Sustained normoglycemia in newly diagnosed type I diabetic subjects. Short-term effects and one-year follow-up.

The impact on remission of normalizing blood glucose levels immediately after diagnosis of type I diabetes was studied in 14 adolescents. Accordingly, in this randomized prospective primary intervention study, 7 of the subjects (i.v. group) received insulin by continuous intravenous (i.v.) infusion via a portable preprogrammed system for 28-62 days and 7 (s.c. group) received conventional subcutaneous (s.c.) therapy. Before therapy, the two groups did not differ significantly with respect to glycosylated hemoglobin, fasting plasma C-peptide, or 24-h urinary C-peptide excretion. During the infusion period, the overall mean fasting plasma glucose (FPG) concentration for the i.v. group was 84 mg/dl with a mean coefficient of variation of 18 +/- 4% (mean +/- SD). During the comparable period for the s.c. group, the mean FPG was 253 mg/dl with a coefficient of variation of 30 +/- 20%. Twenty-four-hour urinary glucose excretions for the two groups were 0.29 +/- 0.06 (mean +/- SEM) and 59 +/- 11 g/day, respectively. Daily insulin requirements in the i.v. group decreased from 1.47 +/- 0.19 U/kg body wt/day at the start to 0.47 +/- 0.10 U/kg/day at the end of the infusion period. Notably, 10-25 days after the infusion period, 5 of 7 subjects experienced a further decrease to a low of 0.27 +/- 0.01 U/kg/day. The mean peak and low requirements in the s.c. group were 0.71 +/- 0.15 and 0.33 +/- 0.13 U/kg/day, respectively, with the only peak requirements being significantly different (P less than 0.01). No patient was able to discontinue insulin therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of temperament on metabolic control in children with diabetes mellitus.

Fifty-one children with insulin-dependent diabetes mellitus (IDDM) and 24 healthy sibling controls were compared on one of two temperament questionnaires completed by parents. Children with IDDM did not provide a characteristic temperament profile or show any problem areas. A regression analysis to predict diabetic control as measured by most recent glycosylated hemoglobin (HbA1c) values revealed that five of the nine temperament scales accounted for a significant 42% of the variance in HbA1c (P less than .001). Children with higher activity levels, greater regularity in routines, milder reactions to external stimuli, distractibility, and negative moods were achieving better glycemic control. The results are discussed in terms of how individual differences in behavioral organization, energy consumption, and stress modulation may affect blood glucose levels.

Insulin resistance developing in children with IDDM.

In a review of the 1000 children with insulin-dependent diabetes mellitus (IDDM) followed at The Hospital for Sick Children, Toronto, between 1970 and 1980, insulin resistance (IR) was noted in 3 after initial sensitivity to exogenous insulin. One was an otherwise typical patient with IDDM. One had cystic fibrosis and is the first such patient with IR to be reported. These two had high insulin antibody titers and responded dramatically to sulfated insulin. The third patient had hyperthyroidism. Although she also had a high anti-insulin antibody titer, IR was controlled only when she was given propylthiouracil. She is the first reported child with IR and hyperthyroidism. The literature on IR in children with IDDM is reviewed. With our 3 patients included, a total of 29 children have been reported. Their mean age is 12.9 yr, 72% are female, and 41% had associated illnesses. Insulin antibodies were elevated in 71% of those in whom measurement was attempted. Of those whose outcome is reported, 67% improved, 21% continued to have IR, and 12% died.

Wolfram syndrome: report of four new cases and a review of literature.

The Wolfram, or DIDMOAD, syndrome consists of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. Diabetes mellitus usually occurs as the first manifestation of this syndrome, followed by the development of optic atrophy, neurosensory hearing loss, and finally diabetes insipidus. We report on four cases with a review of the literature. The diabetes mellitus occurring in these patients is clinically indistinguishable from classic type I diabetes mellitus. Two of three patients continue to have measurable C-peptide secretion 8 yr after onset of diabetes. Two of three patients with Wolfram syndrome had the HLA-DR2 antigen. Combining our cases with those described in the literature, 7 of 11 patients have the HLA-DR2 antigen. The preponderance of the HLA-DR2 antigen in the Wolfram syndrome is different from classic type I diabetes. This is further evidence of the genetic heterogeneity of diabetes mellitus. Although the Wolfram syndrome is rare, it should be considered in diabetic patients with unexplained optic atrophy and hearing loss or with polyuria and polydipsia in the presence of adequate blood sugar control.

Intellectual deficits associated with early onset of insulin-dependent diabetes mellitus in children.

Twenty-seven children with early-onset (less than 4 yr) diabetes (EOD), 24 children with late-onset (greater than 4 yr) diabetes (LOD), and 30 sibling controls were compared in their performance on tests of intellectual functioning and school achievement. The results indicated that children with EOD, particularly girls, scored lower than the other groups of diabetic children and siblings on tests of visuospatial (P less than .05) but not verbal ability. Many of the girls with EOD were also having difficulty at school, and several were receiving special education. Children with EOD had more hypoglycemic convulsions than those with LOD. Both convulsions and age of onset were associated with poorer performance on spatial tasks. Girls with EOD had lower spatial test scores regardless of convulsion history, whereas boys with EOD scored lower only if they had had a convulsion.

Self-monitoring of blood glucose: how accurate are children with diabetes at reading chemstrip bG?

Accuracy of self-monitoring of blood glucose (SMBG) using Chemstrip bG (Bio-Dynamics, Indianapolis, Indiana) was studied in 90 randomly selected children with insulin-dependent diabetes mellitus (IDDM). For 28 children (mean age 8.3 +/- 3.6 yr) a parent routinely read the Chemstrip at home. The remaining 62 children (mean age 13.7 +/- 2.8 yr) read the Chemstrip themselves. Each child or parent analyzed 20 capillary blood samples using Chemstrips and answered a questionnaire on SMBG. The accuracy of SMBG of the group was high (mean correlation coefficient = 0.89 +/- 0.05), but consistency of measurement was variable (mean standard deviation = 1.90 +/- 0.57) and there was a general tendency to underread Chemstrips (mean y-intercept = 1.05 +/- 1.48; mean slope = 0.80 +/- 0.17). For each subject, 0-65% (mean of 34%) of readings were within 10% of the laboratory measurement, and 17-100% (mean 68%) within 20%. These results indicate that most subjects were fairly accurate in reading Chemstrips; however, analysis of accuracy is useful in identifying individuals who are inaccurate or inconsistent in SMBG. Continuing supervision of SMBG is necessary in children with IDDM.

Residual beta-cell function in children with IDDM: reproducibility of testing and factors influencing insulin secretory reserve.

Reproducibility of C-peptide secretion was assessed in 20 children (group 1) by their responses to two Sustacal- (a mixed liquid meal) stimulation tests performed 7-14 days apart. For the 12 C-peptide-positive children (basal C-peptide greater than or equal to 0.03 pmol/ml) there were no differences in the basal or stimulated values between tests 1 and 2. The effect of exogenous insulin on C-peptide secretion was assessed in 20 other children (group 2) by their responses to two Sustacal tests, one test without and one with soluble insulin (0.25 U/kg) injected subcutaneously before testing. Eleven children were C-peptide positive and had no differences in C-peptide response between tests 1 and 2. The results from test 1 in groups 1 and 2 were combined with those from 44 others undergoing a single Sustacal test (group 3, N = 84). There was a close correlation between basal and peak C-peptide concentrations in the 44 C-peptide-positive children (r = .88, P less than .001). Peak C-peptide concentrations correlated inversely with HbA1 (r = -.29, P less than .01), insulin dose in units per kilogram (r = -.40, P less than .001), and duration of diabetes (r = .33, P less than .001) and positively with age at onset of diabetes (r = .34, P less than .001). The C-peptide-positive children had reduced glucose response to Sustacal, lower HbA1 concentration, lower insulin requirement, later age of onset, and shorter duration of diabetes than children who were C-peptide negative.

Brain tumors in children and adolescents--III. Effects of radiation and hormone status on intelligence and on working, associative and serial-order memory.

The effects on intelligence and memory of two post-surgical conditions (radiation treatment, hormone deficiency and supplementation) were explored in 46 children and adolescents with tumors in a variety of brain sites. Verbal intelligence, but not non-verbal intelligence, varied positively with age at radiation treatment. Memory for word meanings was unrelated to either radiation history or to hormone status. Severe deficits in serial position memory occurred with impaired hormone function and an older age at tumor onset. Severe deficits in working memory were associated with a history of radiation and a principal tumor site that involved thalamic/epithalamic brain regions. Radiation treatment and hormone status affect later cognitive function in children and adolescents with brain tumors. Although the greater vulnerability of the verbal intelligence of the younger radiated child and the serial order memory of the child with later tumor onset and hormone disturbances remain to be explained, and although the form of the relationship between radiation and tumor site is not fully understood, the data highlight the need to consider the cognitive consequences of pediatric brain tumors according to a set of markers that include maturational rate, hormone status, radiation history, and principal site of the tumor.

The HLAB*18,BF*F1 in haplotype in type 1 diabetes.

We have investigated the properdin factor B (BF) locus as a possible marker for Type 1 diabetes. The frequencies of the four BF alleles were determined among 70 Type 1 diabetics with onset before age 18 years. The frequency of BF F1 among the diabetics was increased compared with 206 controls (15.7 vs 3.4%). The relative risk (RR) associated with BF F1 was 5.30. The suggestion that BF is most strongly associated with onset of diabetes under 10 years of age was not supported. HBLA 18 was increased (RR = 4.91) and B7 was decreased in frequency (RR = 0.41) among the 62 diabetics tested compared with 238 controls. An association was observed between HLAB 18 and BF F1 among the diabetics. A study of 12 families in which BF*F1 was segregating suggested that BF*F1 and HLAB*18 are components of a haplotype associated with Type 1 diabetes in this population and that both haplotypes of an affected parent may contribute to the development of Type 1 diabetes.

Management of ureteropelvic junction obstruction in neonate.

Fetal hydronephrosis demonstrated by maternal ultrasonography should lead to early investigation during the neonatal period. Postnatal confirmation of the diagnosis of ureteropelvic junction obstruction usually can be established by combining the radiologic modalities of ultrasound, diuretic isotope renal scan, and voiding cystourethrography. Rarely should intravenous urography, antegrade pyelography, or cystoscopy and retrograde pyelography be necessary. Findings of high-grade obstruction and/or significantly diminished function on the affected side(s) should prompt early neonatal reconstruction. Pyeloplasty was performed within the first month of life in 17 infants (20 kidneys) diagnosed as having significant ureteropelvic junction obstruction. Early reconstruction in the neonatal period can be performed successfully with minimal complications and a relatively brief period of hospitalization. This may ultimately achieve maximal preservation of renal function.

Laparoscopic seromyotomy (auto-augmentation) for non-neurogenic neurogenic bladder in a child: initial case report.

An eight-year-old boy with lifelong urinary incontinence secondary to non-neurogenic neurogenic bladder underwent successful laparoscopic seromyotomy (auto-augmentation) with resultant cure of his incontinence. The ability to perform this laparoscopically with its seventy-minute operating time, decreased hospital stay, and improved cosmesis militates for its consideration before a standard augmentation is performed.

Umbilicus preservation with total abdominal wall reconstruction in prune-belly syndrome.

A technique to preserve the umbilicus in abdominal wall reconstruction for prune-belly syndrome is presented. Our technique has been utilized in 5 cases with success.

Diabetes insipidus and nonobstructive dilation of urinary tract.

Two cases of diabetes insipidus (hypothalamic and nephrogenic) with massive nonobstructive trabeculation and dilation of the bladder and hydroureteronephrosis are reported. The cases are evaluated thoroughly--radiologically and urodynamically. Treatment options are discussed, including the use of an important new drug, dDAVP. The general subject of diabetes insipidus and its urologic implications is reviewed.

Spurious impotence after hypospadias repair.

A fifteen-year-old who had undergone multiple hypospadias repairs alleged erectile dysfunction as a result of his surgery. A new method of nocturnal penile tumescence and rigidity monitoring supplied objective evidence to refute this allegation. Attention is drawn to the need for and availability of written documentation when diagnosing the presence or absence of erectile ability.

Laparoscopic vesicoureteroplasty in children: initial case reports.

OBJECTIVE: To determine the feasibility of performing laparoscopic vesicoureteroplasty in children. METHODS: Two pediatric patients, a two-year-old boy and a five-year-old girl, underwent laparoscopic vesicoureteroplasty for vesicoureteral reflux. Operating time was two hours fifteen minutes and three hours fifteen minutes, respectively. RESULTS: The reflux was successfully corrected without morbidity, and they required only short hospitalization (23 hours) and exhibited decreased peri- and post-operative pain as well as improved cosmesis. CONCLUSIONS: These cases represent the first implementation of this technique in humans. We stress that this is a preliminary report and suggest that this technique deserves further study.

Femoral nerve neuropathy after the psoas hitch procedure.

We report the first 3 cases of femoral nerve neuropathy after a psoas hitch vesicopexy, a technique commonly used with ureteroneocystostomy. The condition in 2 patients resolved with conservative therapy, and the third patient required reoperation with removal of an offending suture. All 3 patients recovered completely with no residual neurologic deficit. Urologists who use the psoas hitch must be familiar with this potential complication to prevent its occurrence.

Renal rupture after transplantation.

Nine instances of spontaneous allograft rupture have been identified in a series of 325 renal transplantations. Repair of the graft was accomplished in 4 cases. One graft functioned for five and one-half years, one kidney was removed immediately because of uncontrolled hemorrhage, and two grafts were subsequently removed because of rejection. Immediate nephrectomy was performed in 5 cases of irreversible rejection. The duration of ischemia and method of preservation appeared to have no etiologic importance. Evidence of severe acute rejection was present in all 9 cases. The recent increase in graft rupture parallels the increasing frequency of severe early acute rejection reactions.

Use of saralasin to detect renovascular hypertension in childhood.

Current methods to evaluate renovascular hypertension in the pediatric population often requires a general anesthetic. Saralasin, an angiotensin II competitive inhibitor, is a safe, noninvasive technique which can be utilized at the bedside in a salt-depleted child off antihypertensive medication. Two illustrative cases are presented.

Magnetic resonance imaging of Wilms tumor.

Magnetic resonance imaging (MRI) in 4 children with Wilms tumor suggests the usefulness of this newer imaging modality in evaluating the organ of origin and defining the extent of Wilms tumor. Coronal T1-weighted images were the most useful pulsing sequence for evaluating these children.

Post-transplant hypertension. Normoreninemic severe hypertension treated by bilateral nephrectomies.

In a nineteen-year-old male in whom severe and protracted hypertension developed after a successful renal transplantation, the removal of the diseased kidneys resulted in restoration of normal blood pressure. Prenephrectomy blood samples obtained from the venous drainage of all three renal veins demonstrated no evidence for excessive renin secretion, nor was a significant difference in renin activity found between any two kidneys. It is postulated that the patient may be a clinical variant of the experimental form of renal hypertension with normoreninemia. Alternatively, the remnant kidneys may be implicated to produce a nonrenin pressor substance.