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Biomedical subjects

N C Chiu

Publications and source records attributed to N C Chiu.

30 records · Page 2Linked to original sources

Aicardi's syndrome in a female infant with a family history of miscarried male siblings.

Aicardi's syndrome is thought to be an X-linked genetic disease, although the mechanism for transmission remains uncertain. We report on a four-month-old female patient with Aicardi's syndrome. She was born prematurely at 28 weeks' gestation, weighing 1,500 g. Asymmetric myoclonic jerks developed at one month of age. Her left eye showed chorioretinal lacunae and a coloboma on the optic disc, while the right eye was microphthalmic with total retinal detachment. A CT scan disclosed heterotopia and dysgenesis of the corpus callosum. Abnormal development of the thoracic vertebrae was also evident. The most remarkable aspect of this case was that the patient's mother had suffered three miscarriages. Two are known to have been male, but the other gender is unknown. This family history may support the theory that there is a factor, lethal for males, involved in the genetic transmission of Aicardi's syndrome.

Abnormalities, Multiple↗

[Chylous ascites in infancy].

Chylous ascites is a rare disease in infancy. Onset with acute manifestations should be managed surgically. However, congenital in nature or chronic cases many be treated conservatively with either medium-chain triglyceride feedings or total parenteral nutrition. Five cases of infantile chylous ascites are reported and discussed here.

Chylous Ascites↗

Neurogenic diabetes insipidus in a child with fatal Coxsackie virus B1 encephalitis.

A 5 year-old boy presented with fever, sore throat, diarrhea, and general soreness which evolved into encephalitis. His cerebrospinal fluid showed a cell count of 3 mononuclear cells/microliters, protein 2800 mg/l, and growth of Coxsackie virus B1. Cardiorespiratory arrest was noted after a convulsion and infusion of diazepam. Although he was immediately resuscitated, he remained unconscious with a modified Glasgow coma score of 4 or 3. He developed neurogenic diabetes insipidus 169 hours after the convulsion and died the next day. We conclude that although Coxsackie virus infection is usually benign it may become overwhelming and be complicated with neurogenic diabetes insipidus. It is important to recognize this potential sequel by regularly monitoring weight, intake and output, plasma sodium level, and urine specific gravity.

Cerebrospinal Fluid↗

Tuberous sclerosis with visceral organ involvement.

This study is to determine the incidence of visceral organ involvement in tuberous sclerosis (TS). We reviewed 30 cases of TS diagnosed between 1987 to 1997. There were 17 males and 13 females, ages ranged from one day old to 17 years old. Among the 30 cases, 25 patients had seizures and skin manifestations; 24 had cerebral tubercles; 10 had heart involvement (9 rhabdomyoma, 1 dilated cardiomyopathy); 4 had kidney involvement (3 polycystic kidney disease, 1 renal hamartoma); and 3 had retinal astrocytic hamartoma. Based on our study, the most common visceral organs involved were the heart and kidney. Among the ten patients with cardiac rhabdomyoma, six were less than 1 year old (mean age 1.6 +/- 2.2 years old). One newborn presented with a cardiac mass diagnosed by prenatal sonography and another newborn, noted to have tachycardia after birth, underwent surgical intervention due to impending heart failure. Four patients had kidney abnormalities; three were less than 5 years old (mean age 5.2 +/- 5.2 years). During this 10 year period, there was no mortality seen among patients with visceral organ involvement. We suggest that clinicians treating patients with TS should not overlook the visceral organs, especially heart and kidney, which, if involved can have significant morbidity.

Adolescent↗

Characterization of group D1 non-typhoid Salmonella isolates by serotyping and pulsed field gel electrophoresis.

Non-typhoid salmonella infection is not uncommon in immunocompetent patients in Taiwan. Bacterial factors may play an important role in the pathogenesis of such infections. In a previous study, Salmonella group D1 was found to have the tendency to cause bacteremia with a higher frequency than other serotypes. In the present study, we prospectively collected 94 Salmonella group D1 isolates for serotyping and molecular typing. Salmonella panama and Salmonella dublin seemed more invasive than other serotypes. Pulsed field gel electrophoresis was also done to characterize of Salmonella enteritidis and Salmonella dublin. PFGE type "a" of Salmonella dublin appeared to be more invasive than the other two PFGE types. All six Salmonella dublin isolates were Vi antigen negative. Further study using a larger number of isolates is needed to identify the tendency to invade blood stream of Salmonella dublin and Salmonella panama.

Adult↗

Congenital varicella syndrome as an unusual cause of congenital malformation: report of one case.

Intrauterine infections with varicella-zoster virus following maternal varicella in early pregnancy and resulting in congenital malformations are rare. Herein we report a child with congenital varicella syndrome characterized by low birth weight, cicatricial scarring, hypoplasia of both lower extremities with joint contracture, congenital hip dislocation, corneal opacity, atresia of the sigmoid colon and a rarely associated cloaca anomaly. The varicella IgG remained positive after she was seven months old. Her mother developed chickenpox at the 14th week of gestation. The purpose of this article is to raise pediatricians' index of suspicion for congenital varicella syndrome when an infant is born with multiple congenital malformations with an apparent history of maternal varicella infection.

Abnormalities, Multiple↗

Adenoviral pneumonia in children.

This report reviews the manifestations in fifteen children of proved adenoviral pneumonia. Patients' ages ranged from 43 days to 4 years and 1 month. Twelve cases were younger than 2 years old. Adenoviral infections were proved by positive viral cultures or a four-fold increase of the complement fixation titer. Prolonged fever and cough were found in all cases. In 13 patients, respiratory distress occurred; 5 needed mechanical ventilation. Injected throats, conjunctivae and ear drums were common. Other clinical pictures included abdominal discomfort, hepatomegaly, skin rash, convulsion and bleeding tendency. Abnormal laboratory findings were mild anemia, leukopenia, thrombocytopenia, elevated erythrocyte sedimentation rate and C-reactive protein, impaired liver function test, and prolonged prothrombin time and partial thromboplastin time. Anemia (11 cases), leukopenia (7 cases) and elevated transaminases levels (7 cases) were more common than previously reported. All patients had para-hilar peribronchial infiltrates in chest roentgenography. Segmental atelectasis and compensated hyper-expansion were found frequently. Pleural effusion were noted in six of our cases. Air leak syndrome occurred in three patients who had received mechanical ventilation. Three of the 15 patients expired: one had a preceding measles infection, all had disseminated intravascular coagulopathy. For patients with antibiotic-resistant pneumonia, adenoviral studies should be done. Extrapulmonary manifestations, and some abnormal laboratory findings, i.e., mild anemia, leukopenia, impaired liver function are clues to adenoviral infections, while bleeding tendency can be regarded as a poor prognostic sign for children with adenoviral pneumonia.

Adenoviridae Infections↗

Hemorrhagic shock and encephalopathy syndrome: Report of two cases.

Two young children with a history of diarrhea, for one and two days respectively, were admitted to our hospital due to high fever and conscious disturbance. Disseminated intravascular coagulation, abnormal hepatic and renal functions, and metabolic acidosis were noted as well. Both patients developed shock soon after arrival and expired within one day after admission. Their serum ammonia levels were normal, and their blood and cerebrospinal fluid cultures also brought negative results. Hemorrhagic shock and encephalopathy syndrome was diagnosed by their clinical manifestation and laboratory data. We also discussed their related problems.

Brain Diseases↗

Urinary tract infections in children.

From January 1981 to December 1987, 346 children with urinary tract infections, proved by urine culture, were admitted to the Department of Pediatrics, Mackay Memorial Hospital. The ratio of male to female was 3.0 in children below 2 years, and 0.8 in children above 2 years, of age. The urine specimens were collected from suprapubic punctures in 281 cases (81.2%). Fever was the most common clinical manifestation. In children below two years old, other common symptoms and signs were body weight loss or poor gain, feeding problems, diarrhea, irritability, jaundice, and abdominal distension. In older children, urinary frequency, dysuria, enuresis, loin and abdominal pain were frequently found. Hematuria and edema were occasionally noted in all age groups. Microscopic examination of 329 centrifuged urine specimens revealed: 256 cases (77.8%) had more than 5 leukocytes per high power field, 233 cases (70.8%) had more than 10 leukocytes. Three hundred and seventy positive urine cultures were obtained from these patients. E. coli was isolated in 273 cases (73.6%), followed by Klebsiella spp., 34 cases (9.2%); Proteus spp., 27 cases (7.3%); Enterococcus, 21 cases (5.7%); Enterobacter spp., 9 cases (2.4%); Pseudomonas aeruginosa, 8 cases (2.2%); Citrobacter spp., 7 cases (1.9%); Morganella morganii, 6 cases (1.6%); Acinetobacter spp., 6 cases (1.6%); etc. Candida albicans was isolated from three patients. Two organisms were isolated in 26 cultures; 3 organisms, in 3, and 4 in 1.

Adolescent↗

[Coagulase-negative staphylococcal septicemia].

In the past decade, coagulase negative Staphylococcus (CNS) has become one of the most common pathogens in nosocomial septicemia, especially in neonatal intensive care units. From January 1, 1990 to June 30, 1992, we documented 41 cases of CNS septicemia in the Department of Pediatrics at Mackay Memorial Hospital. Thirty five cases (85%) were found in infants less than 3 months of age. Nineteen patients were premature babies. Of these, 14 had a body weight less than 1,500 gms. All of the patients had intravascular catheters and 40 patients (97.5%) were receiving total parenteral nutrition. The most common causative organism isolated in this study was Staphylococcus hominis. This differs from other reports where Staphylococcus epidermidis is the most common pathogen. Eighty five percent of the organisms were resistant to methicillin and 80% of these methicillin-resistant strains were also resistant to cephalosporins. All of the organisms were sensitive to vancomycin. Because of the possibility of cross-resistance between methicillin and cephalosporin, vancomycin was chosen to treat the methicillin resistant cases. Total parenteral nutrition was discontinued before antibiotic treatment. When CNS septicemia was suspected clinically, we drew two blood cultures from different sites before beginning antibiotic therapy. This was an effort to get a definitive diagnosis. In general, the prognosis of CNS septicemia was good if early diagnosis can be made.

Bacteremia↗

Urinary tract infection in infants less than 2 months of age.

Ninety-five infants, less than 2 months of age, diagnosed as urinary tract infections, from July 1984 to June 1991, were reviewed. Their urinary cultures, obtained either by suprapubic puncture or via catheterization, all had bacterial colony counts of over 10(5)/ml. In this survey, males predominated (91.6%). Fever and gastrointestinal problems were the two most prevalent signs. E. coli was the most common causative organism, and gentamicin was the most effective antibiotic. Vesicoureteral reflux (VUR), the most common anomaly, was found in one-third (25/76) of patients on voiding cystourethrography, with 20% being high grade (Gr. IV or Gr. V). Eleven cases (11%) had bacteremia, and one case had bacterial meningitis. Sixty-seven cases were followed up in our hospital and seven of them had second infections within a year of their first UTI. The mean period between episodes was less than two months. All these patients had urinary tract anomalies and received oral chemoprophylactic drugs for variable lengths of time. Five of the seven recurrences were caused by resistant bacilli. Continuous oral antibiotic prophylaxis and regular follow-up examinations were the rules of prevention for further infection and future renal impairment. These preventive methods are especially important in young infants with UTI.

Bacteremia↗

Cutis marmorata telangiectatica congenita with cerebral and ophthalmic anomalies: report of one case.

A term male neonate had an uncommon congenital vascular disorder of the skin covering the whole body and extremities. These skin lesions were characterized by mottling and persistent telangiectasia. A skin biopsy showed nevus vascularis reticularis which was recognized as cutis marmorata telangiectatica congenita. In addition to the cutaneous involvement, the patient also had fetal ascites, pleural effusion, patent ductus arteriosus, glaucoma, retinal detachment in the left eye, and telangiectasia in the right eye. Neonatal focal seizure developed and expired on the 34th day of life. The cerebral image studies suggested vascular anomalies with secondary cerebral parenchymal damage. Cutis marmorata telangiectatica congenita may be solitary but is frequently associated with other developmental defects. However, the specific findings of cerebral and ophthalmic lesions in our case were rather unusual.

Abnormalities, Multiple↗