Comparison of heart failure in children with enterovirus 71 rhombencephalitis and cats with norepinephrine cardiotoxicity.

The mechanism of heart failure in patients with enterovirus 71 rhombencephalitis (brain stem encephalitis) remains unknown. Our previous reports hypothesized that a catecholamine storm induced by rhombencephalitis may account for the heart failure. The aim of this study was to develop a novel feline model of norepinephrine cardiotoxicity and compare the resulting heart failure to that in children with enterovirus 71 rhombencephalitis. Nine of 75 children (12%) with enterovirus 71 rhombencephalitis (5 boys and 4 girls; age, 4-28 months; median age, 16 months) were complicated with left ventricular hypokinesia (ejection fraction, 31 +/- 9%). Six cats (weight, 3.03 +/- 0.64 kg) were administered intravenous norepinephrine 30 microg/kg/min for 3 hours. Echocardiography assessed the left ventricular diameter and function before and after the administration of norepinephrine. Pathology studies included hematoxylin and eosin stain and in situ terminal deoxyribonucleotidyl transferase-mediated dUTP nick end-labeling assay. In the feline model, norepinephrine induced significant left ventricular dilatation (end diastolic diameter from 1.18 +/- 0.19 to 1.62 +/- 0.22 cm, p = 0.001; endsystolic diameter from 0.54 +/- 0.09 to 1.36 +/- 0.32 cm, p = < 0.001) and hypokinesia (ejection fraction from 87.5 +/- 4.1 to 35.2 +/- 16.3%, p = 0.001). Heart specimens from 4 patients and six cats showed similar pathology findings, including myocardial hemorrhage, cardiomyocyte apoptosis, and coagulative myocytolysis, which is characterized by sarcoplasmic coagulation, granulation, vacuolization, myofibrillar waving, and disruption. Both groups showed no significant inflammatory reaction. In conclusion, heart failure in patients with enterovirus 71 rhombencephalitis is similar to that in cats with norepinephrine cardiotoxicity. Norepinephrine cardiotoxicity may play a role in the pathogenesis of heart failure in enterovirus 71 rhombencephalitis.

Transcatheter closure of a large patent ductus arteriosus in a young child using the Amplatzer duct occluder.

The Amplatzer duct occluder (ADO) provides a safe and effective therapy for patients with moderate- to large-sized patent ductus arteriosus (PDA), but there have been few reports of transcatheter closure of very large PDAs in young children and infants. We report a successful transcatheter closure of a very large PDA, 10.5 mm in diameter at the narrowest point, with a 14/12-mm ADO. To our knowledge, this is the largest PDA ever closed by an interventional method in such a young child.

Cardiac complications of enterovirus rhombencephalitis.

BACKGROUND: Epidemics of enterovirus 71 infection have caused the death of many children throughout the world. Rhombencephalitis, brain stem encephalitis, and heart failure were present in all of the fatal cases. However, no evidence of myocarditis was noted in the heart specimens, and the mechanism of heart failure remains unknown. AIMS: To characterise the presentation of cardiac complications in children with enterovirus rhombencephalitis and discuss its pathogenesis. METHODS: Ninety one consecutive patients with enterovirus rhombencephalitis underwent echocardiography. Of these, 17 patients (nine male, eight female; median age 14 months, range 4-57 months) with left ventricular dysfunction were studied. RESULTS: Tachycardia was noted in all patients and systemic hypertension in 12. Muscle-brain fraction of creatine kinase was >5% in 14 patients. Plasma norepinephrine and epinephrine levels were significantly raised in the three patients in whom these were analysed. Electrocardiographic abnormalities were noted in eight patients. Pulmonary oedema was complicated in 15 patients. The initial ejection fraction of the left ventricle was 22-58% (mean 37%, SD 11%). All patients deteriorated to hypotensive shock within 12 hours and 13 died. Heart specimens from seven patients showed no evidence of myocarditis, but significant coagulative myocytolysis, myofibrillar degeneration, and cardiomyocyte apoptosis were observed. CONCLUSIONS: Acute heart failure was noted in 19% of patients with enterovirus rhombencephalitis, which had a fatality rate of 77%. It was not caused by myocarditis but possibly by neurogenic cardiac damage.

Poor agreement between dipyridamole-stress technetium-99m-tetrofosmin myocardial perfusion single photon emission computed tomography and two-dimensional echocardiography in Kawasaki disease.

To establish a noninvasive diagnostic method for early assessment of cardiac involvement in Kawasaki disease (KD), 28 children with KD were included in this study. Two-dimensional echocardiography (2D-Echo) to detect the aneurysms of coronary arteries (CA), as well as rest and dipyridamole-stress technetium-99m tetrofosmin (Tc-TF) myocardial perfusion single photon emission computed tomography (SPECT) to detect abnormal myocardial perfusion were performed in all of the 28 children with KD and to compare each other. The results showed that (1). 42.9% of cases had no aneurysm and 57.1% had significant aneurysms detected by 2D-Echo; (2). 42.9% of cases had normal perfusion and 57.1% of cases had abnormal myocardial perfusion assessed by Tc-TF myocardial perfusion SPECT; (3). 25.0% of cases showed both normal 2D-Echo and Tc-TF myocardial perfusion SPECT findings and 39.3% of cases showed both abnormal 2D-Echo and Tc-TF myocardial perfusion SPECT findings; and (4). there was poor agreement between 2D-Echo and Tc-TF myocardial perfusion SPECT findings (P>0.05). We concluded that poor agreement exists between aneurysms and abnormal myocardial perfusion detected by 2D-Echo and Tc-TF myocardial perfusion SPECT findings in patients with KD.

Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case.

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5mg/kg/day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE fare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immumoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.

A novel mutation in neonatal isolated sulphite oxidase deficiency.

Isolated sulphite oxidase deficiency (ISOD) is a very rare hereditary metabolic disorder. Imaging findings of the neonatal form of ISOD, including multicystic leukoencephalopathy with brain atrophy, resemble those of severe ischemic changes of the brain. We report the case of a ten-month-old boy who exhibited neonatal seizures on the 24th day after birth. Excessive quantities of sulphite and S-sulphocysteine in the urine and normal blood uric acid were noted. These findings were consistent with those of ISOD. Point mutations were found in two alleles of the sulphite oxidase (SUOX) gene. One of the mutations was a 1029 C > G mutation, which resulted in an amino acid substitution of tyrosine to a stop code (Y343 X); and the other was a 479 G > A mutation, which resulted in an amino acid substitution of arginine to glutamine (R160 Q). Y343 X is a novel SUOX gene mutation. A review of the literature, including data from this report, showed that 3 of 6 cases had typical imaging findings characterized by initial cerebral edema followed by dramatic multicystic leukoencephalopathy. We emphasize that neonatal ISOD should be included in the differential diagnosis of neonates with unexplained hypoxic-ischemic changes on neuroimaging studies.

Growth response and final height in growth hormone-deficient patients treated with biosynthetic growth hormone.

Twenty-three patients with growth hormone deficiency (GHD) followed until their final heights (FH) were reported by retrospective review. Seven patients had spontaneous puberty (group 1) and sixteen required induction of puberty (group 2). Their heights prior to growth hormone (GH) therapy were -3.7 +/- 1.4 and -2.8 +/- 1.4 SDS in men and women respectively. The mean ages at initiation of GH therapy were 17.2 +/- 3.3 in men and 13.0 +/- 1.7 years in women, with a growth velocity < 4 cm/year and a mean bone age (BA) of 12.6 +/- 0.7 years (men) and 9.8 +/- 1.2 years (women). The dose of GH was 0.27-0.83 IU/kg/week, with a total duration of 2.5 +/- 1.0 years. Their consecutive annual mean growth velocities on GH therapy were: 10.6 cm/year, 8.0 cm/year, 6.1 cm/year, 5.1 cm/year and 4.6 cm/year respectively. They reached a mean final height of 167.1 +/- 5.3 cm (-0.4 +/- 1.0 SDS) in men and 157.9 +/- 3.5 cm (-0.1 +/- 0.7 SDS) in women, which is slightly taller than their target height. Demographic factors related to growth response and final heights were analyzed. We conclude that GH therapy is very effective in linear growth promotion. Their final height was correlated with initial height SDS, target height SDS, predicted adult height SDS according to the bone age at the start of GH therapy and height SDS at the onset of puberty.

Breastfeeding knowledge and attitudes of health professionals and students.

Thirty-one physicians, 108 nurses, 105 medical students, and 126 student nurses practicing in the same medical center received a questionnaire to evaluate their knowledge and attitudes about breastfeeding. The mean knowledge score of the 139 staff member was 49.4 +/- 10.8 (The highest possible score was 73); that of the medical students was 31.2 +/- 8.9, and that of the nurse students was 39.5 +/- 7.6. Thirteen percent of the medical students vs. 68.6% of the student nurses reported that they had received breastfeeding education in school. All of the respondents had a positive attitude toward breastfeeding. Occupation and in-service education for breastfeeding could increase the knowledge score. Having been breastfed during infancy could increase both the knowledge score and positive attitude score. We suggest that breastfeeding be integrated into the curricula of both medical and nursing schools. Health professionals should receive in-service education. The mechanism of lactation, management of breast problems and infant problems, contraindications for breastfeeding, the properties of human milk and the benefits of breastfeeding for the infant should all be included.

Precocious puberty associated with growth hormone deficiency in a patient with craniopharyngioma: report of one case.

Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. Growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. Growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. Growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.

Limited cutaneous systemic sclerosis: report of one case.

We report a case of systemic sclerosis, limited type, in a 5 year old boy in Taiwan. He presented with erythema, tight skin over his face and digits, subcutaneous calcification, telangiectasis, as well as Raynaud's phenomenon. The laboratory data showed weakly positive antinuclear antibody (ANA), and Topoisomerase 1 antibody (anti-Scl-70) titers. However his esophagogram, CO diffusion capacity, as well as cardiac and renal function were all normal. He suffered from the skin change for 2 to 3 years before visiting our pediatric clinic when he was 7 years old. We gave him Dipyridamole, D-penicillamine, Colchicine, and Nifedipine. There was some remission of the scleroderma and Raynaud's phenomenon after our treatment.

Hyperbaric oxygen for carbon monoxide poisoning-induced delayed neuropsychiatric sequelae.

The clinical manifestations of delayed neuropsychiatric sequelae after carbon monoxide (CO) intoxication are variable. In addition, there is no specific therapy for these complications. Fortunately, these complications have occurred less frequently in recent years, probably due to the usage of hyperbaric oxygen (HBO) therapy. We report an 8-year-old boy who developed late psychiatric disturbances 2 days after full recovery of consciousness from initial CO intoxication. His neuropsychiatric symptoms included consciousness disturbance, motor dysfunction, chorea, aphasia and agnosias. He received HBO therapy at 2.0 barr for 60 minutes once a day for 7 consecutive days. Three weeks later, he was functioning normally with no neuropsychiatric symptoms. A literature review concluded that HBO may be effective in treating neuropsychiatric sequelae. Moreover, immediate administration of HBO during acute CO intoxication may prevent these complications.

Intra-abdominal actinomycosis with hepatic pseudotumor and xanthogranulomatous pyelonephritis in a 6-y-old boy.

We report the case of a 6-y-old boy with actinomycosis, presenting as xanthogranulomatous pyelonephritis (XGP), hepatic pseudotumor and abdominal abscess. Symptoms included intermittent fever, abdominal pain and significant weight loss. Hepatic and renal tumor masses were suspected on sonography and computerized tomography. XGP and actinomycosis were proven by pathology. The patient recovered well with antibiotic alone.

Clinical spectrum of Kawasaki disease in infants.

BACKGROUND: Kawasaki disease is a common acquired heart disease in children. Only a few reports have been published concerning Kawasaki disease in infants. This study was performed to assess the clinical spectrum of Kawasaki disease in infants. METHODS: Between January 1989 and December 1998, a total of 48 consecutive Kawasaki patients less than one year of age were enrolled and studied retrospectively. Coronary artery dilation was defined as the internal diameter of a coronary artery larger than 3 mm. All cases received 2 gm/Kg of intravenous immunoglobulin. We divided the patients into two groups; group I; coronary artery dilation (+) and group II; coronary artery dilation (-), and compared the clinical and laboratory data. RESULTS: Of 273 patients with Kawasaki disease, 48 (17.5%) were less than one year of age. Among these patients (< 1 year old), the median age was 7.8 +/- 2.8 months (range 2 months to 12 months), and the male to female ratio was 1.52:1. The incidence of atypical Kawasaki disease was 31.2% (compared with an incidence of atypical Kawasaki disease among patient more than one year of age of 7.5%; p < 0.001), and that of coronary artery dilation was 35.4%. Clinical manifestations included fever 100%, extremity change 91.6%, skin rash 89.6%, conjunctivitis 89.6%, oral mucosa change 89.6%, and cervical lymphadenopathy 0%. Laboratory data revealed white blood cell count: 15,403 +/- 6,282/mm3, hemoglobin: 10.1 +/- 1.0 gm/dl, neutrophil: 59.2 +/- 13.7%, lymphocytes: 30.6 +/- 13.1%, platelet count: 456,3000 +/- 216,4000/mm3, and C-reactive protein 8.2 +/- 5.6 mg/dl. Patients with coronary artery dilation had a longer duration of diagnosis, higher incidence of atypical presentation, lower incidence of conjunctivitis, lower incidence of skin rash, lower incidence of extremity change, and lower C-reactive protein. The predictive value of coronary artery dilation based on the combination of atypical presentation, duration of diagnosis, and C-reactive protein was 81.2%. CONCLUSIONS: Kawasaki disease in infants is associated with a high incidence of atypical presentation and increased risk of coronary artery dilation. We suggest that in an infant with insufficient diagnostic criteria for Kawasaki disease, care should be taken to avoid missing atypical Kawasaki disease. Echocardiography is an important tool for diagnosis of atypical Kawasaki disease.

Intracardiac ultrasound assessment of atrial septal defect: comparison with transthoracic echocardiographic, angiocardiographic, and balloon-sizing measurements.

PURPOSE: Accurate evaluation of the size, location and adjacent structure of an atrial septal defect (ASD) is very important in the selection of patients for further management. We directly compared the utility of transthoracic echocardiography, angiocardiography, balloon sizing, and intracardiac ultrasound (ICUS) in the detection of ASD. METHODS: Twenty-one children underwent an ICUS study of ASD after routine clinical and laboratory studies. All patients had received transthoracic echocardiography (TTE), cardiac catheterization, cineangiography, and balloon sizing before the ICUS to evaluate the ASD. RESULTS: There was a significant correlation between the ICUS-derived ASD diameter and the other methods (p < 0.001). The balloon-sizing diameter was estimated by the equation: TTE diameter x 1.09 + 3.9 mm. There was a good correlation between the predicted and measured balloon-sizing diameter (r = 0.963; p < 0.001). CONCLUSION: It is worthwhile spending a few minutes to perform ICUS during cardiac catheterization since it will provide more detailed information on and high resolution images of atrial septal morphology, especially for patients undergoing transcatheter closure by device.

Congenital complete heart block.

Congenital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVI mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.

Zellweger syndrome: report of one case.

Zellweger syndrome is a fatal autosomal-recessive hereditary disease characterized by the absence of peroxisomes in liver and kidneys. The absence of peroxisomes results in impairment of many metabolic pathways, especially beta-oxidation of very long chain fatty acids (VLCFAs). We report a case of a three-month-old male infant with facial dysmorphism, hypotonia, psychomotor retardation, and hepatomegaly. He had an elder brother with the same facial features and hypotonia who died of hepatic failure at four months of age. Biochemical studies revealed elevation of blood pipecolic acid and VLCFAs, compatible with peroxisomal disorder. Electron microscopy of liver biopsy revealed absence of peroxisomes. Zellweger syndrome was diagnosed. Because this syndrome is usually fatal in early life, genetic counseling and prenatal diagnosis are crucial.

Stiff-Baby--an unusual manifestation of cytoplasmic body myopathy: report of one case.

A 2-month-old male baby was admitted to our hospital with episodic cyanosis and respiratory failure which required mechanical ventilation. He was found to have upper limb flexion rigidity and poor weight gain since one month old. Progressive muscle stiffness over the abdomen, chest wall, back and four limbs were also noted. He could not be weaned from the ventilator smoothly due to recurrent CO2 retention. Laboratory tests revealed a high serum creatine kinase level. Cytoplasmic body myopathy was confirmed by muscle biopsy. The unusual initial presentations of generalized stiffness and early onset of respiratory failure were quite different from those of patients reported in the literature, who had floppiness, muscular atrophy and weakness. Prednisolone and Vigabatrin were given and the patient showed slight improvement in muscle stiffness and spontaneous movement.

Transient neonatal hypoparathyroidism: report of four cases.

Neonatal hypocalcemia (NH) is common in the neonatal period. Its cause falls into one of two clinical categories, early NH occurs in first 24-48 hours of life; late NH is observed at the end of the first week of life. NH due to congenital hypoparathyroidism, either permanent or transient, is rare. They both present with hypocalcemia, low levels of intact parathyroid hormones, and hyperphosphatemia. In this paper we report on four cases of neonatal hypocalcemia due to transient hypoparathyroidism. They are all full-term infants with normal birth weights, carried by nondiabetic mothers. The age of onset was 6 days old to 17 days old, there were three male and one female. Seizure was the major symptom except for case 2, who had a high pitch crying, irritability and opisthotonus. Laboratory data revealed calcium: 4.7 to 6.3 mg/dl, phosphorus: 6.8 to 9.2 mg/dl, and magnesium: 1.2 to 2.8 mg/dl. The intact parathyroid hormone levels were abnormally low in two cases (<13 pg/ml and 5.7 pg/ml), yet only subnormal in the other two (25.2 pg/ml and 22.2 pg/ml). Further studies on these four babies showed no evidence of Di George syndrome. Interestingly, two patients' mothers were found to have hyperparathvroidism. In conclusion, in case of neonatal hypocalcemia, measurements of calcium, phosphorus, and intact-PTH in neonates are required to recognize hypoparathyroidism. Pediatricians should always check maternal parathyroid status to rule out maternal hyperparathyroidism.

Anomalous origin of one pulmonary artery from the innominate artery: a report of two cases.

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.