Anaesthesia in a parturient with Noonan''s syndrome

During a randomized double-blind study to assess the antiviral activity of saquinavir (SQV) alone or in combination with zidovudine (ZDV), the emergence of phenotypic resistance was evaluated in 44 patients treated with SQV (13 subjects), ZDV (14 subjects), and SQV plus ZDV (17 subjects). A significant (P< 0.05) lower CD4+ cell count and higher HIV RNA copy number at entry were found in six patients who developed resistant viral strain (3 to ZDV and 3 to SQV) during the first 4 months of treatment. After 1 year, drug-resistant strains (12 to ZDV and 14 to SQV) were isolated in 26 out of 37 patients. A significant higher number of patients treated with ZDV alone (10/13) harbored ZDV-resistant strains compared to patients treated by combination therapy (2/13); whereas more than 50% of patients had SQV-resistant strains aside from treatment. Early SQV-resistant strains were isolated in a limited number of patients treated with SQV alone (3/13). The rates of emergence of resistant strains during ZDV or SQV monotherapies are comparable. Combination therapy may delay the emergence of phenotypic resistance to either drugs in the short term and to ZDV, but not to SQV, at least after 1 year.     

Lupus interstitial cystitis. Apropos of a case]

The poor results obtained with the use of prosthesis in infrapopliteal arterial bypass grafting for critical limb ischaemia led to the introduction of several types of adjuvant arteriovenous fistula to improve the patency and limb salvage rates in patients who have no suitable autologous vein. The main aim of adjunctive arteriovenous fistula in infrageniculate prosthetic bypass is to accelerate the blood flow velocity through the prosthetic graft above the thrombotic threshold level. Since they are subject of great debate among vascular surgeons, the Authors have briefly reviewed the haemodynamic aspects and results reported with the use of such procedures.     

Hair dysplasia in oculo-dento-digital syndrome. Apropos of a mother-daughter case

Oculo-dento-digital syndrome (SODD) as defined by Meyer-Schwickerath in 1957 is a rare entity (84 cases) which belongs to ectodermal dysplasias. It consists of: the characteristic features (long face, pinched nose); syndactyly; ocular, dental and bone abnormalities. This entity is usually transmitted on the autosomal dominant mode. We report two cases (a mother and daughter) with polymalformations which we classed as SODD. Furthermore, agenesis of lacrimal duct and genitourinary abnormalities were noted. The mother had a very particular complex hair shaft dysplasia (incomplete pili torti, "tiger tail" aspect, fractures) with alopecia since she was fifteen years old. The daughter's hair was normal at birth. In SODD, fine and sparse hair is often observed (44 p. 100). Only one patient had hair shaft investigation under polarized light: pili annulati and monilethrix were described but not found in our cases. These two reports incite to the systematic hair shaft study in SODD as other ectodermal dysplasia syndromes.     

Pulmonary plasmacytoma. Apropos of a case. Review of the literature]

Thirty-six infants born at term who developed hyperbilirubinemia were assessed for developmental status at 2, 4, 6, 9 and 12 months of age according to a mental and psychomotor scale for 0-4 year old. The development quotient (DQ) of infants with hyperbilirubinemia were lower than controls, and the reduction was significant at 2 months of age (P = 0.03). When single component of DQ was compared, it was shown that the DQ score for social behavior was significantly lower in patients at 2 months of age than in control (P = 0.007), and except for motor, other four single DQ scores were lower in patients than in control and the reduction was significant in the DQ scores for adaptability and social behavior (P = 0.05, 0.032, respectively). Furthermore, no significant correlation was found between DQ and serum peak bilirubin value or duration of hyperbilirubinemia. The results indicate that hyperbilirubinemia may have a long term impact on mental development of infants. It is necessary to monitor all infants with hyperbilirubinemia, including full term infants with no severe complication.     

Penetrating atheromatous ulcer of the thoracic aorta. Apropos of a case]

The authors report a case of penetrating atheromatous ulceration of the descending thoracic aorta complicated by a haemothorax. Atheromatous ulceration of the aorta is usually observed in elderly hypertensives. It is caused by rupture of the internal elastic layer under an atheromatous plaque extending into the media and resulting in a parietal haematoma of the aorta. Clinically, the differential diagnosis is that of a dissection of the aorta or of fissuration of an atheromatous aneurysm. Nowadays, the investigations of choice are a chest CT scan with injection of contrast and MRI. Transoesophageal echocardiography excludes dissection of the aorta but may miss a direct image of penetrating ulcer when the latter is of small size. The treatment of choice is not established because the natural history of the condition is not well known. Penetrating ulcer would seem to progress slowly to development of an aortic aneurysm. Surgery is mainly reserved for forms complicated by painful recurrences or rupture and for cases involving the ascending aorta.     

Apropos of a case of neuromeningeal viral infection in pediatrics]

The effect of the immunomodulator 2-methyl-4-amino-6-oxypyrimidine (MAOP)on delayed hypersensitivity was studied in experiments on mongrel mice and in C57Bl and CBA mice. The effect of MAOP and that of oxymethyluracil on delayed hypersensitivity to sheep erythrocytes and dinitrofluorobenzene were compared. Immunosuppression of mice was induced by hydrocortisone in a dose of 50 mg/kg.     

Kikuchi syndrome, Hashimoto thyroiditis and lupus serology. Apropos of a case

Histiocytic necrotizing lymphadenitis (Kikuchi's disease) is an uncommon disease of the cervical lymph nodes occurring in young women, commonly associated with various auto-immune or infectious diseases. We describe the case of a 17 year-old girl who presented a Kikuchi's lymphadenitis occurring concomitantly with Hashimoto's thyroiditis and lupus serology as anti-nuclear, anti-DNA, anticardiolipid antibodies and hypocomplementemia. The patient was treated with prednisone and hydroxychloroquine. Thirty months after, she is doing well and hydroxychloroquine is continued.     

Tracheobronchopathia osteochondroplastica . Apropos a case

We present one case of osteochondroplastic tracheobronchopathy in a patient with right laterocervical tumoration as a manifestation of a normofunctional nodular goiter detected in a thyroid morphofunctional study, and tracheal stenosis secondary both to the goiter and the tracheobronchial process. The coincidence of these two processes, probably fortuitous, has never been described before.     

Leiomyosarcoma of the gingiva. Apropos of a case. Review of the literature]

Effects on erythropoiesis and blood pressure as well as physical performance and mental effects were studied in 15 healthy subjects during intermittent exposure to normobaric hypoxia corresponding to either 2000 m (6 persons) or 2700 m (9 persons) above sea level; another group (5 persons) also served as controls at normoxia. The concept "live high-train low" was used for 10 d consecutively and the exposure to hypoxia was 12 h/d. Blood pO2 and oxygen saturation were significantly decreased during the 10 d at hypoxia. [Hb] and Hct decreased significantly after 2 d in hypoxia and then returned to pre-study levels. Erythropoietin was significantly elevated in both hypoxia groups during the initial 3-5 d. Reticulocytes were significantly increased during 7 d of hypoxia. Submaximal and maximal oxygen uptake, blood pressure at rest and during exercise and the profile of mood states (POMS test) did not change during the study. In conclusion, intermittent normobaric hypoxia for 10 d resulted in a significant stimulation of erythropoiesis. Staying at normobaric hypoxia may serve as a complement to an ordinary altitude level sojourn.     

Pseudocystic ureteritis. Apropos of a case

The authors report one case of ureteritis cystica in a young adult. The diagnosis was made by intravenous urography and confirmed by histologic examination. The patient was treated surgically (nephro-ureterectomy). The etiopathogenic, clinical and therapeutic aspects are discussed with a review of literature.     

Odontoid bone. Apropos of a case

Reporting a clinical observation, the authors emphasize upon the extreme scarceness of this variety of so called "congenital" pseudarthrosis of the odontoid process. The congenital origin, due to a lack of fusion, or the acquired one by avascular necrosis following a trauma is explained. The diagnosis may be clinical, consisting either on a vertebral syndrome occurring in childhood, or on a neurological syndrome in the adult age. It is mainly radiological : A scanner investigation is presented here. Owing to the potential neurological severity of the disease, the posterior fusion has to be recommanded.     

Multiple pilomatricoma. Apropos of a case

Pilomatricoma is a benign, solitary tumor. The histopathologic findings of viable basaloid cells in the periphery, shadow cells in the central part and foci of calcification are characteristic of pilomatricoma. The microscopic aspect of multiple pilomatricomas was not different from solitary pilomatricomas. Though, some consider them as a pathognomonic sign of myotonic dystrophy. Complete surgical excision is the best management.     

Xanthogranulomatous pyelonephritis: conservative therapy in the para-nephritic stage. Apropos of a case]

OBJECTIVE: To determine the diagnostic and prognostic test qualities of the enzyme linked immunosorbent assays (ELISA) for rheumatoid factor isotypes in rheumatoid arthritis (RA), and to compare them with the latex fixation test. METHODS: Rheumatoid factor tests were performed in 1988 consecutive new rheumatology outpatients within two months after their first visit to the outpatient clinic of the Department of Rheumatology of Leiden University hospital. The sensitivity, specificity, accuracy, and predictive values of the tests in discriminating RA from non-rheumatoid arthritis and erosive from non-erosive disease after two years of follow up were determined and presented as receiver operating characteristic curves and post-test probability curves. RESULTS: The sensitivity of the ELISA for IgG, IgA, and IgM rheumatoid factor for RA versus all controls at optimal cut off titres was 72%, 44%, and 69%, respectively; the specificity was 52%, 84%, and 86%. For the latex fixation test the sensitivity was 66% and the specificity 91%. The post-test probability of RA, at a clinical prevalence rate of 12%, given a positive test result in the ELISAs for IgG, IgA, and IgM rheumatoid factor and the latex fixation test, was 17%, 27%, 40%, and 49%, respectively; with negative test results the probability was 7%, 8%, 5%, and 5%, respectively. The specificity of all tests in discriminating erosive from non-erosive RA at two years was low: 41%, 44%, 47%, and 58% for the ELISAs for IgG, IgA, and IgM rheumatoid factor and the latex fixation test, respectively. CONCLUSION: The ELISAs for IgG and IgA rheumatoid factor are of no significance in diagnosing RA and in the prediction of erosive disease. The ELISA for IgM rheumatoid factor is a reasonable alternative for the latex fixation test when age and gender are taken in to consideration. The specificity of all rheumatoid factor tests in discriminating erosive from non-erosive RA is low.     

Intramuscular capillary-venous angioma extending into the infratemporal fossa. Apropos of a case]

Tracheobronchial ruptures are rare but potentially lifethreatening events. We report on the case of a 34-year-old suicidal unrestrained car driver, who developed subcutaneous and mediastinal emphysema and right-sided haematothorax following blunt thoracic trauma. Fibreoptical inspection of the tracheobronchial system revealed a rupture (approximately 2 cm in length) of the pars membranacea of the trachea ending shortly above the carina. CT-scan confirmed the diagnosis of mediastinal emphysema, tracheal rupture and, in addition, left-sided pulmonary contusion. A repair of the tracheal tear was performed by right-sided thoracotomy using a double-lumen tube. The left-sided double-lumen tube was used postoperatively to achieve respirator ventilation with low pressure on the tracheal lumen and on the suture of the tracheal tear. On the other hand, sufficient airway pressure with PEEP for the left lung showing contusion could be provided, using the endobronchial tube. The postperative course was without complications. The patient was on respiratory support for three days due to his-pulmonary contusion. Following final endoscopic control of the trachea he was discharged from the ICU one week after the trauma. The clinical and radiological signs of tracheobronchial ruptures are discussed (respiratory distress, haemoptysis, cyanosis, localised pain, hoarseness, coughing, dysphagia, stridor, subcutaneous emphysema and pneumothorax, tension pneumothorax, mediastinal emphysema). Fibreoptic bronchoscopy is the present gold standard for confirming the diagnosis. The surgical and anaesthesiological approach to the management of tracheobronchial ruptures is described reviewing the current literature.