Papers by Aaron Hanukoglu
Pediatrics, Apr 1, 1982
<jats:p>Several weeks after reading the interesting article, "Acute Interstitial Nephr... more <jats:p>Several weeks after reading the interesting article, "Acute Interstitial Nephritis in Children: A Report of 13 Cases and Review of the Literature" (Ellis D, Fried WA, Yunis EJ, et al: Pediatrics 67:862, 1981), a 10-year old girl was admitted to our Department with a history of five days' fever [100.1 to 100.2 F (37.8 to 37.9 C)], progressive fatigue, anorexia, diffuse abdominal pain, and vomiting. The mother noticed, one day prior to her admission, a decreased urinary output.</jats:p>
The Journal of Steroid Biochemistry and Molecular Biology, 2020
Renal pseudohypoaldosteronism (PHA1) is a mild form of an aldosterone-resistance syndrome caused ... more Renal pseudohypoaldosteronism (PHA1) is a mild form of an aldosterone-resistance syndrome caused by mutations in the NR3C2 gene that codes for the mineralocorticoid receptor (MR). The disease is inherited as an autosomal dominant trait characterized by signs and symptoms of salt-losing in infancy. Disease manifestations could be severe in infancy but improve after the age of 1-3 years. Some affected members are asymptomatic and remain so lifelong. In this study, we report the identification of a large deletion in the NR3C2 gene (c.1897+1_1898− 1)_(c.*2955+?)del in renal PHA1 patients from an extended family spanning four generations. We prospectively evaluated the plasma renin activity and serum aldosterone profiles over four decades in symptomatic and asymptomatic affected family members. The benefits of early diagnosis on the clinical outcome were assessed as well. The long-term follow-up showed an age-dependent decrease in both plasma renin activity and serum aldosterone levels over the years. However, aldosterone levels remain high lifelong. Thus, levels of aldosterone are a reliable marker to detect asymptomatic family members. The diagnosis of the proposita led to early diagnosis and therapy in other affected family members, significantly mitigating the clinical course. Despite the extremely elevated serum aldosterone levels during pregnancy, affected pregnant women did not experience any ill effects. However, this should be verified by observations in other adult patients.
Metabolic and pediatric ophthalmology, 1981
The Israel Medical Association journal : IMAJ, 2000
DHEAS, the most abundant steroid secreted by the adrenal cortex, is suggested to have an importan... more DHEAS, the most abundant steroid secreted by the adrenal cortex, is suggested to have an important role in the development of immune reaction by activating T cell function and increasing antibody response, and has been tried as a vaccine adjuvant in elderly people. We examined the correlation between endogenous DHEAS and antibody response in the neonatal period by comparing the serum DHEAS levels with the amount of antibody response against hepatitis B vaccination in neonates. Vaccine was administered to 12 healthy infants within 24 hours of birth (day 0), and blood specimens were obtained on days 0 and 30 for determination of anti-hepatitis B surface antibody concentration and DHEAS levels. DHEAS levels varied widely (range 0.38-3.70 micrograms/ml, mean +/- 1SD 2.14 +/- 0.98). While we could identify two groups of patients--those with high DHEAS levels (2.90 +/- 0.56) and those with lower levels (1.30 +/- 0.56)--there was no correlation between DHEAS levels and the antibody respons...
The Journal of laboratory and clinical medicine, 1993
Carbenoxolone in human patients induces a state that is similar to that seen in the syndrome of a... more 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 cortison...
Journal of Pediatric Gastroenterology &amp Nutrition, 1996
Close Window. Close Window. Thank you for choosing to subscribe to the eTOC for Journal of Pediat... more Close Window. Close Window. Thank you for choosing to subscribe to the eTOC for Journal of Pediatric Gastroenterology and Nutrition. Enter your Email address: Wolters Kluwer Health may email you for journal alerts and information ...
Journal of Paediatrics and Child Health, 2000
Measles is an acute, highly contagious and ancient viral disease. To the best of our knowledge, t... more Measles is an acute, highly contagious and ancient viral disease. To the best of our knowledge, the occurrence of an infectious mononucleosis-like syndrome has never been reported previously in children with measles virus infection. A 9-month-old infant was referred to the Chang Gung Children’s Hospital because of high fever for 3 days. The past medical and family histories were not contributory. There was no history of foreign travel, animal exposure or contact with others with infectious diseases. On physical examination, the child was febrile and a discrete maculopapular rash was present over the face and trunk. He had bilateral palpebral conjunctivitis. Other pertinent findings were cervical lymphadenopathy and hepatosplenomegaly (liver edge palpable 2 cm below the right costal margin and the spleen palpable 3 cm below the left costal margin). Investigations revealed a leucocytosis with atypical lymphocytosis (white blood cell count 17.2 × 109 /L with 44% atypical lymphocytes; Fig. 1a). Liver dysfunction was seen, with an increase in serum levels of asparate aminotransferase (108 U/L), alanine aminotransferase (97 U/L) and alkaline phosphatase (191 U/L). The skin eruption progressed rapidly, covering the whole body. A confluent maculopapular rash was then noted on the face as well as on the upper trunk (Fig. 1b). Skin biopsy showed parakeratosis, dyskeratosis and a diffuse infiltration of atypical lymphoid cells in the dermis. No syncytial epithelial giant cells were found. The skin eruption eventually healed, with abundant membranous exfoliation, and the mononucleosis-like symptoms gradually resolved. Serological tests for cytomegalovirus (CMV), Epstein–Barr virus (EBV), human herpes virus 6 (HHV-6), hepatitis A virus, hepatitis B virus and human immunodeficiency virus (HIV) were all negative. However, IgM antibodies to measles were positive by enzyme-linked immunosorbent assay. The IgG antibodies were undetectable at initial presentation, while seroconversion occurred later, on day 35, with a serum 1 : 64 positive for antimeasles IgG. The serum of the patient’s mother was 1 : 2 positive for antimeasles IgG. Haematological data and clinical findings (fever, extensive skin rash, lymphadenopathy, liver dysfunction and atypical J. Paediatr. Child Health (2000) 36, 520–522
The Journal of Clinical Endocrinology & Metabolism, 1989
The MCR of biosynthetic human GH was studied in 12 prepubertal children, 9 adult women, and 13 ad... more The MCR of biosynthetic human GH was studied in 12 prepubertal children, 9 adult women, and 13 adult men. Subjects received a constant infusion of biosynthetic GH, and clearance was calculated by dividing the infusion rates by steady state serum concentrations of GH. We found that adult men have a significantly more rapid MCR of human GH than women (125.2 +/- 7.6 mL/min.m2 in men; 89.4 +/- 7.7 mL/min.m2 in women) and that both men and women have a significantly more rapid MCR of human GH than prepubertal children (66.8 +/- 7.7 mL/min.m2). Sex differences in GH clearance rates may account at least in part for the lower mean serum GH concentrations in pubertal, but not prepubertal, males compared to those in females. The differences in clearance between prepubertal children and adult women and men suggest that the male-female differences in GH clearance are due to androgen effects on GH clearance or on the relative proportions of free and protein-bound GH in serum.
Infection, 1986
The transmission rate and the clinical and pulmonary manifestations of serologically proven mycop... more The transmission rate and the clinical and pulmonary manifestations of serologically proven mycoplasma infections were reviewed in 34 patients from 11 families consisting of 30 children and adolescents aged 1 to 18 years, and four parents aged 30 to 41 years (total number of family members = 59:37 children, 22 adults). Twenty-seven of the 37 children had pulmonary involvement (73%). The total infection rate was 58%, and the infection rate in children 81%. The roentgenologic findings in children with pneumonia showed no pathognomonic features. The most common X-ray finding was bronchopneumonia (48%). Pulmonary infiltrates occurred in six children (20%) under four years of age. Three of the children (10%) had severe clinical and/or radiological manifestations. These findings depict a pattern of mycoplasma infection that is different from previous reports, i.e. high pulmonary infection rates in families, the occurrence of pneumonic infiltrations in young children and a quite severe clinical and radiological course in some of the patients.
Human Immunology, 2004
The interrelationship between human leukocyte antigen immunogenetics and environmental factors an... more The interrelationship between human leukocyte antigen immunogenetics and environmental factors and their contribution to the emergence of type 1 diabetes (T1D) were studied in Jewish immigrants from Ethiopia in Israel. This community displays high incidence of T1D, and is unique both by its ethnic segregation and its rapid exposure to a new environment after the immigration. The study population consisted of 152 Ethiopian Jews living in Israel, 33 with T1D and 119 unrelated controls. Human leukocyte antigen class II susceptible and protective alleles in the Jewish Ethiopian patients were similar to those in patients of other ethnic groups in Israel and in non-Jewish Ethiopian patients, with a few exceptions. Three haplotypes were markedly associated with diabetes in Jewish Ethiopian patients: DRB1*0301 DQA1*05 DQB1*02 (OR 4.4, p Ͻ 0.001); DRB1*0404 DQA1 03 DQB1*0302 (OR 19.2, p ϭ 0.006), and DRB1*0405 DQA1*03 DQB1*0302 (OR 87.8, p Ͻ 0.001). The highly susceptible allele DRB1*0301 was more common in the general Ethiopian population (25.2%) than in all other ethnic groups in Israel, which may render this community prone to the disease. The age at onset of disease in patients with two susceptible haplotypes was negatively correlated with the duration of living in Israel (r ϭ Ϫ0.621, p ϭ 0.04). We concluded that ongoing exposure of genetically predisposed immigrants from Ethiopia to diabetogenic environmental factors eventually leads to a high incidence of overt diabetes in this ethnic group.
European Journal of Pediatrics, 1980
We describe a boy with an unusual adverse reaction to dyphenylhydantoin (DH). This 12 years old b... more We describe a boy with an unusual adverse reaction to dyphenylhydantoin (DH). This 12 years old boy developed a clinical picture of high fever, scarlatiniform rash, a hemorrhagic (purpuric) skin lesion, on his buttocks and neck, stomatitis and conjunctivitis, within two weeks after DH administration. Laboratory examinations were compatible with the diagnosis of disseminated intravascular coagulation. Cases similar to ours are reviewed and it is stressed that DH should be used cautiously as it is not a harmless drug.
European Journal of Pediatrics, 1990
The severity of bone disease in phosphopenic rickets is not correlated with serum phosphate level... more 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.
Clinical Pediatrics, 1986
Two related families with 15 children, seven of whom developed endocardial fibroelastosis (EFE) a... more Two related families with 15 children, seven of whom developed endocardial fibroelastosis (EFE) are described. Three of the children died during infancy, and the disease was confirmed in one of them at autopsy. The survivors, two sisters age 5 years and 15 months (Family A) and two sisters age 17 and 14 years (Family B), are now symptomless and show a decrease in left ventricular hypertrophy. The mode of inheritance of EFE in our two families appears to be either autosomal or X-linked dominant with reduced penetrance.
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Papers by Aaron Hanukoglu