scholarly journals Hilaiditi syndrome as a cause of chronic duodenal obstruction in children operated on for omphalocele

Author(s):  
A. Yu. Razumovskiy ◽  
N. V. Kulikova ◽  
V. V. Cholostova ◽  
M. A. Amanova ◽  
A. I. Khavkin

Introduction. Chilaiditi syndrome—hepatodiaphragmatic interposition—is a rare condition in which bowel loops are compressed in the space between the liver and the dome of the diaphragm, which can lead to the development of a wide range of complications. In the available literature data on the clinical manifestations of this condition in children with omphalocele are not described.Materials and methods. The article describes rare clinical cases of patients 1 year 1 month, 2 years 11 month and 12 years old, previously operated on in the neonatal period for omphalocele, in whom, in the long term after surgery, signs of hepatodiaphragmatic interposition persisted, which led to the development of chronic duodenal obstruction.Conclusion: It has been proven that the leading role in restoring the normal topography of the abdominal organs in children with omphalocele is played by the complete mobilization of the liver and the great vessels (IVC) fi xing it, which provide the possibility of its subphrenic localization. Chronic duodenal obstruction in children with a similar pathology has not been previously described. The proposed original method of surgical treatment seems to be pathogenetically justifi ed.

2015 ◽  
Vol 172 (5) ◽  
pp. R179-R190 ◽  
Author(s):  
Cristina Capatina ◽  
Warrick Inder ◽  
Niki Karavitaki ◽  
John A H Wass

Pituitary tumour apoplexy (PA) is a rare clinical syndrome that occurs as a result of acute haemorrhage and/or infarction within a frequently undiagnosed pituitary tumour. The sudden enlargement of the pituitary mass undergoing PA is responsible for a wide range of acute symptoms/signs (severe headache, visual loss, diplopia, hypopituitarism, impaired consciousness) which, together with the radiological evidence of a pituitary lesion, establish the diagnosis. The optimal care of PA requires involvement of a multidisciplinary team including endocrinologist, neurosurgeon, neuroophthalmologist and the management strategy that depends on the clinical manifestations, as well as the presence of co-morbidities. Prompt surgical decompression is initially indicated in cases with severe or progressive impairment of the visual acuity or the visual fields or with altered mental state and leads to visual and neurological recovery in most of the patients. The patients with mild, stable clinical picture (including those with isolated ocular palsies) can be managed conservatively (support of fluid and electrolyte balance and stress doses of steroids in most cases) with favourable visual and neurological outcome. Frequent reassessment is mandatory because the clinical course can be unpredictable; if progression of symptoms occurs, later elective surgery is indicated and is beneficial, especially in terms of visual outcome. The endocrinological outcome is less favourable, irrespective of the treatment option, with many patients remaining on long-term replacement therapy. Despite the above guidelines, clear proof of optimal outcomes in the form of randomised controlled trials is lacking. Regrowth of the pituitary tumour years after a PA episode is possible and patients require long-term surveillance.


2021 ◽  
Vol 13 (2) ◽  
pp. 154-158
Author(s):  
K. V. Sobolevskiy ◽  
I. A. Otmakhova ◽  
B. Ya. Gadirova ◽  
Zh. E. Zueva

Parvovirus infection is one of the most widespread infectious diseases in the world. Parvovirus В19 can cause a wide range of clinical manifestations with possible long-term viral persistence. Infectious erythema, arthropathy, fetal infection, and blood cell aplasia are the most common manifestations of parvovirus infection. A case of diagnosis and treatment of a rare variant of parvovirus B19 infection that occurred as acute hepatitis is presented. 


2021 ◽  
Vol 17 (2) ◽  
pp. 6-15
Author(s):  
L.A. Dziak ◽  
O.S. Tsurkalenko ◽  
K.V. Chekha ◽  
V.M. Suk

Coronavirus infection is a systemic pathology resulting in impairment of the nervous system. The involvement of the central nervous system in COVID-19 is diverse by clinical manifestations and main mechanisms. The mechanisms of interrelations between SARS-CoV-2 and the nervous system include a direct virus-induced lesion of the central nervous system, inflammatory-mediated impairment, thrombus burden, and impairment caused by hypoxia and homeostasis. Due to the multi-factor mechanisms (viral, immune, hypoxic, hypercoagulation), the SARS-CoV-2 infection can cause a wide range of neurological disorders involving both the central and peripheral nervous system and end organs. Dizziness, headache, altered level of consciousness, acute cerebrovascular diseases, hypogeusia, hyposmia, peripheral neuropathies, sleep disorders, delirium, neuralgia, myalgia are the most common signs. The structural and functional changes in various organs and systems and many neurological symptoms are determined to persist after COVID-19. Regardless of the numerous clinical reports about the neurological and psychiatric symptoms of COVID-19 as before it is difficult to determine if they are associated with the direct or indirect impact of viral infection or they are secondary to hypoxia, sepsis, cytokine reaction, and multiple organ failure. Penetrated the brain, COVID-19 can impact the other organs and systems and the body in general. Given the mechanisms of impairment, the survivors after COVID-19 with the infection penetrated the brain are more susceptible to more serious diseases such as Parkinson’s disease, cognitive decline, multiple sclerosis, and other autoimmune diseases. Given the multi-factor pathogenesis of COVID-19 resulting in long-term persistence of the clinical symptoms due to impaired neuroplasticity and neurogenesis followed by cholinergic deficiency, the usage of Neuroxon® 1000 mg a day with twice-day dosing for 30 days. Also, a long-term follow-up and control over the COVID-19 patients are recommended for the prophylaxis, timely determination, and correction of long-term complications.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Urmila Anandh ◽  
Rakesh Kumar ◽  
Vishnu Rao

Calcific uremic arteriolopathy is a rare condition affecting chronic kidney disease (CKD) patients on long-term dialysis. The clinical manifestations include subcutaneous skin necrosis and ulcers secondary to calcification of the subcutaneous blood vessels. The necrotic tissue often becomes a nidus of infection. The prognosis is often poor. We present a case of a renal allograft recipient who developed a subcutaneous necrotic lesion which was subsequently infected by Rhizopus spp. The patient underwent surgical debridement and antifungal therapy. The infection resolved completely. Our case represents agrave underlying condition predisposing a rare and serious posttransplant infection. The outcome was favourable because of early identification and treatment of the infection.


2017 ◽  
Vol 11 (2) ◽  
pp. 428-433 ◽  
Author(s):  
Hrudya Abraham ◽  
Sajan Thomas ◽  
Amit Srivastava

Biliary sump syndrome is a rare condition. It is seen as a rare long-term complication in patients with a history of a side-to-side choledochoduodenostomy. In the era before endoscopic retrograde cholangiopancreatography, side-to-side choledochoduodenostomy was a common surgical procedure for the management of biliary obstruction. In the setting of a side-to-side choledochoduodenostomy, the bile does not drain through the distal common bile duct anymore. Therefore, the part of the common bile duct distal from the choledochoduodenostomy anastomosis consequently transforms into a poorly drained reservoir, making this so-called “sump” prone to accumulation of debris. These patients are prone to cholangitis. We present a 64-year-old man with a history of side-to-side choledochoduodenostomy who presented with manifestations of cholangitis. An endoscopic retrograde cholangiopancreatography confirmed a diagnosis of sump syndrome. The etiology, clinical manifestations, and treatment of biliary sump syndrome are discussed in this article.


2020 ◽  
Vol 11 (3) ◽  
pp. 54-59
Author(s):  
S. B. Berezhansky ◽  
A. A. Afonin ◽  
E. A. Papsheva ◽  
N. N. Vostrykh ◽  
G. A. Galkina ◽  
...  

A clinical case of congenital hyperinsulinism, diffuse form, pharmacoresistant course (heterozygous mutation of p. 1361 1363 dup CGG in the GCK gene) in an early child is presented as an example of an orphan severe disease with an extremely unfavorable course and a probability of deterioration of the long-term prognosis. The goal was to highlight the clinical manifestations, course options, and complexity of treatment of this pathology to a wide range of doctors of different specialties in the field of Pediatrics in terms of improving the quality and timeliness of diagnosis, reducing the number of complications with the formation of irreparable consequences. Attention is drawn to the most severe course of hypoglycemic conditions in the early neonatal period, the dependence of the formation of a pronounced neurological deficit on the degree and duration of hypoglycemia, which emphasizes the importance of their timely correction to preserve the quality of life of this contingent of children.


2009 ◽  
Vol 66 (6) ◽  
pp. 434-439 ◽  
Author(s):  
Ljiljana Kesic ◽  
Radmila Obradovic ◽  
Dragan Mihailovic ◽  
Goran Radicevic ◽  
Sasa Stankovic ◽  
...  

Background/Aim. Lichen planus is a chronic, immunologic, mucocutaneous disease with a wide range of clinical manifestations. The aim of this retrospective study was to evaluate the most common forms of oral lichen planus (OLP) and its symptoms and to describe treatment responses in patients during 10-year period. Methods. The study was conduced on 163 OLP patients who came in the Department of Oral medicine and Periodontology between 1997 and September 2007. Each case was classified into one of four clinical subtypes: reticular, atrophic, erosive-ulcerative, bullous. Results. There was no significant difference in patients age. Women were found to be significantly more likely to have OLP (p < 0.001). Corticosteroids were effective in reducing symptoms, erythema and healing ulcers. Improvement was shown over a long term in 61.35% patients. Over the long term 38.65% patients maintained the same type of OLP or it became a more severe type. Two patients (1.22%) developed oral carcinoma during the follow-up period. Conclusion. The response of patients with erosive OLP to a short course of systemic corticosteroids often was quite remarkable. However, symptoms and signs tended to recur after this treatment. Periodic examinations, patient education, medical treatment, monitoring of side-effects as well as follow-up biopsies are necessary for management of OLP patients.


2021 ◽  
Vol 28 (2) ◽  
pp. 104-119
Author(s):  
M. M. Tlish ◽  
P. S. Osmolovskaya

Background. Lichen planus ruber is a common inflammatory disease of skin and mucous membranes with a chronic relapsing course, resistance to conventional therapy, diverse clinical manifestations and capacity of tumourisation. Dermatosis is often comorbid with various underlying gastrointestinal, cardiovascular illnesses and endocrinopathies. Women are more impacted than men (ratio 1.4:1), the disease lasts from 5 to 40 years.Objectives. Assessment of the efficacy and safety of current therapies for lichen planus ruber.Methods. The PubMed, Web of Science, Medline, eLibrary and Scopus databases were mined for randomised controlled studies on treatment of this dermatosis in the period of 2014–2020, and selected impactive publications within 1991–2012 were also included in the review. The following keywords were used: lichen planus [красный плоский лишай], systemic and local drugs [системные и местные препараты], phototherapy [фототерапия], physiotherapeutic methods of treatment [физиотерапевтические методы лечения]. Publications in peer-reviewed journals had priority. Content and descriptive analyses were used as research tools.Results. Therapy for lichen planus ruber is multicomponent including a wide range of techniques, mostly immune-stimulating but often unproved for efficacy and safety. Selected studies are small-sample. The review presents feasible drug-based and physiotherapeutic methods to tackle lichen planus ruber, their application by the disease clinical presentation and prevalence, patient’s personal discomfort, psycho-emotional status and comorbidity. We describe the mechanisms of action, clinical efficacy, application types and noxious side effects in practiced albeit sometimes contentious therapies.Conclusion. Further well-designed research is warranted to aim at enhanced efficacy, safety and long-term remission in therapy for lichen planus ruber.


2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Neeraja Swaminathan ◽  
Jorge M. Vinicius ◽  
Jesse Serrins

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by an overwhelming inflammatory cascade activation which is often associated with rapid progression and high mortality. It may be familial with an underlying genetic mutation or triggered by infection, malignancy, and autoimmune disease. Disseminated histoplasmosis caused by histoplasma capsulatum is a granulomatous fungal disease seen typically in immunocompromised patients with varied clinical manifestations and requires long-term antifungal therapy. We present the case of a 61-year-old immunocompromised female with significant travel history who came with fever, pancytopenia, and liver failure raising suspicion for HLH that prompted a bone marrow biopsy procedure. Hemophagocytic figures consistent with HLH and numerous encapsulated fungi resembling histoplasma were visualized. She was treated with intravenous (IV) liposomal amphotericin B. Etoposide chemotherapy and interleukin-1 (IL-1) antagonist anakinra were deferred in order to limit her immunosuppression, and treatment was focused on antifungal therapy.


2020 ◽  
Vol 182 (3) ◽  
pp. C9-C12
Author(s):  
Clemens Kamrath

Primary adrenal insufficiency (PAI) in children is mostly due to genetic defects. The understanding of the molecular genetics of the causes of adrenal insufficiency in the pediatric population has made significant progress during the last years. It has been shown that inherited PAI can lead to certain clinical manifestations and health problems in children beyond the adrenals. Organ dysfunctions associated with different forms of PAI in children include a wide range of organs such as gonads, brain, heart, bone, growth, bone marrow, kidney, skin, parathyroid, and thyroid. Diagnosing the correct genetic cause of PAI in children is therefore crucial to adequately control long-term treatment and follow-up in such patients.


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