Biomedical subjects
Publications and source records attributed to D Christmann.
A 45-year-old man with severe gout was admitted to the hospital because of Staphylococcus aureus septicemia. He had also a biclonal dysglobulinemia, without signs of myeloma. An asymptomatic lytic lesion of the left pedicle of L5 was discovered on radiographs. Histologic examination of the biopsied lesion showed typical tophaceous gout.
Congenital intracranial tumors are very rare. We report an endovaginal ultrasonographic diagnosis of an anaplastic astrocytoma at 31 weeks' gestation. Other means, such as antenatal magnetic resonance imaging and fetal blood sampling were not shown to have any diagnostic advantage. A detailed literature review of the topic is provided.
The authors report a case history of a male newborn at term, presenting with an accessory diaphragm in the right hemithorax, and an associated lung hypoplasia, revealed by a transient respiratory distress. The review of 31 cases published in the literature confirm the rare occurrence of this malformation. The diagnostic difficulties are discussed. The advantages of both MRI and CT scanning are compared. Pulmonary, vascular, or diaphragmatic abnormalities were due either to the isolated diagnosis or to its possible associated malformations. Operative management is often considered in view of the progressive respiratory complications. Excision of the accessory diaphragm does not necessarily solve the problem of a coexisting lung hypoplasia.
The authors illustrate a case of right neonatal congenital diaphragmatic hernia (CDH) of Bochdalek with major pulmonary hypoplasia and postoperative massive mediastinal displacement to the right, leading to tracheal compression and recurrent respiratory distress at every attempt to wean the infant from the ventilator. Mediastinal stabilization was obtained by placing an expansion prosthesis (of the cutaneous type) in the right hemithorax, to prevent mediastinal obstruction of the main airways. This procedure may prove to be an original and efficient solution for the critical postoperative respiratory obstruction due to severe pulmonary hypoplasia and mediastinal mass effect in the newborn with CDH. However, this may not prevent progressive scoliosis, a serious problem frequently seen in the absence of one lung.
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We report a case of primary pulmonary toxoplasmosis in an immunocompetent pregnant woman. Seroconversion did not occur until 2 weeks after detection of the parasite in bronchoalveolar lavage fluid. It is suggested that toxoplasma infection should be considered in any atypical pneumonia in which the X-ray shows diffuse pulmonary infiltrates.
Central European tick-borne encephalitis is mainly found in Central European countries and Austria where hundreds of cases are reported each year. Apart from 2 cases diagnosed in Alsace in 1968 and 1970 respectively, this disease was hitherto unknown in France. We report 8 new cases observed in Alsace between 1985 and 1990. Clinical presentation in these 10 patients was a pure meningitis syndrome in 4 cases and meningo-encephalitis in 6 cases, very severe in 3 of them. All patients recovered rapidly, and only 3 have slight sequelae. In a seroprevalence survey conducted in 1989 among 619 professional foresters of Eastern France, 8% were found to be seropositive, which suggests that the disease is often unrecognized. A study of the large series published in Austria and in other Central European countries has shown that the prognosis of tick-borne encephalitis is not always as favourable as it was in the Alsatian cases: severe sequelae or death occur in 1 to 2% of the patients. The need for a better detection of the disease and for vaccination of the subjects at risk must be emphasized.
A total of 535 chemotherapy naive, hospitalised patients (263 male/272 female) scheduled to receive cisplatin (50-120 mg m-2)-containing regimens participated in a randomised, double-blind, parallel group study to evaluate the efficacy and safety of three intravenous dose schedules of ondansetron in the prophylaxis of acute nausea and emesis. One hundred and eighty two patients received a loading dose of 8 mg of ondansetron followed by a 24 h infusion of 1 mg h-1 (group 1); 180 and 173 patients received single doses of 32 mg (group II) and 8 mg (group III) respectively, followed by a 24 h placebo infusion. Complete and major control (less than or equal to 2 emetic episodes) of acute emesis was achieved in 74% of patients in group I, 78% in group II and 74% in group III. Seventy seven per cent of the patients in group I, and 75% of patients in groups II and III respectively experienced no or mild nausea during the 24 h observation period. A retrospective stratification of the efficacy data on the basis of patient gender showed the response rate in females to be significant lower (43% vs 67%; less than 0.001). Ondanestron was well tolerated; mild headache was the most commonly reported adverse event (11% of patients) with a similar incidence in the three groups of patients. In conclusion, a single intravenous dose of 8 mg of ondansetron given prior to chemotherapy is as effective as a 32 mg daily dose given as either a single dose of a continuous infusion in the prophylaxis of acute cisplatin-induced emesis.
AIMS: To investigate whether autoantibodies to choroid plexus are present in human senile dementia. METHODS: Serum samples from 40 elderly people presenting with characteristic, diagnostic criteria of senile dementia of Alzheimer's type and 20 age matched healthy controls were tested by indirect immunofluorescence for the presence of autoantibodies to choroid plexuses, using frozen sections of rat or human fetal brain tissue. RESULTS: Significant labelling of choroid plexus basement membrane was observed in 17 of the 40 samples from patients with senile dementia; in the control series one sample of rat but not human plexus labelled positively (p < 0.01). CONCLUSIONS: The antibodies identified in this series of patients with Alzheimer's disease suggest that autoimmune mechanisms might be responsible for some of the changes in cerebrospinal fluid production described in this disorder.
BACKGROUND: Myositis ossificans progressiva is a rare progressive disease of connective tissue and muscle whose early diagnosis, before the lesions become ossified, can be difficult. The congenital malformations that accompany the disease may help in this diagnosis. CASE REPORT: A 12 year-old boy developed a localized swelling in the right trapezius muscle. Muscle biopsy showed fibromatosis lesions. Surgical excision was incomplete and despite chemotherapy, new lesions appeared periodically in the cervical and dorsal regions where they became ossified. Bilateral hallux valgus was noticed at that time and used to rectify the diagnosis as myositis ossificans progressiva. CONCLUSION: Many children suffering from myositis ossificans progressiva have congenital malformations, most commonly of big toes and thumbs. These anomalies are important for distinguishing myositis ossificans progressiva from other inflammatory diseases of muscle.
Ondansetron was compared with metoclopramide for antiemetic efficacy in a randomised double-blind trial in 122 patients with advanced breast cancer. All patients were treated with epirubicin (greater than 50 mg/m2) and cyclophosphamide (greater than 500 mg/m2). 50 patients receiving ondansetron and 60 with metoclopramide were considered evaluable. Ondansetron was at least as effective as metoclopramide in the control of vomiting and nausea. The percentage of patients with complete plus major control was 72% (59-85%) vs. 61% (48-74%) on day 1 (P = 0.230) and 79% (67-91%) vs. 66% (53-78%) on days 2-3 after chemotherapy (P = 0.122). Over the 3-day study period, nausea was absent or mild in 60% of the patients treated with ondansetron, compared to 45% given metoclopramide (P = 0.064). No major drug-related side-effects were reported. 1 patient receiving ondansetron experienced gastrointestinal disturbance and headache. Episodes of diarrhoea, fever, hyperkinetic syndrome, fatigue, restlessness and migraine with vomiting were reported by 5 patients treated with metoclopramide. None of the changes in the biochemical or haematological parameters was attributed to the antiemetic treatments.
Two cases of haemorrhagic renal cyst presenting in the neonatal period are described. Ultrasound examination, excretory urogram, and MRI, gave diagnosis in the second case. The remarkable histopathologic pattern of these two cases has led to propose correct diagnosis of pure cystic mesoblastic nephroma, an unusual variety of mesoblastic nephroma.
The authors report a case of neonatal echovirus encephalitis associated with white matter necrosis. The pattern of illness in the neonatal period was diphasic, marked by hyperthermia and the occurrence of seizures. Echovirus was recovered from the cerebrospinal fluid. Cerebral magnetic resonance imaging (MRI) performed at one month of age showed right periventricular white matter necrosis. The infant exhibited mild left hemiparesis. Cerebral MRI at 6 months of age showed a delay in myelination in the right hemisphere. Echovirus encephalitis in the neonate can cause brain damage.
The human immunodeficiency virus 1 envelope glycoprotein is synthesized as a precursor, gp160, which is subsequently cleaved to generate the external gp120 and the transmembrane gp41. Both of these cleavage products are known to mediate critical functions of the virus. In order to define the best strategy for the development of a vaccine against human immunodeficiency virus 1, it could be important to map the crucial epitopes on gp160. This entire gp160 is uneasy to purify because it is readily subjected to proteolytic cleavage. Furthermore, it is anchored on the cell membrane and needs detergent treatment for purification. We thus used a recombinant gp160 which was engineered to remove the cleavage sites between gp120 and gp41 and the hydrophobic transmembrane in order to investigate the murine immune response. We selected a panel of 8 monoclonal antibodies which recognize different epitopes on the immunizing recombinant soluble gp160. The reactivity of the monoclonal antibodies was checked on virus-derived gp160, gp120, and gp41. Three antibodies reacted only with gp120 but the others were shown to react with gp41 epitopes or with discontinuous epitopes bridging gp120 and gp41. One subregion of these epitopes was located using a synthetic peptide corresponding to the sequence of gp41. This epitope is apparently part of an immunodominant site since it is recognized by three different monoclonal antibodies. We used competitive inhibition experiments to map the epitopes on recombinant gp160; therefore, the results are probably indicative of the folding of the recombinant soluble gp160 used for immunization.
We report our experience with the endoscopic treatment of vesicoureteral reflux by a submucosal injection of Teflon in children. During a period of 20 months, 291 refluxing ureters were injected by 4 members of our surgical team. A cystography was performed 3 months later. Because 53 children have a follow-up of less than 3 months, we only consider the remaining 201 ureters. The success rate is 92% which is similar to that of surgical treatment. We insist on some precautions and contraindications. Endoscopic treatment is an undeniable progress, but surgical treatment is still required in some cases.
In its early stage adrenoleukodystrophy (ALD) is characterized by hypodensity at CT and signal abnormalities at RMI (low-intensity signal on T1-weighted sequences, high-intensity signal on T2-weighted sequences) in the white matter of the parieto-occipital region and the splenium of the corpus callosum. These CT and RMI abnormalities are suggestive of ALD in children with progressive alteration of the superior brain functions, but they are not specific of the disease. The authors present two cases of ALD and underline the almost pathognomonic value of contrast-enhanced ribbons found at the periphery of low-intensity signal plaques after gadolinium injection. These areas of blood-barrier disruption on a background of inflammation and active demyelination appear, on T2-weighted sequences, as ribbons of low-intensity signal within plaques of high-intensity signal. MRI is also superior to CT in detecting abnormalities located in the posterior fossa, notably lesions of the auditory fibres.
The authors report their experience of cerebral magnetic résonance imaging (MRI) in the neonatal period. MRI offers many advantages compared to CT scan or ultrasonography in the study of malformations, tumors, infections and anoxic-ischemic brain injury. However, MRI is limited by the duration of the examination the need for total immobility which is achieved in the neonate via administration of chloral hydrate, and lack of accessibility. MRI is a non invasive method for following in vivo brain development during infancy.
Congenital hyperargininaemia is a rare condition transmitted as an autosomal dominant trait. Following a one-year free interval, repeated vomiting, psychomotor regression and spastic paraparesis with talipes equinus progressively develop. The diagnosis, confirmed by arginine assays in blood and urine, is probably often missed. We report a case of homozygous arginase deficiency belatedly diagnosed at the age of 18 years, when treatment with sodium valproate (VPA) was instituted. This female patient presented with psychomotor regression since the age of 15 months and with paraparesis since she was 3 years' old. These symptoms rapidly became worse. At the age of 18 years, when she was bed-ridden, she was hospitalized for subintrant tonic seizures. EEG showed generalized, continuous spike-wave discharges at the rate of 3.5 c/s. Treatment with VPA was instituted. Five days later, she went into a state of stupor. Blood ammonia level was elevated at 362 mumol/l. VPA was discontinued, and this was followed by a regression of disturbances of consciousness and by a decrease in arterial ammoniaemia, although the ammonia levels remained high, fluctuating between 40 and 100 mumol/l. Several months after VPA treatment was interrupted, the patient had a second episode of stupor, and her ammoniaemia was 500 mumol/l. Serum amino acid chromatography showed hyperargininaemia at 501 mumol/l (N = 30-150 mumol/l). The diagnosis of arginase deficiency was confirmed by the rise of arginine in red cells, cerebrospinal fluid and urine and, above all, by the finding of a deeply depressed arginase activity in erythrocytes. In all cases of intolerance to VPA, arterial ammoniaemia should be measured after withdrawal of VPA, some time after the acute episode.(ABSTRACT TRUNCATED AT 250 WORDS)