Search PubMed⌕ Search

Biomedical subjects

J G Lapierre

Publications and source records attributed to J G Lapierre.

At least 19 recordsLinked to original sources
PubMed2000

Improvement of disseminated lymphangiomatosis with recombinant interferon therapy.

Disseminated lymphangiomatosis is a rare disorder with a poor prognosis. We present a case involving a 3-year-old boy who presented with pulmonary infiltrates, multiple lytic lesions of the ribcage, and small cystic lesions in the spleen. Open-lung and bone biopsies revealed disseminated lymphangiomatosis. Significant clinical and radiologic improvement were observed and persisted after 28 months of treatment with recombinant interferon alpha-2b (IFN alpha-2b). No significant toxicity has been observed.

Angiogenesis Inhibitors↗
PubMed2000

Pulmonary lymphangiectasia revisited.

PURPOSE: Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. METHODS: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. RESULTS: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. CONCLUSIONS: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Abortion, Spontaneous↗
PubMed1999

Pleuropulmonary blastoma: a rare pathology with an even rarer presentation.

BACKGROUND: Pleuropulmonary blastoma is among the rarest tumors of childhood. Three types have been described: cystic, solid, and mixed. To date, bilateral disease has not been documented. METHODS AND RESULTS: A 5-week-old girl presented with a history of fever. Chest x-ray showed bilateral diffuse cystic lesions. Bowel obstruction developed that required laparotomy. Multiple small bowel polyps were resected. The patient was readmitted 4 months later with deteriorating respiratory status. She underwent sequential thoracotomies for resection of multiple bullae under high-frequency oscillatory ventilation. Small bowel polypectomies were again required because of obstruction. Lung lesions were compatible with pulmonary blastoma but could not be correlated with intestinal polyposis. Bilateral cystic renal lesions were seen on ultrasound scan. Her disease progressed, despite chemotherapy, with the appearance of metastatic iris lesions. She again underwent laparotomies for multiple recurrent generalized small bowel polyps that were causing obstruction. Expanding renal cysts affected kidney function, and she died at 14 months of age. CONCLUSIONS: The rare association between pleuropulmonary blastoma and Wilms' tumor or nephroblastomatosis is known but rarely reported. Lacking pathological evidence, we can only speculate that this was the case. We have been unable to demonstrate any histological association between the renopulmonary and digestive lesions. Despite many unanswered questions, we are likely dealing with a "syndrome" of sorts with a dire outcome, despite aggressive treatments.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1999

Airway hyperresponsiveness and symptoms of asthma in a six-year follow-up study of childhood asthma.

BACKGROUND AND AIM: In an inception cohort study of 457 asthmatic children diagnosed at the age of 3 to 4 years, airway hyperresponsiveness (AHR) was assessed 6 years after first diagnosis in a subgroup of 84 children. Our objective was to associate the level of AHR with the symptomatic asthma status at follow-up. METHODS: Information on respiratory symptoms and medication use for the previous 6 years was obtained. Children with reported wheezing episodes during the previous year (n = 169) or for > or = 2 years at any time during the follow-up period (n = 85) were eligible for the challenge test. RESULTS: Among the 254 eligible children, 166 were randomly selected. The parents of 88 of them consented to have their child participate. At the time of assessment of AHR, 19 children (22%) were asymptomatic and 24 others (29%) had symptoms but did not use any medication. Forty-one children (49%) were symptomatic and required medication, including antiinflammatory preparations in 26 instances (31%). All but two children had significant AHR. There was no significant association between the level of AHR and graded symptomatic and medication score. Twenty-four of the 70 children (34%) with greatly enhanced AHR used no medication. CONCLUSIONS: This study shows that (1) almost all children first diagnosed with asthma 6 years ago and with persisting but not necessarily current symptoms of asthma have increased AHR, which satisfies a proposed epidemiologic definition of asthma; (2) AHR was present in 95% of the 20 currently asymptomatic children; and (3) one third of children with greatly enhanced AHR did not use any treatment.

Asthma↗
PubMed1988

Individualized aminoglycoside dosage regimens in patients with cystic fibrosis.

Individualized dosage regimens have recently been recommended for patients treated with aminoglycoside antibiotics. We have developed a calculator-based program for our patients with cystic fibrosis and have studied 93 courses of intravenous aminoglycoside treatment, comparing predicted and measured values in 45 courses. Pharmacokinetic parameters differed notably among subjects: this was reflected by widely variable total daily aminoglycoside dosage requirements. The mean daily dosage requirements (+/- SD) for tobramycin (62 treatment courses) was 13.0 +/- 3.74 mg/kg, and for gentamicin (26 treatment courses) was 11.5 +/- 2.6 mg/kg. The accuracy of the program was evaluated by its ability to predict peak and trough values in individuals: 84 percent of measured peaks were within 2 micrograms/ml of the predicted level. Nephrotoxicity was observed in one patient, ototoxicity in three. This program provides a simple, safe, and effective method of tailoring an aminoglycoside regimen to the patient's needs.

Adolescent↗
PubMed1987

Diffusion capacity and oxygen desaturation effects on exercise in patients with cystic fibrosis.

Although a fall in arterial oxygen saturation (SaO2) during exercise has been reported in patients with advanced lung disease due to cystic fibrosis (CF), not every patient with advanced disease desaturates, and pulmonary function tests have not been considered predictive as to which patient will desaturate. This study evaluated oxygen desaturation by ear oximetry during a progressive exercise test in 21 patients with CF and compared it to forced expiratory volume in one second (FEV1), the forced vital capacity (FVC) and the single breath diffusing capacity for carbon monoxide (DCO), all expressed as percent predicted. During exercise, the SaO2 fell less than 0.25 percent per ml of the maximal O2 consumption per kilogram of body weight to values never less than 90 percent in 15 patients (group A), whereas it fell more than this and always to values at the end of exercise of less than 90 percent in six others (group B). The FEV1 ranged from 103 percent predicted to 37 percent for group A compared to 28 to 17 percent in group B, while the range of FEV1/FVC was 87 to 52 percent for group A and 54 to 40 percent for group B. The range of DCO for group A was 129 to 84 percent compared to 64 to 54 percent. In conclusion, this study found that both the FEV1 and the DCO could separate those that had significant desaturation from those that did not and that no patient with a DCO of 80 percent or greater had significant desaturation during exercise.

Adolescent↗
PubMed1987

Histamine challenge test in children using forced oscillation to measure total respiratory resistance.

This study analyzed the relationship between total respiratory resistance (Rrs) measured by forced oscillation technique and FEV1 during histamine provocation test in 31 children between seven and 17 years of age. Rrs was measured at frequencies between 6 (R6) and 26 Hz (R26). (R6-R26)/R26 was used as an index of frequency dependency of Rrs. A positive histamine test was defined as PC20 less than 8 mg/ml. Seventeen subjects had a positive test, and all of these had increases from baseline of R6 greater than 50 percent and (R6-R26)/R26 greater than 0.45. Of the 14 subjects whose PC20 was greater than 8 mg/ml, only two had changes in R6 and (R6-R26)/R26 of this magnitude. These two subjects had changes in FEV1 of 16 and 18 percent. There was a strong linear relationship between the changes in FEV1 and both R6 and (R6-R26)/R26 from baseline to the final value at the end of the test (r = 0.87 and 0.91 respectively). In conclusion, this study demonstrated that the evaluation of airway reactivity by histamine challenge may be done by forced oscillation technique. It is easy to administer and may allow testing of children unable to perform spirometry.

Adolescent↗
PubMed1986

Psychomaintenance of childhood asthma: a study of 34 children.

Following the study on psychomaintenance of asthma by Kinsman, Dirks, and Jones (1977), we adapted the Battery for Asthma Illness Behavior (BAIB) to children. Thirty-four children aged 9.3 to 15.4 years were tested with this modified BAIB. They were scored simultaneously on a semistructured psychological interview. Three groups emerged out of these data: low-, medium-, and high-panic-fear personalities. Patients were also rated with regard to their pulmonary function and clinical status, including medication prescribed. The high-panic-fear personality was significantly associated with a higher intensity of prescribed medication, in particular corticosteroids. These results were independent of spirometric pulmonary measurements. We conclude that doctors may overreact to the exaggerated distress of high-panic-fear patients. Psychotherapy could be of help for this group.

Adolescent↗
PubMed1985

[Bronchogenic cysts of the mediastinum in children. Apropos of 14 cases].

Fourteen children, aged from 2 months to 14 years, underwent surgical excision of a bronchogenic cyst in the mediastinum. Nine of these children were asymptomatic and the finding of the tumor was purely accidental. Retrotracheal and subcarinal localizations were more often symptomatic but their visualization on standard chest roentgenograms was sometimes difficult. An oesophagogram was helpful for the diagnosis in those circumstances. No postoperative complication occurred in our series.

Adolescent↗
PubMed1985

[Subpleural lipoma in children].

Primary extrapleural intrathoracic tumors are extremely rare in the pediatric patient. The authors report one observation of intrathoracic subpleural lipoma in five-year-old asymptomatic girl. A thoracic CT-Scan was particularly helpful by demonstrating the homogeneous fatty nature of the mass, the density of-120 Hounsfield Units being specific. This allowed us to limit ourselves to a brief non-invasive diagnostic workup. Complete surgical excision is curative and provides histological certainty about the benign nature of the tumour. It remains the current recommended approach.

Child, Preschool↗