Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

Sulfohemoglobinemia secundaria a zopiclona. Casos clínicos / Sulfhemoglobinemia secondary to the use of zopiclone. Report of two cases

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.

Resurgence of pertussis at the age of vaccination: clinical, epidemiological, and molecular aspects / Ressurgimento da coqueluche na era vacinal: aspectos clínicos, epidemiológicos e moleculares

OBJECTIVE: Report the incidence, epidemiology, clinical features, death, and vaccination status of patients with whooping cough and perform genotypic characterization of isolates of B. pertussis identified in the state of Paraná, during January 2007 to December 2013.METHODS: Cross-sectional study including 1,209 patients with pertussis. Data were obtained through the Notifiable Diseases Information System (Sistema de Informação de Agravos de Notificação - SINAN) and molecular epidemiology was performed by repetitive sequence-based polymerase chain reaction (rep-PCR; DiversiLab(r), bioMerieux, France).RESULTS: The incidence of pertussis in the state of Paraná increased sharply from 0.15-0.76 per 100,000 habitants between 2007-2010 to 1.7-4.28 per 100,000 between 2011-2013. Patients with less than 1 year of age were more stricken (67.5%). Fifty-nine children (5%) developed pertussis even after receiving three doses and two diphtheria-tetanus-pertussis (DTP) boosters vaccine. The most common complications were pneumonia (14.5%), otitis (0.9%), and encephalopathy (0.7%). Isolates of B. pertussis were grouped into two groups (G1 and G2) and eight distinct patterns (G1: P1-P5 and G2: P6-P8).CONCLUSION: The resurgence of pertussis should stimulate new research to develop vaccines with greater capacity of protection against current clones and also encourage implementation of new strategies for vaccination in order to reduce the risk of disease in infants.

OBJETIVO: Relatar a incidência, os aspectos epidemiológicos, clínicos, a morte e a vacinação de pacientes com coqueluche e fazer a caracterização genotípica de isolados de Bordetella pertussisidentificados no Estado do Paraná, de janeiro de 2007 a dezembro de 2013.MÉTODOS: Estudo transversal, incluindo 1.209 pacientes com coqueluche. Os dados foram obtidos no Sistema de Informação de Agravos de Notificação (Sinan) e a epidemiologia molecular foi feita por PCR baseada em sequências repetitivas (rep-PCR; DiversiLab(r), bioMerieux, France).RESULTADOS: A incidência de coqueluche no Estado do Paraná aumentou acentuadamente de 0,15-0,76 por 100.000 habitantes entre 2007-2010 para 1,7-4,28 por 100.000 habitantes entre 2011-2013. Os pacientes com menos de um ano foram os mais afetados (67,5%); 59 crianças (5%) desenvolveram coqueluche mesmo depois de receber três doses da vacina e dois reforços com a vacina tríplice DTP. As complicações mais comuns foram pneumonia (14,5%), otite (0,9%) e encefalopatia (0,7%). Isolados de B. pertussis foram agrupados em dois grupos (G1 e G2) e oito padrões distintos (G1: P1-P5 e G2: P6-P8).CONCLUSÃO: O ressurgimento da coqueluche vem para sugerir novas pesquisas com o objetivo se desenvolverem vacinas com maior capacidade de proteção contra os clones atuais e também implantar novas estratégias de vacinação, a fim de reduzir o risco de doenças em lactentes.

Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

Semiología respiratoria en pediatría / Semiology in pediatric respiratory

Una anamnesis cuidadosa y una exploración física completa constituyen las herramientas básicas e indispensables para una adecuada orientación diagnóstica en pediatría. La comunicación es tan indispensable como una buena técnica de examen clínico. El médico debe mantener siempre una actitud de servicio, debe irradiar una imagen que ofrezca confianza y respeto a sus pacientes.

A careful history and physical examination are the basic tools and guidance necessary for an adequate diagnostic tool in pediatrics. Communication is as indispensable as a good technique for clinical examination. The doctor must always maintain an attitude of service, must radiate an image that provides confidence and respect for their patients.

Thinner intoxication manifesting as methemoglobinemia.

Lacquer thinner, commonly used for removing household paints is known to contain mixture of various arometic hydrocarbons; halogenated hydrocarbons and naptha, if ingested may rarely cause methemoglobinemia. We report a 3 year old child who presented to us with history of accidental ingestion of thinner, used for removing household paint. Child was having cyanosis with minimal tachypnoea & the colour of his blood was chocolate brown. Later methemoglibulin level estimations were also done, which were suggestive of this diagnosis. Child was managed conservatively with oxygen therapy & vital monitoring, which lead to complete recovery and discharge from hospital later.

Transposição de grandes vasos corrigida congenitamente e gestação: relato de caso / Congenitally corrected transposition of great vessels and pregnancy: case report

Este é um relato de caso com discussão dos desfechos materno-fetais de gestantes com transposição de grandes vasos congenitamente corrigida (TGVCC). Gestante com transposição de grandes artérias congenitamente corrigida TGACC, internada com 35 semanas de gestação por dispneia e cianose central, evoluindo para parto vaginal com fórcipe e recebendo alta com recém-nascido clinicamente estável. A mesma paciente é internada novamente após 1 ano, gestante e com as mesmas queixas, apresentando dor precordial no trabalho de parto. Foi indicada cesárea por desproporção cefalopélvica. Teve alta clinicamente estável. Os recém-nascidos não apresentaram malformações cardíacas. Em conclusão, gestantes portadoras de TGACC devem receber atenção multidisciplinar diferenciada devido à gestação de alto risco, tendo indicação de parto normal assistido e monitorado, visando diminuir a morbimortalidade materna.

Case report and discussion of maternal-fetal outcomes in pregnant patients with congenitally corrected transposition of the great vessels (CCTGV). A pregnant woman with CCTGV was hospitalized at 35 weeks of gestation for dyspnea and central cyanosis. Her baby was born via forceps delivery and was clinically stable when discharged from hospital. The patient returned to the hospital 1 year later. She was pregnant and presented the same complaints, with precordial pain during labor. Cesarean delivery was indicated due to dystocia. She was clinically stable when discharged from hospital. Both newborns did not have cardiac malformations. Pregnant patients suffering from CCTGV should receive multidisciplinary attention due to the high-risk pregnancy, with recommendation of assisted and monitored vaginal labor to reduce maternal morbidity and mortality.

Fatores prognósticos para ventilação mecânica em lactentes com doença respiratória aguda baixa / Prognostic factors for mechanical ventilation in infants with acute lower respiratory disease

OBJETIVO: Infecções respiratórias agudas são a causa mais comum de internação hospitalar em pediatria. Uma proporção de pacientes internados necessita de ventilação pulmonar mecânica invasiva (VPMI). Este estudo objetivou avaliar fatores prognósticos para VPMI em lactentes hospitalizados por infecção respiratória aguda baixa. MÉTODOS: Estudo de coorte prospectivo, realizado de abril a setembro de 2004, em dois hospitais universitários da região de Campinas, São Paulo, Brasil. Foram avaliados 152 lactentes internados, registrando-se dados epidemiológicos e clínicos na internação e no seguimento. Analisaram-se dois grupos, de acordo com a necessidade de VPMI, comparando-se os fatores prognósticos. A associação entre fatores de risco e desfecho estudado foi analisada por risco relativo (RR) e intervalos de confiança (IC 95 por cento). RESULTADOS: Vinte e um pacientes (13,81 por cento) necessitaram de VPMI. Fatores significativamente associados à VPMI na admissão foram: idade < 3 meses (RR=2,35, IC 95 por cento:1,06-5,22), aleitamento materno < 1 mês (RR=3,15, IC 95 por cento:1,35-7,35) e cianose (RR=7,55, IC 95 por cento:5,01-11,36). Observou-se risco aumentado no grupo de VPMI para internação > 10 dias (RR=13,69, IC 95 por cento:4,92-38,09), oxigenioterapia > 10 dias (RR=13,57, IC 95 por cento:5,41-34,03), uso de antibióticos (RR=3,03, IC 95 por cento:1,34-6,89) e reinternações (RR=5,23, IC 95 por cento:2,12-12,91). CONCLUSÃO: As associações entre a necessidade de VPMI e baixa idade, tempo curto de aleitamento materno e cianose à admissão demonstram as reduzidas reservas fisiológicas no lactente jovem com infecção respiratória baixa. Caracterizam este grupo de pacientes a necessidade de suporte hospitalar prolongado e intensivo e maior taxa de reinternações.

OBJECTIVE: Acute lower respiratory tract infections are the most common cause of hospital admission in pediatrics. A number of admitted patients need invasive mechanical pulmonary ventilation (IMPV). This study aimed to evaluate prognostic factors for IMPV in infants admitted due to acute lower respiratory infection. METHODS: A prospective cohort study was conducted from April to September, 2004, in two university hospitals of the Campinas metropolitan area, São Paulo, Brazil. One hundred, fifty-two infants were enrolled. Epidemiological and clinical data were recorded at admission and follow-up. Two groups were analyzed, according to the need of IMPV, with a comparison of prognostic factors. Association between risk factors and the outcome were studied and assessed by Relative Risk (RR), with confidence intervals of (95 percentCI). RESULTS: Twenty-one patients (13.81 percent) needed IMPV. Factors significantly associated with IMPV on admission were: age < 3 months (RR=2.35, 95 percentCI:1.06-5.22), breast feeding < 1 month (RR=3.15, 95 percentCI:1.35-7.35) and cyanosis (RR=7.55, 95 percentCI:5.01-11.36). In the IMPV group, increased risks for hospitalization > 10 days (RR=13.69, 95 percentCI:4.92-38.09), oxygen therapy > 10 days (RR=13.57, 95 percentCI:5.41-34.03), antibiotic usage (RR=3.03, 95 percentCI:1.34-6.89) and readmission (RR=5.23, 95 percentCI:2.12-12.91) were observed. CONCLUSION: The associations between need of IMPV and early age, reduced breast feeding and cyanosis demonstrate diminished physiological reserves in the young infant with lower respiratory infection. These patients require prolonged and intensive hospital support and readmission.

Splenic abscess in a boy with isolated levocardia.

Levocardia with situs inversus is a rare condition. We present the case of a fourteen-year-old boy with congenital cyanotic heart disease, isolated levocardia and splenic abscess. It is the first report of both these conditions occurring in the same person.

Early mental and neurological sequelae after Japanese B encephalitis.

Japanese B encephalitis is a disease with high mortality and many of those surviving suffer from serious sequelae. During the 1992 epidemic in northern Vietnam 50 patients treated at the Institute for Protection of Children's Health in Hanoi were studied concerning the type of sequelae and the development of the symptoms during the first two months of the disease. The age span was 1 to 15 years. 29 of the patients (58%) did not recover fully during the observation period. Fifteen (30%) showed signs of both neurological and mental disturbances. Nine (18%) only had mental symptoms while 5 (20%) suffered from isolated neurological sequelae. EEG was pathological in 9 out of 30 tested cases (30%); 9 of 23 patients (39%) performed subnormal IQ tests. Deep coma, bronchopneumonia with cyanosis, apnea attacks, prolonged fever and coma were all correlated (without statistical significance) to a higher risk for subsequent sequelae.

Angioplastia transluminal da anastomose aorto-pulmonar da cirurgia de Jatene: aplicaçäo em dois casos / Aorto-pulmonary anastomosis transluminal angioplasty in Jatene surgery: report of 2 cases

Dois pacientes portadores de Transposiçäo de grande artérias submetidos a cirurgia de Jatene aos seis dias e aos seis meses de idade apresentaram no período pós-operatório estenose na anastomose aorto-pulmonar, diagnosticada aos 3§ e 6§ anos de evoluçäo. Procedeu-se a angioplastia transluminal percultânea da referida obstruçäo, com sucesso, seis meses e imediatamente após o diagnóstico, respectivamente. Houve reduçäo dos gradientes de pressäo, respectivamente de 83 mmHg para 24 mmHg, e de 76 mmHg para 13 mmHg, com nítda melhora do aspecto angiográfico. Nenhum dos pacientes apresentou complicaçöes durante ou após o procedimento. Esta aplicaçäo da angioplastia trasluminal percutânea, inédita na literatura, deve ser cogitada como alternativa a correçäo cirúrgica da estenose do "novo" tronco pulmonar da cirurgia de Jatene

Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an altennative to the surgical correction of the new pulmonary trunk stenosis in the Jatene surgery

Hiposfagma: uma ocorrência rara em contusäo abdominal / Hyposfagma: an unusual occurence in abdominal contusion

Os autores apresentam um caso de HIPOSFAGMA decorrente de trauma abdominal fechado. Revisam a etiopatogenia da lesäo e tecem consideraçöes sobre o tratamento e a evoluçäo do paciente

Dissecçäo e rotura da artéria pulmonar associada a persistência do canal arterial: relato de um caso / Dissection and rupture of pulmonary artery associated with patent ductus arteriosus: a case report

É descrito um caso de dissecçäo da artéria pulmonar, com rotura intrapericárdica e tamponamento cardíaco, em menina de 13 anos de idade, portadora de canal arterial grande e valva pulmonar bicúspide. Havia hipertensäo pulmonar com arteriopatia plexogênica grave; nas grandes artérias elásticas, a camada média tinha alteraçöes necróticas e reparadoras profundas. Discute-se o papel da medionecrose, condiçäo que é exacerbada pela hipertensäo e por características individuais, na patogenia do aneurisma dissecante e no caso em questäo. A análise da literatura mostra que, na artéria pulmonar, a dissecçäo é sempre um acidente fatal, por rotura ou obstruçäo arterial. O tratamento desta intercorrência, eminentemente cirúrgico, raramente é possível devido ao caráter fulminante da evoluçäo e à ausência de sinais definidos para o diagnóstico em tempo hábil