Thrombotic Thrombocytopenic Purpura as the First Symptom of Systemic Lupus Erythematosus: A Case Report with Review of Literature / 임상소아혈액종양

Thrombotic thrombocytopenic purpura (TTP) can cause serious morbidity and mortality, and differentiating between this disease and systemic lupus erythematosus (SLE) can prove challenging. Although rare, TTP accompanied by SLE is linked to several complications and a higher mortality rate. Herein, we report a case of a 16-year-old boy who presented with systemic symptoms, such as petechiae, and was diagnosed with acquired TTP following a laboratory test. Steroid treatment was initiated and a diagnosis of SLE was reached after the symptoms had improved. Treatment with low-dose prednisone in addition to hydroxychloroquine was continued. The patient did not develop renal failure or neurologic deficit. No specific symptoms were observed after treatment and during the follow-up period. Early treatment of SLE is crucial, but it is difficult to reach an early diagnosis because the symptoms are similar to those of TTP. In the current study, an early diagnosis of TTP led to prompt treatment, thereby avoiding the fatal symptoms that could be caused by SLE.

Toxic Megacolon as a Complication of Infectious Colitis Caused by Salmonella enteritidis Group D in a Previously Healthy Child

Toxic megacolon is a fatal complication of inflammatory or infectious bowel disease.Prognosis depends on the severity of the disease. In cases of poor prognosis, clinical outcomes range from intestinal resection to death, so early diagnosis and appropriate treatment are very important. However, the prevalence of toxic megacolon in children is very low, and in those without underlying diseases such as inflammatory bowel disease, early diagnosis may be delayed. A previously healthy 12-year-old boy presented to our hospital with lower abdomen pain, fever, and hematochezia. Despite antibiotic therapy, the symptoms worsened. On the third day, abdominal computed tomography revealed severe dilatation of the transverse colon, which indicated toxic megacolon. Stool culture was positive for Salmonella enteritidis group D, and rectal endoscopy showed no signs of inflammatory bowel disease. Ceftriaxone and intravenous methylprednisolone were administered, and the patient's condition improved without any complications. We report a case of toxic megacolon as a complication of infectious colitis caused by S. enteritidis group D, which was diagnosed using early imaging and successfully treated without surgical intervention.

Toxic Megacolon as a Complication of Infectious Colitis Caused by Salmonella enteritidis Group D in a Previously Healthy Child

Toxic megacolon is a fatal complication of inflammatory or infectious bowel disease.Prognosis depends on the severity of the disease. In cases of poor prognosis, clinical outcomes range from intestinal resection to death, so early diagnosis and appropriate treatment are very important. However, the prevalence of toxic megacolon in children is very low, and in those without underlying diseases such as inflammatory bowel disease, early diagnosis may be delayed. A previously healthy 12-year-old boy presented to our hospital with lower abdomen pain, fever, and hematochezia. Despite antibiotic therapy, the symptoms worsened. On the third day, abdominal computed tomography revealed severe dilatation of the transverse colon, which indicated toxic megacolon. Stool culture was positive for Salmonella enteritidis group D, and rectal endoscopy showed no signs of inflammatory bowel disease. Ceftriaxone and intravenous methylprednisolone were administered, and the patient's condition improved without any complications. We report a case of toxic megacolon as a complication of infectious colitis caused by S. enteritidis group D, which was diagnosed using early imaging and successfully treated without surgical intervention.

Predictive value of C-reactive protein for the diagnosis of meningitis in febrile infants under 3 months of age in the emergency department / 영남의대학술지

Background@#Fever is a common cause of pediatric consultation in the emergency department. However, identifying the source of infection in many febrile infants is challenging because of insufficient presentation of signs and symptoms. Meningitis is a critical cause of fever in infants, and its diagnosis is confirmed invasively by lumbar puncture. This study aimed to evaluate potential laboratory markers for meningitis in febrile infants. @*Methods@#We retrospectively analyzed infants aged 10 mg/L.

The Impact of Perioperative Use of a Statin–Magnesium Combination on Opioid Consumption in Patients Who Underwent Cardiac Surgery: A Retrospective Study with Propensity-Score Matching / 전남의대학술지

Both statins and magnesium are associated with NMDA receptors and anti-inflammatory effect. Peripheral NMDA receptors are known to be involved in inflammationinduced pain. This study aimed to investigate the impact of perioperative use of a statin– magnesium combination on opioid consumption in patients who underwent cardiac surgery. This was a retrospective study of 542 patients who underwent cardiac surgery. The patients were divided into two groups according to statin use: the statin–magnesium group (n=375) and the magnesium-only group (n=167). Patients in the magnesium- only group received only perioperative magnesium, but no statins, to prevent atrial fibrillation following cardiac surgery. After propensity-score matching, 228 patients (n=114 in each group) were analyzed to investigate opioid consumption, visual analogue scale (VAS) pain scores over a 72-h period, and pain outcomes according to the duration of statin treatment (<1 or ≥1 yr). The consumed opioid volume was significantly smaller in statin–magnesium group than the magnesium-only group, both before (p=0.010) and after matching (p=0.017). The statin–magnesium combination did not significantly reduce the VAS pain scores compared with magnesium alone. Although the statin–magnesium combination did not significantly reduce the pain intensity compared with magnesium alone, the combination therapy was effective in reducing opioid consumption after surgery.

Prevalence of Pathological Brain Lesions in Girls with Central Precocious Puberty: Possible Overestimation?

BACKGROUND: Brain magnetic resonance imaging (MRI) is routinely performed to identify brain lesions in girls with central precocious puberty (CPP). We aimed to investigate the prevalence and type of brain lesions among Korean girls with CPP and evaluate the need for routine brain MRI examinations. METHODS: This retrospective cross-sectional study evaluated data on 3,528 girls diagnosed with CPP from April 2003 to December 2016, and identified 317 girls who underwent sellar MRI. Exclusion criteria were patients with a known brain tumor or who did not undergo brain MRI due to refusal or the decision of the pediatric endocrinologist. RESULTS: Normal sellar MRI findings were observed in 291 of the 317 girls (91.8%). Incidental findings were observed in 26 girls (8.2%). None of the patients had pathological brain lesions. CONCLUSION: The prevalence of intracranial lesions among girls who were generally healthy and without neurological symptoms but diagnosed with CPP was lower than that previously reported. Furthermore, none of the identified lesions required treatment. It may be prudent to reconsider the routine use of brain MRI to screen all patients with CPP, especially if they are healthy and neurologically asymptomatic, and are girls aged 6–8 years.

Predictors of transient congenital hypothyroidism in children with eutopic thyroid gland

PURPOSE: Congenital hypothyroidism (CH) is the most common cause of preventable mental retardation. Recently, the detection of CH cases with eutopic thyroid gland has increased due to neonatal screening programs. In this study, we aimed to identify and evaluate predictive factors that could distinguish between permanent and transient CH in patients with eutopic thyroid gland. METHODS: We retrospectively reviewed 100 children diagnosed with CH and with eutopic thyroid gland. All subjects were treated with levothyroxine and underwent re-evaluation after 3 years of age. RESULTS: Of the 100 CH patients, 35 (35.0%) were diagnosed with permanent CH (PCH) and 65 (65.0%) were diagnosed with transient CH (TCH). The initial thyroid stimulating hormone levels were significantly lower in the TCH subjects than in PCH subjects. In addition, the mean doses of levothyroxine (µg/kg/day) at the 1st, 2nd, and 3rd year of treatment were significantly lower in subjects with TCH than in PCH subjects with eutopic thyroid gland. Based on the receiver operating characteristic (ROC) curve, the optimal cutoff dose of levothyroxine at 3 years of 2.76 µg/kg/day could predict TCH, and was associated with 87.3% sensitivity and 67.6% specificity, with an area under the ROC curve of 0.769. CONCLUSION: The levothyroxine dose requirement during treatment period has a predictive role in differentiating TCH from PCH in CH patients with eutopic thyroid gland.

Obstetric and Neonatal Outcomes of Teenage Pregnancies

PURPOSE: The number of teenage pregnancy is increasing on these days due to changes in sexual awareness, precocious puberty, lack of proper sex education and abortion restrictions. We evaluated the clinical features including the rate and perinatal complications of mother and neonates caused by teenage births. METHODS: Subjects were mothers whose ages were below 19 at time of admission in Wonkwang University Hospital from January 2000 to July 2011. We reviewed the medical records of teenage mothers and her neonates retrospectively, and investigated the differences between above and below the age of 16. RESULTS: The number of neonates of teenaged mothers was 73, which is 10.4 per 1,000 births and it showed an upward trend during the late decade of 2,000's. 14 (19.2%) of the mothers were multiparous, and 69 (94.5%) unmarried, 25 (34.2%) no prenatal examinations, 11 (15.1%) an abortion history, 47 (64.4%) middle or high school students, and 25 (34.2%) a single parent. Among the maternal complications, premature birth was the most prevalent with 34 cases (46.6%), followed by 11 (4.1%) of premature rupture of membrane. The most common neonatal complication was prematurity in 37 cases (50.7%), followed by 30 (41.1%) low birth weight, 19 (26%) ICU admission. There were no significant differences between above and below the age of 16 in neonatal and obstetrical complications. CONCLUSION: The number of teenage pregnancy is increasing recently, and they had a high prevalence rate of prematurity, low birth weight and respiratory distress syndrome. And, the grouping by age of above and below 16 was no affect the prognosis.

A Case of Hemolytic Disease of Newborn due to Anti-JKa / 대한주산의학회잡지

The Kidd system of blood group is clinically significant since the anti-Kidd antibody can cause immediate or delayed hemolytic transfusion reactions as well as hemolytic disease of newborn (HDN). In contrast to the hemolytic activity of Kidd antibodies in incompatible transfusion, the severity of HDN varies but usually tends to be a benign clinical course with favorable outcomes. And severe HDN due to anti-JKa incompatibility is a rare, which is still not reported in Korea. We experienced a newborn with severe jaundice to be detected within 24 hours from birth due to anti-JKa incompatibility. The laboratory data on admission indicated hemoglobin 13.2 g/dL, reticulocyte 4.08%, total bilirubin 18.7 mg/dL, and direct and indirect antiglobulin tests were positive (2+/2+). But, there are no ABO and Rh (D) incompatibilities between the mother and newborn. Maternal serum was found to contain an antibody that recognized JKa , and also seen in neonate. This patient completely recovered with intensive phototherapy without exchange transfusion. We report this case with a brief review of relevant literature.

A Case of Hemolytic Disease of Newborn due to Anti-JKa / 대한주산의학회잡지

The Kidd system of blood group is clinically significant since the anti-Kidd antibody can cause immediate or delayed hemolytic transfusion reactions as well as hemolytic disease of newborn (HDN). In contrast to the hemolytic activity of Kidd antibodies in incompatible transfusion, the severity of HDN varies but usually tends to be a benign clinical course with favorable outcomes. And severe HDN due to anti-JKa incompatibility is a rare, which is still not reported in Korea. We experienced a newborn with severe jaundice to be detected within 24 hours from birth due to anti-JKa incompatibility. The laboratory data on admission indicated hemoglobin 13.2 g/dL, reticulocyte 4.08%, total bilirubin 18.7 mg/dL, and direct and indirect antiglobulin tests were positive (2+/2+). But, there are no ABO and Rh (D) incompatibilities between the mother and newborn. Maternal serum was found to contain an antibody that recognized JKa , and also seen in neonate. This patient completely recovered with intensive phototherapy without exchange transfusion. We report this case with a brief review of relevant literature.

Hypernatremia and Intraventricular Hemorrhage in Very Low Birth Weight Infants (<1,250 g)

PURPOSE: Hypernatremia most frequently occurs in the immature newborn and be severe in association with intraventricular hemorrhage (IVH). This study examined the frequency, onset and risk factors of hypernatremia, and the relationship between hypernatremia and IVH in very low birth weight (VLBW; or =150 mEq/L) and nonhypernatremia group, and were compared. RESULTS: Incidence of hypernatremia in the VLBW infants was 52.7%, and mean starting time of hypernatremia was 2.8+/-1.3 days. There were no differences in the sodium and fluid intake between the two groups. Weight loss at day 3 after birth was significantly higher in the hypernatremia compared to the nonhypernatremia group (P<0.05); thereafter weight loss was non-significantly higher. The incidence of IVH in VLBW infants was 38.2%, and the difference between the two groups was not significant. CONCLUSION: Hypernatremia occurs commonly in VLBW infants and is most commonly caused by weight loss in the early days after birth. Incidence of IVH is not likely influenced by hypernatremia with marginally elevated sodium concentration.

A Case of Supernumerary Derivative (22) Syndrome Resulting from a Paternal Balanced Translocation

Supernumerary derivative (22) syndrome is a rare genomic syndrome. It is characterized by severe mental retardation, microcephaly, failure to thrive, preauricular tag or sinus, ear abnormalities, cleft and/or high-arched palate, micrognathia, kidney abnormalities, congenital heart defects, and genital abnormalities in males. In 99% of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. To date, there have been about 100 case reports of supernumerary derivative (22) syndrome. In most of the cases, supernumerary derivative (22) syndrome was the result of 3:1 meiotic segregation in the maternal 11;22 translocation carrier. We now report a case of 47,XX, + der(22)t(11;22)(q23;q11.2) resulting from 3:1 meiotic segregation of the paternal translocation carrier.