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Biomedical subjects

A Tejani

Publications and source records attributed to A Tejani.

140 records · Page 8Linked to original sources

Occurrence of HLA types in H. influenzae type B disease.

We have studied 50 Caucasoid children under 7 years of age with Haemophilus influenzae b disease. Half of the patients (Group A) had invasive disease shown by positive blood and/or spinal fluid culture. The other half (Group B) had noninvasive disease characterized by fever, nasopharyngitis, negative blood culture, and positive throat culture. Age, number of other siblings under 12 years old in the family, immune response, antibody production and genetic markers were compared in the two groups. Significant difference between the two groups was only seen in their genetic markers. HLA-B12 was present in 52% of Group A patients as opposed to 16% in Group B patients (P less than .01). HLA-Bw40 was present in 24% of group B patients and absent in all Group A patients (P less than .01). These findings would suggest that susceptibility and resistance towards developing invasive type b disease may be genetically determined.

Antibody Specificity↗

Intestinal laceration secondary to clamping of an occult omphalocele.

An omphalocele is a congenital defect of the abdominal wall, through which intestines and other intraabdominal organs protrude outside the abdominal cavity. The defect in the abdominal wall is covered by a translucent sac composed of peritoneum internally and amniotic membrane externally, through which the viscera can be seen. Such an anomaly is obvious and easily recognized at birth when the defect in the abdominal wall is large, but a small defect, also designated as umbilical cord hernia, is often unnoticed and may, when the cord is clamped, result in an iatrogenic laceration of the gut. We report such an encounter to alert the physician and suggest a method to prevent the iatrogenic misadventure. A careful follow-up of the child, including developmental evaluation, is presented and compared with her healthy twin. A review of the relevant literature shows that the anomaly is not rare and that there is often a delay of several days in establishing the correct diagnosis, leading to a fatal outcome in some cases.

Diseases in Twins↗

Growth, health, and development after neonatal gut surgery: a long-term follow-up.

Growth and development data on 19 children who survived gut surgery in the immediate newborn period are presented. The follow-up period ranges from seven months to seven years six months, with a mean of three years eight months. Neonates who undergo gut resection are at a higher risk for delay in height and weight gain compared to those who have gut surgery without resection. Six of the 12 children who had gut resection showed delay in height gain and seven showed delay in weight gain. Of the seven children who had gut surgery but no resection, only one showed delay in height and weight gain after the age of 1 year. An individual approach and careful serial follow-ups are recommended for all children undergoing gut resection in the newborn period. Of the eight children who had psychometric testing in the gut-resected group, only two are normal. Three of the four older children in this group show signs of perceptuomotor defects, suggesting the need for subtesting such children at about 6 years of age so that remedial help, if necessary, may be provided when formal schooling begins.

Bender-Gestalt Test↗

Intrafamily spread of Haemophilus type b infections.

Meningitis and epiglottitis are the clinical manifestations of severe Haemophilus influenzae serotype b infection. Compared with meningitis, epiglottitis occurs in older children. When secondary cases occur within the family, the type of clinical manifestation produced by this serotype is generally similar in siblings. This report concerns the unusual occurrence of meningitis developing in older child and epiglottitis developing in the younger one. We discuss the possible explanations for this unusual pattern. We also survey the spread of H influenzae both within and outside the family unit and review the present status of histocompatibility antigens and Haemophilus disease.

Alleles↗

Noonan's syndrome associated with polycistic renal disease.

Noonan's syndrome is an eponymic designation that has been used during the last 8 years to describe a variable constellation of somatic and visceral congenital anomalies, which includes groups of patients previously referred to as male Turner's, female pseudo-Turner's and Bonnevie-Ullrich syndromes. It is now recognized that both sexes may show the stigmas of this condition and, unlike Turner's syndrome, there is no karyotype abnormality although there is often a familial pattern. The most commonly observed anomalies include webbing of the neck, hypertelorism, a shield-shaped chest and short stature. Congenital heart disease, principally pulmonary stenosis, and sexual infantilism often with cryptorchidism in the male subject are additional associated anomalies in this syndrome. Renal anomalies have been described rarely and usually consist of rotational errors, duplications and hydronephrosis. We report the first case of an infant who displayed many of the stigmas of Noonan's syndrome and also showed early evidence of frank renal failure secondary to renal dysplasia with cystic disease.

Humans↗

Assessment of objectives of post-doctoral general dentistry programs in Canada.

Objectives of hospital-based post-doctoral general dentistry programs in Canada were assessed by questionnaire. Seventy percent (14 of 20) of the program directors responded. Educational goals and objectives were assessed in professional skills and practice management, public health and preventive dentistry, oral medicine and pathology, special needs patient care, trauma and emergency care, restorative/prosthodontic care, endodontics, orthodontics/pediatric dentistry, oral surgery, periodontics, pharmacology, and functioning in a hospital. High rankings of proficiency were related to primary care, restorative/prosthodontic, endodontic, and surgical care. Emergency care, sedation, and pharmacology were also ranked highly. Lower rankings of proficiency were reported in orthodontics, aspects of public health dentistry, practice management, and advanced oral and maxiliofacial surgery. When the results of the Canadian survey were compared with those of a survey of US post-doctoral general dentistry programs, substantial similarity was seen. The findings support continuing reciprocity in accreditation standards between the Canadian and American Commissions on Dental Education and Dental Accreditation.

Anesthesia, Dental↗

Urinary protein electrophoresis patterns in childhood idiopathic nephrotic syndrome.

We investigated the utility of a standard urinary protein electrophoresis (UPEP) to distinguish among three common variants of childhood idiopathic nephrotic syndrome (NS). The UPEP was performed on 66 urine samples obtained during a disease relapse in 43 children and adolescents with idiopathic NS. There were 15 children with minimal change disease (MCD), 11 with IgM nephropathy (IGMN) and 17 with focal segmental glomerulosclerosis (FSGS). Fourteen of the 26 children (54%) with MCD or IGMN and 16/17 (94%) of the patients with FSGS manifested a frequently relapsing or steroid dependent course. The mean percent albumin and gamma globulin excretion in the UPEP in patients with MCD and IGMN were 75.5 and 2.9 versus 72.6 and 3.9, respectively (p = NS). Both patterns were significantly different from that observed in FSGS, albumin 62.2%, gamma globulin 7.1% (p less than 0.005). Although the percent gamma globulin excretion was inversely related to GFR in children with FSGS, this measurement exceeded a 4.3% cutoff in 9 of these patients while their GFR was normal (less than or equal to 80 ml/min/1.73M2). Therefore, we recommend the use of the UPEP as a marker of urinary protein selectivity and to monitor children with high-risk nephrotic syndrome i.e., those with a frequently relapsing or steroid dependent clinical course, for histological transitions.

Child↗

Long-term outcome of one haploidentical live related donor transplantation in children.

UNLABELLED: We report on 69 children, 6 months to 18 years old, who received a one-haploidentical live related donor transplantation from 11/72 through 4/89. Thirty patients were maternal and 22 were paternal recipients; 23 were Black, 27 Caucasian, 17 Hispanic, and 1 each Oriental and Arabic. The original diagnoses in these patients were aplastic/dysplastic kidneys 13%, obstructive uropathy 30%, focal segmental glomerulosclerosis (FSGS) 23% and lupus nephritis 10%. RESULTS: 1-, 5-, 10- and 15-year actuarial patient and graft survivals were 98, 84, 84, 84% and 94, 70, 41, 30% respectively. Actual 5-year graft survival of paternal recipients was better than maternal recipients (p less than 0.05). No differences were noted comparing actuarial graft survival between Blacks, Caucasians and Hispanics. 1/16 FSGS and 1/7 lupus nephritis patients had recurrence of their original disease. Posttransplant therapy in 37 patients consisted of azathioprine and prednisone while 32 patients received ciclosporin A (CSA) and prednisone. At transplantation, CSA and prednisone patients were younger, i.e., 10.7 years (range 0.5-18) compared to 13.3 years (range 4-18) and (p less than 0.03) in azathioprine and prednisone patients. The 5-year actuarial graft survival of azathioprine and prednisone patients was 57% compared to 76% in CSA and prednisone patients. 16/32 CSA and prednisone patients had their prednisone discontinued for a mean duration of 18 months (3-54 months). Ten of these 16 patients were prepubertal and had their growth hormone levels measured. All had an adequate level (greater than or equal to 10 ng/ml) poststimulation. Five of the 10 patients were less than 6 years of age and exhibited accelerated growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Community acquired infections in children on maintenance cyclosporine therapy.

Cyclosporine has profound immunosuppressive properties. We reviewed our experience of community acquired infections in 62 children who received the drug from 2 to 36 months. 36 children received the drug for a renal transplant for a mean of 18 months. There were 3 minor and one major episode of infection in this group. The frequency of both minor and major infections in cyclosporine recipients was significantly lower when compared with a group of renal recipients treated with azathioprine. 26 children received cyclosporine for 2 months for resistant nephrotic syndrome. This group had one major and one minor episode of infection. Despite the relative safety of cyclosporine recipients from infectious complications, a more prolonged follow up is necessary.

Adolescent↗

Progressive renal damage due to lead intoxication in early life.

We have evaluated the effect of an increased body lead burden in young (3 weeks old) and adult (9 weeks old) Sprague-Dawley rats. Following intraperitoneal administration of lead on two occasions, both renal blood flow and glomerular filtration rates were diminished in the young rats, but not in the adult rats. Our study lends credence to the hypothesis that age at the time of exposure is critical to the development of progressive renal damage.

Age Factors↗

Recurrence of the original disease in pediatric renal transplantation.

Recurrent disease in the transplanted kidney is extremely common and yet accounts for less than 5% of graft loss in the adult population. In children, however, recurrence of the original disease has been responsible for over 6% of index graft failures and 12% of second graft failures in North America. The diseases that commonly lead to recurrence and consequent graft failure in children are primary glomerulo-nephritides such as focal segmental glomerulosclerosis (FSGS), systemic diseases of which hemolytic uremic syndrome (HUS) is best known for this complication and metabolic diseases of which primary hyperoxaluria (PH) is of importance. With the development of new therapeutic and surgical strategies, none of these conditions are now considered a contraindication for cadaveric (CAD) or living related donor (LRD) transplantation. Recurrent disease has also provided investigators a unique opportunity to study the pathogenesis of glomerular disease.

Child↗