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Biomedical subjects

J M Guillard

Publications and source records attributed to J M Guillard.

At least 19 recordsLinked to original sources
PubMed1992

[Mirror movements. Preliminary therapeutic study].

BACKGROUND: Mirror movements are a specific type of synkinesis. The pathogenesis is unknown. Three types have been identified: 1) a hereditary form, 2) a form associated with other neurological diseases, 3) a sporadic form, as is this case. CASE REPORT: A girl was born at term after cesarean section for fetal anoxia. Psychomotor development seemed normal, but mirror movements of fingers, hands, wrists and forearms were noticed when she was 8-9 months old. There were no other neurological manifestations and the imaging techniques were normal. Rehabilitation was begun when she was 20 months old. The girl is now 4 1/2 years old and writes, draws and cuts up normally. CONCLUSIONS: Mirror movements that are not associated with other neurological disorders usually remain unchanged and can be a professional handicap for adults. Prolonged rehabilitation offers a good functional prognosis.

Dominance, Cerebral↗
PubMed1992

[Infantile spasms associated with a brain tumor].

BACKGROUND: Infantile spasms are exceptionally associated with a focal cerebral lesion such as a brain tumor. CASE REPORT: A 15 month-old girl was treated with hydrocortisone and valproic acid for infantile spasms that had appeared a few days earlier. Her psychomotor development was considered normal and the CT scan was normal. Clinical symptoms disappeared within 10 days and the EEG became normal except for a few epileptiform discharges in the frontal areas. The girl developed partial seizures at the age of 5 years. Imaging techniques showed a brain tumor in the right frontal lobe. Surgical excision was followed by radiation therapy. Analysis of the tumor showed an astrocytoma. The girl is normal 18 months after intervention. CONCLUSION: It is difficult to determine a relationship between the infantile spasms and the brain tumor in this case. The moderately asymmetrical pattern of the EEG after infantile spasms, the fact that these spasms appeared relatively late and the persistence of discharges, lead to repeated specialized investigations.

Astrocytoma↗
PubMed1992

[Beta-ketothiolase deficiency: a case of ketoacidosis with hyperglycinemia].

Regarding a case of beta-ketothiolase deficiency revealed by ketoacidosis with hyperglycinemia, the authors show the way to diagnose and to treat this disease. Ketoacidosis without hyperglycemia or lactacidemia suggested this diagnosis. Gas chromatography-mass-spectrography revealed unusual urinary excretion of metabolic products of isoleucine. The enzymological study of fibroblasts confirmed the diagnosis. The treatment of acute episodes consisted of acidosis control and exclusive glucides intake before diagnosis. Afterwards, a controlled proditic diet and L-carnitine must be given and fasting must be avoided.

Acetyl-CoA C-Acyltransferase↗
PubMed1992

[Cardiac failure by major arterial hypertension secondary to nephroblastoma].

The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.

Heart Failure↗
PubMed1992

[Hip diseases and growth hormone deficiency].

Five cases of hip disease (3 Perthes disease, 2 slipped capital femoral epiphyses) occurring in growth hormone deficient children are described. Relationships with the endocrine deficiency and its treatment are discussed. Growth hormone treatment does not appear to be a causative factor.

Adolescent↗
PubMed1991

Infantile acute hemorrhagic edema of the skin: study of ten cases.

Infantile acute hemorrhagic edema of the skin is not included as a separate entity in the current English-language literature as it is in continental Europe. Therefore we have attempted to clarify the nosologic position of acute hemorrhagic edema among cutaneous vasculitides in children, on the basis of our experience in 10 cases. Our study confirms that acute hemorrhagic edema affects infants between 4 and 24 months of age. The two main features are an ecchymotic purpura, often in a cockade pattern, and an inflammatory edema of the limbs and face. Visceral involvement is uncommon. Spontaneous and complete resolution occurs within 1 to 3 weeks; one to four attacks may occur. Histopathologic examination demonstrates a leukocytoclastic vasculitis. Perivascular IgA deposits can occasionally be found. Besides typical acute hemorrhagic edema, some cases in 2- to 4-year-old children appear to overlap with Schönlein-Henoch purpura. We suggest that typical acute hemorrhagic edema should be regarded as a separate clinical entity. This allows an appropriate prognosis to be made for this generally benign disease of infants.

Acute Disease↗
PubMed1990

[Dysautonomia. A clinical study of a case, ultrastructural data].

We report a new case of non-familial dysautonomia. The patient was a boy with no known Jewish ancestry in whom psychomotor retardation in early infancy failed to lead to specialized evaluation. Regressive episodes of ataxia developed at the age of three and infrequent generalized seizures occurred between four and seven. Diagnosis was first considered at the age of seven after neuroparalytic keratitis developed and rapidly became bilateral. This case has remained highly unusual throughout the course since none of the commonly reported complications (swallowing disorders, aspiration pneumonia, and dysautonomic "attacks") has occurred. No life-threatening manifestations have developed. Peripheral nerve biopsy specimens showed that myelinated fibers, especially of large diameters, were abnormally scarce, and that the histogram failed to exhibit the normal bimodal aspect. Ultrastructural studies also disclosed a marked reduction in the caliber of unmyelinated fibers, whose axons were flattened or occasionally missing. No evidence of regeneration was found. The atypical clinical features in our patient are discussed.

Ataxia↗
PubMed1989

[Evolutive atlanto-axial luxation revealing chronic inflammatory rheumatism in children].

Atlanto-axial luxation in uncommon in chronic arthritis in childhood, especially early in the course of the disease. One case is described in which atlanto-axial luxation occurred early. Computed tomographic myelography showed the cervico-medullary compression which was treated by transoral anterior decompression and posterior occipito-cervical fusion.

Adolescent↗
PubMed1989

[Acute infectious cellulitis. Apropos of 28 cases].

Acute infectious cellulitis is a common condition in pediatric patients. We retrospectively studied 28 cases seen over the last four years. Average age of patients was four years. Fever and a decline in general health were often found. Streptococcus pyogenes and Staphylococcus aureus were the most common organisms in our series. Outcome was favorable under early antimicrobial therapy selected according to age and clinical features. Before three months of age, cellulitis suggests a streptococcus B infection and should be treated by ampicillin or parenteral penicillin, combined with an aminoglycoside if called for. Between 4 months and 2 years of age, Haemophilus influenzae b or a pneumococcus are the most likely organisms; Haemophilus cellulitis should be treated by a third generation cephalosporin for ten days or longer, followed by ampicillin or ampicillin-clavulanic acid per os for ten or 15 days. Beyond 2-3 years of age, all organisms may be the cause of cellulitis, but the most likely are Streptococcus pyogenes and Staphylococcus aureus, treatment consists in administration of either a penicillin M or a macrolide, the route being selected according to the general condition and site of the cellulitis.

Acute Disease↗
PubMed1989

["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications].

We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.

Atrophy↗
PubMed1988

[Necrotizing enteritis during the therapeutic induction phase in leukemia. Value of surgical treatment].

A case of typhlitis in a 3,5 year old girl, during induction therapy for acute lymphoblastic leukemia is reported. This typhlitis, or necrotizing enterocolitis involving the coecum and right colon resulted in stercoral peritonitis during the neutropenic phase. After surgery, the patient had a favorable outcome with complete recovery. Knowledge about this uncommon but severe complication of hemopathies leads to follow clinical, microbiologic and radiologic rules of prophylaxis and screening. Typhlitis requires early treatment by supportive care and surgical cure if necessary.

Child, Preschool↗
PubMed1988

[Cerebrovascular ischemic accident and congenital protein C deficiency in children: apropos of a case].

The authors report the case of a 15-month-old boy with left hemiparesia related to a right sylvian ischemic incident, confirmed by CT scan and carotid angiography. Long-term evolution was favourable with anti-aggregant platelet treatment. A large constitutional protein C deficiency (40%) proved to be the etiology. Protein C is a powerful, physiological, vitamin K-dependent anti-coagulant which neutralizes activated factors V and VIII. Congenital protein C deficiency is responsible for recurrent spontaneous thrombosis affecting the young, mainly in the venous areas, since arterial incidents are less frequent. Protein C assays should be included in the systematic etiologic check-up for thrombotic incidents in children.

Brain Ischemia↗
PubMed1987

Postgranulomatous anetoderma associated with Takayasu's arteritis in a child.

Takayasu's arteritis (TA) is a rare chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum and subcutaneous inflammatory lesions of the leg are the most frequent. We studied a boy with TA in whom a papular rash of the trunk preceded the onset of vascular symptoms by many years. Histologically, the lesions were superficial and consisted of middermal noncaseating tuberculoid granulomas, which progressed to atrophy and anetoderma because of elastic network disruption. Granulomas were also found in synovial tissue but not in a temporal artery biopsy specimen, which showed only intimal hyperplasia. Our observations suggest that vascular and skin lesions with elastic tissue may both result from a common granulomatous hypersensitivity process.

Aortic Arch Syndromes↗
PubMed1987

[Medulloblastoma of the posterior cranial fossa. A second tumor after Hodgkin's disease].

The case of a child presenting with Hodgkin's disease (stage I A, mixed cellular type) at the age of 23 months is reported. Chemotherapy (six treatments with MOPP) enabled a clinical remission, but an in situ relapse with the same histological pattern was observed at the age of 3 years and 9 months. After local irradiation, a second complete remission was observed. At the age of 6, a clinical picture of intraskull hypertension with ataxia, lead to the diagnosis of posterior cavity tumor. Complete excision was performed and the pathological examination showed a typical medulloblastoma. Adjuvant radiotherapy was carried out. Eight months later, a fatal plurifocal medullar relapse was observed. Second tumors in Hodgkin's disease are well known. However, this new case appeared to be the first report of a medulloblastoma after Hodgkin's disease.

Cerebellar Neoplasms↗
PubMed1986

[Post-angina septicemia caused by Fusobacterium necrophorum in a 7-year-old child].

A fusobacterium necrophorum septicemia due to a neglected peritonsillar abscess is reported in a 7 year-old boy with no significant past medical history. Osteo-articulary, hepatic and pleuro-pulmonary septic localizations, with an otherwise favourable outcome left severe orthopedic sequelae in the right hip. This resembles the post-peritonsillar abscess septicemia described by Lemierre in 1936 which was due to an anaerobic bacillus (fusobacterium). The reappearance of this pathology should lead to systematic anaerobic blood and abscess studies. Penicillin G and Metronidazole are still efficient in controlling this organism.

Child↗