[A CASE OF STEVENS-JOHNSON SYNDROME SUSPECTED BY A COMMON COLD MEDICATION AS CAUSE].

We describe here the case of a 7-year-old male patient with Stevens-Johnson syndrome (SJS), which was suspected to be caused by treatment with tipepidine hibenzate (Asverin®). The day after taking tipepidine hibenzate and L-carbocysteine (Carbocysteine® DS) for relief of a cold, he began presenting with the following symptoms: fever above 38°C, wheezing, and decreased oxygen saturation. Two days later, mucous membrane rashes, such as erosions on the lips, eye mucosa, vulva, and blisters on the trunk appeared, and SJS was thus diagnosed. Because pseudomembrane formation and corneal epithelial defect in the eyes were also observed, steroid pulse therapy was administered early in the course of the disease, and the patient recovered without sequelae.A drug-induced lymphocyte stimulation test performed to determine the cause of the disease was positive for fixed-dose combination therapy with tipepidine hibenzate plus L-carbocysteine and for tipepidine hibenzate alone. It has now been three years since the onset of the disease, and no sequelae have been observed. Although tipepidine hibenzate is a drug frequently used for pediatric patients, it should be administered with caution because of its potential to cause SJS.

Missed Diagnosis of Subarachnoid Haemorrhage.

A 79-year-old woman presented with left retro-orbital pain, headache and blurred vision. Based on negative radiological tests, life-threatening conditions like subarachnoid haemorrhage (SAH) were ruled out and outpatient follow-up was planned. However, the patient returned to the hospital that night because of progressively declining consciousness and was diagnosed with SAH by head computed tomography. The diagnosis of SAH is often challenging, especially in cases with negative radiological results. We describe some strategies, other than radiological examination, for ruling out SAH, such as performing a lumbar puncture and repeating tests to take account of disease progression, and describe biases which can affect clinical decision-making. LEARNING POINTS: A high diagnostic error rate highlights the difficulty in diagnosing subarachnoid haemorrhage (SAH).Headache together with oculomotor nerve palsy is an important symptom of SAH.If SAH is suspected, further diagnostic measures including lumber puncture are warranted.A strategy of repeating tests to take account of disease progression would also be effective.

Anaphylaxis due to potato starch (possibly caused by percutaneous sensitization).

An 8-year-old boy with poor control of atopic dermatitis could eat potato products such as French fries without restrictions until 21 months of age. However, he developed generalized urticaria after eating potato products at the same age. Therefore, potatoes were excluded from his diet; nevertheless, he continued to consume a very small amount of potato starch but was without symptoms until the age of 8 years. At this age, he developed anaphylaxis after consuming potato starch and required administration of intramuscular epinephrine. He tested positive for potato-specific immunoglobulin E, skin prick test, and basophil activation test. He developed severe eczema with dry skin and erosion. We later discovered that potato starch had been used for play clay at his nursery school. Although he discontinued using potato starch play clay, it remained present in his surroundings for 6 years. His potato allergy may have developed and continued to worsen as a result of making indirect contact with surfaces that had previously been exposed to the allergen. Two-dimensional Western blot analysis on potato starch revealed the presence of proteins binding to the immunoglobulin E of the patient. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis findings showed that 5 of the 6 protein bands had a similar molecular weight as that of potato proteins. Thus far, there are no reports of anaphylaxis due to potato starch. Children with atopic dermatitis or damaged skin may have sensitivity to potato starch and could develop anaphylaxis as noted in this case.

Isolated Superior Mesenteric Artery Dissection Mimicking Impending Rupture of Abdominal Aortic Aneurysm.

BACKGROUND Isolated superior mesenteric artery dissection (SMAD) is a rare vascular disease that is difficult to diagnose. We report a case of SMAD in a patient with an abdominal aortic aneurysm (AAA) that mimicked an impending rupture of the AAA. In addition, we describe several clinical biases that contributed to the delayed diagnosis. CASE REPORT A 66-year-old man presented with a 3-day history of abdominal pain, without a history of trauma, that worsened gradually and caused him to visit our hospital. The patient's medical history included an AAA under observation. The patient was well oriented and initially remained hemodynamically stable, and the abdomen was soft and non-tender on palpation. An emergency contrast-enhanced computed tomography (CT) scan confirmed a 44-mm AAA without any leakage, but with an isolated SMAD. His previous physician confirmed there was no change in the AAA size since 3 months prior to hospital admission. Thus, the symptoms were caused by the isolated SMAD. The patient showed improvement with pain-relieving and antihypertensive management, without anticoagulation therapy or revascularization, and was discharged on day 25 of admission without any complications. CONCLUSIONS The misdiagnosis in this case was attributable to several clinical biases, including search satisfaction, Sutton's slip, and anchoring bias. Physicians should guard against presumptive diagnoses based on patient symptoms or initial plausible findings and instead pursue a thorough workup to reach a definitive diagnosis.

Metastatic spinal cord compression by gastric cancer: a case report.

Metastatic spinal cord compression (MSCC) is one of the serious complications of malignancy. Most cases of MSCC occur from breast or prostate cancer primaries; MSCC secondary to gastric cancer is rare. We herein report a case of a patient with gastric cancer with weakness of the lower limbs and urinary retention on initial presentation. This case demonstrates that although rare, bone metastases and MSCC may occur from gastric primaries. It also highlights the importance of prompt diagnosis and early treatment of MSCC.

Hippocampal diffusion abnormality after febrile status epilepticus is related to subsequent epilepsy.

OBJECTIVE: To assess hippocampal signal changes on diffusion-weighted imaging (DWI) during the acute period after febrile status epilepticus (FSE) and to examine the relationship between DWI and subsequent epilepsy. METHODS: A prospective, multicenter study of children with a first episode of FSE was performed. The patients underwent magnetic resonance imaging (MRI) within 3 days of FSE, and signal intensity was evaluated on DWI. Electroencephalography studies within 3 days of FSE were also assessed. Nine to 13 years after FSE, information on subsequent epilepsy was obtained. RESULTS: Twenty-two children with FSE were evaluated. DWI showed unilateral hippocampal hyperintensity in six patients (27%). Three of six patients with hippocampal hyperintensity had ipsilateral thalamic hyperintensity. On EEG within 3 days of FSE, five of six patients with hippocampal hyperintensity had ipsilateral focal slowing, spikes, or attenuation. Nine to 13 years later, the outcomes could be determined in five patients with hippocampal hyperintensity and in 10 without. All 5 patients with hippocampal hyperintensity had hippocampal atrophy and developed focal epilepsy, whereas only 1 of 10 patients without hippocampal hyperintensity developed epilepsy (P = 0.002). Ictal semiology was concordant with temporal lobe seizures in all patients. Ipsilateral temporal epileptiform abnormalities were seen on EEG in four of five at last follow-up. SIGNIFICANCE: Acute DWI hippocampal hyperintensity was seen in 27% of patients with FSE. Acute DWI hyperintensity suggests cytotoxic edema caused by prolonged seizure activity. Hippocampal DWI hyperintensity is related to mesial temporal lobe epilepsy and can be a target of neuroprotective treatments to prevent the onset of epilepsy.

Evaluation of the results of oral food challenges conducted in specialized and general hospitals.

BACKGROUND: Oral food challenge (OFC) tests are conducted in both specialized institutions and general hospitals. We aimed to compare the severity of the conditions of the patients between these 2 types of institutions in order to consider the role of such institutions in society. OBJECTIVE: We evaluated the results of OFC tests for hen's egg, cow's milk, and wheat that were conducted in a specialized institution (Aichi Children's Health and Medical Center [ACHMC], n = 835) and in 4 general hospitals (n = 327) in Aichi prefecture, Japan. METHODS: The symptoms provoked were scored using the total score (TS) of the Anaphylaxis Scoring Aichi scoring system in combination with the total ingested protein dose (Pro) before the appearance of allergic symptoms. RESULTS: The total ingested dose of the challenge-positive patients in ACHMC was significantly less than that in the general hospitals (p < 0.01). The median TS of the provoked symptoms in ACHMC and the general hospitals did not differ to a statistically significant extent in the hen's egg or cow's milk challenges; however, the median TS in ACHMC was significantly lower than that in the general hospitals for the wheat challenge (p = 0.02). The median TS/Pro values in ACHMC were almost identical to the upper 25% of the TS/Pro values in the general hospitals, suggesting that the specialized institution usually managed more severe patients. CONCLUSION: The specialized institution performed OFC tests at a lower threshold dose, but provoked similar TSs to the general hospitals. This evaluation may help in optimizing the distribution of patients to general hospitals and specialized institutions.

Tissue culture-induced flower-color changes in Saintpaulia caused by excision of the transposon inserted in the flavonoid 3', 5' hydroxylase (F3'5'H) promoter.

The variegated Saintpaulia cultivar Thamires (Saintpaulia sp.), which has pink petals with blue splotches, is generally maintained by leaf cuttings. In contrast, tissue culture-derived progeny of the cultivar showed not only a high percentage of mutants with solid-blue petals but also other solid-color variants, which have not been observed from leaf cuttings. Solid-color phenotypes were inherited stably by their progeny from tissue culture. Petals from each solid-color variant were analyzed by high-performance liquid chromatography and shown to contain different proportions of three main anthocyanin derivatives: malvidin, peonidin, and pelargonidin. Analysis of flavonoid 3', 5'-hydroxylase (F3'5'H) sequences showed no differences in the coding region among the variants and variegated individuals. However, a transposon belonging to the hAT superfamily was found in the promoter region of variegated individuals, and the presence of transposon-related insertions or deletions correlated with the observed flower-color phenotypes. Solid-blue flower mutants contained 8-base pair (bp) insertions (transposon excision footprints), while solid-pink mutants had 58- to 70-bp insertions, and purple- and deep-purple mutants had 21- and 24-bp deletions, respectively. Real-time reverse transcription polymerase chain reaction (RT-PCR) analysis showed that F3'5'H expression levels correlated with insertions and deletions (indels) caused by hAT excision, resulting in flower-color differences. Our results showed that tissue culture of Saintpaulia 'Thamires' elicits transposon excision, which in turn alters F3'5'H expression levels and flower colors.

Lymphangioma of the skull base bones leading to cerebrospinal fluid rhinorrhea.

Lymphangioma localized to the bones of the skull base is rare. The authors report herein the case of a 5-year-old boy who presented with lymphangioma of the bone, localized to the skull base and leading to cerebrospinal fluid (CSF) rhinorrhea with meningitis. Neuroimaging demonstrated lytic destruction with a cyst in the right middle skull base. The patient was successfully treated with resection of the tumor and prevention of CSF leakage. Histopathological examination revealed a lymphangioma. An enlarging lymphangioma can lead to bone destruction. A differential diagnosis of a lytic lesion for a cyst at the skull base is important for proper case management.

Wiskott-Aldrich syndrome is an important differential diagnosis in male infants with juvenile myelomonocytic leukemialike features.

A newborn presented with thrombocytopenia at birth and subsequently developed leukocytosis, monocytosis, and mild hepatomegaly. The bone marrow was normocellular with dysplasia and spontaneous granulocyte-monocyte colony formation was demonstrated. These findings fulfilled the diagnostic criteria of juvenile myelomonocytic leukemia. Then he developed atopic dermatitislike eczema, which led to the consideration of Wiskott-Aldrich syndrome (WAS). Lack of intracellular WASP expression and WASP gene mutation confirmed the diagnosis of WAS. After stem cell transplantation, he is alive in good condition with normal WASP expression. WAS should be considered as a differential diagnosis in male infants with juvenile myelomonocytic leukemialike features.

Treatment of large and/or multiple hepatic malignancies: open surgical approaches of radiofrequency ablation.

BACKGROUND/AIMS: Patients with hepatic malignancies are often poor candidates for resection because of the lack of hepatic reserve as a result of coexisting cirrhosis or the presence of multiple tumors. The purpose of this study was to determine the safety and efficacy of open intraoperative radiofrequency ablation of unresectable hepatic malignancies with size larger than 4 cm in diameter and/or more than three in number. METHODOLOGY: Between May 2000 and September 2003, 30 patients (24 men, 6 women; age range, 59-72 years; mean age, 63 years) with 51 hepatic malignancies. The maximal diameter of all tumors ranged from 1.0 to 10cm (mean +/- SD, 3.2 +/- 1.8). RESULTS: All tumors achieved necrosis completely in a single session. The mean follow-up from the initial ablation in this study was 18.9 +/- 10.1 months (range, 0-41). The 1-, 2 and 3-year overall survival rates were 86.1%, 71.6% and 71.6%, respectively. The 1-, 2 and 3-year disease-free survival rates were 70.9%, 37.6% and 25.1%, respectively. CONCLUSIONS: Open radiofrequency ablation is a safety and efficient approach for hepatic malignancies sized more than 4 cm in diameter and/or located more than three in number.

Pylorotomy in pylorus-preserving pancreaticoduodenectomy.

BACKGROUND/AIMS: The incidence of delayed gastric emptying after pylorus-preserving pancreaticoduodenectomy has been reported to be 30% to 70%. METHODOLOGY: Between January 1996 and December 2002, 43 patients underwent pylorus-preserving pancreaticoduodenectomy, involving pylorotomy, in the First Department of Surgery, Kinki University School of Medicine. The first step in pylorotomy is to cut the duodenal stump obliquely. The next is incision of the pyloric sphincter along its inferior aspect. The incidences of postoperative complications and changes in body weight were collated retrospectively. RESULTS: Delayed gastric emptying was observed in 4 patients (9.3%). However, this complication did not last more than 1 month in any patients. Two patients (4.7%) developed reflux esophagitis 1 month after surgery, but this complication had resolved by 6 months. Weight gain was noted beginning 3 months after surgery. CONCLUSIONS: Pylorus-preserving pancreaticoduodenectomy involving pylorotomy may reduce the incidence of delayed gastric emptying and preserve the long-term quality of life more than similar procedures.

Significance of HBV DNA in the hepatic parenchyma from patients with non-B, non-C hepatocellular carcinoma.

INTRODUCTION: The etiologic and prognostic factors for non-B, non-C hepatocellular carcinoma (HCC), which is defined by its seronegativity for both hepatitis B surface antigen and hepatitis C virus (HCV) antibody, remain unclear. METHODS: Nonneoplastic liver tissue from 46 patients with non-B, non-C HCC were examined for hepatitis B virus (HBV) DNA and HCV RNA using in situ hybridization. Recurrence-free survival rates were compared between patients showing high or low HBV DNA expression. Other potential prognostic factors were examined as well. RESULTS: HBV DNA was detected in nonneoplastic liver specimens from 35 patents (76.1%), whereas HCV RNA was not detected in any case. In patents with high HBV DNA group expression, recurrence-free survival rates at 1 and 5 years after onset were 68.8% and 13.8%, respectively; those with low expression had higher rates of 89.2% and 59.2%, respectively. Multivariate analysis identified high tumor stage (P=0.042) and high HBV DNA expression (p=0.014) as independent negative prognostic factors. CONCLUSIONS: In many patients with non-B, non-C HCC, HBV DNA in the liver appears to be involved in the carcinogenesis, with intense HBV DNA expression predicting poor outcome for patients with these cancers.

Prevention of bile leak after major hepatectomy.

BACKGROUND/AIMS: Bile leak remains a serious complication after major hepatectomy. The usefulness of external biliary drainage to prevent intraperitoneal bile leak was studied. METHODOLOGY: Thirty-nine patients who underwent major hepatectomy from April 1997 through June 2000 were studied. The bile leak test was performed to identify and close leaks following the resection. Patients who still had leakage underwent retrograde transhepatic biliary drainage (RTBD) via a tube inserted through a choledochostomy. Patient's backgrounds, incidence of bile leak, and time until resolution of bile leak were compared between patients who did and did not have an RTBD tube placed. RESULTS: Nineteen patients had an RTBD tube (48.7%). Bile leak developed in 4 patients with the tube (21.1%), and in 4 patients without the tube (20.0%) (not significant). However, the time until resolution of bile leak was 13.3 days for patients with the tube and 51.3 days for patients without the tube (p<0.05). Two patients developed local peritonitis when the tube was removed. CONCLUSIONS: Though some patients had bile leakage even with the RTBD tube, use of the RTBD tube decreased the length of time leakage that occurred. RTBD tube drainage should be done routinely with major hepatectomy.

[The prevalence of iron deficiency anemia among 6- to 18-month-old children in Japan].

BACKGROUND: Iron deficiency anemia is one of the treatable causes of developmental delay in infants and is therefore screened in several countries. However, in Japan, a screening program for anemia among infants has not been introduced and data on the prevalence of iron deficiency anemia and results of therapeutic trial with iron supplementation are limited. OBJECTIVE: To examine the prevalence of anemia, diagnosis was made with venipuncture blood and iron deficiency anemia was confirmed in a therapeutic trial of infants in Japanese communities. PARTICIPANTS: Six- to 18-month-old infants participated in the anemia screening program in Shinshiro city and Shitara districts, Aichi, and Fujisawa town, Iwate, Japan. METHODS: Capillary blood samples in microtubes were obtained by skin puncture, and centrifuged to measure the hematocrit. When the value was low, venipuncture blood was examined. A hemoglobin concentration under 11 g/dl was judged as a positive result. Anemic infants were referred to pediatrics for prescription of ferrous sulfate. Iron deficiency anemia was defined as a hemoglobin concentration elevated by 1 g/dl or more with a 4-week regimen of ferrous sulfate (therapeutic trial). RESULTS: Of 283 eligible infants, 161 were screened (participation rate, 57%). Mean (SD) microhematocrit by skin puncture was 35.9(2.2)%. Thirteen infants (8%, 95% Cl: 4 to 13%) were anemic, and 7 infants (4%, 95% Cl: 2 to 9%) demonstrated iron deficiency anemia in the therapeutic trial. There was no significant difference between study sites in mean microhematocrit, and prevalence of anemia or iron deficiency anemia. CONCLUSIONS: The prevalence of anemia and iron deficiency anemia among infants in the study communities is high enough to warrant considering routine screening. Further studies are needed to determine whether a high prevalence of anemia is widespread in Japan.

Clinical characteristics of hepatitis B core antibody-positive hepatocellular carcinoma.

The pathology and prognosis of hepatitis B surface antigen (HBsAg)-positive hepatocellular carcinoma (HCC) and hepatitis C virus antibody (HCVAb)-positive HCC is well documented. However, patients with HBsAg-negative/hepatitis B core antibody (HBcAb)-positive HCC are included with non-B non-C disease and have been characterized independently. A series of 125 patients who had undergone hepatectomy for HCC were divided into three groups and compared. The HBsAg group comprised 25 HBsAg-positive patients, the HCV group comprised 70 HCVAb-positive patients, and the HBcAb group comprised 22 HBcAb-positive/HBsAg-negative patients. Eight patients of negative virus markers were excluded in this study. Tumors were larger in the HBcAb group (6.2 cm) than in the HBsAg (4.4 cm) and HCV (3.7 cm) groups. Disease-free 1-, 3-, and 5-year survival rates were, respectively, 75.0%, 57.1%, and 57.1% in the HBcAb group; 60.9%, 41.8%, and 41.8% in the HBsAg group; and 88.0%, 54.0%, and 37.8% in the HCV group. HBcAb-positive HCC patients had larger tumors, but their prognosis was relatively good. Although HBsAg and HCVAb are used for conventional screening of patients with hepatic disorders, we believe that screening is also necessary in patients with positive HBcAb titers for early detection of HCC.