated after isolation, via strain selection, of a mutant of S. fradiae that produces this new congener in larger amounts. The structure includes a new chromophore containing aglycone that has not been found before among the angucyclines nor as a natural product generally. In urdamycin H (1) the angucycline four-ring system is enlarged by a (p-OH-phenyl)furan moiety and is closely related to urdamycin C (2). The structure was elucidated by comparison of the physico-chemical data with those of known urdamycins, especially with those of urdamycin C (2), and was confirmed by intensive 2D NMR analysis. Biosynthetic studies showed that tyrosine and not the smaller p-OH-phenylglycine is the precursor of the (p-OH-phenyl)furan moiety.     

Sperm morphology assessment using strict criteria and male fertility under in-vivo conditions of conception

The clinical significance of sperm morphology assessment accordingto very strict criteria was determined using semen samples ofrandomly chosen males from couples not submitted to assistedprocreation techniques, with a median duration of infertilityof 4 years (range 1–17; n = 89). The relationships ofsperm morphological properties to the results of standard spermanalysis, including the differentiation of round cells in semenby monoclonal antibodies and semen cultures, the testing ofsperm functional capacity in vitro with the standardized sperm-cervicalmucus penetration test (SCMPT) and the subsequent pregnancyrate under in-vivo conditions of conception, were evaluatedin a prospective study. The quick staining method (DiffQuickstain) for sperm morphology proved to be practical and suitablefor routine use* The percentage of normal forms according tostrict criteria ranged from 1 to 36%, with a median of 12%.Morphological findings were not markedly related to the medicalhistory, but significant relationships between standard parametersof sperm analysis, in particular the sperm count, the progressivemotlity and standard sperm morphology, were found. The percentageof normal forms was not significantly associated with the microbialcolonization of semen samples but was negatively related tohigh leukocyte rates. Semen samples with a higher percentageof normal spermatozoa (shown, for example, for >4, >7or >14% normal) were significantly more frequent in casesof an adequate SCMPT. The subsequent pregnancy rate within anobservation period of 12 months was 20.2%. The pregnancy rateunder in-vivo conditions was significantly higher when semensamples had a better sperm morphology, with significant differencesfor thresholds at 4, 7 and 14% of strictly normal forms. Althoughsperm morphology is only one among a multiplicity of factorsdetermining fertility, the results suggest that the evaluationof sperm morphology using strict criteria provides valuableinformation during basic infertility investigations.     

Necessity of complete intake of phenylalanine-free amino acid mixture for metabolic control of phenylketonuria

The importance of complete or almost complete intake of the recommended amount of phenylalanine-free amino acid mixture (AAM) for control of blood phenylalanine level in patients being treated for phenylketonuria (PKU) has not been universally appreciated. We observed the effect of complete intake of AAM on plasma phenylalanine levels during hospitalization in 6 patients with PKU (5 pregnant women with PKU and 1 child) who had poor metabolic control because of less than full compliance with prescribed AAM intake. Before hospitalization, all but 1 of the patients had blood phenylalanine levels above 1,000 μmol/L; in 1 patient the blood phenylalanine level was 703 μmol/L. During 9 periods of observation in the 6 patients, the levels of plasma phenylalanine decreased to the recommended range of below 360 μmol/L within 2 to 6 days of hospitalization. These experiences indicate a close relationship between compliance with prescribed AAM intake and control of blood phenylalanine level. We propose that hospitalization be considered when patients with PKU who are consuming a phenylalanine-restricted diet fail to maintain blood phenylalanine levels in the targeted range despite reported compliance with the prescribed intake of dietary phenylalanine and AAM. J Am Diet Assoc. 1999; 99: 1559–1563.