ABSTRACT
Paralytic poliomyelitis presenting with quadriparesis, transient encephalitis and bulbar symptoms in 2 patients in close contact with recently vaccinated children with trivalent live oral polio vaccine is described. Symmetrical lower motor neuron involvement of deltoid muscles with electromyographic confirmation was found. Upper motor neuron signs, with symmetrical hyperactive deep tendon reflexes developed in the lower extremities. Poliovirus Type-2 vaccine-like strain was cultured from one patient and both patients showed significant antibody titers rises to poliovirus. Attention is drawn to the possible clinical differences between vaccine associated poliomelitis and the usual features found in wild strain poliomyelitis. It is suggested that in selected cases, non-immunized contacts be given inactivated polio-vaccine when the vaccinees are immunized with the live oral-vaccine.
Subject(s)
Poliomyelitis, Bulbar/etiology , Poliovirus Vaccine, Oral/adverse effects , Adult , Child , Female , Humans , Infant , Male , Neurologic Examination , Poliomyelitis, Bulbar/diagnosis , Poliomyelitis, Bulbar/physiopathologySubject(s)
Burns/therapy , Fluid Therapy , Resuscitation , Shock/therapy , Age Factors , Burns/complications , Child , Edema/etiology , Edema/therapy , Humans , Plasma , Saline Solution, Hypertonic/administration & dosage , Skin Diseases/etiology , Skin Diseases/therapy , Sodium/administration & dosageABSTRACT
A simple method of evaluating and treating burn shock has been used with very good results and low mortality. The amounts of fluids given in a specific time and the type of fluids given are dependent upon the evaluation of a few simple observations. We believe that shock is a treatable condition and that 'shock kidney' is preventable. We stress the difficulty of trying to compare the results of two different burn units.
Subject(s)
Burns/complications , Shock, Traumatic/therapy , Blood Transfusion , Child , Humans , Shock, Traumatic/etiology , Shock, Traumatic/urineSubject(s)
Genes, Dominant , Hyperostosis, Cortical, Congenital/genetics , Adolescent , Adult , Child , Consanguinity , Female , Humans , Infant , Male , Middle Aged , PedigreeABSTRACT
A 4-year-old girl presented with severe clinical and radiological rickets, and alopecia since the age of 1 year. Laboratory studies revealed: hypocalcaemia, hypophosphataemia, secondary hyperparathyroidism, abnormally low intestinal calcium absorption, and markedly elevated circulating 1,25(OH)2D3 levels. A normal calcaemic response to parathyroid extract was obtained. Treatment attempts with vitamin D2, 1 alpha (OH)D3 and 1,25(OH)2D3 were totally ineffective. Intestinal resistance to the action of 1,25(OH)2D3 appeared well established in this case. Refractoriness of bone to this hormone seems less certain. From this new entity of 'Vitamin D resistant rickets due to end organ unresponsiveness', six cases have been hitherto reported in the literature. However, only two have enough resemblance to our case, to constitute a distinct and well defined nosologic subunit. The molecular basis of this disorder(s) remains to be elucidated.
Subject(s)
Alopecia/etiology , Dihydroxycholecalciferols/therapeutic use , Hydroxycholecalciferols/therapeutic use , Hypophosphatemia, Familial/complications , Calcitriol , Calcium/metabolism , Child, Preschool , Dihydroxycholecalciferols/blood , Drug Resistance , Ergocalciferols/therapeutic use , Female , Humans , Hypophosphatemia, Familial/blood , Hypophosphatemia, Familial/drug therapyABSTRACT
The propositus, who died suddenly at the age of 22 months, was investigated because of an unusual myopathy. Family history revealed two sisters and four cousins who had also died suddenly and unexpectedly. The finding of asymmetric septal hypertrophy by echocardiography in the propositus suggested that the cause of the sudden death in the relatives was an undetected cardiomyopathy accompanying a mild and often subclinical myopathy. The affected children were in two sibships and both sets of parents were first cousins. The mother of one sibship was the sister of the father of the other. It is suggested that a gene causes a mild autosomal recessive myopathy with cardiomyopathy that is often undiagnosed and usually ends in sudden unexpected death in the second year of life. The same gene may manifest on echocardiogram in some heterozygotes as asymmetric septal hypertrophy.
Subject(s)
Cardiomyopathies/genetics , Genes, Recessive , Muscular Diseases/genetics , Sudden Infant Death/etiology , Child, Preschool , Consanguinity , Female , Humans , Infant , Male , PedigreeSubject(s)
Abnormalities, Multiple , Face , Joint Dislocations/congenital , Female , Humans , Infant , SyndromeABSTRACT
A case of salt-loosing syndrome in infancy with hyperaldosteronism is reported. This patient was given high doses of 9 alpha Fluorocortisol (0.1 mg/kg/24 h), together with salt supplements. This treatment corrected the abnormality, while higher doses of salt supplements without such large amounts of Flurocortisol were ineffective.
Subject(s)
Fludrocortisone/therapeutic use , Hyperaldosteronism/diagnosis , Hyponatremia/etiology , Humans , Hyponatremia/drug therapy , Infant , Infant, Newborn , Sodium Chloride/therapeutic useABSTRACT
A case of a benign renal tumor found in an infant of 11/2 months is reported. Its clinical, radiological and operative findings were similar to those described previously as Wilms' tumors. Only pathological examination revealed its benignancy. In view of the occurrence of benign congenital mesoblastic nephroma which is evidently not rare in infancy, and because of the side effects of adjuvant cytotoxic and X-ray therapy, the authors feel that the immediate therapeutic approach to a solid intrarenal tumor in infancy should be surgical only. Further therapy should depend on pathological findings.
