Δευτέρα 13 Απριλίου 2020

World journal of clinical cases.

Laparoscopic repair of complete intrathoracic stomach with iron deficiency anemia: A case report.
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Laparoscopic repair of complete intrathoracic stomach with iron deficiency anemia: A case report.
World J Clin Cases. 2020 Mar 26;8(6):1180-1187
Authors: Yasheng D, Wulamu W, Li YL, Tuhongjiang A, Abudureyimu K
Abstract
BACKGROUND: Giant paraesophageal hiatal hernias (HH) are very infrequent, and their spectrum of clinical manifestations is large. Giant HH mainly occurs in elderly patients, and its relationship with anemia has been reported. For the surgical treatment of large HH, Nissen fundoplication is the most common antireflux procedure, and the reinforcement of HH repair with a patch (either synthetic or biologic) is still debatable.
CASE SUMMARY: We report on a case of giant paraesophageal HH in a middle-aged male patient with reflux symptoms and severe anemia. After performing a series of tests and diagnostic approaches, results showed a complete intrathoracic stomach associated with severe iron deficiency anemia. The patient underwent successful laparoscopic hernia repair with mesh reinforcement and Nissen fundoplication. Postoperatively, reflux symptoms were markedly relieved, and the imaging study showed complete reduction of the hernia sac. More importantly, anemia was resolved, and hemoglobin, serum iron and ferritin level were returned to the normal range. The patient kept regular follow-up appointments and remained in a satisfactory condition.
CONCLUSION: This case report highlights the relationship between large HH and iron deficiency anemia. For the surgical treatment of large HH, laparoscopic repair of large HH combined with antireflux procedure and mesh reinforcement is recommended.
PMID: 32258090 [PubMed]
Growth hormone therapy for children with KBG syndrome: A case report and review of literature.
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Growth hormone therapy for children with KBG syndrome: A case report and review of literature.
World J Clin Cases. 2020 Mar 26;8(6):1172-1179
Authors: Ge XY, Ge L, Hu WW, Li XL, Hu YY
Abstract
BACKGROUND: The incidence of short stature in KBG syndrome is relatively high. Data on the therapeutic effects of growth hormone (GH) on children with KBG syndrome accompanied by short stature in the previous literature has not been summarized.
CASE SUMMARY: Here we studied a girl with KBG syndrome and collected the data of children with KBG syndrome accompanied by short stature from previous studies before and after GH therapy. The girl was referred to our department because of short stature. Physical examination revealed mild dysmorphic features. The peak GH responses to arginine and clonidine were 6.22 and 5.40 ng/mL, respectively. The level of insulin-like growth factor 1 (IGF-1) was 42.0 ng/mL. Genetic analysis showed a c.2635 dupG (p.Glu879fs) mutation in the ANKRD11 gene. She received GH therapy. During the first year of GH therapy, her height increased by 0.92 standard deviation score (SDS). Her height increased from -1.95 SDS to -0.70 SDS after two years of GH therapy. There were ten children with KBG syndrome accompanied by short stature who received GH therapy in reported cases. Height SDS was improved in nine (9/10) of them. The mean height SDS in five children with KBG syndrome accompanied by short stature increased from -2.72 ± 0.44 to -1.95 ± 0.57 after the first year of GH therapy (P = 0.001). There were no adverse reactions reported after GH treatment.
CONCLUSION: GH treatment is effective in our girl and most children with KBG syndrome accompanied by short stature during the first year of therapy.
PMID: 32258089 [PubMed]
Hepatoid adenocarcinoma of the stomach: Thirteen case reports and review of literature.
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Hepatoid adenocarcinoma of the stomach: Thirteen case reports and review of literature.
World J Clin Cases. 2020 Mar 26;8(6):1164-1171
Authors: Zhang ZR, Wu J, Li HW, Wang T
Abstract
BACKGROUND: The aim of the present study was to examine the clinical characteristics of hepatoid adenocarcinoma of the stomach (HAS) and its diagnosis, treatment, and prognosis.
CASE SUMMARY: A retrospective analysis of 13 HAS cases was performed. The mean age of the 13 patients was 66.08 years, and 10 of the 13 patients were male. Prior to treatment, the alpha-fetoprotein levels in the serum were elevated in 7 patients, the tumour was located in the distal or gastric body in 11 patients, and the gastroscopy pathological results showed that 3 patients had poorly differentiated tumours and that 8 patients had moderately/poorly differentiated tumours. Abdominal CT scans showed local stomach wall thickening, and enlarged lymph nodes were visible around the stomach in 8 patients. Of the 13 patients, 11 underwent radical surgery. The clinical pathological staging was as follows: Stage II in 2 cases; stage III in 8 cases; and stage IV in 1 case. A total of 3 patients were lost to follow-up. Otherwise, as of the last follow-up, 3 patients had survived for 56 mo, and the other 7 patients failed to achieve long-term survival (survival period of 1-56 mo).
CONCLUSION: HAS is a special type of gastric cancer, and the prognosis of HAS has improved compared with past prognoses. Measurement of alpha-fetoprotein, early diagnosis, active surgical treatment, and application of new diagnostic and treatment techniques are conducive to improving the prognosis of HAS.
PMID: 32258088 [PubMed]
Cerebral venous sinus thrombosis following transsphenoidal surgery for craniopharyngioma: A case report.
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Cerebral venous sinus thrombosis following transsphenoidal surgery for craniopharyngioma: A case report.
World J Clin Cases. 2020 Mar 26;8(6):1158-1163
Authors: Chang T, Yang YL, Gao L, Li LH
Abstract
BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare condition in patients with craniopharyngioma following transsphenoidal surgery.
CASE SUMMARY: A 56-year-old man who underwent transsphenoidal surgery for craniopharyngioma 26 d ago presented gradual headache and cerebrospinal fluid leakage while vomiting 5 d post-discharge and required readmission to our department of neurosurgery. After admission, head imaging examination showed a hyperdense shadow in the superior sagittal sinus and right transverse sinus, edema at the bilateral parietal lobe, and hemorrhage at the left parietal lobe and right occipital lobe; the venous phase of cerebral angiography revealed CVST. The patient was treated immediately by intravenous thrombolysis, endovascular thrombolysis, and mechanical thrombectomy after the definite diagnosis. However, the neurological status of the patient continued to deteriorate and he died on the fourth day after readmission.
CONCLUSION: For craniopharyngioma undergoing transsphenoidal surgery, it is vital to take an effective strategy to manage the postoperative complications, such as diabetes insipidus, severe electrolyte imbalance, and cerebrospinal fluid leakage. Additionally, the early differential diagnosis of CVST is essential when it develops clinical symptoms, especially in patients following transsphenoidal surgery with a high risk of CVST. Subsequently, the timely and effective treatment of the CVST is critical for preventing neurological deterioration.
PMID: 32258087 [PubMed]
Microscopic removal of type III dens invaginatus and preparation of apical barrier with mineral trioxide aggregate in a maxillary lateral incisor: A case report and review of literature.
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Microscopic removal of type III dens invaginatus and preparation of apical barrier with mineral trioxide aggregate in a maxillary lateral incisor: A case report and review of literature.
World J Clin Cases. 2020 Mar 26;8(6):1150-1157
Authors: Liu J, Zhang YR, Zhang FY, Zhang GD, Xu H
Abstract
BACKGROUND: Invaginated teeth pose greater challenges in clinical management because of their complex configuration. With advancements in equipment and materials, such as the dental operation microscope, cone-beam computed tomography and mineral trioxide aggregate, the preservation rate of type III dens invaginatus could be greatly increased.
CASE SUMMARY: This case report presented a 31-year-old woman with complaints of spontaneous swelling and pain in the right maxillary lateral tooth. With the aid of cone-beam computed tomography, type III dens invaginatus with apical periodontitis was diagnosed and confirmed. Three-visit endodontic treatment was performed. In the first visit, the invagination was carefully removed under the dental operation microscope, and chemomechanical preparation was done. In the second visit, mineral trioxide aggregate apical barrier surgery was performed in this tooth. In the third visit, the canal was finally obturated with thermoplastic gutta-percha to recover the crown morphology. A 26-mo follow-up revealed a satisfied outcome both in the radiographic and oral examinations.
CONCLUSION: In this case, removal of the entire abnormal structure provided great convenience for the follow-up treatment. When confronted with the same clinical case in the future, we can take a similar approach to address it.
PMID: 32258086 [PubMed]
Hyoid-complex elevation and stimulation technique restores swallowing function in patients with lateral medullary syndrome: Two case reports.
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Hyoid-complex elevation and stimulation technique restores swallowing function in patients with lateral medullary syndrome: Two case reports.
World J Clin Cases. 2020 Mar 26;8(6):1142-1149
Authors: Jiang YE, Lyu QQ, Lin F, You XT, Jiang ZL
Abstract
BACKGROUND: A swallowing disorder may occur following a brainstem stroke, especially one that occurs in the swallowing centers. Lateral medullary syndrome (referred to as LMS), a rare condition in which a vascular event occurs in the territory of the posterior inferior cerebellar artery or the vertebral artery, has been reported to lead to more severe and longer lasting dysphagia.
CASE SUMMARY: We report two patients with dysphagia due to LMS and propose a novel technique named hyoid-complex elevation and stimulation technique (known as HEST). The two patients had no other functional incapacity back into life, but nasogastric feeding was the only possible way for nutrition because of severe aspirations. Swallowing function was evaluated by functional oral intake scale, modified water swallow test, surface electromyographic signal associated with video fluorography swallowing study to assess the situation of aspiration, pharyngeal residue, pharyngeal peristalsis, upper esophageal opening and the ability of deglutition. Both patients were treated with the HEST method for dysphagia and recovered quickly.
CONCLUSION: HEST is effective for shortening the in-hospital time and improving the quality of life for patients with dysphagia who suffer from LMS and likely other strokes.
PMID: 32258085 [PubMed]
Metabolic and genetic assessments interpret unexplained aggressive pulmonary hypertension induced by methylmalonic acidemia: A case report.
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Metabolic and genetic assessments interpret unexplained aggressive pulmonary hypertension induced by methylmalonic acidemia: A case report.
World J Clin Cases. 2020 Mar 26;8(6):1137-1141
Authors: Liao HY, Shi XQ, Li YF
Abstract
BACKGROUND: Pulmonary hypertension (PH) causes significant morbidity and mortality in diverse childhood diseases. However, limited information has been reported to obtain a good understanding of pediatric PH. Gaps exist between genome sequencing and metabolic assessments and lead to misinterpretations of the complicated symptoms of PH. Here, we report a rare case of a patient who presented with severe PH as the first manifestation without significant cardiovascular malformation and was finally diagnosed with methylmalonic aciduria (MMA) after metabolic and genomic assessments.
CASE SUMMARY: An 11-year-old female presented with an aggressive reduction in activity capability and shortness of breath for only 4 mo and suffered from unexplained PH. A series of examinations was performed to evaluate any possible malformations or abnormalities of the cardiovascular system and lungs, but negative results were obtained. The blood tests were normal except for manifestations of microcytic anemia and elevated total homocysteine. Computed tomography and magnetic resonance imaging failed to identify any pulmonary diseases. Cardiac catheterization examination identified a small right coronary artery to pulmonary artery shunt and severe PH. During the follow-up, PH progressed rapidly. Then, genome sequencing and metabolic disorder screening were performed, which confirmed a diagnosis of MMA with MMACHC c.80A > G/c and 609G > A mutations. Vitamin B12, betaine and bosentan were then administered as the main treatments. During the 6-mo follow-up, the pulmonary artery pressure dropped to 45 mmHg, while the right ventricle structure recovered. The patient's heart function recovered to NYHA class II. Metabolic disorder analysis failed to identify significant abnormalities.
CONCLUSION: As emerging types of metabolic dysfunction have been shown to present as the first manifestation of PH, and taking advantage of next generation sequencing technology, genome sequencing and metabolic disorder screening are recommended to have a more superior role when attempting to understand unclear or aggressive PH.
PMID: 32258084 [PubMed]
Successful treatment of systemic sclerosis complicated by ventricular tachycardia with a cardiac resynchronization therapy-defibrillator: A case report.
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Successful treatment of systemic sclerosis complicated by ventricular tachycardia with a cardiac resynchronization therapy-defibrillator: A case report.
World J Clin Cases. 2020 Mar 26;8(6):1129-1136
Authors: Chen YY, Yan H, Zhu JH
Abstract
BACKGROUND: Systemic sclerosis is a rare connective tissue disease characterized by localized or diffuse skin thickening and fibrosis, which usually accumulates in various organs throughout the body. Tachyarrhythmia is a common clinical manifestation of cardiovascular damage in systemic sclerosis patients. However, few studies have reported the use of catheter ablation and an implantable cardioverter defibrillator in patients with systemic sclerosis complicated by ventricular tachycardia.
CASE SUMMARY: A 39-year woman with an 11-year history of systemic sclerosis was referred to our hospital due to three syncopal episodes in the past 6 mo. The results of an electrocardiogram and a transthoracic echocardiogram revealed ventricular tachycardia and left ventricular systolic and ventricular septum segmental motion abnormalities, respectively. The results of an electrocardiogram showed a sinus rhythm with complete blockage of the left bundle branch. In light of the progressive nature of systemic sclerosis, the presence of a left bundle branch block, and the decreased ejection fraction, a cardiac resynchronization therapy-defibrillator was implanted. The patient's clinical conditions improved, and at the 3-mo follow-up, the patient was free of ventricular tachycardia and all cardiac symptoms.
CONCLUSION: We report the first case of systemic sclerosis complicated by ventricular tachycardia that was successfully treated with a cardiac resynchronization therapy-defibrillator.
PMID: 32258083 [PubMed]
Hepatoid carcinoma of the pancreas: A case report and review of the literature.
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Hepatoid carcinoma of the pancreas: A case report and review of the literature.
World J Clin Cases. 2020 Mar 26;8(6):1116-1128
Authors: Zeng SX, Tan SW, Fong CTH, Liang Q, Zhao BL, Liu K, Guo JX, Tao J
Abstract
BACKGROUND: Hepatoid carcinoma (HC) is an extremely rare neoplasm that is morphologically similar to hepatocellular carcinoma. HC has been described in various organs; however, HC of the pancreas is extremely rare. To our knowledge, only 38 cases have been reported. We present a case of HC of the pancreas in a 36-year-old male patient.
CASE SUMMARY: A 36-year-old cachexic man with no significant past medical history was transferred to our hospital with a history of painless jaundice, elevated blood glucose and significant weight loss. Lab tests showed elevated serum transaminases, bilirubin and alpha-fetoprotein levels. Magnetic resonance imaging of the upper abdomen showed a diffusely enlarged pancreas, appearing "sausage-shaped". Magnetic resonance cholangiopancreatography showed upstream ductal dilation secondary to stricture of the main pancreatic duct and the common bile duct, which were not visible. Immunohistochemistry of biopsied tissue from a percutaneous pancreatic biopsy showed tumor cell positivity for HepPar1, polyclonal carcinoembryonic antigen and CK19, suggestive of HC of the pancreas. The characteristics of 39 patients with HC of the pancreas were reviewed.
CONCLUSION: HC of the pancreas is more prevalent in males, and patients have a median age of 57 years. It is most commonly asymptomatic or presents as abdominal back pain, and the pancreatic tail is the most common location. At the time of diagnosis, liver metastasis is often present.
PMID: 32258082 [PubMed]
Abnormal serum carbohydrate antigen 19-9 levels in a patient with splenic retiform haemangioendothelioma concomitant with hepatic amyloidosis: A case report.
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Abnormal serum carbohydrate antigen 19-9 levels in a patient with splenic retiform haemangioendothelioma concomitant with hepatic amyloidosis: A case report.
World J Clin Cases. 2020 Mar 26;8(6):1108-1115
Authors: Sun KD, Zhang YJ, Zhu LP, Yang B, Wang SY, Yu ZH, Zhang HC, Chen X
Abstract
BACKGROUND: Carbohydrate antigen 19-9 (CA 19-9) is a glycoprotein that is used as a reliable tool for monitoring pancreatic cancer. Serum CA 19-9 levels are increased in patients suffering from liver, lung, and other non-malignant diseases. Haemangioendothelioma is a vascular neoplasm with a borderline biological behaviour. However, no case of haemangioendothelioma has yet been reported to be associated with CA 19-9.
CASE SUMMARY: A 54-year-old Chinese man was referred to our hospital for discontinuous fatigue and unintentional weight loss for over one year. Laboratory investigations revealed an elevated serum CA 19-9 concentration of 39 IU/mL (reference interval, 0-37 IU/mL) over one year before admission. Afterwards, coagulopathy appeared, and the patient's serum CA 19-9 concentration increased continuously. At the time of admission, abdominal pain and haemorrhagic shock burst occurred, and emergency medical operation was performed. Laboratory investigations conducted upon admission showed a serum CA19-9 concentration of 392.56 IU/mL. Surgical resection of the spleen was undertaken, and pathological examination showed retiform haemangioendothelioma. The patient developed jaundice ten days after surgical excision of the spleen. Pathological examination of needle biopsy samples of the liver yielded a diagnosis of hepatic amyloidosis.
CONCLUSION: We describe a rare case of splenic retiform haemangioenthelioma concomitant with hepatic amyloidosis. Physicians should note abnormal serum CA 19-9 levels with early symptoms of fatigue and unintentional weight loss.
PMID: 32258081 [PubMed]

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