Coverage of the Severely Traumatized Extremity by Emergency Free Flap: Case Report

Emergency free flap has been advocated to cover the severely injured extremity for more than two decades, due to its numerous advantages such as low incidence of flap failure and infection rate and early recovery of function. But there are very few reports about these. The authors report their experience in using the emergency free flap for reconstruction of extremities. For last 10 years, 4 patients ranging from 3 to 27 years old with severely traumatized extremities were treated with emergency free flap transfers. Three were males and the other was a female. Flap size ranged from 2x5 cm2 to 7x22 cm2. The locations of the recipient site were the dorsum of the foot, the cubital fossa, the popliteal fossa and the upper arm. The number of the donor sites used was as follows: one scapular flap, two parascapular flaps, and one radial forearm flap with the radial bone. All of the flaps survived without need of re-exploration. There was no infection or flap loss. Involved joints have recovered a normal range of motion. Therefore, we consider that the emergency free flap is a very safe and reliable method to cover the severely injured extremities.

Adequate Serial Monolayer Passage Number of Human Intervertebral Disc Cells for Cell Therapy: Growth and Phenotype of Cells

STUDY DESIGN: This is an in-vitro experimental study. OBJECTIVES: We wanted to analyze the changes in the growth and phenotype of human degenerative intervertebral disc cells depending on the frequency of subculture in an in vitro monolayer culture system. SUMMARY OF THE LITERATURE REVIEW: A subculture of disc cells is needed to obtain an adequate amount of disc cells for cell therapy, tissue engineering and analysis of the biological characteristics of degenerative disc cells. MATERIALS AND METHODS: The obtained intervertebral discs were divided into the nucleus pulposus (NP) and the annulus fibrosus (AF). The AF and NP cells were cultured in a monolayer manner, respectively. At each subculture time, we analyzed the morphological changes, the adhesion rate, the proliferation rate and the viability. The expressions of types I and II collagen and proteoglycan were analyzed at the mRNA gene level. RESULTS: Both the AF and NP cells gradually showed a fibroblast-like spindle shape while undergoing subculture. The adhesion rate was higher at the second and third times of subculture. The cell proliferation was the highest at the second subculture time. The viability was markedly lower prior to the subculture. On RT-PCR, the type II collagen expression was gradually decreased in the NP cells. In the AF cells, Type II collagen was not expressed from the second time of subculture. The expression of proteoglycan was gradually decreased in both. CONCLUSIONS: Following the 3rd subculture, the degenerative disc cells had completely changed their original growth and phenotypic characteristics. Therefore, we believe that it is not desirable for us to do passage cultures more than three times for cell therapy.

Assessment of Chromosomal Analyses of 1,180 Cases Suspected of Chromosomal Aberrations

PURPOSE: We have performed this study to obtain reference data for the distribution of chromosomal aberrations in Korea. METHODS: We analyzed 1,180 chromosomal study cases from Kwang ju Christian Hospital during the past 25 years. 756 cases suspected of characteristic chromosomal aberration syndromes and 424 cases with hermaphroditism, mild sexual abnormalities, multiple anomalies, or mental and growth retardation were included. RESULTS: The male to female ratio of autosomal aberration syndromes was 1.2 : 1. 78.6% of autosomal aberrations were diagnosed under 1 year of age, whereas 89.8% of sex chromosomal aberrations were diagnosed over 12 years of age. Among 1,180 cases, 612 ones had chromosomal aberrations(51.9%) : 590 of 756 cases suspected of chromosomal aberration syndromes had aberrations(78.0%), whereas 22 of 424 showing the above other features had aberrations(5.2%). Autosomal aberrations appeared in 514 cases(83.8%) and sex chromosomal aberrations appeared in 98 cases(16.2%). The most frequently observed abberation in autosomal aberrations was Down syndrome, followed by E, D, B, A and C group aberrations. The most common abberation in sex chromosomal aberrations was Turner syndrome, followed by Klinefelter syndrome and Fragile X syndrome. CONCLUSION: It is of vital importance that patients suspected of chromosomal aberrations undergo chromosomal analysis. Further advanced chromosomal staining and molecular genetic methods will raise the detection rate of chromosomal aberrations.

The Prognosis and Factors of Neonatal Thyroid Dysfunction

PURPOSE: The function of the neonatal thyroid is affected transiently or consistently by various maternal thyroid diseases, neonatal diseases, drugs, and more as well as thyroid dysgenesis. In this study, we analyzed several factors of neonatal thyroid dysfunction through neonatal screening test and investigated the prognosis. METHODS: From January 1992 to July 1997, we reviewed 66 newborn patients, who showed abnormal thyroid function in neonatal screening test, and analyzed 62 cases except for 3 cases of thyroid dysgenesis and 1 case of TBG deficiency. In the end, we statistically compared the maternal thyroid disease group with umbilical-iodinated disinfection group because the other groups were too small in numbers. RESULTS: Among 62 cases of neonatal thyroid dysfunction, maternal thyroid disease (A) group had 29 cases (46.8%), umbilical-iodinated disinfection (B) group had 22 cases (35.5%), neonatal disease (C) group had 7 cases (11.2%), idiopathic (D) group had 4 cases (6.4%). Group A showed compensated hypothyroidism 79.3%, transient hypothyroidism 13.8% and hyperthyroidism 6.9%, while group B showed compensated hypothyroidism 77.3% and transient hypothyroidism 22.7%. Capillary TSH showed that group B was significantly higher than group A (P<0.05). The serum T4 showed that group B was significantly lower than group A (P<0.05). The incidence of medication showed no significant difference between groups A and B. The duration of normalizing thyroid function showed that group B was significantly longer than group A. CONCLUSOIN: The iodide-containing disinfectant caused transient thyroid dysfunction which was longer and more severe than in the maternal thyroid disease group even though the time of causative exposure differed.

Survey of Harada Scoring of Occurrence of Coronary Aneurysm in Kawasaki Disease Compared to Current Criteria

PURPOSE: Gamma-globulin is effective in preventing coronary aneurysm, the primary complication of Kawasaki disease(KD). However, in order to predict high-risk cases which absolutely require gamma-globulin, because of its high expenses, Harada score(HS) was introduced in Japan in 1990. We attempted to compare HS scoring with the health insurance criteria currently used in Korea. METHODS: Retrospective studies were performed on 123 cases who did not receive gamma-globulin among 283 KD patients who had been treated in this hospital from 1990 to 1997. Chi-square analyses were used. RESULTS: Of the 123 subjects, 91(74%) had HS scores higher than 4, and 32(26%) had HS less than 4, whereas 56(45.5%) belonged to the high-risk group and 67(54.5%) to the low-risk group according to the insurance criteria, with both methods differing significantly(P<0.001). Of 18 cases with the coronary complications only 2 cases had an HS<4, whereas 4 patients belonged to the low-risk group. Even though both methods did not differ significantly in discriminating risky cases, twice as many patients may have been denied adequate treatment if the insurance criteria had been applied. Of various factors, the age and albumin level were found relevant to the coronary complication. CONCLUSION: No statistically significant difference was found between HS and the insurance criteria in predicting the incidence of coronary complications in 123 KD patients. But a less lenient criteria used in the current health insurance policy may result in more cases with coronary complications.

A Case of Band Heterotopia with Lennox-Gastaut Syndrome

Band heterotopia is a rare neuronal migration disorder, resulting in epilepsy and mental retardation. Epilepsy in band heteropopia, of which Lennox-Gastaut syndrome constituted about 20%, varied in nature and degree of severity. Band heterotopia can be diagnosed by brain magnetic resonance imaging (MRI), showing another diffuse layer of gray matter underlying the normal-looking cortex with intervening thin rim of white matter. While positron emission tomography (PET) with [18F]-fluorodeoxyglucose revealed glucose uptake similar to the overlying cortex, single photon emission computerized tomography (SPECT) findings of band heterotopia have not been reported. We report a 8-year-old girl who presented with variable types of generalized seizures and mild mental retardation. She was diagnosed as having band heterotopia with Lennox-Gastaut syndrome by MRI and interictal electroencephalogram (EEG) showing immature background and generalized 2 Hz slow spike and wave complexes. Interictal SPECT, using Tc 99m hexamethyl propylenamine oxime (Tc 99m-HMPAO), revealed the same degree of perfusion in both the areas of band heterotopia and the overlying cortex. By using valproate and lamotrigine, she is now in stable condition with a significant decrease in seizure frequency.

A Case of Cavernous Sinus Thrombosis due to a Mistreated Facial Furuncle

Infections of the face, especially of the medial third or "the danger triangle", are the most frequent primary foci associated with thrombosis of cavernous sinus. Microorganisms entering the facial vein and pterygoid plexus from these sites are easily carried to the sinus through the ophthalmic vein. When the septic thrombosis involves various structures in the sinus, multiple clinical manifestations develop and if appropriate anti-infective therapy is not instituted timely, grave consequences with high mortality is possible. Recently, we experienced such a case. A five-year-old boy had high fever, proptosis, periorbital edema, chemosis and limitation of eye movements. Three days before admission, he had suffered from erythematous swelling spreading over the periorbital area, where simple steroid ointment containing no antibiotics had been applied after a furuncle on the nose had been squeezed. On admission, brain MRI revealed meningeal enhancement, high-signal densities in enlarged right cavernous sinus and narrowing of internal carotid artery. Analysis of cerebrospinal fluid showed bacterial meningitis and the cultures of blood and eye discharge proved to be Staphylococcus aureus. On massive antibiotic therapy, he recovered swiftly without any adverse sequelae. The authors report this case with a warning against wide-spread misuse of steroids and inadequate treatment of the mostly neglected, but once-dreaded minor infections on the face.

A Nationwide Clinical Study of Acute Respiratory Distress Syndrome in Children

PURPOSE: Acute respiratory distress syndrome(ARDS) is the final course of acute lung injury. It results from various etiological origins and pathophysiologic mechanisms, and has a mortality rate of approximately 60-70%. Although the confirmative incidence of ARDS in children is yet unknown, the increasing incidence of ARDS has been reported in Korea. In the present study, we report ARDS diagnosed at the Clinic for Pediatric Allergy and Respiratory Disease in eleven medical centers nationwide. METHODS: The study was conducted on 42 patients diagnosed with ARDS in Pediatric Allergy and Respiratory Clinics from January, 1995 to August, 1997. We analyzed the clinical course and treatment modalities of the 42 cases of ARDS retrospectively. RESULTS: The total number of patients recruited was 42, including seventeen(40.4%) below 1-year-old. The mean age was 2.0+/-2.3(mean+/-standard deviation) years with a range of 2 months to 10 years, and there was no sex predominance(male/female : 27/15). Twenty-one cases(50.0%) occured during the spring(March, April and May). The major triggering factors of ARDS were viral pneumonia(59.5%) and bacterial pneumonia (19.1%). Mechanical ventilation was used in 37 cases(88.1%). Major complications included pneumothorax, DIC, and gastrointestinal bleeding. The mortality rate was 61.9% of which 16 case (61.5%) occurred before 2 years of age. CONCLUSION: We conducted this study to make a rapid diagnosis and appropriate treatment of ARDS in children, who have major risk factors, to reduce its mortality rate.

A Nationwide Clinical Study of Acute Respiratory Distress Syndrome in Children

PURPOSE: Acute respiratory distress syndrome(ARDS) is the final course of acute lung injury. It results from various etiological origins and pathophysiologic mechanisms, and has a mortality rate of approximately 60-70%. Although the confirmative incidence of ARDS in children is yet unknown, the increasing incidence of ARDS has been reported in Korea. In the present study, we report ARDS diagnosed at the Clinic for Pediatric Allergy and Respiratory Disease in eleven medical centers nationwide. METHODS: The study was conducted on 42 patients diagnosed with ARDS in Pediatric Allergy and Respiratory Clinics from January, 1995 to August, 1997. We analyzed the clinical course and treatment modalities of the 42 cases of ARDS retrospectively. RESULTS: The total number of patients recruited was 42, including seventeen(40.4%) below 1-year-old. The mean age was 2.0+/-2.3(mean+/-standard deviation) years with a range of 2 months to 10 years, and there was no sex predominance(male/female : 27/15). Twenty-one cases(50.0%) occured during the spring(March, April and May). The major triggering factors of ARDS were viral pneumonia(59.5%) and bacterial pneumonia (19.1%). Mechanical ventilation was used in 37 cases(88.1%). Major complications included pneumothorax, DIC, and gastrointestinal bleeding. The mortality rate was 61.9% of which 16 case (61.5%) occurred before 2 years of age. CONCLUSION: We conducted this study to make a rapid diagnosis and appropriate treatment of ARDS in children, who have major risk factors, to reduce its mortality rate.

A Case of Hinman Syndrome Successfully Treated by Non-Surgical Measures

Hinman syndrome is a condition caused by an incoordination between the detrusor and external urethral sphincter during bladder contraction. Manifestations include day-and-night wetting, residual urine, infected urine, vesicoureteral reflux, christmas-tree shaped bladder-wall change, and upper tract damage without neurologic lesion or anatomical obstruction. Recently, this incoordination was postulated to be due to over-compensation of the external sphincter which compensates the uninhibitory detrusor contraction and pathological persistence of this "detrusor-sphincter dyssynergia" habit after brain cortical maturation. Accordingly, this syndrome is an acquired psychosocial-behavioral problem, reversible by bladder training and does not necessitate surgical intervention. We report a case of nonneurogenic neurogenic bladder who was successfully treated by biofeedback training, anticholinergic drugs and intermittent catheterization.

Two Cases of Double-Orifice Mitral Valve Detected by Echocardiography

Double-orifice mitral valve is a rare congenital anomaly. Most cases of double-orifice mitral valve are hemodynamically normal and remain symptomless, so that it is usually discovered incidentally in autopsy or during surgical correction of a cardiovascular abnormality. Recently, however, it is increasingly recognized as such, since the echocardiography has gained wide acceptance as a non-invasive diagnostic tool by the M-mode, two-dimensional and color Doppler echocardiogram. Two separate mitral valve apparatuses can be used on the M-mode echocardiogram. In the two-dimensional echocardiography, the parasternal and subcostal short-axis views can show two separate glass-like orifices in the left ventricle, and the parasternal long-axis view as well as the apical four-chamber view can show the anomaly. And the color Doppler echocardiogram can visualize two mosaic-pattern flows between the left atrium and ventricle. We present herewith two cases of double-orifice mitral valve, as diagnosed by means of echocardiography. The first case was an isolated one with mitral stenosis, showing two parachute mitral valves. The second was associated with perimembranous ventricular septal defect, and showed the accessory mitral valve directly attached to the ventricular septum, with the chordae crossing the ventricular outflow tract.

A Clinical Study on Factors affecting Spontaneous Resolution of Vesicoureteral Reflux in Children

We report a case of 53-year-old man with plasmacytoid transitional cell carcinoma of the urinary bladder, which may be confused with plasmacytoma. The patient initially presented with gross hematuria and dysuria for two months. Cystoscopy and radiologic studies revealed multiple intraluminal protruding masses on the urinary bladder invading perivesical fat tissue. After urinary cytologic examination and cystoscopic biopsy, radical cystectomy and pelvic lymph node dissections were done. Urine cytology showed single cells and poorly cohesive cells with round eccentric nuclei, bi-or multi-nucleation, indistinct nucleoli, coarse chromatin, and abundant basophilic cytoplasm within relatively clear background. The cytologic findings of tumor cells were similar to the plasma cells seen in plasmacytoma. The tumor of the bladder was composed of discohesive, individual cancer cells with diffuse pattern that simulated lymphoma or plasmacytoma. Immunohistochemical and electron microscopic studies clearly established the epithelial nature of the neoplasm. Recognition of this plasmacytoid type of transitional cell carcinoma of the urinary bladder can avoid the misdiagnosis.

Prognosis of Bacterial Meningitis based on Cerebrospinal Fluid Culture

PURPOSE: The present study was undertaken to assess whether the results of cerebrospinal fluid (CSF) culture are related to prognosis. METHODS: Subjected to this study were 51 cases admitted to our department who had been diagnosed as bacterial meningitis. They were divided into two groups, Group l (20) with positive CSF culture and Group ll (31) with negative outcome, then both groups were compared. RESULTS: Group l cases tended to be younger than Group ll cases. The male/female ratio was 1.2 in Group l and 1.6 in Group ll. Laboratory findings of CFS were not significantly different in either groups, except for the glucose level, which was markedly lower in Group l than in Group ll. The causative organisms identified from CSF culture were E. coli (5), group B Streptococcus (4) and S. aureus (1) among the 10 cases aged under 2 months. On the other hand, S. pneumoniae were isolated in 7 and H. influenzae in 3 among the 10 older cases. Mortality rate and the incidence of complications were higher in Group l than in Group ll. Mortality rate as related to the causative organism was highest with E. coli, followed by group B Streptococcus, H. influenzae. CONCLUSION: Bacterial meningitis cases with positive CSF culture have significantly lower glucose concentration in the CSF, and also higher rates of mortality and incidence of serious complications than those with negative culture.

A Case of Berry Syndrome associated with Syndactyly

Berry syndrome is a rare association of congenital heart anomalies which consists of a distal aortopulmonary window with aortic origin of the right pulmonary artery and hypoplasia or interruption of the aortic arch. This defect can be corrected only by immediate surgical intervention, so accurate preoperative diagnosis and detailed anatomic depiction of this syndrome are important in prognosis. We experienced a case of Berry syndrome in an 8-day-old male, who had presented with multiple malformation such as syndactyly, high arched palate and brain hemorrhage. The diagnosis was made by two-dimensional echocardiography. The case is presented with a brief review of related literatures.

A Case of Congenital Tuberculosis woth Massive Ascites and Pleural Effusion / 대한주산의학회잡지

We experienced a case of congenital tuberculosis infected in utero, who had been found to have massive ascites and pleural effusion on routine ultrasonography at the 39 weeks' gestation. The emergency C-section delivered a male neonate weighing 2,050 g with poor condition and in severe respiratory distress. He was the first child born of a 27-year-old woman, who was diagnosed as having tuberculous pleurisy after delivery. Detection of acidfast bacilli in gastric aspirates obtained from the newborn as well as the clinical features and maternal history confirmed the diagnosis of congenital tuberculosis. Though rare, congenital tuberculosis requires early detection and treatment because of its severity and high mortality. A brief review of the relevant literature was made.

The Pelvic Changes of Patients with Down Syndrome in Korea

PURPOSE: Since Caffey et al. first described the anomaly in pelvis among the Down patients, decreased iliac and pelvic indexes have been helpful in diagnosing the syndrome. For Korean cases, however, no definitive data are available as yet, prompting us to evaluate the pelvic changes in Korean Down's patients. METHODS: Subjected to this study were 68 children with Down's syndrome. As the control group served 126 patients with other diseases that do not affect the pelvis. The acetabular angle (AA) and the iliac angle (IA) were measured, and the iliac and pelvic indexes wer calculated from the following equations : Iliac index (Ii) = X + Y: pelvic index (Pi) = 0.3X + 0.42Y (where X is the mean of both acetabular angles, and Y is the mean of both iliac angles) RESULTS: 1) The sex ratio of the patients was 1.5 to 1, male prepondering. Their ages ranged from 1 day to 13 years, with the mean of 9.5 months, the majority (55.9%) falling under 1 month. The karyotype most frequently found in them was 21-trisomy (97%), followed by translocation (3%). 2) The AA of the Down patients was 18.49+/-4.92 (M+/-SD), significantly lower than that of the control group with 25.21+/-5.73; and the IA of the affected measured 43.53+/- 5.97, also signficantly (p<0.001) lower than the control (52.68+/-7.93). 3) The Ii and Pi of the Down groups were calculated as 62.01+/-9.04 and 23.83+/-3.38; both values were significantly lower than those of the control with 77.87+/-11.28 and 29.69+/-4.26, respectively. 4) The maximum likelihood estimate of Ii for Down syndrome was found to be 64, and at that level, the specificity 87.30%, and the sensitivity 66.18%. 5) In the Neonatal and infantile period, all parameters of Down syndrome were very significantly lower values when compared with those of the control of corresponding age In the childhood age, however, the AA of both groups did not significantly differ from each other, but the Down children had significantly lower values in the other parameters. The Ii decreased in relation to the increasing age in the control group, while no significant correlation was noted in the Down patients. 6) In the control group, a significant gender difference was found in the Ii, with females having higher values. In the Down patients, however, both sex did not significantly differ in the Ii. CONCLUSIONS: Decreased iliac and pelvic indexes manifested in Korean Down cases were quantitively evaluated in this study and it was found that an iliac index below 64 strongly supports the diagnosis of Down syndrome. For the Down cases, the Ii did not signficantly correlate with age, nor did both sexes differ in the Ii, in contrast to the control group which showed an age-related decrease and higher female values.

Study on Platelet Counts and Indices in Neonates

PURPOSE: The platelet number alone does not give a complete picture of platelet maturity and function, therefore, the platelet indices have been the subject of intensive study in recent years, but they have not been firmly established in the neonates. METHODS: Blood samples collected in K2 EDTA bottle from femoral vein of 176 neonates (54 healthy fullterm, 96 sick fullterm, 10 sick preterm, 9 healthy preterm and 7 healthy small for gestational age) were analyzed with CELL-DYN 1600. RESULTS: 1) Platelet counts (PC) and platelet indices of 70 healthy neonates correlated neither with gestational age, nor with birth weight. 2) Significant correlations were found between PC and plateletcrit and between PC and mean platelet volume with the correlation coefficients being 0.937 and -0.284 respectively. Other indices did not show any significant correlation. 3) There were no significant differences of PC and platelet indices between healthy neonates and sick neonates, between healthy fullterm and sick fullterm, between healthy preterm and sick preterm, between healthy fullterm and healthy preterm, and between healthy fullterm and healthy small for gestational age. 4) Sick fullterm neonates suffering certain diseases showed altered indices, compared with healthy fullterm neonates. In diarrhea, PC, plateletcrit and platelet distribution width were higher than those in healthy fullterm neonates. In hyperbilirubinemia and urinary tract infection, PC and plateletcrit were higher, whereas in sepsis, platelet distribution width was lower. CONCLUSIONS: These data indicate that PC and platelet indices of healthy neonates did not correlate with gestational age, nor with birth weight, and show some significant correlations between PC and platelet indicies. Further studies will be needed in clinical application of platelet indices to each neonatal disease.

Trend of Tuberculous Meningitis since 1970

PURPOSE: The incidence of tuberculous meningitis has reportedly decreased from 4.2% in 1970 to 1.8% in 1990 in Korea; however, tuberculous meningitis, which spreads hematogenously, claiming majority of mortality in childhood tuberculosis, still poses serious problems. This study was performed to evaluate the recent trend of tuberculous meningitis. METHODS: We compared with three groups of tuberculous meningitis, two were our previous reports on tuberculous meningitis, 106 cases1) from 1971 to 1979 were assigned as group I, 60 cases2) from 1982 to 1988 as group II and we reviewed medical record of 16 cases from 1989 to 1994 as group III. RESULTS: 1) The incidence of tuberculous meningitis among the total admitted patients in groups I, II, and III were 0.92, 0.27, and 0.06%, decreasing with a slope of -0.063% per year, and a correlation coefficient of -0.892. 2) The age proportion among children under age 1 year were 17.9, 8.3, and 6.3%, espectively for three groups and those over age 9-10 years were 11.3, 13.4, and 43.8%, indicating that proportion of under age 1 year declined, whereas it increased in older aged children. 3) The overall male to female ratio was 1.43 : 1, and peak seasonal incidence was not noted. 4) There was no differences in tuberculous family history with 41.5% in group I, and 43.8% in group III; BCG vaccination rate was increased significantly from 16.0% in group I up to 68.6% in group III(p<0.001); and the positive tuberculin skin test reactivity significantly increased from 29.6% in group I to 68.8% in group III(p<0.01). 5) On chest X-ray, the rate of finding tuberculous lesion did not significantly change among 3 groups with 69.8, 58.3, and 87.5%, while the rate of associated miliary tuberculosis was significantly reduced with 36.8, 28.3, and 0%(p<0.05). 6) The cure rate was 65.1, 68.4, and 87.5%, respectively and the cure rate of clinical stage 3 improved from 36.1% in group I to 66.7% in group III, but they showed no significant difference between groups(p<0.05). The mortality rate was 5.7, 14.9, and 12.5%, respectively and there was no significant difference between groups(p<0.05). 7) Hospital days under 14 days were 67.9, 23.3, and 6.3%, respectively; those over 21 days were 3.8, 45.0, and 68.8%, respectively, indicating the hospital days became prolonged(p<0.001). 8) Brain CT was performed in group III(13/16), with abnormal findings found in 84.6%. The abnormal findings were hydrocephalus in 5 cases(26.3%, ventriculoperitoneal shunt was done in 3 cases); and basal cisternal enhancement, and infarction 3 cases each. Complication rate was not improved with 35.0% in group I and 31.2% in group III. CONCLUSIONS: During 25 years, the incidence of tuberculous meningitis significantly decreased with the increased BCG vaccination rate; and the age of the affected children tended to increase. It is noted, however, that the mortality rate and neurologic complication of the affected children were not substantially improved, stressing early diagnosis and vigorous treatment on tuberculosis in children.

Changes in Maternal Age and the Incidence of Down Syndrome

PURPOSE: The incidence of Down syndrome, the most common chromosoaml anomaly, increases with the advanced maternal age. Recently, however, the incidence of Down symdrome was reported to have decreased with wide acceptance of prenatal diagnosis and planned parenthood, prompting us to re-evaluate the incidence of Down syndrome in relation to changes in maternal age. METHODS: Subjected to study were 296 Down cases: 26 newborn Down's among the 58,479 deliveries undergone in our hospital (KCH) during the period over 2 decades from April 1974 through December 1994; and 270 cases, whose maternal age at delivery could be traced with certainty, among 366 Down cases confirmed by genetic counseling. The observation period was divided into three: Period I, before 1979 (n=66); Period II, from 1980 to 1989 (n=127); and Period III, after 1990 (n=103). RESULTS: 1) The change in maternal age at delivery Of total 58,479 deliveries, 0.5% were born to mothers younger than 20 years, 21.3% were aged 20-25 years; 57.7% (33,722) were aged 26-30 years; 16.9% fell between 30-35 years; and 3.6% were above 35 years. Deliveries of old mothers (>35 ys) were 4.9% in Period I, which significantly decreased to 2.8% in Period II, increased again to 4.1% in Period III. 2) Changes in Down incidence among those delivered in KCH Overall Down incidence among those delivered in KCH was 0.44/1000 (26/58,479); however, it was 0.93/1000 in Period I, 0.16 in II, significantly lower; and 0.59/1000 in Period III. Of the 26 Downs born in KCH 18 were born to mothers younger than 35 years (0.03%), whereas 8 were born to mothers older than 35 years (0.8%). The Down incidence among mothers younger than 35 years did not significantly differ among the periods, while for the mothers aged over 35 years it decreased from 1.0% (6/576) in Period I to 0% (0/825) in Period II, and back to 0.3% (2/698) in Period III, indicating that Down incidence among old mothers significantly decreased recently, compared with the early period. 3) Changes in maternal age and Down incidence among those confirmed by genetic counseling. Of those 270 Down cases, 226 were born to mothers younger than 35 years, whereas 44 were delivered by old mothers aged above 36 years. Old mothers had Down babies in 23.6% (13/55) in Period I, but it significantly decreased to 11.5% (14/122) in Period II, but it recovered to 18.2% (17/93) in Period III. 4) Relationship between karyotype pattern and maternal age Of total 296 Down cases 260 (87.8%) had typical pattern of 21-trisomy, 32 (10.8%) translocation, 4 (1.4%) mosaicism. Those with typical trisomy had mean age (+/-S.D.) of 30.3 (+/-6.0) year and those with mosaicism 30.8 (+/-6.2) year, whereas those with translocation were significantly younger with the mean age of 26.6 (+/-2.5) year. CONCLUSIONS: During the last 2 decades, the deliveries by old mothers tended to decrease, and also the overall incidence of Down syndrome has significantly decreased, especially among those born to mothers older than 35 years. Recently, however, maternal age tends to increase for various reasons, necessitating due emphasis on prenatal diagnosis.

A Case of Acrodysostosis

Acrodysostosis is a rare multiple congenital malformation syndrome with peripheral dysostosis, nasal hypoplasia, mental retardation and growth failure. In 1968, Maroteaux and Malamut first described this disorder in three patients, and thus far now over 30 published cases have been reported worldwide. Radiographic changes include severe shortening of metacarpals, metatarsals, and phalages, cone-shaped epiphyses of the hands and feet, premature fusion of the epiphyses, and advanced bone age. Most patients do relatively well except for the problems of mental deficiency and arthritic complaints. Progressive restriction of movement of the hands, elbows, and spine may occur. Recently, we experienced a case of acrodysostosis in and 11-year-old boy who presented with short stature, short hands and feet, nasal hypoplasia, mental retardation, and typical radiographic findings including severe shortening of metacarpals, metatarsals and phalanges, cone-shaped epiphyses of the hands and feet.