• Childhood Kidney Diseases
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• v.11 no.2
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• pp.288-293
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• 2007

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.226-228
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• 2009

Henoch-$Sch\ddot{o}nlein$ purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.

• The Journal of Internal Korean Medicine
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• v.42 no.5
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• pp.1074-1081
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• 2021

Objectives: This study demonstrated the positive effect of herbal medication on a suspected idiopathic thrombocytopenic purpura (ITP) patient-a 57-year-old male with suspected ITP and a decreased platelet count. Methods: The patient was treated with an herbal medicine named Nokyongdaebo-tang, and its effect was measured with periodic platelet blood tests. Results: Before taking the herbal medicine, the patient's blood platelet count was 98 K/μl, but following treatment, the blood platelet count increased to 201 K/μl and maintained a continuously elevated level. Conclusion: The results indicated that herbal medication may improve the blood platelet count of suspected ITP patients.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.128-133
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• 2019

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan ($^{99m}Tc$ RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.

• Archives of Plastic Surgery
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• v.48 no.3
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• pp.338-343
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• 2021

Purpura fulminans is a serious condition that can result in severe morbidity in the pediatric population. Although autologous skin grafts remain the gold standard for the coverage of partial- to full-thickness wounds, they have several limitations in pediatric patients, including the lack of planar donor sites, the risk of hemodynamic instability, and the limited graft thickness. In Singapore, an in-house skin culture laboratory has been available since 2005 for the use of cultured epithelial autografts (CEAs), especially in burn wounds. However, due to the fragility of CEAs, negative-pressure wound therapy (NPWT) dressings have been rarely used with CEAs. With several modifications, we report a successful case of NPWT applied over a CEA in an infant who sustained 30% total body surface area full-thickness wounds over the anterior abdomen, flank, and upper thigh secondary to purpura fulminans. We also describe the advantages of using NPWT dressing over a CEA, particularly in pediatric patients.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.130-135
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• 1997

Purpose : Interleukin-6(IL-6), a multifunctional cytokine, has been found to have growth and differentiation activities on a wide variety of tissues and cells, including mesangial cells. It has been known that IL-6 is an autocrine growth factor for the proliferation of mesangial cells. Several studies have been performed for revealing the clinical significance of IL-6 in mesangial proliferative glomerulonephritis. Methods : The author measured serum and urinary IL-6 in 30 patients with $Henoch-Sch\"{o}nlein$ purpura (HSP), 18 patients with HSP nephritis, and 10 normal children as a control group. Results : The serum level of IL-6 was increased significantly in the patients with HSP and HSP nephritis compared to normal control. The level of urinary IL-6 was increased significantly in the patients with HSP nephritis compared to both HSP and normal control groups. The level of urinary IL-6 was not correlated with the level of serum. Conclusion : IL-6 was correlated with the amount of proteinuria. These data suggest that IL-6 may be involved in the pathological proliferation of mesangial cell in HSP nephritis.

• The Journal of Internal Korean Medicine
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• v.28 no.3
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• pp.570-585
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• 2007

A clinical study of 106 cases of $Henoch-Sch{\ddot{o}}nlein$ purpura was made. The author analyzed the result of Korean traditional medical therapy. It was observed more frequently in female at the sex ratio of 1:1.3. The most prevalent age group was $4{\sim}9$ years(56.6%). In seasonal incidence, the cases were frequent during spring(50%), and the main specific history was upper respiratory tract infection(48.1%). The duration of contraction was $420.9{\pm}1079.3$ days. The adults were more chronic than children (p<0.05), The clinical manifestation showed in the skin (100%), the gastrointestinal tract(62.3%), the joints(64.2%), the kidney(25.3%), genitourinary system(11.3%), headache(6.6%), and scalp edema(4.7%) respectively. The first outbreak of symptoms was showing of skin purpura in 77 cases(72.6%) the highest frequency. and the gastrointestinal tract and joints were 16(15.1%) and 12 cases(11.3%) respectively. Of all patients, 22 cases(20.8%) experienced recurrences and 47 patients(44.3%) experienced hospitalization treatment. In the patients group which were recurrence experiences, the recurrence rate was high with patients(63.2%) who usually enjoyed meats and convenience foods and in patients(84%) who treated URI with chemical drugs(p<0.05). The patients group(68.9%) that took steroids also experienced a relatively high recurrence rate. Out of the patients(68.9%) who took treatment with steroids, 46.6% experienced side effects, in orders moon face(100%), weight gain(47.1%), appetite increase(52.9%), and acne. alopecia(11.8%), etc. There was a statistically significant relationship between the period of taking the steroid and the side effects(p<0.05), As the result of the herbal medicine treatment(treatment period: $6.7{\pm}6.2$ weeks, a follow-up survey during $15{\sim}24$ months), 67 patients(91.8%) among the 73 patients(68.9%) under 18 years old recovered completely but 3 cases(4.5%) recurred. 23 patients(69.7%) among the 33 patients(31.3%) above 19 years recovered completely but 5 cases(21.7%) recurred.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.77-80
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• 2007

Purpose: Purpura fulminans is a rare but rapidly progressive, serious, often life-threatening disorder in childhood, which is complicated with septic shock or disseminated intravascular coagulopathy during acute infection. It occurs first as acute-onset petechial rash, and spreads rapidly into full thickness skin and soft tissue necrosis. In the past, it had high mortality rate, up to 80%, but recently, survival rate has increased due to early diagnosis, and rapid advancement of critical care and antibiotics. From our experiences of PF management, we would like to review the pathophysiology and suggest the surgical treatment guideline about meningococcal induced purpura fulminans. Methods: Two cases of purpura fulminans over the last 3 years were reviewed retrospectively about reconstructive management. After they were treated resuscitative management initially by the critical intensive care, reconstructive surgery was performed by plastic surgeon as soon as the patients were vitally and mentally stable. Results: There were 6 procedures in case 1, and 3 procedures in case 2. The mean delayed period from admission with sepsis to the first surgical debridement was 24 days and 42 days, respectively. Total hospitalization period was 103 days and 69 days, respectively. All of them were treated with debridement and split thickness skin graft, but delayed debridement was superior to early one in the point of preserving much more tissues. Conclusion: From our experience, we suggest that conservative therapy to the wounds appears to be the best tool in the initial vitally unstable period in order to preserve as much tissues and functions as possible if no active inflammation and compartment syndrome are detective.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.32 no.3
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• pp.116-135
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• 2019

Objectives : The purpose of this study is to analyze how purpura and vasculitis have been treated with Korean medicine and potentially to present with future direction of research and treatment. Methods : We searched clinical studies from the Korean databases including Oriental medicine Advanced Searching Integrated System(OASIS), Korean Traditional Knowledge Portal(KTKP), National Discovery for Science Leader(NDSL), Research Information Sharing Service(RISS), using keywords related to "Purpura" and "Vasculitis" from January 2000 to May 2019. Results : A total of 20 studies were selected for analysis. More than half of the patients provided with detailed information were under 19 year-olds, and upper respiratory infection, stress and fatigue, seasonal factor were among the most frequently stated as predisposing factors. Among many treatment modalities, herbal medicine was the most frequently used, followed by acupuncture and herbal acupuncture. 23 basic herbal medicine formulas were retrieved from 20 articles, most frequently used being Guibi-tang(歸脾湯加味), Samul-tang(四物湯加味) and Yukmijihwang-tang(六味地黃湯加味). In total, 122 Korean medicine herbs were used, most frequently used herbs being Glycyrrhizae Radix et Rhizoma(甘草), Angelicae Gigantis Radix(當歸), Poria Sclerotium(茯?) and Paeoniae Radix(芍藥). By its category, herbs were mostly classified into Tonyfying and replenishing medicinal(補益藥), Heat-clearing medicinal(淸熱藥) and Exterior-releasing medicinal(解表藥). Patients with total treatment period of 3 months or under were 2/3 of all cases provided with detailed information. Follow up periods were short in general with only 6 cases of over 6 months. The primary motive of patients to receive Korean medicine treatments was unresponsiveness or adverse effects of western medication. Conclusion : Through this literature review, we could find out tendencies of Korean medicine treatments of purpura and vasculitis up to date and some points that may have clinical significance.