Louis Low - Academia.edu (original) (raw)
Papers by Louis Low
Diabetes, 1987
Studies of Caucasian and Japanese patients with insulin-dependent diabetes mellitus (IDDM) have s... more Studies of Caucasian and Japanese patients with insulin-dependent diabetes mellitus (IDDM) have shown that heterozygosity for certain HLA-DR antigens confers a high risk of developing the disease. The HLA antigens of 75 Chinese patients and 100 Chinese controls in Hong Kong were studied to investigate the role of HLA-DR heterozygosity in Chinese individuals. Some of the patients and controls were also tested for allotypic variation in the complement components C2, C4, and BF. Three alleles, Aw33, B17, and DR3, had increased frequencies in patients compared with controls and frequently occurred together in the same phenotype, which suggested their existence as a haplotype. There were no statistically significant differences in complement allotype frequencies between patients and controls, although the C4B null allele seemed to be associated with Aw33, B17, and DR3. No other HLA-DR antigen appeared to be associated with IDDM. However, when the patients were separated on the basis of age at onset, the frequency of DR3/DRw9 heterozygosity was markedly increased in patients presenting in the first decade of life, but there was no increase in patients presenting at >20 yr of age. DRw9 is strongly associated with autoimmune disease in Chinese, whereas DR3 is not. We suggest that the major IDDM susceptibility locus in Chinese is associated with HLA-DR3 and that patients with HLA-DR3 and HLA-DRw9 have an added predisposition to autoimmune disease and therefore develop IDDM earlier than patients without DRw9.
Indian Journal of Pediatrics, 2005
... All the studies have demonstrated that short term GH treatment for one year resulted in signi... more ... All the studies have demonstrated that short term GH treatment for one year resulted in significant increase in the growth Indian Journal of Pediatrics, Volume 72--February, 2005 161 Page 4. Louis CK Low ... 1999 a Katzos Get al. 20004s Sartorio A 2000 Wu KH et al. 2003 .7 ...
Thorax, 2001
Myositis associated with graft-versus-hostdisease (GVHD) typically presents with proximal muscle ... more Myositis associated with graft-versus-hostdisease (GVHD) typically presents with proximal muscle weakness, myalgias, and a raised creatinine phosphokinase (CPK) level. We report a case of a 51 year old man who developed respiratory muscle weakness five years after an allogeneic bone marrow transplant for multiple myeloma. His symptoms included tachypnoea, abdominal paradox, and orthopnoea. Pulmonary function tests revealed diminished vital capacity and maximal inspiratory and expiratory pressures. Serum CPK levels were raised and a peripheral muscle biopsy specimen was consistent with GVHD. He improved with immunosuppressive therapy.
The Indian Journal of Pediatrics, 2005
Hypertransfusion and regular chelation therapy have allowed improved survival in patients with th... more Hypertransfusion and regular chelation therapy have allowed improved survival in patients with thalassemia major (TM). Despite medical advances, growth failure and hypogonadism remain significant clinical problems in these patients in adolescence. Disproportionate truncal shortening which is common especially among adolescents with thalassemia, is due to platyspondyly resulting from a combination of factors like hemosiderosis, desferrioxamine toxicity or deficiency of trace elements. Although growth hormone (GH) deficiency and GH neurosecretory dysfunction have been described in TM patients, most short TM patients have normal GH reserve. The low serum IGF-1 and IGFBP-3 concentrations in TM patients despite having normal GH reserve and serum GH binding protein levels suggest that a state of secondary GH insensitivity exists. The pubertal growth spurt may be impaired in TM patients going through spontaneous or induced puberty and may have a negative effect on final adult height. GH therapy in dosages ranging from 0.5-1.0 IU/kg/wk has resulted in a significant improvement in growth velocity in short TM children without any adverse effects on skeletal maturation, blood pressure, glucose tolerance and serum lipids. There is limited evidence that GH treatment can result in an improved final adult height in short TM children. Careful and regular clinical and biochemical monitoring should be preformed on these patients while they are treated with GH.
Zhonghua er ke za zhi. Chinese journal of pediatrics, 2005
Hutchison's Paediatrics, 2012
Hutchison's Paediatrics, 2012
The Pediatric Infectious Disease Journal, 1998
A 5-year territory-wide retrospective survey of invasive Haemophilus influenzae type b (Hib) dise... more A 5-year territory-wide retrospective survey of invasive Haemophilus influenzae type b (Hib) diseases in Hong Kong established that the annual incidence for children <5 years old was 2.7 per 100000 [95% confidence interval (CI), 2.0 to 3.5]. However, the corresponding annual incidence in Vietnamese refugees in Hong Kong was 42.7 per 100000 (95% CI 17.2 to 87.9), giving a relative risk of 18.5 (95% CI 8.3 to 41.0). The nasopharyngeal carriage rate of Hib was zero in 621 healthy Chinese children and 1.3% (95% CI 0.04 to 2.63%) in 300 healthy Vietnamese refugees 2 months to 5 years old in Hong Kong. The corresponding carriage rate of nontypable H. influenzae was 5.8% (95% CI 1.4 to 7.6%) in Chinese and 65.4% (95% CI 58.9 to 69.8) in Vietnamese. In a larger study of 1812 healthy Chinese children between 6 months and 5 years of age investigated by throat swabs, again no Hib was isolated but 141 children (7.8%) were found to be carriers of nontype b H. influenzae. In a study of 596 healthy Chinese children and adults, 25% had the protective level of anti-Hib antibody of >0.15 microg/ml by 1 year and 90% had reached >0.15 microg/ml by 6 years of age. There was some evidence that these "natural" antibodies against Hib in Hong Kong Chinese were cross-reacting antibodies against antigens on other encapsulated bacteria.
Pediatric Neurology, 1999
Insulin-dependent diabetes mellitus (IDDM) is rare in Chinese children. There have been no report... more Insulin-dependent diabetes mellitus (IDDM) is rare in Chinese children. There have been no reports on the prevalence of peripheral neuropathy in Chinese children with IDDM. This study aimed to determine prevalence of subclinical peripheral neuropathy in Chinese children with IDDM. Motor and sensory nerve conduction studies of both median, ulnar, peroneal, and tibial (motor nerves) and median, ulnar, and sural (sensory nerves) were performed in 38 children with IDDM (18 males, 20 females). The age was 4-21 years (mean ؍ 12.7 years; median ؍ 12 years, 6 months). The duration of diabetes was less than 5 years in 15, 5-10 years in 14, and more than 10 years in nine. Neurophysiologic evidence of subclinical peripheral neuropathy was present in 26 patients (68.4%) of which motor, sensory, or motor and sensory involvement was 26 (68.4%), eight (21.1%), and 26 (68.4%), respectively. Twelve (31.6%) and 14 (36.8%) children had mild and moderate degrees of peripheral neuropathy, respectively. Among the 26 children with abnormal nerve-conduction studies, two (7.7%) had symptoms of numbness and pain in the lower limbs. Thus, two children had symptomatic neuropathy and most (n ؍ 24) had asymptomatic peripheral neuropathy. Two children had systemic hypertension, and one (3.8%) had laboratory evidence of early renal complications. Analysis of demographic and laboratory risk factors for the development of subclinical peripheral neuropathy revealed that the age of onset, duration of diabetes, level of hemoglobin A1c, triglyceride, cholesterol, serum creatinine, and urea, microalbumin/ creatinine ratio, and urinary microalbumin excretion rate were significantly related to the development of subclinical peripheral neuropathy in specific nerves.
Neuroendocrinology, 1991
Growth hormone-releasing hormone (GHRH) stimulates GH secretion in man and the hormonal response ... more Growth hormone-releasing hormone (GHRH) stimulates GH secretion in man and the hormonal response is specific. The attenuation of GH response to bolus GHRH after prior exposure of GHRH of up to 24 h was not demonstrated in normal or GH-deficient subjects after more prolonged exposure. This suggests that the partial loss of responsiveness to GHRH may reflect short-term negative feedback by GH. The stimulatory effect of clonidine and L-dopa on GH release is mediated via GHRH. Other stimuli like hypoglycaemia, arginine and propranolol augment GH release in man by modulating hypothalamic somatostatin secretion. Although GHRH test can differentiate between hypothalamic or pituitary cause of GH deficiency, it is of little diagnostic value in children with short stature. Favourable results have been observed in 60-70% of GH-deficient children treated with GHRH, but the dose and mode of administration are still being explored. We found that low dose (1-2 micrograms/kg) GHRH given subcutaneously every 3 h by a pump was effective in promoting growth in 5 of 7 patients after 1 year. Treatment was continued for 2-4 years in 4 patients and growth velocities ranging from 4.5 to 8.2 cm/year were maintained using a dose of 3 micrograms/kg/pulse.
Journal of Pediatric Psychology, 2000
mary aims, both related to current gaps in the literature. The first aim was to examine risk vari... more mary aims, both related to current gaps in the literature. The first aim was to examine risk variables for emotional distress in the Hong Kong Chinese pediatric diabetes population. Much of what is known about adjustment to chronic illness in youths is based on information from Western samples. The second aim was to investigate the pathway from emotional adjustment to glycemic control. Whereas it has been documented that nonadherent youths show emotional and behavioral problems (e.g., Kovacs, Goldston, Obrosky, & Iyengar, 1992), there is less information about how
Journal of Pediatric Psychology, 2003
Objective To extend longitudinally an earlier study of the pathway from symptoms of emotional dis... more Objective To extend longitudinally an earlier study of the pathway from symptoms of emotional distress (ED) through self-efficacy (SE) and adherence to glycemic control (GC) in youths with diabetes, and to examine the contribution of different specific adherence behaviors to changes in GC. Methods Fifty-six Hong Kong youths with diabetes received a follow-up evaluation 12-24 months after initial participation. ED, SE, self-reported adherence to medical regimen (SRA), and GC were assessed at both evaluations. Results The pathway from ED to SE to SRA to GC was replicated. Participants' SRA to regular checks on blood glucose levels, and taking steps to maintain levels in the recommended range, explained significant variance in changes in GC. Conclusions The model offers strategies to enhance health care in youths with diabetes. Findings support the importance of adherence to the medical regimen but emphasize the complexity of the relationship between adherence behaviors and GC. Selfregulatory behaviors, rather than compliance with fixed instructions, appear to have the most impact on GC.
Journal of Pediatric Endocrinology and Metabolism, 1997
Journal of Paediatrics and Child Health, 2011
Aims: To review the incidence and risk factors of primary hypothyroidism in very low birthweight ... more Aims: To review the incidence and risk factors of primary hypothyroidism in very low birthweight (VLBW) infants in our centre and to assess their neurodevelopmental outcome. Method: Retrospective, descriptive review of VLBW infants with primary hypothyroidism from 1 January 2000 to 31 December 2008 in a perinatal centre. Case control comparisons for neurodevelopmental outcome at 18 months, nested to a prospective VLBW cohort (Vermont Oxford Database). Results: Twelve cases were identified, with the incidence of primary hypothyroidism of 1 in 55 live births in our VLBW cohort during the 9-year study period. Umbilical cord blood thyroid-stimulating hormone was abnormal in less than half of the cases using the current cutoff (Յ14 mIU/L). Five cases were transient in nature, four cases were permanent and the causes for the rest remained undetermined at the time of review. Elevation of thyroid-stimulating hormone was first evident at a mean of 2.4 weeks post-natally. Follow-up assessment reviewed normal development in 83% of cases (10 out of 12 cases). There was no statistically significant difference in Griffith's scores at 18 months between the case and matched controls. Conclusions: The high incidence of primary hypothyroidism in our VLBW cohort deserved stringent monitoring of thyroid function postnatally. Umbilical cord blood screening was not useful as a screening tool because of its low sensitivity. Neurodevelopmental outcome for treated primary hypothyroidism was favourable as assessed at 18 months of age.
Journal of Paediatrics and Child Health, 1992
The role of transplacental transfer of maternal thyrotrophin (TSH)-blocking antibodies causing co... more The role of transplacental transfer of maternal thyrotrophin (TSH)-blocking antibodies causing congenital hypothyroidism in Southern Chinese children was examined in this study. Twenty-two mothers of 24 patients with congenital hypothyroidism were studied 3-5 years after delivery. None of them had thyroid dysfunction at delivery or at the time of study. None had antithyroglobulin or antimicrosomal antibody. Only one mother was found to have TSH-binding inhibitory immunoglobulin (TBII), and her child had agenesis of the thyroid. This women had Graves disease in remission for 2 years before delivery. None had TSH-stimulated cAMP response inhibitory immunoglobulin (TSII). Ten of the 24 congenital hypothyroid children had transient neonatal hypothyroidism, seven had agenesis of the thyroid, six had dyshorrnonogenesis and one had a sublingual thyroid. As none of the mothers who had children with transient neonatal hypothyroidism had blocking antibodies at the time of study, the aetiology of the transient neonatal hypothyroidism remains unclear. These data suggest that maternal TSH-blocking antibodies do not play a role in most cases of sporadic congenital hypothyroidism.
Journal of Paediatrics and Child Health, 1989
Journal of Paediatrics and Child Health, 1987
A boy aged 7.5 years presented with precocious onset of secondary sexual characteristics and symp... more A boy aged 7.5 years presented with precocious onset of secondary sexual characteristics and symptoms and signs of neurological dysfunction. A CT brain scan demonstrated a suprasellar mass lesion. The elevated P-hCG and a-fetoprotein concentrations in cerebrospinal fluid and in serum were consistent with a diagnosis of intracranial embryonal carcinoma, although histological diagnosis was not available. The tumour markers P-hCG and a-FP should be looked for in all children with precocious puberty presenting with midline brain tumours.
The Journal of Clinical Endocrinology & Metabolism, 1989
A subset of patients with the syndrome of acanthosis nigricans and insulin resistance type A is c... more A subset of patients with the syndrome of acanthosis nigricans and insulin resistance type A is characterized by acromegaloid features in addition to hyperinsulinemia, hyperandrogenemia, and an inherent defect in insulin receptor function. It has been proposed that the acromegaloid features result from the interaction of insulin at concentrations encountered in vivo, with a functionally intact insulin-like growth factor-I (IGF-I) receptor closely related to the insulin receptor. We investigated this possibility by examining binding and hormone-stimulated [ 14 C]glucose uptake, [ 3 H]thymidine uptake, and receptor autophosphorylation by both insulin and IGF-I in cultured fibroblasts from two affected patients. In comparison to normal fibroblasts, [ 125 I]insulin binding, insulin-stimulated [ 14 C]glucose, and [ 3 H]thymidine uptake, and insulin-stimulated autophosphorylation were each reduced by approximately 50-60% of the absolute values in controls. In contrast to expectation,
Diabetes, 1987
Studies of Caucasian and Japanese patients with insulin-dependent diabetes mellitus (IDDM) have s... more Studies of Caucasian and Japanese patients with insulin-dependent diabetes mellitus (IDDM) have shown that heterozygosity for certain HLA-DR antigens confers a high risk of developing the disease. The HLA antigens of 75 Chinese patients and 100 Chinese controls in Hong Kong were studied to investigate the role of HLA-DR heterozygosity in Chinese individuals. Some of the patients and controls were also tested for allotypic variation in the complement components C2, C4, and BF. Three alleles, Aw33, B17, and DR3, had increased frequencies in patients compared with controls and frequently occurred together in the same phenotype, which suggested their existence as a haplotype. There were no statistically significant differences in complement allotype frequencies between patients and controls, although the C4B null allele seemed to be associated with Aw33, B17, and DR3. No other HLA-DR antigen appeared to be associated with IDDM. However, when the patients were separated on the basis of age at onset, the frequency of DR3/DRw9 heterozygosity was markedly increased in patients presenting in the first decade of life, but there was no increase in patients presenting at &gt;20 yr of age. DRw9 is strongly associated with autoimmune disease in Chinese, whereas DR3 is not. We suggest that the major IDDM susceptibility locus in Chinese is associated with HLA-DR3 and that patients with HLA-DR3 and HLA-DRw9 have an added predisposition to autoimmune disease and therefore develop IDDM earlier than patients without DRw9.
Indian Journal of Pediatrics, 2005
... All the studies have demonstrated that short term GH treatment for one year resulted in signi... more ... All the studies have demonstrated that short term GH treatment for one year resulted in significant increase in the growth Indian Journal of Pediatrics, Volume 72--February, 2005 161 Page 4. Louis CK Low ... 1999 a Katzos Get al. 20004s Sartorio A 2000 Wu KH et al. 2003 .7 ...
Thorax, 2001
Myositis associated with graft-versus-hostdisease (GVHD) typically presents with proximal muscle ... more Myositis associated with graft-versus-hostdisease (GVHD) typically presents with proximal muscle weakness, myalgias, and a raised creatinine phosphokinase (CPK) level. We report a case of a 51 year old man who developed respiratory muscle weakness five years after an allogeneic bone marrow transplant for multiple myeloma. His symptoms included tachypnoea, abdominal paradox, and orthopnoea. Pulmonary function tests revealed diminished vital capacity and maximal inspiratory and expiratory pressures. Serum CPK levels were raised and a peripheral muscle biopsy specimen was consistent with GVHD. He improved with immunosuppressive therapy.
The Indian Journal of Pediatrics, 2005
Hypertransfusion and regular chelation therapy have allowed improved survival in patients with th... more Hypertransfusion and regular chelation therapy have allowed improved survival in patients with thalassemia major (TM). Despite medical advances, growth failure and hypogonadism remain significant clinical problems in these patients in adolescence. Disproportionate truncal shortening which is common especially among adolescents with thalassemia, is due to platyspondyly resulting from a combination of factors like hemosiderosis, desferrioxamine toxicity or deficiency of trace elements. Although growth hormone (GH) deficiency and GH neurosecretory dysfunction have been described in TM patients, most short TM patients have normal GH reserve. The low serum IGF-1 and IGFBP-3 concentrations in TM patients despite having normal GH reserve and serum GH binding protein levels suggest that a state of secondary GH insensitivity exists. The pubertal growth spurt may be impaired in TM patients going through spontaneous or induced puberty and may have a negative effect on final adult height. GH therapy in dosages ranging from 0.5-1.0 IU/kg/wk has resulted in a significant improvement in growth velocity in short TM children without any adverse effects on skeletal maturation, blood pressure, glucose tolerance and serum lipids. There is limited evidence that GH treatment can result in an improved final adult height in short TM children. Careful and regular clinical and biochemical monitoring should be preformed on these patients while they are treated with GH.
Zhonghua er ke za zhi. Chinese journal of pediatrics, 2005
Hutchison's Paediatrics, 2012
Hutchison's Paediatrics, 2012
The Pediatric Infectious Disease Journal, 1998
A 5-year territory-wide retrospective survey of invasive Haemophilus influenzae type b (Hib) dise... more A 5-year territory-wide retrospective survey of invasive Haemophilus influenzae type b (Hib) diseases in Hong Kong established that the annual incidence for children <5 years old was 2.7 per 100000 [95% confidence interval (CI), 2.0 to 3.5]. However, the corresponding annual incidence in Vietnamese refugees in Hong Kong was 42.7 per 100000 (95% CI 17.2 to 87.9), giving a relative risk of 18.5 (95% CI 8.3 to 41.0). The nasopharyngeal carriage rate of Hib was zero in 621 healthy Chinese children and 1.3% (95% CI 0.04 to 2.63%) in 300 healthy Vietnamese refugees 2 months to 5 years old in Hong Kong. The corresponding carriage rate of nontypable H. influenzae was 5.8% (95% CI 1.4 to 7.6%) in Chinese and 65.4% (95% CI 58.9 to 69.8) in Vietnamese. In a larger study of 1812 healthy Chinese children between 6 months and 5 years of age investigated by throat swabs, again no Hib was isolated but 141 children (7.8%) were found to be carriers of nontype b H. influenzae. In a study of 596 healthy Chinese children and adults, 25% had the protective level of anti-Hib antibody of >0.15 microg/ml by 1 year and 90% had reached >0.15 microg/ml by 6 years of age. There was some evidence that these "natural" antibodies against Hib in Hong Kong Chinese were cross-reacting antibodies against antigens on other encapsulated bacteria.
Pediatric Neurology, 1999
Insulin-dependent diabetes mellitus (IDDM) is rare in Chinese children. There have been no report... more Insulin-dependent diabetes mellitus (IDDM) is rare in Chinese children. There have been no reports on the prevalence of peripheral neuropathy in Chinese children with IDDM. This study aimed to determine prevalence of subclinical peripheral neuropathy in Chinese children with IDDM. Motor and sensory nerve conduction studies of both median, ulnar, peroneal, and tibial (motor nerves) and median, ulnar, and sural (sensory nerves) were performed in 38 children with IDDM (18 males, 20 females). The age was 4-21 years (mean ؍ 12.7 years; median ؍ 12 years, 6 months). The duration of diabetes was less than 5 years in 15, 5-10 years in 14, and more than 10 years in nine. Neurophysiologic evidence of subclinical peripheral neuropathy was present in 26 patients (68.4%) of which motor, sensory, or motor and sensory involvement was 26 (68.4%), eight (21.1%), and 26 (68.4%), respectively. Twelve (31.6%) and 14 (36.8%) children had mild and moderate degrees of peripheral neuropathy, respectively. Among the 26 children with abnormal nerve-conduction studies, two (7.7%) had symptoms of numbness and pain in the lower limbs. Thus, two children had symptomatic neuropathy and most (n ؍ 24) had asymptomatic peripheral neuropathy. Two children had systemic hypertension, and one (3.8%) had laboratory evidence of early renal complications. Analysis of demographic and laboratory risk factors for the development of subclinical peripheral neuropathy revealed that the age of onset, duration of diabetes, level of hemoglobin A1c, triglyceride, cholesterol, serum creatinine, and urea, microalbumin/ creatinine ratio, and urinary microalbumin excretion rate were significantly related to the development of subclinical peripheral neuropathy in specific nerves.
Neuroendocrinology, 1991
Growth hormone-releasing hormone (GHRH) stimulates GH secretion in man and the hormonal response ... more Growth hormone-releasing hormone (GHRH) stimulates GH secretion in man and the hormonal response is specific. The attenuation of GH response to bolus GHRH after prior exposure of GHRH of up to 24 h was not demonstrated in normal or GH-deficient subjects after more prolonged exposure. This suggests that the partial loss of responsiveness to GHRH may reflect short-term negative feedback by GH. The stimulatory effect of clonidine and L-dopa on GH release is mediated via GHRH. Other stimuli like hypoglycaemia, arginine and propranolol augment GH release in man by modulating hypothalamic somatostatin secretion. Although GHRH test can differentiate between hypothalamic or pituitary cause of GH deficiency, it is of little diagnostic value in children with short stature. Favourable results have been observed in 60-70% of GH-deficient children treated with GHRH, but the dose and mode of administration are still being explored. We found that low dose (1-2 micrograms/kg) GHRH given subcutaneously every 3 h by a pump was effective in promoting growth in 5 of 7 patients after 1 year. Treatment was continued for 2-4 years in 4 patients and growth velocities ranging from 4.5 to 8.2 cm/year were maintained using a dose of 3 micrograms/kg/pulse.
Journal of Pediatric Psychology, 2000
mary aims, both related to current gaps in the literature. The first aim was to examine risk vari... more mary aims, both related to current gaps in the literature. The first aim was to examine risk variables for emotional distress in the Hong Kong Chinese pediatric diabetes population. Much of what is known about adjustment to chronic illness in youths is based on information from Western samples. The second aim was to investigate the pathway from emotional adjustment to glycemic control. Whereas it has been documented that nonadherent youths show emotional and behavioral problems (e.g., Kovacs, Goldston, Obrosky, & Iyengar, 1992), there is less information about how
Journal of Pediatric Psychology, 2003
Objective To extend longitudinally an earlier study of the pathway from symptoms of emotional dis... more Objective To extend longitudinally an earlier study of the pathway from symptoms of emotional distress (ED) through self-efficacy (SE) and adherence to glycemic control (GC) in youths with diabetes, and to examine the contribution of different specific adherence behaviors to changes in GC. Methods Fifty-six Hong Kong youths with diabetes received a follow-up evaluation 12-24 months after initial participation. ED, SE, self-reported adherence to medical regimen (SRA), and GC were assessed at both evaluations. Results The pathway from ED to SE to SRA to GC was replicated. Participants' SRA to regular checks on blood glucose levels, and taking steps to maintain levels in the recommended range, explained significant variance in changes in GC. Conclusions The model offers strategies to enhance health care in youths with diabetes. Findings support the importance of adherence to the medical regimen but emphasize the complexity of the relationship between adherence behaviors and GC. Selfregulatory behaviors, rather than compliance with fixed instructions, appear to have the most impact on GC.
Journal of Pediatric Endocrinology and Metabolism, 1997
Journal of Paediatrics and Child Health, 2011
Aims: To review the incidence and risk factors of primary hypothyroidism in very low birthweight ... more Aims: To review the incidence and risk factors of primary hypothyroidism in very low birthweight (VLBW) infants in our centre and to assess their neurodevelopmental outcome. Method: Retrospective, descriptive review of VLBW infants with primary hypothyroidism from 1 January 2000 to 31 December 2008 in a perinatal centre. Case control comparisons for neurodevelopmental outcome at 18 months, nested to a prospective VLBW cohort (Vermont Oxford Database). Results: Twelve cases were identified, with the incidence of primary hypothyroidism of 1 in 55 live births in our VLBW cohort during the 9-year study period. Umbilical cord blood thyroid-stimulating hormone was abnormal in less than half of the cases using the current cutoff (Յ14 mIU/L). Five cases were transient in nature, four cases were permanent and the causes for the rest remained undetermined at the time of review. Elevation of thyroid-stimulating hormone was first evident at a mean of 2.4 weeks post-natally. Follow-up assessment reviewed normal development in 83% of cases (10 out of 12 cases). There was no statistically significant difference in Griffith's scores at 18 months between the case and matched controls. Conclusions: The high incidence of primary hypothyroidism in our VLBW cohort deserved stringent monitoring of thyroid function postnatally. Umbilical cord blood screening was not useful as a screening tool because of its low sensitivity. Neurodevelopmental outcome for treated primary hypothyroidism was favourable as assessed at 18 months of age.
Journal of Paediatrics and Child Health, 1992
The role of transplacental transfer of maternal thyrotrophin (TSH)-blocking antibodies causing co... more The role of transplacental transfer of maternal thyrotrophin (TSH)-blocking antibodies causing congenital hypothyroidism in Southern Chinese children was examined in this study. Twenty-two mothers of 24 patients with congenital hypothyroidism were studied 3-5 years after delivery. None of them had thyroid dysfunction at delivery or at the time of study. None had antithyroglobulin or antimicrosomal antibody. Only one mother was found to have TSH-binding inhibitory immunoglobulin (TBII), and her child had agenesis of the thyroid. This women had Graves disease in remission for 2 years before delivery. None had TSH-stimulated cAMP response inhibitory immunoglobulin (TSII). Ten of the 24 congenital hypothyroid children had transient neonatal hypothyroidism, seven had agenesis of the thyroid, six had dyshorrnonogenesis and one had a sublingual thyroid. As none of the mothers who had children with transient neonatal hypothyroidism had blocking antibodies at the time of study, the aetiology of the transient neonatal hypothyroidism remains unclear. These data suggest that maternal TSH-blocking antibodies do not play a role in most cases of sporadic congenital hypothyroidism.
Journal of Paediatrics and Child Health, 1989
Journal of Paediatrics and Child Health, 1987
A boy aged 7.5 years presented with precocious onset of secondary sexual characteristics and symp... more A boy aged 7.5 years presented with precocious onset of secondary sexual characteristics and symptoms and signs of neurological dysfunction. A CT brain scan demonstrated a suprasellar mass lesion. The elevated P-hCG and a-fetoprotein concentrations in cerebrospinal fluid and in serum were consistent with a diagnosis of intracranial embryonal carcinoma, although histological diagnosis was not available. The tumour markers P-hCG and a-FP should be looked for in all children with precocious puberty presenting with midline brain tumours.
The Journal of Clinical Endocrinology & Metabolism, 1989
A subset of patients with the syndrome of acanthosis nigricans and insulin resistance type A is c... more A subset of patients with the syndrome of acanthosis nigricans and insulin resistance type A is characterized by acromegaloid features in addition to hyperinsulinemia, hyperandrogenemia, and an inherent defect in insulin receptor function. It has been proposed that the acromegaloid features result from the interaction of insulin at concentrations encountered in vivo, with a functionally intact insulin-like growth factor-I (IGF-I) receptor closely related to the insulin receptor. We investigated this possibility by examining binding and hormone-stimulated [ 14 C]glucose uptake, [ 3 H]thymidine uptake, and receptor autophosphorylation by both insulin and IGF-I in cultured fibroblasts from two affected patients. In comparison to normal fibroblasts, [ 125 I]insulin binding, insulin-stimulated [ 14 C]glucose, and [ 3 H]thymidine uptake, and insulin-stimulated autophosphorylation were each reduced by approximately 50-60% of the absolute values in controls. In contrast to expectation,