Question: Is polycystic ovary syndrome (PCOS) a risk factor for nonalcoholic fatty liver disease (NAFLD) in adolescents? Finding: The frequency of NAFLD did not increase in adolescents with PCOS. However, hyperandrogenemia was a risk factor for NAFLD. Meaning: Adolescents with PCOS and hyperandrogenemia should be closely monitored for hepatic steatosis. |
Carnitine (β-hydroxy-γ-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic... |
Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea... |
Transient neonatal diabetes mellitus (TNDM) has been associated with paternal uniparental isodisomy of chromosome 6, paternally inherited duplication of 6q24, or a methylation defect at a CpG island of the ZAC or HYMAI gene. We experienced a case of TNDM in which the patient presented with hyperglycemia, macroglossia, and intrauterine growth retardation, caused by a paternally derived HYMAI. An 18-day-old... |
Purpose : Transient tachypnea of the newborn (TTN) is usually benign and improves within 72 hours. However, it can also progress to prolonged tachypnea over 72 hours, profound hypoxemia, respiratory failure, and even death. The aim of this study is to find predictable risk factors and describe the clinical courses and outcomes of prolonged TTN (PTTN). Methods : The medical records... |
Transient tachypnea of the newborn (TTN), respiratory distress syndrome (RDS), and persistent pulmonary hypertension (PPHN) are the three most common disorders that cause respiratory distress after birth. An understanding of the pathophysiology of these disorders and the development of effective therapeutic strategies is required to control these conditions. Here, we review recent papers on the pathogenesis and treatment of neonatal... |
Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. This disease usually produces no symptoms, but hematuria, uric acid nephrolithiasis or acute renal failure may develop. A defect in the SLC22A12 gene, which encodes the human urate transporter, is the known major cause of this disorder. We describe a 10-month-old boy with idiopathic renal... |
Purpose : The aim of study is to investigate the clinical manifestations and the etiology of markedly-elevated serum alkaline phosphatase(ALP) levels in children without accompanying liver or bone disease. Methods : The serum ALP and other biochemical laboratory data of 4,989 patients attending the pediatric department of Ewha Womans University Mokdong Hospital between January 2002 to December 2002 were studied. The... |
Purpose : Infantile transient methemoglobinemia(ITM) may develop in association with infectious diarrhea without exposure to any toxic oxidizing agents. We observed that the number of ITM associated with infectious diarrhea have increased at the Gyeongsang National University Hospital (GNUH), located in the western area of Kyungnam province during the last 4 years. To determine whether this phenomenon was similarily observed... |
We examined a male infant with transient increase in alkaline phosphatase(ALP) activity. The 25-month-old infant was admitted to a local hospital because of pneumonia of unknown etiology. Initial laboratory investigation revealed a serum ALP of 11,260U/L, which was comparable to that of bone isoenzyme on the electrophoresis pattern. There was no evidence of hepatitis, skeletal or intestinal diseases. The hyperphosphatasemia... |
Sometimes physicians and clinical pathologists find very high levels of alkaline phosphatase(ALP) activity in their patients. This condition is associated with transient, marked increase in serum ALP in healthy infants and children. It has also been described in adults. Clinical and biochemical features of transient hyperphosphatasemia in infancy and early childhood are reviewed in six patients that we have studied. The diagnosis is suggested... |
Purpose : To help differentiate transient hypothyroidism from congenital hypothyroidism, both of which might be confused initially, the clinical characteristics of transient hypothyroidism were compared to those of permanent ones. Methods : An analysis of the clinical data, including perinatal history, TFT(thyroid function test), and radionuclide scanning, was performed. The outcome of 18 patients of transient hypothyroidism was also compared to... |
Transient neonatal pustular melanosis(TNPM) is a benign, self-limited, cutaneous disorder of unknown etiology. Significant physical findings are limited to the skin. Hematologic and serological tests are normal. Cultures of blood and pustule are negative. Biopsy specimens from pustules show a intracorneal or subcorneal separation containing neutrophils and also some eosinophils. TNPM produces only cutaneous lesions and requires no treatment. So... |
Individuals with Down syndrome have a high incidence of hematologic diseases such as transient myeloproliferative disorder(TMD) & acute leukemia. Because it is difficult to distinguish TMD from acute myeloblastic leukemia, the diagnosis in neonate, who have Down syndrome, should be made with extreme caution. TMD usually undergoes spontaneous remission within a few months, but acute leukemia can develope after remission... |
Purpose : In concern of diagnosis and treatment of transient idiopathic hypocalcemia, a major cause of infantile, we studied the onset of age, clinical characteristics, laboratory findings, and duration of treatment and recovery of transient idiopathic hypocalcemia. Methods : The author observed 19 infants with transient idiopathic hypocalcemia who had been admitted to Yonsei university hospital due to convulsion for 4... |
Transient tachypnea of the newborn(TTN) is a benign self-limited disease characterized by early onset and rapid recovery of tachypnea although it may occasionally have a more prolonged and protracted course. A retrospective clinical study was mad on 29 neonates with TTN admitted to NICU of Pohang St. Mary's Hospital from January 1992 to June 1993. The results were as follows: 1) TTN(36.2%) was... |
methemoglobinemia is an uncommon clinicl problem generally caused by inherited disorders of hemoglobin metabolism or environmental toxicity from oxidizing agents. Since methemoglobin has no oxigen carrying capacity, patient with severe methemoglobinemia may have dangerous hypoxia even when arterial oxygen tension is normal. Degree of exposure to oxidants which are benign for older individuals may produce severe methemoglobinemia in newborns. Acidosis without... |
Eight neonates with transient tricuspid insufficiency are presented which was confirmed clinical and two dimensional echocardiographic assessment. We found that two dimensional Doppler echocardiography was very useful in the detection of transient tricuspid insufficiency during neonatal age as noninvasive method. Transient tricuspid insufficiency is a clinical disorder in the newborn period caused by myocardial dysfunction, secondary to asphyxia with or without... |
A transient heart murmur is frequently heard in normal newborn who has no congenital heart disease. The cause of this murmur remains unclear but was speculated as hypoplasia of the pulmonary arterial branches or unusual alignment of the main pulmonary artery with its branches. Pulsed Doppler and two-dimensional echocardiographic studies were undertaken in 19 newborn infants with a transient murmur and... |
We have experienced a case of transient erythroblastopenia of childhood in a 7 years old male patient who was diagnosed by bone marrow aspiration. His hematologic improvement occurred spontaneously. A brief review of related literature was done. |
Transient myeloproliferative disorder has been reported in infants with Down’s syndrome. This disorder is clinically and hematologically indistinguishable from congenital leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. We experienced a case of Transient myeloproliferative disorder with Down’s syndrome. This 4-day-old female neonate showed jaundice, hepatomegaly, fever, dyspnea, mongoloid face and congenital heart... |
Acrdermititis enteropathica is a rare autosomal recessive disorder of zinc absorption and its cardinal symptoms appear at the time of weaning from breast milk which has been referred to as most appropriate formula to prevent and treat this disease. However, recent reports presented Transient Symptomatic Zinc deficiency (TSZD) in infants fed breast milk whom they treated with transitory zinc supplement with no recurrence. We experienced... |
Children with Down syndrome have an increased incidence of acute leukemia. Infants with Down syndrome are also at risk of developing a transient myeloproliferative disorder indistinguishable from acute nonlymphocytic leukemia (ANLL) except by its eventual clinical recovery. We observed 11 patients with acute leukemia or transient myeloproliferative disorder in Down syndrome who had admitted to the Departmetn of Pediatrics, Seoul National University Children’s Hospital,... |
Many authors have reported alterations in the ABR in severe hyperbilirubinemia and ABR is an effective non-invasive method of assessing the functional status of the auditory nerve and brainstem sensory pathway in the management of neonatal hyperbilirubinemia. Perlman et al reported that jaundice in the early neonatal period is associated with significant transient aberration of ABR, suggestive of a transient bilirubin encephalopathy (TBE). And... |
Transient Tricuspid Insufficiency(TTI) of newborn is a feature of myocardial dysfunction caused by myocardial hypoxemia secondary to reduced arterial oxygen content and excessive right ventricular work consequent to pulmonary hypertension.' Since Doppler echocardiography has been used to diagnose cardiac disease, it allows recording of regurgitant turbulent flow pattern. We report one case of TTI in a 36 week-gestational-aged newborn detected by continuous wave Dopplar echocardiography... |
Transient myeloproliferative disorder has been reported in infant with Down's syndrome during neonatal period. This is clinically and hematologically indistinguishable from congenital acute leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. A neonate presented with jaundice, hepatosplenomegaly and mongoloid face was diagnosed as Transient myeloproliferative disorder. We report this case with a review of... |
Transient Neonatal D.M. is rare disease which is characterized by letharginess, dehydration and weight loss with severe hyperglycemia. It occurs frequently which small for data infant and recovers spontaneously if appropriate insulin treatment was taken. We had expirienced a case of Transient Neonatal D.M. in 15 days old female infant. The review of the literature was made briefly. |
The authors studied 36 cases of wet lung disease who were born and admitted to pediatric department of Severance hospital from January 1981 to June 1984. following results were obtained: 1)The incidence of wet lung disease was 0.5% and the predisposing factors were male sex(78%), full term(75%), C-section(58%) and multipara(72%). 2)Number of cases whose Apgar score were above 7 werp... |
Transient myeloproliferative disorder has been reported in infants with Down syndrome. Infants with Down syndrome and this transient myeloproliferative disorder often present with signs and symptoms that are clinically and hematologically indistinguishable from congenital acute myelogenous leukemia. In contrast to congenital AML, complete clinical and hematological recovery occurs within weeks to months of diagnosis without any specific anti- leukemic treatment. A case of transient myeloproliferative disorder... |
We experienced a case of transient complete A-V block, which developed probably due to acute myocarditis of viral etiology. The patient was 13 year old boy, and was admitted to our ward with chief complaints of intermittent abdominal pain and chest discomfort. The auscultation of the heart revealed, the 1st heart sounds varied in intensity, and the E.K.G. findings, checked... |