Risk factors for acute pancreatitis in patients with severe motor and intellectual disabilities

Tamasaki, Akiko; Nishimura, Yoko; Kondo, Noriko; Shirai, Kentaro; Maegaki, Yoshihiro; Ohno, Kousaku

doi:10.1111/ped.12231

Cited by 7 publications

(5 citation statements)

References 11 publications

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“…Patients with multiple profound disabilities have a risk of being subject to systemic complications such as pancreatitis and urinary lithiasis. 8,9 Both acute and chronic pancreatitis are known to result from malnutrition such as anorexia nervosa and malnourishment in children. 10,11 Severe lobular and acinar cell atrophy has been experimentally induced by protein deficiency in bonnet monkeys.…”

Section: Discussionmentioning

confidence: 99%

Autopsy Report of a Woman with Infantile Alexander Disease Who Survived 39 Years

Tsuji

Tanaka

et al. 2020

Neuropediatrics

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Patients with infantile Alexander disease (AxD) usually do not survive beyond their early teens without life support care because of progressive central hypoventilation. We present the autopsy report of a woman with infantile AxD carrying an R239C mutation in the glial fibrillary acidic protein gene, who survived 39 years. She presented with psychomotor retardation in infancy and regressed after age 5. Brain computed tomography scans showed bilateral low frontal white matter density. She became quadriplegic with bulbar palsy and was intellectually handicapped after a measles infection at age 7. Tube feeding was introduced because of dysphagia at age 15. Noninvasive positive pressure ventilation was required due to central hypoventilation in her early thirties. She died of neurogenic respiratory failure at 39 years. Autopsy findings revealed a markedly atrophic brain (709 g, −6.0 standard deviation), especially in the frontal lobe, cerebellum, and brainstem portions. We found demyelination, gliosis, and cystic lesions throughout the brain, and we saw Rosenthal fibers accumulating in the perivascular spaces. We also identified a variety of abnormalities in other organs such as pancreatic necrosis, completely desquamated epithelium in the lower esophagus and stomach, foreign-body giant cells in the colon submucosa, glomerular sclerosis, and multiple bladder stones. This is the first autopsied case report of a patient with infantile AxD with long survival, who showed not only central nervous system characteristic findings, but also unexpected pathological changes in other organs.

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Section: Discussionmentioning

confidence: 99%

Autopsy Report of a Woman with Infantile Alexander Disease Who Survived 39 Years

Tsuji

Tanaka

et al. 2020

Neuropediatrics

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“…In terms of other aspects specific to SMID, a suboptimal nutritional status may result in lower serum albumin level, which in turn increases the free fraction of AEDs in the blood, leading to greater augmentation of toxicity than that expected from measured blood levels [5]. Hypoalbuminemia may also be linked to a high-er risk of acute pancreatitis in SMID, which may enhance the patient's' vulnerability to VPA-induced pancreatitis [6]. A higher incidence of VPA-induced Fanconi syndrome has been recognized in patients with SMID, which may be due to subclinical damage to the kidney caused by hypoxic-ischemic insults during perinatal asphyxia, a common etiology of brain lesions in these patients [7].…”

Section: Discussionmentioning

confidence: 99%

Adverse effects of antiepileptics potentially serious in individuals with severe motor and intellectual disabilities

Saito¹,

Hirabayashi²,

Kita³

et al. 2020

E&S

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To descr ibe the potentially ser ious adver se effects of antiepileptic dr ugs (AEDs) in individuals with severe motor and intellectual disabilities (SMID) and to improve the quality of drug treatment for these patients. Methods: The data of 99 SMID patients with epilepsy staying at an institute for mor e than 1 year were retrospectively reviewed. Each patient was taking 3.4 ± 1.6 (range, 1-8) kinds of AEDs, and clinical seizures were still prevalent in 68 patients. Results: Discontinuation or dose r eduction of AEDs amelior ated agitation and/or sleep disturbance, somnolence, vomiting, appetite loss, and stridor in the corresponding patients, and additionally resolved serious gastrointestinal or cardiorespiratory problems in three patients whose clinical courses were detailed. Patient 1 manifested aerophagia-related chronic distension of the abdomen causing mechanical ileus, requiring repeated intestinal resection. The abdominal distension improved significantly after discontinuation of acetazolamide. Patient 2 experienced progressive motor deterioration during young adulthood, with simultaneous sudden elevation in blood phenytoin level. Although motor disability persisted, resolution of hypoventilation was noted after cessation of phenytoin. Patients 3 had long-term use of carbamazepine and exhibited episodic bradycardia (heart rate as low as 25 bpm) that resolved after drug discontinuation. Conclusions: Patients with SMID sometimes fail to effectively ver balize subjective adver se effects of medications to healthcare providers. The chronic adverse effects of AEDs, including those described in this study, should be broadly evaluated for better patient management.

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“…In patients with cholecystitis, cholangitis or pancreatitis, laboratory data were collected at least 1 month before the onset of infection, when the condition of patients was generally good. These infections included cholecystitis, cholangitis, pancreatitis, and other infectious diseases 9 …”

Section: Methodsmentioning

confidence: 99%

“…These infections included cholecystitis, cholangitis, pancreatitis, and other infectious diseases. 9…”

Section: Data Collectionmentioning

confidence: 99%

Gallstones in patients with severe motor and intellectual disability

Zuo

Sawai

Kanehiro

et al. 2022

Pediatrics International

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Background: Asymptomatic gallbladder stones may be detected with ultrasound; some gallstones produce symptomatic diseases, such as cholecystitis, cholangitis, or pancreatitis. Identifying the clinical features of symptomatic gallstones may help prevent severe complications by providing intervention for asymptomatic gallstones. We aimed to investigate risk factors associated with developing symptomatic disease in pediatric patients with severe motor and intellectual disabilities (SMID). Methods: This retrospective study enrolled 30 patients with SMID who were treated at the Nara Medical University between March 2016 and March 2019. We examined the prevalence of gallstones, and the rate at which associated symptoms of gallstones were observed in patients. Furthermore, we compared the clinical features of patients with and without gallstones. Results: Among 30 patients with SMID, 7 (23%) had gallstones, with 6 (86%) of them being symptomatic: 3 patients had acute pancreatitis and 3 had cholecystitis or cholangitis. Among 23 patients without gallstones, 2 had acute pancreatitis and 2 had biliary sludge. Patients with gallstones had significantly lower daily calorie intake than those without gallstones (P = 0.042). Furthermore, the incidence of gallstones was higher in patients who received total parenteral nutrition than in those who did not (P = 0.031). Comparative analysis between symptomatic and asymptomatic patients was not performed because almost all cases were symptomatic. Conclusion: Gallstones were detected in 23% of patients with SMID. The gallstones were symptomatic at a very high rate. Considering this fact, patients with SMID should be surveyed for gallstones; careful management may be needed in such patients.

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Risk factors for acute pancreatitis in patients with severe motor and intellectual disabilities

Cited by 7 publications

References 11 publications

Autopsy Report of a Woman with Infantile Alexander Disease Who Survived 39 Years

Autopsy Report of a Woman with Infantile Alexander Disease Who Survived 39 Years

Adverse effects of antiepileptics potentially serious in individuals with severe motor and intellectual disabilities

Gallstones in patients with severe motor and intellectual disability

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