• Childhood Kidney Diseases
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• v.15 no.2
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• pp.184-190
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• 2011

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Sch$\ddot{o}$nlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.982-986
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• 2007

Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. Results : Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than $1,000/mm^3$, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. Conclusion : IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.428-431
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• 2010

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.

• The Journal of Internal Korean Medicine
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• v.43 no.6
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• pp.1045-1062
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• 2022

Objectives: The aim of this study was to analyze the latest clinical studies on Korean medicine treatment of idiopathic thrombocytopenic purpura (ITP) in the Chinese National Knowledge Infrastructure (CNKI) database. Methods: We searched the last 6 years of clinical studies discussing Oriental medicine-based treatments for ITP in the CNKI database. A meta-analysis of 13 RCTs was performed by synthesizing the outcomes, including the measured platelet count and total effective rate. The quality of the studies was assessed using Cochrane's risk of bias (RoB) tool. RevMan 5.4.1 software was used for data analysis. Results: Of the 15 selected studies, 1 was a non-randomized controlled trial (nRCT), 2 were case series, and 12 were randomized controlled trials (RCTs). Treatments in all studies included oral herbal medicine. The most frequently used herbal decoction was the Liangxue Jiedu prescription (凉血解毒方), and the most commonly used herb was Agrimonia pilosa (仙鶴草), Astragali Radix (黃芪), Fossilia Glycyrrhizae Radix et Rhizoma (甘草), and Rehmannia glutinosa Liboschitz ex Steudel (地黃). The meta-analysis showed significantly better improvement in platelet counts and total effective rate for ITP in the treatment group than in the control group. Conclusion: Treatment with herbal medicine was effective in treating ITP. However, the significance of this conclusion is somewhat limited due to the low quality of the available studies. Multifaceted and scientifically designed clinical studies are required to develop treatments for ITP based on Korean medicine. The results of this study could be used as basic data for further ITP studies.

• Journal of Yeungnam Medical Science
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• v.40 no.3
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• pp.241-246
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• 2023

Immune thrombocytopenia (ITP) is a disease in which thrombocytopenia occurs because of immune-mediated platelet destruction and decreased platelet production. Although many pediatric patients with ITP experience spontaneous remission or reach remission within 12 months of first-line therapy, approximately 20% progress to chronic ITP. Patients who do not respond to first-line treatment or experience frequent relapses are of great concern to physicians. This review summarizes recent treatments for second-line treatment of pediatric chronic ITP.

• BMB Reports
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• v.35 no.5
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• pp.524-531
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• 2002

Thrombotic thrombocytopenic purpura (TTP) is characterized by widespread platelet thrombi in arterioles and capillaries. Unusually large or multimeric von Willebrand factor, as well as one or ore platelet-agglutinating factors, have been implicated in the pathogenesis of TTP. But, the actual mechanisms of platelet agglutination have not been satisfactorily explained. Recent studies suggested the 37-kDa platelet-agglutinating protein (PAP) p37 to be partially responsible for the formation of platelet thrombi in patients with TTP. We studied mobility in SDS-PAGE, the sequence of N-terminal amino acid residues, DNA and antigenic characteristics of PAP p37, which might be related to the pathogenesis of TTP. PAP p37 was purified from the plasma of a 31-year-old male Korean patient with acute TTP. The findings are as follows: (1) We compared PAP p37 with thrombin through the use of SDS-PAGE, either with or without $\beta$-mercaptoethanol. PAP p37 did not appear to be cleaved between the A- and B-chains of prethrombin 2. However, thrombin did cleave between those of prethrombin 2, but linked with disulfide bridge. (2) N-terminal 21 amino acid sequence of PAP p37 was T-F-G-S-G-E-A-D-X-G-L-R-P-L-F-E-K-K-S-L-E. It appeared to be identical to that of 285-305 amino acid residues of human prothrombin (prethrombin 2). (3) No prothrombin gene DNA mutation was revealed. (4). The antigenicity of PAP p37 was similar to thrombin, which was a result of the competitive binding against the anti-thrombin antibody. With these results, we conclude that PAP p37 has similar characteristics to prethrombin2.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.931-937
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• 2007

The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.

• Clinical and Experimental Pediatrics
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• v.63 no.5
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• pp.158-163
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• 2020

IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

• Journal of Oriental Neuropsychiatry
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• v.18 no.2
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• pp.143-151
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• 2007

This study report the good reslut for chronic Idiopathic thrombocytopenic purpura(ITP) patient with Herbal medication treatment and Counseling therapy. The case was a 56 years old female who worried about small matters too much. This patient treated in western medicine, However, didn't see any improvement. So, the patient admitted in our hospital. We diagnosied this patient as 'The Defiency of the Spleen energy', caused by too much thinking and concern. Herbal medication(Nokyonggunbitang)and psychotherapy applied to this patient for hospitalized 22 days. The result showed that blood platelet increased at discharged moment(37,000 / ${\mu}{\ell}$) compared with the admission time(19,000 / ${\mu}{\ell}$). The present result indicated that Herbal medication treatment and psychotherapy may play a role elevate and maintain the number of blood platelet.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.62-68
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• 2007

Purpose : The cause and pathogenesis of Henoch-Sch$\ddot{o}$nlein purpura (HSP) are unknown, but recently the hypothesis that immunoglobulin A may have an important role in the pathogenesis of HSP is being published and HSP associated with mycoplasma infection has been also reported. The aim of this study is to discover relation between HSP and mycoplsma infection. Methods : From Jan. 2002 to Dec. 2005, we retrospectively evaluated 98 children who were diagnosed as HSP at Ilsan Paik Hospital. 84 patients were not associated with mycoplasma infection (group A) and 14 patients were associated with mycoplasma infection (group B). We compared both groups about clinical features. Results : The ratio of male to female patients in group A and B were 1.21:1 and 1.80:1. The number of patients in group A was most in November and December, and in group B was most in November. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities and joint involvement was most common on the feet and ankle in both groups. 57 (67.9%) cases in group A and 9 (64.3%) cases in group B complained of abdominal pain. And 21 (25.0%) cases in group A and 5 (35.7%) cases in group B had nephritis. Elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients (14.3%). In this study, there was one child with HSP preceded by typical mycoplasma pneumonia (mycoplasma antibody titer 1:1280). Conclusion : In this study, elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients and the difference of clinical features between group A and B was not specific. The role of mycoplasma infection in the etiology of HSP may have been implicated, so the association with mycoplasma infection should have to be proved by further controlled studies.