Primary hyperparathyroidism is a rare endocrine disease in children. It involves bone and joint, urinary tract, gastrointestinal tract and cardiovascular system. The main cause of these involvement is high level of PTH in serum, resulting in hypercalcemia.An 11 years old male patient who had complained of limping gait since last 18 months, showed typical laboratory and radiological findings of primary hyperparathyroidism. At the ultrasonography, computed tomography and radionuclide scanning, a well defined mass(10 X 15mm) was found on the posterior aspect of the right thyroid lobe. The mass was confirmed histologically as adenoma of parathyroid gland. The patient was successfully treated with subtotal parathyroidectomy and temporal administration of calcium and vitamin D.We report this case of primary hyperparathyroidism with brief review of the literatures.
Adenoma ; Calcium ; Cardiovascular System ; Child ; Endocrine System Diseases ; Gait ; Gastrointestinal Tract ; Humans ; Hyperparathyroidism, Primary ; Joints ; Male ; Parathyroid Glands ; Parathyroidectomy ; Thyroid Gland ; Ultrasonography ; Urinary Tract ; Vitamins

The authors have experienced with two cases of acute pulmonary edema; one underwent an intestinal resection under local anesthesia for panperitonitis due to typhoid perforation, and the other under general anesthesia received splenectomy and mesocaval shunt for portal hypertension and splenomegaly. There are many predisposing factors for acute pulmonary edema, namely, left sided heart failure due to cardiac diseases or overloading, pulmonary capillary endothelial damages from bacterial infections, toxins or irritant gases, oxygen poisoning, water (especially salt water) drowning, rarely central nervous system injuries and pulmonary hypersensitivity reactions. For the cases presented, we believe that overloading was the causative factor. There are many preventive measures and treatment for acute pulmonary edema. However in such cases as these, we conclude that prompt recognition and attention by the anesthesiologisis are the most important preventive measure.
Anesthesia* ; Anesthesia, General ; Anesthesia, Local ; Bacterial Infections ; Capillaries ; Causality ; Central Nervous System ; Drowning ; Gases ; Heart Diseases ; Heart Failure ; Hypersensitivity ; Hypertension, Portal ; Oxygen ; Poisoning ; Pulmonary Edema* ; Splenectomy ; Splenomegaly ; Typhoid Fever ; Water

Granulocytic sarcoma is an uncommon extramedullary localized tumor composed of granulocytic precursor cells. The majority of cases have been reported in association with acute myeloid leukemia(AML) especially in children, but infrequently may occur in patients with myeloproliferative disorders or myelodysplastic syndromes. Most common sites of involvement were bone, soft tissue, lymph nodes and skin, but gastrointestinal tract, testis, central nervous system are also involved rarely. We present an unusual case of granulocytic sarcoma of the intracranium and the retro-orbital soft tissue occurring in a patient with concurrent myelodysplastic syndrome, diagnosed by brain magnetic resonance images and ultrasound guided needle biopsy.
Biopsy, Needle ; Brain ; Central Nervous System ; Child ; Gastrointestinal Tract ; Granulocyte Precursor Cells ; Humans ; Lymph Nodes ; Myelodysplastic Syndromes* ; Myeloproliferative Disorders ; Sarcoma, Myeloid* ; Skin ; Testis ; Ultrasonography

PURPOSE: The periventricular leukomalacia(PVL) tends to increase the risk of developing motor neurologic sequelae, delayed cognitive development, visual impairment, and epilepsy. Although several developmental screening test methods are being used, one of the oldest and best known developmental screening test was restandardized and revised as Denver Development Screening Test II(Denver II). The objective of this study is to analyse the correlation between the degree of PVL on MRI and the results of Denver II. METHODS: Among the children brought into Pusan National University Hospital between January 1996 and August 1999 with developmental delay, all of the 36 children with PVL on MRI were selected for the study. Denver II was checked in all these patients for screening of developmental delay with the review of medical records. Depending on the grade of PVL, total sample was classified into three groups, and it was based on abnormally increased signal intensity in periventricualr white matter or a reduced amount of periventricular white matter or both, and compensatory focal ventricular enlargement. We analysed the relationship of the grade of PVL and the results of Denver II. RESULTS: The 36 total patients were composed of 22 boys and 14 girls, with the age distribution between 11 to 72 months and the mean of 34.4 months. Delayed occurrence of gross motor sector were 5 cases(44.4%) in group 1, 16 cases(80.0%) in group 2, 7 cases(100.0%) in group 3. Incidence of delay was significantly higher in the high grade PVL group. Delayed rate of other sectors(fine motor-adaptive, personal-social) were higher in the high grade PVL group. But it was not statistically significant. Delayed rate of language sector has no correlation with grade of PVL on MRI. CONCLUSION: Incidence of dealy of gross motor sector was significantly higher in the high grade PVL group. However, language sector has no correlation with grade of PVL on MRI.
Age Distribution ; Brain* ; Busan ; Child ; Epilepsy ; Female ; Humans ; Incidence ; Magnetic Resonance Imaging* ; Mass Screening* ; Medical Records ; Vision Disorders

PURPOSE: Periventricular leukomalacia(PVL) is associated with various neurologic sequelae such as cerebral palsy and cortical blindness. The aim of this study was to analyse the correlation between the degree of PVL on MRI and clinical features or its severity. METHODS:Thirty-eight children with PVL on MRI among children brought to Pusan National University Hospital between January 1996 and August 1999 with development delay, cerebral palsy or epilepsy were included into the study group. We reviewed medical records of the patients including the gestational age, birth weight and neurologic sequelae. The grade for PVL was divided into 1 to 3, and it was based on abnormally increased signal intensity in periventricular white matter or a reduced amount of periventricular white matter or both, and compensatory focal ventricular enlargement. We analysed the relationship of the grade of PVL and various clinical findings. RESULTS: The age ranged from 11 months to 13 years old with a mean of 42 months. History of cesarean delivery and ventilator care were significantly frequent for the severe grade of PVL. Birth weight was significantly lower according to grade of PVL. Gestational age was lower according to the grade of PVL, but was not significant statistically. Incidence of cerebral palsy was significantly higher on the severe grade of PVL. Incidence of epilepsy and mental retardation were 34.2Yo and 23.9M, not associated with the grade of PVL. CONCLUSION: Incidence of cerebral palsy was associated with the severe grade of PVL, so we think MRI can be helpful in predicting neurodevelopmental outcome. (J Korean Pediatr Soc 2000;43 798-805)
Adolescent ; Birth Weight ; Blindness, Cortical ; Brain* ; Busan ; Cerebral Palsy ; Child ; Epilepsy ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Leukomalacia, Periventricular* ; Magnetic Resonance Imaging* ; Medical Records ; Ventilators, Mechanical

Familial hemiplegic migraine(FHM) is an autosomal dominant subtype of migraine with aura, characterized by the occurrence of hemiplegia during the aura. Two subforms of FHM families exist; pure FHM in 80% and FHM families with cerebellar symptoms in 20%. Half of the known FHM families show genetic linkage to chromosome 19p13, and in these families FHM is caused by missense mutations in a neuronal P/Q type calcium channel alpha-1 subunit gene(CACNA1A gene). Linkages to 1q31 and 1q21-23 have also been established. Other families are linked neither to chromosome 19 nor 1. Clinical variabilities are partially associated with the various types of CACNA1A gene mutations. FHM is distinguished from more frequent migraine types by a clear, dominant inheritance pattern and the relative absense of other headache types. Further investigation of FHM will help to clarify the genetics of more common migraine. We describe a male patient with FHM with a brief review of the literature.
Calcium Channels ; Chromosomes, Human, Pair 19 ; Epilepsy ; Genetic Linkage ; Genetics ; Headache ; Hemiplegia ; Humans ; Inheritance Patterns ; Male ; Migraine Disorders ; Migraine with Aura* ; Migraine without Aura ; Mutation, Missense ; Neurons

PURPOSE: This study was performed to find out the role of routine lumbar puncture in children presented with their first seizure with Fever. METHODS: This study included 220 children, over a 5 year period, from April 1999 to March 2003, who visited or were admitted at Dae Dong Hospital with their first febrile convulsion. Lumbar puncture was performed in all children. We analyzed their age, family history, type of seizure, duration of seizure, cause of fever, and the results of lumbar puncture. RESULTS: In the sex distribution, males(58.6%) outnumbered females(41.4%) and the ratio was 1.2:1. 81.7% of the patients had febrile convulsion from 6 months- to 3 years of age(P<0.05). 30.5% also had family history of febrile convulsion. The types of seizure were generalized tonic-clonic(72.7%), generalized tonic(17.7%), and generalized clonic(6.4 %). For the duration of seizure, 90.4% of the patients were estimated less than 15 minutes(P<0.05). The causes of fever were pharyngotonsilitis(40.5%), gastroenteritis(19.1 %), pneumonia or bronchitis(13.2%), meningitis(11%), otitis media, urinary tract infection, and exanthem subitum. However, 9.1% of the patients were diagnosed meningitis, with 18 of 20 patients under 3 years of age. CONCLUSION: In the first seizure with fever, lumbar puncture is a useful method for meningitis, especially under 3 years of age.
Child* ; Exanthema ; Fever* ; Humans ; Meningitis ; Otitis Media ; Pneumonia ; Seizures* ; Seizures, Febrile ; Sex Distribution ; Spinal Puncture* ; Urinary Tract Infections

We retrospecively analyzed MRI findings of 14 patients with intracranial tuberculosis according to the disease process before anti-tuberculous medication. We also analyzed the correlativity between these findings and the clinical outcome after anti-tuberculous medication in 8 patients who had taken follow-up MR imaging. MR imaging was performed with either a 0.5T(1 patients) or 1.0T(13 patients) superconducting system with spinecho pulse sequences. Abnormalities on MRI were seen in 87%(12/14) of the patients. MRI findings consisted of meningeal enhancement(n=10), parenchymal tuberculoma(n=6), hydrocephalus(n=5), and infarction(n=5). Most of them were found within 3 months from symptom-onset. Parenchymal tuberculomas were found after 1 month of symptom-onset. All cases of hydrocephalus and infarction were associated with meningeal enhancement. On follow-up MRI after anti-tuberculous medication, changes of MR imaging did not correlate with clinical outocome in 4(50%) of 8 patients. Improvement of MRI findings were observed in patient treated for more than 3 weeks. In conclusion, MRI is a good modality in the diagnosis of intracranial tuberculosis, however, the MRI findings are not always correlated with clinical outcome after, anti-tuberculous medication.
Diagnosis ; Follow-Up Studies ; Humans ; Hydrocephalus ; Infarction ; Magnetic Resonance Imaging* ; Tuberculoma ; Tuberculosis*

Postpartum hemorrhage is an important cause of maternal mortality and morbidity. Especially uterine atony is the most common cause of postpartum hemorrhage. Conventional method to control postpartum uterine atonic bleeding is based on the use of oxytocin and ergot preparations. Prostaglandin F2alpha analogue such as carboprost can be used to promote contraction when these agents fail to produce uterine contraction. Prostaglandin E1 analogue, misoprostol has uterotonic effect by oral or vaginal administration. They are used to induce labor and first or mid trimester abortion. In postpartum uterine atonic bleeding, misoprostols cannot be used via oral or vaginal route. Recently we have experienced that postpartum uterine atonic bleedings unresponsive to conventional methods were controlled by rectal misoprostols. So we report these cases with a brief review of literatures.
Administration, Intravaginal ; Alprostadil ; Carboprost ; Dinoprost ; Hemorrhage* ; Maternal Mortality ; Misoprostol* ; Oxytocin ; Postpartum Hemorrhage ; Postpartum Period* ; Uterine Contraction ; Uterine Inertia

BACKGROUND/AIMS: According to the recent research, mutations in the HBV pre-S region may have an impact on the progression of hepatitis B virus(HBV)-related liver disease. The aim of this study was to clarify the frequency and location of naturally occurring mutations in the pre-S region of HBV, and their possible effects on the clinical course of HBV-associated chronic liver diseases. METHODS: HBV DNA was extracted from the sera of 15 patients (8 with liver cirrhosis and 7 with hepatocellular carcinoma). The pre-S sequence was amplified via polymerase chain reaction, subcloning and sequenced. RESULTS: All patients had point mutations in the pre-S region. Nine of 10 mutation sites (90%) in the pre-S1 region, and 4 of 5 mutation sites (80%) in the pre-S2 region were identical in both liver cirrhosis and hepatocellular carcinoma. Deletions were detected in seven patients (4 with liver cirrhosis and 3 with hepatocellular carcinoma). Among the 4 patients with liver cirrhosis, three had deletion in 5'-end of the pre-S2 region and one spanning the 3'-end of the pre-S1 to 5'-end of the pre-S2 region. All 3 patients with hepatocellular carcinoma had deletions in 5'-end of the pre-S1 region, and two patients had simultaneous deletion spanning the 3'-end of the pre-S1 to the 5'-end of the pre-S2. CONCLUSION: The pre-S mutants were frequently detected in HBV-associated liver cirrhosis or hepatocellular carcinoma and the point mutations or deletions in the pre-S gene were clustered in specific regions.
Carcinoma, Hepatocellular* ; DNA ; Hepatitis B ; Humans ; Liver Cirrhosis* ; Liver Diseases ; Liver* ; Point Mutation ; Polymerase Chain Reaction