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1.
Bone Marrow Transplant ; 48(1): 74-9, 2013 Jan.
Article in English | MEDLINE | ID: mdl-22635245

ABSTRACT

To clarify the clinical features and outcome of Stenotrophomonas maltophilia infection among hematopoietic SCT (HCT) recipients, we retrospectively reviewed the records of 1085 consecutive HCT recipients and identified 42 episodes in 31 HCT recipients with S. maltophilia infection. We compared these recipients with 30 non-HCT patients with S. maltophilia infection. The mortality rate in HCT recipients was significantly higher than that in non-HCT patients (relative risk 5.7, P=0.04), and we identified seven patients with pulmonary hemorrhage due to S. maltophilia, exclusively in the HCT cohort. Six of these latter seven patients died within 1 day from the onset of hemorrhage and the isolate was identified after death in most cases; one patient, who received empiric therapy for S. maltophilia and granulocyte transfusion, survived for more than 2 weeks. The patients with pulmonary hemorrhage had a more severe and longer duration of neutropenia, persistent fever despite of the use of broad-spectrum antibiotics, complication by pneumonia and higher C-reactive protein levels than those without pulmonary hemorrhage. In conclusion, S. maltophilia was associated with fulminant and fatal pulmonary hemorrhage in HCT recipients. Empiric therapy with antibiotics before the onset of pulmonary hemorrhage may be effective in HCT recipients who carry the conditions identified.


Subject(s)
Gram-Negative Bacterial Infections/physiopathology , Hematopoietic Stem Cell Transplantation/adverse effects , Hemorrhage/etiology , Immunocompromised Host , Lung Diseases/etiology , Pneumonia, Bacterial/physiopathology , Stenotrophomonas maltophilia/immunology , Adolescent , Adult , Anti-Bacterial Agents/therapeutic use , Child , Child, Preschool , Cohort Studies , Drug Resistance, Multiple, Bacterial , Female , Gram-Negative Bacterial Infections/immunology , Gram-Negative Bacterial Infections/microbiology , Gram-Negative Bacterial Infections/therapy , Hemorrhage/epidemiology , Hemorrhage/mortality , Humans , Immunocompromised Host/drug effects , Incidence , Japan/epidemiology , Lung Diseases/epidemiology , Lung Diseases/mortality , Male , Middle Aged , Neutropenia/epidemiology , Neutropenia/etiology , Neutropenia/physiopathology , Pneumonia, Bacterial/immunology , Pneumonia, Bacterial/microbiology , Pneumonia, Bacterial/therapy , Retrospective Studies , Severity of Illness Index , Stenotrophomonas maltophilia/drug effects , Stenotrophomonas maltophilia/isolation & purification , Young Adult
2.
Rev Sci Instrum ; 83(10): 10E529, 2012 Oct.
Article in English | MEDLINE | ID: mdl-23127036

ABSTRACT

Developments in measuring sound velocity of matter under ultrahigh pressure are described. We employed a time-resolved x-ray shadowgraph technique to measure the sound velocity of shock-compressed diamond and iron foils at around melt. The sound velocity significantly dropped at melting, a behavior that has been difficult to clarify by conventional measurements by subtracting particle velocity from shock velocity (u(s) - u(p)). In addition to sound velocity, other important parameters were also obtained simultaneously.

3.
J Dent Res ; 91(7 Suppl): 29S-37S, 2012 Jul.
Article in English | MEDLINE | ID: mdl-22699664

ABSTRACT

Described for the first time in 1971, Schimke immuno-osseous dysplasia (SIOD) is an autosomal-recessive multisystem disorder that is caused by bi-allelic mutations of SMARCAL1, which encodes a DNA annealing helicase. To define better the dental anomalies of SIOD, we reviewed the records from SIOD patients with identified bi-allelic SMARCAL1 mutations, and we found that 66.0% had microdontia, hypodontia, or malformed deciduous and permanent molars. Immunohistochemical analyses showed expression of SMARCAL1 in all developing teeth, raising the possibility that the malformations are cell-autonomous consequences of SMARCAL1 deficiency. We also found that stimulation of cultured skin fibroblasts from SIOD patients with the tooth morphogens WNT3A, BMP4, and TGFß1 identified altered transcriptional responses, raising the hypothesis that the dental malformations arise in part from altered responses to developmental morphogens. To the best of our knowledge, this is the first systematic study of the dental anomalies associated with SIOD.


Subject(s)
Arteriosclerosis/complications , Immunologic Deficiency Syndromes/complications , Nephrotic Syndrome/complications , Osteochondrodysplasias/complications , Pulmonary Embolism/complications , Tooth Abnormalities/etiology , Alleles , Anodontia/etiology , Arteriosclerosis/genetics , Bicuspid/abnormalities , Bone Morphogenetic Protein 4/analysis , Cell Culture Techniques , Cell Proliferation , Cell Survival , Cells, Cultured , DNA Helicases/analysis , DNA Helicases/genetics , Fibroblasts/pathology , Humans , Immunologic Deficiency Syndromes/genetics , Molar/abnormalities , Mutation/genetics , Nephrotic Syndrome/genetics , Odontogenesis/genetics , Osteochondrodysplasias/genetics , Primary Immunodeficiency Diseases , Pulmonary Embolism/genetics , Skin/cytology , Tooth Germ/pathology , Tooth Root/abnormalities , Tooth, Deciduous/abnormalities , Transcription, Genetic/genetics , Transforming Growth Factor beta1/analysis , Wnt3A Protein/analysis
5.
Acta Anaesthesiol Scand ; 53(4): 476-82, 2009 Apr.
Article in English | MEDLINE | ID: mdl-19226297

ABSTRACT

BACKGROUND: Although many investigators previously reported the sympathovagal effect of spinal anaesthesia, there is no information about the sympathovagal effects of supplementation with fentanyl. The aim of this study was to evaluate the sympathovagal effects of intrathecal or intravenous fentanyl added to spinal anaesthesia. METHODS: One hundred and twenty patients undergoing elective transurethral surgery under spinal anaesthesia were randomly allocated to receive intrathecally either isobaric bupivacaine alone (Group B), bupivacaine supplemented with intrathecal (Group Ft) or with intravenous fentanyl (Group Fv). Heart rate variability was estimated using the MemCalc method (Tarawa, Suwa Trust, Japan) before and after spinal anaesthesia. RESULTS: In all groups, spinal anaesthesia significantly decreased low frequency/high frequency (LF/HF) as a marker of sympathovagal balance. However, patients in Group B with a low block height developed a marked increase in LF/HF after spinal anaesthesia, which was attenuated in Group Ft. Meanwhile, intravenous fentanyl did not attenuate this response. CONCLUSION: We conclude that sympathetic activation observed in patients with a low block height was attenuated by intrathecal fentanyl but not by intravenous fentanyl.


Subject(s)
Analgesics, Opioid/pharmacology , Anesthesia, Spinal , Fentanyl/pharmacology , Heart Rate/drug effects , Sympathetic Nervous System/drug effects , Vagus Nerve/drug effects , Adult , Aged , Electroencephalography/drug effects , Female , Fentanyl/administration & dosage , Humans , Injections, Intravenous , Injections, Spinal , Male , Middle Aged
6.
AJNR Am J Neuroradiol ; 30(3): 629-34, 2009 Mar.
Article in English | MEDLINE | ID: mdl-19112063

ABSTRACT

BACKGROUND AND PURPOSE: The causative gene of the common congenital malformation referred to as CHARGE syndrome is CHD7. Affected individuals often undergo head and neck imaging to assess abnormalities of the olfactory structures, hypothalamus-pituitary axis, and inner ear. We encountered a few children with severe hypoplasia of the basiocciput during a radiologic assessment of patients with CHARGE syndrome. To our knowledge, this anomaly has not been reported. Our purpose was to evaluate the incidence and severity of this anomaly in this syndrome. MATERIALS AND METHODS: Sagittal MR images of 8 patients with CHARGE syndrome were retrospectively reviewed by 2 radiologists who consensually evaluated the status of the basiocciput of the patients with CHARGE syndrome, as either normal or hypoplastic; and associated anomalies, which include basilar invagination, Chiari type I malformation, and syringomyelia, as either present or absent. The length between the basion (Ba) and the endo-sphenobasion (Es) and between the basion and the exo-sphenobasion (Xs) was measured on midsagittal MR images of the 8 patients and 70 age-matched controls. We searched for trends related to age in the length of Ba-Es and Ba-Xs of the control children by using a matched t test. RESULTS: Basioccipital hypoplasia was identified in 7 of the 8 patients with CHARGE syndrome and was severe in 6. Of those, 5 had associated basilar invagination and 1 had Chiari type I malformation with syringomyelia. CONCLUSIONS: Basioccipital hypoplasia and basilar invagination are prevalent in patients with CHARGE syndrome.


Subject(s)
Abnormalities, Multiple/pathology , Cranial Fossa, Posterior/abnormalities , Sphenoid Bone/abnormalities , Abnormalities, Multiple/epidemiology , Abnormalities, Multiple/genetics , Adult , Child , Child, Preschool , Choanal Atresia/epidemiology , Choanal Atresia/pathology , Coloboma/epidemiology , Coloboma/pathology , DNA Helicases/genetics , DNA-Binding Proteins/genetics , Ear, Inner/abnormalities , Growth Disorders/pathology , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/pathology , Humans , Hypothalamo-Hypophyseal System/abnormalities , Incidence , Olfactory Bulb/abnormalities , Retrospective Studies , Severity of Illness Index , Syndrome
9.
Heart ; 94(4): 429-33, 2008 Apr.
Article in English | MEDLINE | ID: mdl-17646194

ABSTRACT

OBJECTIVES: To evaluate the in-vivo plaque composition and characteristics in patients with type 2 diabetes mellitus (DM) using Virtual Histology intravascular ultrasound (VH IVUS). METHODS: In 90 patients with stable angina pectoris, de novo target vessels were studied and plaque components were analysed. Patients were divided into two groups: a diabetic group (36 vessels) and a non-diabetic group (54 vessels). RESULTS: The percentage area of necrotic core and dense calcium were significantly larger in the DM group than the non-DM group (necrotic core: 11.0% (interquartile range (IQR): 7.2-15.2%) vs 7.6% (IQR 5.6-13.2%), p = 0.03; dense calcium: 5.6% (IQR: 2.3-7.3%) vs 2.9% (IQR: 1.7-4.9%), p = 0.01). The DM group presented with a significantly higher presence of at least one VH IVUS-derived thin-cap fibroatheroma (VHD-TCFA) (75% vs 41%, p = 0.001) and VH IVUS-derived fibrocalcific atheroma (VHD-FCA) (75% vs 40%, p = 0.001). In the DM group, 53% of the vessels had both VHD-TCFA and VHD-FCA, which was significantly higher than non-DM group (17%, p = 0.0004). CONCLUSIONS: Coronary plaque characteristics in DM patients showed an increased amount of dense calcium and necrotic core, as well as a higher frequency of VHD-TCFA and VHD-FCA. Atherosclerosis of the target vessel was more advanced in diabetic patients.


Subject(s)
Coronary Artery Disease/pathology , Diabetes Mellitus, Type 2/pathology , Diabetic Angiopathies/pathology , Adult , Aged , Aged, 80 and over , Calcinosis/diagnostic imaging , Calcinosis/pathology , Coronary Artery Disease/diagnostic imaging , Coronary Vessels/pathology , Diabetes Mellitus, Type 2/diagnostic imaging , Diabetic Angiopathies/diagnostic imaging , Female , Humans , Male , Middle Aged , Necrosis , Ultrasonography, Interventional/methods
11.
Acta Anaesthesiol Scand ; 51(9): 1161-5, 2007 Oct.
Article in English | MEDLINE | ID: mdl-17714571

ABSTRACT

BACKGROUND: The objective of this study was to determine whether pre-operative heart rate variability (HRV) estimated using the newly developed MemCalc method predicts hypotension after spinal anaesthesia (SA). METHOD: Fifty-two patients with American Society of Anesthesiologists physical status I or II, aged 38-84 years, scheduled to undergo elective transurethral surgery were investigated in this study. Ultra short-term entropy (UsEn) as a non-linear index of HRV was estimated before and after SA using the MemCalc method. The patients were then assigned to two groups (Group LO and HI) according to pre-operative UsEn. SA was performed by intrathecal injection of 17.5 mg of isobaric bupivacaine via interlumbar spaces L3-4 or L4-5. After SA, the haemodynamic fluctuations were recorded and compared between the two groups. RESULTS: SA significantly decreased LF/HF (a ratio of the low frequency component of HRV to that of the high frequency component) but did not affect UsEn. The percentage decrease in systolic blood pressure (SBP) after SA was 11.4 (9.9)% in group HI, whereas it was 17.5 (9.8)% in group LO (P < 0.05). Furthermore, the number of patients who developed hypotension was significantly higher in group LO. CONCLUSION: SA significantly decreased LF/HF without affecting UsEn. Patients with a low UsEn developed a greater decrease in SBP after SA. Furthermore, the incidence of hypotension after SA was higher in patients with a low UsEn.


Subject(s)
Anesthesia, Spinal , Heart Rate , Hypotension/diagnosis , Adult , Aged , Aged, 80 and over , Chi-Square Distribution , Cohort Studies , Electrocardiography/methods , Entropy , Female , Humans , Male , Middle Aged , Signal Processing, Computer-Assisted
12.
Anaesthesia ; 62(2): 117-21, 2007 Feb.
Article in English | MEDLINE | ID: mdl-17223801

ABSTRACT

The objective of this study was to compare linear and non-linear analysis of heart rate variability (HRV) in terms of correlation with haemodynamic fluctuation during induction of general anaesthesia. Pre-operatively, HRV was estimated by the MemCalc method in 114 patients scheduled for general anaesthesia. After anaesthesia was induced with propofol, fentanyl and vecuronium bromide, tracheal intubation was performed. Haemodynamic fluctuations during induction of anaesthesia were recorded and the correlation between pre-operative HRV and haemodynamic fluctuation was examined using logistic regression analysis. As an index of non-linear analysis of HRV, ultra short-term entropy (UsEn) correlated better with blood pressure fluctuation than did the ratio of the power of low frequency component of HRV to that of high frequency component (LF/HF). In contrast, although LF/HF significantly correlated with heart rate increase caused by tracheal intubation, the correlation between UsEn and heart rate fluctuation was not significant.


Subject(s)
Anesthetics, General/pharmacology , Heart Rate/drug effects , Signal Processing, Computer-Assisted , Adult , Aged , Aged, 80 and over , Anesthesia, General , Blood Pressure/drug effects , Body Constitution , Electrocardiography/methods , Entropy , Female , Humans , Intubation, Intratracheal , Male , Middle Aged
15.
Acta Anaesthesiol Scand ; 50(4): 509-11, 2006 Apr.
Article in English | MEDLINE | ID: mdl-16548866

ABSTRACT

Heart rate variability (HRV) was investigated using a new technique for time series analysis combining the maximum entropy method and non-linear least squares method -- the 'MemCalc method' -- in patients undergoing general anesthesia with sevoflurane and fentanyl for elective surgery. As the occurrence of junctional rhythm coincided with the measurement of these variables in two patients, we successfully evaluated the entropy, low (LF) and high (HF) frequency component of the HRV during junctional rhythm and found that the occurrence of junctional rhythm is associated with marked decreases in the entropy, LF and HF of HRV. When evaluating autonomic control of the heart using HRV analysis, the decrease in HRV caused by the occurrence of junctional rhythm must be taken into account.


Subject(s)
Anesthesia, General/adverse effects , Anesthetics, Combined/administration & dosage , Anesthetics, Inhalation/administration & dosage , Anesthetics, Intravenous/administration & dosage , Arrhythmias, Cardiac/physiopathology , Fentanyl/administration & dosage , Heart Rate , Methyl Ethers/administration & dosage , Adult , Arrhythmias, Cardiac/etiology , Female , Humans , Middle Aged , Sevoflurane
16.
Br J Nutr ; 94(5): 691-7, 2005 Nov.
Article in English | MEDLINE | ID: mdl-16277770

ABSTRACT

The effects of two kinds of Escherichia coli (E. coli) strain, wild-type E. coli W3110 and E. coli nir-Ptac, which has enhanced NO(2) reduction activity, on oral CH(4) emission and NO(3) toxicity in NO(3)-treated sheep were assessed in a respiratory hood system in a 4 x 6 Youden square design. NO(3) (1.3 g NaNO(3)/kg(0.75) body weight) and/or E. coli strains were delivered into the rumen through a fistula as a single dose 30 min after the morning meal. Escherichia coli cells were inoculated for sheep to provide an initial E. coli cell density of optical density at 660 nm of 2, which corresponded to 2 x 10(10) cells/ml. The six treatments consisted of saline, E. coli W3110, E. coli nir-Ptac, NO(3), NO(3) plus E. coli W3110, and NO(3) plus E. coli nir-Ptac. CH(4) emission from sheep was reduced by the inoculation of E. coli W3110 or E. coli nir-Ptac by 6 % and 12 %, respectively. NO(3) markedly inhibited CH(4) emission from sheep. Compared with sheep given NO(3) alone, the inoculation of E. coli W3110 to NO(3)-infused sheep lessened ruminal and plasma toxic NO(2) accumulation and blood methaemoglobin production, while keeping ruminal methanogenesis low. Ruminal and plasma toxic NO(2) accumulation and blood methaemoglobin production in sheep were unaffected by the inoculation of E. coli nir-Ptac. These results suggest that ruminal methanogenesis may be reduced by the inoculation of E. coli W3110 or E. coli nir-Ptac. The inoculation of E. coli W3110 may abate NO(3) toxicity when NO(3) is used to inhibit CH(4) emission from ruminants.


Subject(s)
Escherichia coli/physiology , Methane/metabolism , Nitrate Reductase/metabolism , Nitrates/adverse effects , Rumen/microbiology , Animals , Carbon Dioxide/metabolism , Escherichia coli/enzymology , Escherichia coli/genetics , Fermentation , Infusions, Parenteral/methods , Male , Metabolic Clearance Rate , Methemoglobin/analysis , Nitrates/administration & dosage , Nitrates/pharmacokinetics , Nitrites/blood , Nitrites/pharmacokinetics , Oxygen Consumption/physiology , Sheep
17.
Aliment Pharmacol Ther ; 21 Suppl 2: 60-6, 2005 Jun.
Article in English | MEDLINE | ID: mdl-15943849

ABSTRACT

BACKGROUND: The incidence and severity of non-steroidal anti-inflammatory drugs (NSAIDs)-induced gastro-duodenal ulcer have not been extensively studied in Japan. AIM: We performed a prospective study to clarify NSAIDs-induced gastro-duodenal injury, focusing especially on low-dose aspirin (L-A). METHODS: Two hundred and thirty-eight patients with bleeding peptic ulcers admitted to our hospital. History of taking NSAIDs and anti-ulcer drugs was obtained from all patients who underwent endoscopic examinations. The lesion scores of patients taking L-A were classified numerically from zero (no lesion) to five (ulcer). RESULTS: The NSAIDs were associated with 28.2% of hemorrhagic ulcers. The rates of patients using L-A, loxoprofen, diclofenac, and combination of two of these drugs were 27, 16, 10 and 9%, respectively. Co-administered anti-ulcer drugs were cytoprotective anti-ulcer drugs (27%), H2 receptor antagonists (16%), PPI (4%), and none (53%). In patients taking L-A, H2 receptor antagonists were used most frequently. The HP was positive in 63% of L-A-induced ulcer cases and in 69% of NSAIDs other than low-dose aspirin-induced ulcer cases. The lesion scores of patients taking L-A with H2 receptor antagonists or PPI were significantly lower than those of patients who were taking only L-A (P < 0.05). CONCLUSIONS: Approximately one-third of hospitalized patients with NSAIDs-induced hemorrhagic ulcer showed an association with L-A. Prospective randomized controlled trials including H2 receptor antagonists are required to establish preventive efforts aimed at L-A-induced gastro-duodenal injury.


Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Anti-Ulcer Agents/therapeutic use , Aspirin/adverse effects , Histamine H2 Antagonists/therapeutic use , Peptic Ulcer Hemorrhage/prevention & control , Adult , Aged , Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Aspirin/administration & dosage , Endoscopy, Gastrointestinal , Female , Helicobacter Infections/complications , Helicobacter pylori , Hemostasis, Endoscopic , Humans , Male , Middle Aged , Peptic Ulcer Hemorrhage/chemically induced , Prospective Studies , Recurrence
18.
J Anim Sci ; 83(3): 644-52, 2005 Mar.
Article in English | MEDLINE | ID: mdl-15705761

ABSTRACT

The effects of two kinds of Escherichia coli strains, wild-type E. coli W3110 or E. coli nir-Ptac, which has enhanced nitrite reduction activity, on in vitro CH4 production and nitrate and nitrite reduction in cultures of mixed ruminal microorganisms was investigated using continuous incubation systems. Escherichia coli nir-Ptac, a derivative of wild-type E. coli W3110, was constructed by replacing self promoter of nir BD operon encoding subunits of nitrite reductase in E. coli W3110 by tac promoter to make the expression of nir BD higher and constitutive. The nitrite reductase activity of E. coli nir-Ptac was approximately twice as high as E. coli W3110. The culture media consisted of 400 mL of strained ruminal fluid taken from two nonlactating Holstein cows receiving a basal diet of orchardgrass hay at maintenance level (55 g of DM/kg of BW0.75 daily), and 400 mL of autoclaved artificial saliva. Treatments were arranged in two separate 3 x 3 factorials consisting of nitrate (NaNO3; 0, 5, or 10 mM) without E. coli or inoculated with E. coli W3110 or E. coli nir-Ptac, or nitrite (NaNO2; 0, 1 or 2 mM) without E. coli or inoculated with E. coli W3110 or E. coli nir-Ptac. The control culture contained no chemical or microbial additives. Escherichia coli cells were inoculated into in vitro mixed ruminal cultures at approximately 2 x 10(8) to 10(9) cells/mL. Methane production by ruminal microorganisms was decreased markedly (P < 0.001) by the addition of nitrate and nitrite, and by the inoculation of cultures with E. coli W3110 or E. coli nir-Ptac (P < 0.01). With mixed nitrite-containing cultures, E. coli nir-Ptac inhibited (P < 0.001) in vitro nitrite accumulation and CH4 production more than E. coli W3110, which may be due to the tac promoter-enhanced nitrite reductase activity of E. coli nir-Ptac accelerating electrons to be consumed for nitrite reduction rather than CH4 biosynthesis. In conclusion, anaerobic cultures of E. coli W3110 or E. coli nir-Ptac may decrease CH4 production in the rumen. The inoculation of E. coli W3110 or, especially, E. coli nir-Ptac to mixed ruminal microorganisms may decrease nitrite toxicity when ruminants consume high-nitrate-containing forages and when nitrite is applied to abate ruminal CH4 production.


Subject(s)
Escherichia coli/enzymology , Methane/biosynthesis , Nitrates/metabolism , Nitrite Reductases/metabolism , Nitrites/metabolism , Rumen/metabolism , Animals , Cattle , DNA Primers/chemistry , DNA, Recombinant , Female , Fermentation/drug effects , In Vitro Techniques , Methane/analysis , Nitrates/pharmacology , Nitrites/pharmacology , Organisms, Genetically Modified/metabolism , Polymerase Chain Reaction/veterinary , Random Allocation , Rumen/microbiology , Time Factors
19.
Conf Proc IEEE Eng Med Biol Soc ; 2005: 6908-11, 2005.
Article in English | MEDLINE | ID: mdl-17281863

ABSTRACT

This paper describes an efficient system for transmitting a few words (verbal information) to a prosthetic hand user by using a two-channel electrotactile stimulation capable of generating apparent movements, which is applied to the upper arm area of the left hand via surface concentric electrodes. If the prosthetic hand user gets verbal information about interaction between the prosthesis and the environment, e.g., the prosthetic hand just touched a doorknob, he/she will be able to open or close the door even in the darkness, and also to handle an object on the top of a shelf out of his/her view. In this study, verbal information was provided regarding which finger touches the object (three states), and the fingers angle (two states). Consequently, six states of verbal information could be sent. We therefore designed five stimulation patterns (duration 600 - 800 ms) comprising several burst profiles in which each burst was composed of several pulses (interval of biphasic pulses, 2.85 ms) modulated in amplitude. The recognition experiments using 10 healthy subjects showed that the mean percentage of recognition was about 94% (standard deviation: 3.9). These results suggested the possible applicability of the proposed system to assist prosthetic hand users.

20.
Acta Paediatr ; 92(6): 698-703, 2003 Jun.
Article in English | MEDLINE | ID: mdl-12856981

ABSTRACT

AIM: To investigate the final adult heights and pubertal growth patterns in Japanese patients with congenital hypothyroidism (CH) detected by neonatal screening. METHODS: A retrospective chart review was conducted of female patients >15 y of age (n = 18) and male patients >18 y of age (n = 9), who were detected by neonatal screening and kept on continuous thyroid hormone replacement therapy. Final height standard deviation scores (FHSDS) and target height standard deviation scores (THSDS) were determined. Parameters characterizing the pubertal growth process (such as age at onset of pubertal growth spurt and age at peak pubertal growth) were obtained from each patient's growth rate chart. Menarchial age was determined in each female patient by reviewing the medical record. The impact on FHSDS of the etiology of CH, the severity of CH, the time of initiation of therapy and the adequacy of treatment during the first year of life was assessed. RESULTS: All patients had received initial thyroid hormone treatment no later than 50 d of age, and had reached their final height. The mean FHSDS for female and male patients were +0.17 +/- 0.99 and -0.03 +/- 0.99, respectively. The mean FHSDS-THSDS for female and male patients was +0.09 +/- 0.77 and -0.19 +/- 0.53, respectively. No difference was seen in pubertal growth parameters for either gender compared with that of the reference population, except for a greater peak height velocity and pubertal height gain in male patients. The mean menarchial age was identical to that of the reference population. No significant relationship was found between the FHSDS and any of the factors investigated. CONCLUSION: The adult height of patients with CH detected by neonatal screening was equivalent to that of the reference population and their target height. As long as early intervention and satisfactory management are ensured, severe CH does not appear to reduce final adult height.


Subject(s)
Body Height , Growth , Hypothyroidism , Adolescent , Adult , Congenital Hypothyroidism , Female , Gestational Age , Humans , Hypothyroidism/diagnosis , Infant, Newborn , Japan , Male , Neonatal Screening , Retrospective Studies
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