Biomedical subjects
Publications and source records attributed to J M Guillard.
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.
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.
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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.
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.
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.
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.
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.
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.
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.
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.
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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.
One case of subarachnoid haemorrhage due to a spinal cord tumor, seen in a child, is reported. This exceptional etiology, whose the look is often misleading must be called to mind when meningeal and rachidian syndromes are associated. Ependymoma of cauda equina is the most common tumor.
After observation of a 10 month-old infant hospitalised for acute thrombopenia associated with signs of marked radiological hyperostosis, the initial medullograms showed a rich bone marrow free of blast cells. Subsequent complications of the thrombopenia were anemia, neutropenia and circulating blastosis. Marrow aspirations at various sites were unsuccessful. X-rays showed a worsening of the conditions and the child suffers attacks of intense pain. Ultrastructural cytochemistry and Tdt disclosed by immunofluorescence showed that the majority of blasts possessed Tdt and 2-3% were PPO positive. The presence of these 2 markers may correspond either to the presence of a promegakaryoblastic leukemia with aberrant Tdt, or to the coexistence of 2 types of blasts cells.
The authors analyse 40 cases of erythema multiforme (including twenty children under fifteen) seen over a five-year period at the Sick Children's Hospital in Bordeaux, Bullous erythematous target lesions of the skin were associated, in most cases, with pluri-orificial ulcerations on the mucous membranes and, less frequently, with more or less severe systemic or visceral symptoms. Borderline cases were observed, associating features of erythema multiforme simplex and of Lyell disease with variable degrees of dermoepidermal blistering and epidermal necrosis. Infection (32.5% of cases) is a more common etiology in children than in adults; the main pathogens are herpes simplex virus, vaccinia pox virus, and Mycoplasma pneumoniae. Drug-induced forms (37% of cases), which are more often seen in adults than in children, are usually due to sulfonamides or antiinflammatory agents. In 30% of cases, no etiology could be demonstrated. Attention is drawn to the frequency of facial vespertilial erythema, as well as the possible occurrence of severe conjunctival sequellae. The connections between erythema multiforme, fixed drug-induced eruptions, and Lyell disease are discussed: only the last, which implies dermoepidermal cleavage, can be categorized with erythema multiforme. The staphylococcal scalded skin syndrome, in which the epidermolysin of Staphylococcus aureus type II 71 is responsible for a superficial cleavage, proceeds from entirely different mechanisms and should be regarded as totally distinct from erythema multiforme.
Five recent cases of urticarial rash with cow-milk allergy are reported. They all occur at the time of weaning in children who have been sucking during at least three weeks. In three of those cases, digestive manifestations were also found. The histological pattern of the skin showed a vasculitis with deposits of fibrinogen and complement. The steadily high rate of IgE and positive cow-milk RAST in five cases give arguments for reaginic allergy in extra digestive manifestations of cow-milk proteins allergy. The treatment is the suppression of cow-milk and of any products which might contain milk proteins. Our cases had a favorable evolution.
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