Infectious mononucleosis-like illness in an infant with acute herpesvirus 6 infection.
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Antibodies, Viral↗
Biomedical subjects
A Hanukoglu
Publications and source records attributed to A Hanukoglu.
Cyclic psychosis associated with the menstrual cycle.
Psychotic symptoms are not included under accepted definitions of premenstrual syndrome (PMS). We present a 14-year-old girl with PMS, who developed a late luteal cyclic psychosis during two consecutive premenstrual periods, which resolved completely after the onset of menses. She was treated with dehydroxyprogesterone for two cycles, and later with placebo for the next three consecutive cycles. Psychotic symptoms did not reappear following two psychotic cycles, and the PMS resolved within the next menstrual cycle. We suggest that cyclic psychoses associated with the menstrual cycle may be a specific benign entity, not included under the recognised functional psychoses. In some cases these psychoses could be classified as a subgroup of PMS.
Adolescent↗
The effect of carbenoxolone on the peripheral metabolism of cortisol in human patients.
Carbenoxolone in human patients induces a state that is similar to that seen in the syndrome of apparent mineralocorticoid excess. The mechanism in both the drug-induced and the naturally occurring disorder is thought to be the inhibition of a normal mechanism for preventing access of cortisol to the mineralocorticoid receptor, namely 11 beta-hydroxy dehydrogenation. We took the opportunity to study the effect of carbenoxolone on the peripheral metabolism of cortisol in the course of evaluating the drug's therapeutic effectiveness in pseudohypoaldosteronism. Carbenoxolone, at a dose that induces mineralocorticoid effects in patients with normally responsive mineralocorticoid receptor systems, did not lead to significant changes in the urinary cortisol: cortisone tetrahydrometabolite ratio. There was, however, a marked inhibition of ring A reduction of both cortisol and cortisone to tetrahydro metabolites. Urinary cortisol level was not significantly changed, but urinary cortisone level was decreased and the cortisol:cortisone ratio markedly increased. We conclude that the urinary cortisol:cortisone tetrahydrometabolite ratio is not necessarily a valid measure of effective inhibition of 11 beta-hydroxy dehydrogenation. A better measure of the inhibitory effect of carbenoxolone on 11 beta-hydroxy dehydrogenation is the urinary free cortisol:cortisone ratio.
11-beta-Hydroxysteroid Dehydrogenases↗
Benign cystic mesothelioma of the peritoneum: the occurrence of an adult entity in a child.
Benign cystic mesothelioma of the peritoneum is considered a disease of adulthood occurring predominantly in women. We report a case of benign cystic mesothelioma of the peritoneum in an 11-year-old boy. The innumerable cystic lesions that varied in size occupied the entire peritoneal cavity. He underwent two laparotomies in a period of 15 months during which a partial removal of the tumor was performed. Four years after the second operation the child is well and active, despite significant tumor tissue left behind after the first two operations. The management of this disease is discussed, and the importance of a conservative approach minimizing the number of laparotomies and avoiding radical surgical intervention especially in childhood is emphasized.
Child↗
Type I pseudohypoaldosteronism includes two clinically and genetically distinct entities with either renal or multiple target organ defects.
Type I pseudohypoaldosteronism (PHA) is a hereditary disease characterized by salt wasting resulting from target organ unresponsiveness to mineralocorticoids. We have studied two kindreds including a total of nine patients with PHA. In kindred I, the propositus presented with renal salt wasting in infancy (vomiting, failure to thrive, short stature, hyponatremia, hyperkalemia) and responded dramatically to a high salt diet (2.5 g/day). Sodium supplementation was discontinued at the age of two. In seven additional family members from three generations, clinical expression of PHA varied from asymptomatic to moderate. In affected members (propositus, mother, and two brothers), hyperaldosteronism persisted over 13 yr; however, the PRA decreased gradually to near normal values. Persistent hyperaldosteronism in the face of a decrease in PRA indicated the development of tertiary hyperaldosteronism due to autonomously functioning zona glomerulosa. The pedigree was consistent with an autosomal dominant mode of transmission with variable expression. In kindred II, the propositus, who was the product of a consanguineous marriage, developed severe renal salt losing at age 9 days. She had also increased salivary and sweat electrolytes consistent with PHA resulting from multiple organ unresponsiveness to mineralocorticoids. Life threatening episodes of salt wasting recurred beyond the age of 2 yr. At 5 yr of age she still requires high amounts of salt supplements (14 g/day). A sister died at 9 days of age with PHA symptoms. Six close relatives (parents, three siblings, maternal uncle) showed no biochemical abnormalities. This pedigree was consistent with an autosomal recessive mode of inheritance. In view of the findings on these two kindreds and the analysis of those in the literature, we conclude that type I PHA includes two clinically and genetically distinct entities with either renal or multiple target organ defects.
Adult↗
Mechanism of corticotropin and cAMP induction of mitochondrial cytochrome P450 system enzymes in adrenal cortex cells.
We studied the kinetics of corticotropin (ACTH) induction of mitochondrial cytochromes P450scc and P450c11 and their electron transport proteins, adrenodoxin and adrenodoxin reductase, in bovine adrenal cortex cells in primary culture. The mRNA levels of these enzymes increase and reach a peak within 3-12 h after ACTH addition. The protein levels of adrenodoxin reductase and P450scc show an increase only nearly 24 h after ACTH addition. After ACTH addition, the intracellular level of cAMP reaches maximal levels within 5 min, and then decreases gradually over 60 min. Hence, we examined the effect of a pulse of ACTH or cAMP analogs on enzyme and mRNA levels. Exposure of the cells to ACTH for 1-2 h was sufficient for maximal induction of the enzymes and P450scc mRNA. In contrast, the induction of the enzymes and the mRNA by cAMP analogs or forskolin required the continuous presence of these agents for over 12 h. But, these agents stimulated cortisol secretion to the medium quickly, indicating that they can activate some intracellular processes while not showing any effect on enzyme induction. The absence of any effect of prolonged cAMP pulses on enzyme and mRNA levels weakens the previous hypothesis that cAMP is the sole second messenger for the ACTH induction of steroidogenic enzymes in adrenal cortex cells. The inductive ability of a brief pulse of ACTH indicates that ACTH can rapidly initiate a series of reactions that result in enzyme induction many hours later.
8-Bromo Cyclic Adenosine Monophosphate↗
Does growth hormone influence the severity of phosphopenic rickets?
The severity of bone disease in phosphopenic rickets is not correlated with serum phosphate levels. In order to determine whether growth hormone (GH) secretion may influence rachitic changes, we evaluated the 24 h integrated concentration of growth hormone (IC-GH) in five children with phosphopenic rickets. Two patients with marked clinical and roentgenographic rachitic abnormalities had normal IC-GH levels. In contrast, three patients with low IC-GH levels had mild rachitic changes. We suggest that the level of spontaneous GH secretion may be one factor which influences the severity of phosphopenic rickets.
Adolescent↗
Human pancreatic polypeptide in children and young adults.
Measurement of human pancreatic polypeptide may be useful for assessment of gastrointestinal function, integrity of the parasympathetic nervous system or screening for endocrine neoplasia. In adults hPP levels have been reported to increase with age. However hPP levels throughout childhood have not been well characterized in comparison with the adult range. We studied fasting human pancreatic polypeptide (hPP) from 45 pediatric patients, from infancy - 15 years, and 18 older adolescents and adults aged 16-45 years. The mean hPP level of children (233 +/- 147 pg/ml) was significantly higher than that (113 +/- 35 pg/ml) of adults (P less than .0001). There was no difference in mean hPP levels of children with normal growth hormone secretion compared to growth hormone deficient patients. There was no effect of gender or body mass index on hPP levels. We conclude that fasting hPP levels must be interpreted with respect to the age of the subject, children particularly, in that preteens may have higher fasting levels than older teenagers and adults.
Adolescent↗
Quantitative monitoring of brain function, vital signs, and hormonal response during acute insulin-induced hypoglycemia.
Acute insulin-induced hypoglycemia provokes changes in central nervous system activity and release of counterregulatory hormones. The clinical relationship between central nervous system activity, hormone secretion, and vital signs has not to our knowledge been previously reported. We used computerized electroencephalographic (CEEG) analysis to monitor 5 nondiabetic subjects during acute insulin-induced hypoglycemia (0.75 U/kg intravenous push). Their glucose nadir was 38 +/- 6 mg/dl (mean +/- 1 SD). A three-phase pattern of change in CEEG power in response to hypoglycemia was observed: phase 1 was characterized by an increase in total CEEG power (natural log of activity = 9.1 +/- 1.3 microV2) over baseline (8.7 +/- 1.2 microV2) in the theta, delta, and beta frequency bands. This phase preceded and coincided with the glucose nadir. During phase 2, power in all frequency bands fell significantly below baseline. A nadir in CEEG power (8.0 +/- 1.6 microV2) occurred 40 to 55 minutes after insulin injection as glucose levels were rising. During phase 3 there was a return to baseline in CEEG power and frequency spectra. Heart rate increase just before phase 1; peak heart rate (91 +/- 8 beats/min) coincided with peak CEEG power and was significantly higher than basal rate (71 +/- 11, P less than 0.05). A significant increase in respiratory rate occurred during phase 1 of the CEEG and persisted through phase 2. A significant decrease in mean blood pressure (nadir = 73 +/- 6 mm Hg) below preinsulin blood pressure (81 +/- 8 mm Hg, P less than 0.05) coincided with the nadir of CEEG power in phase 2.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Surgically curable hypophosphatemic rickets. Diagnosis and management.
Childhood hypophosphatemic rickets (HR) is most often caused by a defect in renal tubular resorption of filtered phosphorus. However, HR can also be caused by secretion of a phosphaturetic factor from a tumor. The presentation of patients with the different HR syndromes may be identical. Distinguishing between the HR syndromes is essential, however, because HR caused by renal defect requires life-long therapy with Vitamin D and phosphate replacement, but tumor-associated HR is cured by removal of the tumor. A case of hemangiopericytoma occurring in bone and causing HR is reported. Children with HR typically have normal levels of serum calcium and parathyroid hormone but very low levels of serum phosphorus. In a child with HR, the following features should prompt a thorough evaluation for a causative tumor: lack of other family members who have hypophosphatemia; presence of aminoaciduria, particularly glycinuria. Causative lesions are most commonly found in the bone or skin.
Child↗
The effect of testosterone therapy on spontaneous growth hormone secretion in boys with constitutional delay.
Testosterone treatment is known to improve growth hormone (GH) secretion in boys with constitutional delay (CD). To determine whether spontaneous GH secretion is normal after treatment, we assessed GH secretion before and after a four- to five-month course of testosterone enanthate in eight adolescents with CD. Before testosterone therapy, the mean (+/- 1 SD) 24-hour integrated concentration of GH (IC-GH) by constant blood withdrawal technique was 1.7 +/- 1.0 micrograms/L (normal range for age, 3.2 to 11.5 micrograms/L), and the IC-testosterone was 1.8 +/- 2.7 nmol/L. Two patients restudied during treatment had normal IC-GH values. After testosterone treatment, the mean IC-GH of the entire group was 3.3 +/- 2.6 micrograms/L, and the IC-testosterone was 6.5 +/- 5.3 nmol/L. Five of eight patients had IC-GH values that were again subnormal. A subnormal IC-GH associated with CD may persist after testosterone therapy is discontinued. Deficiency of spontaneous GH secretion may contribute to short stature and slower growth rates in this patient group. Whether GH therapy in these patients would have a beneficial effect on final height is unknown.
Child↗
Late-onset hypocalcemia, rickets, and hypoparathyroidism in an infant of a mother with hyperparathyroidism.
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Breast Feeding↗
Growth hormone deficiency is not associated with impaired vagal cholinergic nervous system activity in children.
Central cholinergic mechanisms mediate release of growth hormone (GH) as well as peripheral secretion of pancreatic polypeptide (PP). To determine if impaired ability to secrete GH is associated with defective PP response, we studied the PP, epinephrine, and norepinephrine responses to insulin-induced hypoglycemia in 31 children evaluated for GH deficiency by insulin-arginine stimulation (IATT) and 24-h integrated concentrations of GH (IC-GH). Eleven patients had normal GH by IATT and IC-GH (controls), 10 patients had normal GH by IATT but subnormal IC-GH, 10 patients had GH deficiency by both IATT and IC-GH. PP levels peaked at the time of glucose nadir, and remained elevated for 20 min thereafter. The peak PP and incremental PP change from baseline were not significantly different among the three groups. The log peak PP response was inversely correlated with the glucose nadir (r = -0.5, p less than 0.005). Peak PP levels were also significantly correlated with the peak epinephrine levels (r = 0.6, p less than 0.001) but not with norepinephrine. Our findings suggest that 1) GH deficiency disorders are not associated with impaired vagal cholinergic response to hypoglycemia; 2) in children the magnitude of PP response is inversely related to the degree of hypoglycemia; and 3) the peripheral hormonal manifestation of autonomic nervous system responses to hypoglycemia as measured by PP and epinephrine levels are closely correlated.
Child↗
Metabolic clearance rates of synthetic human growth hormone in lean and obese male rhesus monkeys.
The MCR of synthetic human GH was studied in eight adult male rhesus monkeys (Macaca mulatta). Four monkeys were lean (less than 20% body fat), and four were obese (greater than 35% body fat). The monkeys were given a single bolus injection of GH (2.5 micrograms/kg BW), followed by a constant infusion of GH (250 micrograms/h) for 2.5 h. Venous blood samples were collected before the infusion and every 10 min during the infusion. In both groups a plateau of the plasma GH concentrations, indicating a steady state, was reached 70 min after the start of the infusion. The MCR of GH was calculated from the ratio of the constant GH infusion rate and the plateau plasma GH concentration in each monkey. The MCR of synthetic GH was 12.7 +/- 1.7 (+/- SD) L/24 h in the lean group and 19.5 +/- 2.9 L/24 h in the obese group (P less than 0.007). However, the MCR/kg ratio in the lean monkeys was the same as that in the obese animals. We conclude that 1) MCR of GH is directly proportional to body weight; and 2) the lower plasma GH levels in obesity may be due to an increase in its MCR not compensated for by an appropriate increase in the rate of GH secretion.
Age Factors↗
Radiological case of the month. Periappendicular abscess with intraperitoneal gas formation.
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Abscess↗
Fatal aplastic anemia in a child with Down's syndrome.
An infant with Down's syndrome developed severe persistent neutropenia at the age of 9 months and fluctuating anemia and thrombocytopenia at one year of age which terminated as full-blown aplastic anemia at 26 months of age. Immunological evaluation revealed increased peripheral and bone marrow lymphocytes and impaired blood OKT4: OKT8 ratio. Bone marrow granulocyte-macrophage colony forming cells (GM-CFC) were markedly increased, while peripheral blood mononuclear cells (PBMN) produced normal numbers of colonies. The patient's PBMN and serum were both somewhat inhibitory to normal bone marrow derived GM-CFC, suggesting the existence of a suppressor activity both in his serum and PBMN. This unusual course of aplastic anemia and the abnormalities in T-cells and hematopoiesis in Down's syndrome are discussed.
Anemia, Aplastic↗
Loss of voice as sole symptom of subglottic foreign-body aspiration.
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Foreign Bodies↗
Myocarditis after triple immunisation.
We describe a 3 month old infant who developed myocarditis several hours after diphtheria, tetanus, and pertussis vaccination. The time of occurrence of symptoms, the clinical course, and the negative virological studies suggest a possible cardiogenic adverse reaction to the vaccine.
Diphtheria Toxoid↗