• Journal of Chest Surgery
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• v.22 no.3
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• pp.514-517
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• 1989

Congenital esophageal stenosis is a rare disease in childhood. By virtue of its complex embryological development, the esophagus is the site of many congenital abnormalities. Congenital Esophageal stenosis is one tenth as rare as tracheoesophageal fistula with esophageal atresia and is very rare in the cervical esophagus, which mostly occurred below mid-esophagus. Congenital esophageal web may be caused by the resorption failure of the epithelium following the vacuolization stage in embryonic development in the esophagus. Recently, we experienced 1 cases of congenital esophageal web, as the symptoms of life-long dysphagia. According to her history of dysphagia, radiologic and clinical findings, her esophageal stenosis was considered as congenital. For dilatation and relief of dysphagia, she underwent the Heineke-Mikulicz type of esophagoplasty. The results of surgical treatment were relatively good without any clinical events. So we reported it with its literature review.

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.6-10
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• 2016

Poststreptococcal glomerulonephritis (PSGN) is one of the most recognized diseases in pediatric nephrology. Typical clinical features include rapid onset of gross hematuria, edema, and hypertension, and cases are typically preceded by an episode of group A ${\beta}$-hemolytic streptococcus pharyngitis or pyoderma. The most common presenting symptoms of PSGN are the classic triad of glomerulonephritis: gross hematuria, edema, and hypertension. However, patients with PSGN sometimes present with unusual or atypical clinical symptoms that often lead to delayed diagnosis or misdiagnosis of the disease and increased morbidity. Additionally, the epidemiology of postinfectious glomerulonephritis (PIGN), including PSGN, has changed over the past few decades. This paper reviews atypical clinical manifestations of PSGN and discusses the changing demographics of PIGN with a focus on PSGN.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.89-93
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• 2010

Hyponatremia which is a very common electrolyte abnormality in hospitalized patients is defined as a plasma sodium concentration less than 135 mEq/L. Hyponatremia is generally caused by intravascular volume depletion, excessive salt loss and hypotonic fluid overload. It also can be caused by intravascular osmotic agent. Although most cases are mild and asymptomatic, acute severe hyponatremia can cause severe neurologic symptoms, such as seizures and coma. We report a rare case of severe hyponatremia induced by radiographic contrast agent.

• Archives of Plastic Surgery
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• v.46 no.2
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• pp.171-175
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• 2019

Forearm fractures are common injuries in childhood. Median nerve entrapment is a rare complication of forearm fractures, but several cases have been reported in the literature. This case report discusses the diagnosis and management of median nerve entrapment in a 13-year-old male who presented acutely with a both-bone forearm fracture and numbness in the median nerve distribution. Following the delayed diagnosis, surgical exploration revealed complete nerve entrapment and a nerve graft was performed.

• Childhood Kidney Diseases
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• v.25 no.2
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• pp.133-139
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• 2021

Herein, we report two rare cases of renal infection. The first case was renal subcapsular urine reflux in a 8-month-old girl with recurrent urinary tract infection and the second was subcapsular abscess in a 14-year-old girl with diabetes, who was successfully treated with percutaneous drainage. It has been suggested that renal subcapsular abscesses could be caused by the direct reflux of urine into the subcapsular space, rather than spread of infection from an existing parenchymal lesion, and that complete recovery can be achieved if percutaneous drainage is performed in a timely manner. We propose primary subcapsular reflux, in which urine directly refluxes upwards into the subcapsular space of the kidney, as one of the mechanisms for development of renal subcapsular abscesses.

• Childhood Kidney Diseases
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• v.26 no.2
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• pp.97-100
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• 2022

In response to the global coronavirus disease 2019 (COVID-19) pandemic, vaccines were developed and approved quickly. However, numerous cardiovascular adverse events have been reported. We present two adolescent cases who developed a hypertensive crisis following NT162b2 mRNA COVID-19 vaccination. Patient 1 was an 18-year-old male and his systolic blood pressure was 230 mmHg one day after the second vaccine. He was obese. No secondary cause of hypertension other than the vaccine was identified. Patient 2 was an 18-year-old male who complained with palpitation after the first vaccine. His blood pressure was 178/109 mmHg. He had autosomal dominant polycystic kidney disease. Both were treated with continuous infusion of labetalol followed by losartan, and blood pressure was controlled. Patient 2 received second vaccination and his blood pressure did not rise. It is warranted to measure blood pressure in adolescents at high risk of hypertension after NT162b2 mRNA COVID-19 vaccination.

• Childhood Kidney Diseases
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• v.26 no.2
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• pp.111-115
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• 2022

Kleefstra syndrome is a rare genetic disease characterized by mental retardation, hypotonia, and a characteristic facial appearance. Furthermore, in some cases, Kleefstra syndrome is associated with various anorectal and genitourinary complications, including imperforated anus, vesicoureteral reflux, hydronephrosis, and chronic kidney disease. Herein, we present a case of Kleefstra syndrome with recurrent urinary tractinfections associated with vesicoureteral reflux and rectourethral fistula, which was treated by a multidisciplinary approach.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.133-138
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• 2023

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

• Pediatric Infection and Vaccine
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• v.31 no.1
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• pp.122-129
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• 2024

Chediak-Higashi syndrome (CHS) is a rare haematological and immunodeficiency disorder that occurs in childhood leading to recurrent infections, bleeding tendencies and progressive neurological dysfunction. Partial oculocutaneous albinism occurs in almost all cases. The exact prevalence is unknown, and the disease is caused by over 70 identified mutations in the lysosomal trafficking regulator gene. The presence of a bright polychromatic appearance from hair shaft and abnormally large intracytoplasmic granules, especially within neutrophils and platelets in the bone marrow is highly suggestive. Treatment is largely supportive, and the only curative treatment is through an allogeneic hematopoietic stem cell transplant. Without transplant, most patients will enter an accelerated phase of hemophagocytic lymphohistiocytosis (HLH) which carries a high mortality rate. We present a young male with CHS who we had followed through and eventually developed a fulminant accelerated phase. We believe this is only the second reported case of CHS in Malaysia.

• Journal of Chest Surgery
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• v.31 no.11
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• pp.1102-1105
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• 1998

Chronic granulomatous disease in childhood is a rare inheritable disorder of phagocytic cells in which defective production of the reactive intermediates of oxygen predisposes the patient to severe recuring pyogenic infections. The lung is the most common site of infection and pulmonary disease is the primary cause of death for greater than 50% of children with chronic granulomatous disease. Although the role of surgery in management of this disease remains undefined, rapid diagnosis of the underlying pulmonary problem is crucial to determine the most appropriate antimicrobial therapy and surgical techniques such as lobectomy of involved areas lead to more rapid recovery and thus allow the antibiotics to be more efficacious in these cases. We have treated a one month old male baby who had the chronic granulomatous disease with pulmonary infection. Wide surgical resection of the affected lobe and use of antibiotics and antifungals were carried out with good clinical results. He was well after the operation.