Full-blown Syndrome Research Articles

Neuro-psychiatric symptoms in directly and indirectly blast exposed civilian survivors of urban missile attacks

BackgroundBlast-explosion may cause traumatic brain injury (TBI), leading to post-concussion syndrome (PCS). In studies on military personnel, PCS symptoms are highly similar to those occurring in post-traumatic stress disorder (PTSD), questioning the overlap between these syndromes. In the current study we assessed PCS and PTSD in civilians following exposure to rocket attacks. We hypothesized that PCS symptomatology and brain connectivity will be associated with the objective physical exposure, while PTSD symptomatology will be associated with the subjective mental experience.MethodsTwo hundred eighty nine residents of explosion sites have participated in the current study. Participants completed self-report of PCS and PTSD. The association between objective and subjective factors of blast and clinical outcomes was assessed using multivariate analysis. White-matter (WM) alterations and cognitive abilities were assessed in a sub-group of participants (n = 46) and non-exposed controls (n = 16). Non-parametric analysis was used to compare connectivity and cognition between the groups.ResultsBlast-exposed individuals reported higher PTSD and PCS symptomatology. Among exposed individuals, those who were directly exposed to blast, reported higher levels of subjective feeling of danger and presented WM hypoconnectivity. Cognitive abilities did not differ between groups. Several risk factors for the development of PCS and PTSD were identified.ConclusionsCivilians exposed to blast present higher PCS/PTSD symptomatology as well as WM hypoconnectivity. Although symptoms are sub-clinical, they might lead to the future development of a full-blown syndrome and should be considered carefully. The similarities between PCS and PTSD suggest that despite the different etiology, namely, the physical trauma in PCS and the emotional trauma in PTSD, these are not distinct syndromes, but rather represent a combined biopsychological disorder with a wide spectrum of behavioral, emotional, cognitive and neurological symptoms.

An Ayurvedic Review on Poly-Cystic Ovarian Disease

PCOD is the most common endocrine disorder in women of reproductive age group, affecting 5% to 10% of women exhibiting, the full blown syndrome of hyperandrogenism, chronic anovulation and polycystic ovaries. PCOD is a disease which is related to the cystic changes in the ovary. It is the most common cause of Menstrual Irregularities, Obesity, anovulatory infertility and hyperandrogenism, etc. PCOD are not compiled as a disease or syndrome in Ayurveda, most of them have been described as symptoms of separate diseases or conditions. Ayurvedic management principles Samshodhana, Agneya dravya, Nidan parivarjana as well as modern treatment describe here. The present article is an attempt to highlighting on details of Ayurvedic review on poly cystic ovarian diseases.

MO108: Paraneoplastic Nephrotic Syndrome in a Man with Breast Cancer

Abstract BACKGROUND Nephrotic syndrome is associated with many solid tumours and haematologic malignancies. Membranous nephropathy (MN) is the most commonly reported paraneoplastic glomerulonephritis; about 10% (rate 5–20%) of adults with MN have been reported to have a malignancy [1]. Less frequently, minimal change disease, focal segmental glomerular sclerosis, membranoproliferative and crescentic glomerulonephritis, IgA nephropathy [1] and thrombotic microangiopathy [2] have been associated with cancer. Differentiating primary from secondary glomerular disease in patients with malignancy can be difficult. The probability of a secondary form is higher when the appearance of proteinuria or nephrotic syndrome precedes by few months or develops within a year of cancer diagnosis. The suspicion of paraneoplastic nephropathy is even stronger when tumour remission is associated by significant fall in proteinuria and when recurrence of malignancy is followed by the development of clinical glomerular disease. Solid tumour most commonly associated with glomerular diseases (mainly MN) is lung cancer, followed by prostate and colorectal cancer. Paraneoplastic glomerular diseases are rarely linked to breast cancer. In two reviews, breast cancer was reported only in 3% (5 out of 159 patients) [1] and 8% (6 out of 85 patients) of patients with MN and solid tumours. Breast cancer is the most common malignancy in women, while it is a rare disease in men. Male breast cancers account for less than 1% of all diagnosed cases, and the mean age is usually over 60 years [1]. METHODS We report the case of a breast cancer in a man associated with a paraneoplastic nephrotic syndrome. A 76-year-old man was managed at our renal department since 2016 for chronic kidney disease. He was overweight and affected by non-insulin dependent diabetes mellitus, arterial hypertension and coronary artery disease. Since 2016 to December 2020, he had stable renal function with serum creatinine levels ranging from 2.5 to 3.0 mg/dL and a normal urinary protein excretion rate (100–150 mg/24 h). In March 2021, the patient developed a right periareolar breast swelling, pain and erythema with yellowish nipple discharge. In the same period, a full-blown nephrotic syndrome with heavy proteinuria (8 g/24 h) was observed, and serum creatinine increased to 3.5–4.0 mg/dL. Immunological blood tests were negative, including anti-PLA2R and anti-THSD7A antibodies. A kidney biopsy could not be performed due to bilateral renal cysts. Mammogram examination and ultrasound imaging revealed ductal ectasia and microscopic calcifications at the site of right breast lesion, so a diagnosis of cancer was made. RESULTS In September 2021, the patient underwent radical right mastectomy and histopathological examination confirmed early-stage breast cancer (pT1a, pN0, Mx R0, ER 100%, PgR 0, Ki67 5%, CerbB2 neg). In the following month, the 24-h urinary protein excretion markedly reduced to 1.4 g and the nephrotic syndrome resolved completely, confirming its secondary origin. CONCLUSIONS Paraneoplastic nephrotic syndrome is a rare complication of cancer. Its recognition and detection of an undiagnosed malignancy could be life-saving. To our knowledge, this is the first case reported in literature of paraneoplastic nephrotic syndrome in a man affected by breast cancer.

Prevalence, comorbidity, quality of life and use of services of obsessive-compulsive disorder and subthreshold obsessive-compulsive symptoms in the general adult population of Greece

Objective: We aimed to describe the epidemiology of obsessive-compulsive disorder (OCD) and related subthreshold symptoms in the general population of Greece and to assess the comorbidity, use of health services and impact on quality of life of these syndromes in the general population.Methods: This was a secondary analysis of the 2009–2010 general population Greek psychiatric morbidity survey (4902 participants living in private households, response rate 54%). Psychiatric disorders were assessed with the revised Clinical Interview Schedule (CIS-R). Quality of life was assessed with the EuroQoL EQ-5D.Results: 1.69% of the participants (95% confidence interval [CI]: 1.33%–2.05%) met criteria for current OCD while 2.79% met criteria for subthreshold obsessive-compulsive symptoms (95%CI: 2.33%–3.26%). In the adjusted analysis few sociodemographic associations remained statistically significant. Although the full-blown syndrome was more severe in terms of comorbidity and quality of life our results showed that even subthreshold obsessive-compulsive symptoms were associated with significant comorbidity and reductions in quality of life. Use of mental health services was small.Conclusions: OCD is an under-treated public health problem that needs more attention in Greece and elsewhere. Provision of specialised mental health services should be a priority for publicly funded national health systems.KeypointsOCD is common in the general population of Greece with few sociodemographic associations apart from subjective financial difficulties.The comorbidity pattern of the full-blown syndrome versus the subthreshold obsessive-compulsive symptoms is similar and differs in magnitude in the expected way.Quality of life in OCD is greatly reduced especially when there is chronicity and/or comorbidity with depression.Use of services is limited but comorbidity with depression may increase the chances of consultation with a mental health professional.

Serum betatrophin levels are reduced in patients with full-blown polycystic ovary syndrome

Betatrophin is defined as a new marker in glucose homeostasis and lipid metabolism. We aimed to investigate the role of serum betatrophin in full-blown polycystic ovary syndrome (PCOS) patients and 47-aged healthy women, 51 full-blown PCOS patients were included in this cross-sectional study. Betatrophin concentrations were significantly lower in PCOS group and displayed a positive correlation only with serum tryglyceride in control group (p < .05). A cutoff level (464.5 ng/L) was determined for betatrophin according to Receiver Operating Characteristic curve. Using this value, 64.7% of PCOS patients were classified as below the cutoff and in this group betatrophin was found to correlate negatively with fasting glucose, fasting insulin, and homeostasis model assessment of insulin resistance (p = .038, p = .020, and p = .014, respectively), and positively with total testosterone (p = .041). In the rest of PCOS cases (35.3%) who had betatrophin higher than cutoff, positive correlation was found with low-density lipoprotein cholesterol (p = .009). In conclusion, betatrophin levels are reduced in full-blown PCOS patients who had worse metabolic phenotype.

Incomplete Distal Renal Tubular Acidosis and Kidney Stones.

Renal tubular acidosis (RTA) is comprised of a diverse group of congenital or acquired diseases with the common denominator of defective renal acid excretion with protean manifestation, but in adults, recurrent kidney stones and nephrocalcinosis are mainly found in presentation. Calcium phosphate (CaP) stones and nephrocalcinosis are frequently encountered in distal hypokalemic RTA type I. Alkaline urinary pH, hypocitraturia, and, less frequently, hypercalciuria are the tripartite lithogenic factors in distal RTA (dRTA) predisposing to CaP stone formation; the latter 2 are also commonly encountered in other causes of urolithiasis. Although the full blown syndrome is easily diagnosed by conventional clinical criteria, an attenuated forme fruste called incomplete dRTA typically evades clinical testing and is only uncovered by provocative acid-loading challenges. Stone formers (SFs) that cannot acidify urine of pH<5.3 during acid loading are considered to have incomplete dRTA. However, urinary acidification capacity is not a dichotomous but rather a continuous trait, so incomplete dRTA is not a distinct entity but may be one end of a spectrum. Recent findings suggest that incomplete dRTA can be attributed to heterozygous carriers of hypofunctional V-ATPase. The value of incomplete dRTA diagnosis by provocative testing and genotyping candidate genes is a valuable research tool, but it remains unclear at the moment whether they alter clinical practice and needs further clarification. No randomized controlled trials have been performed in SFs with dRTA or CaP stones, and until such data are available, treatment of CaP stones are centered on reversing the biochemical abnormalities encountered in the metabolic workup. SFs with type I dRTA should receive alkali therapy, preferentially in the form of K-citrate delivered judiciously to treat the chronic acid retention that drives both stone formation and bone disease.

Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital.

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder; there is limited experience regarding its clinical course and therapeutic response. To describe the clinical profile, investigations, and therapeutic outcome in pediatric OMAS. Fourteen children (age: 27.1 ± 7 months; male: female = 1:2.3) suffering from OMAS seen over a period of 10 years (2006-2015) were included in the study. Their clinicodemographic profile, investigations, therapeutic outcome at follow-up, and relapses were reviewed. Ten children reported antecedent events (respiratory infection: 7; gastrointestinal infection: 1; vaccination: 2). The most common referral diagnosis was acute cerebellitis (n = 8). Hypotonia (n = 9), abnormal behavior (n = 10), and neuroregression (n = 6) were also the frequent manifestations. Brain magnetic resonance imaging, cerebrospinal fluid, and urinary vanillylmandelic acid were normal in all the patients. Seven patients had an underlying tumor (abdomen: 4; thorax: 2; neck: 1) detected by ultrasound (n = 2/14), computed tomography (CT) (n = 6/12), and fluorodeoxyglucose - positron emission tomography (n = 2/2). CT scan identified the tumor in 2 patients where metaiodobenzylguanidine scintigraphy was negative. All patients received steroids for 22.3 ± 20 months (3 months to 5 years). Eight required prolonged immunomodulation (>12 months). Complete remission after follow-up of 31.3 ± 19 months (7 months to 5 years) was noted in 5 patients, whereas the rest had persisting behavioral and cognitive abnormalities. Relapses were noted in 6 patients related to intercurrent infections (n = 5) and discontinuation of steroids (n = 1). The patients presented with isolated symptoms of the full-blown syndrome during their relapses. OMAS in children runs an indolent course requiring careful monitoring and long-term immunomodulation. An abnormal behavior is common and the outcome is variable.

Lytic viral replication and immunopathology in a cytomegalovirus-induced mouse model of secondary hemophagocytic lymphohistiocytosis

BackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare immunological disorder caused by unbridled activation of T cells and macrophages, culminating in a life-threatening cytokine storm. A genetic and acquired subtype are distinguished, termed primary and secondary HLH, respectively. Clinical manifestations of both forms are frequently preceded by a viral infection, predominantly with herpesviruses. The exact role of the viral infection in the development of the hemophagocytic syndrome remains to be further elucidated.MethodsWe utilized a recently developed murine model of cytomegalovirus-associated secondary HLH and dissected the respective contributions of lytic viral replication and immunopathology in its pathogenesis.ResultsHLH-like disease only developed in cytomegalovirus-susceptible mouse strains unable to clear the virus, but the severity of symptoms was not correlated to the infectious viral titer. Lytic viral replication and sustained viremia played an essential part in the pathogenesis since abortive viral infection was insufficient to induce a full-blown HLH-like syndrome. Nonetheless, a limited set of symptoms, in particular anemia, thrombocytopenia and elevated levels of soluble CD25, appeared less dependent of the viral replication but rather mediated by the host’s immune response, as corroborated by immunosuppressive treatment of infected mice with dexamethasone.ConclusionBoth virus-mediated pathology and immunopathology cooperate in the pathogenesis of full-blown virus-associated secondary HLH and are closely entangled. A certain level of viremia appears necessary to elicit the characteristic HLH-like symptoms in the model.

Full blown nephrotic syndrome due to unilateral obstruction of the uretero-pelvic junction

Full blown nephrotic syndrome due to unilateral obstruction of the uretero-pelvic junction

Anti-N-methyl-D-aspartate receptor(NMDAR) antibody encephalitis presents in atypical types and coexists with neuromyelitis optica spectrum disorder or neurosyphilis

BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases of anti-NMDAR encephalitis with incomplete forms, 3 cases of which were accompanied by neuromyelitis optica spectrum disorder or neurosyphilis respectively.Case presentationA 33-year-old man presented with dysarthria, movement disorder and occasional seizures. He had 6 relapses in 28 years. When suffered from upper respiratory tract syndrome, he developed behavioral and consciousness impairment. Cranial MRI was normal. Viral PCR studies and oncologic work-up were negative. Anti-NMDAR antibody was detected in CSF and serum.A 21-year-old female manifested dizziness and diplopia ten months and six months before, respectively. Both responded to steroid therapy and improved completely. This time she presented with progressive left limb and facial anesthesia, walking and holding unsteadily. Spinal cord MRI follow-up showed abnormality of medulla oblongata and cervical cord(C1). Anti-AQP4 and anti-NMDAR were positive in CSF. Steroid-pulse therapy ameliorated her symptoms.A 37-year-old male experienced worsening vision. He was confirmed neurosyphilis since the CSF tests for syphilis were positive. Protein was elevated and the oligoclonal IgG bands(OB) and anti-NMDAR was positive in CSF. Anti-aquaporin 4(AQP4) antibodies and NMO-IgG were negative. Cranial MRI showed high FLAIR signal on frontal lobe and low T2 signal adjacent to the right cornu posterious ventriculi lateralis. Treatment for neurosyphlis was commenced with gradual improvement.A 39-year-old male, developed serious behavioral and psychiatric symptoms. Examination showed abnormal pupils and unsteady gait. He was confirmed neurosyphilis according to the CSF tests for syphilis. Anti-NMDAR was positive in CSF and serum. Cranial MRI showed lateral ventricles and the third ventricle enlargement and signal abnormality involving bilateral temporal lobe, corona radiate and centrum semiovale. PenicillinG, pulsed methylprednisolone and intravenous immunoglobulin was administered. He was stable.ConclusionAnti-NMDAR encephalitis can present in atypical types. When relapsing, it may present with partial aspects or with isolated symptoms of the full-blown syndrome. Anti-NMDAR encephalitis may be related to neuromyelitis optica spectrum disorder or neurosyphilis.

«Verschroben»-phenomenon in schizophrenia and schizophrenia spectrum disorders: aspects of systematics

Psychopathological analysis and typological differentiation of «verschroben»-phenomenon as the full-blown syndrome of defect in schizophrenia and schizophrenia spectrum disorders. Fifty patients, 33 female and 17 male, mean age 44.8±8.5 years, with ICD-10 diagnosis of schizotypal disorder (F21) and residual schizophrenia (F20.5) were included in the study. Patients were examined using psychopathological and psychometric (SPQ-74) methods. There was clinical heterogeneity of the «verschroben» type defect: the differentiation of primary defect syndrome and the «second life» defect was confirmed. This typological classification is based not only on the differences in psychopathological symptoms and impaired social functioning but on the trajectory of the development of deficit syndromes related with the features of the course of endogenous disease. The typology would be useful for future studies of deficit changes in view of clinical prognosis, social context of schizophrenia spectrum disorders with negative «verschroben» type changes.

Acute Post-Streptococcal Glomerulonephritis in Children

Acute post-streptococcal glomerulonephritis is the most common form of glomerulonephritis in children, continues to be a major concern for pediatricians and nephrologists worldwide. The disease is caused by prior streptococcal infection caused by -hemolytic streptococcus of A group. Nephritis-associated plasmin receptor and streptococcal pyrogenic exotoxin B are considered to be major renotrophic antigens. Clinical findings of the disease can vary from asymptomatic gross hematuria to the full-blown acute nephritic syndrome (edema, gross hematuria, hypertension, renal failure). The main laboratory findings of acute post-streptococcal glomerulonephritis include an abnormal common urine analysis (dysmorphic erythrocytes, varying degrees of proteinuria, red blood cell casts, and segmentonuclear neutrophils), an elevated titer of antibodies to antistreptococcic antigens, and hypocomplementemia. Treatment of patients is mainly supportive and is focused upon the elimination of hypervolemia and its complications (edema, hypertension). In this situation, the most effective agents are loop diuretics and antihypertensive agents. The prognosis in children is generally favourable. The review analyses the current understanding of the pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of acute post-streptococcal glomerulonephritis.

Early hyperreactive malarial splenomegaly and risk factors for evolution into the full-blown syndrome: a single-centre, retrospective, longitudinal study.

BackgroundThe hyperreactive malarial splenomegaly (HMS) represents a chronic, potentially fatal complication of malaria. Case definition includes: gross splenomegaly, high level of anti-malarial antibody and IgM, response to long-term anti-malarial prophylaxis. In this study, a large series of patients not fully meeting the case definition was tentatively classified as early hyperreactive malarial splenomegaly (e-HMS). The main research questions was: does “e-HMS” tend to evolve to the full-blown syndrome? And if so, what are the main factors influencing this evolution?MethodsRetrospective, longitudinal study. The patient database was searched to retrieve all potentially eligible patients. e-HMS was defined by splenomegaly of any size (with or without raised IgM), high anti-malarial antibody titre and exclusion of other causes of splenomegaly. The clinical outcome at following visits was analysed in relation to re-exposure to malaria, and to treatment (only part of the patients with e-HMS were treated with a single anti-malarial treatment and advised to follow an effective anti-malarial prophylaxis, if re-exposed). The association of the outcome with the main independent variables was first assessed with univariate analysis. A stepwise logistic regression model was then performed to study the association of the outcome with the main independent variables.ResultsOne hundred and twenty-six subjects with e-HMS were retrieved. Eighty-one had at least one follow-up visit. Of 46 re-exposed to malaria for a variable period, 21 (46 %) had progressed, including 10/46 (22 %) evolving to full-blown HMS, while of 29 patients not re-exposed, 24 (93 %) had improved or cured and five (7 %) progressed (p < 0.001). At logistic regression re-exposure was confirmed as a major risk factor of progression (OR 9.458, CI 1.767–50.616) while treatment at initial visit was protective (OR 0.187, CI 0.054–0.650).Conclusione-HMS should be regarded as a clinical condition predisposing to HMS. Although the case definition may include false positives, e-HMS should be treated just as the full-blown syndrome. A single anti-malarial treatment is probably adequate, followed by effective prophylaxis for patients exposed again to malaria transmission.Electronic supplementary materialThe online version of this article (doi:10.1186/s12936-015-1015-6) contains supplementary material, which is available to authorized users.

Neurodevelopmental Animal Models Reveal the Convergent Role of Neurotransmitter Systems, Inflammation, and Oxidative Stress as Biomarkers of Schizophrenia: Implications for Novel Drug Development.

Schizophrenia is a life altering disease with a complex etiology and pathophysiology, and although antipsychotics are valuable in treating the disorder, certain symptoms and/or sufferers remain resistant to treatment. Our poor understanding of the underlying neuropathological mechanisms of schizophrenia hinders the discovery and development of improved pharmacological treatment, so that filling these gaps is of utmost importance for an improved outcome. A vast amount of clinical data has strongly implicated the role of inflammation and oxidative insults in the pathophysiology of schizophrenia. Preclinical studies using animal models are fundamental in our understanding of disease development and pathology as well as the discovery and development of novel treatment options. In particular, social isolation rearing (SIR) and pre- or postnatal inflammation (PPNI) have shown great promise in mimicking the biobehavioral manifestations of schizophrenia. Furthermore, the "dual-hit" hypothesis of schizophrenia states that a first adverse event such as genetic predisposition or a prenatal insult renders an individual susceptible to develop the disease, while a second insult (e.g., postnatal inflammation, environmental adversity, or drug abuse) may be necessary to precipitate the full-blown syndrome. Animal models that emphasize the "dual-hit" hypothesis therefore provide valuable insight into understanding disease progression. In this Review, we will discuss SIR, PPNI, as well as possible "dual-hit" animal models within the context of the redox-immune-inflammatory hypothesis of schizophrenia, correlating such changes with the recognized monoamine and behavioral alterations of schizophrenia. Finally, based on these models, we will review new therapeutic options, especially those targeting immune-inflammatory and redox pathways.

How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia

AbstractDifferentiation syndrome (DS), formerly known as retinoic acid syndrome, is a relatively common and potentially severe complication seen in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and/or arsenic trioxide. The full-blown syndrome consists of unexplained fever, weight gain, dyspnea with pulmonary infiltrates, pleuropericardial effusion, hypotension, and renal failure. Most measures currently used for management of DS have very little evidence-based support, and therefore, many remain controversial. Despite the lack of evidence supporting DS prophylaxis, several groups have adopted a preventive strategy with corticosteroids, especially for patients with leukocyte levels higher than from 5 to 10 × 109/L. DS diagnosis should be suspected in the presence of any of the above-mentioned signs and symptoms, and preemptive treatment with dexamethasone should be started immediately. Other supportive measures can also be crucial for the correct management of DS, especially in those patients with life-threatening complications. Temporary discontinuation of all-trans retinoic acid or arsenic trioxide is indicated only for patients in very poor clinical condition or with severe renal or pulmonary dysfunction, sometimes requiring admission to the intensive care unit. Recognition of specific biomarkers and a better understanding of DS pathogenesis can be helpful for the development of specific therapies to counteract DS in a timely manner.

New Tests to Detect Antiphospholipid Antibodies: Anti-Domain I Beta-2-Glycoprotein-I Antibodies

Beta-2 glycoprotein I (β2GPI) is the main antigenic target for antiphospholipid antibodies (aPL), the serological markers of antiphospholipid syndrome (APS). Domain I (DI) of β2GPI has lately been identified as the main epitope targeted by antibodies reacting against β2GPI. DI is a cryptic epitope, becoming available for autoantibody binding when β2GPI opens from a circular to a fish-hook configuration. Antibodies targeting β2GPI-DI are more frequently detected in patients with a full-blown syndrome than in asymptomatic aPL carriers or in patients with infectious diseases that have reactivity toward the whole molecule. Interestingly, anti-DI antibodies are strongly positively correlated with thrombotic and pregnancy manifestations, enabling identification of patients at higher risk of clinical events. However, available tests to detect anti-DI antibodies still lack standardization. Moreover, some APS patients develop antibodies reacting against β2GPI epitopes other than DI, suggesting that other anti-β2GPI antibody subsets may be clinically relevant. Available evidence on anti-DI antibodies in APS is herein critically reviewed.

Portal Vein Thrombosis in Minimal Change Disease

Among the possible venous thromboembolic events in nephrotic syndrome, renal vein thrombosis and pulmonary embolism are common, while portal vein thrombosis (PVT) is rare. This report describes a 26-year-old man with histologically proven minimal change disease (MCD) complicated by PVT. The patient presented with epigastric pain and edema. He had been diagnosed with MCD five months earlier and achieved complete remission with corticosteroids, which were discontinued one month before the visit. Full-blown relapsing nephrotic syndrome was evident on laboratory and clinical findings, and an abdominal computed tomography revealed PVT. He immediately received immunosuppressants and anticoagulation therapy. An eight-week treatment resulted in complete remission, and a follow-up abdominal ultrasonography showed disappearance of PVT. In conclusion, PVT is rare and may not be easily diagnosed in patients with nephrotic syndrome suffering from abdominal pain. Early recognition of this rare complication and prompt immunosuppression and anticoagulation therapy are encouraged to avoid a fatal outcome. (Ewha Med J 2014;37(2):131-135)

Focus

Focus

Refeeding Syndrome or Refeeding Hypophosphatemia

Nutrition support clinicians refer to the abnormalities in laboratory data and changes in clinical signs and symptoms that follow refeeding of starved or malnourished patients as refeeding syndrome. Theoretical descriptions of refeeding syndrome include a complex and extensive list of changes, such as hypophosphatemia, hypomagnesemia, hypokalemia, hyponatremia, hypocalcemia, hyperglycemia, and vitamin deficiency--all of which are accompanied by clinical signs and symptoms. In practice, clinicians see asymptomatic refeeding hypophosphatemia more often than a full-blown syndrome with multiple laboratory and clinical abnormalities. Confusion results because there is no widely accepted or uniformly applied set of defining characteristics for diagnosing refeeding syndrome. To gain insight into the clinical characteristics of refeeding syndrome described in the literature, a systematic review of reported cases and case series was conducted. Since 2000, 20 authors described 27 cases that contained sufficient data for review. Hypophosphatemia occurred in 26 patients (96%). While 19 patients (71%) experienced at least 1 other laboratory abnormality, only 14 (51%) exhibited a consistent pattern of abnormally low phosphorus and magnesium levels. Seven patients had hypocalcemia (26%), and hyponatremia was reported in 3 patients (11%). There were no reports of hyperglycemia. Mean data reported in case series containing data from 63 patients showed that hypophosphatemia was a consistent finding but that other abnormalities were not consistently identified. Findings suggest that refeeding hypophosphatemia is not accompanied by a consistent pattern of biochemical or clinical abnormalities among case reports or case series of patients reported to have refeeding syndrome.

Analysis of relapses in anti-NMDAR encephalitis

The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis. We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously. A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode. Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.