• Childhood Kidney Diseases
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• v.7 no.2
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• pp.229-233
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• 2003

Mortality and morbidity of malignant hyperthermia has decreased markedly by the avoidance of succinylcholine, and the earlier detection and introduction of dantrolene. We report a fourteen-year-old boy who developed malignant hyperthermia during general anesthesia. He showed the earlier clinical signs, such as elevation of end-tidal $CO_2$, tachycardia, and hypertension. After prompt administration of dantrolene, operation was continued with profopol and midazolam. Rhabdomyolysis and myoglobinuria followed, and were managed by hydration and alkalinization of urine. Azotemia did not occur, and he was discharged without any sequelae on the $10^{th}$ postoperative day.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.83-90
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• 2005

Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of Idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin h(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehyIPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained In the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.94-99
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• 2010

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.132-136
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• 2013

Parainfluenza virus infection is one of the causes of fatal rhabdomyolysis. Rhabdomyolysis can be aggravated by mitochondrial fatty acid ${\beta}$-oxidation disorders during prolonged periods of fasting. Moreover, in patients with late-onset isovaleric acidemia, hyperammonemia may occur following catabolic stress. In the present report, we describe a case of a 4-year-old boy with parainfluenza virus infection and late-onset isovaleric acidemia that rapidly progressed to coma, seizures, and cardiorespiratory collapse. His serum ammonia and creatinine kinase (CK) levels were $385{\mu}Mol/L$ and 23,707 IU/L, respectively. Continuous renal replacement therapy (CRRT) was initiated using continuous venovenous hemodiafiltration, after which the ammonia and CK levels returned to normal. Thus, we recommend the immediate initiation of CRRT in the management of patients with life-threatening rhabdomyolysis and hyperammonemia.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.120-125
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• 2020

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.74-78
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• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Journal of Korean Neurosurgical Society
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• v.29 no.4
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• pp.564-568
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• 2000

The authors present a rare case of subependymal giant cell astrocytoma which successfully treated with endoscopic tumor resection. A 15-year-old boy was presented with multiple episode of generalized seizure and intermittent headache for 5 years. The patient had facial adenoma sebaceum, multiple nevi on his back, and mental retardation. Cerebral MRI scans showed a round tumor located at the frontal horn of right ventricle near the foramen monro. The tumor was totally removed with endoscopic procedure and histologically confirmed as a subependymal giant cell astrocytoma. Subependymal giant cell astrocytomas can cause all the clinical problems that are associated with other types of intracranial tumor. However, surgical indication is not well standardized, because of the lack of basic knowledge about the tumor and the likelihood of surgical morbidity. We believe that endoscopic surgery, a minimally invasive technique, can circumvent the surgical disadvantage while achieving the surgical goal.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.209-214
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• 2013

Multiple caries in a pediatric patient often requires dental treatment under general anesthesia, especially when the patient is suffering from a systemic disease. The patient was a 6-year-old boy with Marfan syndrome and needed extensive dental treatment. Marfan syndrome is an inherited disorder resulting from mutations in Fibrillin-1 gene. Patients are known to have mainly cardiovascular, ocular, and musculoskeletal problems. Although clinical symptoms of the syndrome are age-realted, thus hindering early diagnosis of the disease in young children, our patient had been confirmed by a gene study at a younger age. Medical history of the patient revealed moderate to severe mitral regurgitation and aortic root dilatation, which required mitral valve replacement surgery with a mechanical valve. As a result, the patient was taking warfarin post-operatively and changes in medication had to be made before the dental treatment. Also, prophyalctic antibiotics had to be given before the treatment for prevention of (to prevent the) infective endocarditis. With careful control of the medications and bleeding tendency, general anesthesia and the treatment were done successfully without any complications.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.203-207
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• 2013

Aplastic anemia (AA) is a serious hematologic disease characterized by hypocellular bone marrow and deficient production of erythrocytes, granulocytes and platelets. Serious complications such as uncontrolled bleeding and bacteremia can occur. A case of severe AA are presented with dental considerations. A 4-year-old boy had been referred from Seoul National University Hospital for dental examination before the hematopoietic stem cell transplantation (HSCT). Treatments were planned under general anesthesia, due to the poor compliance. Following medical consult, dental treatments were performed after platelet transfusion and antibiotic prophylaxis. Postoperatively, neither significant bleeding nor complictation was observed. On the time of the treatment planning. the anesthesiologist and dentist should perform a complete hematological assessment. It is imperative not only platelet counts but also other leukocyte counts are under safe boundaries. It is mandatory to follow strict aseptic precautios for all anesthetic and surgical maneuvers. In severe thrombocytopenic patients, platelet transfusion should be considered. Also, it is recommended to establish a good oral hygiene.

• Archives of Craniofacial Surgery
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• v.12 no.2
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• pp.102-106
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• 2011

Purpose: It is accepted universally that correction of the cleft lip nasal deformity requires multiple stages of surgery. Following primary lip repair in infancy or early childhood, secondary surgery to improve the deformity of the lip and nose is frequently necessary. A suitable surgical procedure to correct the accompanying deformity, such as cleft palate and alveolus, must be carried out at an appropriate age. In developing countries, it is common for patients with cleft lip nasal deformity to present severe secondary deformities in adolescence, because of poor follow-up and inappropriate surgery. Methods: The first patient was a 12 year old Mongolian boy. He presented prominent lip scar, short lip, wide columella, asymmetric nostril, palatal fistula, cleft alveolus, and velopharyngeal incompetence. He underwent cheilorhinoplasty, transpositional flap, alveoloplasty by iliac bone graft, and sphincter pharyngoplasty. On follow-up, a bilateral maxillary hypoplasia and a class III malocclusion developed. He underwent LeFort I osteotomy and maxillary advancement at the age of 16 years. The second patient was an 18 year old Eastern Russian girl. She presented with a deviated nose, right alar base depression, short lip, protrusion on vermilion, large palatal fistula, and severe VPI due to short palate. She underwent the combined procedure of cheilorhinoplasty, corrective rhinoplasty, tongue flap for palatal fistula, and superiorly based pharyngeal flap. And the tongue flap was detached at postoperative 3 weeks. Results: The overall results have been extremely pleasing and satisfactory to patients. There were no postoperative complications. Conclusion: We discovered the one stage operation for radical correction was sufficient procedure to provide excellent clinical outcomes in patients with severe cleft lip nose deformity.