Anatomical basis of sleep-related breathing abnormalities in children with nasal obstruction

OBJECTIVE: To define, in a group of children with nasal obstruction, the anatomical differences that differentiate those with quiet, unobstructed nocturnal respiration from those with obstructive sleep-related breathing abnormalities (snoring and obstructive sleep apnea). DESIGN: Case series. PATIENTS: Fifty-nine children aged 3 to 13 years (35 boys and 24 girls) with nasal obstruction and without tonsillar hypertrophy, known craniofacial syndromes, or neuromuscular diseases were included in the study. MAIN OUTCOME MEASURES: Each patient was categorized as to severity of nocturnal obstructive breathing symptoms. Angular and linear cephalometric measurements were used for assessment of craniofacial features. Clinical symptom scores were correlated with the cephalometric measurements. RESULTS: Significant craniofacial abnormalities were identified in patients prone to obstructive breathing patterns: increased flexure of the cranial base and bony nasopharynx, opening of the gonial angle, shortened mandibular length, dorsocaudal location of the hyoid, reduced posterior airway space, and increased velar thickness. CONCLUSIONS: A number of anatomical abnormalities may contribute to sleep-related abnormal breathing in otherwise normal children with nasal obstruction. Our results suggest that symptomatic children show some of the same skeletal and soft-tissue configurations that are found in adults with obstructive sleep apnea. While adenoidectomy is generally an effective treatment in children with obstructive sleep-related breathing abnormalities, the underlying craniofacial variances that remain after adenoidectomy may predispose these patients to redevelopment of obstructive breathing abnormalities in adulthood.     

Postnatal changes in plasma ceruloplasmin and transferrin antioxidant activities in preterm babies

Postnatal changes in plasma ceruloplasmin ferroxidase and transferrin iron-binding antioxidant activity were studied in 10 healthy preterm babies during the first 6 weeks of life. Ceruloplasmin levels and ceruloplasmin ferroxidase activity were low at birth, remained stable for the first 3 weeks, and increased between 3 and 6 weeks. The transferrin levels were also low at birth, and this finding persisted throughout the 6-week study period. However, although the plasma iron-binding antioxidant activity was correspondingly low at birth, it thereafter rose and remained high. In four cord blood samples, but not in subsequent postnatal samples, peroxidation was actually stimulated in the assay measuring plasma iron-binding antioxidant activity. We have previously shown that this phenomenon is probably due to the presence of non-protein-bound iron.     

Interferon α in the treatment of polycythemia vera

Interferon alpha (IFN) inhibits the growth of the abnormal clone in patients with myeloproliferative disorders, leading to a reduction of the clinical and laboratory signs of the pathologic myeloproliferation. The therapeutic efficacy of IFN in polycythemia vera (PV) is demonstrated by the summarized treatment results of 279 patients participating in 16 prospective nonrandomized studies and in three case reports. The initial IFN dose ranged from 3 to 35 million IU/week. In 82% of the patients the frequency of phlebotomies was reduced. In 50% a complete remission was achieved, defined as a stable hematocrit of 45% without concomitant phlebotomies. Reduction of splenomegaly was seen in 77% and control of pruritus in 81% of the patients. The median observation time of the studies was 13 months (ranging from 3 to 84 months). Individual cases were followed for up to 126 months. In 21% of the patients IFN was terminated, owing mostly to side effects. The selective suppression of the malignant clone by IFN was demonstrated by the induction of cytogenetic remissions in sporadic cases with a chromosomal marker and by the observation of unmaintained remissions that lasted up to 4.8 years. IFN has no known mutagenic or teratogenic effects. The data presently available demonstrate that IFN is an effective alternative to the present forms of treatment in PV. Controlled prospective studies are essential to clarify whether the favorable biologic properties are also reflected by a benefit in clinical course and survival, and whether IFN may reduce the rates of acute leukemia and myelofibrosis. A randomized study that compares IFN and hydroxyurea in patients with PV has recently been initiated by the Suddeutsche Hamoblastosegruppe (SHG) in Germany.     

Standards, Options, and Recommendations for brachytherapy in patients with prostate cancer: efficacy and toxicity]

CONTEXT: The "Standards, Options and Recommendations" (SOR) collaborative project was initiated in 1993 by the Federation of the French Cancer Centres (FNCLCC), with the 20 French Regional Cancer Centres, several French public university and general hospitals, as well as private clinics and medical specialty societies. Its main objective is the development of serviceable clinical practice guidelines in order to improve the quality of health care and the outcome of cancer patients. The methodology is based on a literature review, followed by a critical appraisal by a multidisciplinary group of experts. Draft guidelines are produced, then validated by specialists in cancer care delivery. OBJECTIVES: Produce clinical practice guidelines for the brachytherapy of prostate cancer using the methodology developed by the Standards, Options and Recommendations project. METHODS: The FNCLCC and the French Urology Association (AFU) first designated the multidisciplinary group of experts. Available data were collected by a search of Medline and lists selected by experts in the group. A first draft of the guidelines was written, they validated by independent reviewers. RESULTS: The main recommendations are: 1/Brachytherapy with permanent seeds alone is a possible curative treatment for prostate cancer patients with the following prognosis factors: tumour stage T1 or T2a (TNM 1992), Gleason score < or = 6 and PSA < 10 micrograms/L. 2/Combined treatment with brachytherapy and hormonal therapy could be more efficient than brachytherapy alone for prostate cancer patients with Gleason score > 7 and/or PSA > 10.3/Combination of brachytherapy and external beam radiation therapy can be proposed to prostate cancer patients with intermediate prognosis. 4/Before and after seed implantation, risks of infection must be prevented by appropriate antibiotic therapy (recommendation). 5/Brachytherapy must not be performed within 2 months of transurethral prostate resection. 6/The height of the urethra receiving more than 200% of the prescribed dose must be reported. The portion of the rectum receiving 100 and 120% of the prescribed dose must be limited to 10 and 5 mm length, respectively.     

Photosensitivity in HIV-infected individuals

OBJECTIVE: To characterize photosensitivity in HIV-infected individuals using minimal erythema dosage (MED) UVA (ultraviolet A light) and UVB (ultraviolet B light) photoprovocation light testing. DESIGN: Prospective, controlled analytical study. SETTING: University of California, San Francisco, between March 1995 and January 1997. PATIENTS: 13 HIV-seropositive patients with clinical and pathological features consistent with photodermatitis, 13 HIV-seropositive patients with biopsy-proven eosinophilic foliculitis (EF), and 10 HIV-seropositive patients with CD4 (T helper cell) count below 200 cells/uL and no history of photosensitivity or EF. INTERVENTION: Each patient underwent MED testing for UVB. All 13 patients with suspected photodermatitis underwent full photochallenge testing with UVA and UVB for up to 10 consecutive week days. RESULTS: Mean MED to UVB in patients with clinical photosensitivity and EF was lower (p = 0.004 and p = 0.022 respectively) than that of patients without a clinical history of photodermatitis. There were no significant differences in mean CD4 count or Fitzpatrick skin type. Positive photochallenge tests (papular changes at site of provocative light testing) to UVB (9 of 13 patients) were much more common than reactions to UVA (3 of 13 patients) in the photodermatitis group. All patients with clinically active photodermatitis developed papular changes at the site of UVB photochallenge testing, but only 1 of 5 patients with photodermatitis in remission developed papular changes with UVB photochallenge testing. Seven of the 13 patients with photodermatitis had Native American ancestry. Photosensitive patients were commonly taking trimethoprim-sulfamethoxazole (TMP-SMX), but no more commonly than EF or control patients. CONCLUSIONS: Photosensitivity in HIV-infected individuals appears to be a manifestation of advanced disease. Most patients are sensitive to UVB. The most severely affected individuals are both UVB and UVA sensitive, and may show reactions to visible light. A significant Native American ancestry may be a risk factor for development of photodermatitis in patients with advanced HIV disease. Finally, patients with eosinophilic folliculitis may be subclinically photosensitive.     

Diagnostics and surgical treatment strategy for rectal cavernous hemangiomas based on three case examples

A 20-year-old man with a congenital vascular malformation extending from the anal canal into the distal sigmoid had had recurrent perianal blood loss as a neonate. A hemangioma was diagnosed for the first time in 1978. The patient received regular and frequent gastroenterological treatment until admission. Decisive for the indication for surgery was the patient’s need for blood infusions and shorter bleeding intervals in June 1998. Surgical therapy consisted of deep anterior rectosigmoid resection with coloanal pouch anastomosis. In a second case of a 27-year-old woman a sigmoid hemangioma was diagnosed in conjunction with emergency sigmoid resectioning. Because of recurrent hemorrhages a coloanal pouch was also established here in a second step. The third case involved a 19-year-old woman with a 12-year history of repeated perianal hemorrhages. After sigmoid discontinuity resection we carried out proctectomy with descendostoma creation due to renewed severe intractable perianal bleeding. The histological examination revealed a rectal hemangioma that had caused the repeated perianal hemorrhages. Surgical reconstruction was then achieved by coloanal pouch anastomosis. In view of the good functional and perioperative results, current surgical therapy should aim at preserving continuity and continence by coloanal pouch anastomosis. Accepted: 12 November 1999