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BACKGROUND: Kidney transplantation (KT) in children and adolescents with severe motor and intellectual disabilities (SMID) has been a topic of controversy. A multicenter study in Japan showed that KT was not contraindicated for children with multiple handicaps, but no consensus has been reached on KT for patients with SMID. This study aimed to determine whether KT is a viable treatment option for children and adolescents with SMID. METHODS: A single-center, retrospective study was conducted on children and adolescents with SMID who underwent KT. SMID was defined based on Oshima's classification. Clinical information was collected through a review of medical records. RESULTS: Of 453 children and adolescents who underwent KT between 1983 and 2023 in our institution, six (1.3%) patients with SMID were identified. One patient received KT twice. All patients underwent living KT. Five patients used medical devices, including gastrostomy and a ventriculoperitoneal shunt, prior to KT. Perioperative complications, including hemothorax related to central venous catheter insertion, ventilator-associated pneumonia, and common iliac artery thrombosis requiring graftectomy, occurred in three patients. One patient required vesicostomy owing to refractory urinary tract infection. There was no significant difference in the graft survival rate between patients with SMID and those without SMID. One patient developed graft failure and died after selecting conservative kidney management. CONCLUSION: Our study showed a favorable graft survival in children and adolescents with SMID who underwent KT. Although careful perioperative management and continued medical care are required, KT may be a viable option for these patients.
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BACKGROUND: Exophiala dermatitidis is a dematiaceous fungus isolated from various environmental sources. Systemic E. dermatitidis infections can lead to fatal outcomes, and treatment has not yet been standardized. Although E. dermatitidis is also known to cause cutaneous infection, it has not been previously reported to appear as ecthyma gangrenosum (EG), an uncommon cutaneous lesion in neutropenic patients that is mainly caused by Pseudomonas aeruginosa. CASE PRESENTATION: A 2-month-old male infant with mixed-phenotype acute leukemia presented with prolonged fever unresponsive to antibacterial and antifungal agents during myelosuppression due to remission induction therapy. He also presented with skin lesions on the left wrist and left lower quadrant of the abdomen. The abdominal lesion gradually turned black and necrotic, which was consistent with the findings of the EG. E. dermatitidis was isolated from the blood, stool, wrist skin, and endotracheal aspirate. During hematopoietic recovery, consolidation in both lungs was evident. Multiagent antifungal treatment failed to eliminate E. dermatitidis from blood. In order to salvage the central venous catheter, ethanol lock therapy (ELT) was adopted, following which the blood culture became negative. The abdominal lesion that evolved as a necrotic mass connecting the small intestine and subcutaneous tissue adjacent to the skin was surgically resected. After these interventions, the general condition improved. CONCLUSION: Disseminated E. dermatitidis mycosis in the neutropenic infant was successfully managed with a multidisciplinary treatment consisting of multiagent antifungal treatment, ELT, and surgery.
Assuntos
Ectima , Leucemia , Micoses , Masculino , Humanos , Antifúngicos/uso terapêutico , Micoses/tratamento farmacológico , Ectima/tratamento farmacológico , Leucemia/tratamento farmacológico , Doença Aguda , Antibacterianos , Etanol , FenótipoRESUMO
BACKGROUND: Campylobacter fetus is an uncommon Campylobacter species, and its infections mainly cause infective endocarditis, aortic aneurysm, and meningitis rather than enteritis. It is more likely to be detected in blood than Campylobacter jejuni or Campylobacter coli, specifically reported in 53% of patients. In our case, C. fetus was detected in both blood and cerebrospinal fluid (CSF) cultures. CASE PRESENTATION: A 33-year-old woman, who was on maintenance chemotherapy for acute lymphoblastic leukemia (ALL), presented to our clinic with chief complaints of severe headache and nausea. Blood and CSF cultures revealed C. fetus. We administrated meropenem 2 g intravenously (IV) every 8 h for 3 weeks, and she was discharged without neurological sequelae. CONCLUSION: We encountered a case of C. fetus meningitis without gastrointestinal symptoms, neck stiffness or jolt accentuation in a patient with ALL. Undercooked beef was considered the source of C. fetus infection in this case, suggesting that the need for a neutropenic diet and safe food handling be considered.
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Infecções por Campylobacter , Campylobacter fetus/isolamento & purificação , Ceftriaxona/administração & dosagem , Doenças Transmitidas por Alimentos , Meningites Bacterianas , Meropeném/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Antibacterianos/administração & dosagem , Infecções por Campylobacter/sangue , Infecções por Campylobacter/líquido cefalorraquidiano , Infecções por Campylobacter/diagnóstico , Infecções por Campylobacter/tratamento farmacológico , Diagnóstico Diferencial , Tratamento Farmacológico/métodos , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/diagnóstico , Feminino , Doenças Transmitidas por Alimentos/complicações , Doenças Transmitidas por Alimentos/diagnóstico , Doenças Transmitidas por Alimentos/tratamento farmacológico , Doenças Transmitidas por Alimentos/microbiologia , Humanos , Meningites Bacterianas/diagnóstico , Meningites Bacterianas/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Resultado do TratamentoRESUMO
MYH9-related disease (MYH9-RD) is characterized by congenital macrothrombocytopenia, progressive kidney failure, and sensorineural hearing loss. We describe a patient with MYH9-RD and a normal platelet count. A 13-year-old boy with a normal platelet count presented with proteinuria and hematuria and underwent a kidney biopsy. Light microscopy showed mild mesangial matrix expansion. Electron microscopy showed thinning of the glomerular basement membrane and splitting of the lamina densa. A tentative diagnosis of Alport syndrome was made. Unexpectedly, genetic analysis revealed a de novo MYH9 gene variant (p.Gln1068_Leu1074dup). A peripheral blood smear examination showed giant platelets and leukocyte inclusion bodies, confirming a diagnosis of MYH9-RD. In summary, we described a patient with MYH9-RD without thrombocytopenia who showed glomerular basement membrane abnormalities similar to Alport syndrome. Peripheral blood smear examinations may be helpful for an appropriate diagnosis of MYH9-RD, even in patients with proteinuria and a normal platelet count.
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In fluctuating environments, many microorganisms acquire phenotypic heterogeneity as a survival tactic to increase the likelihood of survival of the overall population. One example of this interindividual heterogeneity is the diversity of ATP concentration among members of Escherichia coli populations under glucose deprivation. Despite the importance of such environmentally driven phenotypic heterogeneity, how the differences in intracellular ATP concentration emerge among individual E. coli organisms is unknown. In this study, we focused on the mechanism through which individual E. coli achieve high intracellular ATP concentrations. First, we measured the ATP retained by E. coli over time when cultured at low (0.1 mM) and control (22.2 mM) concentrations of glucose and obtained the chronological change in ATP concentrations. Then, by comparing these chronological change of ATP concentrations and analyzing whether stochastic state transitions, periodic oscillations, cellular age, and intercellular communication-which have been reported as molecular biological mechanisms for generating interindividual heterogeneity-are involved, we showed that the appearance of high ATP-holding individuals observed among E. coli can be explained only by intercellular transmission. By performing metabolomic analysis of post-culture medium, we revealed a significant increase in the ATP, especially at low glucose, and that the number of E. coli that retain significantly higher ATP can be controlled by adding large amounts of ATP to the medium, even in populations cultured under control glucose concentrations. These results reveal for the first time that ATP-mediated intercellular transmission enables some individuals in E. coli populations grown at low glucose to retain large amounts of ATP.
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Escherichia coli , Glucose , Humanos , Glucose/análise , Comunicação Celular , Trifosfato de Adenosina/análiseRESUMO
BACKGROUND: Currently, sequential morphological changes of vascularized scapular bone for mandibular reconstruction have remained unreported. METHODS: We retrospectively analyzed 18 patients with defects on the lateral segment of the mandible who underwent postoperative computed tomography (CT) scanning at 1, 6, 12, and 24 months postoperatively. Using the cross-sectional coronal CT images during each assessment, we measured the height and thickness of the grafted scapular bone at points 10 and 30â¯mm away from the mesial segment and evaluated their differences over time. Then, the relationship between the morphological changes of the grafted scapular bone and the distance from the mesial bone segment, the number of occlusal supports on the contralateral side of the mandible, and the correlation of sex and age were evaluated. RESULTS: The height of the grafted scapular bone decreased by approximately 11% and its thickness increased by 14% within 24 months postoperatively. The bone thickness increase was greater at point 30â¯mm from the mesial bone segment. Patients with two occlusal contacts (premolar and molar) on the contralateral side had a significantly higher rate of bone thickness increase. Furthermore, the graft morphology was not associated with age or sex. CONCLUSION: A vascularized scapular bone thickens over time, which depends on the distance from the mesial bone segment and the occlusal contact regions on the contralateral side of the mandible.
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Mandíbula/diagnóstico por imagem , Mandíbula/patologia , Reconstrução Mandibular/métodos , Escápula/irrigação sanguínea , Escápula/transplante , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Retalho Miocutâneo , Estudos Retrospectivos , Escápula/diagnóstico por imagem , Estresse FisiológicoRESUMO
The enantioselective total synthesis of (+)-hexachlorosulfolipid, a cytotoxin found in the Adriatic mussel Mytilus galloprovincialis, is described. The unique chlorinated hydrocarbon motif of the lipid is successfully furnished by a series of dichlorination reactions of chiral epoxides with chlorophosphonium reagent generated in situ from Ph(3)P/NCS. The present total synthesis has allowed the confirmation of the absolute configuration of the natural cytotoxic (+)-hexachlorosulfolipid originally proposed by Fattorusso, Ciminiello, and co-workers.
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Lipídeos/síntese química , Mytilus/química , Animais , Simulação por Computador , Lipídeos/química , Lipídeos/isolamento & purificação , Conformação Molecular , EstereoisomerismoRESUMO
Yellow nail syndrome (YNS) is a rare clinical syndrome characterized by a triad of yellow thick nail, lymphedema and respiratory diseases. We experienced 2 cases of YNS with diffuse panbronchiolitis (DPB)-like pulmonary manifestation. Since YNS might be hidden to those who have been diagnosed with DPB, physicians should be alert to recognize nail signs of YNS in case of DPB refractory to macrolide therapy. We hereby review previous case reports of YNS and discuss its pulmonary manifestations.
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Antibacterianos/uso terapêutico , Bronquiolite/tratamento farmacológico , Bronquiolite/etiologia , Infecções por Haemophilus/tratamento farmacológico , Infecções por Haemophilus/etiologia , Síndrome das Unhas Amareladas/complicações , Síndrome das Unhas Amareladas/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Resultado do Tratamento , Síndrome das Unhas Amareladas/diagnósticoRESUMO
A novel method to prepare 2-H-imidazolines from glyoxylic acid monohydrate and 1,2-diamines is described; the key reaction of this method is the oxidative decarboxylation of the 2-carboxy imidazolidines by NBS.
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Ácidos Carboxílicos/química , Diaminas/química , Imidazolinas/síntese química , Imidazolinas/química , OxirreduçãoRESUMO
A convergent asymmetric total synthesis of (+)-danicalipin A is accomplished, in which two chlorinated fragments are stereoselectively joined by 1,3-dipolar coupling, leading to the confirmation of the absolute configuration of the natural product.
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Lipídeos/síntese química , Lipídeos/química , Estrutura Molecular , Ochromonas/química , EstereoisomerismoRESUMO
The first asymmetric total synthesis of spongotine A is described. The oxidative synthesis of the imidazoline/ketone unit from keto aldehyde and diamine is a key step in this synthesis. The absolute stereochemistry of the asymmetric center of natural spongotine A is revealed as the (S)-configuration.