Molecular basis of Refsum disease: identification of new mutations in the phytanoyl-CoA hydroxylase cDNA

OBJECTIVE: This study aimed to review the management of cerebrospinal fluid (CSF) fistulae in the setting of developmental inner ear deformity. STUDY DESIGN: The study design was a case review, close examination of preoperative radiology, and corresponding intraoperative images. TECHNIQUE: A definitive method of CSF fistula closure is described using previously known techniques used commonly in skull base surgery. CONCLUSIONS: The use of a multiple-level, reinforced wound closure technique is necessary to definitively close CSF fistulae in extreme inner ear deformity and to prevent further episodes of CSF leak and meningitis.     

Neonatal infection by Streptococcus agalactiae. Multicenter study in the area of Barcelona, Spain

BACKGROUND: Streptococcus agalactiae or streptococcus group B (SGB) is the main etiologic agent of early neonatal sepsis. A multicenter study was performed with the aim of determining the incidence and characteristics of this disease in our medium and contribute the design of an adequate prevention protocol. METHODS: Ten hospitals and two primary health care centers were implicated in the study; 103 microbiology confirmed episodes of SGB neonatal sepsis (blood and/or LCR positive) were reported from 1994 to 1996. RESULTS: The incidence of early SGB neonatal sepsis was 1.48/1,000 live births with a mortality of 8.7%. The cultures, for detecting the state of the SGB carrier were performed in only 26 (25%) of the patients. At least one of the factors described for risk of perinatal SGB infection was observed in 46% of the mothers, with the most frequent being prolonged amniorrhea (26%), intrapartum fever (17%), and early delivery (14%). At the time of delivery only 10.7% of the mothers received endovenous antibiotherapy. CONCLUSIONS: From these results the following recommendations have been made: a) detection of SGB carriers by the systematic practice of blood cultures in the last weeks of gestation and b) the administration of intrapartum antibiotic prophylaxis in both early births (< 37 weeks) and in all the SGB carriers should be undertaken. With these measures we aim to decrease the neonatal infections by streptococcus group B.     

Percutaneous CT-guided catheter drainage of infected acute necrotizing pancreatitis: techniques and results

OBJECTIVE: The objective of this paper was to assess the safety and efficacy of percutaneous catheter drainage for initial treatment of infected acute necrotizing pancreatitis. MATERIALS AND METHODS: Thirty-four patients with acute necrotizing pancreatitis shown with contrast-enhanced CT were treated for sepsis with percutaneous catheter drainage. Extent of necrosis was less than 30% in 10 cases, 30-50% in 10 cases, and greater than 50% in 14 cases. Fourteen patients had central necrosis. Eighteen patients were critically ill with multiorgan failure. RESULTS: Sixteen (47%) of the 34 patients were cured with only percutaneous catheter drainage, including four (29%) of the 14 patients with central gland necrosis and 12 (60%) of the 20 with body-tail necrosis. Sepsis was controlled (defervescence of fever and return of WBC to normal) in an additional nine patients, allowing elective pancreatic surgery for control of pancreatic duct fistula. Eight patients failed to show clinical improvement after drainage and required necrosectomy. No patient experienced catheter-related complications. Mortality was 12% (all four deaths occurred after necrosectomy because of multiorgan failure). CONCLUSION: Percutaneous catheter drainage is a safe and effective technique for treating infected acute necrotizing pancreatitis. Overall, sepsis was controlled in 74% of patients, permitting elective surgery for treatment of pancreatic fistula, and 47% of patients were cured with no surgery required. No catheter-related complications occurred.     

Radical oncologic surgery affects the circulatory levels of interleukin 10

A 52-year-old woman presented with a clinical picture consistent with bacterial meningitis 3 years after functional endoscopic sinus surgery. Diagnosis of a cerebrospinal fluid (CSF) fistula was made clinically, and the site of the fistula was confirmed using magnetic resonance cisternography. The utilization of this technique in the diagnosis of CSF disorders is gaining popularity. Its usefulness in the context of other imaging modalities is discussed.     

Duodeno-colic fistula due to cancer of the right colon]

The author presents a case of duodeno-colic fistula revealing carcinoma of the right hepatic flexure showing the difficulty of surgery. A right hemi-colectomy enlarged to the duodenum and pancreas is the ideal operation from a curative point of view, but rarely possible owing to the age of the patient and the constitutional background. The risk of breakdown of duodenal sutures, which is sometimes technically difficult after colonic removal, is far from negligible. The results of by-pass operations, with or without exclusion of the fistula, are poor and uncertain.     

A phase 2 study of cisplatin in patients with hepatocellular carcinoma

Candida meningitis is a growing problem today. We report a 21-day-old male baby who was a victim of Candida albicans meningitis with the initial presentations of fever, mild stiff neck, poor feeding and activity. He had been treated with intravenous antibiotics and ventilator therapy prior to admission Initially, he was treated as a case of bacterial sepsis after admission with intravenous antibiotics. Due to positive cultures of cerebrospinal fluid for Candida albicans twice, intravenous amphotericin B was started from the 13th hospital day and was continued for 38 days. The successive three sets of CSF fungus culture yielded negative results and the patient was doing well without fever. Meanwhile, the brain sonogram revealed normal findings and he was discharged in a stable condition. We report this case and review some literature in an attempt to know more about this unusual disease, which is becoming more frequent as progress in intensive care grows.     

Group B streptococcal meningitis in adults: report of twelve cases and review

Group B streptococcus (GBS) is the leading etiologic agent of bacterial meningitis and sepsis during the neonatal period, but it is an infrequent cause of meningitis in adults. We report 12 episodes of group B streptococcal meningitis in adults and review 52 cases reported in the literature. A total of 24 men and 40 women were included in the study; the mean age (+/- SD) was 49.2 +/- 20.5 years (range, 17-89 years). All the patients had cerebrospinal fluid cultures positive for GBS. Eighty-six percent of the patients had comorbid conditions, 50% had a distant focus of infection, and blood cultures yielded GBS for 78.7%. The overall case-fatality rate was 34.4% (22 patients). Factors associated with a poor outcome were advanced mean age (+/- SD) (61.5 +/- 17.4 years vs. 42.8 +/- 19.2 years; P = .0003) and the presence of complications on admission (P = .0001). Seven percent of survivors had neurological sequelae. Group B streptococcal meningitis in adults has become increasingly frequent in recent years; it tends to occur in patients with severe underlying conditions and is associated with a high case-fatality rate. Factors associated with a poor prognosis are advanced age and the occurrence of neurological and extraneurological complications.     

Uretero-iliac fistula

OBJECTIVE: To describe a case of uretero-iliac fistula, an extremely rare condition that is not easily diagnosed. METHODS/RESULTS: Herein we describe a 76-year-old female who had undergone repeated pelvic surgery for adenocarcinoma of the sigmoid. She had a right ureteral fistula that had been managed conservatively by insertion of a ureteral catheter. Diagnosis was made by selective arteriography of the iliac arteries. The patient was submitted to surgery; primary closure of the arterial fistula and nephroureterectomy were performed. CONCLUSIONS: Uretero-iliac fistula should be suspected in patients with a history of repeated pelvic surgery and ureteral catheter placement that present with massive hematuria.     

Management of acquired rectourinary fistulas: outcome according to cause

PURPOSE: Acquired rectourinary fistulas, an infrequent complication of pelvic conditions, remain a therapeutic problem for which neither a widely accepted classification nor long-term outcome data are available. This study was designed to provide a new etiologic classification system and examine the success of various surgical therapies. It also looked at the need for permanent fecal or urinary diversion or radical excision depending on the cause of the fistula, i.e., benign vs. malignancy-related. METHODS: A retrospective analysis was made of 41 patients treated for acquired rectourinary fistulas between 1980 and 1995. Acquired rectourinary fistulas were classified as 1) benign but caused by Crohn's disease, trauma, perirectal sepsis, or iatrogenic injury; and 2) malignancy-related fistulas secondary to neoplasm, radiation, surgery, or combined tumor and treatment effects. Surgical interventions were classified as repair, excision, fecal diversion, and urinary diversion. RESULTS: Thirty-seven males and 4 females with acquired rectourinary fistula were identified with a mean age of 62 (range, 28-90) years. Nineteen patients had fistulas involving their urethras, and 22 patients had fistulas involving the bladder. Eight patients were not treated surgically; one was not treated because of an advanced malignancy, three because of patient preference, three because of sepsis, and one because of a poor general condition. Of the remaining 33 patients, nine had benign fistulas of which two were the result of Crohn's disease, two were the result of trauma, two were from an iatrogenic response, and three were from perirectal sepsis. Twenty-four patients had malignancy-related fistulas, and five patients had neoplasm at their fistula sites. The remaining 19 patients had malignancy-related fistulas that were the result of cancer treatments. Of the 19 malignancy-related fistulas, 5 were from radiation, 9 were from surgical trauma, and 5 were from radiation and surgical trauma. Forty-nine percent of the patients had undergone attempts at fistula treatment before referral. A resolution of symptoms after initial and reoperative surgery occurred more often in patients with benign fistulas (44 and 100 percent; mean, 1.8 surgeries per patient) compared with malignancy-related fistulas (21 and 88 percent; mean, 2.1 surgeries per patient). The rates of permanent fecal, urinary, and fecal plus urinary diversion were also lower for benign fistulas (11, 0, and 33 percent) compared with malignancy-related fistulas (13, 8, and 54 percent). Permanent diversion was avoided in 56 percent of the benign fistulas but in only 25 percent of the malignancy-related fistulas. The rates of excisional and radical (ileal conduit) surgery were lower for benign fistulas than for malignancy-related fistulas (44 and 11 percent vs. 50 and 54 percent). CONCLUSION: Successful management of rectourinary fistulas typically requires aggressive reoperative therapy with permanent diversion more often required for malignancy-related fistulas. Better outcomes can be anticipated for benign fistulas.     

The role of drainage and antibiotic prophylaxis in thyroid surgery

BACKGROUND: It is our habit to employ an open drainage after thyroid surgery in our department. We have also found a large number of surgical infections in these patients (5.8% vs 2.5). Aim of the study is to evaluate prospectively if contamination happens during surgical procedure or in a later time according to the presence of the open drainage. METHODS: From October 1995 to November 1996, 113 patients who underwent a subtotal thyroidectomy were randomized into two groups: group A with antibiotic prophylaxis (57 patients) and group B without it (56 patients). RESULTS: One case (1.7%) of sepsis among 57 patients of group A and 2 cases (3.4%) among 56 patients of group B were observed. CONCLUSIONS: No statistical difference was found between the two groups despite antibiotic prophylaxis covering surgical procedure. It is personal opinion that sepsis arose after surgical procedure, due to the presence of the open drainage.     

Bilateral vestibular loss as a post-infection complication of yersiniosis?

BACKGROUND: Yersinia infections other than plaque are caused by Yersinia pseudotuberculosis and Yersinia enterocolitica. Food and water contamination as well as animal-to-person and person-to-person contact are common pathways of transmission. Clinical manifestations include enteritis, enterocolitis, acute appendicitis, inflammation of the terminal ileum, and mesenteric adenitis. Y. enterocolitica may cause bacteremia with subsequent septicemia predominantly in patients with underlying illnesses such as diabetes mellitus or malignancy. More frequently enteritis is followed by immunological post-infectious syndromes such as arthritis and erythema nodosum. The present case report discusses bilateral vestibular loss possibly caused by an infection with Y. enterocolitica. PATIENTS: A 27-year-old caucasian woman initially presented with the otologic symptom of spinning vertigo accompanied by nausea and vomiting. RESULTS: Physical exam revealed spontaneous nystagmus to the left. Bithermal caloric responses were absent. Pure tone audiometry showed a bilateral symmetric high-frequency sensorineural hearing loss. Neurologic exams did not reveal involvement of the central vestibular system. Perilymphatic fistula on the left side was excluded by tympanoscopy. Serology for rheumatoid factors and HLA B27 was negative. Lead or mercury intoxication was also excluded. In her medical history the patient reported intermittent watery diarrhea and stress dependent arthralgia that had commenced during a stay in Argentina three years ago. Serology was positive, revealing elevated titers for Y. enterocolitica type 3 (1:200) and type 9 (1:400). DISCUSSION: Bilateral vestibular loss is rare. The main cause is aminoglycoside ototoxicity or meningitis. Yersina infections have not yet been described as inducing disease of the labyrinth. Present pathophysiologic knowledge of yersinia infections is described as follows: After peroral infection, gastrointestinal permeability is increased. Low-molecular-weight substances may enter the bloodstream and stimulate the formation of circulating immune complexes. These are held responsible for extraintestinal manifestations of yersinosis. Whether these circulating immune complexes and antibodies against Y. enterocolitica have an effect on the inner ear remains unclear. CONCLUSION: Because the coincidence of yersiniosis and a bilateral vestibular loss with no other identified cause, a postinfectious immune response is suggested as possible pathogenic mechanism.     

Colonization with group B streptococci in neonates and premature infants from selected neonatal departments

Group B streptococci are considered an important etiological agent of sepsis and meningitis in neonates and, particularly, in premature infants. There is a close correlation between colonization with these bacteria and the frequency of symptomatic infection. It is estimated that symptomatic infections occur in 1.0% of colonised neonates. The purpose of this work was to investigate the frequency of neonate colonization with group B streptococci for determination of the risk of symptomatic infection.     

Collagenous gastritis revealed by severe anemia in a child

Non-traumatic duodenocaval fistulae are rare, but may be the source of massive gastrointestional bleeding with associated fever and sepsis. These fistulae result from penetrating duodenal peptic ulcers or right nephrectomy and subsequent radiation to the upper abdomen. The outcome depends on early diagnosis and surgery before a potentially fatal hemorrhage occurs. The therapy of choice includes closure of the fistula and repair of the duodenum and inferior vena cava. We describe the seventh case with radiogenic duodenal ulcer. Gastrointestinal bleeding occurred 10 years after radical nephrectomy and radiation (60 Gy). The patient survived following partial pancreatoduodenectomy (Whipple).     

Longitudinal study of susceptibilities of species of the Bacteroides fragilis group to five antimicrobial agents in three medical centers

A total of 579 clinical isolates of the Bacteroides fragilis group collected from three Canadian hospitals were tested for susceptibility to five antimicrobial agents by using an agar dilution method. During the 4-year survey, isolates from intra-abdominal infections were collected from the following sites: abdominal abscesses (48%), peritoneal fluid (39%), blood (10%), and bile (3%). B. fragilis was the most prevalent species (35.4%), followed by B. thetaiotaomicron (19.2%), B. ovatus (15.9%), and B. vulgatus (11%). No metronidazole- or imipenem-resistant strains were found during the survey. Resistance profiles varied among the different species tested: 7.8, 2.9, and 7.3% of B. fragilis strains (n = 205) and 68.1, 17.2, and 9.4% of non-B. fragilis strains (n = 373) were resistant to cefotetan, cefoxitin, and clindamycin, respectively. B. fragilis and B. vulgatus demonstrated lower resistance rates than B. thetaiotaomicron, B. ovatus, B. distasonis, and B. caccae. During the study, rates of resistance to cefotetan and clindamycin fluctuated but rates of resistance to cefoxitin increased, particularly at one center. These data indicate a need to determine the susceptibility patterns of the B. fragilis group periodically at each hospital.     

Bacterial meningitis in the United States in 1995. Active Surveillance Team

BACKGROUND: Before the introduction of the conjugate vaccines, Haemophilus influenzae type b was the major cause of bacterial meningitis in the United States, and meningitis was primarily a disease of infants and young children. We describe the epidemiologic features of bacterial meningitis five years after the H. influenzae type b conjugate vaccines were licensed for routine immunization of infants. METHODS: Data were collected from active, population-based surveillance for culture-confirmed meningitis and other invasive bacterial disease during 1995 in laboratories serving all the acute care hospitals in 22 counties of four states (total population, more than 10 million). The rates were compared with those for 1986 obtained by similar surveillance. RESULTS: On the basis of 248 cases of bacterial meningitis in the surveillance areas, the rates of meningitis (per 100,000) for the major pathogens in 1995 were Streptococcus pneumoniae, 1.1; Neisseria meningitidis, 0.6; group B streptococcus, 0.3; Listeria monocytogenes, 0.2; and H. influenzae, 0.2. Group B streptococcus was the predominant pathogen among newborns, N. meningitidis among children 2 to 18 years old, and S. pneumoniae among adults. Pneumococcal meningitis had the highest case fatality rate (21 percent) and in 36 percent of cases was caused by organisms that were not susceptible to penicillin. From these data, we estimate that 5755 cases of bacterial meningitis were caused by these five pathogens in the United States in 1995, as compared with 12,920 cases in 1986, a reduction of 55 percent. The median age of persons with bacterial meningitis increased greatly, from 15 months in 1986 to 25 years in 1995, largely as a result of a 94 percent reduction in the number of cases of H. influenzae meningitis. CONCLUSIONS: Because of the vaccine-related decline in meningitis due to H. influenzae type b, bacterial meningitis in the United States is now a disease predominantly of adults rather than of infants and young children.     

Short-term maternal oxygen administration in fetuses with absence or reversal of end-diastolic velocity in umbilical artery: pathophysiological and clinical considerations

This case report describes our experience in treatment of a child with Crohn's disease involvement of ileocecal and sigmoid regions complicated with a severe abdominal wall abscess. A combined therapeutic approach of the acute episode was based on intestinal diversion, abscess drainage, antibiotherapy, nutritional support and post-operative immunosuppression with azathioprine. The definitive treatment of the ileocolonic involvement occurred 6 months after the acute episode and consisted of limited resection, multiple strictureplasties, primar" closure of ileosigmod fistula and undiversion. This operative procedure was followed by a short period of total parenteral nutrition and progressive re-establishment ofenteric nutritional support and immunosuppression. At 18 months follow-up, under low dose azathioprine revealed, a normal growth with a 20 Kg weight gain and 10 cm height gain, no evidence of fistula recurrence or restenosis and suggests the safety of strictureplasty in paediatric patients as a way to conserve as much small bowel as possible.     

In vivo efficacies of quinolones and clindamycin for treatment of infections with Bacteroides fragilis and/or Escherichia coli in mice: correlation with in vitro susceptibilities

Therapy with ofloxacin, ciprofloxacin, and lomefloxacin (alone or in combination with clindamycin) and therapy with sparfloxacin, clinafloxacin, and temafloxacin alone were given to mice with subcutaneous abscesses. The abscesses were caused by two Bacteroides fragilis isolates, one of which was susceptible and one of which was resistant to ofloxacin, ciprofloxacin, and lomefloxacin, alone or in combination with Escherichia coli. The abscesses were examined 5 days after inoculation. Numbers of B. fragilis organisms reached log10 10.2 to 11.8 per abscess, and numbers of E. coli organisms reached log10 10.6 to 11.8 per abscess. All of the quinolones reduced the number of susceptible B. fragilis isolates (log10 3.6 to 6.9) and E. coli isolates (log10 5.7 to 6.8). However, ciprofloxacin and lomefloxacin failed to reduce the number of resistant B. fragilis organisms in single-organism or mixed infections. The addition of clindamycin to either ofloxacin, ciprofloxacin, or lomefloxacin reduced the numbers of both susceptible and resistant B. fragilis organisms (log10 3.8 to 7.8). In contrast, sparfloxacin, clinafloxacin, and temafloxacin were effective as single therapy in eradicating B. fragilis resistant to ofloxacin, ciprofloxacin, and lomefloxacin. These in vivo data confirm the in vitro activity of these quinolones and suggest that although ofloxacin, ciprofloxacin, and lomefloxacin are occasionally effective as single agents in eradicating mixed infection by susceptible strains of B. fragilis and E. coli, addition of an agent with activity against anaerobic organisms will ensure their efficacy. Quinolones with good efficacy against B. fragilis may be effective as single-agent therapy of mixed infections.     

Magnetic resonance imaging of fistula-in-ano: STIR or SPIR?

Patients with clinically suspected anorectal sepsis were studied using MRI in order to determine if T2 weighted sequences with fat suppression conveyed any additional benefit over conventional short tau inversion recovery (STIR) sequences. 23 consecutive patients (16 male) undergoing MRI for suspected perianal sepsis were studied prospectively using a 1.0 T whole body system and body coil. Axial and coronal T1 weighted turbo spin echo sequences were obtained, followed by STIR and T2 weighted spectral fat saturation inversion recovery (SPIR) sequences. Images were assessed for the presence of sepsis or fistula, and information provided by the sequences compared. Active disease was diagnosed in 17 patients, 14 of whom had fistula-in-ano; one intersphincteric, 10 transsphincteric and three extrasphincteric. Internal openings were identified in all of these 14 patients; anal in 10, rectal in two, and both in two. Diagnosis and fistula classification was possible in all of these 14 subjects on the basis of STIR sequences alone. The anal sphincters and pelvic floor musculature were better resolved by STIR than SPIR, leading to easier and more confident determination of fistula anatomy in eight of the 14 (57%). In no case did STIR sequences fail to resolve inflammation seen subsequently on SPIR, despite reduced track intensity. T1 weighted sequences were generally non-contributory. Both STIR and SPIR sequences are adequate to classify fistula-in-ano, but classification was easier with STIR due to superior resolution of pelvic floor structures.     

Topical preparations for burns and their evaluation

Omission of a temporary ileostomy in patients undergoing restorative proctocolectomy is controversial. Although fewer operations may be required and some complications avoided, the risks of anastomotic dehiscence and pelvic sepsis may be greater. Patients undergoing restorative proctocolectomy with no ileostomy (Group NI, n = 72) were compared retrospectively with patients given a conventional loop ileostomy (Group I, n = 30). Criteria for avoiding faecal diversion included: absence of severe acute colitis, good nutritional status and favourable surgery with creation of a sound, tension-free anastomosis. Steroid intake was not a contraindication to single-stage surgery. Delayed stomas were necessary in 8% of Group NI. For Groups NI and I, the rates of anastomotic leak (3% vs 3%), pelvic sepsis without demonstrable leak (3% vs 0%), pouch fistula (3% vs 10%) and intestinal obstruction (8% vs 3%) were similar. Closure of the temporary ileostomy in Group I was associated with a 10% complication rate. Cumulative post-operative hospital stay was significantly less in Group NI (median 11 vs 16 days). Functional results at 1 year were similar. A temporary loop ileostomy can be safely avoided in carefully selected patients undergoing restorative proctocolectomy.     

Pitfalls of hyperfractionation: theoretical considerations of effect of repair time on late radiation damage]

We conducted a 3-year Taiwan-wide hospital-based survey of invasive Haemophilus influenzae infections in children less than 15 years of age. From January 1992 to December 1994, 105 cases (57 boys, 48 girls) were reported. Seventy-three patients (69.5%) had meningitis and 32 patients had other diseases (12 pneumonia, 10 sepsis, 7 cellulitis, 3 arthritis). Fourteen patients (13%) died, all of whom had meningitis or sepsis. Among the 63 patients who survived meningitis, 17 (27%) had neurologic sequelae and eight (47%) had hearing impairment. The number of cases of H. influenzae meningitis (30%) and other H. influenzae diseases (29%) peaked in children between 6 and 12 months of age. Patients with invasive infections (82%) and meningitis (73%) were younger than 24 months of age. Only 12 patients (11%) were older than 5 years of age and four had underlying diseases. The annual incidence of invasive H. influenzae infections in children less than 5 years old was 1.9 per 100,000 per year. During the same period a survey of purulent meningitis in children younger than 15 years of age was also conducted in 20 hospitals. A total of 198 patients, in whom the causative organisms were identified, were included; 94 patients were 2 months of age or under and the most frequent pathogen was group B streptococci (35 cases, 37%). Among the 104 patients who were older than 2 months of age, H. influenzae was the leading cause (38 cases, 37%). In conclusion, invasive H. influenzae type b (Hib) diseases exist in Taiwan but have an incidence lower than in Western countries. Hib meningitis is still the most common cause of purulent meningitis in children in Taiwan and is an important cause of mortality and morbidity. Continuous active surveillance of invasive H. influenzae infections is suggested to determine the best time to introduce an Hib conjugate vaccine in Taiwan.