Pregnancy in Women With Cerebral Palsy.

Cerebral palsy is a permanent, non-progressive, irreversible, non-curable condition with high co-morbidities and lifelong complications. Brain lesions may be present at birth or shortly after that. It may be congenital or acquired, prenatal, or abnormal brain development. The damage to the brain is non-progressive. It mainly affects movement, coordination, strength, and posture. Cerebral palsy is believed to increase women's chance of unfavorable pregnancy outcomes. According to studies, the main outcome of cerebral palsy in pregnant women is premature birth. Secondary outcomes like LSCS, labor induction, low 5-minute APGAR, small for gestational age (SGA), large for gestational age (LGA), and stillbirth point to the necessity for increased surveillance during prenatal treatment. A 27-year-old primigravida with a known case of dystonic Cerebral palsy since childhood presented with a history of nine months of amenorrhea, pain in the abdomen, and backache for one day. Per abdominal examination, the uterus was 34 weeks in size with Breech presentation, mild contractions were present, and a fetal heart rate of 146 beats per minute, which was regular. On per-vaginal examination cervical os was one finger loose, the show was present. The patient underwent a planned Lower segment caesarean section after neuro physician and anesthesiologist clearance and delivered a healthy female baby of 2.4 kg. Both mother and baby were stable.

Severe refeeding syndrome after starvation ketoacidosis requiring stopping feeds.

INTRODUCTION: Starvation ketoacidosis (SKA) is a rare cause of ketoacidosis in the general population but can be seen with malignancy. Patients often respond well to treatment, but some rarely develop refeeding syndrome (RFS) as their electrolytes drop to dangerous levels causing organ failure. Typically, RFS can be managed with low-calorie feeds, but sometimes patients require a halt in feeds until their electrolyte imbalances are managed. CASE REPORT: We discuss a woman with synovial sarcoma on chemotherapy who was diagnosed with SKA and then developed severe RFS after treatment with intravenous dextrose. Phosphorus, potassium, and magnesium levels dropped precipitously and remained fluctuant for 6 days. She also developed normal sinus ventricular tachycardia, premature ventricular beats, and bigeminy. She could not tolerate calorie supplementation at that time. She was managed with electrolyte repletions until clinically stable and then progressed to a liquid diet. DISCUSSION: We present a unique case of severe SKA that resulted in RFS requiring nihil per orem (NPO) treatment for 6 days. There are no specific guidelines for SKA or RFS management. Patients with pH < 7.3 may benefit from baseline serum phosphorus, potassium, and magnesium levels. Clinical trials are needed to further study which patients may benefit from starting at a low-calorie intake versus those that require holding nutrition until clinically stable. CONCLUSION: Completely stopping caloric intake until a patient's electrolyte imbalance improves is an important management aspect of RFS to underscore and study, as grave complications can occur even with cautious refeeding regimens.

A Case of Severe Metabolic Acidosis in the Setting of a Strict Ketogenic Diet.

Patients with metabolic acidosis often present with obscure, multifactorial etiologies, making efficient diagnosis and treatment key to preventing poor clinical outcomes. This case report describes a patient with severe metabolic acidosis in which the underlying cause was not immediately apparent. After a thorough work-up and history taking, the patient's strict ketogenic diet was identified as the most likely source of his illness. Over multiple days, the patient improved as he resumed a normal diet and was treated for refeeding syndrome. This case highlights the importance of taking a comprehensive social and diet history when assessing a patient with metabolic acidosis. It also highlights the need for physicians to understand and be ready to counsel on the possible effects of fad diets, such as the ketogenic diet.

Non-diabetic Euglycemic Ketoacidosis in an Adult Patient With Spinal Muscular Atrophy Type II.

Spinal muscular atrophy (SMA) is a rare neuromuscular disease that develops as a result of the degeneration of the anterior horn cells in the spinal cord and lower brainstem motor nuclei, resulting in progressive muscle weakness and atrophy. While the initial presentation of this disease involves diffuse muscular atrophy at an early age, patients with an established diagnosis and later-stage disease often present with gastrointestinal symptoms related to metabolic imbalances. Here, we examine the case of an adult patient with SMA type II who presented with complaints of 12 hours of intractable nausea and vomiting. The patient was found to be in euglycemic ketoacidosis (EKA), an uncommon, but not unheard of, metabolic derangement in SMA patients with severely decreased muscle mass.

Starvation Ketoacidosis With Hypoglycemia in a Patient With Chronic Pancreatitis.

Chronic pancreatitis is a pancreatic inflammation that can result in endocrine pancreatic insufficiency. We present a case of starvation ketoacidosis in a 44-year-old Japanese man with chronic alcoholic pancreatitis. On admission, he exhibited hypoglycemia and severe acidosis. Intravenous glucose and vitamin B1 were administered in the emergency department, and nutritional management for presumed starvation ketoacidosis was begun. Because the patient did not have diabetes mellitus, his insulin secretion and insulin resistance were examined. A diagnosis of pancreatic diabetes caused by chronic pancreatitis was made based on decreased insulin secretion, normal insulin resistance, and negative anti-glutamic acid decarboxylase antibody. Intensive insulin therapy was initiated, and he was discharged 15 days after hospitalization. Although starvation rarely causes hypoglycemia and severe ketoacidosis, they can be induced by short-term fasting in patients with decreased pancreatic function.

Starvation Ketoacidosis Induced by Ketogenic Diet and Consumption of Ketone Supplement.

The ever-changing landscape of dieting and its correlation with health outcomes have continued to evolve with time. New diets appear and disappear just as quickly as they gain notoriety. This is a rare case of a 67-year-old female with a history of type II diabetes who presented with generalized weakness, nausea, and vomiting, and was found to have severe anion gap metabolic acidosis. In an effort to lose weight, she was combining a ketogenic diet with prolonged fasting and exogenous ketone supplement use that she purchased online. The patient reported drinking an exogenous ketone ester supplement that contained 30 g of D-beta hydroxybutyrate (BHB) per serving, three times per day. This case is unique in that the patient was initially thought to be in diabetic ketoacidosis upon arrival, but after further investigation into her initial labs, medication, and social history, the underlying factor for hospitalization became evident; that is, a combination of a ketogenic diet with prolonged fasting and exogenous BHB-induced ketoacidosis in the setting of type II diabetes. Thus, this case highlights the importance of thorough history taking, the dangers of over-the-counter supplement consumption, and the risks consumers inherit with trend dieting.

Characterization and outcome of 11 children with non-diabetic ketoacidosis.

OBJECTIVES: To study the clinical and laboratory features, management, and outcome of pediatric non-diabetic ketoacidosis (NDKA). METHODS: Between May 2018 and April 2020, we prospectively collected children under 18 years who presented with ketoacidosis, defined as ketosis (urinary ketones ≥++ and/or serum ß-hydroxybutyrate level ≥3 mmol/L) and metabolic acidosis (pH <7.3 and HCO3 - <15 mmol/L). Children with HbA1c level ≥6.5% at initial presentation and those meeting the diagnostic criteria for DM during follow-up were excluded. Data were collected on demographics, clinical and laboratory features, management, and outcome. RESULTS: Eleven children with 19 episodes of NDKA were identified. The median age was 12 months (range from 5 months to 5 years). They manifested dehydration and disturbed conscious level (all cases), convulsions (n=6), hypoglycemia (n=6), hyperglycemia (n=2) and significant hyperammonemia (n=4). Most cases required intensive care management. Death or neurodevelopmental impairment occurred in six cases. Seven cases had inborn errors of metabolism (IEMs). Other cases were attributed to starvation, sepsis, and salicylate intoxication. CONCLUSIONS: This is the largest case series of pediatric NDKA. Ketoacidosis, even with hyperglycemia, is not always secondary to diabetes mellitus. IEMs may constitute a significant portion of pediatric NDKA. Increased awareness of this unfamiliar condition is important for prompt diagnosis, timely management, and better outcome.

Starvation ketoacidosis on the acute medical take.

Starvation ketoacidosis (SKA) represents one of three metabolic acidoses caused by the accumulation of ketone bodies within the bloodstream. While easily treated, it is a diagnosis that can be easily missed in patients with an unexplained metabolic acidosis.In this case report, we discuss two patients presenting with a starvation ketoacidosis and psychiatric illness. This link between SKA and psychiatric disease is especially pertinent as this is a cohort of patients who may not be able to give an accurate history, which can potentially lead to a delay in diagnosis and treatment.Furthermore, the patient cohort described here have higher rates of alcohol dependence and are therefore at risk of alcoholic ketoacidosis. It is important to recognise that these conditions may coexist and should be managed as such, with thiamine prior to carbohydrate replacement in all at-risk patients.

Review article: Ketoacidosis in the emergency department.

Diabetic ketoacidosis, a life-threatening complication of type 1 diabetes mellitus, is a common cause of presentation to EDs. Two new drug classes have been found to cause ketoacidosis with distinctive presentations. The sodium-glucose transport protein 2 inhibitors used in the management of type 2 diabetes mellitus may present with ketoacidosis with normal glucose levels. Ketoacidosis with these medications may be prolonged and recur after initial resolution. Checkpoint inhibitors may present with fulminant diabetic ketoacidosis in individuals with previously normal glucose tolerance. Ketoacidosis may also occur as a result of starvation and alcohol excess, as well as a number of rare causes. Other causes of metabolic acidosis with both high and normal anion gap need to be considered in the differential diagnosis of ketoacidosis. Diabetic ketoacidosis may also present with biochemical changes suggestive of myocardial ischaemia and pancreatitis in the absence of these pathologies. The present paper reviews ketone body metabolism, ketone testing and the causes and differential diagnosis of ketoacidosis with particular relevance to emergency medicine.

Basal Vacuolization in Renal Tubular Epithelial Cells at Autopsy and Their Relation to Ketoacidosis.

Basal vacuolization of renal tubular epithelial cells is a useful postmortem marker for ketoacidosis. To investigate its incidence and relationship to the severity of ketoacidosis, 158 autopsy cases with elevated ß-hydroxybutyrate (>1 mmol/L) over a 7-year-period were retrospectively reviewed. Sixty-eight cases (43%) exhibited basal vacuolizations (vitreous ß-hydroxybutyrate: 1.16-29.35 mmol/L, mean 10.28 mmol/L), and 90 cases (57%) did not (vitreous ß-hydroxybutyrate: 1.03-13.7 mmol/L, mean 2.84 mmol/L). Quantitative analysis revealed on average a fourfold elevation in ß-hydroxybutyrate in cases with basal vacuolizations compared to those without; 10.3% of cases with ß-hydroxybutyrate concentrations between 1.01 and 2.00 mmol/L had basal vacuolizations, and this incidence increased to 33.3% with concentrations between 4.01 and 6.00 mmol/L. A marked increase in incidence to >70% was observed with concentrations >6.00 mmol/L, and basal vacuoles were invariably present (100%) with concentrations >14.01 mmol/L. This study demonstrates that basal vacuolizations are a sensitive marker for significant ketoacidosis and reaffirms its use as an indicator for likely cases of fatal ketoacidosis at autopsy.

Life-threatening ketoacidosis in a pregnant woman with psychotic disorder.

Pregnancy is an insulin resistant state. Hyperglycaemia and gestational diabetes mellitus are well-recognised complications even in women without existing metabolic syndrome or obesity. Pregnant women also appear to be more vulnerable to ketoacidosis, particularly after short periods of reduced oral intake in the third trimester, and may present with very severe starvation ketoacidosis, prompting emergent delivery. We present a case of a woman with a background of depression and psychotic episodes. Olanzapine had been commenced after a psychotic episode at 20 weeks' gestation. Gestational diabetes mellitus was diagnosed at 28 weeks, and she was then admitted at 31 weeks with severe euglycaemic ketoacidosis following a short period of vomiting. She underwent caesarean section when the metabolic disturbances did not resolve with medical treatment. We believe atypical antipsychotic therapy contributed to the profound insulin resistance seen here, and that obstetricians, physicians and psychiatrists must be aware of the risks conferred by these agents in pregnancy.